IMMUNEIMMUNE GGLOBULINLOBULIN CCOMMUNITYOMMUNITY wwww.IGLiving.comww.IGLiving.com AApril-Maypril-May 2001212

Prescribing the Right Amount

How to Diagnose Infants with PIDD

Understanding & Treating CVID

Defending Kids Against Bullying

Diagnosing Specific Antibody Deficiency

E NTER IG L IVING ’S E SSAY C ONTEST ! — P AGE 11 • Thrombotic events have occurred in patients receiving IGIV GAMUNEX®-C therapy. Monitor patients with known risk factors for thrombotic Immune Globulin Injection (Human) 10% events; consider baseline assessment of blood viscosity for those at risk of hyperviscosity. Caprylate/Chromatography Purified • Aseptic Meningitis Syndrome (AMS) has been reported with GAMUNEX-C and other IGIV treatments, especially with high HIGHLIGHTS OF PRESCRIBING INFORMATION doses or rapid infusion. These highlights do not include all the information needed to • Hemolytic anemia can develop subsequent to IGIV therapy due use GAMUNEX®-C safely and effectively. See full prescribing to enhanced RBC sequestration. Monitor patients for hemolysis information for GAMUNEX-C. and hemolytic anemia. GAMUNEX-C, [Immune Globulin Injection (Human) 10% Caprylate/Chromatography Purified] • Monitor patients for pulmonary adverse reactions (transfusion- related acute lung injury [TRALI]). Initial U.S. Approval: 2003 • Volume overload

WARNING: ACUTE RENAL DYSFUNCTION and FAILURE • GAMUNEX-C is made from human plasma and may contain infectious agents, e.g., viruses and, theoretically, the See full prescribing information Creutzfeldt-Jakob disease agent. for complete boxed warning. • Passive transfer of antibodies may confound serologic testing. • Renal dysfunction, acute renal failure, osmotic nephrosis, and death may occur with immune globulin ------ADVERSE REACTIONS------intravenous (IGIV) products in predisposed patients. • Renal dysfunction and acute renal failure occur more • PI – The most common adverse reactions (Ն5%) with commonly in patients receiving IGIV products containing intravenous use of GAMUNEX-C were headache, cough, sucrose. GAMUNEX-C does not contain sucrose. injection site reaction, nausea, pharyngitis and urticaria. The most common adverse reactions (Ն5%) with subcutaneous • For patients at risk of renal dysfunction or failure, use of GAMUNEX-C were infusion site reactions, headache, administer GAMUNEX-C at the minimum concentration fatigue, arthralgia and pyrexia. available and the minimum infusion rate practicable. • ITP – The most common adverse reactions during clinical trials (reported in Ն5% of subjects) were headache, vomiting, fever, ------INDICATIONS AND USAGE------nausea, back pain and rash. GAMUNEX-C is an immune globulin injection (human) 10% liquid • CIDP – The most common adverse reactions during clinical indicated for treatment of: trials (reported in Ն5% of subjects) were headache, fever, • Primary Humoral Immunodeficiency (PI) chills, hypertension, rash, nausea and asthenia. • Idiopathic Thrombocytopenic Purpura (ITP) To report SUSPECTED ADVERSE REACTIONS, contact Talecris • Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Biotherapeutics, Inc. at 1-800-520-2807 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch. ------CONTRAINDICATIONS------DRUG INTERACTIONS------• Anaphylactic or severe systemic reactions to human immunoglobulin • The passive transfer of antibodies may transiently interfere with the response to live viral vaccines, such as measles, mumps • IgA deficient patients with antibodies against IgA and a history of hypersensitivity and rubella. Passive transfer of antibodies may confound serologic testing. ------WARNINGS AND PRECAUTIONS------USE IN SPECIFIC POPULATIONS ------• IgA deficient patients with antibodies against IgA are at greater risk of developing severe hypersensitivity and anaphylactic • Pregnancy: no human or animal data. Use only if clearly reactions. Have epinephrine available immediately to treat any needed. acute severe hypersensitivity reactions. • Geriatric: In patients over 65 years of age do not exceed the • Monitor renal function, including blood urea nitrogen, serum recommended dose, and infuse GAMUNEX-C at the minimum creatinine, and urine output in patients at risk of developing infusion rate practicable. acute renal failure. • GAMUNEX-C is not approved for subcutaneous use in ITP patients. Due to a potential risk of hematoma formation, do not administer GAMUNEX-C subcutaneously in patients with ITP. • Hyperproteinemia, with resultant changes in serum viscosity Talecris Biotherapeutics, Inc. and electrolyte imbalances may occur in patients receiving IGIV Research Triangle Park, NC 27709 USA 08939771/08939782-BS therapy. U.S. License No. 1716 Revised: October 2010

The PROOF is everywhere you look GAMUNEX-C has proven effi cacy and patient outcomes in CIDP, PI, and ITP*1

Important Safety Information for GAMUNEX-C Gamunex-C, Immune Globulin Injection (Human), 10% Caprylate/Chromatography Purifi ed, is indicated for the treatment of primary humoral immunodefi ciency disease (PI), idiopathic thrombocytopenic purpura (ITP), and chronic infl ammatory demyelinating polyneuropathy (CIDP). Renal dysfunction, acute renal failure, osmotic nephrosis, and death may occur with immune globulin intravenous (IGIV) products in predisposed patients. Patients predisposed to renal dysfunction include those with any degree of pre-existing renal insuffi ciency, diabetes mellitus, age greater than 65, volume depletion, sepsis, paraproteinemia, or patients receiving known nephrotoxic drugs. Renal dysfunction and acute renal failure occur more commonly in patients receiving IGIV products containing sucrose. Gamunex-C does not contain sucrose. For patients at risk of renal dysfunction or failure, administer Gamunex-C at the minimum concentration available and the minimum infusion rate practicable. Gamunex-C is contraindicated in individuals with acute severe hypersensitivity reactions to Immune Globulin (Human). It is contraindicated in IgA defi cient patients with antibodies against IgA and history of hypersensitivity. Gamunex-C is not approved for subcutaneous use in patients with ITP or CIDP. Due to the potential risk of hematoma formation, Gamunex-C should not be administered subcutaneously in patients with ITP. Hyperproteinemia, increased serum viscosity, and hyponatremia may occur in patients receiving IGIV therapy. Thrombotic events have been reported in association with IGIV. Patients at risk for thrombotic events may include those with a history of atherosclerosis, multiple cardiovascular risk factors, advanced age, impaired cardiac output, coagulation disorders, prolonged periods of immobilization and/or known or suspected hyperviscosity. There have been reports of noncardiogenic pulmonary edema [Transfusion-Related Lung Injury (TRALI)], hemolytic anemia, and aseptic meningitis in patients administered with IGIV. The high dose regimen (1g/kg x 1-2 days) is not recommended for individuals with expanded fl uid volumes or where fl uid volume may be a concern. Gamunex-C is made from human plasma. Because this product is made from human plasma, it may carry a risk of transmitting infectious agents, e.g., viruses, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent. After infusion of IgG, the transitory rise of the various passively transferred antibodies in the patient’s blood may yield positive serological testing results, with the potential for misleading interpretation. In clinical studies, the most common adverse reactions with Gamunex-C were headache, fever, chills, hypertension, rash, nausea, and asthenia (in CIDP); headache, cough, injection site reaction, nausea, pharyngitis, and urticaria with intravenous use (in PI) and infusion site reactions, headache, fatigue, arthralgia and pyrexia with subcutaneous use (in PI); and headache, vomiting, fever, nausea, back pain, and rash (in ITP). The most serious adverse reactions in clinical studies were pulmonary embolism (PE) in one subject with a history of PE (in CIDP), an exacerbation of autoimmune pure red cell aplasia in one subject (in PI), and myocarditis in one subject that occurred 50 days post-study drug infusion and was not considered drug related (in ITP). * CIDP=Chronic infl ammatory demyelinating polyneuropathy; PI=Primary immunodefi ciency; ITP=Idiopathic thrombocytopenic purpura. Reference: 1. Data on fi le, Grifols. You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088. Please see adjacent page for brief summary of GAMUNEX-C full Prescribing Information.

To get GAMUNEX-C call 1-888-MY GAMUNEX (694-2686) USA Customer Service: 1-800-243-4153 Evidence based. Patient proven. www.gamunex-c.com © 2011 Grifols Therapeutics Inc. All rights reserved. November 2011 GX175-1111

AApril-Maypril-May 2001212 Contents Up Front Features 5 Contributing Experts Writers in this issue How Much Exercise Is 16 Too Much or Too Little? 6 Editorial It’s Tough Being a Kid By Matthew Hansen, with a Chronic Illness DPT, MPT, BSPTS By Ronale Tucker Rhodes, MS 7 Celebrating New Opportunities Diagnosing PIDD By Kris McFalls 20 in Infants Did You Know? By Melvin Berger, MD 8 Immunology 101 Understanding and Diagnosing Specific Antibody 26 Treating Common Variable Deficiency: Interpreting Pre- and Immunodeficiency Post-Immunization Pneumococcal Vaccine Responses By Ronale Tucker Rhodes, MS 11 Industry News Research, science, product and insurance Sources updates 45 Book Corner New and Useful Reading Lifestyle 46 Resource Center Community foundations, 32 Let’s Talk! associations, forums By Trudie Mitschang and other resources 34 Ask Kris By Kris McFalls Kaaa-Booms! About IG Living 36 IG Living is the only magazine dedicated to bringing comprehensive healthcare information, immune globulin information, By Cheryl L. Haggard community and reimbursement news, and resources for successful living directly to immune globulin consumers and their healthcare providers. 38 SCIG Game Plan IG Living , (ISSN 1949-4548), published bimonthly, is a community service provided by FFF Enterprises, 4 1093 County By Ever Fecske Center Drive, Temecula, CA 92591, (800) 843-7477 x 1362, fax (951) 699-9655. Subscriptions to IG Living are free, and readers may subscribe at www.IGLiving.com or by calling (800) 843-7477 x1351. Transitions: 39 The opinions expressed in IG Living are those of the authors alone and do not represent the opinions, policies or positions Crossing the Pond of FFF Enterprises, the Board of Directors, the IG Living Advisory Board or editorial staff. This material is provided for general information only. FFF Enterprises does not give medical advice or engage in the practice of medicine. FFF for Better Health Enterprises under no circumstances recommends any particular treatment for any individual and in all cases recommends By Carla Schick that individuals consult with a physician before pursuing any course of treatment. All manuscripts should be submitted in MS Word, in Arial font. Manuscripts should be between 650 and 1,300 words Parenting: in length, with unjustified margins and without any other formatting. Submission guidelines are available for download 42 from the Contact Us page on www.IGLiving.com. Email manuscripts to [email protected]. IG Living retains the right to Defending Kids edit submissions. The contents of each submission and their accuracy are the responsibility of the author(s) and must be original work that has not been, nor will be, published elsewhere, without the written permission of IG Living . A copyright Against Bullying agreement attesting to this and transferring copyright to FFF Enterprises will be required. Acceptance of advertising By Mark T. Haggard for products and services in IG Living in no way constitutes endorsement by FFF Enterprises. ©2012 FFF Enterprises Inc. Advertising in IG Living IG Living Magazine is read by 30,000 subscribers who are patients who depend upon immune globulin products and their healthcare providers. For information about advertising in IG Living , download a media kit at www.igliving.com/ Advertise.aspx. Or contact Cheryl Brooks at (800) 843-7477 x1177 or [email protected].

4 April-May 2012 www.IGLiving.com IG Living! Contri buti ngExperts

MELVIN BERGER, MD KRIS MCFALLS Senior Medical Director for Patient Advocate, IG Living magazine Immunology Research, CSL Behring

Diagnosing PIDD in Infants Medicare and IG “With modern diagnostic testing, a definitive diagnosis “Both intravenous IG (IVIG) and subcutaneous IG (SCIG) can be made in most cases of PIDD, and the precise therapy are covered under Medicare Part B.” molecular defect often can be identified .” Simple But Effective Home Exercise Equipment MARK T. HAGGARD “While there may not be a cure for most chronic illnesses, High School Teacher, Football Coach regular exercise has been proved to decrease pain, increase and Parent of PIDD Children stamina and improve overall health.”

Defending Kids Against Bullying “Kids who show outward signs of illness make them MATTHEW HANSEN, DPT, MPT, BSPTS a target of opportunity. ” Physical Therapist How Much Exercise Is Too Much or Too Little? TERRY O. HARVILLE, MD, PHD “The easiest formula for finding the right amount of Medical Director, Special Immunology exercise is for a patient to take note of how they feel Laboratory, University of Arkansas for afterward. ” Medical Sciences Diagnosing Specific Antibody Deficiency: Interpreting Pre-/Post-Immunization Pneumococcal Vaccine Responses “Whether a patient makes specific antibodies can be determined by performing pre-/post-immunization antibody titer analyses to pneumococcal vaccine .”

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Sign up for the Teleforums now by emailing [email protected] or calling (800) 843-7477, ext. 1362.

April-May 2012 www.IGLiving.com IG Living! 5 Editorial

Our mission is to support the IG community through education, It’s Tough Being a Kid communication and advocacy with a Chronic Illness A community service from FFF Enterprises, Inc. Being a kid is tough. Being a kid with a With all the added challenges of chronic chronic illness is tougher. Just ask those illness, it takes courage and mental tough - Advisory Board kids, their parents, the doctors and the ness to grow up as a sick kid. Those Nancy Creadon, RN Vice President advocates on their behalf who diagnose, attrib utes aptly describe 20-year-old Sarah VaxAmerica treat and support them. In this issue of IG Eames, who was diagnosed at age 15 with Erika Lawrence, PhD Living , we look at the topic of chronic myasthenia gravis. Our interview with Associate Professor illness in children from all perspectives. Sarah in our Let’s Talk column reveals that Department of Psychology In our feature article, Diagnosing PIDD for her the most difficult part of being a University of Iowa in Infants, Dr. Melvin Berger, a specialist in teen with a chronic illness was coming to Todd Levine, MD pediatric immunology, shares his vast terms with it herself and not letting it Director, Department of Neurophysiology Good Samaritan Hospital, Phoenix knowledge about the various forms of define her. Now, her experience has led to pri mary immunodeficiency diseases, their her desire to work with kids, and her Assistant Professor of Clinical Neurology University of Arizona symptoms, the stages of diagnostic evalu - advice to other children who are chronic- Fred Modell ation and the urgent need for greater ally ill is to “ keep going forward even Co-founder of the awareness about these diseases. Dr. when the going gets tough.” Jeffrey Modell Foundation Berger is one of the few physicians in this Thankfully, there is a lot of support for Marc Riedl, MD, MS specialized area who can answer the children with chronic illness and their par - Assistant Professor often-asked questions: “Why does it take ents to make life a little less tough. Due to Department of Medicine so long to diagnose an immune deficiency efforts by physicians like Dr. Berger, organi - Clinical Immunology and Allergy UCLA, David Geffen School of Medicine in children?” “What are the telltale signs zations that devote effort to raising the and tests to recognize and confirm an awareness of immune deficiency diseases , Publisher Patrick M. Schmidt immune deficiency in kids?” And, “What and advances in diagnostic testing, these Editor Ronale Tucker Rhodes, MS needs to be done to increase awareness diseases are becoming more recognizable, Assistant Editor Cheryl Brooks Creative Director Sheryl Perez among medical professionals about and many more children are getting the IG Artistic Director Allan Bean immune deficiencies to help children get treatment they so desperately need. Graphic Artists Allan Bean diagnosed sooner and allow them to lead Support for kids such as Sarah and their Ben Drolet healthy, productive lives?” parents also comes from patient advocates. Proofreader Jackie Logue Diagnosis is key to helping kids with IG Living is proud to offer patients every - Contributing Writers chronic illness, but there are other con - where access to a patient advocate, and Melvin Berger, MD cerns. Oftentimes, having a chronic illness we have been very blessed to have had Kris Ever Fecske can exacerbate issues that all kids may McFalls on our team for the past seven Cheryl L. Haggard face such as bullying, a problem that years providing advice and guidance that Mark T. Haggard affects some 2.7 million students nation - has helped so many of our readers. As Kris Matthew D. Hansen, DPT, MPT, BSPTS Terry O. Harville, MD, PhD wide. As Mark Haggard, a father of two explains on the adjoining page, she has Kris McFalls children with common variable immune accepted a new challenge. It is with heartfelt Trudie Mitschang deficiency, explains in his article, gratitude for her work with us and for all she Carla Schick Defending Kids Against Bullying, chroni - has done and continues to do for the patient cally ill children often show outward signs community that we wish Kris all the best, and of illness, which makes them “a target of we support her in her new endeavors. opportunity” for bullies. Mark discusses why kids bully, and he provides some ©2012 FFF Enterprises Inc. All rights reserved. guidance for kids and their parents about Please direct editorial, advertising and marketing communications to how to respond. Ronale Tucker Rhodes, MS, Editor 41093 County Center Drive Temecula, CA 92591 Ph: (800) 843-7477 Email: [email protected] www.IGLiving.com UpFro nt

Celebrating New Opportunities

It has been nearly seven years since I began my position stewards of the rare disease community very seriously. with IG Living ’s founder and publisher, FFF Enterprises. Therefore, positive, forward change will continue to happen And, I remember feeling then what I still feel now: It was at IG Living , and you can be part of it. I encourage all of you a wonderful opportunity full of challenge and promise. to keep telling your story, continue to comment on the arti - Not only have I had the chance to work for a company cles, and continue to make suggestions for new articles. like FFF Enterprises, which has integrity and a patient- Your needs can only be met if you let them be known! first philosophy I have long admired, I have had the I would like to thank my sons, Konner and Keegan, for chance to connect with all of you — patients within the allowing me to make their lives so public. It is only through immune globulin (IG) community — through IG Living sharing our trials that others can understand them. And I magazine. It has been an honor and a privilege to serve am grateful they allowed me to share some of our experi - as a writer for IG Living , as well as a patient advocate for ences with you. Additionally, I would like to thank the IG many of you. I take pride in the amazing work that has Living staff for their wonderful support and for making me been done to serve the IG community. That is why it is look like a bona fide writer. Many on our staff go unrec - with mixed emotions that I am announcing I have once ognized and unnoticed, but I can promise you, they all again been blessed with a wonderful new opportunity. I have accepted the position of manager of reimburse - ment with CSL Behring, and this will be my last issue as My colleagues here at a member of the IG Living staff. IG Living celebrate with me as I The funny thing about opportunities is that sometimes when you least expect them, they find you. And I assure all of embark on this new journey, and you that is the case for me. My colleagues here at IG Living celebrate with me as I embark on this new journey, and while while I won’t be as visible, I will I won’t be as visible, I will still be very much involved with this community. I can tell you, without a doubt, that the work is still be very much involved far from done, and I will always be a part of it in some way. Winston Churchill was quoted as saying: “There is noth - with this community. ing wrong with change, if it is in the right direction.” I believe at IG Living we have made postive changes in the care as much as I do. I must give a special thanks to Patrick right direction, always with an eye toward our mission to Schmidt, FFF’s CEO, who has had the vision, caring and support the IG community through education, communi - determination to invest in this community and to back a cation and advocacy. For instance, our social media outreach publication that is patient-focused. Thanks also go out to has allowed readers from all over the globe to connect the IG Living advertisers who continue to believe in and with one another. Many of our articles are now being support this publication and our community. Without this writ ten by experts in the fields of immunology, rheuma - support, IG Living would not be able to continue. Last, I tol ogy and neurology. And, in 2011, we launched the first would like to thank all of you, our readers, for allowing me of what we plan to be an annual writing contest through the honor of being a small part of your lives. I encourage which several of our readers shared the intimate details of you to celebrate your opportunities, rise to your challenges their personal battles with chronic illness. and embrace them all with passion. Know that, as I step I have enjoyed reading your letters, emails, Facebook into my new professional role, my personal role as part of comments, as well as comments submitted with your sub - this wonderful community and as a Facebook fan and avid scription requests. And I can promise you that I have person - IG Living reader will continue. ally read every single one of them, as have many others on our IG Living staff. We take the responsibility to be good Kris McFalls, IG Living’s Patient Advocate

April-May 2012 www.IGLiving.com IG Living! 7 Did You Know

Immunology 101: Diagnosing Specific Antibody Deficiency: Interpreting Pre- and Post-Immunization Pneumococcal Vaccine Responses By Terry O. Harville, MD, PhD

AS WE DISCUSSED in previous columns, an antibody This grew to seven, 10, 12, deficiency disorder can be diagnosed in two ways: 1) defi - 14, 15 and, now, to the full cient serum IgG levels, usually accompanied by deficient 23 serotypes. Many studies IgA and/or IgM levels, in a patient who also fails to make comparing the assay regard - appropriate specific antibodies (such as in X-linked agam - ing the potential for diag nos - maglobulinemia) or 2) normal IgG, IgA and IgM serum ing an antibody deficiency lev els in a patient who fails to make specific antibodies. have looked at the responses Whether a patient makes specific antibodies can be of 10 to 15 serotypes. Based determined by performing pre- and post-immunization on these studies, a consensus antibody titer analyses to pneumococcal vaccine. For has developed concerning instance, a poor response to pneumococcal vaccination, the interpretation of the results. In general, a titer value whether a patient has low IgG (and/or IgA and IgM) levels exceeding 1.3 mcg/mL is defined as protection against the or normal serum IgG, IgA and IgM levels, can be a deter - specific pneumococcal serotype tested, whereas values less minant of a specific antibody deficiency (SAD). However, than 1.3 mcg/mL are defined as not protective. Previously, many medical and insurance personnel may be confused the titer value was considered a meaningful immune by a SAD diagnosis for patients with normal serum IgG, response if there was a fourfold increase between the IgA and IgM levels, even though the se patients respond pre- and post-immunization titers (example : if pre = 4 poorly to pneumococcal vaccination. It’s a common mis - mcg/mL, then post >16 mcg/mL would be considered conception that if the IgG serum levels are normal, then nor mal). Now, if the pre-immunization titer is greater than the humoral immune system must be normal. That is 1.3 mcg/mL, then a twofold increase may be accepted as wrong! The humoral immune system (antibodies and nor mal (example : if pre = 4 mcg/mL, then post >8 mcg/mL complement proteins) may be within normal parameters is considered normal). For nonprotective pre-immunization only when appropriate specific antibody levels are made titers, the post-immunization titer values should increase and maintained after pneumococcal vaccination. into the protective range, with a threefold increase Currently, 23 different strains of pneumococcal bacteria expected. For very low pre-immunization titers, a fourfold are used to make up the pneumococcal vaccine. Since increase into the protective post-immunization range is each strain will result in the production of a “unique” anti - expected. body, the term “serotype” is used to denote each strain The next issue considered is the “number” of appropri - and the antibody directed against it. Therefore, 23 pneu - ately protective post-immunization serotype titers. For mococcal serotypes can be identified in the vaccine, and chil dren between 2 and 6 years of age, “normal” is defined they are composed of 23 unique antibodies and the by having more than 50 percent of the post-immunization “strength” of each antibody response, which is called the titers well into the protective range, with titer increases “titer.” Thus, 23 serotype titers can be determined from of twofold to fourfold, depending on how low the the pneumococcal vaccine. pre- immu nization titers were. For adults, it is 75 percent . To perform a pneumococcal assay, first a blood sample is taken from a patient (pre-immunization serum), and TERRY HARVILLE , MD, PhD, is medical director of the Special the n the pneumococcal vaccine is given. In approximately Immunology Laboratory at the University of Arkansas for four weeks, another blood sample is obtained for the Medical Sciences , and a consultant for immunodeficiencies, post-immunization serum. Both sera are then assayed to autoimmunities and transplantation . obtain specific titer values. The number of serotype titers analyzed has changed over Editor’s Note: This Immunology 101 column, introduced in the April-May the years. Initially, as few as four serotypes were analyzed. 2010 issue of IG Living , is intended to be a basic course in immunology.

8 April-May 2012 www.IGLiving.com IG Living! 1 3/7/12 4:19 PM

FDAFDA aapprovedpproved fforor CIDP,CIDP, PIPI andand IITPTP

Take Care with GAMMAKED

WARNING:WWAARNING: AACUTECUTE RRENALENAL DDYSFUNCTIONYSFUNCTION AANDND ACUTEACUTE RENALRENAL FAILUREFFAAILURE s2ENALDYSFUNCTION ACUTERENALFAILURE OSMOTIC NEPHROSIS ANDDEATHMAYOCCURWITHIMMUNE GLOBULIN INTRAVENOUS )')6 PRODUCTSINPREDISPOSEDPATIENTS0ATIENTSPREDISPOSEDTORENALDYSFUNCTIONINCLUDETHOSEWITHANYDEGREE OFPRE EXISTING RENAL INSUFFICIENCY DIABETES MELLITUS AGEGREATER THAN VOLUMEDEPLETION SEPSIS PARAPROTEIN EMIA ORPATIENTSRECEIVINGKNOWNNEPHROTOXIC DRUGS s2ENALDYSFUNCTION ANDACUTERENALFAILUREOCCURMORECOMMONLYINPATIENTSRECEIVING)')6PRODUCTS CONTAINING SUCROSE;='!--!+%$DOESNOTCONTAINSUCROSE s&ORPATIENTSATRISKOFRENALDYSFUNCTIONORFAILURE ADMINISTER'!--!+%$ATTHEMINIMUMCONCENTRATIONAVAILABLE ANDTHEMINIMUMINFUSIONRATEPRACTICABLE ((ssseeee WWaWarningsarrnniinnngggss aanandnd PPrPrecautions)rreeccaauttiiioonnsss))

Please see the GAMMAKED Brief Summary of Prescribing Information on the following page for additional prescribing details. References: 1. GAMMAKED [prescribing information]. Fort Lee, NJ: Kedrion Biopharma, Inc. 2012

©2012 Kedrion Biopharma, Inc. All rights reserved. Printed in USA March 2012 KEDUSA0213 US Customer Service 1-855-353-7466 www.gammaked.com 1 3/7/12 4:17 PM

% Hyperproteinemia, increased serum viscosity and % After infusion of IgG, the transitory rise of the hyponatremia may occur in patients receiving IGIV various passively transferred antibodies in the treatment, including GAMMAKED. It is clinically patient’s blood may yield positive serological testing critical to distinguish true hyponatremia from a results, with the potential for misleading Brief Summary pseudohyponatremia that is associated with interpretation. Passive transmission of antibodies to concomitant decreased calculated serum osmolality erythrocyte antigens may cause a positive direct or See full Prescribing Information for complete or elevated osmolar gap. indirect antiglobulin (Coombs’) test. information % Thrombotic events have been reported following Adverse Reactions WARNING: ACUTE RENAL DYSFUNCTION IGIV treatment and may occur in patients receiving AND ACUTE RENAL FAILURE Clinical Trials IGIV treatment, including GAMMAKED. Patients at PI - The most common adverse reactions ( 5%) with % Renal dysfunction, acute renal failure, osmotic risk may include those with a history of % intravenous use of GAMMAKED were headache, nephrosis, and death may occur with immune atherosclerosis, multiple cardiovascular risk factors, cough, injection site reaction, nausea, inflammation globulin intravenous (IGIV) products in advanced age, impaired cardiac output, coagulation predisposed patients. Patients predisposed to disorders, prolonged periods of immobilization of the throat, and hives. Vomiting was reported more frequently in pediatric patients. The most common renal dysfunction include those with any degree and/or known or suspected hyperviscosity. Consider adverse reactions ( 5%) with subcutaneous use of of pre-existing renal insufficiency, diabetes baseline assessment of blood viscosity in patients at mellitus, age greater than 65, volume depletion, risk for hyperviscosity. For these patients, administer GAMMAKED were infusion site reactions, headache, fatigue, joint pain, and fever. sepsis, paraproteinemia, or patients receiving GAMMAKED at the minimum rate of infusion known nephrotoxic drugs. practicable. % ITP - The most common adverse reactions during % Renal dysfunction and acute renal failure occur clinical trials (reported in 5% of subjects) were % Aseptic Meningitis Syndrome (AMS) may occur more commonly in patients receiving IGIV infrequently with IGIV treatment, including headache, vomiting, fever, nausea, back pain and products containing sucrose. GAMMAKED does rash. GAMMAKED. Discontinuation of IGIV treatment not contain sucrose. has resulted in remission of AMS within several days % CIDP - The most common adverse reactions during % For patients at risk of renal dysfunction or without sequelae. The syndrome usually begins clinical trials (reported in 5% of subjects) were failure, administer GAMMAKED at the within several hours to two days following IGIV headache, fever, chills, hypertension, rash, nausea minimum concentration available and the treatment. AMS is characterized by the following and weakness. minimum infusion rate practicable. symptoms and signs: severe headache, nuchal Postmarketing Experience Indications rigidity, drowsiness, fever, photophobia, painful eye % Hemolytic anemia and aseptic meningitis have been movements, nausea and vomiting. Cerebrospinal GAMMAKED consists of immune globulin injection identified and reported during the post marketing use fluid (CSF) studies are frequently positive with of GAMMAKED. (human) 10% liquid that is used: pleocytosis up to several thousand cells per cu mm, The following adverse reactions have been reported % As replacement therapy of Primary Humoral predominantly from the granulocytic series, and with during the overall post marketing use of IGIV products: Immunodeficiency (PI). elevated protein levels up to several hundred mg/dL, Respiratory: Apnea, Acute Respiratory Distress % To treat patients with Idiopathic Thrombocytopenic but negative culture results. Conduct a thorough % Purpura (ITP) to raise platelet counts to prevent neurological examination on patients exhibiting such Syndrome (ARDS), TRALI, cyanosis, hypoxemia, bleeding or to allow a patient with ITP to undergo symptoms and signs including CSF studies, to rule pulmonary edema, dyspnea, bronchospasm surgery. out other causes of meningitis. AMS may occur more % Cardiovascular: Cardiac arrest, thromboembolism, frequently in association with high doses (2 g/kg) To treat Chronic Inflammatory Demyelinating vascular collapse, hypotension % and/or rapid infusion of IGIV. Polyneuropathy (CIDP) to improve neuromuscular % Neurological: Coma, loss of consciousness, disability and impairment and for maintenance % IGIV products, including GAMMAKED, may seizures/convulsions, tremor contain blood group antibodies which may act as therapy to prevent relapse. % Integumentary: Stevens-Johnson syndrome, hemolysins and induce in vivo coating of red blood Contraindications epidermolysis, erythema multiforme, cells (RBCs) with immunoglobulin, causing a bullous dermatitis % GAMMAKED is contraindicated in patients who positive direct antiglobulin reaction and, rarely, have had an anaphylactic or severe systemic reaction hemolysis. Delayed hemolytic anemia can develop % Hematologic: Pancytopenia, leukopenia, hemolysis, to the administration of human immune globulin. subsequent to IGIV therapy due to enhanced RBC positive direct antiglobulin (Coombs test) % GAMMAKED is contraindicated in IgA deficient sequestration, and acute hemolysis consistent with % General/Body as a Whole: Pyrexia, rigors patients with antibodies against IgA and history of intravascular hemolysis, has been reported. Monitor % Musculoskeletal: Back pain patients for clinical signs and symptoms of hypersensitivity. Gastrointestinal: Hepatic dysfunction, abdominal hemolysis. If signs and/or symptoms of hemolysis are % Warnings and Precautions pain present after GAMMAKED infusion, perform % Severe hypersensitivity reactions may occur with appropriate confirmatory laboratory testing. Drug Interactions IGIV products, including GAMMAKED. In this case, Noncardiogenic pulmonary edema may occur in % Passive transfer of antibodies may transiently discontinue GAMMAKED infusion immediately and % patients following treatment with IGIV products, interfere with the response to live viral vaccines, such institute appropriate treatment. Medications such as including GAMMAKED. Transfusion-related Acute as measles, mumps and rubella. This may confound epinephrine should be available for immediate Lung Injury is characterized by severe respiratory serologic testing. Inform the immunizing physician of treatment of acute hypersensitivity reaction. distress, pulmonary edema, hypoxemia, normal left recent therapy with GAMMAKED so that % Assure that patients are not volume depleted prior to ventricular function, and fever. Symptoms typically appropriate measures may be taken. the initiation of the infusion of GAMMAKED. occur within 1 to 6 hours after treatment. Monitor Use in Specific Populations Periodic monitoring of renal function and urine patients for pulmonary adverse reactions. If TRALI is % Pregnancy Category C. There is no human or animal output is particularly important in patients judged to suspected, perform appropriate tests for the presence data. It should only be given to a pregnant woman have a potential increased risk for developing acute of anti-neutrophil and anti-HLA antibodies in both only if clearly needed. renal failure. Assess renal function, including the product and patient serum. TRALI may be measurement of blood urea nitrogen (BUN)/serum managed using oxygen therapy with adequate % Geriatric: In patients over 65 years of age, do not creatinine, prior to the initial infusion of ventilatory support. exceed the recommended dose, and administer GAMMAKED and at appropriate intervals. If renal GAMMAKED at the minimum infusion rate The high dose regimen (1g/kg x 1-2 days) is not function deteriorates, consider discontinuation of % practicable. recommended for individuals with expanded fluid GAMMAKED. For patients judged to be at risk for volumes or where fluid volume may be a concern. developing renal dysfunction (e.g., any degree of pre- Rx Only existing renal insufficiency, diabetes mellitus, age % Because GAMMAKED is made from human blood, Manufactured by: greater than 65, volume depletion, sepsis, it may carry a risk of transmitting infectious agents. Talecris Biotherapeutics, Inc. paraproteinemia, or patients receiving known No cases have ever been identified for Research Triangle Park, NC 27709 Distributed by: nephrotoxic drugs) administer GAMMAKED at the GAMMAKED. ALL infections suspected by a physician possibly to have been transmitted by this Kedrion Biopharma, Inc. minimum infusion rate practicable. 400 Kelby Street product should be reported by the physician or other % Do not administer GAMMAKED subcutaneously in Fort Lee, NJ 07024 healthcare provider to Talecris Biotherapeutics, Inc. patients with ITP because of the risk of hematoma [1-800-520-2807] formation. © 2012 Kedrion Biopharma, Inc. Did You Know

Contest Research Enter IG Living’s Second Autoimmune Annual Essay Contest! Disease Linked • Write no more than 600 words, to Non-Healing and be sure the essay is typed and double-spaced. Wounds • Include a title for your essay, and make sure it includes the author's name, complete address, email, phone number and word count. • Submit your entry electronically as a Microsoft Word attachment to [email protected], or submit it by mail to: IG Living Essay Contest, 41093 County Center Drive, Temecula, CA 92591, Attention: Carla Schick. • Mail your entry by June 1, 2012 (must be postmarked by that date). IG Living’s judges will rate the A study by a Georgetown rheuma - entries on a scale of one to 10 on five tologist found that patients who had criteria: open wounds that were very slow to • Organization (the writing flows heal also had underlying autoimmune logically with clear structure) diseases. The study reviewed charts IG Living is hosting its second annual • Mechanics (spelling, capitaliza - of 340 patients treated at a high- essay contest open to IG patients and tion and punctuation are correct) vol ume wound clinic at Georgetown their caregivers ages 18 and older. • Content (subject is discussed University Hospital in Washington, Similar to last year’s contest, we are clearly, and the reader is left with a D.C., for mostly leg ulcers during a asking entrants to start their essay finished feeling) three-month period in 2009. Forty- with an identical phrase. This year, • Creativity (content is compellingly nine percent of those patients had we have chosen the topic of risk- interesting for our audience) diabetes, which is a typical rate, taking. • Effectiveness (the whole entry is yet 23 percent also had underlying When have you taken a risk, no effective in its purpose for our audience) autoimmune diseases. Of those 78 matter how small? Have you tried an Winners will be announced on patients who had autoimmune dis - exotic food to ease GI issues, July 1st. The first-place winner will eases, most had rheumatoid arthritis, learned a new language to take your be awarded a new Kindle eReader, lupus or livedoid vasculopathy, a mind off your illness, or flirted with and their essay will be published in type of vascular disease. According a complete stranger to feel a little IG Living magazine. Second- and to the researchers, the connection more “normal”? How did things third-place winners will be awarded between these relatively rare disor - turn out? What did you learn? To a $50 gift card, and their essays ders and wounds that don’t heal is participate in the contest, begin your will be published in an IG Living unrecognized. The findings also essay by completing this sentence: blog. showed that autoimmune disease- The last time I did something for the This is your opportunity to get associated wounds were significantly first time, I... pub lished in IG Living ! For additional larger at the patient’s first visit, and Guidelines for essay submittal are entry rules, refer to the IG Living blog that skin grafts were more likely to as follows: contest page at www.IGLiving.com . fail in these patients.

April-May 2012 www.IGLiving.com IG Living! 11 Did You Know

Research Combined IVIG Treatment Effective to Treat Kawasaki Disease

Researchers from Kitasato University scores of 2.5 or more at one month School of Medicine in Japan found were higher in the IVIG group than in that combining intravenous methyl - the combined treatment group. prednisolone pulse and intravenous Adverse events of the combination immunoglobulin (IVIG) to treat patients therapy included hypothermia, with Kawasaki disease appears safe bradycardia and hypertension in and effective. The researchers looked some patients; however, these events at data on 122 patients with Kawasaki were transient and not serious in disease who were randomly assigned either group. “Approximately 15 to either the combined treatment or percent to 20 percent of patients IVIG alone. Fever abated more quickly with [Kawasaki disease] are not in 19 of 22 patients in the combined responsive to initial IVIG treatment, group compared with six of 26 and these patients are at a higher identify these patients because they patients in the IVIG group. In addi - risk for coronary artery lesions,” the might benefit from more aggressive tion, coronary artery dimension z researchers wrote. “It is important to initial treatment.” 

                                      

 
 
          
Did You Know

Research Two in Five Adults with RA Are Inactive A new study finds that two in five such as motivation for physical activity, improve public health. “While there is adults (42 percent) with rheumatoid obesity and pain. In addition to the inac - much evidence of the benefits of physi - arthritis (RA) are inactive. The study, tivity findings, researchers found that 53 cal activity, RA patients are generally not which was funded by a grant from the percent of study participants lacked physically active, and physicians often National Institutes for Arthritis and strong motivation for physical activity, do not encourage regular physical Musculoskeletal and Skin Diseases, ana - and 49 percent lacked strong beliefs in activ ity in this patient population,” lyzed data on 176 RA patients 18 years the benefits of physical activity. explains Dr. Jungwha Lee, an assistant of age and older enrolled in a random - Until the early 1980s, medical experts professor in the Department of ized controlled trial to assess the effec - recommended medication and rest for Preventive Medicine at Northwestern tiveness of an intervention promoting those with RA. However, current med - University Feinberg School of Medicine physical activity. Researchers evaluated ical evidence suggests that regular mod - in Chicago, Ill., and the study’s lead pre-intervention data for inactivity, erate physical activity benefits arthritis researcher. “Our study aims to expand which was defined as no sustained 10- sufferers by maintaining joint flexibility, understanding of the risk factors associ - minute periods of moderate to vigorous improving balance, strengthening mus - ated with inactivity among adults with physical activity during a week. They cles and reducing pain. Therefore, tak - RA and encourage clinical interven - also assessed the relationships between ing measures to motivate RA patients to tions that promote participation in inactivity and modifiable risk factors increase their physical activity will physical activity.”

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XXXIJHIøPOFFEMFTDPN HIGHLIGHTS OF PRESCRIBING INFORMATION t
Hyperproteinemia, increased serum viscosity and hyponatremia occur in patients receiving IGIV therapy. These highlights do not include all the information needed to t
Thrombotic events have occurred in patients receiving IGIV therapy. use Octagam, Immune Globulin Intravenous (Human), safely and Monitor patients with known risk factors for thrombotic events; effectively. See full prescribing information for Octagam. consider baseline assessment of blood viscosity for those at risk of hyperviscosity. Octagam® [Immune Globulin Intravenous (Human)] t
Aseptic Meningitis Syndrome has been reported with Octagam 5% 5% Liquid Preparation liquid and other IGIV treatments, especially with high doses or Initial US Approval: 2004 rapid infusion. t
Hemolytic anemia can develop subsequent to IGIV therapy due to WARNING: ACUTE RENAL DYSFUNCTION and RENAL FAILURE enhanced RBC sequestration. See full prescribing information for complete boxed warning. t
IGIV recipients should be monitored for pulmonary adverse reactions (TRALI). - Renal dysfunction, acute renal failure, osmotic nephrosis, and death t
The product is made from human plasma and may contain infection may be associated with Immune Globulin Intravenous (Human) agents, e.g. viruses, and theoretically, the Creutzfeldt-Jakob (IGIV) products in predisposed patients. disease agent. ------ADVERSE REACTIONS------Renal dysfunction and acute renal failure occur more commonly in patients receiveing IGIV products containing sucrose. Octagam 5% Most common adverse reactions with an incidence of >5% during a liquid does not contain sucrose. clinical trial were headache and nausea. To report SUSPECTED ADVERSE REACTIONS, contact Octapharma at 1-866-766-4860 or - Administer IGIV products at the minimum concentration available FDA at 1-800-FDA-1008 or www.fda.gov/medwatch. and the minimum infusion rate practicable. ------DRUG INTERACTIONS------t
The passive transfer of antibodies may confound the results of ------RECENT MAJOR CHANGES------serological testing. t
The passive transfer of antibodies may interfere with the response Warnings and Precautions – Hyperproteinemia 8/2008 to live viral vacancies. ------INDICATIONS AND USAGE------USE IN SPECIFIC POPULATIONS------t


Octagam is an immune globulin intravenous (human), 5% liquid, t
Pregnancy: no human or animal data. Use only if clearly needed. indicated for treatment of primary humoral immunodefi ciency (PI). t
In patients over age 65 or in any person at risk of developing renal ------DOSAGE AND ADMINISTRATION------insuffi ciency, do not exceed the recommended dose, and infuse Intravenous use only. Octagam 5% liquid at the minimum infusion rate practicable. Indication Dose Initial Infusion Maintenance ------HOW SUPPLIED------rate infusion rate (if tolerated) 1g 2.5g 5g 10g 25g Size 20ml 50ml 100ml 200ml 500ml PI 300-600mg/kg 0.5mg/kg/min 3.33mg/kg/min NDC# 67467-843-01 67467-843-02 67467-843-03 67467-843-04 67467-843-05 Every 3-4 weeks NDC# 67467-843-01 67467-843-02 67467-843-03 67467-843-04 t
Ensure that patients with pre-existing renal insuffi ciency are not MANUFACTURED BY: volume depleted; discontinue Octagam 5% liquid if renal function deteriorates. OCTAPHARMA Pharmazeutika t
For patients at risk of renal dysfunction or thrombotic events, Produktionsges.m.b.H. administer Octagam 5% liquid at the minimum infusion Oberlaaer Strasse 235 rate practicable. A-1100 Vienna, Austria ------DOSAGE FORMS AND STRENGTHS------DISTRIBUTED BY: Octagam 5% liquid is supplied in 1.0g, 2.5g, 5g, 10g, or 25g Octapharma USA, Inc. single use bottles 121 River Street, Suite 1201 Hoboken, NJ 07030 ------CONTRAINDICATIONS------Tel: 201-604-1130 Fax: 201-604-1131 Anaphylactic or severe systemic reactions to human t
www.octapharma.com/usa immunoglobulin. t
Immunoglobulin A (IgA) defi cient patients with antibodies against IgA and a history of hypersensitivity. Revised: September 2009 t
Patients with acute hypersensitivity reaction to corn.

------WARNINGS AND PRECAUTIONS------t
IgA defi cient patients with antibodies against IgA are at greater risk of developing severe hypersensitivity and anaphylactic reactions. t
Epinephrine should be available immediately to treat any acute severe hypersensitivity reactions. t
Monitor renal function, including blood urea nitrogen and serum creatinine, and urine output in patients at risk of developing acute renal failure. t
Falsely elevated blood glucose readings may occur during and after the infusion of Octagam 5% liquid with some glucometer and test strip systems.

1:04 PM RESEARCHED REFINED REASSURED

Proven clinical effi cacy Updated manufacturing Validated in patients with primary process to enhance pathogen safety immunodefi ciency (PI)1,2 thromboembolic safety3

For more information, please contact us:

Octapharma USA, Inc. To report suspected adverse reactions, Medical Affairs: contact Octapharma USA, Inc. 121 River Street [email protected] 866-766-4860 or Suite 1201 888-429-4535 FDA at 1-800-FDA-1088 or Hoboken, NJ 07030 www.fda.gov/medwatch 201-604-1130 Reimbursement: www.octapharma.us [email protected] Tel: 800-554-4440 Customer Service: Fax: 800-554-6744 [email protected] 866-766-4860

IMPORTANT SAFETY INFORMATION Octagam® is contraindicated in individuals with intolerance to immunoglobulins, especially in immunoglobulin A (IgA) defi ciency, when the patient has IgE mediated antibodies to IgA. Immune Globulin Intravenous (Human) (IGIV) products have been reported to be associated with renal dysfunction, acute renal failure, osmotic nephrosis, and death. Other possible side effects with Octagam include: aseptic meningitis, hemolysis, transfusion-related acute lung disease (TRALI) and thrombotic events. Immune Globulin Intravenous (Human) products have been reported to be associated with various minor reactions, such as headache, chills, backache, chest pain, fever, allergic reactions, arthralgia, dizziness, and changes in blood pressure, cutaneous reactions and/or nausea and vomiting. Cases of reversible aseptic meningitis and migraine and isolated cases of reversibly hemolytic anemia and reversible increases in liver function tests have been observed with Octagam. Immediate anaphylactic and hypersensitivity reactions are a remote possibility. As with all medicine made from human plasma, the risk of spreading infections agents, including viruses, cannot be completely eliminated. Some types of blood glucose testing systems falsely interpret the maltose contained in Octagam as glucose. This has resulted in falsely elevated glucose readings and, consequently, in the inappropriate administration of insulin, resulting in life-threatening hypoglycemia.

References 1. Octagam®, Immune Globulin Intravenous (Human) 5% Liquid Preparation, complete Prescribing Information. 2009. 2. Ochs HD, Pinciaro PJ. Octagam® 5%, an intravenous IgG product, is effi cacious and well tolerated in subjects with primary immunodefi ciency diseases. J Clin Immunol 2004;24:309-14. 3. Roemisch J, et al. Identifi cation of activated FXI as the major biochemical root cause in IVIG batches associated with thromboembolic events. analytical and experimental approaches resulting in corrective and preventive measures implemented into the Octagam® manufacturing process. WebmedCentral Immunother 2011;2:WMC002002.

Please see Highlights of Prescribing Information

1:04 PM By Matthew D. Hansen, DPT, MPT, BSPTS

For chronically ill patients, the amount of exercise needed depends upon what is right for each individual, but inactivity is not an option.

16 April-May 2012 www.IGLiving.com IG Living! s human beings, we often struggle with knowing every exercise session, the effect of exercise on anx iety how much is too much and how much is too little reported in our study may be underestimated.” 1 Ain many aspects of our lives. For instance, we Overactivity certainly can lead to greater exhaustion. won der about how much sleep, food, drink, attention, However, somewhat ironically, inactivity deconditions the work, entertainment, relaxation, fiber, etc., we really need. body and may not only lead to exhaustion, but it can But, for those patients who live with a chronic illness, few actually make symptoms of the condition even worse. So, questions have consequences that are more far-reaching what’s the answer to this seeming “catch-22”? If the than: “How much exercise/activity is too much, and how solu tion isn’t inactivity, and it’s not just grinning and bearing much is too little?” it, it must be — as is often the case in life — somewhere in between. The easiest formula for finding the right amount of exercise is for patients to take note of how they The easiest formula for feel afterward. All people may experience some tiredness and muscle soreness after exercising (especially when they finding the right amount are just getting started); however, those feelings should not be debilitating, and after patients have had a chance of exercise is for patients to rest, they should usually feel better than they did previous to exercising. If any pain or exhaustion persists to the point to take note of how they that it is interfering with patients’ normal daily routine the next day, they have likely overdone it ( gotten too much exercise). If they don’t have any discomfort or exhaustion feel afterward. after exercising, but aren’t experiencing any of the apparent benefits either, they can probably afford to increase at least One day of really overdoing exercise may put someone one component of their routine (intensity, frequency with a chronic illness out of the game for the rest of the and/or duration). week; worse yet, with some diagnoses, it may actually Seems simple enough, right? It may seem simple, but exacerbate the condition! Consequently, many patients finding the proper balance can be a confusing and find it easier to simply justify not exercising at all. “After some times frustrating process. In order to assist in the all,” they think, “why risk getting worse when I barely have the energy to get started anyhow?” Unfortunately, this is a fallacy in thought that is all too common, and it’s not just found with patients who experience a chronic illness.

Risks of Inactivity Scientific research has long established many of the risks of not exercising, including high blood pressure and/or heart disease, obesity and associated type 2 diabetes, possible increased risk of certain cancers, osteoporosis, increased risk of muscular and soft tissue injuries, anxiety and depression. Living with a chronic illness can present enough anxiety. Why add any more? A meta-analysis published in the February 2010 issue of Archives of Internal Medicine reviewed 40 studies on the effects of exercise in nearly 3,000 participants living with a chronic illness. In 90 percent of the studies analyzed, those who exercised regularly reported an approximately 20 percent reduction in anxiety symptoms compared with those who did not exercise. Co-author Rod Dishman, PhD, explained that “because not all study participants com pleted

April-May 2012 www.IGLiving.com IG Living! 17 Figure 1.

• How patient felt four hours after EXERCISE LOG Date: ______Day: M T W T F S S exercising

Exercises Sets Reps Minutes Distance Notes (Intensity Level, etc.) • How patient felt the day after exercising • Goals for the next exercise session Again, there’s no standard format. Patients can use narratives, check boxes, graphs or whatever they like

Energy Level (Before Exercise) 1 2 3 4 5 to track these or other factors of their Pain Level (Before Exercise) 1 2 3 4 5 own. The key to using an exercise log is for patients to find or develop a sys - Energy Level (Immediately After Exercise) 1 2 3 4 5 tem that works for them and to use Pain Level (Immediately After Exercise) 1 2 3 4 5 it ! This means not only recording in Energy Level (Four Hours After Exercising) 1 2 3 4 5 their exercise log daily, but also Pain Level (Four Hours After Exercising) 1 2 3 4 5 reviewing past entries to discover

Energy Level (Day After) 1 2 3 4 5 pat terns, such as what exercises Pain Level (Day After) 1 2 3 4 5 made them feel better, what exercises made them feel worse, how far they Goals for Next Exercise Session: can walk on a flat surface without becoming exhausted, and how many repetitions of a given exercise they can perform before it causes pain.

Visual Chart A regular element of each of the This exercise log can be downloaded for use at www.IGLiving.com/IGResources.aspx. exercise logs that I helped to develop is a visual chart to monitor exhaustion task, I have found two tools to be invaluable: the exercise and pain. I prefer to keep it simple and use a graduated log and visual charts used to monitor pain and exhaustion. five-point scale (five- and 10-point scales seem to be the norm, though there isn’t anything that says that a seven-point Exercise Log An exercise log is basically a journal that records patients’ activities for the day and their physical response A regular element of each to those activities (see Figure 1). It also can be used to set goals, measure progress and serve as a visual motivator. If patients have ever kept an infusion or diet log, they’re of the exercise logs that already familiar with the concept of keeping these types of records. However, I highly recommend that they personalize I helped to develop is a their log to meet individual needs and style. Here are several components for consideration and an example of a log visual chart to monitor that patients may use: • Date and time exhaustion and pain. • How patient felt before exercise • Type of exercise(s) performed • Length of time of each exercise scale can’t be used if it’s someone’s favorite number). • Intensity level ( one to five ) experienced while performing Patients may also choose to use a color scale and/or exercise(s) fig ures to represent their energy and/or pain status. (See • How patient felt immediately after exercising Figure 2.)

18 April-May 2012 www.IGLiving.com IG Living! How to Avoid Doing Too Much exercise routine. However, consistency is important, It’s almost always better for patients to ease into activity because deconditioning may occur even more quickly if no versus overdoing it and getting discouraged as a result. exercise is performed over an extended period of time. However, as a rule of thumb, I like to recommend that Exercise tolerance can vary daily in the chronically ill patients use their current activity level as their baseline and patient population. It’s all right to avoid exercise during a increase activity (intensity and/or duration) by no more symptom flare-up, scale back to a prior level, or substitute than 10 percent to 20 percent per week until they experience for an exercise with an easier activity or light stretching. any hint that they may be starting to overdo it. At that Pacing techniques also are important. Oftentimes, it’s safer point, it’s important to slightly scale back until the initia tion and just as effective for chronically ill patients, depending of symptoms disappears. Despite my rules (and my thumbs), patients with a chronic illness should always visit with their doctor prior to beginning a new exercise program or significantly increasing an established one. Exercise tolerance can vary What might the 10 percent rule look like? Patients who may be able to walk five minutes without stopping to rest, daily in the chronically ill for example, may attempt to begin by increasing their rou - tine to walking five and a half minutes without stop ping. Maybe they are able to walk 100 feet, arm curl 5 pounds, patient population. or perform 10 knee bends. The point is to increase this amount by just 10 percent to 20 percent per week. As you can imagine, by increasing activity level in small intervals, on the purpose of the program, to exercise several times a it may take chronically ill patients a number of weeks day for shorter intervals than to lump it all together into (even months) to establish their maximum symptom-free one longer session. If a planned activity becomes too much, it’s better to stop than to push through the exercise

Figure 2. and pay for it later. Meditation techniques also can be used as a warm-up to 1 2 3 4 5 aerobic or strengthening exercises, and will oftentimes help patients to direct their session. Moreover, meditation is a FULLY MILD MODERATE ENERGIZED RESTED FATIGUE FATIGUE EXHAUSTED fantastic stress-reducing activity that can be utilized in just about any situation. It can be performed through the use of yoga, tai chi, stretching or simply sitting still and listening. By learning to listen to their own body, and using tools such as exercise logs and visual charts to assist, patients

will soon discover how to tell how much exercise is too NO PAIN SLIGHT MILD MODERATE SEVERE DISCOMFORT PAIN PAIN PAIN much and how much is too little. The rest is a walk in the park, or at least it could be — that’s up to the patient! OR MATTHEW DAVID HANSEN , DPT, MPT, BSPTS, is a practicing physical therapist in Utah, and president of a healthcare consulting 1 2 3 4 5 and fitness product company, SOMA Health, LLC. The company's latest product, the Freedom2Move exercise program and video series, FULLY MILD MODERATE E N ERGIZED RESTED FATIGUE FATIGUE EXHAUSTED was inspired by an IG Living Readers Teleconference. Matt completed his formal education at the University of Utah in Salt Lake City.

Reference 1. Herring, MP, and O’Connor, PJ. The Effect of Exercise Training on NO PAIN SLIGHT MILD MODERATE SEVERE Anxiety Symptoms Among Patients, Archives of Internal Medicine, DISCOMFORT PAIN PAIN PAIN (reprinted) February 2010.

April-May 2012 www.IGLiving.com IG Living! 19 Diagnosing PIDD in By Melvin Berger, MD

There are many forms of PIDD, most of which can be diagnosed early and correctly by recognizing the signs and using a variety of testing methodologies.

rimary immune de ficiency diseases (PIDDs) are caused requires awareness and astute observation by primary care by genetic abnormalities that increase patients’ physicians. The National Institutes of Health, the Immune Psus ceptibility to infection. Since the immune system Deficiency Foundation (IDF) and the Jeffrey Modell cannot readily be seen when we look at a baby or child, Foundation (JMF) devote considerable effort to raising the most forms of PIDD are diagnosed only after the patient awareness of PIDD among physicians and the general has suffered unusual, severe or recurrent infections. public, and they have developed guidelines for the recog - Recognizing those patterns of infection that suggest PIDD nition, diagnosis and management of these diseases .1,2,3

20 April-May 2012 www.IGLiving.com IG Living! SCID is caused by mutations in genes that control the development and most basic functions of the immune sys tem, which makes these patients nearly defenseless. Surprisingly, in most cases, there is no outward sign that a newborn baby has SCID. As a consequence, that child may experience d evastating infections that result from expo - sure to germs that are everywhere in the environment — germs that are harmless to people with normal immune systems. Once a serious or life -threatening infection sets in, expensive and complicated intensive care may be needed, and damage to organs such as the lungs or liver may make subsequent definitive corrective therapy difficult. There are some conditions that can mimic SCID , but they are not nearly as serious .5 Therefore, a correct and early diagnosis is extremely important . Fortunately, there are now tests for SCID that can be performed on the dried blood spots obtained from all babies in the U.S., allowing physicians to detect SCID before infection occurs. 6 Essentially all forms of SCID result in very low levels of T cells ,4 and SCID babies have extremely low levels of a unique kind of DNA fragment called T cell receptor excision circles (TRECs) , which are formed when T lympho - cytes are made. Tests for TRECs performed on the dried blood spots have been adopted in several states to screen newborns for SCID . Once diagnosed with SCID, these babies can receive stem cell transplants that provide them with a new immune system. When stem cell transplants are performed in the first few months of life, the long -term survival rate is greater than 95 percent , and many of these babies can be considered cured .7 Unfortunately, only a few states offer newborn screening for SCID. Where screening is not available, awareness by the primary care physician and/or principal caregiver is key. Infants The family history can provide important clues, particularly if male relatives on the mother’s side died or had serious infections in infancy.

Diagnosing SCID Diagnosing Other Serious PIDDs The most serious form of PIDD is called severe combined Several of the most common PIDDs, including an impor - immune deficiency (SCID ). The incidence of SCID is about tant form of antibody deficiency known as Bruton’s type one in 50,000 to one in 100,000 live births .4 In the 1970s, of agammaglobulinemia (antibody deficiency), as well as a patient with SCID whose story has been widely recounted Wiskott-Aldrich syndrome , are called “X-linked” because was kept free from infection only by having him live inside the mutation that cause s the disease is carried on the a sterile plastic chamber to completely isolat e him from all X- chromosome. Females have two X chromosomes, so infectious agents . This led to the adoption of the term even if one has a PIDD-causing mutation, they are usually “bubble boy disease” for SCID. Today, many forms of SCID healthy carriers. On the other hand, males have only one can now be corrected by stem cell transplantation, gene X chromosome, so if that has a mutation, the male will therapy or enzyme therapy. have the disease.

April-May 2012 www.IGLiving.com IG Living! 21 Some serious immune deficiencies such as DiGeorge’s syndrome, Wiskott-Aldrich syndrome, hyper-IgE syndrome and others are associated with other abnormalities that Immune Deficiency Foundation Diagnostic & Clinical may be more readily apparent. Unusual cases of pneumonia, Care Guidelines Patient for Primary Immunodeficiency Diseases & Family severe or prolonged diarrhea with failure to thrive , and Handbook

For Primary certain skin rashes all can be indicative of serious immune Immunodeficiency Diseases deficiencies. Evaluation of suspected immune deficiencies in infants should include a careful physical exam with documentation

of the presence or absence of tonsils and lymph nodes, as well as a search for other abnormalities that might suggest The Immune Deficiency Foundation offers several publications, including immune deficiency. Laboratory evaluation by the primary Diagnostic and Clinical Guidelines for PIDD and the Patient and Family care physician should include a chest X-ray for assessment Handbook for Primary Immunodeficiency . of the presence or absence of the thymus and a complete blood count with differential. The latter test may reveal on their own, and they have very low levels of IgA and low numbers of lymphocytes, suggesting a deficiency of IgM. This is because IgG is transferred from the mother T cells or of neutrophils, which are needed for engulfing across the placenta during the last three months of preg - and killing bacteria and other invaders. It is important to nancy. This IgG gives some protection to the new born, so PIDDs that mainly affect antibody production (more than half of all know n types of PIDD) are often not detected The second stage of the in early infancy. However, by the time the baby is 4 to 6 months old, t he IgG from the mother is metabolized ( used up ), and babies with antibody deficiencies or PIDDs other diagnostic evaluation than SCID usually will start to have an increased severity and/or frequency of infections after that age. The frequen - includes tests for specific cy and severity of infections, though, are highly variable because of differences in exposure to infectious agents. antibodies to immunizations For example, a first-born baby living at home with his or her mother is much less likely to be exposed to germs that patients should have cause ear infections and pneumonia than a baby who starts in day care or preschool at an early age, or a baby who has multiple school-aged siblings. School-aged received as part of the regular chil dren (and adults with a high degree of exposure to children and/or each other) are much more likely to be ill childhood immunization with frequent viral and bacterial infections. As with older children and adults, awareness by the schedule. pri mary care physician is the most important element in appropriate diagnosis and management of these PIDDs. Unfortunately, the diagnosis is often delayed — by as compar e blood count results against age-adjusted normal much as 10 years in many cases. The JMF publishes a values, since babies should have higher numbers of poster titled the “10 Warning Signs of Primary lym phocytes than older children and adults. If these tests Immun odeficiency ”. These warning signs have been suggest a serious PIDD, consultation by an experienced for mulated and periodically updated by expert immu - immunologist should be sought without delay. nolo gists. The poster is available in both text and cartoon formats, in versions applicable to adults and chil dren, Diagnosing More Common PIDDs and in many languages. Full-term infants are born with the same levels of IgG as If multiple and/or particularly severe infections occur in normal adults, even though they cannot make much antibody babies and children who also have histories of diarrhea,

22 April-May 2012 www.IGLiving.com IG Living! poor weight gain and/or failure to thrive , or in adults who variable immune deficiency (CVID) and other defects in are losing weight and/or having unexplained fevers or antibody production. cough and other lung symptoms, a careful physical exam The third tier of testing involves determination of the may reveal additional signs of PIDD , such as the absence numbers of each different type or “subset” of lymphocytes of tonsils and lymph nodes (in Bruton’s disease), enlarge - in the blood using a technology called “flow cytometry .” ment of the spleen and/or liver, atypical rashes, and/or This test is usually performed at regional referral centers chronic changes due to infection. Parents or patients and often leads directly to a definitive diagnosis of the themselves who are concerned about the possibility of specific type of PIDD the patient has. This level of testing PIDD may find helpful information on the websites also includes tests of the lymphocytes’ ability to respond to listed in the Sources section of IG Living , and may be stimulation in the laboratory and/or of the ability of certain interested in downloading or requesting a free hard white blood cells to generate active oxygen molecules that copy of the Patient and Family Handbook for Primary kill bacteria. Immunodeficiency Diseases from the IDF . That publication Finally, specific tests and mutation analyses performed in is now in its four th English edition, and it is also available specialized research laboratories can identify the exact in Spanish and French versions. The JMF also publishes a molecular defect responsible for many, but not yet all , poster explaining the four stages in the diagnostic evaluation spe cific types of PIDD. This information is very useful for of patients suspected of having PIDD. genetic testing and for developing a specific treatment As noted above, the initial screening steps such as a plan for each type of PIDD. When developing a treatment complete blood count with differential, a chest X-ray and plan, t he IDF Patient and Family Handbook for Primary a test for immunoglobulin levels can be obtained in virtually Immunodeficiency can be an invaluable aid to understanding any practice or community hospital in the U .S. These tests what the doctors are talking about when they slip into alone usually can distinguish between those patients who their medical jargon, as well as a guide to knowing what can be reassured that they are unlikely to have a PIDD and treatment options are available. In addition, the IDF makes that their infections are within the range expected for their degree of exposure, those who should be watched more closely, and those who should be referred to an immunologist. Caution must be used , however, to be sure the results are compared with age-adjusted normal values, since the absolute lymphocyte count (obtained from the CBC and differential) and immunoglobulin (IgA, IgG and IgM) levels vary during a child’s development into adulthood. If any physician does not know to whom to refer a sus - pected PIDD patient, t he IDF , the Clinical Immunology Society and the JMF maintain directories of immunologists with expertise in PIDD . The IDF also publishes a booklet titled Diagnostic and Clinical Gui delines for PIDD , which is very useful and represents a consensus among experts in PIDD. The second stage of the diagnostic evaluation includes tests for specific antibodies to immunizations patients should have received as part of the regular childhood immunization schedule .8 These tests may also involve look ing at the specific antibody response before and after certain vaccines like Pneumovax. Blood samples for these tests are frequently sent to large national reference labo - ratories, but they can be ordered by any doctor. These tests help to distinguish between patients who have low or borderline IgG levels but whose antibody production is This poster is available from the Jeffrey Modell Foundation in both text really normal , and those who have PIDDs such as common and cartoon formats.

April-May 2012 www.IGLiving.com IG Living! 23 available a guide for teachers and other school personnel , which can be helpful in en suring that appropriate provisions are taken when a child with PIDD is in the classroom.

PIDDs and Childhood Vaccinations Although most of the early childhood vaccines can be given safely to babies with SCID and other PIDDs, a few of these vaccines contain live viruses and should be avoided. The only live vaccines currently recommended for routine childhood immunization 8 in the U .S. are those against rotavirus, measles , mumps and rubella (MMR), chicken pox (varicella/zoster) and the nasal influenza vaccine. Routine diphtheria, tetanus and pertussis ( DTaP ), injectable key first step in appropriate evaluation and diagnosis of influenza, inactivated (Salk) polio vaccine, hepatitis A and PIDD. With advances in replacement of IgG and other B vaccines, and hemophilus and pneumococcal vaccines important proteins, stem cell transplants, and even gene may or may not be effective in patients with PIDD, but therapy in some cases, there are now many treatments they do not pose special risks for them . If there is any that can help PIDD patients live happy, productive lives. question about the safety of vaccines for any child, a CBC We must all work to en sure that these treatable diseases and differential count to rule out lymphopenia (low are recognized and properly diagnosed so the right therapy lym phocyte counts) should be conducted , and inquiries can be given to the right patient . should be directed to an expert immunologist. MELVIN BERGER , MD, PhD, is senior medical director for immunology research and development at CSL Behring, and adjunct professor of pediatrics and pathology at Case With modern diagnostic Western Reserve University in Cleveland, where he practiced testing, a definitive diagnosis clinical immunology for 25 years. References can be made in most cases 1. National Institutes of Health. When the Body’s Defenses Are Missing: Primary Immunodeficiency. Accessed at www.nichd.nih.gov/publications/ pubs/primary_immuno.cfm . See also: www.niaid.nih.gov/topics/immune of PIDD, and the precise Deficiency/Understanding/Pages/ Default.aspx . 2. Immune Deficiency Foundation . Patient and Family Handbook for Primary Immune Deficiency Diseases, 4th edition. Accessed at molecular defect often primaryimmune.org/publications/patient-family-handbook . See also: primaryimmune.org/about-primary-immunodeficiency-diseases . 3. Jeffrey Modell Foundation . Primary Immunodeficiency Resource can be identified. Center and 10 Warning Signs of Primary Immunodeficiency. Accessed at www.info4pi.org. 4. Lindegren , ML, Kobrynski , L, Rasmussen , SA, et al. Applying Public Advances and Education Are Health Strategies to Primary Immunodeficiency Diseases: APotential Key to Diagnosing PIDD Approach to Genetic Disorders. MMWR Recomm endation Rep ort . 2004 Jan 16; 53(RR-1):1-29. Accessed at www.ncbi.nlm.nih.gov/pubmed/ With modern diagnostic testing, a definitive diagnosis 14724556 . can be made in most cases of PIDD , and the precise 5. Buckley, RH. Primary Immunodeficiency or Not ? Making the Correct molec ular defect often can be identified. None of the Diagnosis. Journal of Allergy and Clin ical Immunol ogy . 2006; 117: 756-8 . sophisticated laboratory tests or special techniques will be 6. Puck , JM. Neonatal Screening for Severe Combined Immunodeficiency. Curr ent Opin ion in Pediatr ics . 2011 Dec; 23(6):667-73. of any value, however, if PIDD is not suspected and the 7. Buckley , RH. Transplantation of Hematopoietic Stem Cells in Human patient is not referred. The JMF , the IDF and several other Severe Combined Immunodeficiency: Longterm Outcomes. Immunol ogy professional organizations devote considerable effort to Res earch . 2011 Apr; 49(1-3):25-43. 8. Advisory Committee on Immunization Practices. Recommended educating physicians about PIDD. However, awareness by Immunization Schedules for Persons Aged 0 Through 18 Years. the patient’s family and the primary care doctor(s) is the Accessed at www.cdc.gov/vaccines/recs/schedules/default.htm .

24 April-May 2012 www.IGLiving.com IG Living!    Understanding and Treating Common Variable Immunodeficiency

By Ronale Tucker Rhodes, MS

While CVID is a “common” immune deficiency disease, as its name implies, it is far from a common disease. And in the majority of cases, it is diagnosed after years of illness.

t could be said that Jenny Gardner is the face of CVID affects approximately one person in 50,000 world - com mon variable immune deficiency (CVID). Not because wide and occurs in males and females equally and in all Ishe is alone in suffering from the disease. Hardly. It’s races. It can occur in infants, young children, adolescents because she has become one of the many strong vocal or even those ages 20 years to 40 years and older. And, advocates for patients with CVID and other immune defi - while peaks of onset occur in children ages 1 to 5 years ciency diseases. Jenny’s story is a glaring example of what and in young adults ages 16 to 20 years, more than two- many individuals endure in their lifelong struggle with this thirds of patients are 21 years or older when diagnosed, 2 disease. It is a story of suffering through years of illness and in the majority of patients, diagnosis is not made until before finally being diagnosed, fighting a losing battle the third or fourth decade of life. 1 with insurance companies to be reimbursed for the That is the case with Jenny, who was sick all of her life expen sive treatment, and continuing to battle the effects but who wasn’t diagnosed until age 45. This was after of the illness despite treatment. being told many times that her illness was psychological and that she was a hypochondriac. “I was so frustrated,” What Is CVID ? explains Jenny. “I told one doctor that he needed to get People with CVID have deficient numbers of serum more creative, as I’d been told that before. But something immunoglobulins (IGs), which are antibodies that fight off was wrong with me, and I wanted answers.” infection. Why people develop CVID is unknown, but the disease is the most common of the immune deficiency Symptoms of CVID dis eases, hence the name “common,” and the degree and Symptoms of CVID can occur in the first few years of type of IG deficiency varies, hence the name “variable.” life, as with Jenny, or they may not develop until the secon d Some patients treated with immune globulin (IG) have a or third decade, or even later. 1 deficiency in both IgG and IgA antibodies, while others Symptoms include recurring infections of the respiratory may be deficient in all three types of antibodies: IgG, IgA tract, such as sinus infections, otitis, bronchitis and pneu - and IgM. 1 monia, which is caused by bacteria, including streptococ -

26 April-May 2012 www.IGLiving.com IG Living! cus pneumonia, hemophilus or moraxella. 3 If severe lung Causes of CVID infections repeatedly occur, it’s possible that permanent Despite 40 years of research, what actually causes CVID damage to the bronchial tree may result and a chronic con - is unknown. It’s possible that genetic factors may be dition of the bronchi can develop, causing widening and involved. For instance, in approximately 20 percent of scarring of the breathing tubes (known as bronchiectasis). 1 CVID patients, a first-degree family member has a selective Patients also frequently experience diarrhea and chronic IgA deficiency. When more than one family member is lambliasis (an intestinal infection). Occasionally, infections affected with CVID, approximately 5 percent of patients of the skin, urinary tract and herpes zoster (shingles) may have a concurrent IgA deficiency. However, no clear pattern occur. And, rarely, patients will experience cytomegaly of inheritance has been observed. 4 virus infection, viral meningitis and tuberculosis. Jenny has no family members who also have been Approximately 20 percent of patients also develop diag nosed with CVID. However, she has tried to make a autoimmune complications, such as rheumatoid arthritis genetic connection, since her physician told her it is not (in the knees, ankles, elbows and wrists), vitiligo (a skin uncommon for this disease to skip a generation or two, condition), hemolytic anemia, thrombocytopenia and and it’s possible that someone in her family may have had neu tropenia. 3 And, CVID patients have an increased risk of the disease but not known it. Her brother “seems to get cancer, especially cancer of the lymp hoid system, skin and everything that comes around,” says Jenny, but he is able gastrointestinal tract. 1 to fight off the infections with the help of antibiotics. Fortunately, unless complications have developed, CVID Research also has shown the involvement of a small patients do not experience physical abnormalities. However, group of genes in some patients. Patients with CVID some patients may have an enlarged spleen and lymph appear to have normal numbers of B lymphocytes, but those fail to mature into plasma cells capable of making the different types of IGs. Other CVID patients lack T CVID affects approximately lym phocyte function, which is necessary for a normal anti - body response. And, yet other CVID patients have excessive numbers of cytotoxic T lymphocytes, whose role in the disease one person in 50,000 is unclear. 1

worldwide and occurs in Diagnosing CVID To diagnose CVID, the patient’s history is ex amined to males and females equally look for types of infections and their severity and frequency, and the family history is explored to determine whether and in all races. there have been relatives diagnosed with primary immun - odeficiency or those who have an unusual susceptibility to infections. This is followed by a physical exam to rule out nodes. And, if chronic lung disease has developed, patients other possible reasons for a high rate of infections, which may not be able to exercise due to decreased lung capacity. could include the suppression of normal immune responses In some cases, gastrointestinal disorders may cause due to malnutrition, injuries such as burns, drugs such as decreased growth in children or weight loss in adults. 1 corticosteroids, diseases such as leukemia and infections Jenny’s symptoms ran the gamut. “If any bug was going such as mononucleosis, measles, chicken pox and AIDS. In around, I got it and was sick the longest. Strep throat was children, the physical examination should show whether a constant problem,” says Jenny. When she was 8, she had the child is growing well and is well-nourished. A severely rheumatic fever, which kept her out of school for three immunodeficient child is likely to look sickly and pale. months, and she was sick so much throughout junior high If an infection is present, samples of mucus, sputum and and high school that the school district sent someone to stool will be cultured to determine the type of germs her home to investigate her parents. “I might have been involved. Common bacteria typically elicit antibodies. the first home-school patient,” says Jenny. As an adult, in Therefore, sinus and respiratory infections, which often addition to repeatedly becoming ill, including a bout with are due to bacteria, suggest an antibody deficiency. On the German measles, she went through three sinus surgeries. other hand, viruses and fungi stimulate T cells. Therefore,

April-May 2012 www.IGLiving.com IG Living! 27 infections caused by viruses and fungi point to a T cell defect. bronchiectasis, physical therapy and postural drainage will Recurrent infections involving the skin or soft tissues often remove the secretions from the lungs and bronchi. 1,5 can be traced to problems with phagocytes, and blood- Jenny was started with gamma globulin injections, and borne infections caused by encapsulated bacteria, including when that didn’t work, she was treated with IVIG. She meningitis, may be linked to complement deficiencies. was first administered 5 grams, and that was increased to If CVID is suspected, lab tests are necessary. These 10, and then 15, 20, 30 and 40. However, she continued include a complete blood count to measure the levels of to get sick, although not quite as often, and she suffered red and white blood cells, as well as platelets; a quantita - from terrible IVIG side effects, including migraines and tive immunoglobulin test that measures IgG, IgM and IgA nausea, and she would often need to recuperate in bed antibody levels in the blood; a blood test to show if the for two or three days afterward. Because of the side blood contains antibodies to the usual childhood immu - effects, Jenny was sent to another doctor for a second nizations (i.e., tetanus, measles, pertussis and diphtheria); opinion: He ran extensive tests and told her that her a complement test using a sample of the blood to indicate dosage was too low. After being prescribed 50 grams, her how effectively the complement system is working; and insurance carrier cut it back to 40 and she again was fre - skin tests that are similar to tuberculosis tests to show how quently ill. Jenny is also frequently treated with antibiotics . well T cells are functioning. 5 While many people never have a problem with reimburse - ment for their IG treatments, unfortunately many others do. Jenny just happens to be one of those. In addition to not Despite 40 years of getting the number of grams of IG she needed, Jenny also began experiencing another problem. Her insurance carrier research, what actually was forcing her doctor to buy the least expensive IG brand , so every month, Jenny was infused with a different IG product. causes CVID is unknown. Then, in 2006, her insurance company canceled her policy, and after her COBRA plan expired, she was unable to con - vert that plan into a private individual plan. And, while she Jenny suffered from infections and illness for years qualified for disability in the state of Florida, where she before she finally got lucky and was diagnosed. This hap - resides, she is ineligible for a Medigap/supplemental insur - pened when she was required to change insurance carri - ance plan until age 66 , leaving her responsible for pay ing ers and was able to pick from a new list of doctors. “I for 20 percent of the cost of her IVIG treatments. She called a friend who is a nurse, and I asked her to tell me appealed, but she was told that “it was cheaper to let me who was the best at allergy and immunology,” says Jenny. die than to treat me,” says Jenny. “That really got to me.” Her friend referred her to a doctor who, after unsuccess - Age 66 is still five years away for Jenny, and she still has no fully treating her for allergies, tested her IG numbers and insurance. Today, she relies on both clinical trials and patient diagnosed her with CVID. assistant programs to receive her IVIG. “Every day, I am fear ful that I will not have either of these available to me, Treatment for CVID and it really worries me,” explains Jenny. While there are a number of treatment options for CVID, the most common is IG therapy to provide the patient with Managing Day to Day protective antibodies. IG almost always brings improvement Even with treatment, CVID patients may still experience of symptoms. Generally, intravenous IG (IVIG) is dosed at infections and their consequences . For Jenny , who was a 400 mg/kg every three to four weeks. Subcutaneous admin - vice president of a national bank in Florida, chronic illness istration of immunoglobulin (SCIG) also can now be caus ed her to leave that job and work as an administrator per formed with a variety of dosing schedules to suit the at her church , where her pastor insisted that her illness and preference of the patient, with the overall goal of adminis - doctor appointments would not be a problem. However, tering a total of 400 mg/kg every three to four weeks. that pastor moved on to another church and his replace - Prophylactic antibiotics may help patients with chronic ment was not so sympathetic. Jenny quit that job as well, sinusitis or chronic lung disease. Antibiotics also may help and since January 2000, she has been on disability. control small bowel bacterial overgrowth. For those with While patients can’t prevent infections from occurring,

28 April-May 2012 www.IGLiving.com IG Living! EnhancingEnhancingg life’s defenses Positive efficacy outcomes &OR0)PATIENTSRECEIVING'AMMAPLEX THEREWERE .OREPORTSOF!CUTE3ERIOUS "ACTERIAL)NFECTION *USTDAYSPERYEAROFSUBJECTS HOSPITALIZED /NLYDAYSPERSUBJECTYEAROUT OFWORKSCHOOLDAYCARE Low IgA levels 4HECONTENTOF)G!IS«GM, Convenient infusion schedule )NFUSIONRATECANBEINCREASED EVERYMINUTESTOAMAXIMUM RATEOFM,KGMIN Robust 3-step virus reduction !NEXTREMELYLOWRISKOFVIRALTRANSMISSION Room temperature storage 'AMMAPLEXCANBESTORED BETWEENª#ANDª#ª&TOª& UNOPENEDFORYEARS

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SERIOUS !%S WERE CONSIDERED RELATED TO 'AMMAPLEX TREATMENT Transfusion-related Acute Lung Injury (TRALI) THROMBOSISANDCHESTPAIN 4HREEOTHERSUBJECTSWITHDREWFROM .ONCARDIOGENIC PULMONARY EDEMA ;4RANSFUSION RELATED !CUTE THESTUDYDUETOTHEFOLLOWING!%SPARESTHESIA BRONCHOSPASM See WARNINGS AND PRECAUTIONS and DOSAGE AND ,UNG)NJURY42!,) =MAYOCCURINPATIENTSFOLLOWING)')6TREATMENT ANDPREGNANCY ADMINISTRATION sections in the Package Insert for 3YMPTOMSFEVER SEVERERESPIRATORYDISTRESS PULMONARYEDEMA important information intended to reduce the risk of acute HYPOXEMIABUTNORMALLEFTVENTRICULARFUNCTION TYPICALLYAPPEAR $URING THIS STUDY NO SUBJECTS TESTED POSITIVE FOR INFECTION DUE renal failure. WITHINTOHOURSFOLLOWINGTREATMENT)F42!,)ISSUSPECTED TEST TOHUMANIMMUNODEfiCIENCYVIRUS()6 HEPATITIS"VIRUS("6 FORANTI NEUTROPHILANTIBODIESINBOTHTHEPRODUCTANDTHEPATIENTS HEPATITIS#VIRUS(#6 OR0ARVOVIRUS" "ECAUSE THIS PRODUCT IS MADE FROM HUMAN PLASMA IT MAY SERUM 3EE 7!2.).'3 !.$ 02%#!54)/.3 -ONITORING CONTAIN INFECTIOUS AGENTS EG VIRUSES AND THEORETICALLY THE ,ABORATORY4ESTS -ANAGEMENTINCLUDESOXYGENANDAPPROPRIATE -ANUFACTUREDBY #REUTZFELDT *AKOB;#*$=AGENTTHATCANCAUSEDISEASE4HERISK VENTILATORYSUPPORT "IO0RODUCTS,ABORATORY,IMITED HASBEENREDUCEDBYSCREENINGPLASMADONORSFORPRIOREXPOSURE $AGGER,ANE TESTING DONATED PLASMA AND INACTIVATING OR REMOVING VIRUSES Laboratory Tests %LSTREE DURING MANUFACTURING $ESPITE THESE MEASURES 'AMMAPLEX &OR APPROPRIATE MONITORING SEE PREVIOUS SECTIONS ON 2ENAL (ERTFORDSHIRE CARRIESANEXTREMELYREMOTERISKOFTRANSMISSIONOFVIRALDISEASES (YPERPROTEINEMIA (EMOLYSISAND42!,) 7$"8 4HE PHYSICIAN SHOULD DISCUSS THE RISKS AND BENEfiTS OF THIS 5NITED+INGDOM PRODUCTWITHTHEPATIENT BEFOREPRESCRIBINGITTOTHEPATIENT Drug Interactions: 0ASSIVE TRANSFER OF ANTIBODIES MAY 53,ICENSE.O TRANSIENTLY INTERFERE WITH THE IMMUNE RESPONSE TO LIVE VIRUS !LL INFECTIONS SUSPECTED BY A PHYSICIAN POSSIBLY TO HAVE BEEN VACCINES SUCH AS MEASLES MUMPS RUBELLA AND VARICELLA 3%% TRANSMITTEDBYTHISPRODUCTSHOULDBEREPORTEDTO   0!4)%.4#/5.3%,).').&/2-!4)/.).0!#+!'%).3%24  53$ISTRIBUTOR OR EMAIL "0,INFO ,ASH'ROUPCOM ON BEHALF OF "IO 0RODUCTS "0,)NC ,ABORATORY,TD Pregnancy Category C: !NIMAL REPRODUCTION STUDIES HAVE 3IX&ORKS2OAD 3UITE NOT BEEN CONDUCTED WITH 'AMMAPLEX )T IS NOT KNOWN WHETHER 2ALEIGH 'AMMAPLEX )MMUNE'LOBULIN)NTRAVENOUS(UMAN ,IQUID 'AMMAPLEXCANCAUSEFETALHARMWHENADMINISTEREDTOAPREGNANT .ORTH#AROLINA SHOULDONLYBEADMINISTEREDINTRAVENOUSLY WOMANORCANAFFECTREPRODUCTIONCAPACITY'AMMAPLEXSHOULDBE 53! GIVENTOAPREGNANTWOMANONLYIFCLEARLYNEEDED PRECAUTIONS General ADVERSE REACTIONS 4HE PRODUCT SHOULD BE USED PROMPTLY AFTER PIERCING THE CAP General !NY PARTIALLY USED OR UNUSED PRODUCT SHOULD BE DISCARDED 'AMMAPLEX )MMUNE 'LOBULIN )NTRAVENOUS (UMAN  ,IQUID 6ISUALLYINSPECTEACHBOTTLEBEFOREUSE$ONOTUSEIFTHESOLUTION CONTAINS NO REDUCING CARBOHYDRATE STABILIZERS EG SUCROSE ISCLOUDYORTURBID3OLUTIONTHATHASBEENFROZENSHOULDNOTBEUSED MALTOSE ANDNOPRESERVATIVE

Hypersensitivity Postmarketing Experience 3EVERE HYPERSENSITIVITY REACTIONS MAY OCCUR )N CASE OF 4HEFOLLOWINGADVERSEREACTIONSHAVEBEENIDENTIfiEDDURINGPOST HYPERSENSITIVITY DISCONTINUE 'AMMAPLEX INFUSION IMMEDIATELY APPROVALUSEOF)')6PRODUCTS AND INSTITUTE APPROPRIATE TREATMENT -EDICATIONS SUCH AS Infusion reactions:HYPERSENSITIVITYEG ANAPHYLAXIS HEADACHE EPINEPHRINESHOULDBEAVAILABLEFORIMMEDIATETREATMENTOFACUTE DIARRHEA TACHYCARDIA FEVER FATIGUE DIZZINESS MALAISE CHILLS HYPERSENSITIVITYREACTIONS mUSHING URTICARIAOROTHERSKINREACTIONS WHEEZINGOROTHERCHEST DISCOMFORT NAUSEA VOMITING RIGORS BACKPAIN MYALGIA ARTHRALGIA Renal dysfunction/failure ANDCHANGESINBLOODPRESSURE %NSURE THAT PATIENTS WITH PRE EXISTING RENAL DEfiCIENCY ARE NOT Renal:!CUTERENALDYSFUNCTIONFAILURE OSMOTICNEPHROPATHY VOLUME DEPLETED BEFORE INFUSION OF )')6 0ERIODIC MONITORING Respiratory:!PNEA !CUTE2ESPIRATORY$ISTRESS3YNDROME!2$3 OF RENAL FUNCTION AND URINE OUTPUT IS PARTICULARLY IMPORTANT 42!,) CYANOSIS HYPOXEMIA PULMONARY EDEMA DYSPNEA IN PATIENTS CONSIDERED TO BE AT INCREASED RISK OF DEVELOPING BRONCHOSPASM ACUTERENALFAILURE2ENALFUNCTION INCLUDINGBLOODUREANITROGEN Cardiovascular: #ARDIAC ARREST THROMBOEMBOLISM VASCULAR "5. AND SERUM CREATININE SHOULD BE ASSESSED BEFORE COLLAPSE HYPOTENSION ADMINISTERING'AMMAPLEXANDATAPPROPRIATEINTERVALSTHEREAFTER Neurological: #OMA LOSS OF CONSCIOUSNESS SEIZURES TREMOR )FRENALFUNCTIONDETERIORATES CONSIDERDISCONTINUING'AMMAPLEX ASEPTICMENINGITISSYNDROME Integumentary: 3TEVENS *OHNSON SYNDROME EPIDERMOLYSIS Information for patients: 0ATIENTS SHOULD BE INSTRUCTED TO ERYTHEMAMULTIFORME DERMATITISEG BULLOUSDERMATITIS  REPORT THE FOLLOWING SIGNS AND SYMPTOMS TO THEIR HEALTHCARE Hematologic: 0ANCYTOPENIA LEUKOPENIA HEMOLYSIS POSITIVE PROFESSIONALDECREASEDURINEOUTPUT SUDDENWEIGHTGAIN flUID DIRECTANTIGLOBULIN#OOMBS TEST RETENTIONEDEMA ANDORSHORTNESSOFBREATHWHICHMAYSUGGEST Gastrointestinal:(EPATICDYSFUNCTION ABDOMINALPAIN KIDNEYDAMAGE  General/Body as a Whole:0YREXIA RIGORS $ECEMBER53'X

there are some things they can do to help to manage their Jenny has been on numerous local and national lobbying disease. For instance, they should eat a normal, balanced trips. “If anyone had told me 10 years ago that I would be diet with sufficient vitamins and fiber. Those with chronic doing this and that I would actually find it very fulfilling, I gastritis or diarrhea should make sure to peel fruit, wash would have said ‘no way,’” exclaims Jenny. “It can be very salads well and cook vegetables, and they should keep daunting, especially in the beginning. When I first went to away from raw eggs, mussels and whipped ice cream. Washington, it was all about me. But it only took one day While immune stimulants have not been proved in any clin ical trials to help, they do in some cases. However, patients should take caution if immunosuppressive substances While there are a number have been prescribed. Light fitness training is recommended, but high-performance sports training is not advis ed. of treatment options for Patients should be sure to get annual checkups and reg - ular blood checks. Should patient s be exposed to someone who is ill, they should keep their distance as best as possible. CVID, the most common is If patients become ill, they should be examined by a physician to determine the causal bacteria type and to be prescribed immune globulin (IG) the appropriate antibiotics. It should be noted that blood screening tests for bacterial antibodies in CVID patients therapy to provide the usually result in false negative readings. A serious infection may be present with or without a fever. patient with protective Another precaution: Live virus vaccines such as polio, measles and yellow fever should not be administered to antibodies. CVID patients. Last, women with CVID who are pregnant may require an increase in the frequency of IgG infusions. And, for the to talk to other patients and hear their stories to realize safety of the child, the choice of antibiotics may be an that I needed to do this for the patient s. I am especially issue. These patients also should discuss the risk of inheri - concerned for the children with this disease. It’s not about tance with their doctor. 3 me anymore; it’s about the entire patient community. Sometimes, I think we are just spinning our wheels, and Future Outlook then I look back and see what has been accomplished, Fortunately, IG therapy combined with antibiotic therapy and I realize that baby steps turn into victories.” has greatly improved the outlook of CVID patients, help - ing them to decrease the number of infections and, in RONALE TUCKER RHODES , MS, is the editor of IG Living many cases, prevent the development of chronic lung magazine. dis ease. However, the future for patients depends on how much damage occurred prior to diagnosis and treatment, References 1. Immune Deficiency Foundation. Patient & Family Handbook for as well as their ability to get treatment. This is why advocacy Primary Immunodeficiencies , 4th ed. Chapter 2: Common Variable efforts to spread the awareness of CVID and its symptoms Immune Deficiency (CVID). Accessed at www.immunedisease.com/ for more timely diagnoses for patients, as well as to enact about-pi/types-of-pi/common-variable-immunodeficiency-cvid.html. 2. Schwartz, RA. Common Variable Immunodeficiency Epidemiology. legislation that will provide adequate reimbursement for Medscape Reference. Accessed at emedicine.medscape.com/ the expensive IG therapy, is crucial. article/1051103-overview#a0199. Jenny , who advocates at both the local and national 3. Schwartz, RA. Common Variable Immunodeficiency Causes. Medscape Reference. Accessed at emedicine.medscape.com/ level , advises that others who advocate for patients need arti cle/1051103-clinical#a0217. to make sure that work is done at home before going to 4. Schwartz, RA. Common Variable Immunodeficiency History. the national level. Her first advocacy trip was to her state Medscape Reference. Accessed at emedicine.medscape.com/ Capitol in Tallahassee, Fla. She was then asked by the arti cle/1051103-clinical#a0218. 5. National Institutes of Health. When the Body’s Defenses Are Missing: Immune Deficiency Foundation to accompany the organi - Primary Immunodeficiency. Accessed at www.nichd .nih.gov/publications/ zation on a lobbying trip to Washington, D.C. Since then, pubs/primary_immuno.cfm.

April-May 2012 www.IGLiving.com IG Living! 31 Life Style Let’s Ta lk! By Trudie Mitschang

I had MG as a baby. I had failure to thrive at 2 weeks old and was given only a 20 to 50 percent chance of survival. When I turned 2 years old, it simply went away. The disease came back strong when I was 12 years old , and I started losing a ton of weight and was catching pneu - monia and viral illnesses. When I was in seventh grade, the disease almost took my life , and I ended up in Children’s Hospital in the intensive care unit (ICU) . I had severe pneumonia , which is thought to be caused by MG . I believe God saved my life. A year later, I ended up in ICU again with a severe case of the flu , fol - lowed by pneumonia. My neurolo - gist ordered a blood test and it was positive for MG . At that time , I was If given the opportunity, few of us Sarah: I have a very tough, seldom started on Mestinon. would choose to be 15 again; the life-threatening, autoimmune neuro - Trudie: Tell me about the time teen years are challenging by anyone’s muscular disease called myasthenia fol lowing your diagnosis. standards, even under the best of gravis (MG) . Right now, the disease Sara: A few months after my cir cumstances. That’s why we were so is stable. I am on very low-dose diagnosis, I had my first MG flare. I inspired by Sarah Eames’s story. Sarah steroids, an immune booster called couldn't walk, stand, sit up or grip is a strong and optimistic young Naltrexone. I have potassium every anything with my hands. I had to hav e woman who was diagnosed with life- altering myasthenia gravis shortly after her 15th birthday. Five years later, the When I was first diagnosed, 20-year-old has resolved not to let the disease define her or limit her choices I was 15, although it is thought that in life. An active college student, Sarah draws strength from her family and I had MG as a baby. her faith, is dedicated to enjoying life to the fullest, and hopes to inspire day and high-dose intravenous physical therapy, speech therapy and other young people living with immune globulin (IVIG) treatments. occupational therapy. I was started chronic disease to do the same. Trudie: How were you diagnosed? on IVIG infusion and it helped me Trudie: Tell me about your disease Sarah: When I was first diagnosed, out a lot. After a week in the hospital, state. I was 15 , although it is thought that I was back to my old self. I was

32 April-May 2012 www.IGLiving.com IG Living! Life Style

started on IVIG infusions a month infusions and how often? later. My doctor then took a scan of Sarah: I have monthly IVIG the thymus and found that I had a infusions at Overlake Hospital condition called thymic hyperplasia: as an inpatient in Bellevue, my thymus was larger than it should Wash . have been. I was started on high Trudie: How has having a doses of prednisone for over a year chronic illness affected your to keep me out of the ICU and also friendships and relationships? had a thymectomy , which is like Sarah: Having a chronic ill - open heart surgery. The disease was ness has not really affected then put into partial remission , and my social life much except for I had restoration of the swallowing when I am sick or when I am muscles that used to be paralyzed, in the hospital . I’m fortunate as well as restoration of vision , which I because my friends are very had partially lost because of weak - understanding. ness of the eye muscles. I was also Trudie: Tell us about your able to get off the high dose of hobbies. steroids. Sarah: I love swimming, hiking, walking, jogging, going to the club, going out I am learning to to movies, going out to eat, Sarah Eames was diagnosed with myasthenia gravis at age 15, despite having symptoms when she was as going out for ice cream, young as 2 weeks old. Today, IVIG helps her to live a accept this disease going to sleepovers, going to nor mal life, and she is pursuing a college degree. youth group and hanging out and not to let MG with friends. Trudie: What helps you maintain Sarah: My advice to younger IVIG define who I am. a positive outlook? patients is to keep going forward Sarah: I stay positive by praying, even when the going gets tough Trudie: As a young person, what going to church and by exercising. and not let their disease define has been the most difficult thing Trudie: What are your plans/goals who they are. about living with chronic illness? for the future? Sarah: The most difficult thing for Sarah: I am working toward a me about living with a chronic illness general education college degree has been accepting it and fighting and pursuing the early childhood off infections. I am learning to education program and also the accept this disease and not to let certified nursing assistant program TRUDIE MITSCHANG is a MG define who I am. I try to stay at Bellevue College. I would love to staff writer for IG Living magazine. positive when the going gets tough. work in a preschool environment o r When I do have a flare, I start back a daycare center. I also love taking on prednisone , which brings me music classes at Bellevue. In my If your life depends on immune right out of the flare .The IVIG spare time , I design Christmas globulin and you have a infusions really help me, as I am not cards for Seattle Children’s unique experience to share, prone to catching pneumonia any - Hospital and try and take art we want to feature you in more and I don’t end up in ICU that classes whenever I can. this column! Email us much anymore. Trudie: What advice would you at [email protected] . Trudie: Where do you get your give to younger IVIG patients?

April-May 2012 www.IGLiving.com IG Living! 33 Life Style Ask Kris By Kris McFalls

Patient : My new insurance policy states that subcuta - instance, consider a prescription that directs the patient to neous immune globulin (SCIG) medication must be pur - take 12.5 milligrams of drug XYZ. If drug XYZ comes in chased through a pharmacy benefits manager (PBM) . As a only 5 or 10 milligram dose s, the retail pharmacist may result, the spec ialty pharmacy that I must use is charging suggest the prescription be filled with 5 milligram tablets. me double the copay that my insurance policy dictates . In The patient would then split one of the tablets and take addition, because my dose requires two different vial two and one- half tablets daily. That way , the patient has sizes, the PBM insists I now have to pay two copays even only one copay but still receives the needed amount though it is one prescription. The specialty pharmacy told of medication. me these kinds of things are becoming a real problem for Unfortunately, this is a little more difficult to do with IG SCIG patients using a PBM. What are my options? products because of the limited number of vial size options , but it can be done. You could speak with your doctor and pharmacist about dosing that will allow you to be treated with one vial size. This solution may require the Medicines that require use of several smaller vial sizes or one larger one. Before you do that, however , a better option would be different doses and that fall to contact your PBM and ask for a supervisor or case man ager. Explain that your physician is happy to change under the pharmacy benefit your prescription to accommodate their copay policy , but you would prefer to keep costs down and not waste may be charged multiple copays. med ication or use up more ancillary supplies that will be needed when using multiple smaller vial sizes. While they Kris: The double copay issue should not be hard to may not change their policy, they may , at least in your resolve . Simply provide a copy of your policy to both your case, see your logic and grant you an exception. specialty pharmacy provider and your PBM and ask them Last , I would suggest contacting the manufacturer of to read it. If you continue to have a problem, inform your your medication. Speak with someone in the company’s employer’s human resource s (HR) department . Chances reimbursement department about your experience to are it is simply a misunderstanding between the PBM and make sure they are aware of the problem. Chances are, the specialty pharmacy that also may be affecting your they have already heard from other patients , and they may co- workers , and your HR department should be able to fix have some other suggestions . it for everyone. While I have not encountered the issue concerning KRIS MCFALLS has two adult sons with chronic copays for different vial sizes, I did speak with a CSL diseases treated with IG. She is formerly a physical Behring representative who says they have recently therapist assistant, and currently is IG Living ’s full- time patient advocate. received s imilar reports from patients using SCIG therapy . Although th is practice is not yet prevalent for IG products, it is quite common for oral medications in retail pharma - cies . When medications fall under the pharmacy benefit, Have a question? patients can be charged multiple copays if filling their Email us at [email protected] . prescription requires the use of different doses . When Your information will remain confidential possible, retail pharmacists can work with the patient and unless permission is given. doc tor to find a solution that will result in one copay. For

34 April-May 2012 www.IGLiving.com IG Living!

Life Style Kaaa-Booms! By Cheryl L. Haggard

“PHEW! We r almost there, Honi!” I texted my husband, Mark. A few moments later , my cell phone chirped, signaling Mark’s response: “Yup! ‘nother 6 grd yr on the books! Way 2 go momma!” Pride swelled in my heart knowing that Mark and I got another one of our three primary immune deficient disease (PIDD ) kids through a year of middle school — and survive d. Sixth grade seem ed to be this mother’s Achilles’ heel , as this was the year Galileo Middle School learn ed , ahem, about the birds and bees (read: ugh!) .Icould have use d my upbringing as comfort and guidance , but my parents got out of “it ”scot - free , or should I call it scotch-free. Flashback to the very late 1970 s. It had to be a Wednesday afternoon because I and the rest of Girl Scout Troop 782 were excited about our meeting after school: We were touring a landmark bakery in my hometown. The mystery of a sudden assembly had us well-scheduled 10- to 11- year-old female-types completely freaked out. Even more puzzling was the fact that our parents were exiting the caf é-gym-atorium just as we were being escorted in. They fell into my lap sobbing. Some girls interrupted laundry duties with “Mom, closed the door behind Troop 782 begged for their mommies , while can we have ‘the talk ’?” what he and the rest of the sixth-grade girls others needed straitjackets. Two really asked me to do was confuse from Eagle Elementary, waving and days later, a fifth -grade informant and frustrate him. Period. blowing comforting kisses. snitched on our parents: While we Panicking, I grabbed a washcloth, Soon after, a public health nurse were being tortured by a public placed it over my head so I didn’t explained why our near future health nurse, our mom s and dad s have to look my firstborn in the eye, included various belts, sanitary passed the proverbial buck while and instructed : “Calvin, this is a nap kins and — gasp! — tampons. sucking down adult beverages. woman’s uterus.” Calvin stood in I sat in shock while my best friend So , when our eldest son, Calvin, quiet and mortified submission as

36 April-May 2012 www.IGLiving.com IG Living! Life Style

younger sister Molly’s rolled-up training expected to do the mundane and her family enjoys visiting grandparents bra became ovaries, and a pair of routine only — and never without and how they go to the lake and “tighty whities” traveled ever so supervision. Never mind that I know watch fireworks for the Fourth of softly down the fallopian tubes. A which antibiotic is good for a sinus July. The word “normal” entered my knock on my washcloth interrupted infection or that I’m quite capable thoughts as I listened to Alexis describe my health lesson. of infusing my daughter’s intravenous their summer plans, dousing any hope “What in the world are you doing, immune globulin . None of this of our friendship going past the fringe Cheryl?” Mark demanded. Calvin mat tered to the Normal Mothers of of her blanket. stood silent; his furrowed brow told America and, like the kids, I was I looked at Alexis’ son and asked : me he was either in shock, confused counting down to the last day of “So , are you going to see the ‘ boom or both. Needless to say, it has taken school before summer vacation so I boom s’ go off at the lake for the Mark two years of constant didn’t have to face them any longer. Fourth of July?” “conception ” therapy to straighten The last day of school finally arrived , He cocked his head with a puzzled out my boy. To this day , I can’t get and I decided to go to the traditional look on his face. Turning to his mom, either of them to do the whites! school wide picnic with my then -third - he reached out both hands and did For us parents of PIDD kids, we get grader, Molly. As I panned the vast the unthinkable, unimaginable and a double whammy. You see, not field of normalcy trying to spy my the completely abnormal: the 4- year - only do we field the nightmarish Molly, the smell of the everyday cliché old tot placed one chubby, jelly-stained “Where do babies come from? ” PB&J s wafted in the soft breeze. This hand on each of Alexis’ well -endowed, question, but we also must answer place even reeks of normal , I griped. uh ,“boom booms ” and began groping excruciatingly painful inquiries about Out of the corner of my eye , I saw his mother. Mortified, Alexis swatted our PIDD kid s’ immune disease. I don’t Molly sharing a blanket with a good away her son’s befuddled limbs and think it’s fair! I don’t mean to be an friend , Wintyr, her little brother and explained : “Uh, my sister and I decided infantile whiner , but do “normal ” — gasp! — her mother. to have a secret code for our privates. parents of pre pubescent children “Will you come join us?” asked So when we were complaining about know how lucky and blessed they Alexis, Wintyr’s mom , welcomingly female ‘stuff’ the kids wouldn’t have really are? making space for me on their blanket. a clue. They figured it out anyway. Since humiliating myself with the Only if you want a freak show to I’m so embarrassed.“ Calvin fiasco, I’ve spent much time interrupt your normalcy, I thought , “Alexis, I feel so close to you,” I mulling over being an “abnormal ” as I nodded and smiled, accepting cried. And as I shared my story of parent. When I walked the halls of Alexis’ offer. washcloths and tube socks , the my PIDD kid s’ junior high school ,I Alexis and I shared small talk as “abnormal ” title I had given myself didn’t know who felt more awkward : the girls ate. Alexis’ son played with a faded away with every laugh we me or the hormonal middle schoolers truck, creating a road with his tortilla shared. who brushed by the bulletin board chips. On the Fourth of July , I got a text I worked on. It felt as if a large talk I began to ease into our picnic, from Alexis that read : “@ the lake . bubble loomed over my head that letting my guard down just a smidge Herez hopin’ ur boom booms r flying read : “Mrs. Haggard doesn’t know as I noshed on smoked turkey on high, exciting & perky! ” how her children got here .“ wheat. I carried my burden throughout “So, do you have plans for this the school year , and then word got summer, Cheryl?” Alexis asked. out among the PTA about how I “Y eah, we’re going to do a little CHERYL L. HAGGARD is a stay-at-home mom and has misguided my child — of all things camping. Nothing too exciting, “I three children, two of whom — using a load of whites. That was shared. “And you? Do you have have CVID. She and her husband, Mark, all I needed. I was banished to the anything exciting in store for your also operate Under the Hood Ministries at Isle of Misfit Mothers and was family?” Alexis responded with how www.underthehoodministries.org.

April-May 2012 www.IGLiving.com IG Living! 37 Life Style SCIG Game Plan By Ever Fecske

I HAVE REACHED a milestone. to do the deed, it wasn’t so bad . M y After six years of infusing immune favorite nurse , Lois, in my immunologist ’s globulin intravenously (IVIG) , I have office trained me . She explained that switched to infusing subcutaneously I would be infusing in the office (SCIG) . It wasn ’t a difficult decision three times, and if I passed the test to make . I suddenly started having and did everything right by the third severe reactions to my every -two- time, I would give my self my fourth week IVIG infusions, so my doctor infusion at home . Lois also taught and I concluded that the subcuta - me a trick. She taught me to hold neous route would be safe r. the needle in one hand and to pinch was able to go home to my cozy The idea of switching to SCIG was my skin tightly where the needle personal space. intriguing. I wouldn’t have to leave would go with my other hand . Then, Changing routines was more diffi - the house! I would no longer have as I poked in the needle, I was to cult tha n I had anticipated. I had to to drive 30 minutes or arrange for blow out my breath and , what do step back and gain some perspective . someone to pick me up on infusion you know , I didn ’t feel a thing! On The whole reason I switched to SCIG day. And, I wouldn’t have the limited the day of my third SCIG infusion ,I was because it was better for my 12 channels on an archaic, squeaky did everything by myself with Lois ’ health. This was going to be the accordion television! I could stream supervision and I passed with flying solution to all of the scary side effects Netfli x if I wanted to. I could order a colors . I made a cake to celebrate! I was experiencing with IVIG. And, pizza to be delivered, and I could pin A week went by , and it was time I was going to be comfortable at and pin and pin on Pinterest.com — to fly solo. I was so anxious . I looked home. I decided the best way to all while I was infusing at home. This around my little apartment, and I cope was to think positively and set was going to be great, I thought! So realized that this was my new infusion up an infusion day game plan. When much freedom! center. I cleaned my kitchen table I am not infusing , everything goes But, when I first switched, I was a twice and laid out all of my supplies back in to the designated ”Infusion little sad . I actually miss ed my four- in the order I needed to use them. Day Box ” in the closet . This way, my hour Benadryl -induced naps ; they It took me about an hour to get apartment is clear of any medical were an excuse to lie down and not com pletely set up, filling the syringes, reminders and more of a home. answer my phone for an extended priming the tubing and finding the But, when infusion day rolls around , period of time. Also, b efore the perfect places to poke the needles. I allow myself to go all out : nothing scary IVIG side effects began , I As I clicked my Freedom 60 pump but me, trash T V and a Zebra looked forward to my infusion day s; to the ”on ” position, I felt accom - Snuggie! they were a n escape from the plished, but also a little sad. There demands of life — a day when I was medical equipment all over my could just worry about myself and house ; I had over lapped my two nothing more. worlds. I felt I had made a mistake. EVER FECSKE was diagnosed I was also nervous. The idea of Was n’t my home supposed to stay with CVID and interstitial sticking six needles into myself was my medical -free zone — with the lung disease in 2004. She is a fashion design student, loves spending less than appealing. After all, who exception of a bag of pill bottles ? time with her fiancé, family and bulldog, wants to voluntarily inflict pain on With IVIG, the medical procedures Dunkin, and can’t get enough of writing, themselves? But, when it came time stayed in the sterile hospital, and I cake decorating and anything that sparkles!

38 April-May 2012 www.IGLiving.com IG Living! Life Style Transitions By Carla Schick

Crossing the Pond for Better Health

Change is often a challenge. Now and then change is necessary, but oftentimes it is a decision patients make to improve their well-being . For instance, they may decide to change their physician if they feel that he or she is not providing them with adequate care. Or, they might switch their intravenous immune globulin (IVIG) brand to lessen the occurrence of side effects. But , occa - sionally, they might have to make the decision to change those elements, and more , nearly simultaneously. Raye Churchill made that choice when she and her husband, John, were determined to pick up stakes and move from the Arizona desert to the United Kingdom. For Raye Raye Churchill was diagnosed in 1992, at age 51, with common variable immunodeficiency (CVID), allergic rhinitis and pulmonary coccidioidomycosis. and John, crossing the pond to improve her health has made all the difference. ciency (CVID) , allergic rhinitis and Social Security, so they began lookin g pulmonary coccidioidomycosis . for a place to enjoy their retirement Coming to Terms For the next 16 years , Raye and years. When Raye was 49 years old, she John did their best to cope with had two open-heart surgeries. After her declining health and increasing A Blessing in Disguise her second surgery, Raye was “over - me dical bills. But with advancing In 2008, the couple “took a trip whelmed with health issues and see - age and the stress of his wife’s to England to look around and make ing specialists to try to get a proper ill ness, John, a native of Great sure John didn’t want to return to diagnosis as to why I wasn’t getting Britain, began to “develop migraines his homeland.” To their surprise, Raye better.” After numerous tests, Raye and syncope events.” He made the says , they “found it quite refreshing was diagnosed in 1992 , at age 51 , decision to stop working. By this time, and to our liking, since the desert with common variable immunodefi - both Raye and John were receiving was causing allergies to begin to

April-May 2012 www.IGLiving.com IG Living! 39 Life Style

appear in both our health patterns.” get to England and need an infusion her health on her birthday , and all During their visit, Raye experienced a with a new doctor in an unfamiliar necessary tests are scheduled at that blessing in disguise when she devel - place. “I needed to be free to settle visit. If she needs treatment and oped an ear infection and had to go in and not need an infusion ASAP,” she’s too ill to travel to her GP’s for medical help. While being treated , explains Raye. office, the doctor will visit her home Raye and John “found the U.K. health - Through her involvement in IG 24 hours a day. When her symptoms care [system] was more to our [taste] Living reader teleconference calls to are beyond her GP’s field of knowl - as seniors.” Raye remembers thinking: learn how patients were dealing edge, she is automatically referred to “If healthcare in the U.K. could fill with SCIG at home, as well as her a specialist, and according to Raye, the bill on medical , it could be the acquaintance with a nurse at the if she “has someone in mind … a answer to remove a great deal of infusion center who had previously referral letter is sent by the GP to stress , and we would be in John’s been involved with SCIG home treat - that specialist. At that point, the country of birth.” ment, Raye successfully made the specialist then does the necessary As soon as they returned home switch from IVIG therapy to SCIG treatment and reports back to the from England , Raye began to research at- home treatments. GP’s office. ” the possibility of moving to another Raye did have one setback, how - country, with a new healthcare system Making the Move ever. It took a little time to find an and different infusion therapy options. Just months after Raye’s visa was immunologist with whom she was She contacted IG Living ’s patient approved , Raye and John moved to happy. Initi ally, her GP referred her advocate , Kris McFalls, who told her England in January 2010 . “ The to “an immunologist closer to where about the Primary Immunodeficiency healthcare system is very different we lived, but we did not like the Association (PIA) . Through the PIA from the U.S. ,” says Raye. “Seniors sys tem in that area, so we asked for website, Raye eventually began are highly respected and well taken a second referral to the Royal London emailing a gentleman in the U.K. care of. Medical insurance is set up Hospital [RLH] where I had originally who was being treated with IVIG so that citizens are given a National contacted Dr. Hilary Longhurst while and who told her that SCIG is the preferred method of delivery by most primary immunodeficiency As soon as they returned home from dis ease ( PID D) patients in the U.K. With Raye’s permission, her informa - England, Raye began to research the tion was forwarded to a specialist nurse in hopes that he would know possibility of moving to another country, more about the Vivaglobin supply in the U.K. The nurse informed her that with a new healthcare system and Vivaglobin was the most widely used SCIG product in England and most different infusion therapy options. of Europe. While she waited for her visa to be approved, Raye decided to make the Health Insurance [NI] number , which I was still in the states.” The RLH switch to SCIG to “see if I could tol - they acquire from their local ‘surgery, ’ was farther from their home, but erate the SCIG with all my allergies a facility that staffs several doctors. ” since seniors are able to ride the and issues … since I had been on From there, patients can make an British railway for a discounted price, IVIG for 16 years.” It was important appointment to see a general practi - the longer distance wasn’t an issue, to Raye that the switch to SCIG be a tioner ( GP ) whose office is close to especially because Raye liked the success because she didn’t want to their residence. Raye ’s GP reviews people and the RLH system better.

40 April-May 2012 www.IGLiving.com IG Living! Life Style

When Raye arrived at the RLH , “it was very refreshing and totally dif - ferent from the one closer to our residence ,” she says . “Blood work, extensive exams and review of all my immunology information from the U.S. [were] sent in advance. The

Raye and John “found the U.K. healthcare [system] was more to our [taste] as seniors.” doctors have been very helpful, friendly and eager to take care of us.” And the best part is that every - thing is “covered under the National Health Service [NHS ], including pre scriptions, blood tests, X-rays and scans.” An added advantage of the NHS is that there is no paperwork for IG Since moving to England, her husband John’s homeland, as well as switching to SCIG, Raye has found she gets improved medical care, has fewer infections and is able to be more active. reim bursement once patients are regis - tered with an NI number. Raye says that “everything is computerized. If with my GP and the immunologist, U.S. She also is a member of the PIA you need to move from one part of they see an improvement.” While and plans to get involved with other the U.K. to another, your medical they were living in the U.S. , they support groups. records are programmed to follow were paying $20,000 per year for ”L ife is less stressful , and we have you.” medical expenses. Now , everything time to relax!” exclaims Raye. Overall, their move to the U.K. is covered. Comfortably settled in a has allowed Raye and John to enjoy suburb of London, Raye still stays in CARLA SCHICK is a staff writer for IG their retirement years and their contact with her old friends in the Living magazine. improved health. “I have had fewer infections and my health has allowed me to be active .… My breathing is If you have a life-transitioning story brought about with the help far better — deeper than it’s been in of IG, we want to share it with our readers. Email us years — and I believe the fresh air at [email protected] . has been good for me. In speaking

April-May 2012 www.IGLiving.com IG Living! 41 Life Style Life Style

Defending Kids Against Bullying

Children who are ill are often prime targets of bullying, and parents can intervene as well as help their kids learn how to deal with it.

By Mark T. Haggard

MY 20 YEARS as an educator have making a more concerted effort to every year, while about 2.1 million given me keen insight into the ado - target these instigators who bully students engage in bullying. More lescent community. Much of it is their way across our children’s cam - than half of students say they have quite inspiring; some of it is not. I puses. A nd as a parent of kids with witnessed incidents of bullying at have broken up a number of fights on an immune deficiency disease, this is school, while one student in seven is high school campuses. Unfortunately , a welcome sight. a victim of bullying. 90 percent of those fights are insti - Nationwide , the statistics attrib - gated by 10 percent of the kids . In uted to bullying are astounding. What Is Bull ying? the past few years , schools and law About 2.7 million students between Dan Olweus, Norwegian psychologist enforcement agencies have started kindergarten and 12th grade are bullied and the creator of the Olweus Bullying

42 April-May 2012 www.IGLiving.com IG Living! Life Style Life Style

Prevention Program, states that “a per - Other researchers suggest that many assert themselves in the face of a son is bullied when he or she is exposed, bullies are abused at home and it is a bully and firmly tell the bully to leave repeatedly and over time, to negative learned behavior. Bullies want to feel them alone. In contrast, kids with low actions on the part of one or more that they are in control — that they self-esteem are not likely to assert other persons, and he or she has are stronger, smarter or better than themselves, making them a direct dif ficulty defending himself or herself.” others. Some see others bullying and tar get of bullies . Confident kids also Bullying may take the form of threats copy the behavior as a way to fit in have a solid group of friends that acts or name -calling, stealing or destroy - with the crowd. Some see bullying as as support against bullies. This is why ing personal property, being excluded a preemptive strike: It is better to be children should become involved in by others, or physical abuse such as the bully than to be bullied. one of the many activities offered at hitting, kicking, shoving or spitting. A Kids who show outward signs of the school — be it sports , band or one study by the National Institutes of illness often become a target of of the other campus clubs — where Health report s that boys are more opportunity. This is because their they will find immediate support. likely to bully their classmates physi - recurring illnesses often cause them For kids who don’t have strong cally, while girls are more likely to to be less physically adept than their self-esteem, the eas iest answer is for bully by spreading rumors, making bullying class mates , who are generally them to avoid the parts of the cam - sexual comments and by snubbing. bigger than those they bully . In addition, pus where the bullies hang out. A newer form of bullying is numerous absences due to illness may However, that isn’t always possible, “cyberbullying,” which occurs when make it harder for sick kids to make so kids should be taught to stand firm hurtful messages are sent via the friends. Unfortunately, in trying to look in the face of bull ies and to tell them Internet, text messages or social net working sites. It is different from traditional face-to-face bullying because Kids who show outward signs of illness messages and images can be sent any time. Types of cyberbullying include often become a target of opportunity. sending mean messages to a person, spreading rumors or lies about a per son online, threatening or harassing out for the best interests of their chil - to leave them alone. This may require another person online, taking pic - dren, parents often insist their kids go practice at home. But, bullies are tures of a person and posting them to school even when they are sick and usu ally insecure themselves , and they online, or hacking into someone’s physically weakened , sometimes unaware will likely back down when confronted. account and using it to send hurtful that they are a target for bullies. If a child becomes the victim of a messages to a third person. Forty -five bully ing incident , it must be reported percent of teens report viewing back- How to Respond to Bullying to school or law enforcement offi - and-forth rude or mean behavior on Ultimately , despite their illness, kids cials. Principals are possessive of their a social networking site . are simply kids, subject to the same campuses , and they do not want social forces as everyone else on their schools to gain a reputation as a Why Do Bullies Pick on Sick Kids ? cam pus. And, parents have an impor - haven for bullies. When my own son Why do bullies threaten and engage tant role to play in protecting their was bullied in fifth grade , we brought in physical violence? According to kids against bullying. The most the behavior to the attention of the Olweus , many kids bully because they important defense against bullying is principal of the school. The next fall , have a strong need for power and to help kids maintain a strong sense the bully was no longer enrolled. dominance. Some find satisfaction of self-esteem. Students who carry Although a child may complain that from causing injury to another person , themselves well on campus are not reporting the incident seems like “tat - or they receive some material and psy - often targets of bullies. Self-confi - tling” or that the bully has threatened chological reward for their actions . dent students are more likely to further harm, telling the authorities

April-May 2012 www.IGLiving.com IG Living! 43 Life Style Life Style

puts the bully on notice that the con - they believe is worse than bullying. this behavior by going to the “con tact” sequences of his or her next act If a child becomes a victim of cyber - page and sending copies of the might be a juvenile detention facility. bullying , the authorities may need to unwanted messages. Unfortunately, become involved if it does not stop. if these actions do not work at deter - Responding to Cyberbullying Most importantly, children must be ring a cyberbully, the last option may As children begin their lives told not to retaliate . R etaliation gives be to change the child’s e mail “on line,” it is important they under - the bully a sense of gratification and address . stand that cyberbullying, like any leads to further nasty messages and other form of bullying, is wrong, no continued retaliation. This back-and- An Arsenal Against Bullying matter who started it. Children forth behavior on line is referred to as Although children with an illness should be completely insulated while “flaming,” and it is attractive to may be attractive target s for bullies , working on their computers. They those who are likely to join in on the there are defense mechanisms in should have passwords that are diffi - unwanted behavior. Rather than place to protect them. The first and cult to decipher, and they should retaliate, kids should either ignore most important line of defense is never share their passwords, except bullies or respond with short, unemo - instilling in them a strong sense of with their parents. Furthermore , tional responses like : “Knock it off.” self-worth. If a bully continues his or they should never open anything Parents also can block the e mail her unwanted behavior, school from someone they do not know. address of the bully . However, if administrators and law enforcement Nor should children ever share unwanted communication continues , are bound by the law to intervene on personal information on line, since parents should keep messages as parents’ behalf. And p arents are anything they post stays in cyber - evi dence in building a case against responsible for the behavior of their space forever. the bully . The parents of the bully are children ; even the “hardest” parents can be mollified when threatened with jail time. Especially on line, The most important defense against where cyberbullying has become prevalent, parents are not alone in bullying is to help kids maintain a seeking help. They have a powerful arsenal to defend their children strong sense of self-esteem. from those who wish to threaten and abuse.

Parents can become more deeply legally responsible for their children’s MARK T. HAGGARD is a high school involved in their kids’ on line activities actions , and if they do not do any - teacher and football coach, and has three children, two of whom have CVID. He by keeping their computer in a high- thing to make it stop, they may face and his wife, Cheryl, also operate Under traffic area of the house. In addition, legal consequences. the Hood Ministries at www.underthe kids should be required to “friend” If a cyberbully is sending messages hoodministries.org. their parents on their social network - anonymously or with a fake name , ing sites so the parents can see the Internet Service Provider can Sources messages their kids receive . This is not track the sender . I f the messages are Clemson University and Hazelden Foundation. spying on them ; it is for their protec - threatening or damaging, parents Olweus Bullying Prevention Program . Accessed at www.olweus.org . tion. Kids also should be encouraged can go to the police. Many e mail United States Department of Health and to speak up if they feel they are being providers or social networking sites Human Services . Stop Bullying. Accessed bullied on line. Many teens will not tell will shut down an account if they are at www.stopbullying.gov . their parents about cyberbullying out provided evidence that it is being Bullying Statistics . Accessed at www.bullyingstatistics.org . of fear that the parents will take their used for cyberbullying . Parents can Teen Bullying . Accessed at computer privileges away — a fate notify social networking sites about www.teenhelp.com .

44 April-May 2012 www.IGLiving.com IG Living! Sources Book Co rner

Living With Chronic love them will see that they are not alone. New York Illness: A Prescription Times bestselling author Richard A. Cohen spent for Advocacy three years chronicling the lives of five diverse “citi - Authors: Jennifer C. Jaff, Shelley C. zens of sickness”: Denise, who suffers from ALS; Stoll, Yi-An Ko, Emily Youatt, Nicole Buzz, whose Christian faith helps him deal with his Netkin-Collins, Noreen M. Clark non-Hodgkin’s lymphoma; Sarah, a determined young Publishers: Advocacy for Patients woman with Crohn’s disease; Ben, a college student with Chronic Illness, with muscular dystrophy; and Larry, whose bipolar www. advocacyforpatients.org disorder is hidden within. The five are different in age and gender, race and economic status, but they are Almost half of all Americans have a chronic health determined to live life on their own terms. Though con dition, and by the year 2030, half of the United each individual’s illness wreaks havoc in a different States population will have a chronic illness. People way, Cohen shows how their experiences are strik - with chronic conditions are the most frequent users of ingly similar and offers lessons for all — on self- health care in the United States, accounting for 81 percent deter mination, on courage in the face of adversity of hospital admissions, 91 percent of prescriptions filled and public igno rance, on keeping hope alive, and and 76 percent of all physician visits. From March 2008 on finding strength and peace under the most difficult to March 2009, more than 1,500 patients with chronic of circumstances. illnesses completed a detailed survey reporting on their experiences as a patient and, in particular, the obstacles Diabetes Weight Loss — they face and the strategies they use to try to surmount Week by Week: A Safe, these obstacles. The rich trove of data that resulted sug gests important directives for advocates. In this paper, Effective Method for that data is summarize d and evaluated to reveal what Losing Weight and these advocates believe are important points to be Improving Your Health con sidered when seeking to improve the experience, both within and outside of the healthcare system, of individuals with chronic illnesses. Author: Jill Weisenberger, MS, RD, CDE Publisher: American Diabetes Association

Strong at the This book is a no-fad, no-trick method to lose Broken Places weight and keep it off for a lifetime. Written by a reg istered dietitian and a diabetes educator with more Author: Richard Cohen than 20 years’ experience helping people meet their Publisher: HarperCollins, weight and health goals, it is a one-year journey to www.harpercollins.com weight control while managing diabetes. Each week, readers learn new skills that build on previous lessons, including how to set reasonable, achievable goals, recover from dietary setbacks, avoid low blood sugar Strong at the Broken Places was written out of the while losing weight and exercising, snack with a purpose, desires by many who suffer from chronic illness to share and ask for the support they need. The book will be their stories in the hope that the sick and those who available in July.

April-May 2012 www.IGLiving.com IG Living! 45 Sources

For a more comprehensive list of resources, visit the Resources page at www.IGLiving.com.

General Resources Disease-State Resources These organizations provide information about various disease states, which can be Ataxia Telangiectasia (A-T) found by conducting a search of the disease state name. • Advocacy for Patients with Chronic Illness: www.advocacyforpatients.org Websites • The Alliance for Biotherapeutics (fair access to plasma therapies): • A-T Children’s Project: www.atcp.org www.bioalliance.org • American Autoimmune Related Diseases Association (AARDA): www.aarda.org Chronic Inflammatory Demyelinating • American Chronic Pain Association (ACPA): www.theacpa.org Polyneuropathy (CIDP) • Band-Aides and Blackboards: www.lehman.cuny.edu/faculty/jfleitas/bandaides Websites • GBS/CIDP Foundation International: www.gbs-cidp.org • eMedicine from WebMD: emedicine.medscape.com • The Neuropathy Association: www.neuropathy.org • FamilyDoctor.org: www.familydoctor.org • Johns Hopkins Medicine: www.hopkinsmedicine.org Evans Syndrome • KeepKidsHealthy.com (pediatrician’s guide to children health and safety): www.keepkidshealthy.com Online Peer Support • Kids Health (medical and emotional impact of caring for an ill child): • Evans Syndrome Research and Support Group: www.evanssyndrome.org www.kidshealth.org/parent/system/ill/seriously_ill.html • Mayo Clinic: www.mayoclinic.com Guillain-Barré Syndrome (GBS) • National Committee for Quality Assurance (detailed report cards on health Websites plans, clinical performance, member satisfaction and access to care): • GBS/CIDP Foundation International: www.gbs-cidp.org www.ncqa.org • The Neuropathy Association: www.neuropathy.org • National Infusion Center Association: www.infusioncenter.net Online Peer Support • National Institute of Neurological Disorders and Stroke (NINDS): • GBS Support Group: www.gbs.org.uk www.ninds.nih.gov/disorders/disorder_index.htm • GBS/CIDP Foundation International Discussion Forums : • National Institutes of Health: www.gbs-cidp.org/forums http://www.niams.nih.gov/Health_Info • National Organization for Rare Disorders ( disease-specific support groups and virtual communities for patients and caregivers): www.rarediseases.org Idiopathic Thrombocytopenic Purpura (ITP) • Office of Rare Diseases Research: rarediseases.info.nih.gov Websites • ITP Support Association – UK: • Patient Advocate Foundation (patient access to care, maintenance of www.itpsupport.org.uk employment and financial stability): www.patientadvocate.org • National Heart, Lung and Blood Institute: The nonprofit Patient Services Incorporated, www.nhlbi.nih.gov/health/dci/Diseases/Itp/ITP_WhatIs.html www.patientservicesinc.org , specializes in health insurance premium, pharmacy co-payment and • Platelet Disorder Support Association: www.pdsa.org co-payment waiver assistance for people with chronic illnesses. (800) 36 6-7741 Kawasaki Disease • WebMD (medical reference): www.webmd.co m Websites IG Manufacturer Websites • American Heart Association : • Baxter: www.baxter.com www.heart.org/HEARTORG/Conditions/More/CardiovascularConditionsofChildhood/ Kawasaki-Disease_UCM_308777_Article.jsp#.T1T2boePWE0 • CSL Behring: www.cslbehring.com • American Academy of Family Physicians: • Grifols: www.grifolsusa.com www.aafp.org/afp/2006/1001/p1141.html • Kedrion: www.kedrionusa.com • Kawasaki Disease Foundation: www.kdfoundation.org • Octapharma: www.octapharma.com • KidsHealth: http://kidshealth.org/parent/medical/heart/kawasaki.html

46 April-May 2012 www.IGLiving.com IG Living! Sources

Mitochondrial Disease • Myositis Support Group: www.myositissupportgroup.org Websites • Myositis Support Group – UK: www.myositis.org.uk • United Mitochondrial Disease Foundation: www.umdf.org • MitoAction: www.mitoaction.org Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus (PANDAS) Multifocal Motor Neuropathy (MMN) Websites • Guide to P.A.N.D.A.S. Syndrome: www.pandas-syndrome.webs.com Websites • The Neuromuscular Center at Washington University: • P.A.N.D.A.S. Network: pandasnetwork.org http://neuromuscular.wustl.edu • Behavioural Neurotherapy Clinic – Australia: www.adhd.com.au/PANDAS.htm • The Neuropathy Association: www.neuropathy.org Pemphigus and Pemphigoid Multiple Sclerosis (MS) Websites Websites • The International Pemphigus and Pemphigoid Foundation : www.pemphigus.org • All About Multiple Sclerosis: www.mult-sclerosis.org/index.html • Multiple Sclerosis Association of America: www.msaa.com Peripheral Neuropathy (PN) • Multiple Sclerosis Foundation: www.msfocus.org/learn-about-multiple-sclerosis.aspx Websites • National Multiple Sclerosis Society: www.nationalmssociety.org The Neuropathy Association, www.neuropathy.org , is devoted exclusively Online Peer Support to all types of neuropathy, which affects • Friends with MS: www.FriendsWithMS.com upwards of 20 million Americans. The Association’s mission is to increase • MSWorld’s Chat and Message Board: www.msworld.org public awareness of the nature and extent of PN, facilitate information exchanges about the disease, advocate the need for early intervention and support research into the causes and treatment of neuropathies. Myasthenia Gravis (MG) (212) 69 2-0 66 2

Websites and Chat Rooms • Neuropathy Action Foundation: www.neuropathyaction.org • Myasthenia Gravis Foundation of America (MGFA) : www.myasthenia.org • Calgary Neuropathy Association: www.calgaryneuropathy.com Online Peer Support • Genetic Alliance: www.geneticalliance.org Primary Immune Deficiency Disease (PIDD) Myositis Websites Websites The Immune Deficiency Foundation (IDF), www.primary immune.org , is the national patient organization dedicated The mission of The Myositis Association, to improving the diagnosis, treatment and quality of life of www.myositis.org , is to find a cure for inflammatory and other related persons with primary immunodeficiency diseases through myopathies, while serving those advocacy, education and research. (800) 296-4433 affected by these diseases. (703) 299-4850 The Jeffrey Modell Foundation, www.info4pi.org , is dedicated to • International Myositis Assessment and Clinical Studies Group: early and precise diagnosis, meaningful treatments and, ultimately, http://www.niehs.nih.gov/research/resources/collab/imacs/main.cfm cures for primary immunodeficiency. (212) 81 9- 0200

The Cure JM Foundation • The National Institute of Child Health and Human Development (NICHD), www.curejm.com www.nichd.nih.gov , is part of the National Institutes of Health. Go to the (760) 487-1079 “Health Information and Media” tab on the website and do a search under • American Academy of Allergy, Asthma & Immunology: www.aaaai.org Online Peer Support • International Patient Organization for Primary Immunodeficiencies (IPOPI): • Juvenile Myositis Family Support Network: www.ipopi.org www.curejm.com/family_support/index.htm • Michigan Immunodeficiency Foundation: • Myositis Association Community Forum: www.myositis.org www.facebook.com/groups/108048062584350 April-May 2012 www.IGLiving.com IG Living! 47 Sources

• National Institute of Child Health and Human Development (NICHD) (Click on • U.S. Department of Health and Human Services, Office of Civil Rights: "Health Information" then "A to Z health & human development topics" and www.hhs.gov/ocr/office/news/2008/discrimdisab.html select "P" for "Primary Immunodeficiency"): www.nichd.nih.gov Spells out your rights under Section 504 of the Rehabilitation Act. • New England Primary Immunodeficiency Network: www.nepin.org Medical Research Studies • Rainbow Allergy-Immunology: www.uhhospitals.org/tabid/132/Default.aspx • ClinicalTrials.com: www.clinicaltrials.com This site has a registration form to request that you be notified about • Team Hope (for families and patients in New England): www.teamhope.info recruitment for future studies. O nline Peer Support • ClinicalTrials.gov: www.clinicaltrials.gov • IDF Common Ground: www.idfcommonground.org A registry of federally and privately supported clinical trials conducted in • IDF Discussion Forum: http://idffriends.org/forum the United States and around the world. • IDF Friends: http://idffriends.org Food Allergies • Allergic Disorders: Promoting Best Practice: www.aaaai.org • Jeffrey Modell Foundation Message Board: www.info4pi.org • American Partnership for Eosinophilic Disorders: www.apfed.org • Rhode Island peer group: http://health.groups.yahoo.com/group/RhodeIslandPIDD • Food Allergy and Anaphylaxis Network: www.foodallergy.org Scleroderma • National Institutes of Health, National Institute of Allergy and Infectious Diseases (2004). Food Allergy: An Overview (NIH Publication No. 04-5518): Websites www.niaid.nih.gov/topics/foodallergy/Pages/default.aspx • Scleroderma Foundation: www.scleroderma.org • Sicherer, S.H. (2006). “The Complete Peanut Allergy Handbook: Understanding • Scleroderma Research Foundation: www.srfcure.org and Managing Your Child’s Food Allergies,” Johns Hopkins Press. • Scleroderma Center: • World Allergy Organization: www.worldallergy.org http://www.hopkinsmedicine.org/rheumatology/clinics/scleroderma_center.html Product Information Online Peer Support • Influenza and the influenza vaccine: www.cdc.gov/flu or call (800) CDC-INFO: • Scleroderma Support Forum: curezone.com/forums/f.asp?f=404 (800) 232-4636 • International Scleroderma Network: www.sclero.org/support/forums/a-to-z.html • IVIG Carimune NF: www.cslbehring-us.com/docs/586/523/CarimuneNF_Oct%2010_PI,1.pdf Stiff-Person Syndrome (SPS) • IVIG Flebogamma 5% DIF and 10% DIF: www.grifols.com/portal/en/US/bioscience?div=8883 Websites • IVIG/SCIG Gammagard Liquid: www.gammagardliquid.com • American Autoimmune Related Diseases Association Inc.: www.aarda.org • IVIG Gammagard S/D: • Genetic Alliance: www.geneticalliance.org www.baxter.com/patients_and_caregivers/products/gammagard_sd_5.html • Living with Stiff Person Syndrome (personal account): www.livingwithsps.com • IVIG/SCIG Gammaked: www.gammaked.com • Stiff Person Syndrome: www.stiffpersonsyndrome.net • IVIG Gammaplex: www.gammaplex.com Other Resources • IVIG/SCIG Gamunex-C: www.gamunex-c.com/html/patient-what-is-gamunex-c.htm Education and Disability Resources • IVIG Octagam: www.octapharma.com/en/about-octapharma/products-therapies/ immunotherapy/octagamr10.html • Americans with Disabilities Act of 1990: www.ada.gov Provides protection for people with disabilities from certain types of discrimination, • IVIG Privigen: www.privigen.com and requires employers to provide some accommodations of the disability. • SCIG Hizentra: www.hizentra.com • Continuation of Health Coverag e—Consolidated Omnibus Budget Pump and Infusion Sets Websites Reconciliation Act (COBRA): www.dol.gov/dol/topic/health-plans/cobra.htm • EMED Corporation: www.safetymedicalproducts.com • Disability.gov: www.disability.gov U.S. Federal government’s disability-related information and resources. • Graseby Marcal Medical: www.marcalmedical.com • Individuals with Disabilities Education Improvement Act of 2004: • Intra Pump Infusion Systems: www.intrapump.com http://idea.ed.gov/explore/home • Micrel Medical Devices: www.micrelmed.com • National Disability Rights Network: www.ndrn.org . This website offers a search tool • Norfo lk Medical: www.norfolkmedical.com to find resources in your state to assist with school rights and advocacy. • Repro Med Systems, Inc: www.rmsmedicalproducts.com • Social Security: www.ssa.gov/disability • Smith Medical: www.smiths-medical.com/brands/cadd • U.S. Department of Education Website: www.ed.gov . This federal government website offers a parents section titled “My Child’s Special Needs.” Have something to add to these pages? Please send your suggestions for additions to the IG Living Resource Directory to [email protected]. 48 April-May 2012 www.IGLiving.com IG Living! CSL Behringg ThromboticThromboticc Events ThromboticThrombotic eevents may occur with use of human immune globulin products3-5. PPatientsatients at BRIEF SUMMSUMMARYMARRYY OF PRESCRIBING INFORMAINFORMATIONAATIONTION increased risriskk may include those with a history of atherosclerosisatherosclerosis,rosis, multiple cardiovascularcardiovascular risk HizentraHizentra®, factorsactors,, advadvancedanced ageage,, impaired cardiac output, hypercoagulablehypercoagulaable disordersdisorders,, prolonged periods of immobilization,immobilizzation, and/or known or suspected hyperviscosity.hyperviscositysity. Because of the potentially Immunee Globulin SubcutaneousSubcutaneeous increased risksk of thrombosis,thrombosis, consider baseline assessmentt of blood viscosity in patients at risk for hypehyperviscosity,rviscosity, including those with cryoglobulins,cryoglobulins, fastingfaasting chylomicronemia/markedlychylomicronemia/markedly (Human),(Humann), 20% Liquid high triacylglycerolstriacylglycerols (triglycerides), or monoclonal gammopathies.gammopathiesathies. ForFor patients judged to be at risk of devdevelopingveloping thrombotic eventsevents,, administer HizentraHizentra at the minimum raterate practicable.practicable. BeforeBefore prescribing,prescrribing, please consult full prescribingprescribingg information, a brief Hemolysis summary of whichw follows.follows. Some text and referencesreferences referrefer to full prprescribingescribing HizentrHizentraa cann contain blood group antibodies that may actct as hemolysins and induce inin information. vivovivoo coatingg of red blood cells (RBCs) with immunoglobulin,bulin, causing a positive direct 1 INDICATIONSINDICAATIONSTIONNS AND USAGEUSAGE antiglobulin (Coombs’) test result and hemolysishemolysis..6-86 8 DelayeDelayedd hemolytic anemia can develop HizentrHizentraa is an IImmunemmune Globulin Subcutaneous (Human) ((IGSC),IGSC), 20% Liquid indicated subsequent to immune globulin thertherapyapy due to enhancedd RBC sequestration,sequestration, and acute as replacement thertherapyapy for primary humorhumoralal immunodeficiencycy (PI). ThisThis includes,includes, but is hemolysishemolysis,, consistentcoonsistent with intravascularintravascular hemolysis,hemolysis, has beenen reported.9 not limited toto,, thethhe humorhumoralal immune defect in congenital agammaglobulinemia,mmaglobulinemia, common Monitor recirecipientsipients of HizentrHizentraa for clinical signs and symptomstoms of hemolysis.hemolysis. If these are vvariableariable immunoimmunodeficiency,odeficiency, X-linkX-linkeded agammaglobulinemia, Wiskott-AldrichWisskott-Aldrich syndromesyndrome,, and present afteafterr a HizentrHizentraa infusion, perform appropriate confirmatorynfirmatory laboratorylaboratory testing. If severe combinedd immunodeficienciesimmunodeficiencies.. transfusiontransfusion is indicated for patients who develop hemolysissis with clinically compromising 4 CONTRAINDICATIONSCONTRAINDDICAATIONSTIONS anemia afterr receiving HizentrHizentra,a, perform adequate cross-matchingmatching to avoid exacerbating HizentrHizentraa is contraindicatedcontrraindicated in patients who have had an anaphylacticanapphylactic or severe systemic on-going hemolysis.hemolysis. reaction to the administradministrationation of human immune globulin or to components of HizentrHizentra,a, Transfusion-RelatedTransfusion-Relatedn-Related Acute Lung Injury (TRALI) such as polysorbatepolysorbate 80. Noncardiogeniconcardiogeenic pulmonary edema may occur in patientss administered human immune HizentrHizentraa is contrcontraindicatedaaindicated in patients with hyperprolinemia becauseause it contains the stabilizer globulin products.productsducts.10 TRALI is characterizedcharacterized by severe respiratoryrespiraatory distress,distress, pulmonary edema, L-proline (seesee DescriptionDeescriipption [11][11]).) hypohypoxemia,xemia, normaln left ventricular function, and fever.feverr.. Typically,Typiccallyy,, it occurs within 1 to 6 hours HizentrHizentraa is contraindicatedcontraindicated in IgA-deficient patients with antibodiestibodies against IgA and a followingg trtransfusion.aansfusion. PPatientsatients with TRALI mayy be managedagedg usingg ooxygenxygenyg therapytherapypy with history of hypersehypersensitivityensitivity (seesee DDescriptionescriipption [11][11]).) adequate veventilatoryntilatory support. 5 WARNINGSWARNINGS AND PRECAUTIONSPRECAUTIONS Monitor HizeHizentraentra recipients for pulmonary adverse reactions.reactionss. If TRALI is suspected, perform 5.1 HyperHypersensitivitysenssitivity appropriate tests for the presence of anti-neutrophil antibodiesbodies in both the product and Severe hypersenhypersensitivitysitivity reactions may occur to human immuneimmunne globulin or components patient’patient’ss serserum.um. of Hizentra,Hizentra, suchh as polysorbate 80. In case of hypersensitivity,hypersensitivitytyy,, discontinue the HizentraHizentra 5.4 TTransmissibleransmmissible Infectious Agents infusion immediaimmediatelyately and institute appropriate treatment. Because HizentrHizentraentra is made from human plasma, it may carryy a risk of transmittingtransmitting infectious Individuals with IgA deficiency can develop anti-IgA antibodiess and anaphylactic reactions agents (e(e.g.,.g., viruses,viruses, and theoretically,theoretically, the Creutzfeldt-JCreutzfeldt-Jakobakob disease [CJD] agent). TheThe risk (including anaphanaphylaxishylaxis and shock) after administradministrationation of blood components containing IgA. of infectiouss agent trtransmissionansmission has been reduced by screeningreening plasma donors for prior PatientsPatients with knknownown antibodies to IgA may have a greater risrisksk of developing potentially exposure to certain virusesviruses,, testing for the presence of certaintain current virus infectionsinfections,, and severe hypersenshypersensitivitysitivity and anaphylactic reactions with administradministrationtration of Hizentra.Hizentra. HizentraHizentra including virusus inactivinactivation/removalation/removal steps in the manufactumanufacturingring process for HizentrHizentra.a. contains )50 mcg/mLmccg/mL IgA (seesee DescriptionDescriipption [11][11]).) Report all infectionsnfections thought to be possibly trtransmittedansmitted by HizentraHizentra to CSL Behring 5.2 Aseptic MeningitisMeeningitis SyndromeSyndrome (AMS) Pharmacovigilancegilance at 1-866-915-6958. AMS has been reportedreported with use of IGIV1 or IGSCIGSC.. TheThe syndrome usually begins within severalseveral 5.5 LaborLaboratoryaatory TTestsests hours to 2 days ffollowing immune globulin treatment. AMS is characterizedchharacterized by the following VVariousarious paspassivelysively trtransferredansferred antibodies in immunoglobulinimmunoglobulin preparpreparationsations may lead to signs and symptoms:symptoms: severe headache,headache, nuchal rigidity,rigidity, drowsiness,drowsinesswsiness, fever,feverr,, photophobia, misinterpretamisinterpretationation of the results of serological testing. painful eye movemovements,ements, nausea, and vomiting. Cerebrospinal flfluid (CSF) studies frequently 6 ADVERSEADVERSSE REAREACTIONSCTIONS show pleocytosiss up to severseveralal thousand cells per cubic millimeter,millimetereterr,, predominantly from the TThehe most cocommonommon adverse reactions (ARs), observed in *5% of study subjects receiving granulocyticgranulocytic series,serieses, and elevelevatedated protein levels up to severalseveral hundredhunndred mg/dL. AMS may occur HizentrHizentra,a, wewereere local reactions (i.e(i.e.,., swelling, redness,redness, heat, pain, and itching at the injection site), headache,headacche, vomiting, pain, and fatiguefatigue.. more frequently in association with high doses (>2 g/kg) and/oror rrapidapid infusion of immune globulin product.product. 6.1 Clinicalal TTrialsrials Experience PatientsPatients exhibitiexhibitingng such signs and symptoms should receivee a thorough neurological Because clinicalnical studies are conducted under widely vvaryingaryinng conditions,conditions, AR ratesrates observed examination, incincludingluding CSF studiesstudies,, to rule out other causes off meningitismeningitis.. Discontinuation in clinical studiesudies of a product cannot be directly compared to rratesates in the clinical studies of of immune globuglobulinulin treatment has resulted in remission of AMS within severalseveral days without another prodproductduct and may not reflect the rratesates observed in clinical prpractice.actice. sequelae.sequelae. TThehe safety off HizentrHizentraa waswas evaluatedevaluated in a clinical study forr 15 months in subjects with PI 5.35 3 Reactions Reported to Occur WWithith IGIV TTreatmentreatment who had beenbeeen treated previouslyp y with IGIV everyy 3 or 4 weeks.weeeks. TheThe safetyy analysesy included TheThe following rereactionsactions have been reported to occur with IGIVV treatment and may occur 49 subjects in the intention-to-treat (ITT) population. TheThee ITT population consisted of all with IGSC treatment.treatmment. subjects whoo received at least one dose of HizentrHizentraa (seesee CClinicallinical StudiesStudies [14][14]).) Renal Dysfunction/FailureDysfuncttion/Failure Subjects werere treated with HizentrHizentraa at weekly doses rangingrangiing from 66 to 331 mg/kg body Renal dysfunctiodysfunction/failure,on/failure, osmotic nephropathynephropathy,, and death mamayay occur with use of human weight duringng the wwash-in/wash-outash-in/wash-out period and from 72 too 379 mg/kg during the efficacy immune globulinn productsproducts.. Ensure that patients are not volume depleted and assess renal period. TheThe 49 subjects received a total of 2264 weekly infusionsfusions of HizentrHizentra.a. function, includinincludingng measurement of blood urea nitrogen (BUN) and serum creatininecreatinine,, before No deaths orr serious ARs occurred during the study.study. TwoTwo subjectssubjects withdrew from the study the initial infusioinfusionn of HizentrHizentraa and at appropriate intervintervalsals thereafter.thereeafter. due to ARsARs.. One subject experienced a severe injection-site reaction one day after the third PeriodicPeriodic monitormonitoringing of renal function and urine output is particparticularlycularly important in patients weekly infusion,sion, and the other subject experienced modermoderateaate myositis.myositis. Both reactions were judged to have a potential increased risk of developing acute renalnal failurefailure..2 If renal function judged to bee “at least possibly related” to the administradministrationatioon of Hizentra.Hizentra. deteriordeteriorates,ates, conconsidersider discontinuing HizentrHizentra.a. ForFor patients judgejudgeded to be at risk of developing TableTable 2 sumsummarizesmmarizes the most frequent adverse events (AEs)AEs) (experienced by at least 4 renal dysfunctionn because of pre-existing renal insufficiency or predisposition to acute renal subjects), irrespectiveirrrreespective ooff ccausalityausalittyy. Included are all AEs anda those considered temporallytemporally failure (such as thoset with diabetes mellitus or hypovolemia, thosethhose who are overweight or associated with the HizentrHizentraa infusion, i.e.,i.e., occurring during or within 72 hours after the end use concomitantt nephrotonephrotoxicxic medicinal products,products, or those whoo are over 65 years of age), of an infusion.on. Local reactions were the most frequent AEsA observed, with injection-site administer HizenHizentrantra at the minimum rrateate prpracticable.acticable. reactions (i.e.,(i.ee., swelling, rednessredness,, heat, pain, and itching at the site of injection) comprising 98% of locall reactionsreactions..

TTableable 2: IncidenceInciddence of Subjects WWithith AdverseAdverse EventsEvents (AEs)* (Experienced 6.2 PPostmarketingostmarkarketing Experience by 4 or MorMoree Subjects) and Rate per Infusion, IrrespectiveIrrrespectiveespective of Causalityy (ITT BecauseBecause ppostmarketingostmarrkkettiing rreportingeporttiing ofof adverseadverrsse rreactionseactions iiss vvoluntaryoluntarryy aandnd ffrfromrom a ppopulationopulattiion PPopulation)opulation) ooff uuncertainncertain size,sizee,, iitt isis nnotot alwaysalwayyss ppossibleossible ttoo rreliablyeliabllyy eestimatesttiimate tthehe frffrequencyrequenccyy ooff tthesehese AAEs* Occurring During rereactionsactionss oorr eestablishstabliishsh a ccausalausall relationshiprelationshiipp toto pproductroduct eexposure.exxposure. All AEs* oor WithinWithin 72 HoursHours of TheThe followingg adverse reactions have been identified and reportedported during the postmarkpostmarketingeting Infusion use of IGIV productsroducts11: Number Number Number Number s InfusionInfusion reactions:rreeacttiions: Hypersensitivity (e.g.,(e.g., anaphylaxis),, headache,headache, (Rate†) (Rate†) (%) of (%) diarrhea diarrhea,a, tachycardia, fever,feverr,, fatigue,fatigue, dizziness,dizziness, malaise,malaise, chills,chills, flushing, of AEs of AEs AE (*4 SubjectSubjects)ts) Subjects of Subjects (n=2264 (n=2264 urticariaurticariaa or other skin reactionsreactions,, wheezing or other chestest discomfort, (n=49) (n=49)( Infusions) Infusions) nausea, , vomiting, rigorsrigors,, back pain, myalgia,myalgia, arthrarthralgia,algiaa, and changes in Local reactions‡ 49 (100)1340 (0.592) 449 (100) 1322 (0.584) blood ppressure Other AEs: s Renal:Renal: AcuteA renal dysfunction/failure,dysfunction/failurey , osmotic nephropathyp opathyp y Headache 13 (26.5) 40 (0.018) 1122 (24.5) 32 (0.014) s Respiratory:Respiratattoorryy: Apnea, Acute RespirRespiratoryatory Distress Syndromeme (ARDS), TRALI, Cough 8 (16.3) 9 (0.004) 5 (10.2) 6 (0.003) Diarrhea 7 (14.3) 8 (0.004) 5 (10.2) 6 (0.003) cyanosis,cyanosis, hypoxemia,hypoxemia, pulmonary edema, dyspnea, bronchospasmbronnchospasm FatigueFatigue 6 (12.2) 6 (0.003) 4 (8.2) 4 (0.002) s Cardiovascular:Cardia ovavascular: Cardiac arrest, thromboembolism, vvascularasscular collapse,collapse, Back pain 5 (10.2) 11 (0.005) 4 (8.2) 5 (0.002) hypotensionhypotennsion Nausea 5 (10.2) 5 (0.002) 4 (8.2) 4 (0.002) s Neurological:Neurological: Coma, loss of consciousnessconsciousness,, seizuresseizures,, tremor,trremorr,, aseptic Abdominal pain,paain, upper 5 (10.2) 5 (0.002) 3 (6.1) 3 (0.001) Rash 5 (10.2) 7 (0.003) 2 (4.1) 3 (0.001) meningmeningitisgitis syndrome PainPain in extremityextremmity 4 (8.2) 7 (0.003) 4 (8.2) 6 (0.003) s Integumentary:Integummentarryy: Stevens-Johnson syndromesyndrome,, epidermolysis,epidermolysis, erythema MigraineMigraine 4 (8.2) 5 (0.002) 3 (6.1) 4 (0.002) multiforme,multiforrme, dermatitis (e(e.g.,.g., bullous dermatitis) PainPain 4 (8.2) 5 (0.002) 3 (6.1) 4 (0.002) s Hematologic:Hematoologic: PPancytopenia,ancytopenia, leukleukopenia,openia, hemolysishemolysis,, popositiveositive direct Epistaxis 4 (8.2) 6 (0.003) 2 (4.1) 3 (0.001) PharyngolaryngealPharyngolarynngeal pain 4 (8.2) 6 (0.003) 2 (4.1) 2 (<0.001) antiglob antiglobulinbulin (Coombs’) test ArthralgiaArthralgia 4 (8.2) 5 (0.002) 2 (4.1) 3 (0.001) s Gastrointestinal:Gastroiintteesttininal: Hepatic dysfunction, abdominal pain * Excluding infections.infections. s General/BodyGenerall//Boddyy aass a WhWWhole:hole: Pyrexia, rigors † Rate of AEs per infusion.ion. ‡ Includes injection-site reactions as well as bruising, scabbing, pain, irritation, cystscysts,, eczema,ema, and nodules at the injection site.site. TToo report SUSPECTEDUSPECTED ADVERSE REAREACTIONS,CTIONS, contact CSLL Behring Pharmacovigilance at TheThe rratioatio of infusionsusions with temportemporallyally associated AEsAEs,, includingding local reactionsreactions,, to all 1-866-915-69586958 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.wwww..ffdda.govv//medwatch. infusions wwasas 131338338 to 2264 (59.1%; upper 95% confidence limit of 62.4%). Excluding 7 DRUGDRUG ININTERACTIONSNTERACTIONS local reactionsreactions,, thehe corresponding rratioatio wwasas 173 to 2264 (7.6%;6%; upper 95% confidence 7.1 Live VVirusirrus VaccinesVaccines limit of 8.9%). TThehe passive trtransferansfer of antibodies with immunoglobulin administradministrationdministration may interfere with TableTable 3 summarizeszes the most frequent ARs (i.e(i.e.,., those AEs considconsidereddered by the investigators to the responsee to live virus vvaccinesaccines such as measlesmeasles,, mumpsmumps,mps, rubella, and varicellavaricella (see be “at least possipossiblyibly related” to HizentrHizentraa administradministration)ation) experieexperiencedenced by at least 2 subjectssubjects.. PPatientatient CCounselingounseling IInformationnffoorrmmation [17][177]]).) TTableable 3: Incid Incidencedence of Subjects WWithith AdverseAdverse Reactionsns (Experienced by 2 or 7.2 SerSerologicalologgical TTestingesting MorMoree Subjects)) to HizentrHizentraa and Rate per Infusion (ITT Population)Poopulation) VVariousarious passpassivelysively trtransferredansferred antibodies in immunoglobuimmunoglobulinulin preparpreparationsations may lead to AdverseAdverse ReactReactiontion Number (%) of Subjects Number (Rate*) of misinterpretamisinterpretationation of the results of serological testing. (*2 Subjects) (n=49) AdverAdversese Reactions 8 USE IN SSPECIFICPECIFIC POPULAPOPULATIONSATIONSTIONS (n(n=22642264 Infusions) 8.1 PrPregnancyegnaancy Local reactions† 49 (100)1338 (0.591) Pregnancy CaCategoryategory CC.. Animal reproduction studies have notot been conducted with HizentrHizentra.a. Other ARs: It is not knownwn whether HizentrHizentraa can cause fetal harm whenhen administered to a pregnant Headache 12 (24.5) 36 (0.016) woman or canan affect reproduction capacitycapacity.. HizentrHizentraa shoulshouldld be given to pregnant women VomitingVomiting 3 (6.1) 3 (0.001) only if clearlyy needed. PainPain 3 (6.1) 4 (0.002) 8.3 NurNursingsingg MotherMotherss FatigueFatigue 3 (6.1) 3 (0.001) HizentrHizentraa has not been evevaluatedaluated in nursing mothersmothers.. Contusion 2 (4.1) 3 (0.001) 8.4 PPediatricediatrric Use Back pain 2 (4.1) 3 (0.001) TThehe safety anandnd effectiveness of HizentrHizentraa have been establisestablishedhed in the pediatric age groups Migraine 2 (4.1) 3 (0.001) 2 to 16, as ssupported by evidence from adequate and well-controlledell-controlled studiesstudies.. HizentrHizentraa Diarrhea 2 (4.1) 2 (<0.001) wwasas evevaluatedaluateed in 10 pediatric subjects with PI (3 children and 7 adolescents) in a study Abdominal pain,paain, upper 2 (4.1) 2 (<0.001) conducted in the US (see Clinical Studies [14]]) and in 23 pediatricatric subjects with PI (18 children Nausea 2 (4.1) 2 (<0.001) and 5 adolescents)scents) in EuropeEurope.. TTherehere were no differences inn the safety and efficacy profiles Rash 2 (4.1) 2 (<0.001) as compared with adult subjectssubjects.. No pediatric-specific dose requirements were necessary to ArthralgiaArthralgia 2 (4.1) 2 (<0.001) acheive the desiredd serum IgG levelslevels.. * Rate of ARs per infusinfusion.ion. † Includes injection-site reactionsr as well as bruising, scabbing, pain, irritation, cysts,cysts, eczema,eczemma, and nodules at the injection site.site. Safety and efeffectivenessffectiveness of HizentrHizentraa in pediatric patients belowlow the age of 2 have not been established. TableTable 4 summarisummarizeszes injection-site reactions based on investigatorator assessments 15 to 45 minutes after thee end of the 683 infusions administered duringg regularly scheduled visits 8.5 GeriatrGeriatricric Use (every 4 weeks). Of the 49 subjectsbjects evevaluatedaluated in the clinical study of Hizentra,Hizentra, 6 subjects were 65 years of age or olderolder.. No overoverallall differences in safety or efficacy were observedbserved between these subjects TableTable 4: InvestigatorInveestigator Assessments* of Injection-Site ReactionsR by Infusion and younger subjectssubjects.. Injection-Site ReactionR NumbeNumberer† (Rate‡) of Reactions (n=683(nn=683 Infusions§) Edema/indurEdema/indurationaation 467 (0.68) Erythema 346 (0.50) Local heat 108 (0.16) Local ppain 88 (0.13) Itching 64 (0.09) * 15 to 45 minutes afteafterer the end of infusions administered at regularly scheduled visitsts (every 4 weeks). Manufacturedd by: Distributed by: † FForor multiple injection sitessites,, every site wwasas judged, but only the site with the strongestst reaction wwasas recorded. ‡ Rate of injection-site reactions per infusion. CSL Behringg AGAG CSL Behring LLC § Number of infusions administered during regularly scheduled visitsvisits.. Bern, SwitzerlandSwitzerrland Kankakee,KankakKankakeeee, IL 60901 USA Most local reactioreactionsons were either mild (93.4%) or modermoderateate (6.3%(6.3%)%) in intensityintensity.. US License NNo.o. 1766 BBasedased on FFebruaryebruary 2011 revision

If you live with primary immunodeficiency disease (PIDD)… Make the leap to Hizentra

The Sub-Q Ig therapy that fits your life Hizentra is a subcutaneous immune globulin (Sub-Q Ig) therapy that was deliberately designed to give you freedom and flexibility with your Ig treatment. s The 20% concentration delivers an Ig dose in half the volume of 10% solutionsa s Convenient room temperature storage for up to 30 months s Always ready for immediate use

aBased on an equivalent dose in grams.

To learn about the benefits of Hizentra, visit www.LearnAboutHizentra.com Ask your doctor about Hizentra today.

Important Safety Information Hizentra is indicated for the treatment of various forms of primary The most common drug-related adverse reactions (seen in 5% or more of subjects immunodeficiency (PI). in the clinical trial) were local reactions (swelling, redness, heat, pain, and itching If you have a history of anaphylactic or severe systemic response to immune at the injection site), headache, vomiting, pain, and fatigue. globulin preparations or selective immunoglobulin A deficiency, check with your Your physician will monitor for potentially serious reactions associated with physician, as Hizentra should not be used. intravenous immunoglobulin treatment that might also occur with Hizentra, including renal dysfunction/failure, osmotic nephropathy, thrombotic events, aseptic meningitis Hizentra is to be infused under your skin only; do not inject into a syndrome (AMS), hemolysis, and transfusion-related acute lung injury (TRALI). blood vessel. Ig administration may impair the effect of virus vaccines, such as measles, Hypersensitivity reactions may occur with Hizentra. If your physician suspects mumps and rubella. Before getting any vaccination, inform your doctor that you you are having a negative reaction or are going into shock, treatment will be are using Hizentra. discontinued. Because Hizentra contains proline, you cannot be treated with Hizentra if you have hyperprolinemia (a high level of proline in your blood). Please see brief summary of full prescribing information for Hizentra on previous pages. Hizentra is derived from human plasma. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) You are encouraged to report negative side effects of prescription drugs agent, cannot be completely eliminated. to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

Hizentra is manufactured by CSL Behring AG and distributed by CSL Behring LLC. Hizentra is a registered trademark of CSL Behring AG. ©2011 CSL Behring LLC 1020 First Avenue, PO Box 61501, King of Prussia, PA 19406-0901 USA www.CSLBehring-us.com www.Hizentra.com HIZ09-11-0011 9/2011