February 2008 • Volume 18 • Issue 1 Parent Empowerment Newsletter When You Lose Your Health Insurance

BY LAURIE KELLEY

As the New Year rolls in, anxiety about healthcare coverage for hemophilia families is at an all-time high. The government and insurance companies continue their crusade to cut costs, and employers may make it tougher for employees to find adequate cov- erage. In an economy reeling from subprime lending collapse and massive debt, jobs may be more difficult to find. And an entire post- HIV generation is preparing to graduate from college—but finding itself without insurance. Now more than ever, it’s vital for every person with hemophilia, every family, and every graduating student to understand: You need some kind of insurance to obtain factor. This article will help you find that coverage. For a family with hemophilia, one of the most frightening scenar- ios is the loss of health insurance. You can lose your health insur- ance when… • you change jobs or companies • you resign from a company or are laid off • you depend on, and then lose, your spouse’s insurance • you reach your lifetime cap • your employer cancels your insurance coverage Depending on the policy, your child can lose the insurance he receives through you when… • he turns 18 or 19 and is not a college student • he finishes college • he is a college student and turns 24 Many parents live in fear of losing their insurance, particularly when the local economy is depressed, jobs seem threatened, or the family depends on a single insurance policy. Some parents remain stuck in unsatisfying jobs because they fear that switching jobs will lead to losing their insurance. Often, both spouses must work to inside ensure adequate coverage. 3 As I See It: The Making of a Gentleman It’s possible to maintain adequate insurance, even when you 4 Inhibitor Insights: The Boy Scout Who Never Gives Up change jobs to improve your career or move to another state for a 5 Project SHARE: Zimbabwe: Life on the Edge new job. But such moves must be carefully planned. 6 Storm Watch: The Wicked Switch of the West? continued on page 11 7 Homefront: New Year’s Resolutions 8 Transitions: Making Their Mark Excerpted from Raising a Child With Hemophilia: A Practical Guide for Parents, 9 Richard’s Review: No Dueling Allowed! 4th edition

LA Kelley Communications, Inc. welcome PARENT EMPOWERMENT NEWSLETTER FEBRUARY 2008 While the presidential candidates are EDITOR-IN-CHIEF Laureen A. Kelley stumping to sound-bite promises of CONTRIBUTING WRITERS Paul Clement “change” and “hope,” millions of Americans Ziva Mann are facing change, but not much hope. Richard J. Atwood Sherrell Portrait Design Persistent insurance cost-cutting measures EDITOR Sara P. Evangelos continue to warp the outline of American LAYOUT DESIGNER Tracy Brody healthcare. For those of us with hemophilia, PROJECT SHARESM DIRECTOR Julia Q. Long home care switching may be our number-one EXECUTIVE ASSISTANT Zoraida Rosado worry. In this issue, find out what to do PEN is a newsletter for families affected by bleeding when things seem hopeless. Our feature disorders that is produced and edited by a parent of a child article guides you through the steps to with hemophilia. It is a forum that promotes an active recovery when you lose your insurance. And Storm Watch exchange of information and support among divergent groups tracks the involuntary switching that many states currently in the national and international hemophilia community. face from Blue Cross Blue Shield franchises. PEN does not accept advertising and uses brand Want to put life into perspective while wrestling with payer product names and company names pertaining only to problems? Read about hemophilia care in Zimbabwe and my news and education. visit there just two months ago. At least we have factor—no All names, addresses, phone numbers and letters are one in America goes without that. Zimbabweans with hemo- confidential and are seen only by the PEN editorial staff. philia suffer on all levels, in a country that was once heralded PEN publishes information only with written consent. Full as a model for Africa. Through our own Project SHARESM, names will be used unless otherwise specified. PEN is we are offering aid to keep hope alive. Read more about my privately sponsored; sponsors have no rights to production, trip on HemaBlogTM, updated every Monday morning, at content or distribution, and no access to files. The views www.kelleycom.com/blog. expressed by various contributors to PEN do not necessarily Ziva Mann offers comic relief with her testament to New reflect those of the editor. PEN is in no way a substitute for Year’s resolutions in Homefront, giving us a peek at the crazy medical care. Parents who question a particular symptom life of a family with hemophilia. Speaking of crazy, check out or treatment should contact a qualified medical specialist. tattoos in Transitions—that is, check out Tommy’s tattoo. Learn Articles may be reprinted from PEN only with express how they’re created, what they mean, and how best to handle written permission and with proper citation. PEN may not the subject with your transitioning child. In Inhibitor Insights, be be published, copied, placed on websites, or in any way inspired by Paul Clements’ look at the remarkable Huerta distributed without express written permission. family of California. The Huertas refused to accept the med- Funding provided through generous grants ical community’s passivity in the face of an inhibitor, and with from our corporate sponsors (page 19) tenacity and hope, overcame tremendous challenges. Finally, we say goodbye to my good friend and co-author, community advocate Renée Paper. Renée passed away on LA Kelley Communications November 7 at age 49. Her death prompted accolades, letters LA Kelley Communications, Inc. of grief, and condolences from around the world. Napoleon 68 East Main Street, Suite 102 • Georgetown, Massachusetts 01833 USA once defined a leader as a “giver of hope.” In a world of 978-352-7657 • 800-249-7977 • fax: 978-352-6254 change, Renée was undoubtedly a leader. [email protected] • www.kelleycom.com

I AM A MOTHER OF TWO BOYS WITH SEVERE HEMOPHILIA, inbox ages 10 years and 21 months. I am also an RN with a small home care company that provides excellent serv- ice. We have an insurance dilemma. Our company’s THANK YOU SO MUCH FOR SENDING YOUR LITERATURE. MY insurance provider, Blue Cross Blue Shield, is switching father had hemophilia B, which made me a carrier. I was to a single provider for hemophilia care. This provider blessed that my son did not inherit it, but my daughter is a is a large company that, in my eyes, is inadequate. I carrier. Now that my grandson has been diagnosed, I am try- don’t agree with single-source providers, as that limits ing to educate myself on all the advances that have been our choice as patients. And while this company may made over the past 50 years. My goal is to get our family out look great on paper, I would consider its expertise in of the mindset of fear so that my grandson can lead a nor- hemophilia care for patients in my area dubious. As a mal, happy life. Thank you for educating us. registered nurse, I usually need only our factor and Susan Francis supplies, but who knows? I have a toddler who can be Florida continued on page 19

Parent Empowerment Newsletter • February 2008 2 as I see it

BY MONICA GRUHER The Making of a Gentleman Gruher family

Player of the Year: Ben Gruher

stood on the cart path, watching the protocols, even in children. little blond-haired boy in the red, white I have now met many I and blue cap stride across the putting youngsters and young adults who play inherent vulnerability of having a bleed- green, remove his cap in traditional PGA golf. Every one of these young people has ing disorder, participating in golf at any style, and shake his opponent’s hand impressed me. It’s easy to see how the skill level builds confidence and self- with a smile, saying, “Nice game.” My requirements and expectations of golf have esteem, which a chronic health problem son Ben took second place that day, suc- influenced and developed their characters. can sometimes damage. cumbing to defeat after a two-hole playoff. As a mom, I like the dress codes for Although golf is often perceived as an Not so long ago, this might have resulted courses and tournaments—again the gen- expensive sport, we’ve found it no more in a teary ending. Such a small action— tlemanly influence. Gone are the T-shirts expensive than many other sports. The removing his cap and congratulating the and jean shorts; they’re replaced by col- “First Tee Program” at our local course winner—I might not even have noticed, lared polo shirts, neatly tucked in, khaki focuses on kids in our metro area. It except for the slow but continual changes shorts, and... no, not the dreaded… belt! offers low-cost lessons and greens fees, taking place. My boy, who at age three My son’s petite size two saddleshoe-style and gives low-income grants. began to hitting wiffle balls with plastic golf shoes, and his ball cap with the Golf has been a godsend for us in pro- clubs around the yard, is becoming a lit- attached ball marker, complete the look. viding a safe sport, but the making of our tle gentleman at age eight. Recently, I even noticed that he didn’t gentleman has truly been the blessing of all I was planning to title this piece “The offer resistance to the suit jacket I time. For parents looking for a path for their Making of a Champion,” until I realized required him to wear to a wedding. boy, I encourage you to follow this one. what I really have on my hands. Yes, my In golf, there are no participation tro- son is becoming a winner, but the evi- phies. You place, or you go home empty- Monica Gruher lives in Camas, Washington, dence of his transformation is not the col- handed until the next tournament. This with her husband Jim, son Benjamin, and lection of trophies and medals displayed is a learning process for life: to be a gra- daughter Jade (age three, also a golfer). She in his room. It’s the kind of person I see cious “non-winner” by the tender age of has been a mentor for mothers with newly developing before my eyes. A boy that I eight; to cultivate the strength of head diagnosed children, and hopes to continue her will, in the not-so-distant future, call a man. needed to strategize and gain mental support and encouragement for other families With confidence, I now accept invitations toughness; and to develop the strength of with a hemophilia diagnosis. Ben was the for him, at age eight, to play in foursomes heart to be a gracious winner. 2006 NHF/CSL Behring Jr. National with adults. He keeps up the pace, knows Oh yeah, by the way, he has hemo- Golf Champion at age seven. the lingo, and completely comprehends philia. Severe factor VIII deficiency. The First Tee Program is a national and practices the rules and protocol of Could there be a safer sport from a phys- nonprofit available in 48 states at various the game—like don’t walk across some- ical standpoint? How lucky I feel: I don’t golf courses. Its mission is to provide one’s line on the putting green! have to worry about a head injury or “young people of all backgrounds an It seems that golf demands a certain continual joint problems. Once, the built- opportunity to develop life-enhancing type of behavior or you don’t last long. up adrenalin and stress of a two-hole tie values such as confidence, perseverance, Good manners, control over your lan- playoff produced a fairly severe nose- and judgment through golf and character guage, and a “gentlemanly” demeanor are bleed as we walked up to accept the first- education.” Visit www.thefirsttee.org as important as your score—if not more so. place medal. But fortunately, this is the for more information. Tournament play definitely requires these worst that has happened. Given the

3 ParentParent Empowerment Empowerment Newsletter Newsletter•• February August 2002 2008 inhibitor insights

BY PAUL CLEMENT

Dustin Huerta, Inhibitor Insights is a PEN column sponsored by The Boy Scout Novo Nordisk, Inc. Who Never Gives Up

n February 1995, Pattie Huerta ordered blood tests. 13 needlesticks before nurses could experienced an exceptionally diffi- While in the ER, Dustin received access a vein for infusion. The next I cult labor, and her obstetrician several blood transfusions and fresh day, at the site of an infusion in his resorted to forceps and vacuum to frozen plasma in an effort to control right arm, Dustin’s little elbow was deliver the new baby. Parents Pattie his bleeding. When he was four days swollen to six and one-half inches in and Richard were sent home with old, the results of the blood tests diameter. The swelling spread, causing their new baby, Dustin, just 12 hours showed that Dustin had hemophilia A. his hand to grow to several times its after they had arrived at the hospital. He was immediately given a dose of normal size. Blood then traveled up But something seemed wrong. The factor VIII concentrate. Now the his arm and across his chest, causing routine heel stick would not stop bleed- bleeding from his heel stick and umbil- his arm to swell, as Pattie describes it, ing. The next morning brought a star- ical stump made sense. The vacuum “like the Michelin Man’s.” tling shock—Dustin’s head was swollen and forceps had caused bleeding Dustin’s bleeding caused a danger- and lopsided. As Pattie monitored between his skull and scalp, causing ously high build-up of pressure within Dustin, his head continued to swell. his head to swell and distort. Dustin the sheath of connective tissue cover- When Dustin became lethargic, his received several doses of factor and ing the arm muscles, known as the parents knew something was seriously was released from the hospital when “compartment.” The pressure eventu- wrong and rushed him to the emer- he was six days old. ally shut down blood flow within the gency room. The ER physicians at first Over the next few months, Dustin muscle, a condition known as acute suspected shaken baby syndrome, but had several bleeds that didn’t respond compartment syndrome. Acute compart- when Dustin’s umbilical stump was well to infused factor. Dustin bled ment syndrome is a medical emer- accessed for a vein and kept bleeding, from the infusion sites. After learning gency. Without a supply of blood car- they suspected a bleeding disorder and from NHF about inhibitors, Pattie rying essential nutrients and oxygen, muscle cells and nerve cells die. photos: Huerta family asked her son’s physi- cian several times to Without treatment, this can lead to have inhibitor tests paralysis, loss of limb, or death. done. The hematologist Dustin’s physician finally ordered an simply replied that inhibitor test, which revealed a titer of Dustin didn’t have an 73 BU. Dustin was transferred from inhibitor, and refused to the local hospital to a medical center order a test. As a result, in Los Angeles, where a venous access Dustin would suffer device was implanted. A second another severe bleed, inhibitor test showed the titer had with long-term conse- risen to 135 BU. quences. By now the damage had been done. In April 1996, Pattie Pressure from the bleed had com- and Richard infused pressed the blood vessels in Dustin’s Dustin four times in arm so much that blood flow was two days for an ankle reduced, and his arm was cold to the bleed, but with no touch. The pressure also caused nerve effect. They took damage—he lost sensation in his arm Dustin to the local hos- and hand, and was unable to move his pital, where he endured arm. Dustin’s paralysis caused multi- Dustin’s childhood hasn’t been easy: Dustin with father Richard Huerta continued on page 14

Parent Empowerment Newsletter • February 2008 4 a Project SHARE story ZIMBABWE: BY LAURIE KELLEY Life on the Edge

hen Zimbabwe gained inde- Peter Dhlamini, who has hemophilia, I pendence from the UK in toured Harare, the capital, and W 1980, hopes were high. The Bulawayo, the second-largest city and small African nation, nestled between Peter’s home town. We met with doc- Zambia and South Africa, was regarded tors, the health ministry, patient groups, as the model for all African nations. and influential people who might help Robert Mugabe, a teacher turned politi- us. Our goal was to resurrect the cal reformist, became the country’s first Zimbabwe Haemophilia Association black president. He won kudos for dou- (ZHA) and start a plan to get help. Kelley Communications, Inc. photos: LA bling school enrollment, raising the min- Each day was a struggle. Where to imum wage by 50%, offering free get gas? How to exchange my dollars? healthcare to the poor, and subsidizing (I paid $10 US for a glass of Coke one Day-to-day survival: vegetable seller in Harare prices. In his 1982 landmark book The day; using the official exchange rate, Africans, David Lamb wrote that you could go broke quickly.) Electricity Mugabe had proved himself “perhaps shut down frequently. We ate only two the most capable leader in Africa.” meals a day because food was so hard How things have changed. With to get. I shook my head, thinking rue- Mugabe still in power after 27 years, fully about how many Americans will economic mismanagement, corruption pledge to lose weight in 2008—here, and political terror have left Zimbabwe you have no choice. numb, like a traumatized child at the One night we visited Elton, a 17-year-old hands of an abusive father. The last with severe hemophilia A. He lives on white prime minister, Ian Smith, died the outskirts of Harare on a red, dusty just days before I arrived, and his fatal- road. His house was dimly lit when we ism about self rule in Zimbabwe seems arrived, and mosquitoes constantly to be fulfilled: the country is starved, nipped our ankles. Elton, gangly and Fatalism: Laurie Kelley with Elton, who dysfunctional, and fearful. Whites are tall, has the worst case of synovitis I does not expect to get better leaving in droves and blacks suffer daily. have ever seen. He told me his lower leg I visited Zimbabwe for ten days in fall had gone numb from the swelling. His 2007, my third trip to a country I admit blood supply was being strangled, and I still love. I admire the lush countryside, he said he might lose his leg. Resigned, the stunning beauty of Victoria Falls, his family didn’t bother to take him to the safaris, and above all, the gracious the hospital. We immediately arranged Zimbabweans, always polite and civil. to get Elton taken to Harare to see a But I was shocked to see the country’s surgeon or, better still, taken to South deterioration since 2001, when I attend- Africa, where they have better care. ed our first hemophilia camp. The econ- SHARE would pay all expenses. omy has collapsed by 50%. Inflation is By week’s end, we had a clearer pic- rampant at 12,000%, the world’s high- ture of how to begin helping in est. Gas stations are empty, and banks Zimbabwe. Project SHARE does a lot No factor for infection: are routinely closed. Unemployment more than just dole out free factor. We Owen Kanozeya suffers in a hospital has skyrocketed to 80%. Bread is use free factor as a tool—to get assess- mostly unavailable, in a country that ments, to get accountability; to get local and for anyone else who needs it. We’ll used to be called Africa’s Breadbasket. hemophilia patients, doctors and health buy two new refrigerators to store the fac- Hemophilia care? Nonexistent. There ministry officials working with us to tor, and buy a printer for the ZHA office. are no hematologists in the country, improve long-term care. The health min- And best of all, we will hold a camp again except for one volunteer Cuban doctor, istry was candid: It would be a long in 2008 to provide medical care, factor, fun, who is scheduled to leave in April. The time before there would be money for and hope for a better future. only factor comes from Project SHARE factor. So it’s up to us, the international and our partner, AmeriCares. I visited hemophilia community, to care for our Read about Laurie’s trip on HemaBlogTM at to assess needs, and I hardly knew brothers in Africa. We will continue to www.kelleycom.com/blog where to begin. With my colleague ship factor. We will get care for Elton,

5 ParentParent Empowerment Empowerment Newsletter Newsletter•• February August 2003 2008 storm watch

BY LAURIE KELLEY Th e Wicked Switch of the West?

ome care switching has hit the nity must have hemophilia community hard choice to access H in the past few months, and expert care in treat- patients increasingly feel its sharp ing this condition— change to either the 340B program at pinch. Insurers continue to negotiate not an insurance your HTC or the insurance-owned new contracts with home care providers, company that has pharmacy PrecisionRx?” and in some cases, even start their own absolutely no knowledge, experience Switching isn’t happening only with specialty pharmacies to purchase and or expertise in providing care to our PrecisionRx, of course. Chris Blair, deliver factor. Hemophilia home care very unique needs. Hemophilia care is president of Hemophilia and Bleeding switching may no longer mean just not factor in a box.” Disorders of Alabama (HBDA), writes being switched from one well-known The switching has been followed by that Blue Cross Blue Shield of provider to another; it may mean being some hemophilia patients’ complaints Alabama has also entered into a single- switched to a new entity, one owned by about services from these new entities source provider, or exclusive, contract an insurance company. At the forefront (see “A Letter to Aetna,” PEN, May with a large healthcare company for of the recent hemophilia switching wars 2007). WellPoint is quick to point out patients in Alabama with hemophilia. is a specialty pharmacy called that whatever the problems, they aren’t Anyone in Alabama who is insured PrecisionRx Specialty Solutions, due to lack of knowledge or through BCBS, and whose factor owned by expertise: Wellpoint plans provider is not the one selected by WellPoint/Anthem. all have medical direc- BCBS, will be transferred automatical- In July 2007, tors, case managers ly to the new BCBS provider effective WellPoint/Anthem “Hemophilia and pharmacy direc- February 1, 2008. policyholders in tors. PrecisionRx Despite the articles and warnings Nevada, Colorado care is not employs nurses, previously sent to families,2 the news and California were pharmacists, reim- still comes as a shock. And families still notified that as of bursement special- cling to what will soon be outmoded September 2007, they factor in ists, and technicians strategies. They express two fears: would be switched trained in the specific from their previous home a box.” needs of the bleeding 1. Losing the long-term relationship care company to disorders community. It they have with their current PrecisionRx, or to a local 340B also has a medical director with provider. entity. WellPoint/Anthem follows more than 40 years’ experience in the 2. Poor service by the new home care Aetna, Cigna, HealthNet, Kaiser, and bleeding disorders community. company regarding assay manage- other smaller plans that now own inter- Still, change isn’t easy to make, or ment, delivery time, supplies, and 1 nally-integrated specialty pharmacies. easy to take. And our community is knowledge about hemophilia. “This issue has been creeping up on reacting swiftly as the switching Trust is built on long-term relation- the bleeding disorders community for spreads eastward. Warned one town ships. Sudden switching snaps apart some time now,” says Bill Jamison, a crier in the online chat group the bond of trust between hemophilia person with hemophilia from Hemophilia Support, “For those of you families and home care; families want Harrisburg, Pennsylvania. “Choice is who have Anthem/Blue Cross in the to keep the representative they knew, paramount to our wellbeing. states of Indiana, Kentucky, Missouri, who understands their unique needs. WellPoint/Anthem is testing the waters Ohio, and Wisconsin… did you know But there is an added wrinkle. The and eliminating adequate [home care] that your in-network providers for hemophilia factor-provider industry is choice for the consumer. The commu- your delivery of factor are going to continued on page 16

1 Additionally, many insurance plans have developed an exclusive relationship with vendors for hemophilia care. For example, Coventry, IBC, Humana, and BCBS of Alabama all have exclusive carve-outs for hemophilia; some are with well-established and well-known hemophilia home care companies. 2 See “The Coming Storm,” PEN, February 2005, and “PBMs Take Center Stage,” PEN, May 2005.

Parent Empowerment Newsletter • February 2008 6 homefront

BY ZIVA MANN New Year’s Resolutions

blinked, and already there are Christmas trees on the • I will remember that doing housework is less important curb. Where did 2007 go? I had such plans for 2007—but than reading stories with my boys. I then again, I recall having plans for 2006. Sigh. Onwards Note to self: Hire housekeeper. we trudge, then. It’s resolution time. Note to self: Get job to pay for housekeeper. Failing that, win lottery. • I will get Shai to school on time. It’s only 27 minutes away, so how hard could this be? • I will learn about advocacy. I’m tired of fighting with our Note to self: Put little brother Akiva in car the night before. insurance company, and it gets worse every year. Is there something I can do to protect us? • I will be more patient with lost gloves, forgotten (and Note to self: Call local NHF/HFA chapter and ask about advocacy. moldy) lunchbags, and the seventeenth wail of (Stephen King novel vs insurance battles? Insurance battles are far, “Muuuum, I’m huuuungry!” far scarier.) Note to self: Pack more snacks in diaper bag. Consider a pack mule when diaper bag is full. • I will go to bed before I drop in my tracks. Note to self: Tasks before going to bed: clean up dinner dishes, laun- • I won’t laugh when Shai limps theatrically, forgets, and dry, make school lunch, set out tomorrow’s school clothes, paperwork, then starts dancing to “Walk Like an Egyptian.” Or at put away toys, restock diaper bag, laundry, return phone calls, spend least, I’ll laugh quietly. I will try not to roll my eyes time with husband, email, blog, prep for morning factor infusion. noticeably when he says something hurts, but after con- sidering the implications (factor? ice? rest?), decides that • I will eliminate some tasks. he feels just fine. Note to self: Which ones? Husband may have an opinion. • I will remember to water the plants. And feed the fish. Consult him. Note to self: Failing the above, get better at hiding—or flushing—the • I will not be intimidated by the goals I’ve set for myself. evidence. Except that I am. Dang, that’s a really long list.

• I will thank my husband Ezra profusely and repeatedly Hmm. Maybe I should try and whittle this down. Okay, let’s for doing more than his share of morning infusions. An try again. extra 20 minutes of sleep is wonderful. • I will learn to accept imperfection. The mittens will be Note to self: zzzzzzzzzzzzzzzzzzzzz. lost, the lunchbag will contain something horrible and rotting, the gym is unlikely, and yes, I will probably roll • I will file things. The Big Pile O’ Paper is not (repeat, not) my eyes from time to time. But maybe I could reduce the a filing system. I will file all the EOBs, the notes from amount of horrified screeching? arguing with people about the EOBs, the lists of lab results, my old factor log sheets. • I will try to forgive mistakes—mine included. Because oh Note to self: Develop filing system. boy, are we going to make a lot. Note to self: Find place to put filing system. Use same methodology Note to self: Find therapist. This rolling with the punches idea may used to create extra hours in the day. Failing that, label a cardboard be harder than I think. Do perfectionists ever roll? box INSURANCE 2008 and toss everything in there. Note to self: Persuade self that a box is very, very different from a pile. Happy 2008, everybody! May your year bring joy, love and surmountable challenges. Oh, and a good filing system.

7 Parent Empowerment Newsletter • February 2008 transitions BY LAURIE KELLEY

Transitions is a PEN column sponsored by Making Baxter BioScience Their Mark Tommy learns Latin: Honesty, Courage, Generosity, Beauty

What does gene therapy have to do with tattoos? The first hemophilia gene therapy peer pressure, or impulsiveness. Many teens and young adults see it differently. Wtrial, conducted in Boston in 1999, used years from now? Twenty years from To them, tattooing means emerging fibroblasts as host cells for an implanted now? Will he respect other people’s feel- autonomy and identity expression. vector. The vector contained a gene that ings by avoiding racially, politically, sex- Tattoos are fun, flirty and daring. I would produce factor VIII. Fibroblasts ually or religiously charged art? Can he know one representative in the hemo- are dermal cells, lying beneath the epi- handle the various reactions he’ll get: philia pharmaceutical industry who dermis. Unlike most other cells in the Disgust? Shock? Admiration? Concern? body, fibroblasts are long-lived and sports a modest one, and an executive don’t slough off like the outer skin layers. director of an NHF chapter who flaunts DOs and DON’Ts This makes them attractive cells for what a large, colorful one. we hoped was a permanent gene therapy. Tommy has no adolescent angst; his Educate yourself and your young adult about the procedure, pain, follow-up Fibroblasts are also great for tattoos. tattoo is all about positive expression. care, and potential complications. In a Ink injected into fibroblasts stays in Last year, he surprised us with a tattoo nutshell, here are some things for you place, making tattoos permanent. The on his right deltoid: a vibrant green both to consider: gene therapy didn’t pan out, but tattoos and gold shamrock. Hard to object are all the rage. when you’re Irish. But this Latin one is • Be sure your child is of legal age; in most states, this is 18. More than 20,000 tattoo parlors oper- more serious. It’s big, it’s black, it’s on ate in our nation, according to U.S. his forearm, and it’s forever. As a con- • Call the hematologist first to discuss. News & World Report. A 2003 Harris Poll temporary musician, he feels this is Always infuse before tattooing. estimates that 36% of people aged appropriate “body art,” as tattoos are • Be sure you child understands that 25–29 have one or more tattoos.1 now called. tattoos are permanent. And remem- Chances are, your teen or young adult If you have a transitioning teen with ber, skin sags after age 45. As with hemophilia will consider getting a hemophilia who is considering a tattoo, Robin Williams once said, that tattoo sometime. please prepare him. Have the Tattoo barbed-wire tattoo will look like a But enough about statistics. Tommy Talk. Don’t think you’ll be planting picket fence when you’re 60! came home just after Christmas to show ideas about getting one—because • Choose a licensed shop and a quali- me his gift to himself—a large tattoo on chances are, he’ll think of it himself. fied professional. his left forearm of four Latin words in When your child is transitioning, you • Make sure the artist uses new or Old English script. Pius. Animosus. need to help him make better decisions. sterile equipment for each client. For that, he needs to know your values Liberalis. Bellus. Talk about mixed • Try a temporary tattoo first, to see and feelings on the subject, but he also feelings: It’s Latin! It’s a tattoo! It’s how friends, family—and you—react. needs good information. beautiful! Is he crazy? Or try a small tattoo in an incon- Discuss the reasons your teen wants a spicuous place. tattoo. What message does he want to Why a Tattoo? convey? What does the tattoo mean to For many parents, tattooing connotes him? How might he feel about it several rebellion, self-mutilation, conformity to continued on page 15

1 Conducted online between July 14 and 20, 2003, by Harris Interactive® in a nationwide sample of 2,215 adults.

Parent Empowerment Newsletter • February 2008 8 richard’s review

BY RICHARD J. ATWOOD No Dueling Allowed! A look at hemophilia in the early twentieth century

Diseases of the Kidneys and of the Spleen, Treatment for bleeds included tonic Hemorrhagic Diseases remedies, ergot, acetate of lead, H. Senator and M. Litten, 1905 hydrastis canadensis or opiates, and Philadelphia, PA: W.B. Saunders & Company nitrate of silver. Mechanical treatment involved elevating the 815 pages affected limb, local styptics such as chloride of iron, cautery, packing, rubber bandages, and compression. t’s fascinating to examine the early twentieth century’s view of hemo- Early hemophilia care also included some social rules: Iphilia and its treatment, if only to • Playing with children should be supervised by an adult, appreciate the advanced treatment we and playthings should not inflict injury. enjoy today. In 1967, Robert K. • The family physician should consult with the child’s Massie published Nicholas and teacher about hemophilia symptoms. Alexandra, his Pulitzer Prize-winning • Children should not perform gymnastic exercises. history of the last Russian tsarist • Children should never be punished physically. family. Alexis, the tsarevich and heir • Dueling is forbidden. to the throne, had hemophilia. Massie stressed the key role of • People with hemophilia should avoid any profession or hemophilia in shaping the course trade, including military service, with violent bodily exer- of events that led to the Bolshevik cise or exposure to slight injuries. revolution. For contemporary • Recommended employment: office work, drafting, or the medical insight, he cited Dr. M. “learned professions.” Litten, who published Hemorrhagic • Occupations to avoid: watch maker, engraver, wallpaper Prince Alexis: In 1914, Russia had Diseases in 1905, when Alexis was hanger, goldsmith, barber. only 11 known individuals in one year old. The combined Expertise in care and treatment originated in areas where seven hemophilia families work by Drs. Senator and Litten hemophilia was prevalent, and Germany furnished the majority included a 95-page section on hemorrhagic dis- of cases. In 1905, of a worldwide prevalence of 194 known eases, where Litten summarized hemophilia (“Bleeders’ Disease”) hemophilia families and 630 people with hemophilia, 93 fami- in just 25 pages. At the time, hemophilia’s prognosis was poor. lies and 258 individuals lived in Germany. Compare this to Only 40% of those affected reached age eight, and just 11% lived Russia, with only 11 individuals in seven hemophilia families. to age 22, although the outlook slightly improved after puberty. Why did Massie choose to cite the Litten text on hemophilia In the early twentieth century, hemophilia was known to be in Nicholas and Alexandra? Possibly because it was readily avail- transmitted by female “conductors” to male “bleeders.” It was able in its English translation and was published during Alexis’s also known that females could be affected, in a ratio of one childhood. And probably because the more experienced woman to 13 men. Hemophilia’s cause was not understood, German physicians presented the standard of hemophilia care although an unidentified bacterial infection was suspected; possi- in 1905. Interestingly, the Russian physicians may not have fol- ble culprits even included psychic influences during pregnancy, lowed all of the German medical advice because of professional and a susceptibility linked to anxiety. Ironically, hypotheses jealousy or national pride, as the Russian dislike of Germany at involving the vascular system and the blood itself had failed rig- the time was well known. But regardless of treatment efforts, orous scientific examination. While scientists investigated the Alexis was assassinated by the Bolsheviks at age 14. He was blood’s reduced clotting ability, along with fibrin levels and the perhaps the most famous person with hemophilia, and his life fragility of vessel walls, they reached no conclusions. has been immortalized in many books and films. There was suggested treatment. A special diet cut out all bev- erages that “excite the vascular system” (alcohol, tea, coffee) For more on living with hemophilia in the early twentieth century, read and stressed bland, solid food with fresh vegetables. Sea- Legacy: The Hemophilia of Yesterday by Matthew Dean Barkdull. To bathing was recommended, and cold dips followed by rubs. learn about the first known American family with hemophilia, read “The Life in the country was considered beneficial. Drugs used to Appletons: America’s ‘First Family’ With Hemophilia,” by Richard J. treat hemophilia included chloride of iron, mineral acids, subac- Atwood and Sara P. Evangelos, PEN, November 2002. Order both etate of lead, sulphate of magnesia, and sulphate of sodium. resources through LA Kelley Communications at www.kelleycom.com.

9 Parent Empowerment Newsletter • February 2008 In Memoriam: Renée Paper

ith deep sadness we note the passing of our com- From your acorns you saw great oaks grow, now the branches munity’s greatest advocate for people with von are reaching far and wide.” W Willebrand Disease. Parimal Debnath, Haemophilia Society of Bangladesh, wrote, Renée Paper died November 7, at age 49, after an eight-week “It’s really sad news. My condolences for her family. May God hospital stay following a fall. Renée had been on disability rest her soul in eternal peace.” Dr. Carol Kasper, renowned leave for the previous three years. She had just received an hematologist, emerita professor of medicine, University of achievement award at NHF’s 59th Annual Meeting in Southern California, Orthopaedic Hospital, and Renée’s close November, in recognition of her outstanding life achievement colleague and friend, best sums up Renée’s legacy and memory: in helping others with bleeding disorders. People around the Renée Paper had a vision for Nevada, its own hemophilia world knew Renée, as she traveled and lectured frequently foundation, its own hemophilia treatment center, and she prior to her disability. As many of you already know, after her made them happen. It wasn’t easy. Renée was blessed gastric bypass surgery, Renée lost a tremendous amount of with energy, enthusiasm and perseverance. She had a weight in a short time. She seemed to recover and was doing great ability to organize and to inspire. She also knew how well, but then her health issues compounded. She had VWD to have fun. She was the life of the party! I remember driv- and also battled hepatitis C, along with various other prob- ing with her down a country road in Ireland, in sheep lems. Many of her friends stayed in touch by phone or by visit- country. Sheep strayed across the road. Renée stopped ing her in Las Vegas over the past several years. and shooed them off, and you have no idea how hard it is Renée was a personal friend, and also my co-author of A to shoo a sheep. We cleared a bit of road, drove on Guide to Living With von Willebrand Disease. She traveled with my around a curve, and more sheep! We wound up in gales family and me to Puerto Rico and the Dominican Republic on of laughter as we continued to shoo the sheep, and shoo business. Renée and I gave presentations together in places as and shoo. close as Connecticut and as far away as Australia. She was bril- I am grateful to Dr. Jonathan Bernstein and nurse Becki liant; she was fun-loving. She could be irreverent, yet compas- Berkowitz, also Dr. Heather Allen, all of Las Vegas, who sionate. She loved animals, and one of her favorite gifts to her watched over Renée in her last illnesses, whenever friends each New Year was to send a photo of herself with a Renée’s spirit of independence would allow it. I shall different animal from different parts of the world: kissing a dol- remember her achievements, but the images of her exu- phin in the Caribbean, draping a boa constrictor around her berance and hilarity are foremost in my mind at this time. neck in Mexico, nuzzling an alligator in New Orleans, atop a Her name will be remembered. camel in the Canary Islands. Renée knew how to grab life by the horns and tame it, and she wanted others to do that, too, Renée is survived by her father, Harry Paper, of Encino, regardless of disorder or disability. California. Harry expressed his deepest appreciation to all who Renée was a certified critical care nurse with more than 20 were touched by Renée’s work and took the time to write to years’ experience in emergency nursing. She thrived in the him to share their thoughts, feelings and stories about Renée. midst of excitement and chaos with her typical take-charge per- Good-bye to a tremendous leader, warm and loving human sonality. She served as a team member of several boards and being, educator, visionary, and friend. There was ever only one committees for NHF, WFH, and other organizations. Renée Renée, and we will miss her. founded Hemophilia Foundation of Nevada, bringing care and advocacy to hundreds. Nevada was an appropriate Laurie Kelley and co-author Renée Paper, Boston, 2003 place for her to live, as her motto has always been “Just play the hand you’re dealt.” After Renée’s passing, messages poured in from around the world. Helen Campbell of the UK, from the MSN group WomenwhoBleed, wrote: “Renée has been a great inspiration to me personally (and to other women) in raising awareness and promoting LA Kelley Communications, Inc. LA advocacy within, and outside of, the bleeding disor- ders community regarding the issues surrounding women and their bleeding disorders. Her work has touched the lives of many both directly and indi- rectly. It is a great loss for the hemophilia communi- ties and yet must be a greater loss for her family. Thank you, Renée. You made such a difference.

Parent Empowerment Newsletter • February 2008 10 When You Lose Your Health Insurance... continued from cover

I was once offered a position that I really wanted, but I had to turn it down because of the insurance policy. Within three months, we were able to get my son covered under my wife’s policy. So I accepted the job I had want- ed, with the understanding that I would have to wait a year before my son was covered. To my dismay, we found out a year later that this could not be—after I had already changed jobs. The insurance industry has dictat- ed that my wife must work forever. —Ken Moffat, Ohio If you’re lucky enough to be offered the job of a lifetime, first get written approval that you will be covered by your new employer’s insurance. Make sure you get written answers to your questions concerning hemophilia, especially the new poli- cy’s pre-existing condition clause. Why do I stress written? Trust me: If you ever need to battle out a disagreement with your payer or employer through a mediator, or even in court, you’ll need these written promises to support you legally. Try to have some savings on hand as a backup, in case the pre-existing con- dition clause requires a waiting period. Ask a reimbursement specialist about pharmaceutical companies’ patient assistance programs, which sometimes grant free factor to needy families. Help is available—but first help yourself by getting prepared. Even when you are prepared, sometimes the inevitable hap- pens: You lose your insurance. What are your options?

Advantages? COBRA lets you keep all the benefits and cov- Consolidated Omnibus Budget erage you had before. You can use this extended coverage for Reconciliation Act (COBRA) 18 to 36 months, depending on your reason for eligibility. If you lose your job, you can continue coverage for only 18 If you lose your job or switch jobs, your first question should months. Following divorce or separation, coverage is 36 be, “Am I eligible for COBRA?”1 The COBRA Act of 1985 months for the spouse and dependent child.3 was established to allow you to pay for continuing your insur- ance coverage, temporarily, at the group rate you received Best of all, when you start a new job, you can be covered through your employer. COBRA applies only to certain quali- by your previous COBRA policy even when you begin fying events: when you lose your job, experience a reduction a new insurance policy. If your new policy imposes a in hours, anticipate legal separation or divorce, or lose a pre-existing condition clause or waiting period, you’ll spouse who has provided insurance. continue to be covered by your COBRA policy throughout the waiting period. Your employer2 must notify its insurance plan administrator within 30 days of the date of the qualifying event. Within 14 days after this notification, the plan administrator must then Public Assistance notify you of your COBRA rights. You may elect COBRA coverage any time within 60 days after your insurance cover- What if you can’t afford the expensive premiums and other age terminates, or 60 days after the date you received notice payments associated with COBRA? You can work with your from the plan administrator. HTC social worker or reimbursement specialist to find assis- tance programs that help pay premiums and other costs. If Drawbacks? Coverage through COBRA is expensive, you’re unable to find such a program (like PSI; see p. 13), although group coverage rates are lower than those of individ- you may need to consider public assistance. Fortunately, all ual plans. You must pay the full premium and usual costs, state governments have assistance programs for people facing such as deductible and copayments, on time. Missed or medical expenses, and 33 states have specific hemophilia pro- delayed payments result in immediate termination of your policy. grams. Public assistance programs include Medicare,

1 For COBRA information, call the Department of Labor hotline at 202-523-8784. Employers with more than 19 employees and a sponsored group program must allow the terminated employee to continue coverage through COBRA. However, the federal government is exempted from offering COBRA, so federal employees are not eligible for this continued coverage. 2 Or your spouse’s employer, if you are divorcing and you are dependent on your spouse’s plan. 3 Under the new HIPAA rules (see PEN, February 2007), if a person is determined disabled within the first 60 days of COBRA coverage, he or she is allowed to purchase an additional 11 months beyond the usual 18-month coverage period. Family members of a disabled individual also qualify for the additional 11 months of coverage.

11 Parent Empowerment Newsletter • February 2008 Medicaid, Supplemental Security Income (SSI), and erally lower than COBRA or individual policy premiums. You Comprehensive Health Insurance Programs (CHIPs). may have to pay a deductible, sometimes as much as $1,000. Medicare is a federal health insurance program for people Call to find out if your state offers CHIPs and exactly over age 65 and people with disabilities or end-stage renal what is covered.6 Usually, you are eligible if… disease. Medicare’s medical insurance covers only 80% of the • you have had 18 months or more of previous coverage cost of factor therapy, so all hemophilia patients should under a group health, government, or church plan obtain a supplemental insurance policy that pays the addi- • you have had no lapse in coverage longer than 63 days tional 20% coinsurance. Because Medicare doesn’t cover all • you are not eligible for another group plan, Medicare, or health services, find out what’s covered, and learn about any Medicaid limits on hemophilia treatment. Medicare is divided into two • you do not have another source of health insurance parts. Part A covers hospital charges, and Part B covers • you have used COBRA, and physician-based services. Under Part B, Medicare covers all • you were not dropped from your former plan due to hemophilia factor products and reimburses you whether you nonpayment or fraud use factor as an inpatient or outpatient.4 State Children’s Health Insurance Program (SCHIP) is Medicaid is a state-run medical assistance program that offered in all states. SCHIP provides health insurance cover- pays medical bills for low-income families. The good news is age to uninsured children under age 19 in families that meet that you will get full coverage for your factor. The bad news? certain financial guidelines. States may provide SCHIP cover- To qualify for Medicaid, your annual income must be below age by expanding their existing Medicaid program, by creat- a specified poverty limit, which varies state-to-state. To be eli- ing a separate state program, or with a combination of both. gible for continued assistance, you must keep your income The goal of SCHIP is to help children in working families low—often so low that many two-income families must return with incomes too high to qualify for Medicaid, but too low to 5 to one income and sacrifice many things they once enjoyed. afford private family coverage. Although benefits vary state- We have no insurance; we reached our $1 million cap. to-state, children are usually eligible for regular checkups, We had to apply for Medicaid and give up lucrative immunizations, eyeglasses, doctor visits, prescription drug income in order to be eligible. It’s really tough living coverage, and hospital care. It’s important for families to under current income restrictions. investigate the coverage options available in their states.7 —Anonymous If you still need assistance, and you don’t qualify for the programs listed, check out other public programs that may One SSI (see below) provision, 16-19-B, allows you to be eli- help people with hemophilia pay for medical expenses or gible for continued Medicaid coverage even when you hold a receive additional services. job, as long as your adjusted income falls within certain approved levels. Since each state runs its own Medicaid pro- Title V Children With Special Healthcare Needs gram according to federal guidelines, coverage varies state-to- Programs (CSHCNP). Each state has special programs state. But all Medicaid programs offer choice of provider, as under Title V government funding. Your state health depart- long as that provider accepts Medicaid patients. Ask your ment can supply more information about these programs. doctor or HTC if they accept Medicaid patients. If they When you need instant, personalized advice on accessing don’t, you may need to use public clinics, which normally state and federal assistance and entitlement programs, turn to lack specialized care for hemophilia. Supplemental Security Income (SSI) and Social Security Disability Income (SSDI) are programs provided by Social Security. SSI pays monthly benefits to families with low incomes and limited assets that can be liquidated. Children with hemophilia may qualify for SSI if they meet the SSI definition of “disabled,” and if their family’s income and assets are low. SSDI is a social insurance program that offers Social Security tax benefits through your employer. If you are disabled, you can receive benefits based on your earnings. Comprehensive Health Insurance Programs (CHIPs) are offered by most states. These are high-risk insurance pools for people who can’t get insurance elsewhere. Under a CHIPs plan, you pay a premium that varies state-to-state, but it’s gen-

4 In January 2006, the Medicare Modernization Act (MMA) became effective. The MMA does not change Part B coverage for factor products, but it does change how much your distributor will be reimbursed. In addition, the MMA will mandate prescription drug coverage for all Medicare recipients. 5 See the Centers for Medicare and Medicaid Services at www.cms.hhs.gov. 6 An excellent resource guide, Comprehensive Health Insurance for High-Risk Individuals: A State-by-State Analysis, can be purchased through Communicating for Agriculture at 218-739-3241. 7 For more information regarding eligibility and coverage, call 877-Kids-NOW or 877-543-7669.

Parent Empowerment Newsletter • February 2008 12 ance premiums or provide free factor. While it may take some juggling, frequent phone calls, and a lot of paper- No Insurance: work, you can use each of these programs to help fill the gaps in insurance. What Next? Patient Services, Inc. (PSI). This nonprofit organization, founded by a person with hemophilia, is registered in all 50 You can lose your insurance when you states. PSI operates as a safety net to help maintain coverage change jobs or companies, resign or are laid while people with chronic disorders transition from one poli- off, depend on and then lose your spouse’s cy to another. It assists with premium payments for patients insurance due to divorce or death, reach who can’t afford them, and helps patients locate health insur- your lifetime cap, or your employer cancels ance. PSI services are free.9 insurance coverage. Research trials. Check with your HTC for new research COBRA allows you to continue your insur- studies that may offer free factor concentrate to study ance coverage after you’ve lost your job at participants. the group rate you received through your employer, under certain conditions. Free trial programs. To entice customers to switch products, factor manufacturers now offer free factor You may qualify for public assistance or through special programs. Some programs require that entitlement programs. Medicare is a federal you make a request through your HTC; others can ship health insurance program for people over product directly to you. Check all the manufacturers’ age 65 and people with disabilities or end- websites to see if your factor manufacturer offers such a stage renal disease. Medicaid is a state-run program. Supplies will be limited. medical assistance program that insures low- income families. Coupon programs. Some manufacturers provide factor at no charge when you lose insurance, if you enroll in their Along with public assistance programs, look coupon program while you are still insured. Some offer up to into Social Security and Comprehensive three years of free factor if you’re a regular customer and Health Insurance for High-Risk Individuals. have lost your insurance. This is a way to solicit customers PSI offers temporary free insurance premi- and build brand loyalty. It’s also a fabulous idea. Contact um coverage to qualified individuals, so you your manufacturer immediately to enroll in its program. But can stay insured while you work through you must be registered before you lose your insurance. If you insurance transition or loss. aren’t registered now, please get registered! There are many ways to get free factor: Compassionate care programs. Pharmaceutical and research trials, free trial programs, manufac- home care companies sometimes offer compassionate care turer coupon programs, and compassionate programs that provide free factor concentrate to families care services. who can prove financial hardship. This is usually a one- time shipment. Your HTC social worker or nurse can help When insurance change threatens or occurs, you with this resource, or you can visit the pharmaceutical contact your HTC social worker, local websites directly. You can also call us at LA Kelley hemophilia chapter, or reimbursement special- Communications, Inc.10 ist with your home care company or factor manufacturer. The entire hemophilia community—patients, HTC staff, home care companies, pharmaceutical companies—realizes that insurance is the foundation of good healthcare. Strive to maintain your current insurance, and know your Advocating for Chronic Conditions, Entitlements and Social options when you change insurance. If you lose insurance, Services (A.C.C.E.S.S.). This free service is funded by be aware of the safety nets available to you. Preparation is Hemophilia Health Services and offers in-depth information your best hope for preventing a disastrous situation: no about public insurance from experienced hemophilia experts.8 insurance and no options. The waters of hemophilia insur- ance are choppy, full of obstacles that can shipwreck you. Navigate these waters well with the help of all the Other Options for Obtaining resources listed here. An entire community is waiting to Insurance and Factor assist you.

Some families may not qualify for public assistance but are threatened with the loss of insurance. The hemophilia community has special programs that can help cover insur-

8 Contact A.C.C.E.S.S. at 800-999-LIFE. 9 Contact PSI at 800-366-7741. 10 Contact LA Kelley Communications, Inc., at 800-249-7977.

13 Parent Empowerment Newsletter • February 2008 Inhibitor Insights... continued from page 4 soon shot up to 800 BU. Who’s Reading PEN? Fortunately for Dustin, the toleriza- tion program was successful. Within We have many readers throughout the world, a year, his titer dropped to zero. but who reads PEN here at home? Look below Dustin’s childhood hasn’t been to see who’s reading PEN in your neck of the “Boy easy. He has suffered many compli- woods. It’s not surprising that our top readership Scouts… cations of hemophilia. He has had the best two Broviacs® and two Port-a- mirrors the states with the biggest populations, thing Caths®. He’s had four infected but look at Maryland! Way to go! In 2008, help Dustin access devices that required surgical us reach more families by making it a point to ever did.” removal. One Port-a-Cath eroded share PEN with people in your state. How does through his skin, another clotted off several times, and a third developed your state measure up? a leak. Now age 13, Dustin has arthritis NUMBER OF NUMBER OF in his ankles, knees, hips and STATE READERS STATE READERS elbows. His ankles are target joints, California ...... 218 Colorado...... 27 and he developed synovitis in both. Ohio ...... 199 Alabama ...... 26 In the winter, Dustin’s ankles and Pennsylvania . . . . . 192 Nebraska ...... 25 knees are often so painful that he Texas ...... 164 Maine ...... 22 can’t get out of bed. For four years, New York ...... 153 New Hampshire . . . . 22 Dustin relied on a daily low dose of Michigan ...... 136 Nevada ...... 19 VIOXX® to reduce his pain and Florida ...... 130 Idaho ...... 16 inflammation. When the drug was Massachusetts . . . . 125 New Mexico ...... 16 pulled from the US market in 2004, Illinois ...... 113 West Virginia ...... 14 Dustin’s ankle bled more frequently Indiana ...... 86 Mississippi ...... 13 and became increasingly painful. Virginia ...... 84 North Dakota ...... 13 Dustin had a radionuclide synovec- New Jersey...... 82 Montana ...... 11 tomy performed on his left ankle. Minnesota...... 76 Rhode Island ...... 9 Due to the thickness of his synovium North Carolina . . . . . 73 South Dakota ...... 7 (his ankle was the size of a grape- Missouri ...... 71 Delaware ...... 6 fruit), the synovectomy successfully Louisiana ...... 67 Vermont ...... 6 removed only half the synovium. Tennessee ...... 67 Alaska...... 5 Seven months after his synovectomy, Maryland...... 59 District of Columbia a cortisone injection in Dustin’s Washington...... 57 (DC) ...... 5 Hawaii...... 5 ankle provided significant relief. Wisconsin ...... 57 ple problems. He gnawed his right Kansas ...... 52 Wyoming...... 4 index finger almost to the bone. He But life for Dustin is finally look- Iowa ...... 49 suffered bleeds because he was ing up. Getting tolerized to factor— Arizona ...... 46 Puerto Rico...... 12 unable to stop himself with his right and having the synovectomy and Georgia...... 43 arm when he fell. Dustin underwent cortisone injection—has allowed him Connecticut ...... 41 three years of physical therapy to to go about his daily routine with Kentucky...... 41 regain the use of his right arm. only minor arthritic pain. Since the South Carolina . . . . . 39 Although he can now use it, he still synovectomy, he’s had far fewer Utah ...... 39 lacks fine motor skills in his right bleeds in his left ankle. A little over Arkansas ...... 38 hand, and simple tasks, such a tying a year ago, Dustin joined the Boy Oregon ...... 38 a shoelace, are difficult. Scouts. He has participated in an Oklahoma ...... 28 To get the specialized medical eight-mile hike, four-mile backpack- care he needed, Dustin was ing trip, and 25-mile bicycle ride. switched to an HTC in Orange Recently, Dustin went rock climbing County, where he was started on with the scouts—no problems—and Immune Tolerance Induction, with looks forward to enjoying future monthly infusions of IVIG, a prod- outings. Pattie says, “Joining the uct that maintains adequate anti- Boy Scouts is the best thing Dustin body levels to prevent infections. At ever did.” With proper care from the beginning of treatment, Dustin’s educated parents and an excellent inhibitor titer was 185 BU, and it HTC, Dustin is finally living life to the fullest.

Parent Empowerment Newsletter • February 2008 14 Transitions ... continued from page 8 • Be sensitive to people’s reactions: a Here’s how: • Don’t pick at scabs. tattoo carries a message. • Wash the tattoo an hour after appli- • Tattoos can get infected. If the skin • Removing a tattoo always leaves a cation with warm, soapy water; pat around the tattooed area becomes scar. dry with a clean towel. Do not rub very red, swollen or tender, see a • Research tattoo removal: laser treat- the tattoo. Apply the antibacterial doctor; you might need antibiotics. ointment given to you by the tattoo ment, dermabrasion, salabrasion, Even when you try to talk him out of artist and cover the tattoo. Repeat scarification. Painful, painful. it, even when he takes proper precau- this process daily for about a week. • Never put a girl’s name on your tions, shows good taste (can’t go wrong body unless it is your mother’s. • Try not to scratch it, although it will with Latin, right?), and shares his zeal (Or a guy’s name. Just ask leak some fluids, become itchy and with you, you may still scratch your Angelina Jolie.) peel. head and wonder, why? Why do they do • Don’t soak your tattoo while it heals. it? Possunt quia posse videntur: They can This means no swimming or baths. because they think they can. Motto for • Don’t expose your tattoo to direct the young, and for the young at heart. sunlight while it heals. Use sun- screen when outdoors.

Want to be Cutting-edge? Use a Sharps Container—Correctly! Know the Health Risks “Sharps container” is a generic name for any single-use hard container made for disposing of used medical needles and syringes. Proper Tattooing requires repeated needle disposal of needles and syringes is important—for you, and for those pricks that inject dye. This is done with who take your trash. To prevent the potential spread of blood-borne a small machine. With every puncture, diseases, and to avoid needlesticks, do not throw needles, syringes and the needle inserts tiny ink droplets. The sharps containers into household trash or recycling bins. Bring them to a tattooing process may last from 20 min- drop-off site or hospital, or return them using a medical mail-back service. utes to several hours, depending on tat- too size. It may cause bleeding and Purchase sharps containers from a medical supply store, or call swelling—a huge concern for someone your home care company. with a bleeding disorder. Bring a sharps container when traveling. The Mayo Clinic says, “Getting a tat- too at an accredited professional tattoo Do not put sharps containers in the garbage or with recyclables. parlor is relatively risk-free, but getting a Put used syringes in a sharps container immediately. tattoo still puts you at risk.” What are the risks? Do not throw used syringes in the garbage. • serious infectious diseases, such as Do not flush used syringes down the toilet or drop into storm drains. hepatitis C, hepatitis B, HIV or tetanus Do not clip, bend or recap needles. • skin infections, such as impetigo Keep needles away from children and pets. • dermatitis • allergic reactions Use a new needle every time you inject. • thick scars called keloids Get tested to learn your HIV/viral hepatitis status. A person with hemophilia should Never share needles or syringes. always consult a hematologist before get- ting a tattoo. Check out these sharps container mail-back services. All provide a variety of container sizes and prepaid mailing cartons. Take Care of the Tattoo Medasend 800-200-3581 www.medasend.com A tattoo is an open wound until it heals, Sharps Compliance 800-772-5657 www.sharpsinc.com usually within a week. During that time, Stericycle 800-355-8773 www.stericycle.com your young adult must take care of it.

15 Parent Empowerment Newsletter • February 2008 Storm Watch... continued from page 6 unlike any other—it employs a large choice. Tom Albright of Arkansas is number of bleeding disorder patients furious that patients are being and parents as representatives. Parents switched to PrecisionRx. In a letter to or patients who work for home care PrecisionRx, Tom wrote, “I like to companies often purchase factor have a choice in who my provider is. I through their employer, providing the believe I know my home care provider employer with a stream of revenue. far better than Blue Cross ever will. These home care companies also con- What reason does Anthem/Blue Cross tract with insurance companies to pro- have for demanding its hemophilia vide health coverage to their employ- patients use PrecisionRx? Is your com- ees, regardless of whether they have pany also going to tell patients what hemophilia. But if the home care com- brand of factor they can get and how pany’s insurance provider switches sud- many doses they can receive at a time?” denly to a competitor home care com- Tom’s concerns touch on another pany as the plan’s factor provider, this fear: Where will the switching end?3 creates an awkward dilemma: Are How much power do these companies “We must accept that some change is hemophilia patients or parents, who are have in our lives? WellPoint is an inevitable.” —Bob Robinson, HFI also home care reps, going to lose their insurance giant, the largest publicly- jobs? Were they hired for their skills traded, commercial health benefits and contacts, or for their consumption company in the nation. It serves more patients can do? Document every of factor, which provides revenue? than 34 million lives, and is an inde- transaction, every phone call, and Insurers will ask the same questions, pendent licensee of the Blue Cross and every delivery. Find out if the new since in some cases, the costs to Blue Shield Association. One of every company to which you are switched is employ a patient or even to waive a nine Americans is a member of a truly incompetent or negligent. And copay are passed through to the insur- WellPoint health plan. The company check the per-unit cost. If you can er. Are patient complaints about wields considerable power. But at the show that it’s less expensive to use switching based solely on the fear of current rate of mergers and acquisi- your preferred company, you might losing a unique long-term relationship, tions of home care and specialty phar- have a case. or more on the fear of losing revenue macies, the industry may indeed be The handwriting is on the wall. for the employer? Arguing about los- left with only four or five providers, Home care switching is on the rise. ing the long-term relationship with an all powerful. Chris Blair writes, “If we do not work empathetic rep may be a weak strategy The second consumer concern is together to prevent this now, we will all once the insurer learns that the patient about lack of expertise—and how it ultimately be at the mercy of whichever or parent earns his or her revenue will impact access to and delivery of healthcare company becomes the next from the home care provider. product. This concern is more likely potential low bidder selected to service Still, patients with no ties to industry to get the insurer’s attention, particu- our unique healthcare needs. We need are also being affected. Many advo- larly if the insurer owns the specialty to stand united in our efforts to keep cates believe the battle is purely about pharmacy. The best thing families and from being controlled by insurers and their selection processes.” Bob Robinson, executive director of the Hemophilia Foundation of Illinois, Did You Know? is trying to preserve choice of home care, but concedes, “We must accept Starbucks spends more that some change is inevitable and that than $200 million annually we are going to have to learn to com- promise. There is just no way we can to provide health insurance have ‘our old way’ anymore.” for its employees—more And Bill Jamison warns, “If than it pays for its this approach is successful for WellPoint/Athem, look for the other coffee beans. Big Blues to follow.” He adds, “Our community must stand up and be New York Times Book Review, cited in heard or our choice of provider will go The Week, Dec 28, 2007 away. Educate, advocate and motivate yourself to stand up against this issue. Our lives depend on it.”

3 PrecisionRx has indicated that its open access to all hemophilia therapies will remain. It follows MASAC guidelines of a 2% +/- assay guarantee, and claims it will not mandate a formulary that eliminates choice of product or brand.

Parent Empowerment Newsletter • February 2008 16 headlines

“Bloodstopper” — Herbal Relief? NEW Turkish scientists claim to have developed an herbal product that stops external bleeding in seconds. Factor IX Products According to Professor brahim Haznedaroğlu of the IN DEVELOPMENT hematology department, Hacettepe University, this product is not a medicine, although it has been Inspiration Biopharmaceuticals has licensed by the Turkish Ministry of Health. signed an agreement with Cook Currently available in ampule and spray forms, the Pharmacia to develop its lead biologic liquid is intended for direct application to a wound. therapy, an injectable recombinant Scientists have not disclosed the names of the herbs factor IX. Founded in 2004, used in the mixture. Inspiration Biopharmaceuticals focuses on revolutionizing hemophilia treatment For information: www.turkishdailynews.com.tr in two ways: 1) developing lower-cost versions of existing intravenous recom- binant therapies; and 2) developing More FIX Humate-P non-invasive administration of therapies. Enhancements For information: www.inspirationbio.com No More Studied Refrigeration Baxter International has begun pre- According to an FDA clinical work on a genetically engi- news release on neered product for bleeding in patients October 19, 2007, with hemophilia B. Nektar ® Humate-P can now Therapeutics of San Carlos, be stored at room tem- California, and Baxter are working in Helixate FS perature. Almost all partnership to create a more long-last- NEW New 2000 IU Vials coagulation products ing treatment for hemophilia B. This can now be stored at is the second agreement between room temperature. Nektar and Baxter. In September For information: 2005, the two companies announced see product insert or that they would work together to www.cslbehring.com develop PEGylated, or longer-lasting, therapies for hemophilia A. For information: www.hemophilia galaxy.com CSL Behring’s new, larger vial size of Helixate® FS reduces reconstitu- tion time by eliminating the need to Noninjectable Factor IX? mix and pool multiple vials. This helps improves patient compliance Nastech Pharmaceutical possibility of developing an and convenience. CSL Behring also Company, Inc., has begun a alternative delivery method of introduces its new HeliTraxSM feasibility study with an undis- factor IX without an injection. system. The internet-based encrypted closed global leader in the pro- For information: database is designed for profession- duction of plasma products. www.nastech.com als to track treatment. It includes a The study will examine the wireless, hand-held electronic diary for patients to log infusions and Recombinant Factor IX treatments with Helixate FS. Product Researched For information: www.cslbehring.com or 888-508-6978 GTC Biotherapeutics, Inc., of Blacksburg, Virginia. Framingham, Massachusetts, Currently, the only existing has purchased a license to recombinant factor IX product ® Pharmaceutical develop a new recombinant fac- is BeneFIX , made by Wyeth tor IX product with Pharmaceuticals of King of ProGenetics LLC of Prussia, Pennsylvania.

17 ParentParent Empowerment Empowerment Newsletter Newsletter•• February August 2003 2008 NoseBudd Helps Stop NOSEBLEEDS Coram Community NoseBuddTM is a gel-pack that can be placed on the nose to stop Conference Calls bleeds, in combination with light Tired of emailing people or pinching. Designed by a person visiting chat rooms to talk with hemophilia. about hemophilia? This program is a great way to For information: www.NoseBudd.com stay in touch with others in the bleeding disorders community—live. The next conference call, sponsored by Bayer, is February 27, 2008, NEW Children’s Book on Hemophilia! 7:00 pm EST. Featured guest is Nancy Roy, clinical nurse I am Nate tells the story of how hemophilia affects four-year- specialist at Palmetto Richland old Nate—and how it doesn't! Infusions are a weekly part of Health HTC in Columbia, Nate’s life, just like feeding his pets, dressing himself, and South Carolina. Dial toll-free going to school. In his preschool class, Nate feels that he’s 866-213-1962 about five “just like everyone else,” and he looks forward to going to minutes before the call, kindergarten. Written by Chris Perretti Barnes, mother of a then enter 2522783 child with hemophilia. Sponsored by Bayer and BioRx. when prompted. For information: www.biorx.com Home Care

Advocate for Factor IX Meeting Choice and Hemophilia Care! The Coalition for Hemophilia B, Inc., is hosting NHF’s Washington Days takes place on March 5 – 7, 2008, in a one-day consumer symposium on Saturday, Washington, DC. Join your community, and visit state senators March 8, 2008, 9:30 am – 5 pm, at the and legislators to make them aware of our insurance problems. Millennium Broadway Hotel in New York City. NHF credits last year’s event with the increase in federal funding The NYC-based nonprofit provides informa- to the CDC’s subsidization of HTCs, now at $18.2 billion a year. tion, education and support to people with For information: www.hemophilia.org hemophilia B and their families. For information: 212-520-8272 or [email protected]

HFA 2008 Meeting Bleeding Disorders Hemophilia Federation of America’s 2008 Annual Educational Symposium HOTLINE will be held May 1 – 4, 2008, in Little for Insurance Help Rock, Arkansas. The meeting brings together families, treaters, and busi- Call toll-free 800-520-6154 with your nesses related to hemophilia for a questions about insurance changes, weekend of education and fun. reforms, restrictions, and general For information: information. Funded by www.hemophiligfed.org Baxter, initiated by the Lone Star Chapter of NHF. Nonprofit

Parent Empowerment Newsletter • February 2008 18 Medical International national level toadvocate for this.I would love to getinvolved ona company may have limits,too.I may notgetasgoodapolicy, orthe our plan.Butthattakestime, andwe insurance companiesasproviders of with privateinsurancecompanies. they allsay they’ve gottennowhere HFA, COTT,NHF, andothers,but We have mademany phonecallsto that unacceptableformy children. ence withbleedingdisorders. Ifind your area andhasyears ofexperi- instead ofonewhoisactuallyin to anurseacross thecountry— hemophilia,oran 800-number about someone whomay have noidea sonal. Theyofferaphonecallto get larger, more imper- theybecome trust completely. I can’teven usethecompany thatI and Ithinkthatishorrible.Andnow I know thisishappeningtoothers, he were tohave repeated treatments. difficult tostickattimes,especiallyif Inbox... continuedfrom page2 My company islookingintoother As thebighomecare companies are nolongerindicatedforthe Desmopressin intranasalformulations hyponatremia a severenew informationabout toincludeimportant desmopressin the prescribing informationfor thatmanufacturers update requested 4,2007: TheFDA December AlertonDesmopressin FDA Hemophilia Treatments China 33.4 millionunits.InChina,three 33.4 while production in2007 wasonly millionunits, was48.9 tion in2006 companies. Total produc- factorVIII by Chineseblood product factor VIII year. Thisledtoreduced production of from theprevious in2006 50% about Plasma supplyinChinadropped by : Lack ofPlasmaBlamedfor Shortageof nd seizures. electrolyte imbalance. illnesses that may lead to interruptedduringacute should be treatment withdesmopressin tablets with a hyponatremic patientsor usedin Theyshouldnotbe enuresis. treatment ofprimarynocturnal Source: in HenanProvince. Engineering and HualanBiological Green Co., Cross ChinaBiotheological BloodProducts Co., Shanghai RAAS produce clottingfactorVIII: pharmaceutical manufacturers K of that affectmy children. refuse toletanything remotely like andI in1990, lost my dadtoHIV choice ofmedicationortreatment. I provider, our buteventually itwillbe choices. Today it’sourchoiceof and privateinsuranceslimitingour reform forhealthcareofficials about our localandnationalgovernment year, andhave writtentomany of plan togoWashington Days this Ziva inthefuture! tomorrow. Hopetoseemore from am takingittoourson’steachers extended familyover the holidays and hemophilia. Ishared itwithour andteachers ofboysgivers with It isamust-read forallparents, care- Son withHemophilia”by ZivaMann. “From Boys toMen:NurturingYour DSO THE ON UDOS PEN Texas Shanna Garcia Name withheld history ofhyponatremia. PNE www.Xinhuanet.com , especiallythecover story N OVEMBER 19 fluid and/or 2007 ISSUE Parent Empowerment Newsletter Parent Empowerment Newsletter www.novonordisk-us.com/biopharm corporate sponsors www.hemophiliagalaxy.com www.HemophiliaHealth.com acknowledges our www.hemophiliavillage.com [email protected] Reimbursement HELPline PEN gratefully www.kogenatefs.com www.cslbehring.com Baxter’s website for website for Baxter’s hemophilia families Customer Service Service Customer 800-243-4153 800-288-8374 800-727-6500 800-423-2862 800-800-6606 888-999-2349 888-508-6978 • 2008 February • August 2003 sign up to receive name is available either in hard copy or electronically in PDF format. To receive PEN electronically in a PDF file, you must download Acrobat Reader (free through Adobe at www.adobe.com). PDF files save us postage and arrive about two weeks before the hard copy. organization is free to patients, families, hospitals, nonprofit organiza- tions and corporate partners of LA Kelley Communications. Other interested readers may subscribe for $24.95/year (mail/hard copy) or address $14.95/year (email/PDF). To sign up, simply complete this form and return it to the address below with a check or money order made payable to LA Kelley Communications, Inc. city/state/zip/country LA Kelley Communications, Inc. 68 East Main Street, Suite 102 Georgetown, MA 01833 USA daytime phone email address Or subscribe online at www.kelleycom.com Check any that apply: I would like to receive by: name and date of birth of child(ren) with hemophilia patient email only (PDF file) parent post only (hard copy) factor deficiency type and severity medical treater both educator Join Our Research Team hemophilia Do you want maintains a special network of patients and parents to provide us organization to join the with information for upcoming articles and projects. We want to get your hemophilia Research ideas, opinions and experiences periodically through telephone surveys, company Team? interviews, or written questionnaires. If you’d like to be on our elite team, cut along the dotted line Yes! No check “Yes” in the box at left, and send or email this form to us.

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