Volume 6, Issue 1

Winter 2015 Children’s Heart Center THE HEART BEAT

Goryeb Children’s Morristown Medical Center HRISTINE ONNELLY 100 Madison Ave. T HE D IRECTOR ’ S C UT C D , M D Morristown, NJ 07962

Goryeb Children’s Center Heart History: Pioneers in world of children born with Overlook Medical Center Pediatric -The cyanotic heart defects but in 99 Beauvoir Ave. Collaboration of Helen the birth of pediatric cardio- Summit, NJ 07901 Taussig, Alfred Blalock vascular medicine. Newton Medical Center and Vivien Thomas “to elen Taussig obtained 175 High Street seek a newer world” – the her doctor of medicine Newton, NJ 07860 th H 70 year anniversary degree from Johns Hopkins Flemington Specialty Pediatric Care his year is the 70th anni- University in 1927, a time 194 Route 31 versary of the first pallia- when few women were phy- Flemington, NJ 08822 T tive operation to help chil- sicians and some medical Physicians dren born with severe cya- schools did not even accept Christine Donnelly, MD-Director notic congenital heart de- women as students. Under vessel was able to bypass Stuart Kaufman, MD the area of obstruction and Donna M. Timchak, MD fects. There is no other pro- the mentorship of Dr. Ed- Suzanne Mone, MD cedure in the history of pedi- ward Park, she was placed provide blood to the lungs. Lauren Rosenthal, MD, MPH atric cardiology that changed in charge of the cardiac unit She was convinced that Aparna Prasad, MD the lives of so many and so at the Harriet Lane Home for most of her cyanotic patients Leonardo Liberman, MD-EP Consult. could be helped by creation quickly than what is now Invalid Children at Johns Nurses known as the Blalock- Hopkins Hospital and began of an “artificial ductus.” She Maria Lawton, RN, BSN Taussig shunt. In honor of her life’s work in the care of needed, however, to con- Megan Dickinson, RN, BSN vince a potential “creator” of Andrea Winner, RN, BSN, CEN this landmark event, we are patients with congenital reprinting the story of this heart disease. After a dec- such. Technologists achievement from our first ade of observations and care r. Taussig approached Colleen Henderson, RCS, RDCS- issue of The Heart Beat. of her “blue babies” she de- PE,FE, Lead Clinician D Dr. Alfred Blalock, the Alexis Terlaje, RCS, RDCS-PE,FE, Blalock, Taussig and Thom- termined that most of her newly appointed Chief of Lead Technologist as were true pioneers and patients were blue because at Hopkins with her Bhavisha Pandya, RDCS the world of pediatric cardiol- of insufficient blood to the idea. Dr. Blalock with the Alla Greenberg, RDCS Ashley DeRosa, RCCS ogy will be forever indebted lungs. What is truly remark- aid of his surgical assistant Kelli Vranch, RDCS to this remarkable team. able is that she was able to Vivien Thomas had previ- Ginamarie Casale, RCS, RDCS, RVS oday, there are as many determine this through her ous experience with creating clinical skills and armed with this artificial ductus but for a Medical Assistants T adults living with con- Joanne Kobylinski genital heart disease as rather rudimentary diagnos- different indication. Over the Kimphany Chillious there are children. This tic tools of a stethoscope (an next year, Vivien Thomas Martha Henao amplified one since Dr. was able to create an animal speaks to the advances and Social Worker successes in pediatric cardi- Taussig was nearly deaf), model of “cyanotic heart Margaret Micchelli, LCSW ovascular medicine that fluoroscopy (X-ray) and a disease” and tested the new have occurred over the past crude electrocardiogram. operation in his laboratory. Administrative Staff (There were no echocardio- Lynn Vanderyajt- Business Coord. decades. It is hard to imag- Sooner than anticipated, the Diann Vivar ine, therefore, a time in the gram machines or cardiac operation was performed on Sandy Segreto not too distant past when catheterization laboratories one of Dr. Taussig’s patients Tina Brown at that time). She observed because of her rapidly dete- Pat Orlando children born with congenital heart disease, especially that her patients with critical riorating condition. On No- Phone: (973) 971-5996 those defects resulting in pulmonic stenosis which vember 29, 1944, Dr. Alfred Fax: (973) 290-7979 cyanosis causing the “blue limited blood flow to the Blalock with Vivien Thomas Visit the Children's Heart Center Web lungs did better if their Site babies” rarely survived past standing behind him for childhood. And yet such ductus arteriosus ( a natu- guidance, “created” the first Edited by: was the case in the 1940s rally occurring vessel that “artificial ductus” shunt in 15 Margaret Micchelli & Stuart Kaufman when a remarkable collabo- connects the aorta and pul- month old Eileen Saxon and ration resulted not only in a monary artery before birth) history was made. After revolutionary change in the remained patent, since this (continued on page 3)

Save the Date: Valentine Party February 15, 2015

The Heart Beat Volume 6, Issue 1 Page 2

N URSES ’ N OTES S TAFF H IGHLIGHT M ARIA L AWTON , R N , B S N P EDIATRIC N URSE All You Need to Know About Sports began working in the division of pediatric cardiology in February Clearance I of 2006. Many of you know me from encounters during office Andrea Winner, RN, BSN visits or perhaps from dialogue on the phone. In either case, it’s a privilege to work for this department and be part of a team to which  Even if your child has you entrust your most cherished gem, your children. no activity restrictions attended nursing school at Seton Hall University and after gradu- from a cardiac perspec- I ation my journey in healthcare began. Working in various areas tive, the school may still and trying to find what it was that would bring me satisfaction and a need a sports clearance sense of accomplishment was my goal. One afternoon, I met a form. family whose child had significant heart disease and I recall being very curious and intrigued.  Please familiarize your- For weeks afterwards, it consumed my thoughts and I found myself reading about cardiac self ahead of time with anomalies. It was then I realized I had to pursue this interest in pediatric cardiology. your school’s specific hen I’m not working, my time is spent with my husband and two children. I enjoy time requirements and outdoors at sporting events, gardening, swimming, bike riding, jogging, cooking and forms. W entertaining my family and friends. My absolute favorite pastime is sitting back with my hus-  The best time to request band as we watch and listen to the sound of our children’s laughter and happiness. I relish sports clearance is at these times and hope that my involvement in any child’s life will allow many parents to treasure your regularly sched- similar moments. uled pediatric cardiology appointment.  Think ahead! If your appointment is in the CARDIOLOGY AND G ENETICS D ARIUS J . A DAMS , M D spring, you can still re- quest the form for fall or ver the past decade, numerous discoveries have provided evidence winter sports. O about the genetic etiologies of congenital heart defects and the roles  Due to high volume at that these genes play. Although there is still much to be learned, these the beginning of the advances point to better care for pediatric cardiology families. school year, we may be hen cardiac defects are seen in junction with other anomalies, ra- unable to respond im- W ther than presenting as a single medical problem, the etiology is mediately to your sports often easier to identify. However, such syndromes account for less than clearance request. 20% of congenital heart defects identified.  An RN answers phone hromosome analysis, by blood test or cheek swab, can identify nu- messages on Monday- C meric chromosome abnormalities and sometimes provide information Friday from 9am-4pm. about deletions and duplications of large amounts of genetic material. We have been testing Calls are returned in for syndromic conditions involving heart defects, such as DiGeorge syndrome (22q deletion) order of medical urgen- and Williams syndrome, by this method for years. cy (chest pain, Rx re- owever, there are a large number of single gene disorders that have cardiac involvement. fills, etc.) with sports H Specific gene panels are available to test for Marfan’s or thoracic aneurysm-related dis- clearance as a lower orders or Noonan syndrome which is genetically heterogenous. Recent technology, such as priority. whole exome sequencing, has identified mutations that play a role in congenital heart disease  OUR NEW PROCE- that have not been included in these panels before. DURE: Please submit a ardiomyopathy and Long QT syndrome are heart conditions, not immediately identifiable stamped, self- C at birth or in early childhood, that also have a genetic etiology. Therefore, testing earlier in addressed envelope a child’s lifetime due to a family history of these conditions may provide better opportunities to with the form and it will proactively manage these diseases. be mailed back within verall, genetics plays a much greater role in the field of cardiology than was previously 10 days. O thought. It’s important that as we proceed with genetic testing that we take part in col-  Thanks for working with laborative databases to better understand the role that genetics plays and how to best help the nursing team to affected families. Clinical and research laboratories are encouraged to share identified muta- make this a smooth tions and a description of the patient’s symptoms in order to better understand the genetic process for all! basis of cardiac conditions. n this new arena for care, Atlantic Health now has the benefit of the Jacobs Levy Equity

I Management Personalized Genomic Medicine program staffed by myself, a board-certified genetics physician, and a genetic counselor. Genetic testing and post-test counseling are provided on matters related to DNA health and wellness.

The Heart Beat Volume 6, Issue 1 Page 3

D IRECTOR ’ S C UT ( CONT ’ D ) Did you publication of the results of the first 3 opera- received from a 12-year old French patient, tions in JAMA in 1945, Dr. Taussig’s clinic Jean Pierre Cablan which she kept framed was engulfed by patients, and over the next on her mantelpiece: “Je suis maintenant un know?? 4 years, over 700 operations were per- tout autre petit garcon..je vais pouvoir aller  Jumping rope involves al- formed which became known as the Blalock jouer avec mes petits comrades.” (“I am most every muscle in your -Taussig shunt. Physicians came from all now a completely different little boy… I can body. over the world to learn the technique of the now play with my friends”) - a new world operation from Dr. Blalock and Vivien Thom- indeed!  Using free weights builds as and the world of children born with con- or a dramatized account of the first muscle mass faster than genital heart disease was changed forever. using weight machines. The impact of this operation was tremen- F Blalock-Taussig shunt, see the TV mov- dous, if not miraculous, but is most poign- ie “Something the Lord Made.” antly summarized in a letter that Dr. Taussig  More than 80% of teenag- ers don’t meet the recom- mended guidelines for aer- obic activity. F AMILY C ONNECTION  Underwater swimming is ou know the look. The one that other the only time you should Y parents give you when you say your baby hold your breath while ex- had emergency open-heart surgery when he ercising. was born. That sympathetic, but “Thank God pumping and I thank God he is alive. And in it’s not me” look. Before we had Tyler, I prob- the same thought, I don’t feel lucky when I  Being dehydrated reduces ably was one of those moms who gave the think about everything we endured as parents exercise performance. not knowing if he was going to live when his look without realizing it. chest was being cut open. I don’t feel lucky t’s so tough to explain to someone else  Hula hooping for 30 knowing all that he went through after he was what goes through your mind everyday minutes burns about 210 I born. I don’t feel lucky when I know one day I about your heart baby. It’s hard to explain that calories. when I hear that particular song on the radio, will need to explain what he went through as a newborn. the one that was playing in the NICU when he  Watching TV for 30 he upside is that every minute of every was recovering with tubes & wires sticking out minutes burns about 23 day, I feel exceptionally blessed to have of him, how it brings me right back to that hos- T calories. pital room and hits me like a ton of bricks. him as our son. His scar is who he is. He Like it happened yesterday. wouldn’t be Tyler without his scar (which Julianna says is his belly button). He is so  Doing karate for 30 minutes e have a 4 year old daughter, Julianna, burns about 300 calories. W who wasn’t born with a CHD and a 1 incredibly happy and full of life, so innocent 1/2 year old son, Tyler, who was born with and perfect, and I could hold him in my arms infra-diaphragmatic TAPVR (total anomalous until the end of time. I want to protect him  Skateboarding improves pulmonary venous return). His defect was from everything in the world because I feel like balance, as well as coordi- successfully repaired at 8 days old at Colum- he has been through so much already. I know nation between your eyes, bia CHONY. there are other CHD babies who do not live legs, feet and arms. very long and/or have multiple in

o far, at 18 months old, he runs and plays their lifetime. Every situation is different. So, S like any other child. But anytime I see yes, we are lucky & blessed that Tyler is with  How was Zumba invented? him run around, I can’t help but think “Is today us and he is healthy, however I do not feel going to be the day that he has a heart-related lucky for going through what we went through. issue?” When I put Tyler to bed at night, I give him just a little longer of a kiss, and a little s time goes on, we tend to put the ‘heart longer of a nose-nuzzle on his fuzzy head. I A defect’ on the back burner of our thoughts, even though his scar is a daily re- talk very softly to him, telling him how much we love him and feel so incredibly blessed to minder of how our little boy is so unique. have him in our lives. He is such an extraordi- hen we were first going through this, nary little boy and is absolutely in our lives for W our family & friends offered great sup-

a reason. port. Then we were told about the CHD sup-

ere comes the weird part. There is a fine port group at the hospital where we talked to tapes. merengue and

other CHD parents. It was the mental relief line as to how I feel about Tyler’s CHD. salsa his with improvised

H that we needed, listening to other CHD par- Sometimes people say that we are lucky be- he So, class. aerobics cause he only had to have one open-heart ents who felt the same way we did and were

surgery. Only one surgery - Are we really going through the same thing we were. The group his for music bring

lucky? Yes, he is alive and healthy now, but I meetings really allowed us to express our to forgot instructor, fitness don’t feel lucky every day when I see his little feelings and helped us to feel not so alone. a Perez, Alberto Answer: scar. I touch it and feel his beautiful heart Skye, mother of a 1 1/2 year old son

The Heart Beat Volume 6, Issue 1 Page 4

A MERICAN A CADEMY OF P EDIATRICS :PARENT R ESOURCES

he Internet is overloaded with information. In fact,  Building Resilience in Children and Teens T there is so much information available to parents that  CyberSafe: Protecting & Empowering Kids in the Digital it may be difficult to distinguish between what’s reliable World of Texting, Gaming and Social Media and what’s not. (And that’s not including the varying quali- Sleep: What Every Parent Needs to Know ty of blogs.) If you have questions about parenting, where  do you turn to get guidance and ideas?  Sports Success Rx! Your Child’s Prescription for the Best he American Academy of is a resource for Experience T current, accurate and helpful information on topics of  Food Fights: Winning the Nutritional Challenges of interest to parents. Check out some of the book titles that Parenthood Armed with a Bottle of Ketchup are available through their website: http://  Dad to Dad: Parenting Like a Pro www.aap.org/en-us/aap-store/parent-resources/ Pages/parent-resources.aspx.

S UMMER C AMP FOR C HILDREN WITH CHD

ould you like your child to have the experience of over- The Edward J. Madden Open Hearts Camp W night camp with the reassurance that qualified staff can Ages 8 to 16 address your child’s cardiac needs? Some of our youngsters Great Barrington, MA have attended these specialized camps and have great things (413) 528-2229 to say about it. They have met new friends, developed more www.openheartscamp.org independence and had lots of fun. Please feel free to contact Hope with Heart Camp the camps to get more information and to see if it’s right for Ages 7 to 17 your child. Harriman, NY (973) 728-3854 www.hopewithheart.org

CHD COALITION WALK A SUCCESS

ver 1,000 people attended the CHD Awareness Walk and Family Fun Day on 9/21/14 at Darlington Park in Mahwah. The O AHS pediatric cardiology team members of the “The Heart Throbs” were joined by many of our families to show support and increase awareness of congenital heart defects. It’s also a fun day for children with crafts, games, snacks and live music. he CHD Coalition, a non-profit parent organization, offers support to families, donates to CHD research, provides scholarships T to high school seniors and distributes heart bags for children having surgery. To find out more, please call (973) 291-4676.