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HearttoHeartHighlights...
Volume 9, Number 1 • Spring • Summer 2012
Identifying MolecularMechanisms ofInflammation in theDevelopment of DCMDaniela Cihakova, M.D., Ph.D.Assistant Professor, Departmentof Pathology and Director of theImmunologic Disorders Laboratory,Johns Hopkins University
Dr. Cihakova’s study, Driversof Pediatric Dilated Cardiomyopathy,focuses on how mediators of inflammationcalled cytokines lead to DCM. Using amouse model of DCM, Dr. Cihakova andher colleagues recently discovered thatan immune product, cytokine IL17A, isessential to the development of thedisease.The proposed study will determineif another cytokine (granulocyte-macrophage colony-stimulating factoror GM-CSF) is a partner of IL17A inthe development of DCM.
The second part of the project concentrateson possible treatment options. Becauseinflammation of the heart (myocarditis)often precedes DCM, the study will neutralizecytokine IL17A in mouse models withmyocarditis. If the strategy succeeds in
RESEARCH BRIEFINGS
• CCF’s 2012 ResearchGrant Recipients ..........pg. 1
• Findings from CCF-Funded Studies Publishedand Presented ..............pg. 3
• Pediatric CardiomyopathyRegistry Receives Multi-Million Dollar Grant....pg. 5
CCF UPDATES
• 4th AnnualPoker Event ..................pg. 6
• Highlights from CCFFamily Fundraisers ......pg. 7
• CCF SponsorsDocumentary,TheHeart of the Matter ....pg. 8
• Cardiomyopathy BillIntroduced to theSenate ............................pg. 8
FOR FAMILIES
• Ben Breedlove’sMessage of Hope ........pg. 1
• Lizzy Craze:A Journey with aNew Heart ....................pg. 9
• CCF Family AssistanceProgram Grant ..........pg. 11
CCFAnnounces its2012 Research Grant Recipients
“Mom,Dad, come here.You have tosee this!” Deanne and Shawn Breedlove,ran to their daughter,Ally, who was in theliving room. On her laptop was their belovedteenage son, Ben, who had died the daybefore on Christmas day. He appeared aliveand smiling, sharing his story of strugglingwith hypertrophic cardiomyopathy (HCM),surviving three cardiac arrests and seeing thebright light that had comforted him eachtime his heart had given out.
Little did they know that Ben’s two-part videousing simple, handwritten note cards wouldgo viral on YouTube—viewed more than11.5 million times and covered by internationalmedia outlets. His video, “This is my story,”called attention to the uncertainties of livingwith cardiomyopathy, but it was Ben’s under-lying message of faith andhope that touched
Ben Breedlove’sMessageof Hopecontinued, page 4
Daniela Cihakova, M.D., Ph.D., Mark Friedberg, M.D., Carmen Sucharov, Ph.D.
2012 Research Grant Recipients
continued, page 10
Ben Breedlove’sMessageof Hope
The Children’s Cardiomyopathy Foundation(CCF) is pleased to announce that $144,550in seed funding will be awarded in 2012to three pediatric cardiomyopathy studiesproposed by Daniela Cihakova, M.D., Ph.D.of Johns Hopkins University; Mark Friedberg,M.D. of Toronto Hospital for Sick Children;and Carmen Sucharov, Ph.D. of Universityof Colorado in Denver.
Each study will focus on an aspect ofdilated cardiomyopathy (DCM), theleading cause of heart failure and trans-plantation in infants and children. DCMaccounts for approximately half of thosewith pediatric cardiomyopathy, and currentlythere are no therapies available to delay orstop the progression of the disease, decreasethe need for a heart transplant or improvethe long-term survival of children with DCM.
HearttoHeartVOLUME 9, NUMBER 1 • SPRING/SUMMER 2012
The Heart to Heart newsletter is publishedbiannually by the Children’s CardiomyopathyFoundation (CCF), a national organizationdedicated to saving lives and improving thequality of life for children with cardiomyopathy.CCF’s mission is to accelerate the search fora cure by supporting research, educatingphysicians and patients, and increasing awarenessand advocacy related to the needs of childrenwith cardiomyopathy and their families.CCF is a publicly supported tax-exemptorganization as described under section501(c)(3) of the Internal Revenue Service.
Board of Directors
Lisa Yue, President & FounderBrian Nold, Treasurer
RaymondYue, SecretaryCarney HawksCarolyn KongIan SandlerEddie Yu
Medical Advisory Board
Wendy Chung, MD, PhDColumbia University Medical Center
Steve Colan, MDChildren’s Hospital Boston
Daphne Hsu, MDChildren's Hospital at Montefiore
Steve Lipshultz, MDUniversity of Miami Medical Center
Jeff Towbin, MDCincinnati Children’s Hospital
Staff
Lisa Yue, Executive DirectorSheila Gibbons, Development &Communications Senior Manager
Joanne Bloomstein, Development &Communications Coordinator
Christine Colón, Patient Outreach& Support ManagerHarriet Salk, Patient Outreach& Support Coordinator
Becky Delgado, Administrative Assistant
All submissions and correspondence regarding thenewsletter should be directed to:
Children’s Cardiomyopathy FoundationP.O. Box 547
Tenafly, NJ 07670Tel: 866.808.CUREFax: 201.227.7016
The Children’s Cardiomyopathy Foundation, including allparties to or associated with Heart to Heart will not beheld responsible for any actions readers take based on theirinterpretation of articles from this newsletter. As always,readers are encouraged to discuss medical evaluations andtreatments with their own physicians.
© Copyright 2012 Children’s Cardiomyopathy Foundation.All rights reserved.
2 Heart toHeart Spring• Summer 2012
From LisaYue,CCF Founder & President
Dear Families,
In mid-April, I traveled toWashington, D.C. for a series of meetingswith senators and representatives from the American Heart Associationand American College of Cardiology to garner more support for theCardiomyopathy HEARTS Bill. I also met with directors at the Officeof Rare Diseases Research and National Heart, Lung and BloodInstitute to thank them for their multimillion-dollar grant to the PediatricCardiomyopathy Registry (article on pg. 5), and to discuss other ways toincrease research on cardiomyopathy. I returned from the trip invigorated andmore committed than ever to raising the profile of pediatric cardiomyopathy.
Sharing the stories of our cardiomyopathy families was the highlight of thetrip and helped policy makers understand what it is like to live with or, sadly,to lose a child to the disease. In this issue you will hear the story of twoextraordinary individuals who have had to face the ultimate challenge with thedisease – heart transplantation and death. 30-year-old Lizzy Craze, diagnosedwith DCM at 13 months, was the youngest transplant recipient at the time(article on pg. 9), and Ben Breedlove was the courageous teen who lost his lifeto HCM but inspired millions with his story on YouTube (article on pg. 1).
As 2012 unfolds, advocacy remains a top priority for CCF. In the nextfew months, we will be developing an Advocacy Toolkit and launchingour grassroots initiative to get you more involved (article on pg. 8). Makingour voices heard will influence members of congress and the Administrationto prioritize pediatric cardiomyopathy research, education and awareness.We have a lot to do in this area, and I look forward to hearing your story ofhow cardiomyopathy has impacted your life.
LisaYue
SPRING APPEAL 2012CCFTeen Making a Difference
Watch your mail for a letter from Joe DiSanto, CCFteen ambassador, who tells his story of how in lessthan a year he had gone from feeling fine and playingsports to being one of the sickest patients at Children’sHospital of NewYork.
We want to continue helping kids, like Joe, and theirfamilies have a fighting chance. Please support ourefforts and contribute to our Spring Appeal.
• A heartfelt thank you to all our 2011 sponsors and donors.
• Read CCF’s 2011 Annual Report online under “About CCF/Financials.”
In 2011, CCF awarded $250,000 to the National Heart, Lungand Blood Institute’s Pediatric Cardiomyopathy Registry(PCMR) for multiple studies using the registry’s nationwidepatient data.The findings from three studies have been publishedin the Journal of the American College of Cardiology and Circulation,as well as presented at the 2012 American College ofCardiology Scientific Session.This increases the total numberof CCF-funded PCMR publications and presentations to 17(ten manuscripts and seven presentations).The dissemination ofthese study results could have potentially important ramificationsfor the treatment of children with dilated cardiomyopathy (DCM).
Incidence of and Risk Factors for Sudden CardiacDeath in Children with Dilated CardiomyopathyUntil now, the incidence of sudden cardiac death (SCD) andrisk factors for sudden cardiac arrest have not been identifiedin children with DCM. In the absence of accurate data, infantsand children have undergone placement of implantablecardioverter defibrillators (ICDs) based on recommendationsfrom studies of adults with DCM.While implanting an ICD mayprotect those at risk of SCD by normalizing their heart rhythm,the use of an ICD in a child carries certain risks and complicationsthat may offset or even exceed its benefits. Risks include leadfractures, need for lead and defibrillator replacement, implant-related complications due to body size, and the psycho-socialaspects of inappropriate shocks.
In this study, Elfriede Pahl, M.D. and colleagues determined theincidence and risk factors of SCD in children with DCM.Thestudy, which was published in the Journal of the American Collegeof Cardiology in February 2012, analyzed the clinical data of1,800 children enrolled in the PCMR. Researchers found thatthe 5-year rate of SCD in children with DCM is 2.4 percent,which is far lower than that in adults. Based on their analysis ofchildren’s echocardiography tests, they were able to identify thehighest risk factors for SCD, which include age at diagnosisyounger than 14 years of age, left ventricular dilation and leftventricular thinning. Gender, ethnicity, cause of DCM and familyhistory were not risk factors for SCD.These results help toestablish a criteria for determining which children would benefitmost from ICD therapy.
The data from the PCMR was critical for the study’s completion.“No one center sees enough children with DCM to reliablydetermine the rate of SCD or to assess current recommenda-tions for ICD placement,” says Steve Lipshultz, M.D., PCMRprincipal investigator and chairman of pediatrics at the Universityof Miami Miller School of Medicine. “This study supports theconcept that universal implantation of ICDs is probably not
warranted. However, risk-stratification is possible, and if apatient’s condition meets all the high-risk criteria for anextended period, ICD placement should be consideredfor children with DCM.”
Competing Risks for Death and CardiacTransplantation in Children with DilatedCardiomyopathyThe vast majority of children with DCM are diagnosed duringtheir first year of life, and many children with DCM are inneed of a heart transplant. In fact, DCM is the leading causeof pediatric heart transplants between 2 and 18 years of age.Currently, the eligibility criteria for heart transplants does nottake into account the various causes of DCM.As a result, somechildren are not being listed for a transplant, while other childrenare being transplanted when there is a chance that their heartmay stabilize or recover.
This study, led by Jorge Alvarez, M.D., Ph.D., used a new statisticalapproach to identify risk factors for death and transplantationin 1,731 children with idiopathic DCM. Published in Circulationin August 2011, the study discovered some important criteriain children who die that had not been used previously todetermine who should be listed for a heart transplant. Notably,the study found that impaired growth or short stature increasedthe risk of death but not transplantation. Progression to congestiveheart failure and increased age (above 6 years) also lead toworse outcomes. Dilation of the left ventricle, which has beencommonly used as a criteria for transplantation, was not foundto be predictive of death. Other differences were discoveredamong children with neuromuscular disease and myocarditis.
These findings indicate that the cause of DCM should beconsidered when evaluating the importance of predictivefactors such as ventricular dilation. As explained by a pressrelease issued by the National Institutes of Health, “… thecauses of DCM strongly affected which risk factors werepredictive of death and/or transplantation.This suggests thatestablishing the cause of the disease early on is critically important.”
Using risk factors identified in the study should help physiciansbetter identify which high-risk children will likely benefit from aheart transplant. “If the findings of this study were implementedfully and used to decide which children with DCM should belisted for cardiac transplantation, we estimate that about 50children in the U.S. annually would live who would otherwisedie,” explains Dr. Steve Lipshultz.
Progressive LeftVentricular Changes Predictthe Likelihood of Survival in Pediatric DilatedCardiomyopathyAlthough it has been established that dysfunction in theheart’s left pumping chamber (left ventricular dysfunction) isassociated with death and heart transplantation in children
Spring• Summer 2012 Heart toHeart 3
RESEARCH BRIEFINGS
continued, page 8
RESEARCH HIGHLIGHTSFindings from CCF-Funded PediatricCardiomyopathy Registry StudiesPublished and Presented
RESEARCH HIGHLIGHTS
4 Heart toHeart Spring• Summer 2012
RESEARCH BRIEFINGS
preventing DCM in these mice, the sameapproach using a drug to counteract theeffect of IL17A will be proposed fortreating humans.
According to Dr. Cihakova, “Our overallgoal is to identify the cellular and molec-ular mechanisms by which inflammationinitiates the development of DCM.Wehave already shown that we can preventprogression to DCM in mouse models byneutralizing the cytokine IL17A.We nowwant to determine whether we can arrestor even reverse already established DCM,improve cardiac function and improvesurvival.”Wendy Chung, M.D., Ph.D., CCFmedical advisor and director of clinicalgenetics at Columbia University MedicalCenter, believes that “This study couldopen a new avenue for the developmentof biologic treatments with potentiallyrapid application.”
Dr. Cihakova has been investigatingmolecular and cellular processes ofautoimmune diseases since 1998, andhas focused specifically on autoimmunemyocarditis since 2003.
Identifying Benefits of CardiacResynchronizationTherapy forChildren with DCMMark Friedberg, M.D.Associate Professor in Pediatrics,University of Toronto and Cardiologist,Hospital for Sick Children
Dr. Friedberg’s study, Patterns andClinical Significance of Electro-MechanicalDyssynchrony in Pediatric DilatedCardiomyopathy, aims to determinewhich children with DCM may benefitfrom cardiac resynchronization therapyusing a specialized pacemaker tore-coordinate the heart’s pumping action.
In some children with DCM, slow orabnormal electrical signals in the heartmuscle may cause different regions ofthe heart to contract at different times(cardiac dyssynchrony), which can lead toinefficient pumping and heart failure.
Cardiac resynchronization therapy, or CRT,uses an implantable pacemaker to sendelectrical impulses to stimulate the left and
right chambers of the heart to squeeze atthe same time. CRT has been shown toimprove quality of life and life expectancyin adults with heart failure.
In spite of its success in adults, CRT israrely used in children with DCM becauselimited experience has suggested thatchildren with DCM do not always respondto this therapy. Dr. Friedberg, however,has seen dramatic improvement in somechildren with DCM following CRT.“Some of our patients have been ableto come off the heart transplant waitinglist because their heart function improvedso significantly,” says Dr. Friedberg.
Because systematic studies have not beendone to test the use of CRT in childrenwith DCM, it is unclear which childrenwith DCM may benefit from CRT andwhich children would show the greatestimprovement with this therapy. Withoutfurther investigation, treatment guidelinescannot be developed for this therapyin children.
With CCF funding, Dr. Friedberg plansto identify patterns of cardiac contractionthat may be treated by CRT in childrenwith DCM. The research team will analyzeultrasound pictures from 150 healthychildren and 60 children with DCM.This comparison will help them to betterunderstand exactly what is abnormal inDCM and identify which patients wouldbe most likely to benefit from CRT. Theyalso will analyze tissue samples from 15children undergoing heart transplant tounderstand the relationships between thegenes associated with DCM, scarring in thetissue and abnormal cardiac contraction.
“If we can identify which patterns ofcardiac dyssnychrony are associatedwith heart failure and death, and whichcan be effectively treated with CRT, thispotentially lifesaving therapy could beoffered to more appropriate childrenwith DCM,” says Steve Lipshultz, M.D.,CCF medical advisor, and professor andchairman of pediatrics at University ofMiami School of Medicine.
Dr Friedberg is a Canadian Institute ofHealth Research and Canadian Heart
and Stroke Foundation-funded expertin the assessment of cardiac function byechocardiography in children. Since 2004,he has been researching mechanismsof cardiac dysfunction and dyssynchronyby echocardiography in children withcardiomyopathy and congenital heartdisease.
Predicting Outcomes andSeverity of Disease withBiomarkersCarmen Sucharov, Ph.D.Associate Professor in Cardiology,University of Colorado at Denver
Dr. Carmen (Kika) Sucharov’s study,MicroRNA Expression in Children withHeart Failure, aims to determine whethermicroRNAs can be used as biomarkersto predict which children with idiopathicdilated cardiomyopathy (DCM) will most
Research Grant Recipients continued from page 1
Request for ResearchApplications
Dilated, Hypertrophic, Restrictive,LeftVentricular Non-Compaction orArrhythmogenic RightVentricular
Cardiomyopathy
Funding Opportunity:The Children’s Cardiomyopathy Foundation(CCF) is inviting investigator-initiatedresearchapplications for innovative basic, clinical,population or translational studies relevant tothe cause, diagnosis or treatment of primarycardiomyopathy in children under the age of18 years. Funding is available in the range ofUS$25,000 to US$50,000 for one year oftotal direct costs.
Eligibility Requirements:The principal investigator must hold anM.D., Ph.D. or equivalent degree and have afaculty appointment at an accredited U.S. orCanadian medical institution.
Application Deadline:The 2012 deadline for application submissionis Friday, September 7. Grant guidelines andapplication form are available online atchildrenscardiomyopathy.org/site/grants. php.
For more information, contactLisa Yue, CCF president at866-808-2873, ext 901 or
continued, page 5
Spring• Summer 2012 Heart toHeart 5
RESEARCH BRIEFINGS
likely progress to heart failure and which are likely to have betteroutcomes.
Although many children with DCM progress to heart failure,15 to 35 percent of children do recover from the disease.Yetreliable guidelines to predict outcomes among infants and childrenwith DCM do not exist, and pediatric cardiologists are not alwaysable to determine which therapeutic options are best for theirpatients. Because of the risks and complications associated withheart transplantation and device therapy, a criteria for riskstratification of infants and children with heart failure couldimprove outcomes for this high-risk population.
MicroRNAs are small nucleic acids that modulate the expressionof many genes, and their presence in the blood is associated withcardiovascular disease, cancer and certain other diseases. CirculatingmiRNAs in adult patients with DCM can be associated with pooroutcomes, but their levels have not yet been studied in childrenwith DCM. Dr. Suchorov’s study will analyze microRNAs in thecirculating blood of children with DCM to determine thefeasibility of using them as biomarkers to predict pediatricoutcome and disease severity.
According to Dr. Sucharov, “If we can identify which patients arelikely to recover with the use of medical therapy, we can avoid
placing these children on the heart transplant waitlist. If we canpredict which patients are likely to benefit from a ventricular assistdevice, then we may be able to achieve better outcomes forthese challenging patients.”
Dr. Sucharov’s team will analyze circulating microRNAs in theblood of 28 children who are listed for heart transplantation –nine children at the time of transplantation, nine children whohave received a ventricular assist device and ten control subjects.A goal of the study is to identify a “signature” of circulatingmicroRNAs in children with DCM as a basis for future prognosticstudies.This would be the initial step in defining whethermicroRNAs may represent a new biomarker for childrensuffering from heart failure.
“If microRNA profiling is found to be associated with severedisease, it would open an avenue for further research andpotentially identify areas of therapeutic research,” states DaphneHsu, M.D., CCF medical advisor and chief of pediatric cardiologyat Children's Hospital at Montefiore.
A member of the genetics and epigenetics study section of theAmerican Heart Association peer review committee, Dr. Sucharovhas studied the molecular mechanisms of pathologic cardiacgrowth and heart failure for the past 10 years.
The Pediatric CardiomyopathyRegistry (PCMR) will receive twomultimillion-dollar, 5-year researchgrants from the National Heart,Lung and Blood Institute (NHLBI)to improve diagnosis and treatmentfor children with cardiomyopathy.
“We believe this unprecedentedsupport from the NHLBI for pediatriccardiomyopathy research shows howimportant this disease is,” said CCFMedical Advisor, Steve Lipshultz, M.D.Dr. Lipshultz also is the PCMR studygroup leader and professor and chairmanof pediatrics at the University of MiamiMiller School of Medicine.
The first study will use state-of-the arttechnology to map every gene for 600diagnosed children to find new gene
mutations that cause dilated, hypertrophicand restrictive cardiomyopathy, as well asidentify gene mutations that explain theage at which symptoms appear and theseverity of those symptoms.
The second study will look at the relation-ship between specific blood tests, calledcardiac biomarkers, along with echo-cardiogram and cardiac MRI images todetermine if these tests can predictshort- and long-term outcomes forchildren with dilated cardiomyopathy.The study will determine if specificcardiac biomarkers can indicate thepresence of myocardial fibrosis, athickening of the heart, in cases ofhypertrophic cardiomyopathy.
Results for these two research projectsshould improve the cardiologist’s ability tobetter diagnose and more effectively treat
children with cardiomyopathy. It also canimprove screening of other family membersfor the disease and help in the developmentof new cardiomyopathy therapies forchildren.“We are confident that the resultsof this research will likely lead to bettertreatments, which will improve the lives ofpeople with cardiomyopathy and theirfamilies,” adds JamesWilkinson, M.D., M.P.H.,professor of pediatrics and epidemiologyand director of the PCMR administrativecoordinating center.
The PCMR is a national database of 3,500patients age 18 and under with selectedcardiomyopathies from nearly 100medical centers in the United States andCanada. CCF has been actively involvedwith the PCMR over the years fundingauxiliary studies, working group meetingsand the establishment of a linked DNAand tissue repository. “CCF is an impor-tant and integral partner in all that wedo,” said Dr.Wilkinson.The PCMR studygroup meets biannually to analyze newstudy data, discuss updates on currentPCMR manuscripts and determine howstudy findings should be disseminated.
Pediatric Cardiomyopathy Registry ReceivesMULTIMILLION-DOLLARRESEARCH GRANTS fromNHLBI
Research Grant Recipients continued from page 4
It was a royal flush for CCF’s All-In ForA Cure No-Limit Texas Hold ‘EmTournament. With a sold-out crowd of235 people and 30 corporate sponsors,the event raised more than $245,000—a 15 percent increase from last year—forCCF’s research and education initiatives.
CCF Board Member Carney Hawkskicked off the event with openingremarks. “Thank you for attending ourFourth Annual Poker Tournament duringNational Heart Month,” said Hawks.“Your support allows us to continueour mission of helping kids with cardio-myopathy have strong, healthy hearts.”
Guests and players from top law firmsand financial companies in the metro areaenjoyed cocktails and appetizers at the
start of the evening and movedon to a rousing game of pokerthat lasted well intothe night.
Grand-prize winnerDavid Croll ofImperial won a$10,000 entry tothe 2012 WorldSeries of Poker MainEvent in Las Vegas.David works atImperial Capital withJason Alpin, winnerof CCF’s 2009 PokerTournament.
Second prize of anall-inclusive trip fortwo to LasVegas went to James Cullinaneof RBS. Gary Lehrman of 1798 GlobalPartners won the third prize, an AtlanticCity getaway for two to the Borgata. Fourthprize of golf and lunch at Hudson NationalGolf Club went to Brian Potash of IntradeGroup. David Yaker was the fifth-prizewinner with four tickets to a NewYorkGiants game. Colin Lancaster,Travis Hogan,George Goudelias, Bob Franz and DaleStohr won the sixth through tenth prizes,gift certificates to popular NewYork Cityrestaurants.
6 Heart toHeart Spring• Summer 2012
CCF UPDATES
2012 Event Sponsors: Rob Barrett; Jay Bharadwa; Brigade CapitalManagement; Benji Cheung/Bob Franz/Jed Kelly; Credit Suisse; Robert & KarenDishner; John Florio/Andy Stock; Eric Friel; Rob Gallivan; Gibson Dunn & Crutcher;Bill Goebelbecker; Imperial Capital; Katten, Muchin & Rosenman; Knight CapitalGroup; Kramer Levin Naftalis & Frankel; John Lugano; Chris Mikosh; EricNeedleman; Mike & Beth Neumann; Gene Pagnozzi; Richards Kibbe & Orbe;Riva Ridge Capital Management; Royal Bank of Scotland; Edgar Sabounghi; IanSandler; Gary Stanco;Andrew Susser;Taconic Capital Advisors;Tullett Prebon/Chris Berry/Matt Lambert; UBS; Eddie Yu.
SAVETHE DATE!
Montclair Golf ClubWest Orange, New Jersey
Monday July 16, 2012Join CCF for this popular
and fun-filled event!Visit our event web page
under “News & Events/Golf”for more information.
More event photos are online under “News & Events/Poker.”
Lisa Yue and grand prize winner David Croll
Above: Ashley Singer, Bob Franz and Bob Frahm
Below: Participants enjoy a lively evening playing poker
CCF POKER EVENTDeals aWINNINGHAND
Four Years in a Row
CCF POKER EVENTWINNINGHAND
The Third Annual Cinca ShoppingExtravaganza in Cocoa Beach Fla. hadanother successful year, raising morethan $21,000 for CCF. More than 150people attended the event in honorof Cristina Cinca, age 11, who was
diagnosed with hypertrophic cardiomyopathy when she was 7 years old.
Held on November 19, 2011, attendees enjoyed a day of food, shopping andfabulous entertainment courtesy of Cristina, her friends and her 7-year-old brother,Parker, who enthralled the crowd with hismagic tricks. More than 26 vendors, including31 Gifts, Avon, Jockey and Creative Memoryparticipated in the event. Funds were raisedthrough a percentage of event sales, sponsorsand silent auction proceeds.
“This is Cristina's favorite day of the year, and itis worth every minute I put into it just for that!”says Heather Cinca who is already hard atwork planning the next year’s Extravaganza.
Spring• Summer 2012 Heart toHeart 7
CCF UPDATES
Dedicated CCF Staff Member GivesFoundation SupportCCF Patient Outreach and Support Coordinator, HarrietSalk, organized a fundraiser lunch at her daughters’ schoolduring Heart Awareness Month. Held at J. Spencer SmithElementary School inTenafly, N.J. on February 9, more than250 students and teachers participated.The student lunchroomwas decorated with red and white balloons, and studentsenjoyed a pizza lunch along with heart-shaped cupcakes.“Itwas a great day in honor of CCF, ” said Harriet.
Crossingthe Finish Linefor CCFWith almost 100 percenthumidity and temperatureswell into the 80s, KavithaBaratakke ran a half-marathonin San Antonio,Texas. with animpressive time of 2:07:33.She ran in honor of herdaughter, Sharanya, who has
hypertrophic cardiomyopathy.Through her efforts, Kavitharaised more than $2,000. “I'm so glad I was able to dosomething for CCF because the Foundation has helpedme enormously in my journey,” said Kavitha.
Raising Heart Rates, Raising FundsJared Markiewicz of Functional Integrated Training inVerona,N.J. held a “Get Fit” boot camp and raised more than $1,000for CCF.The hour-long fitness training was held at Glacier EdgeElementary School. “The boot campwent off without a hitch and wasawesome,” said Jared. It was inhonor of a camper whose son hashypertrophic cardiomyopathy.
Holiday Donation DriveFrank and Kristi Shippers held anonline holiday fundraiser in honor oftheir son Cooper, who was diagnosedwith hypertrophic cardiomyopathy andleft ventricular non-compaction (LVNC).They sent emailsto family and friends asking for online donations on theircrowdwise.com gift page.“Because LVNC is a rare disease,we created this fundraising page in hopes of raising moneyfor CCF to help with research,” said Frank.
Will CCF Be MineFor a second year in a row, the Tyngsborough ElementarySchool in Massachusetts held a February Heart Month fund-raiser in honor of Assistant Principal Scott Middlemiss’ son Joewho has hypertrophic cardiomyopathy. Led by teachers EricaYandow and Jen Trischitta, the school’s student council madeand sold valentines to students and staff on Valentine’s Day.
FAMILY FUNDRAISERS
CINCA FAMILYFUNDRAISER KEEPSGETTING BETTER
Above: Heather, Cristina and Parker Cinca
Right: Parker Cinca and friend
8 Heart toHeart Spring• Summer 2012
CCF UPDATES
CCF SponsorsDocumentary,THE HEART OFTHE MATTER
CCF is working with the Children’s Heart Foundation to sponsor The Heart of theMatter, a one-hour documentary on congenital heart defects (CHD).The program,which focuses on the world’s #1 birth defect, premiered Sunday, May 6 onWTTW-Channel 11 in the Chicago area.
Produced by The Children’s Heart Foundation and TMK-Productions, the documentaryis narrated by Golden Globe nominated actor Chris O’Donnell and examines allaspects of congenital heart disease from the development of the heart in utero throughadulthood. The Heart of the Matter includes interviews with the top cardiologists andcardiothoracic surgeons in the field as well as families whose children have beendiagnosed with congenital heart defects and other heart diseases. Among the manyinterviews, Brian Littrell of the The Backstreet Boys talks about the impact of livingwith CHD and Chicago Bears’ cornerback, Charles Tillman, shares his story abouthis daughter’s cardiomyopathy and subsequent heart transplant. CCF’s involvement willbe acknowledged in the documentary credits, website and promotional materials.
The program will be available for broadcast to all PBS station throughout the U.S. afterJune 1.The trailer and a listing of PBS stations and broadcast times are available on thedocumentary’s website congenitalheartdocumentary.com
First-EverCardiomyopathy LegislationIntroduced to the SenateOn February 17 during National Heart Month, Senators Frank R. Lautenberg(D-NJ) and Robert Menendez (D-NJ) introduced the Cardiomyopathy HealthEducation, Awareness, Risk Assessment and Training in the Schools (HEARTS) Actto the Senate (S.2124).
Since the December introduction of the HEARTS House Bill (H.R.3625) by CongressmanFrank Pallone (D-NJ6), the Children’s Cardiomyopathy Foundation (CCF) has beenactively working with the New Jersey Senators to introduce this companion bill inthe Senate (S.2124). CCF has secured 25 organizations, including the American HeartAssociation and members of the Sudden Cardiac Arrest Coalition, to support boththe House and Senate HEARTS Bill.The bill also has the bipartisan support of nineco-sponsors in the House of Representatives. CCF continues to make the rounds onCapital Hill to impassion more legislators to co-sponsor this bill, which can help toidentify more undiagnosed children with cardiomyopathy at risk of sudden cardiac death.
In August, CCF will introduce its Advocacy Toolkit with basic information on thelegislative process, a step-by-step guide on being an effective advocate, and sampleletter and email templates.These resources are being developed to make it easier forCCF supporters and families to approach their district congressman and state senatorsto cosponsor the Cardiomyopathy HEARTS Act.
with DCM, the effect of left ventricularwall thinning in combination withdilation has not been identified.
Led by Paul Kantor, M.D., this studyanalyzed echocardiogram records from1,457 DCM patients to determine howprogressive changes in the size, thick-ness and function of the left ventricleare predictive of death, heart trans-plantation and survival at 12 months.
They found that when progressivechanges in left ventricular size, wallthickness and function were measured,rather than just the degree ofabnormality at the time the child wasoriginally diagnosed, the outcome wasmore clearly predictable. An increasinglyenlarged left ventricle or one thatcontinued to show poorer functionover time was more associated witha poor outcome (death or transplan-tation) than one that did not worsen,while a smaller left ventricle with athicker left ventricle wall was linked tosurvival. According to Dr. Kantor, “Thesefindings suggest that serial assessmentsof echocardiographic changes should beconsidered when determining treatmentplans for children with dilated cardio-myopathy.”This study emphasizesthe importance of ongoing, carefulassessment of children with cardio-myopathy to identify whether theirrisk is increasing or not.
This study was selected for oralpresentation at the March 2012meeting of the American College ofCardiology. “This selection indicatesthe importance of its findings, aspediatric epidemiology studies arenot often accepted for presentationduring this conference,” states JamesWilkinson, M.D., M.P.H., PCMR studyinvestigator and director of the PCMRadministrative coordinating center.
Research Highlights continued from page 3
“...these study results couldhave potentially important
ramifications for the treatmentof children with
dilated cardiomyopathy.”
THE HEART OFTHE MATTER
FOR FAMILIES
Spring• Summer 2012 Heart toHeart 9
Dressed as Ernie,her favorite characterfrom Sesame Street,Lizzy Craze spenther Halloweentrick-or-treatingthrough the whitecorridors of Stanford Hospital in Palo Alto, Calif. Justweeks before, on October 8, 1984, Lizzy made medicalhistory as the youngest person to undergo a successfulheart transplant in the U.S. Staff beamed at the brave, littlegirl they taught to ride a tricycle during her one-month stay.
Lizzy, diagnosed at 13 months old with dilated cardio-myopathy (DCM), was only 2 years old when she receiveda new heart. Her parents lost three children to the diseasebefore Lizzy was born and her brother, Andy, who also hasDCM but a milder form, had a heart transplant a yearbefore Lizzy when he was 16 years old. Lizzy’s mothercalls her two living children “miracles.”
Today, 30-year-old Lizzy, an event coordinator for Facebook,has been featured in many publications including a 2009article in People magazine. She takes her “celebrity” status instride. “I don’t tell a lot of people,” says Lizzy. “I let them findout on their own.” When they do learn of her story, Lizzysays, “they’re blown away.”
Even though Lizzy’s transplant was considered a milestonein medical history, Lizzy was too young to understand thesignificance of the transplant. She lived with the new heartfrom such an early age she never knew anything different. Itwas something she always accepted.That is, until at age 15the anti-rejection medication she was taking for her heartcaused her kidney to fail. Needing a kidney transplant madeher feel vulnerable for the first time. Until then, she hadnever realized the seriousness of her condition.
Now Lizzy takes nothing for granted. During a crispafternoon while brewing beer with her boyfriend —a fun
hobby they took up together three years ago—theyuncovered an old “Life To Do List” she had jotted down
on a brew log. She is now busyaccomplishing the goalson her list. Last August shecompleted a 20-mile, 2-daybackpacking trip in Yosemite,and recently she received herscuba diving certification aftertraining in Fiji.
“Being active is so important,”advises Lizzy to other teenand young adults with cardio-myopathy. “The medicines canincrease your appetite and makeyou feel sluggish.The steroidsmake your face puffy,” says Lizzywho maintains a regular workoutregime at the gym and monitors
her heart rate. “Even if you don’t feel like it, get out and dosomething. Go for a brisk walk or bike ride; it’s likely you’llfeel better when you’re done.”
About 370 children each year receive a heart transplantwith an average wait of six months. Lizzy knows first-hand,from waiting for both a heart and kidney, how lonely being“listed” can be. Shenow volunteers ona transplant patientadvisory panel atStanford Hospital,which was estab-lished to helpimprove thepatient transplantexperience. Shealso works directlywith hospitalpatients providingsupport andmentoring asthey wait for anew heart. Lizzyadvises others to“Try to have apositive attitude.It can affect yourmind as well asyour body.”
LIZZY CRAZE:A Journey with a
NEW HEART
LIZZY CRAZE:A Journey with a
NEW HEART
Lizzie Craze and Sentinel Dome inYosemite National Park.
Lizzie Craze hiking inYosemite National Park.
10 Heart toHeart Spring• Summer 2012
FOR FAMILIES
thousands of people who had never methim, including one famous rapper.
CCF recently reached out to Deanne andShawn Breedlove, Ben’s parents, to learnmore about Ben and their family’s journeywith cardiomyopathy. In her quiet, southerndrawl, Deanne spoke lovingly about her son,the courageous young man in the videowho captivated the world. "It’s exciting toknow that Ben planted a seed in people'smind to begin thinking about things thatreally do matter in life," said Deanne.
Ben was diagnosed with HCM at 13months due to a heart murmur. He wasgiven medication and remained asympto-matic until at age 4 he developedabnormal heart rhythms (arrthymia).As Ben says in his video, he “cheateddeath three times.” His first suddencardiac arrest happened at age 4 followinga severe seizure. During his near-deathexperience, he describes a light fromabove that calmed his fears.
Although the seriousness of his heartcondition was now known, Ben grew uphappily and, for the most part, unbotheredby his cardiomyopathy. He, along with hissiblings Ally and Jake, enjoyed an activechildhood inTexas filled with swimmingand wakeboarding on the lake behindtheir house. At the same time, he followedhis cardiologist’s guidelines to take it slow.
“We found smart ways to be active,”says Deanne.
Deanne and Shawn tried to keep life asnormal as possible for Ben, even thoughthere were concerns related to living withan unpredictable heart condition.Theydidn’t change their parenting style for Ben;they modified it. Ben had chores just like hissiblings.“As parents, it’s our responsibility toteach our children values and responsibility,”says Deanne. One of Ben’s chores wastaking out the trash, but if he was particu-larly tired that day or the trash was tooheavy, his parents modified the task toaccommodate his condition.
The Breedloves have always valued a strongwork ethic.When the Breedlove childrenturn 16, they are expected to find a job.Ben was no different. His first job wasworking at a local restaurant, but the jobwas physically challenging. Instead, Shawnsuggested that Ben work from home andrecord a weekly YouTube show for hispeers. He was responsible for developingthe content, taping and editing.
Ben, from behind a desk like a late nighttalk show host, covered topics on studying,dating and relationships. It was matureadvice from a handsome and charming18-year-old, who told girls they werebeautiful for just being themselves. His twovideo blogs, BreedloveTV and OurAdvice4You,developed a devoted following and startedto generate ad revenue. Ben was clearly anatural in front of the camera, making it theperfect outlet for Ben’s final story.
Even though he appeared confident onscreen, the high school years proved to bechallenging for Ben.“With peer pressure,team sports and the need to be like hisfriends, Ben began to test the limits,” saysDeanne. Seeing his friends develop intomore serious athletes, Ben felt sidelined byhis condition for the first time.“I was neverallowed to play all the sports that my friendsdid,” said Ben in his video.“I really just hopedI could be the same as everyone else.”
Deanne was worried when Ben beganlifting weights. She did not want to tell himhe could not continue so she gently relayedher concerns,“This scares me. Let’s talk toyour doctor at our next appointment and
develop a plan.” Deanne felt this approachworked because,“including Ben in thedecision making process with his doctorsmade him feel more in control of hiscondition.”
Ben was on several different medicationsthroughout his life to treat his arrhythmias.In 2009 Ben was told he needed animplantable device, which was both acardioverter defibrillator (ICD) and apacemaker, to treat his abnormal heartrhythms. Ben was opposed to the deviceuntil a 6’6”, 295-pound football playerwith an ICD counseled Ben and toldhim he didn’t have a choice. After that,Ben was ready and willing.
After the implant, Ben had more energy—enough strength to do a back flip on thewakeboard. However, by 2011 Ben’s heartbegan to deteriorate. Frequently exhaustedand weak, Ben missed weeks of school andsuffered two more cardiac arrests—onein the hospital and a second one at school.“My heart stopped and I wasn’t breathingfor three minutes,” said Ben.With thesesecond and third near-death experiences, heonce again saw the light that called for himin the same way it had when he was four.
With Ben’s heart continuing to fail, theBreedloves began the process of listinghim for a new heart. Unfortunately, Bencollapsed unexpectedly on Christmas daywhile playing outside with his brother.This time he could not be resuscitated.
How could his ICD and pacemaker failto save him, so many cardiomyopathyfamilies ask? According to Deanne, Benwas not experiencing fast, irregularheart rhythms (ventricular tachycardia,ventricular fibrillation) that normally wouldtrigger the ICD to fire, but his bloodpressure was extremely low. Because ofhis dilated and weakened heart muscle,there simply was not enough pressureto pump blood through his body tosustain life.
“The important message here is that anICD is not a cure for HCM. It reduces theincidence of sudden death, and overall itprolongs survival for patients,” saysSteve Colan, M.D., CCF medical advisorand professor of pediatrics at Boston
Ben Breedlove’s Message of Hopecontinued from page 1
Ben Breedlove
FOR FAMILIES
Spring• Summer 2012 Heart toHeart 11
CCF hasawarded itsfirst FamilyAssistanceProgramgrant to aworthy familyin Minnesota.The CCF Family Assistance Program was launched in December2011 at nine medical centers across the U.S. to assist low-incomefamilies with cardiomyopathy-related medical and non-medical needs.The Rippy family from Anoka County, Minn. received financial assistance fromCCF for their rent, utilities and health insurance.The Rippys have two daughters,Madison and Sydney, both of whom have dilated cardiomyopathy. Madison is doingwell after a 2011 transplant, and Sydney recently received a heart transplant at theMayo Clinic.
“When I found out we had been awarded a grant from CCF’s Family AssistanceProgram, I just sat down at the kitchen table and cried,” says Linsey Rippy, momto Madison and Sydney. “My husband hasn’t gotten paid in six months, and I hadto quit my job to stay home with the girls. I just didn’t know what we weregoing to do.”
After hearing stories of cardiomyopathy families struggling financially to providecare to their child, CCF established the program with annual appeal donationsfrom CCF families.The assistance program will reimburse qualified families fortreatment-related costs; rent, mortgage, utilities and displacement fees during achild’s treatment and health insurance premiums, deductibles and co-pays.
“Thank you so much to CCF and to all the wonderful families who have donatedto the fund,“ says Linsey. “It was so frustrating having two very sick kids and gettingbehind in our bills. Now we have a little bit of peace in our lives, knowing ourmedical insurance and other bills are paid for a while.We won’t need help forever,but just for a few months until we can get back on our feet.”
CCF is actively working with other hospital social workers to identify more qualifiedfamilies. For more information, including program guidelines and an applicationform, please contact Chris Colón at [email protected] make a contribution to the Family Assistance Program, contact Sheila Gibbonsat [email protected].
CCF Family Assistance ProgramAwards FIRST GRANT
Children’s Hospital. “ICDs are better thanany previously available therapy; however,patients are still at risk for sudden cardiacdeath and heart failure.”
While not much time has passed sinceBen’s death, Deanne doesn’t dwell onpainful memories or give consideration towhat could have been done differently forBen. Instead she remembers the beautyBen brought to life.“We strongly believewe each have a certain amount of time onthis earth that we are gifted with, whichis beyond any attempts we may make atextending our own life,” says Deanne.“We are willing to accept that Ben wasgiven 18 wonderful, fantastic years, whichwe were blessed to share with him.”
The funny and light-hearted Ben thefamily knew was much more serious andcontemplative in his final message.Unknown to his family and friends, hecreated a new channel to broadcast his twothree-minute videos. It was Ben’s holiday giftfor others to find—a hopeful message thateven in the midst of fear and uncertainty,faith and courage would carry us through.
Deanne continues to share Ben’s messageto live life as fully as possible.“Rememberto live every day as a gift,” says Deanne.“Everyone has challenges, but we all havereal hope and he (Ben) saw that.”
6-year-old Madison and 3-year-old Sydney Rippy
Awards FIRST GRANT
Ben Breedlove: This is my story video
HearttoHeart
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Volume 9, Number 1 • Spring • Summer 2012
CCF Forum GuestQ&A Sessions
• Pediatric Cardiomyopathyand MedicationsElizabeth Blume, M.D.Children’s Hospital Boston
June 4-11
• Pediatric HeartTransplantationAnne Dipchand, M.D., F.R.C.P.C.The Hospital for Sick Children
September 10-17
Local SupportGroups
• North CarolinaDuke University Medical CenterDurham, N.C.
May 24 at 6:30 p.m.
Room 4902, McGovern-DavidsonChildren’s Health Center
CCF offers a variety of ways for members toshare information on pediatric cardiomyopathy and
provide support to one another.
CCF offers a member forum, local support groups and phone sessions. For moreinformation about these services or the scheduled events below, please contactChris Colón at [email protected].
MEMBER SUPPORT SERVICES Now available:CCF 2010-2011CYBERGUESTCOMPILATION
Answers fromthe Experts: CCFCyberguests, acompilation ofQ& A pediatriccardiomyopathyforum sessionsis now available.Topics include
“Helping Your Child Cope withMedical Tests or Hospitalization,”“The Basics on Health Insurance,”“Parenting Teens with Cardio-myopathy or a Heart Transplant”and several others. Compilationsfor Q&A sessions from 2006-2007and 2008-2009 also are availableupon request.
Children’s Cardiomyopathy Foundation
Scan thiscode withyour smartphone toview CCF’swebsite
