Poem: My Son

The following poem was submitted and written by David, self-described "husband to the most beautiful wife and the two most beautiful children (Thomas David, 2, HLHS) and Cadence (HH) a father could ask for." Thank you, David, for sharing your work! David created a support site at www.kentuckianalefthearts.com.

My Son

My Son was born on a warm summer's day
With me pacing the floor in a proud dad's normal way
Not knowing of challenges that were still yet to come
That would change my life in more ways than one

Holding my son for the first time I cried
Thinking of all of the years that his mommy and I tried
Still not knowing how sick and tired he would be
After his heart was wearing out even before week three

We were going home and it was just a day away
When something happened that almost ended our day
The doctor came in and showed us his heart
The news was so shocking it tore my life apart

The shock so horrible and the news so bad
My life was now ending I was wishing that it had
Our journey had begun one we didn't want to start
How a boy as sweet as him could be born with half a heart

It must be a bad dream and it can't possibly be fair
For a moment life stood still as we looked with a blank stare
I was numb all over and my tears dried on my face
Our doctor had informed us to get ready for the race

It would take our emotions from valleys to peaks
One day we are happy to the next no one speaks
We prayed God would show David mercy and grace
As we sat down to ponder with a look of fear on our face

We made it through the Norwood with help from up above
God surrounded us with people that we would grow to love
Those people were doctors and nurses and family and such
That showed our family they cared with the love in their touch

After a month of a struggle for our little guy
We were allowed to take him home with a gleam in his eye
I remember like it was yesterday as I welled up with tears
My eyes kept filling up it seemed like for years

Happiness filled the room as we started to pack
Still not knowing the future or what to expect
I gained my composure and prepared to go home
Not knowing it had changed me and my life yet to come

For you see spreading awareness has become my whole life
Not wanting a parent to have to suffer this strife
We have been through so much I still think we have won
Because everyday I wake up I still have MY SON

A Day to Remember

On this day in 1944, the pioneers in surgical repair of congenital heart defects completed the first successful CHD surgery. So many of us owe our lives, or our childrens' to these two innovators. Steve, of Adventures of a Funky Heart, gives us a brief history lesson today... not just on these two incredible people, but on the unsung hero who REALLY made our, and our children's lives possible in the face of CHDs.

As part of a prolonged weekend of thanks giving, take a moment to send a little thought or prayer of thanks to the hundreds of unsung heroes working to save us.

From Adventures of a Funky Heart...

Vivien Theodore Thomas was born on August 29, 1910 in Lake Providence, Louisiana. After graduating high school in 1929, he planned to attend Tennessee Agricultural and Industrial State Normal School, (now known as Tennessee State University) with hopes of becoming a doctor.

He had been in school two months when the stock market crashed, causing him to lose his part-time carpentry job. Forced to drop out of college, Thomas still found work as a Lab Assistant at Vanderbilt University Medical School, working for surgeon Dr. Alfred Blalock. Although hired to sweep floors and clean out cages, Vivien Thomas impressed Dr. Blalock with his intelligence. Blalock was so impressed that he trained Thomas to be his Surgical Technician.

Thomas began assisting Blalock in the study of shock during surgery. Shock is caused by a sudden drop in blood flow through the body, and can be fatal. Working together, Blalock and Thomas developed ways to prevent shock from occurring during an operation. By World War II most of their theories were in use, saving the lives of countless injured soldiers.

In 1941, Dr. Blalock was hired by Johns Hopkins Hospital in Baltimore, Maryland, to serve as the hospital’s Chief Surgeon and as a Surgical Professor in the Hopkins Medical School. The doctor asked his trusted assistant to go with him, and Thomas agreed. But while Blalock was responsible for training every surgeon in the school, Thomas had to enter the building through the service entrance. He was also listed on the hospital payroll as a handyman.

The two men respected and trusted each other, but were hardly equal. At one time, Blalock was paid ten times more than Thomas. Often the doctor hired Thomas to serve drinks in his home during a social event. And never was Thomas allowed in the Operating Room.

It was at Johns Hopkins that the two men met Dr. Helen Taussig. Taussig had been hired in 1930 to oversee the Cardiac Clinic of the Harriet Lane Home, and quickly grew interested in “Blue-Baby” diseases.

Usually, blood coming into the heart is routed first to the lungs, where it absorbs oxygen. The oxygen rich blood then goes back to the heart, where it is pumped throughout the body. Blue Babies are born with a badly formed heart or blood vessels that cannot provide enough oxygen to the blood. Their skin has a distinctive blueish tinge, especially in the fingertips. At that time Blue Baby diseases were incurable, and almost all of the patients died very young.

Dr. Taussig approached Dr. Blalock with an idea: if a Blue Baby’s heart couldn’t provide oxygen to the blood naturally , then why couldn’t a surgeon re-route the major blood vessels? Taussig’s plan was interesting but extremely dangerous. The operation would have to take place near the heart, and heart surgery was so risky it was almost never recommended. Any accidental damage to the heart would have to be repaired within 4 minutes, or the patient died.

Busy with his teaching duties, Blalock asked Vivien Thomas to work out the details of how such an operation could be done. Thomas began by studying medical textbooks, drawings and diagrams of hearts, and even real hearts taken from dead bodies. Then he operated on dogs, intentionally creating Blue Baby hearts in them. Later he would operate again, repairing the heart and making careful notes of everything he did. It was a slow process, learning exactly what had to be done. Many dogs died, and several of the surgical tools he needed didn’t even exist. Quite often, Thomas would invent them.

X-rays of the patient were another problem. X-ray films provide a good still photograph of the workings of the body. But Taussig preferred to use a fluoroscope. A fluoroscope image is best described as “X-ray TV”– It provided moving images of the interior of the body. If the patient accidentally moved, so did the picture. There was no way to record the fluoroscope image, so the three doctors would have to study their patient’s fluoroscope scans carefully and commit them to memory.

At last they felt they were ready, and Taussig began to search for a proper patient. On November 29, 1944, they operated on a little girl named Eileen. Although fifteen months old, Eileen only weighed nine pounds.

Thomas had planned to be in the observation room, watching the operation. Blalock said no – he felt more comfortable with Thomas close enough to give him advice. In preparation for the operation, Thomas had performed the procedure over 100 times on animals. Blalock had been taught the procedure by Thomas, but had actually done it only once. Breaking all the rules of the time, Thomas entered the operating room and guided Blalock through the operation.

Eileen’s heart never stopped beating and her blood vessels were only as thick as a matchstick. After about 90 minutes, Blalock was finished. Everyone held their breath as he removed the last clamp from a blood vessel. After a long pause, Helen Taussig said “Al, the baby’s lips are a glorious pink color.”

Proven to be a success, Blalock’s team performed nearly 300 operations in less than a year. Surgeons came from around the world to study Blalock’s new surgical procedures, only to learn that Thomas was the expert, not Blalock or Taussig. Still, the operation was known as the “Blalock-Taussig Shunt,” named for the surgeon who performed it and the doctor who suggested it.

Blalock retired in 1964 and died four months later. For six years, Thomas continued to teach but took on no major project – almost as if he were in mourning. But as the 1970’s began, more and more African-Americans were entering the Hopkins Medical School. To them, Vivien Thomas was not just one of their teachers, he became their mentor. And just as he had guided Blalock so many years before, Thomas’ advice and support guided a new generation of doctors through medical school.

Thomas died in 1985, just a few days before his autobiography was published.* Today, Vivien Thomas is almost unknown to the general public. But Dr. Alfred Blalock never forgot him. If someone stood too close to his right shoulder during an operation, Blalock would tell them to back away. “Only Vivien may stand there.”

* Thomas’ autobiography has been reissued with a new title: Partners of the Heart: Vivien Thomas and his work with Alfred Blalock.

Stem Cells May Help Repair Valve Defects!

Bloomberg Science, Nov. 10 -- Cardiologists at the University Hospital of Munich believe that umbilical cord blood, rich in stem cells, may bre the raw material needed to repair the hearts of thousands of children born each year with defective heart valves. The researchers reported the findings at the November 10 annual meeting of the American Heart Association. They believe they are 5 to 7 years away from transplanting new valves created from cord blood with patient's faulty valves.

The Munich researchers are ready to begin trials in lambs, using collected cells (frozen for preservation) to seed into biodegradable polymer scaffolds. Thus far, eight bio-engineered valves have acted much like natural heart valves when tested for blood flow and pressure. The scaffolds will disolve over time, leaving behind a fully formed structure made from the cells, they assert. The next step will be to transplant the heart valves into hearts of young lambs, watching them to see how they grow and function over time. The experiments are hoped to begin next year.

Click here to read more...

No Drop in IQ Seen After Bypass

ScienceDaily, Nov 10, 2008 -- Good news for older children facing cardiopulmonary bypass, or CPB. Scientists at the Cardiac Center of Children's Hospital recently conducted a study of children aged five to 18 who underwent heart surgery while on a heart-lung machine to circulate their blood. The research found that the use of CPB does not cause short-term neurological problems in children and teenagers after surgery for less complex heart defects (such as valves or ASDs/VSDs), according to pediatric researchers. The new finding contrasts favorably with previous studies that showed adverse neurological effects after newborn surgery for more complex heart conditions.

Click here to read more...

We NEED Your Story!!

As a parent of a child with CHD or a CHD survivor, we often can feel lost in a sea of other causes. Now you can take the helm of awareness and steer it toward change, by sharing your story with your congress representatives.

Many of you have expressed a willingness to share your personal stories of battles, both those lost and won, in an effort to raise public awareness of CHD. This is an excellent way to allow the voices of our children, family members and ourselves to be heard!

In effort to provide a united front as the National Congenital Heart Coalition (watch for their new website, to be launched this week!), a Mended Little Hearts volunteer, Amy, has volunteered to create books that will be provided to congress men and women during Lobby Day 2009.

Guidelines for Submission:

1. Content and Accuracy: Emotion is a powerfully persuasive tool. So are numbers when supporting the financial hardship that can result from CHD. Please try to be accurate and describe the details of your story, and try to convey the burden faced by survivors, families and friends. Remember, the purpose is to emotionally engage the reader regarding the impact of CHD, increasing awareness and compassion for the lifelong disease.
   
2. Format: Stories need to be 1-2 pages, in a .doc or .txt format. Please check for
   spelling and grammar. Along with your story, please submit two, high quality photos, one of which is in a medical setting, such as during a hospital stay, doctors office visit or procedure.
3. Disclaimer: Please understand that your story may need to be edited to fit our standardized models and formatting needs!
   

DEADLINE: December 15, 2008

Click here to submit your story.

ACHA's Top 10 for Adults with CHD

Source: EmaxHealth, 11/8/08

Today, most children born with CHD have a good chance of living well into adulthood, thanks to research and innovation. However, the increasing adult CHD population poses a problem - many adult cardiologists are unfamiliar with the resulting heart anatomy and physiology that results from surgical repair. Adults with CHDs are encouraged to see specialists that are familiar and experienced with CHD. Check out Kansas City's Fox Affiliate for a recent health update and interview!

In an effort to assist cardiologists in their every day decisions for adults with CHD, the American College of Cardiology and the American Heart Association have jointly released a comprehensive set of practice guidelines. The guidelines—the first of their kind in the United States—appear in the December 2, 2008, issue of the Journal of the American College of Cardiology (JACC).

ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease include:

1. Given current surgical mortalities of less than 5%, in the next decade almost 1 in 150 young adults will have some form of congenital heart disease (CHD).
2. There should be enhanced education of adult cardiovascular specialists and pediatric cardiologists in the pathophysiology and management of adult CHD (ACHD) patients.
3. Practitioners are provided with logical well-conceived care plans for patients with simple (e.g., isolated small VSD), moderate (e.g., tetralogy of Fallot) and complex (e.g., cyanotic congenital heart defects) lesions.
4. Consultation before pregnancy, including genetic counseling, so that both men and women with ACHD should have a thorough understanding of the risks of transmitting CHD to their offspring.
5. ACHD patients should carry a complete medical “passport,” listing specifics of their past and current medical history and contact information for immediate access to data.
6. A formal transition process should be used to help teenagers and young adults cross the bridge from their pediatric cardiologist to an adult cardiologist.
7. ACHD patients must be informed of their potential risk for infective endocarditis and should be provided with the AHA wallet card with instructions for prophylaxis.
8. Outreach and education programs should be organized to bring patients back into the healthcare system if they are no longer receiving appropriate care and follow-up.
9. Health care should be coordinated by regional ACHD centers of excellence.
10. Every ACHD should have a primary care physician
    

Source: Warnes et al ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease). http://circ.ahajournals.org/cgi/reprint/CIRCULATIONAHA.108.190690

An Insurance Story

Lately we've been receiving quite a few stories from families that wish to share information that may help others. We'll now begin posting them regularly! Meet Lauren, a CHD mom who describes her battle with insurance...

I'd like to share our story. When I got pregnant with our 1st daughter I was covered by my employer's health insurance. When we found out about her heart issues - HRHS, pulmonary atresia, Ebstein's Anomaly - we were told we'd have to deliver in the lower 48 as we live in Alaska and there are NO heart surgeons here who do kids. We chose Seattle.

The Children's Hospital said I should apply for Medicaid; a lot of their kids from AK were on it, they said. I at first thought "no way" because I thought that since I have a job and insurance that I didn't need it, wouldn't qualify. We got on and were lucky we did as her delivery, 1st surgery, and all costs for her 1st year were covered 100% - including her 2nd surgery, travel to Seattle, staying there, as well as all the PT and OT services she needed. When she got to be 1, we heard about a program called TEFRA, which in Alaska also covers kids with complex medical conditions; provided they also meet a certain "level of care" threshold. She got on that and was covered until it came time for our 2nd renewal - before she turned 3. They said she didn't meet the "level of care" requirement. We said "BS" and appealed, got an attorney, the whole 9 yards. We said that she has the complex condition, and she hasn't even had all her surgeries - she still needs 1 more. Plus she takes a Heart med and aspirin, and she still needs therapy services - documented now by the school district, since she qualified for special needs preschool due to her need for therapy. They still denied us - their rationale for deciding she didn't meet the criteria??? I worked and I had to put her at a babysitter's house!!!!

So, now I am lucky to work for a large company, widely recognized as one of the best places in the country to work, so I get good benefits (thank heavens). Before I got hired, I was pregnant again, and in a last ditch effort to provide full insurance for my daughter, I applied for Medicaid again. I got on because I was pregnant. She didn't. There are 2 different income levels they use for qualifying you, and she was evaluated on a lower one because she technically already had coverage (I was a contractor for the Feds and got the barest of bare bones coverage from that). However, when I got my new job, during open enrollment, I saw that a high deductible plan covered everything at 100% after you meet the deductible. I quickly signed up for that for this year because I was pregnant, so I knew we'd meet the deductible. I also knew I would never have to pay a penny of it because Medicaid would pay all costs insurance didn't, and by the time our daughter needed a 3rd surgery, this last March, I thankfully did not have to pay a penny of it - though we did have to pay for airfare, and a token fee at the Ronald McDonald house, as well as food.

Next year, I don't know what I'll do, I just pray she stays healthy and out of the hospital.Thanks for letting me share!

~Laurel Nelson
Anchorage Alaska

Thank you for sharing your experience navigating the insurance maze, Laurel.

Do you have a story you want to share? Tips for CHD survivors or parents? Send them to us at learnaboutchd@gmail.com and we'll be happy to share them here in cyberspace!

Free Webcast on Complications of Adult CHD

Webcast, Patient Power®, to discuss
“Under-recognized Complications of Adult Congenital Heart Disease”
Wednesday, November 5th

6:00 pm – 6:30 pm Pacific
Oregon Health and Science University presents
“Ask the Experts” with Andrew Schorr at www.patientpower.info

Significant amounts of people are born with heart defects, many of them with congenital lesions. Because of new improvements in surgical and medical therapies, increasing numbers of these children are now patients in adulthood. Still, these patients remain vulnerable to dangers and complications of congenital heart disease. Join two Oregon Health and Science University experts, Dr. Craig Broberg and Dr. Joseph Weiss, as they unravel misconceptions and underline important factors for a disease that is often under-recognized. Both experts will help us understand long-term issues that commonly arise, the role of congenital heart disease in pregnancy, and heart rhythm disturbances. Tune in to hear a conversation with highly knowledgeable experts for a closer look into a condition many are living with.

WHO (FEATURED GUESTS):
Craig Broberg, M.D.: Pediatric Cardiologist, Oregon Health and Science University
Joseph Weiss, M.D, Ph.D.: Cardiologist, Oregon Health and Science University
Andrew Schorr: Host and leukemia survivor
WHEN: Wednesday, November 5th, 2008 from 6:00 pm – 6:30 pm Pacific

HOW TO PARTICIPATE: Listen at www.ohsuhealth.com/experts

ABOUT PATIENT POWER: Patient Power debuted in February 2005 and is a weekly show hosted by Andrew Schorr, eleven-year leukemia survivor, patient educator and patient advocate. The show features renowned medical experts on topics that include cancer, pain, diabetes, and heart specialists, as well as experts in clinical trials and top pharmacists. The show serves to bring patients together in a radio and Internet community to help navigate an often inhospitable healthcare system. Patient Power takes questions from callers and Internet listeners on topics such as how to find the right doctor, how to advocate for effectively, when to get a second opinion from a specialist, and how to evaluate one treatment option over another.

Become a CHD Advocate!

When you are a survivor, or a parent of a child that is or was affected by a CHD, you often feel helpless in a sea of insurance, legislation and lack of information. Many many of us never knew that 1 in 120 children born each day are affected with CHD before we received a diagnosis. And almost everyone affected wants to change that.

Many groups across the U.S. are working on providing support and comfort to people affected by a CHD diagnosis. But who is working with our legislators and government agencies to bring about desperately needed change? How can we create a better future for CHD survivors?

The answer? The National Congenital Heart Coalition. Formed just over a year ago, this coalition is the culmination of six major, US national groups' efforts to create a climate of change for our disease. We seek to create better avenues of awareness and advocacy, which in turn will lead to greater focus on research and technological advancements that will save lives through earlier diagnosis, better screening, advancements in quality of life and surgical breakthroughs. Consisting of the Adult Congenital Heart Association (ACHA), the Children's Heart Foundation (CHF), Children's Heart Information Network (CHIN), Mended Little Hearts (MLH), Saving Little Hearts, It's My Heart (IMH) and Little Hearts, this newly founded organization is already making strides in unifying our cause. Current projects include the creation of a unified message, creating a presence in government agencies who monitor CHD and distribute funds for research and, most recently, a unified effort in lobbying our government for a central registry of CHD statistics.

Lobby Day has been a significant effort undertaken by the ACHA over the two years. This year, the entire National Coalition (NCHC) is behind the effort, and YOU are invited to help us make a difference! You are welcome to attend the lobby day, which will be held on February 10, 2009 in Washington DC. Or, you may learn how to participate in your own local area.

Online registration is now open and available at: www.achaheart.org/getinvolved/lobbyday2009.php.

CHD Death Incidence in Infancy

Source: Patient Health Information, 13 October 2008

MedWire News: A significant number of babies are dying soon after birth because of undiagnosed congenital heart disease, a US study suggests.

To examine the extent of the problem, researchers from the University of California used the California death registry for the years 1989−2004 to assess data on 898 infants who died of congenital heart disease between birth and 1 year of age.

They found that, in total, 152 of the babies had a missed diagnosis of congenital heart disease. The average age at death was 14 days and more than half of the babies died at home or in the emergency department, indicating that they had been discharged from the postnatal ward. The team concluded that current screening methods are inadequate.

That's roughly 1 in 8 deaths with a condition previously undiagnosed that died at about 14 days. It is not clear that these children could have been saved if they had been diagnosed on time. Only time, and the creation of an adequate registry for CHD, will provide these answers.

This is one front that all CHD organizations, large and small, should be united on - creating an adequate survey of patients, births and deaths related to CHD, so that we can truly begin to understand this series of anomolies.

The Moral Obligation to Give Back

If you haven't yet subscribed to Adventures of a Funky Heart, (written by an adult CHD survivor and packed with humor and insight), I highly encourage you to peruse the interview he recently conducted with Amy Verstappen, President of the Adult Congenital Heart Association (ACHA) and a survivor of Congenitally Corrected Transposition of the Great Arteries. In her interview with Steve, Amy discusses her "moral obligation" to give back to the CHD community after her personal experiences as an adult survivor. Her personal story is an interesting one, but the action she has taken is nothing short of inspirational.

In the past year, the ACHA has recently joined with several other Congenital Heart Defect organizations to form the National Congenital Heart Coalition. Amy is actively involved in creating new legislation to bring much needed research and attention to CHD issues.

“I am 100% positive that in the long run we will have an integrated, high quality care system for all survivors,” Amy says in the interview. “Our really long term goal is to have a very unified system with research, a registry, a way to quantify outcomes, all the centers talking to each other, sharing information and building knowledge together...”.

Ms. Verstappen is determined to educate parents on the long-term needs of CHD patients. She tells Steve that she “...presented a pilot version of [a new brochure designed by ACHA specifically for parents] to a group of Heart Moms - all leaders of some of the advocacy groups for children - three of them told me that no one had ever told them that their child was going to need this level of care.” Amy goes on to describe some of the questions that parents of children with a heart defect should be asking.

I can tell you, as one of those moms who didn't know or understand the long term implications (I have a child with Transposition of the Greater Arteries who I was told was "fixed!"), that the information provided is valuable and too important not to take a moment to read.

I highly encourage you to take a minute and check out the latest entry at Adventures of a Funky Heart! Not only is it a great personal story of one of the movers and shakers of the CHD community, but it is also a great source of information for parents, teens and adults alike!

Online Discussion on Adult CHD to be held October 2

Next Thursday, October 2, 2008 at 12 noon (EST) Cleveland Clinic will host an online health chat on Adult Congenital Heart Disease.

During this chat, Dr. Richard Krasuski, Director of Adult Congenital Heart Disease Services and a staff cardiologist in the Section of Clinical Cardiology, Department of Cardiovascular Medicine at Cleveland Clinic Heart and Vascular Institute, explores the treatment options available for adults living with a congenital heart disease. More information on this chat can be found at http://www.clevelandclinic.org/health/ChatReg/Krasuski.html.

Dr. Krasuski's specialty interests include adult congenital heart disease including atrial septal defects, patent foramen ovale, ventricular septal defects, coarctation of the aorta, Ebstein anomaly, Eisenmenger syndrome, transposition of the great arteries, Tetralogy of Fallot and patent ductus arteriosus. He also specializes in pulmonary hypertension, mitral valve disease, cardiac catheterization, transesophageal echocardiography and mitral, aortic and pulmonic valvuloplasty.

Too Close To Home...

A recent article in The Sun (13 Sept) asserts that a teenage girl with a congenital heart defect died after telling her gym teacher she did not feel well. She was warming up for hockey practice when she asked to stop because of chest pain but, according to classmates, the gym teacher told her to keep going. The classmates then watched her collapse on the pitch.As a heart parent, I can tell you that this is one of my biggest nightmares. I've been unable to let go of the bond with my daughter into another's care (other than family members and the occassional night out while the neighbors come over). Granted, she's only two at this point, but I worry about the day when she will enter public or private school, out from beneath my watchful eye. I've heard so many stories about children with special directives and orders who are overlooked or ignored by their teachers, counselors, coaches, etc. How will I protect my daughter from such a tragedy, yet encourage her toward independence?

Of course, no one can fully protect their child. I understand this. But one can take steps to help ensure a tragic accident such as this one does not occur. The parents of the girl who passed away at hockey practice had made the school aware of Sasha's condition, so they assumed that this information would be passed on to her teachers. Apparently, it was not.

As parents, it is our responsibility to teach our children how to live with their heart condition, to know their bodies and be able to judge accordingly when "enough is enough." The father of the deceased child said, “Sasha had lived with her heart condition since she was born and she knew her own body. Yet even though the school knew she had this problem, they still didn’t listen to her when she said she was unwell.”

Clearly there is something to be learned from this tragic incident. I know I personally take the following from it:

1. Teach your child about their heart condition. Help them to understand their body.
2. Make sure your child's caregivers are aware of his or her condition, and the potential stresses that could lead to a serious medical crisis.
3. Teach your child that it's OK to take breaks when you need to. Even if a coach, friend or other person is pushing you to go farther, your child is the best judge of his/her body. Don't take unnecessary risks.
4. Talk to gym teachers, coaches and other sport instructors that your child will be working with. Make sure they understand the child's condition and any special considerations and precautions that should be taken.
   

What do you do to protect your child? How have you taught him to read his body's signs? How do you help her to engage in a healthy, active lifestyle without providing undue stress to her already fragile heart?

Real CHD Survivors on Reality TV!

GROWN UP “HEART KID” FEATURED ON SEASON FINALE OF LIFETIME TELEVISION’S HIT SERIES “HOW TO LOOK GOOD NAKED"

PHILADELPHIA – SEPTEMBER 04, 2008 – The Adult Congenital Heart Association (ACHA) announced today that congenital heart defect (CHD) survivor, Heather Magee, will be featured on the season finale of Lifetime Television’s hit series, “How To Look Good Naked,” airing September 9 at 10:00 PM EST.

Magee, a survivor of two childhood surgeries to correct a congenital heart defect that left her with only one working ventricle, the most recent of which was performed in October 2007, confronts her body image on the show in the wake of multiple scars, both physical from the incisions, and the mental impact of coping with a congenital heart defect as an adult.

“For almost twenty years I lived under a false sense of security that my heart condition was a childhood problem that was fixed long ago,” said Magee. “When I discovered four years ago that CHD is a lifelong struggle that requires ongoing care, it threw me for a tailspin. Not only did I suffer physically from my condition and the resulting corrective surgery, but mentally as well. I participated in ‘How To Look Good Naked’ both to raise my self esteem after a traumatic time in my life, and also to raise awareness among the millions of adults living with CHD that they must seek treatment for their condition if they aren’t already doing so.”

Approximately 1.8 million adults in the United States are living with congenital heart defects. Most, like Magee, underwent open-heart surgery as an infant or child, and drop out of special congenital heart care, as they believe themselves to be “fixed.” However, recent evidence shows that heart procedures performed on young children may weaken over time, leading to serious health complications as early as young adulthood.

Magee’s appearance on the season finale of “How To Look Good Naked” will do more than just increase awareness of congenital heart defects among the viewing audience, it will help raise the funds necessary to continue the ACHA’s mission. For the first time in the series’ history, the episode also served as a fundraiser. The show featured Magee and other adults living with CHD in a charity fashion show that raised $10,000, which will be divided between the ACHA and the Ahmanson/UCLA Adult Congenital Heart Disease Center (Ahmanson/UCLA ACHD Center), where Magee was treated for her condition.

More about the show can be found at Lifetime's website.

For More Information:

Anna Espe, Development Associate
Adult Congenital Heart Association
215-849-1260
aespe@achaheart.org

The Tallest Kid in the Room

There's a great new blog out there that I'd love to point our readers to... Adventures of a Funky Heart. The blog is written by Steve, a 41-year old survivor of Tricuspid Atresia. He's allowed us to reprint a portion of a recent entry here. Enjoy, and be sure to check out Adventures of a Funky Heart!

July 28, 2008 by Steve

They always have Highlights for Children. It’s a common staple of any Pediatrician’s office, but it never seemed that they made the magazine available for home delivery. I always wanted a subscription to Highlights, because I enjoyed all the puzzle pages. But that was then. Times have changed, and now I’d rather hae a subscription to a good baseball magazine. Most children aren’t that interested in baseball, so I need to bring my own.

I walk into the doctor’s office and I can feel the eyes boring into my back. I can’t see them, but I know that everyone is looking at me. When someone my age comes into a pediatrician’s office without a child, everyone stares. What’s he doing here? If they are wondering now, just wait until the nurse checks me in and tells me to have a seat.

Usually all of the adult chairs are occupied. It’s impolite to stand in the waiting room – there’s no rule against it, but I’ve never seen it done unless every seat is taken – so I sit in one of the children’s chairs. That’s an adventure unto itself. You sit down as you normally do, but you just keep going down until your bottom hits the chair with a bone jarring THUMP and your knees are level with your eyes. Now I look stupid, so I stretch out my legs. Not only am I blocking the walkway, but now I really look stupid, so I pull my legs back in. Hopefully this won’t be as bad as the time that I had pneumonia: my doctor admitted me to the hospital, and after a 4 hour wait in the ER I learned the only available bed was on the Children’s wing. It was a loooooooooong way from my rear end to the child sized toilet, and I was too far down to stand up! I literally had to roll off the seat and then get to my feet. But there was no room in the inn, you take what you can get, and beggars can’t be choosers.

Until I was 30 years old I saw my local pediatrician for non-emergencies. It works the same way for kids with heart problems. “Adult” Cardiologists can’t deal with us – they are trained to deal with heart attacks, clogged arteries, and all the problems that your heart develops as you age. They usually don’t have experience dealing with Congenital Heart Defects (CHD). A friend of mine – also a Cardiologist – once said that if you chose to specialize in Adult Cardiology, you received about two hours of training in CHDs. Basically just enough to know that they exist. So no matter our age, Cardiac Kids are still patients of a children’s doctor.
But now there’s a new specialty, known as an Adult Congenital Heart Defect doctor. Adult Congenital Defect care is not taught at any medical school (yet); many of the ACHD docs are really Pediatric Cardiologists. Since their patients stay with them practically all their life, these “children’s doctors” found themselves dealing with questions about work, pregnancy, dating, insurance… questions that Adult Cardiologists usually have to answer. And so the field of ACHD Cardiology was born.

You won’t find one on every corner, but ACHD care can be found. Usually, you’ll need to go to a major medical center. I live in rural South Carolina; the ACHD centers in the South include Duke University, Emory University, and the University of Alabama at Birmingham (UAB). Other major centers include The Mayo Clinic, Stanford, the Philadelphia Adult Congenital Heart Center and Children’s Hospital of Boston. There are other clinics, of course, but those are the ones that spring immediately to mind. And now, the waiting rooms have people who are more in my own age range.

I miss the visits to the Pediatric Cardiologist, though. While I’m sitting there trying to get comfortable, absorbing all the stares, the kids don’t pay a bit of attention to me. Kids are kids, they are usually congregated in the play area, getting along famously and having a great time. For once, they aren’t being left behind on the playground – everyone is moving at about the same pace.

The parents are the ones sitting there with the worried look on their face, with good reason. I always hope that one of the parents will speak to me, perhaps ask if my child is sick. If they do, I’ll tell them that no, I’m the one with the heart defect. My parents were in the same boat you were. I’m 41 now, and still going strong. And hopefully someone will come to believe that with good medical care and a little good fortune, their child will do well.

Research Yeilds Answers on Aortic Arch Defect Causes

By The Pittsburgh Tribune-Review
Tuesday, July 8, 2008

Research led by University of Pittsburgh scientists is the first to describe how two common congenital circulatory problems form, the university announced today.

The team found that a gene called unc45a plays a critical and previously unknown role in the formation of aortic arch vessels. The vessels contribute to several of the body's major arteries and often develop improperly, causing a wide range of vascular defects.

The team also found that arteriovenous malformations, or AVMs, happen when an artery fuses with a vein, diverting blood flow, and result from genetic and physiological factors. Previously doctors had believed its origins were solely genetic.

The research is published in the journal Developmental Biology. Scientists at Georgetown University Medical Center and the National Institutes of Health also participated.

First Hearts United Conference held for HLHS families

PRLog (Press Release) Bloomington, MN, USA. Jun 23, 2008 -- The non-profit organization Hypoplastic Right Hearts held its first-ever medical and educational conference, Hearts United 2008, in Bloomington, MN June 18-21, 2008. Families with children born with hypoplastic right heart syndrome (HRHS) from around the US and Australia gathered to meet in person for the first time, and some families had never met another child in person with the same diagnosis.

The members of Hypoplastic Right Hearts planned and fundraised for almost 3 years to make this special event happen.

Five years ago when Amanda Adams of Colorado had a baby boy born with a complex, life-threatening congenital heart disease (CHD) where the right ventricle of the heart does not develop properly, she felt all alone as her newborn son faced multiple open heart surgeries to have a chance at survival. Consequently, Adams founded and became president of the online non-profit group, Hypoplastic Right Hearts (www.hypoplasticrighthearts.org). Hypoplastic Right Hearts provides emotional support for parents, primary caregivers, survivors and their spouses, and bereaved parents of children with CHDs that result in a hypoplastic right ventricle. What started with a handful of “heart moms” as a Yahoo! Group in 2003 has now grown into a worldwide membership of over 450 families.

For more information about Hypoplastic Right Hearts group and Hypoplastic Right Heart Syndrome: http://www.hypoplasticrighthearts.org

Long-term Care of CHDs

As a parent of a child with Transposition of the Greater Arteries (TGA), I will never forget the moment when the surgeon sat down with me after her open-heart surgery and told me "She's fixed." When I asked what I could expect from then on out he told me "She'll be able to live a normal, active life" and "she'd only need a check-up once a year or so." What he didn't tell me is that she was NOT fixed. She will always need to be watched closely. It is likely that she will face complications down the road.

Sure, it's every pediatric cardiac surgeon's dream to tell a parent that a child is "fixed." It doesn't happen often. Some of the more severe defects require many surgeries, none of which are a "fix," but are aimed at extending the lifespan until a fix or alternative treatment can be found. But is it really fair for a surgeon to sugar-coat the truth? No, it isn't.

That's why I was upset when I found out that my daughter's defect is considered severe, requiring life-long care. I had entered into a world where TGA seemed like a minor defect. The truth was like a slap in the face that woke me up from my complacency.

The truth about Congenital Heart Defects is that many DO require life-long care and follow up - not with an adult cardiologist (that is often not specialized in CHD-care), but with a specialist in Adult Congenital Heart Defects. The Adult Congenital Heart Association is working to spread the message, as are many CHD specialists across the world.

For an example, check out today's (17 June) news article from KSPR News in Springfield, Missouri:

Heart Defects need medical attention through adulthood
By KSPR News (visit the link for video footage)

Fifty years ago babies born with congenital heart defects weren't expected to live into adulthood. But thanks to advances in medicine, most babies with heart defects now live long, full lives.

"What it's called is tetralogy of fallot. It's one of the most common occurring complex defects."

Kyra's heart had four abnormalities. The pulmonic valve was narrowed, and there was a hole in the septum, or wall between the right and left ventricles. Also, a main artery was in the wrong place. These issues caused her right ventricle to get thick because of all the extra pumping it had to do. At age 4, Kyra had surgery. She was considered cured.

"Then I started developing symptoms. Shortness of breath, electrical conduction problems, arrhythmias."

It turns out she needed another valve replacement. The repaired valve started to leak. Dr. Keith Oken says Kyra was a victim of a common misconception.

"Which is that she was cured with the surgery she had. That is rarely the case."

Kyra needs continuous, life-time care. But there are not many cardiologists trained to manage cases like hers. That's because until recently most people with congenital heart defects did not survive into adulthood. So to make sure Kyra stays healthy, she sees her regular doctor and experts at mayo clinic. Plus she's doing whatever she can to keep her heart strong...

Doctor Oken says people with adult congenital heart defects need to continue seeing their doctors... And to see a heart defect specialist.

Stereoscopic Glasses May Aid Beating-Heart Surgery

Source: iTNews, 10 June 2008

In a recent study, the three-dimensional glasses have been used in a successful operation on pigs with a common form of congenital heart disease. The newly-tested stereoscopic glasses enable depth perception by splitting computer images in two, and cocking them at slightly different angles to provide surgeons with ultrasound images of the beating heart as a hologram.

Researchers expect that clinical trials of beating-heart surgery with the patching system could begin in children with ASDs this year.

Social Security Benefits for Adults with CHD

Source: Cavey|Barrott Attorneys at Law Blog

Social Security Disability Benefits are paid for congenital heart disease. The Social Security Administration uses Listing 4.06 to evaluate symptomatic congenital heart disease. You show the results of specific medical testing and meet very stringent medical findings to qualify for listing.

More often than not, Social Security disability benefits for congenital heart disease will be awarded based on vocational criteria.

Growing up with CHD, Part 3

Growing up with CHD: A Mother-Daughter chat


By Sarah Clark and Connie Walker


Hey everyone! I apologize for the delay on this post. this week has been a bit crazy in the Clark and Walker households, with me and my husband closing on our first house next friday and my folks packing up for a family visit in Virginia. Thanks again for your patience! After the draining experience that writing (and probably reading!) the last week's column turned out to be, we're going to dial things back a notch. Mom and I were talking on the phone the night before my second column posted, and liked my idea of co-writing the final article in this series. In fact, we're going to change things up a bit, and this article will be written as a interview with me asking and Mom answering questions about how my CHD impacted our family, what we're really proud of, and what, if we had it to again, we might do differently or wish someone had told us.

Also, what's next for my involvement with the CHD blog? Well, As you see, Kim has bestowed on me the honor of co-authoring this blog, and while she's certainly more of an expert on raising a kid with CHD, I'm looking forward to adding my two cents from the other side of the fence, so to speak. After my life settles back down from our move and a few work commitments I'd kind of like to put my librarian hat on for an article or two and share some tips on doing medical research. When I was young the issue for average folks was finding any information at all. Now thanks to the Internet, we're all drowning in data on any medical subject you can think of, of quality varying wildly from expert to quackery. I'll help you figure out where to search, what terms to use, how to effectively use your local public and university libraries, and how to evaluate what you find and discuss it intelligently and assertively with your kid's medical team.

Last, I want to share my contact info and mention a resource I've found useful. As I mentioned, I didn't really have any CHD role models as I grew up, came to terms with some very strange and heavy stuff, and became the productive and upstanding grownup (?) that I am today. Again thanks to the Internet, that no longer needs to be the case no matter where you live. While I spend far more time on Lifehacker, LOLcats and Writing-oriented blogs and forums than in the heart defect corner of the net, I'm a proud and slightly goofy member of ACHA-this association lobbies for the needs and interests of adults with congenital heart defects, sponsors research and education in ACHD issues, hosts a bi-annual convention for adults with CHDs, family, friends, and physicians, and provides a message board where teens and adults with weird plumbing can share their victories, support each other through tough times, or (mostly) be silly! You can drop me a private message there (I'm on as OKLibrarian), or simply zap me an email at oklibrarianATgmail.com. If you're looking for the parental perspective, you can reach my mom and dad at connie52ATcox.net and cwalker71ATcox.net respectively. (Thanks again for volunteering to be spammed, Mom & Dad!)

Now for part 3:

Sarah: I know I've said this before, but why not let the whole internet know? I got very lucky to have you and Dad as parents. As I've mentioned, you helped me and Daniel survive some very...interesting times during our childhood, and we've somehow managed to both become mature, upstanding adults with spouses, mortgages and careers. What do you think were the most important things you and/or dad did that helped us get to adulthood with good mental and physical health?

Connie: We loved you. That simple--and that complicated. I'd like to believe it would have been the same regardless of health concerns or birth order, but who can say for sure. Life happens as it happens.

Whatever was happening in our lives, it was important to me that home meant safety--a retreat and refuge. We valued you two for who you were--not who we wanted you to be. We paid close attention to your interests and talents, and did our best to nurture them in every way we could. We also did our best to turn every perceived "failure" into a learning experience. By the time you were born, we had learned that resilience was the most important tool for a successful life. We made it our highest responsibility to make sure both of you had that tool!

Sarah: On the flip side--if you had it to do all over again, is there anything you would have done differently, or wished you'd known about in advance about raising a kid with CHD, or just parenting in general? (let's take not bringing me to see ET as read. *smirk*)

Mom: You've been very kind not to mention my worry-wart nature. It's my lifelong "defect" (can you tell I take strong exception to that word and what it implies...). When that worrying nature was applied to my deepest love--my family--I probably added unnecessary burdens to your lives. I wish I could have been braver. But as I've told you before, it could have been much worse! Without sharing my fears with you & Daniel, and forcing you both to prove to me you would be smart and safe, I would probably just have locked you in the house forever. It is a generalized fear...applied in its turn to my beautiful, first born, "blue" baby, crossing the street alone, riding the bike to a friend's house, dating, driving, calls from a deserted train station in Rome at 2 a.m. on Christmas Eve...you know, silly stuff like that. (And on the topic of ET, if it's still traumatic, I'll be happy to make you another ET costume and let you parade up and down the neighborhood, like we did all those years ago!)

(Note: The author would like to commend her Mom for her very savvy desensitization of her daughter's ET-Phobia, and also remind her that she had specifically requested a phone call on Christmas Eve, and it was the only phone booth available by the time we left midnight mass @ the Vatican! The only people around anyway were a policeman, my backpacking buddies and a very polite transvestite panhandler...)

Sarah: My younger brother Daniel is the coolest guy on the planet, and hasn't gotten nearly enough attention in the first two parts of our story. Among other gifts, he has more musical talent in his little toe than I will ever hope to possess, and plays keyboards part-time with local jazz and funk bands in addition to his career as an Industrial Engineer. However, he and I are very different people-he is a logical, laid-back guy, quiet where I am outgoing, mellow where I am melodramatic. How did you handle raising two children, both very gifted in their own ways but with two very different personalities and needs, and how did you try to make sure that your "normal" kid didn't feel like he was shortchanged?

Mom: See answer to question # 1! Truthfully, he probably did feel shortchanged from time to time. Probably you did too. Sibling rivalry is what it is. But disrespect, in action or word, was not allowed between the two of you (at least not when I knew about it). Do you remember when one of you would say, "that's not fair...you love ____ more than you love me!" I always answered "yep, you must be right". That always shut off the pity party, because you both knew that was silly talk. We valued you both, and expected you to value each other. That mutual love, respect, and support of each other is still my strongest desire for the two of you. Different is good!

Sarah: I often tell friends that I had a idyllic 80's sitcom suburban childhood "with a few twists"- Often I don't think they believe me until they've seen enough of us all interacting to realize it isn't some weird passive-aggressive act! How did you and Dad find a balance between acknowledging the medical challenges I (and later Dad) had to deal with growing up with giving me and Daniel as "normal" and stable a childhood as possible? Also, do you think everything we went through made you and dad a stronger couple, and us a stronger family?

Mom: Well, your dad and I are at least as different from each other as you and Daniel are! I think it is extremely important to have both parents involved in a child's life. I know it isn't always possible. In fact, for a good portion of your childhood, dad was too sick to be as actively involved as he had dreamed of being. It took all of his energy just to keep on working, so we would be provided for. That makes us sad when we look back on the childrearing years. But those kinds of regrets don't really serve any purpose except to keep people from making the most of the circumstances they find themselves in. We just did the best we could with each day that was given to us.

To answer your question, reference my overprotective, worrying nature. One day, after listening to my many plans for keeping you safe from any danger, harm, hurt or substandard educational experiences, your dad, in exasperation, said, "Connie, we don't live in Mr. Rogers' Neighborhood!" He had hit the nail on the head. That was exactly where I WANTED to live. And I told him so. But it also made me laugh at myself. I knew you (and Daniel) couldn't live there forever, even if we could afford a house in the Neighborhood of Make-Believe. And so it went, with every "crisis"--potential, undeniable, or conjured up--we both spoke up about our fears, opinions and reasons, communicated (sometimes loudly) and came up with a solution we could both live with. The one thing we always knew was that we both loved both of you, and each other, very much.

Sarah: I'm not a parent yet- some days I barely have it together enough to take care of our cat! :-) Because of that, I'm guessing that I'm missing some stuff you think is important for other parents to keep in mind. Is there anything you'd like to say that I didn't ask you about?

Mom: Becoming active in a support group was probably the one thing we did that saved our marriage, family, and perhaps even your life. Lifeline, the support group at Children's Hospital in Oklahoma City, was the saving grace when you were first born. I dealt with guilt (what had I done wrong), anger (what kind of God would let these things happen to innocent children), fear (if she doesn't live, I can not bear to live either), and overwhelming love (surely everyone knows this is the most beautiful, marvelous child who was ever born). I worried about every sneeze, about helping you gain enough weight for the surgery, about how you were progressing compared to others your age. Some of that was heart, and some of that was first baby. The worries were real. But the focus of Lifeline (named by a family who called their child's scar her lifeline) was hope, support, education and friendship. I never thought of you, or any of those other wonderful children we grew to love, as defective in any way. You all were perfectly, wonderfully made.

Your correction eventually had an excellent result, but there were problems from the post surgery complications that we had to deal with. It is possible we might have defined you in terms of those problems, except for what I call "God's beautiful gift wrapped in a very ugly package". You had a respiratory arrest after the correction at 18 months. It was my blinding light on the Damascus road. All my anger at God about why--why you--why precious little children--why child abuse--all those hard things I saw at Children's Hospital--fell away when I saw them holding the paddles over your tiny, wired body. I knew in that instant that you never really belonged to me. You belonged to God. Regardless of my plans, actions or thoughts, I had no power of life or death. You were in my heart for as long as I had you, and for as long as I lived. My precious privilege, but not my possession. My child, but also God's child. And so, that very minute, I repented of that anger. I gave you back to God, and starting praying for wisdom and courage to raise you and support you in the life YOU were meant to lead. By the time Daniel came along, I knew what to do. I gave him back the day he was born! And I prayed for wisdom and courage to raise him and support him in the life he was meant to lead.

That is my advice to parents, CHD or any other. The only power you have is the power of love. See your children as what they truly are--God's children. Pray for wisdom and courage to raise them and support them in the lives they are meant to lead. It is that simple--and that complicated.

~~~~~~~~~~~~

Thanks Mom, and I don't think I can really improve on her ending.

So, Is there a moral to my mini-autobiography? I don't know. You and your kids will have to visit and revisit this issue occasionally through their lives, no matter how "perfect" their outcome is. Aside from less than ideal physical stamina, and a few very minor hearing and motor control glitches created by my brain damage, I'm normal in pretty much every way you could define. And yet, every so often I've had to mull over these issues in my life, come to understand them in a deeper, more mature way, and make peace with them for another few years. It last happened around the time Kevin and I got married, this time it seems to be related to turning 30 and buying our first house, and it'll probably happen when/if we have kids and again when I retire. I suspect it's the same for anybody who's had traumatic experiences in their lives, i.e. 100% of the human population.

I started writing these posts for those of you, like Kim, whose kids have passed the crisis, and who are wondering "what now?" about things like self image and dating and all the soft psychological stuff that you can't really ask your cardiologist about. But I have to be honest and say that I was helped at least as much as anyone here by telling my story. Without realizing I was doing it, I gave myself permission to let go of a lot of my lingering guilt about being born as I was, yanking out that stupid vent tube when I was 18 months old, panicking that night when I was standing by the telephone, and all the other big regrets of my early life. I can't speak for all people with CHD any more than I can speak for all redheaded short chubby Librarians who watch too much Food Network. But in my case, having parents who encouraged us to share our feelings even when they were ugly or scary, who taught us resilience, and who encouraged us to achieve as much as our talents allowed helped me and my brother become strong, successful adults who work every day in our own ways to make the world better. And that, so I'm told, is the goal of every parent. I hope that hearing my story and Mom's will help in some way as you parent your kids. If you'd like to know more, we're no more than an email or comment away.

Thanks for reading,
Sarah

US Cardiac Specialists to Assist in China

Team Of Top US Cardiac Specialists To Perform Critical Operations On Children In China
Medical News Today

16 May 2008. Heart disease is the world's most common birth defect, affecting one in every 100 children. Without treatment, one third of these children die before their first birthday. In developed countries, children are typically diagnosed and treated at birth. Children's HeartLink, an international medical nonprofit, is reaching out and helping build sustainable pediatric cardiac programs in the underserved regions of the world, to help more children have the same opportunity. May 17 - 24, Children's HeartLink will be sending a team of volunteers from New York hospitals on a cardiac medical mission to Changchun, China, to provide advanced training and participate in lifesaving operations on needy children.

Last September at the Clinton Global Initiative in New York, the Bracco/Children's HeartLink Initiative was recognized as it announced the commitment's estimated total value of $1,000,000. The partnership will provide life-saving training, equipment and direct patient funding to Jilin Heart Hospital, in Changchun, Jilin Province, China -- reaching more than 12,000 children in the next 3 years.

The project will involve the training of medical personnel at the Jilin Hospital in China. The Bracco/Children's HeartLink Initiative will reach an estimated 4,000 children a year with emphasis on improving the quality of care. "In China, congenital heart disease is the primary cause of death of infants. This Initiative has the ability to impact thousands of lives" said Elizabeth Bickel, President of Children's HeartLink.

More info...

Those of us in the US are often so focused on how under-served our children are as well as the lack of awareness that it's often easy to forget how very lucky we are to have the facilities and care available, even if it is at a significant, life-changing cost... many in other countries do not. We're looking forward to seeing the results of this significant effort undertaken by Children's HeartLink!

$1 Million Gift to Support Earlier CHD Detection

Washington, DC (PRWEB ) May 15, 2008 -- The Elsie & Marvin Dekelboum Family Foundation has announced a gift of $1 million to Children’s National Medical Center. The gift will allow the Children’s National Heart Institute to launch a Congenital Heart Defect Screening Study. Children’s National will name a three-bed bay in the new Cardiac Intensive Care Unit in honor of the Elsie & Marvin Dekelboum Family Foundation.

The heart defect screening study will focus on the effectiveness of an inexpensive device called a pulse oxymeter that could help identify more than 80 percent of life-threatening congenital heart defects. If initial findings are validated, the Dekelboum gift will enable Children’s National to promote its use in hospitals across the country and around the world – improving early detection and health outcomes for children everywhere.

An Award

A very special thanks to Emily, author of Lovely and Amazing and Gabriel's Heart for presenting this site with the Arte y Pico award for its creativity, interesting design and content. Check out my personal blog if you're interested in seeing who I'm recommending for the Arte y Pico award, as well as how to participate.

Thanks to Emily for her support, great blogging and wit! It's amazing who you meet in cyberspace...

Growing up with CHD: Part 2

Part 2: And now the Bad (or at least challenging) Stuff

By Sarah Clark

First, my apologies for the length—as somebody once wrote, if I'd had more time to edit I would have made this piece shorter. Second, you're going to get tired of hearing this, but growing up with CHD was a relative non-issue, especially when I compare myself to people who suffered various types of abuse or neglect. However, that's not to say CHD wasn't an issue at all.

The first 10 years of my life were hard. About 3 years after my medical drama, my dad started having seizures, and it took several brain surgeries, countless drugs, and ten years of searching to find the cause and get them under control. (And he worked full time during all that!) So much happened to my family in my early life that it's hard to say what caused a given problem, and what just made it worse. However, the ones I'm going to focus on the issues that were there before my Dad got sick. I'm also glazing over some of the obvious fears you may have for your kids like body image and gym class traumas, because their effects aren't that much different or worse than any other kid's psychic bumps and bruises, and are treated in the same way. I'm a deep thinker, so I'd rather focus on the Deep stuff I faced, and that your kids might face as well.

Guilt:

The summer after my 3rd grade year, Mom and Dad went to UCLA for his big brain surgery. We shuttled between several relatives and family friends back in Oklahoma, who each gave us varying amounts of attention and affection. I could probably write a book about the experiences of that summer, but for the purposes of this article, I'm going to stick to the "Blacks". The Blacks were one of a few families my folks had kept in touch with from the CHD days. Their oldest, "Amy", had been a couple years older than me, and hers was the first funeral I attended (about 5 years prior to the summer we spent with the Blacks). She looked like something from a fairy tale with her blue eyes and blond curls. We'd played together some, but really all I remembered was that she was pale and kind of bossy. The Blacks had two younger kids whose ages roughly corresponded to me and my little brother Daniel, so we all got along well, and spent the several weeks we stayed there romping in the backyard and doing Kid Stuff. The only weird thing was the staircase.

The wall alongside the stairs to the second floor was a shrine to Amy. Every morning when I ran down to breakfast, I was torn between averting my eyes from and gazing in fascination at the dozens of photos, certificates, and plaques that lined the walls. Somehow I felt ashamed looking at them, as if I had no right to feel a slight pang of envy for the cute girl who'd gotten to be in front of so many TV cameras and meet so many interesting people in her short life. One afternoon I came back to the house when I got pooped from too much racing on my bike. I came in the back door and grabbed the first book I came across (I left books in my wake like Hansel and Gretel left breadcrumbs), and flopped down in Mr. Black's squashy armchair to read for a bit. I heard Mrs. Black's voice from the kitchen, and realized she was on the phone. Being 9 and nosy, I did the best I could to listen in. I can only assume she didn't hear me enter.

"She'd be thirteen soon…I know God wanted her because she was so special. She's probably the prettiest angel in His garden…," Mrs. Black continued, sharing her dreams for her late daughter to the person on the other end of the line. I sat in the chair, unseen, wanting to throw up. Maybe it was my carefully suppressed fear that I'd never see Dad again, or simply the fact that I was getting older, but for the first time in my life, her overheard words caused the full implications of the eternal question "Why?" to hit me like a ton of bricks. My mind whirled with rage at God, confusion at Mrs. Black's comments, and a strange dark tug at my soul that I eventually recognized as guilt. Did this mean that I was more special than Amy—which made no sense—she was angelic (when grownups were looking) even when she was alive. Or did it mean that I wasn't special enough? Had I done something bad to be born sick, or worse--was my survival some sort of bargain that made Dad sick? Had I made Amy die by praying to God to get back at her for pulling my hair? Her defect hadn't been that much worse than mine--why save a nearsighted geeky clutz with mouse-brown hair and no social skills? Why me and not Amy?

Nearly 25 years later I still have no clue on that one. If somebody here figures that one out, please let me know. It's certainly one of the strongest arguments I've come across for the non-existence of God.

Anxiety:

I've had panic attacks, generalized anxiety, and phobias of various types and degrees pretty much since the day I woke up from my mustard procedure and the ensuing complications. Call it PTSD, brain damage, damaged seratonin receptors from spending so much time on the heart-lung machine--ultimately the cause doesn't matter. Store closings, unfamiliar doctors, elevators, Bs on report cards, any of those things could and did send me into panic attacks at various points in my life. Think twice before you put ET in the DVD player for the first time—I was 4 when I saw that in theaters and I went into a full-tilt inconsolable shrieking meltdown when ET "died". In fact, the only thing I seriously regret in my life occurred because at a moment when someone I loved needed my help desperately, I had a panic attack.

It was a week or two before Christmas. Mom was out for a "meeting with Santa's elves", and Dad was reading us a bedtime story in my brother's bedroom. He finished the story, closed the book, and stood up, preparing to tell me to brush my teeth and go to my bedroom. Then his voice trailed off, and he got a real funny look in his eyes, like he was staring 100 miles into the distance. Then he collapsed into convulsions, and knocked himself unconscious on Daniel's toy piano. Daniel (who was no more than a year old) started screaming, and I swung into action. So what if I was 5? I knew what to do, 799-HELP, I had the phone in my hand, I started dialing—and I froze.

All of a sudden I couldn't breathe, my heart was pounding a million miles a minute, and I started thinking a million different things—what if Dad was dead, and I was supposed to be doing CPR? Would the paramedics be mad if I called and he was dead and I'd wasted their time? What if dad woke up, and then the paramedics would really be mad because it wasn't a real emergency? Would they say "aw, you're just a kid" and hang up? No matter how I tried, I couldn't dial that phone. I hung up and crawled into bed weeping until Dad came to from his first seizure. Dad, of course, long ago told me there was nothing to forgive. I hope one day I can figure out how to be that generous to my younger self.

Since that night 25 years ago when I learned what my panic could make me do (or not do), I've fought a ruthless pitched battle against my terror in all its forms. It's been a foe more formidable than my circulation, by far. It's not just the sweaty palms and the 3 AM catastrophizing over whether I CC'd the right people on an email or if I paid the light bill. The person I loved most in the world needed me, and I failed him. I've come to terms with that (at least intellectually), but it will always gnaw at me, and give my anxiety the most potent ammunition possible when I try to fight it down. I have beaten it down to a dull murmur, but I will always have to be on the defensive--it often sneaks up when I least expect it.

Perfectionism:

Guilt + Anxiety=Perfectionism. Good enough is never good enough for me—I had a serious depression in my early teens and one of the greatest things that fed it was that I simply lacked the energy or motivation to do homework because I was brooding over my "deformed" body, my sick father, or the heavy existential questions mentioned above-- or hiding from that brooding via compulsive reading. That of course lowered my grades, made me feel worse about myself, and the cycle continued down for several years before I pulled myself up through therapy and sheer force of will. The perfectionism remains though—it took me 24 hours after the greatest physical success of my life, climbing the first third of the Great Wall of China at Badaling, to stop berating myself for not training hard enough to conquer the whole thing and to take pride in what I *had* accomplished. I've mellowed a lot since my "emo" teen years and become more balanced as I've matured. I've learned to use that impulse for good--it's certainly helped me out in my career.

The Kid Thing:

"Sarah, It would be a very good idea if you decided not to have children. Pregnancy could be very dangerous for you."

That was NOT the reaction I'd expected when I told Dr. Razook about my first serious boyfriend! I'd expected him to grin, remind me he'd told me so when I'd worried over my scars making me unattractive in previous checkups, and generally play the proud if protective surrogate dad he'd been through my childhood. Instead he asked to talk to me without mom in the room, asked a few delicate questions about what "physical contact" we'd done ("Peter" was president of the Bible Club and Math team and I was barely 16—we were both too nerdy and too scared of eternal retribution to try very much), and then Dr. Razook hit me with that bombshell. Peter and I parted the following year, but I will always remember how he came right over that night when I called, and held me as I cried, telling me that he still thought I was just as beautiful as before. Peter had his faults, but I've held every man I've dated to that standard when I've told them my medical history and its implications. I've been married for 6 years to a man who passed that test with flying colors.

Everyone's situation is different. People with TGA can and do decide to have children, and 15 years on I realize Dr. Razook's blanket disapproval was probably mostly his extra-cautious nature, and partially his impulse to slow down a teenager who was in too big a rush to grow up. However, I've read the studies, talked to people who've had both good and bad outcomes, and I came to the same decision. I feel it would be unethical to knowingly put a child at increased genetic risk of a CHD, and considering that a pregnancy might well complicate my uncomplicated outcome, I will NOT subject my child to growing up with a chronically ill parent if at all possible. Your kids' mileage may vary. Remember that "anxiety" stuff above? I'm a devout believer in Murphy's law.

Being a "miracle"

I'm sure you say it all the time—you've probably said it to your kids, or your friends, or had others say it to you. "You're a miracle". "Her recovery was miraculous." "God blessed us with a miracle". In the sense that at least one event happened to me that could not then be fully explained by medical science, I am a "miracle". I learned the story of my early life, surgery, and (at least metaphorical) rebirth as a wee child, right alongside Cinderella, Snow White, and Sleeping Beauty. My defect was never something to be ashamed of, and my parents told me to wear my scars proudly. From early on I knew I was destined for Great Things—God had saved my life, had He not? (I was still a little young yet to ponder either the "Amy Counter-argument", or wonder Who had been in charge of creating the situation where my life needed saving in the first place)

In addition to having a rather more dramatic origin story than most, I was intelligent. Scary, precocious, read off the menu at the restaurant when I was 2 intelligent. (apparently when the waitress complimented me on my literacy, my granny whipped up my dress to show her my scars, to my parents' horror…). Between those two things, every adult in my life was CONVINCED that I was going to do something amazing with my life—and from my earliest days through 3^rd grade I complied. When maturity, hormones, and the events of the first decade of my life finally slammed into me as mentioned above, not only did I feel depressed for those reasons, my despair deepened because I wasn't living up to what I was Supposed To Do with my life, the mysterious, still-unknown reason I had been saved and made a Miracle.

When I clawed my way out of my funk in my mid-teens, I set myself to figure out what Great Work I had to accomplish to pay back the life debt of my miracle. I put myself under a lot of pressure and agony, and it was definitely a big part of the perfectionism. In recent years I finally got mature enough to throw up my hands and essentially say "God, if you exist and I have an outstanding bar tab, you're going to have to send me the bill via some sort of signal I'll understand. Otherwise I'm just going to muddle through the best I can, and try my best not to feel guilty over that." I've yet to get a bill, so I assume I'm doing fine.

As for the "miracle" thing? As a kid, I wore it with uncomfortable pride, like an Easter dress that was too itchy with starch and made me want to change back into anonymous jeans and a t-shirt. As a teen and young adult I hid from the descriptor or rejected it outright. Now? I don't even know if I still believe I'm a miracle (sorry mom), much less what I think about what that implies. I really am uncomfortable with and to an extent resent all the baggage that has come with being slapped with that 7-letter albatross. Then again, one doesn't get to pick and choose the good and bad in life. If not for the "Miracle" label…Would I be this determined, or this intelligent? Would I be wasting away in a job I hated instead of doing something I love? Would I be living such an (over)examined life?

The point to all of this:

I hope I haven't scared you too much, or convinced you that your children are doomed to a life of brooding and anxiety. Remember Part 1: I am so strong, and lead such a wonderful life in large part BECAUSE I faced these demons at an early age and came out whole on the other side. I don't mean to sound like a Pollyanna, and it is very likely that your children will need your help and possibly professional help in answering the very adult questions and fears that surround their very existence. Fortunately I had that. My mom practiced desensitization therapy long before it was in the books by gradually and carefully exposing me to my phobic triggers until they no longer set me off. Dad is a living example of how one can have a chronic medical issue and still live a productive and fulfilling life. My parents considered no topic taboo, and helped me talk through a lot of my early struggles. At crucial moments they got me to therapists who helped me sort out my thoughts and fears. My parents are the real heroes of this story—in my most honest moments I know that I'm afraid to have children because I simply could not do what they did—and what you do now. And while I feel somewhat unqualified to suggest things to you all, simply because I'm not a parent of a child with a heart defect, I'd like to wrap up this series next week with a list of Dos and Don'ts for raising a kid with a CHD. Hopefully I can talk Mom into co-writing…

Important: Digitek Recall

Though the recall happened on April 25, I talked to a parent today who did not know about this, so felt it was important to post.

If you or your child take Digitek, please see the recall on the FDA website. Please spread the word to anyone who takes or may take this medication, as it seems that there has been little publicity, and many pharmacies are not aware of, or have been ignoring the recall.

"The voluntary all lot recall is due to the possibility that tablets with double the appropriate thickness may have been commercially released. These tablets may contain twice the approved level of active ingredient than it appropriate.

Digitek® is used to treat heart failure and abnormal heart rhythms. The existence of double strength tablets poses a risk of digitalis toxicity in patients with renal failure. Digitalis toxicity can cause nausea, vomiting, dizziness, low blood pressure, cardiac instability and bradycardia. Death can also result from excessive Digitalis intake. Several reports of illnesses and injuries have been received."

I read a blog today by one family (if I get permission, I'll add the link) that had been affected by the recall - their daughter was taking the double-dosage tablets. They posted the following photo showing the difference between the normal size (in this photo, Digoxin, the replacement for the recall) versus the recalled Digitek, which is quite larger.

Send Flowers to Mom & Help Raise Funds for Research!

LINCOLNSHIRE, Ill., May 5 /PRNewswire/

The Children's Heart Foundation today announced a new partnership with ProFlowers(R) -- the online fresh flower marketplace -- just in time for Mother's Day! Shoppers can visit http://www.proflowers.com/CHF and choose from a variety of beautiful, fresh-from-the-field flower arrangements. All year long, whenever flowers are purchased through the CHF page on ProFlowers, ten dollars from each purchase will benefit The Children's Heart Foundation (check out their new website!).

"Sending flowers to the mothers and grandmothers in your life is a great way to show your appreciation and celebrate their love -- and now it also can help raise much-needed funding to find better treatments and, hopefully someday a cure, for congenital heart defects" said William Foley, Executive Director, The Children's Heart Foundation.

The Children's Heart Foundation is the country's leading organization solely committed to congenital heart defect (CHD) research funding and is comprised of parents, family members and friends of those affected by a congenital heart defect. The goal of The Children's Heart Foundation is to increase awareness, support the recently diagnosed through educational materials and continue funding the most promising research to find better treatments.

Growing up with a CHD: The good, the bad, and the…*interesting*

By Sarah Clark

Part 1: First, the good news

I’m probably not very different from some of the younger parents who follow this blog. I’m a 31 year old living in Northeast Oklahoma with my husband of 6 years and our cat, Tawnya. We’re moving into our first house in about a month, and we’re looking forward to filling up our new bookshelves, setting up a whole-house wireless network, and starting a proper vegetable garden. I’m a librarian at a local university, and I spend my days keeping up with overdue notices, teaching information literacy classes, and helping our online students get the research help they need for their classes. I was born with Transposition of the Great Arteries, VSD, and PDA, all corrected by the time I was 18 months old. My husband Kevin is a software developer. We walk a few times a week (when we’re being good), and attempt to eat a reasonably healthy diet. We hang out with friends, spend too much time online, and could really stand to clean out the bedroom closet this weekend. All in all, we’re your typical middle class DINK couple, though perhaps a little geekier than most.

Just in case you missed the point of this recitation of our nice little life in suburbia, let me make it clear: I lead a normal, happy, reasonably healthy life, and as far as I can tell, my life is pretty typical of adults with my defect (the symptom-free survival rate at 30 years post-correction is over 80%, and most of us had Mustards or Sennings instead of the new and improved arterial switch procedure). I’ve had no physical complications to speak of, and I’ve accomplished most of the modest physical goals I’ve set for myself. My CHD is merely one of a laundry list of things that are notable about my life. I’ve led a generally happy life (with a few bumps in the road I’ll hit in part two), I’ve graduated bachelor’s and master’s programs with excellent grades, I studied in Scotland during my sophomore year of college, and I’m the first TGA that I know of to climb (a very small chunk of) the Great Wall of China. The most important thing I want to share is that most of those things didn’t happen in spite of my defect—in a very real way they happened because of it. Let me explain with a brief list of the benefits of being born with a congenital heart defect.

1. Immortality? What immortality? 24 hours after my mustard procedure, I managed to disconnect myself from the ventilator, coded, spent the next month in a drug-induced coma, and was expected to wake up with major brain damage. At two years old it looked like I might be developing pulmonary hypertension. Pretty much every CHD kid I knew well before the age of 4 died of their heart defect—I believe I went to my first funeral when I was about 5. I never believed I was immortal or untouchable. That’s probably a big part of the reason I’ve never touched drugs or nicotine, drink no more than one or two cups of coffee a week, and consider a glass of wine with dinner a wild night on the town. (Well, that and I'm a nerd who grew up in the 80s…)

2. Carpe Diem: I don’t know my life expectancy. Some of the first patients to have surgically corrected heart defects are now in their 80s and the picture of health. Then again, there are people younger than me with my defect who are in heart failure and on disability. All those cheeseburgers I ate on the run in my dot-com days (and am still working off) could catch up with me tomorrow (or never). For that matter, I could get hit by a bus. I save as though I’m going to be one of the first TGA centenarians (which is the plan), but I don’t put off my dreams. If I find myself in a crappy job or relationship, I start working to get out of it. If I want to see the world I save up for a plane ticket and go. If I want the triple cheese decadence special, I eat it with very little guilt (this one’s a double-edged sword, though…) In a nutshell, everyone’s life is too short to put up with nonsense, but I can’t assume I’ll have as many “later”s as other people. And if I make it to that 100th birthday, I’ll have a LOT of cool stories to tell at my party.

3. Overachiever and proud of it: I play hard, and I work hard. I always have to have some sort of goal or project going, and while I’m no prodigy, I’ve accomplished a good bit in my first 30 years. I keep striving for the next accomplishment or the next refinement, which is a good thing in general but is another one of those double-edged swords. (again, see part two for the dark side of this) As a wee kid my cardiologist Dr. Razook told me that if I set my mind to it, I could accomplish anything. I was young/naïve enough to take him at his word, and for the most part he was right.

4. I don’t wait to tell people how I feel about them. Good or bad.

5. I feel an obligation to use my talents to leave the planet better off than I found it.

6. I don’t particularly care what other people think of my choices. I do a job most people would see as the height of boredom. I ignore people who tell me I need to watch some soap opera or “fix” some sartorial flaw of my husband’s. I march to my own drummer—in childhood because I didn’t know how to fit in as a bespectacled hyper-intelligent neurotic wimp, but later on because I didn’t particularly care to fit in. As I’ve gotten older, I’m more willing to wear the right outfit or schmooze with the right people to get to my goals, but the subterfuge is still only skin deep. And I don’t hide my brains for anyone.

To boil this thousand word description of the upside of TGA to its essence, I long ago realized that life was too short to put up with bullshit. I’m driven to make the most of my life, and I also care a lot about using my talents to help others improve their own lives. For the most part these are good things, and I wouldn’t change that for anything. However, that drive, and the drive of other adult CHD survivors that I know of, is powered by a decreasing but eternally present fuel of anxiety, perfectionism, survivor’s guilt, and by memories of the long-dead friends, acquaintances, and playdates that were no less deserving of the healthy, normal, and seemingly improbable life I enjoy today. Not only did I live while other children died, I live *because* other children died, and provided the knowledge needed to save the life of a chubby neurotic drama queen.

For better and worse, all of my life’s experiences have been marked by that essential truth. I don’t know whether that will be true for your child—but all I can do is share my experiences, good and bad, and hope that you can find some use for them in parenting your child. But remember—I still wouldn't change a thing.

Next week: The Bad.


Blog Note - Sarah is one of many people who I have befriended online from this blog. I hope you enjoy her perspective and experience as much as I do! Sarah has kindly agreed to provide a three-part series for LearnAboutCHD, and we can't wait for the next installment.

If you have a story, perspective or news item you would like to share, please email learnaboutchd@blogspot.com. The more we share, the more we learn and the more we can accomplish!