Saturday, November 29, 2008

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.

Wednesday, November 12, 2008

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

Tuesday, November 11, 2008

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

Monday, November 10, 2008

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).

Monday, November 3, 2008

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 and we'll be happy to share them here in cyberspace!

Sunday, November 2, 2008

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

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.

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


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.