It works better when you work together!

It’s hopeless.

We can’t do anything.

That is what parents of babies diagnosed with Hypoplastic Left Heart Syndrome (HLHS) were told not many years ago. The word Hypoplastic means “underdeveloped”, and that is exactly what has happened: the heart’s Left Ventricle is much too small to pump blood to the body. The hypoplasia extends much further than the Ventricle; the Aortic and Mitral valves are tiny (or nonexistent), and the Aorta itself is tiny. The child is alive only because of a Patent ductus arteriosus, a “built in” heart defect everyone is born with.

The Ductus Arteriosus is a small connection between the Pulmonary Artery and the Aorta that allows the heart to function while still in the womb. Once the child is born, the connection will close by itself, usually in less than two weeks. A connection that is “stuck open” — a Patent ductus arteriosus — is easily corrected. But in this case, when the ductus closes, blood flow drops and the child goes into shock.

HLHS was the defect the doctors had no answer for. Obviously, a surgical repair was the only option, but how? How do you fix a heart that is, for all intents and purposes, missing the left half? A heart transplant was an option, but rarely was an infant’s heart available, and there was almost no time to find one.

But in the early 1980’s the answer came. Rather than try to fix everything at once, make the repair in stages. Three operations, (known collectively as the Norwood Procedure) performed in sequence, allowed the child to survive. Certainly it was understood that this wasn’t a cure, but the child could live if he or she received lifelong medical care.

Fast forward to 2009. The first generation of HLHS survivors are now in their early to mid 20’s. And with no older survivors, no one has any idea what future health problems may affect them. Like Edmund Hillary climbing Everest, no one’s been here before, and no one has a clue what's ahead.

The CHD Blog and Adventures of a Funky Heart will both be in Washington, DC on February 10, 2009 to support the National Congenital Heart Coalition (NCHC) effort to create a national registry of patients with heart defects. The NCHC is made up of several different CHD organizations, working together for the first time. The American College of Cardiology, the Adult Congenital Heart Association, It's My Heart, the Congenital Heart Information Network, Mended Little Hearts, and the Children's Heart Foundation have all come together to work for this important cause. (The group information is taken from the cover of the informational packet that was mailed to attendees; there may have been more groups that have joined the effort since then!)

If data is gathered about those of us living with a Congenital Heart Defect right now, it could be analyzed and used to predict our future health care needs. It can also be used to make things a little easier for the next generation of CHD patients. As an example, I take 11 different medications. Yes that is a lot, but some CHD’ers take more, and some take less. If the knowledge gained from a national registry could eliminate one medication that each of us has to take, the savings would be phenomenal.

Much too often you read in your local newspaper about the child who needs surgery for a major heart defect (Infant Survives Heart Surgery, reads the title of the one and only article about me in the local Daily Planet) but what happens after that? Usually you don't see a follow up article in the local press. But Cardiac Kids grow up; we get jobs, pay our taxes, fall in love…

... In other words, we live our lives.

Pick them up and put them down!

It's a proven fact that exercise improves your fitness level. If you have a CHD, that's important: Strengthening a damaged heart can make it work better, function longer, and be more resistant to shock if you happen to have a health crisis. In short, exercise can extend your life.

For any exercise to be effective, your heart and respiratory rate have to increase - safely! So be sure to consult your doctor before beginning any exercise program. If your doctor clears you for exercise, be sure to ask if he/she recommends a certain type of exercise. Be sure to start slowly and build your strength.

Music can make your exercise routine go by faster and keep you going when your body says "Wait a minute!" You also tend to move with the beat: faster music makes you work harder. Don't believe me? American Pie is a classic song, but just try to exercise to it. 500 Miles by The Proclaimers works much better!

This website will sell you "workout music" with faster or slower beats, depending on how intense you want to exercise. Or you can download your favorite MP3's. Either way, if your doctor approves, get out and get moving!

Put down the Vicks!

No, not Michael Vick, the former NFL quarterback... that subject is an entirely different post for an entirely different blog! We're talking about Vicks VapoRub, which can be found in nearly every medicine cabinet in America. I bought a bottle myself the last time I was at the drugstore. We all have memories of our moms coating our chests with VapoRub when we were sick. Recent research has proven that we were all doing it wrong.

"The ingredients in Vicks can be irritants, causing the body to produce more mucus to protect the airway," said Bruce K. Rubin, MD, of Wake Forest University. While that's not good for anyone, it's especially not good for small children. And even though the report doesn't mention this, it can't be a good thing for someone with any airway issues... including a Congenital Heart Defect.

The study showed that VapoRub increased mucus secreation 59% in healthy, uninfected lungs. Even when a child is healthy, that much mucus could lead to airway inflammation and irritation, possibly closing the airway. Also, VapoRub doesn't actually make it easier to breathe. The menthol triggers a cold sensation as it enters the nasal cavities, which tricks the brain into believing there is increased airflow.

Newborn Screening Test Could Save Lives

Research paper: Impact of pulse oximetry screening on the detection of duct dependent congenital heart disease: A Swedish prospective screening study in 39,821 newborns

The British Medical Journal

Routine screening of blood oxygen levels before discharge from hospital improves the detection of life threatening congenital heart disease in newborns and may save lives, according to a study published on bmj.com today.

The low false positive rate of pulse oximetry screening and the reduced need for treatment because of a timely diagnosis also makes this a cost effective intervention, say the authors.

Pulse oximetry screening has been suggested for early detection of congenital heart disease, but its effectiveness is unclear.

The authors found that in apparently well babies ready for discharge a combination of clinical examination and pulse oximetry screening had a detection rate of 82.8% for duct-dependent heart disease. The detection rate of physical examination alone was 62.5%. Pulse oximetry also had a substantially lower false positive rate (0.17%) compared to physical examination alone (1.90%).

However, some babies had been detected before discharge examination, meaning that the introduction of pulse oximetry screening in West Götaland improved the total detection of duct dependent heart disease to 92%. This was significantly higher than the 72% detection rate in other regions not using the screening technique. Interestingly, improved detection was achieved by a maximum of just five minutes of extra nursing time per baby.

Links for CHD Families

Here are several very important links for you to read today. Read them online, download them, or print the file, whichever you choose. If you try to download the file as a .pdf document, you won't be able to... the writers are trying to protect their work, you can read the details on the download page. But you can print the entire file, which is just as good! The only bad thing is that these links are from medical journals, written by doctors for other doctors. It's almost 100% medical terminology, and you'll probably have to have an interpreter.

Tetralogy of Fallot (ToF) in a seven year old girl

Some thoughts on Tetralogy of Fallot (ToF) surgery

The Fontan Procedure... what's down the road?

This next link refers to the Glenn Shunt, and has a very good illustration. The illustration, however, is of the Classic Glenn Shunt, which was first used in the 1950's. The modern version, the Bi-directional Glenn Shunt, was first described in 1966 and not popular until the 1970's.

Click the .pdf button to be taken to a three page file concerning both versions of the Glenn Shunt. This is important reading for anyone who has a Glenn!

This link has more layers of information than a seven layer Chocolate Cake! Above the illustration, click the heading labeled "Contents"; there you can find 40+ links to heart defects, surgical procedures, and case studies. There is lots of information here, and not just on the Glenn Shunt.

Who We Are

So who are we?

We’re 1 out of every 120 people... there’s a new one of us every 14 minutes. We are both genders and we are all ages. A million of us are adults, and about 800,000 of us are children.

We’ve made it through surgeries, hospital stays, infections, Endocarditis, pacemakers, and Heaven know what else. We’ve given gallons of blood, one vial at a time. We’ve fought back against tremendous odds. We’ve been so sick that we’ve scared the world’s best doctors witless… and then amazed them even more when we recovered.

We aren’t related by blood but we are a family. We call ourselves the Cardiac Kids and Heart Warriors. We have an amazing inner strength, but we are terribly fragile at the same time. We know that most of us shouldn’t even be here and so we live every moment as if it is our last. We’ve celebrated our victories and we’ve mourned our losses. Our heroes include a Flying Tomato, a golfer, and a former movie star, because we are them and they are us. Our parents are Heart Dads and Heart Moms, and we consider those titles as Badges of Honor. They deserve it; they were the first ones to discover that a heart defect doesn’t just break one heart... it breaks three.

We work, we play, we pay our taxes and we live our lives. We’re in your community, in your church, in your school, in your office, and quite possibly in your home. We move a little slower, do some things a little differently, but we usually get along without causing a fuss.

We are people living with Congenital Heart Defects.

Welcome, Steve!

It is with great pleasure that the CHD Blog introduces a new contributor... Steve!

Steve, who blogs at Adventures of a Funky Heart, is a 42 year old male born with Tricuspid Atresia. Despite three heart operations, a stroke, and a pacemaker, he lives and thrives in rural South Carolina.

Over the past several months, Steve has allowed us to cross-link to some of his excellent entries. Now, Steve will be adding content to the CHD Blog on a more regular basis. We welcome Steve, and look forward to the wealth of information and insight he'll be providing! Be sure to visit his blog, which provides a front-row seat to the issues (and joy, and pain and pleasures) relating to life as a CHD survivor.

Second Opinions: It Never Hurts

The following letter arrived in the CHD Blog's inbox from Farshid Bayat...

My daughter Ava was born with DORV, D-TGV, PDA, large VSD, ASD and COA on 21 October 2006. In a nutshell, Ava had two surgeries at the ages of 2 and 12 months. Her final surgery took place on 27 April 2008. Although most of the cardiac pediatricians we had visited before were of the opinion that Ava's life could not be saved, the excellent pediatric cardiac surgeon Dr. Navabi Shirazi managed to thoroughly repair the main defects in a ten-hour surgery. Ava was discharged from the hospital after a ten-day stay.

Today Ava acts like any other child with no physical restrictions. She is a bundle of energy who loves running and playing football with her older friends. Parents of children with CHD should remember to ask for a second opinion before choosing a surgeon. Had we known about Dr. Navabi from the very beginning, Ava would have been treated by only one surgery.

I wish I could say that it's the only time I've heard of such a story, but it simply is not the case. Until we have a central registry of CHD cases and better reporting information for CHD cardiac care facilities, the need for second opinions will remain vital in your (or your loved one's) quality care. Many times, at diagnosis, we have little time to prepare. We may feel comfortable with the individual(s) who provide the diagnostic results and recommendations, and be lulled into a sense of comfort in working with them. But I urge patients and parents alike to be diligent - ask those tough questions, research the facility and its staff carefully and talk to fellow CHD survivors and families when you can - it can make a tremendous difference.

No Flu for me, thank you!

It's a given that Congenital Heart Defect Survivors need to try to keep themselves as healthy as they can. Our weakened bodies are susceptible to just about every illness that comes along, from the common cold to that winter monster... the Flu!

The first thing you should do, of course, is check with your cardiologist. If he/she recommends it, get the flu shot! This should be second nature to us by now - when the weather gets cold, we put on an extra layer of clothes, try to remember where we stored our gloves and scarf, and get the flu shot.

There are two web based resources that can also be useful in fighting the flu bug: The Centers for Disease Control (CDC) maintains a web page dedicated to information about the flu. They also offer an online weekly flu report. The weekly report usually refers to last week - it take a little time to compile all the information - but it is a very detailed assessment of Flu Activity in the United States. If you love numbers, charts and graphs, the weekly flu report is just your thing!

Google also offers a "flu tracking service", in a sense. They have flagged the words "flu" and "flu symptoms" in their search engine, and also flagged words that describe most of the major symptoms of the flu. They figure that when people start feeling sick, a lot of them will sit down at their computers and Google their symptoms. A lot of people do exactly that - and Google collects and tracks that information.

The result is Google FluTrends, a chart showing the number of people typing the symptoms into Google's search engine, along with a map showing the states those searches are coming from. Google says that unlike the CDC weekly report, their system anticipates the Flu. Because people tend to look up their symptoms as they appear, Google claims FluTrends is actually a day or two ahead of an outbreak. According to FluTrends, Texas is not the place to be right now!

Who's right? CDC has been tracking contagious illness for a long time, and they are good at it. But Google seems to be on to something... so why not use both?