Posts Tagged ‘HLHS’

Joshua

October 20, 2010

When Joshua Haskins passed away earlier this month there was a great deal of controversy. This post is not about Circumcision safety issues, but about a related and equally important topic.

Joshua’s mother is heartbroken – she’s supposed to be her child’s advocate, his defender, but now he’s gone and she blames herself. But I don’t – she had been a Heart Mom for seven weeks. There was a lot that Jill Haskins didn’t know, including one critical fact that I didn’t mention.

As regular readers of this blog know, I have a hernia. A hernia can be fixed with a simple operation;  when my dad had his hernia repaired it required an overnight hospital stay, but even that may not be required anymore. The average hernia can be repaired pretty easily, but mine can’t.

My Cardiologist has vetoed a hernia repair more than once, because my blood oxygenation level (the familiar PulseOx reading) is low. A Heart Healthy person will register a PulseOx of nearly 100%; but mine is normally 80 to 82%. “I don’t have any doubt we can put you to sleep,” my Cardiologist says. “The real challenge is waking you back up!”

Not only is anesthesia an issue, there’s more. “I could spend all day describing your cardiac anatomy to the operating team,” my heart doctor continues. “And I don’t know if they would understand it any better when I finished.” That’s quite a statement, considering my doctor has won several teaching awards in his career. I’m 44, and one of the open heart repairs I have had has changed greatly over the years. Another one just isn’t done anymore – there are only three places that I know you can find it: 1) in a musty old surgical textbook; 2) in the brain of a very old cardiac surgeon; and 3) in my chest.

If my hernia were to cause enough trouble that it had to be repaired, I couldn’t just have it done anywhere. I can’t run the risk of having a surgeon who has done this operation 12,759 times before and considers me just another patient, because I’m not. Because of my heart, the plan is to go to Emory University Hospital and have my hernia repaired with a Congenital Heart Surgeon in the room, just in case of trouble. Afterwards they would keep me in the Intensive Care Unit for at least 24 hours – again just in case.

I know this… and I never mentioned it to Joshua’s parents. I don’t recall them mentioning the circumcision to me before it occurred, but that doesn’t matter. I should have said “Look, with Joshua’s heart, there is never going to be a ‘simple’ medical procedure. More than likely he’ll even need antibiotics before and after he sees the dentist, so you really need to think about any kind of operation or procedure that can be avoided.”

But I didn’t say that.

We are each put on this Earth for a reason, to accomplish something. Perhaps Joshua’s task was to remind us all that when you have a Congenital Heart Defect, no medical procedure can be considered “routine.”

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Joshua needs you!

August 20, 2010

Just read that Joshua is having a difficult night. His heart rate, Blood Oxygen level, and Blood pressure all fell. Keep him in your thoughts tonight, please.

Update at 6:51 AM (10: 51 AM GMT): Joshua’s mom posted a message to her facebook page that he had “a stable night.”

You’ve come a long way, baby

August 17, 2010

Jill and Shane continue to report on Joshua. Diagnosed prenatally with Hypoplastic Left Heart Syndrome (HLHS), Joshua was delivered at a major medical center and was immediately sent to a Neonatal Intensive Care Unit. By contrast, I was born in a community hospital of less than 100 beds. My grandfather – only an occasional smoker – was so nervous that when I didn’t show up on time, he unwrapped the celebratory box of cigars that he planned to give away and lit up. He was chased outside (smoking was accepted almost everywhere in the mid 1960’s, but a nurse wasn’t comfortable with anyone smoking around the newborns) so he stood under a dogwood tree and smoked until he just had to find out how things were going. Then he jammed his cigar into a fork of the tree and rushed back inside. He never said anything about retrieving his cigar, so for all I know, it could still be there.

Joshua’s parents knew they would be on a roller coaster ride from the moment he arrived. I looked normal. There were no prenatal tests in the 60’s, not even a way to tell if the unborn child was a boy or a girl. Only my mother’s intuition led her to notice that something wasn’t exactly right, and she consulted a pediatrician. The pediatrician that they had used for my older brother was out of town so they talked to the New Guy.

The New Guy realized he was looking at a heart problem but wasn’t sure of the details, so he sent my parents and me to the BIG HOSPITAL 200 miles away. The Doctors at the BIG HOSPITAL figured out that I had Tricuspid Atresia… and stopped. They had never successfully treated a Tricuspid before, so they gave my folks the diagnosis and told them to take me home and enjoy the time I had. I was not long for this world.

New Guy hit the roof. BIG HOSPITAL may not be able to do anything, but he knew of places that would at least try. He found one, and to make a long story short, I was a patient of New Guy until I was 20 years old. The “new” had worn off by then, but he’s one of only two doctors to really understand what is going on inside of me – him and my current Cardiologist. I still see him around town every so often!

By contrast, Joshua was pegged as a Cardiac Kid long ago, while he was still being knit together in his mother’s womb. The moment he was born he was handed off to a skilled care unit. Their only job is to keep him stable until it is time for his repair. And while we thought 200 miles was quite a way to travel to a hospital, that’s no longer true. 200 miles is a short trip.

We’ve literally come a long way.

We’ll meet Joshua soon!

August 8, 2010

We have a new Cardiac Kid in our Blogroll, Joshua.The link is to his mom’s blog, as officially Joshua hasn’t made his appearance yet. He’s scheduled to be here on Monday, August 16.

Joshua will be born with Hypoplastic Left Heart Syndrome (HLHS) and his Mom and Dad will be glad to finally meet him… but understandably, they are worried. Any good parent would be! As of right now his first surgery is scheduled for Thursday, August 19.

I am asking each of you to consider passing our story along to your friends, family, and churches. Either just pass our names, a little snippet of what we will be facing soon, or my blog address, his mom writes.

We can do that! In fact, we can do better than that… Everyone take a moment to head over to Joshua’s Mom’s blog and let her know that we’ll all be pulling for them.


Haunted Dreams

June 23, 2010

Here’s a blog post from a friend and a Heart Mom that you need to read:

Last night as I watched Mary Clare zoom by me in her mini airplane laughing so hard tears were streaming, I couldn’t help but think of what is ahead of us.  The mother in me prayed a silent prayer and pleaded to God that this will somehow make it up to her.  Maybe if she has the most fun summer, she’ll not hate me for what I know must be done to her.  I’ve noticed Mel has also been thinking this way.  He has promised to paint her toenails everyday while in the hospital this fall.  He told her she could pick out 40 different colors, or however many days we are there, so they can be a different color everyday.  He also promised her a trip to Disney World to see all of her beloved Princesses afterward.

How can I do this again?  How can she do this again?  Yet again.  How am I going to be able to hand her over this time, not just for a heart cath, a simple procedure by comparison, but a surgery which rips her tiny body apart?  How am I going to be able to watch her on a vent again?  Lines, leads, monitors.  All over again.  Having her torn away from me at birth and sit by and watch all that she went through, then again to have the same thing happen merely 6 months later was horrific.  She has to do it all over again, but this time she will ask.  Why is this happening?  Why can’t we go home?  Why do I hurt?  Why can’t you put a band-aid on it?  These are the questions which haunt my dreams.

Wow. What a burden to carry around. How could anyone make the decision to send another person – especially a child – to heart surgery? And especially their child?

This Heart Mom obviously knows the answer to those questions: To save her life. To give her the best chance to have a reasonably normal existence.  And to perhaps one day, tell her own children that her mom stood up for her when she was a child; tell how Grandma and Grandpa never gave up on her. Even when it looked like their lives were heading right over the edge of the cliff, they dug their nails in and they hung on.

Because that’s what Heart Parents do. They ask one more question. They explore one more option. They go one more mile.

And please keep this young family in your thoughts. Drop by their blog and wish them the best!

HLHS Advice

June 22, 2010

Hypoplastic Left Heart Syndrome (HLHS) is a known killer. It is 100% fatal if left untreated, and before 1981 there was no treatment. For several years after that there were very few survivors as doctors learned that a surgical correction could not be done in one step, but rather took two (and eventually three) separate operations. Infant Heart Transplantation was a dream until 1984.

So doctors have only been able to combat HLHS for 30 years, and there is still a lot that is unknown. The hows and the whys of the defect are still only being guessed at. The operations are delicate: not only are they demanding on a newborn’s Circulatory System, but so much depends on blood flow and pressures in the heart. All of the blood now flows through the Tricuspid Valve, which wasn’t designed to move that much blood. The proper “balance” often depends on the patient and there is no “standard” series of operations for HLHS. Each one must be customized to the individual.

Which leads to this interesting report from the March 2010 issue of the  medical journal Pediatrics. 749 Pediatric Cardiologists were surveyed concerning their management recommendations when giving a diagnosis of Hypoplastic Left Heart Syndrome. 99.7% of all Pediatric Cardiologists recommended the three surgery approach. 67% of the doctors discussed a heart transplant with the new parents, and 62.2% discussed Compassionate Care without a surgical intervention. (The high numbers in each category shows that the doctors were not limited to one response.) However, only a small percentage – 14.9% discussed all three options.

Also, the doctors were asked what they would recommend when confronted with a prenatal diagnosis of HLHS. Almost all of them (98.8%) recommended continuation of the pregnancy with surgery after birth. 53.5% argued for continuation of the pregnancy with Compassionate Care/no surgery; and a very high 74.3% discussed termination of the pregnancy. Only 36.5% discussed all three options.

Interesting numbers, and much to think about. The bottom line is that most Cardiologists would tell you to fight for your child. But why would so many of them recommend ending the pregnancy when HLHS is diagnosed prenatally?

UPDATE: A reader has brought to my attention (See comments) that the research report states that the Cardiologists/Surgeons discuss these options rather than recommend them. A discussion, of course, is not a recommendation. Some patients may treat it as such (“The doctor will probably know best…”) but it does answer my closing question much better – and reflect the experiences of my readers who have chosen to comment.

I have stated before that I am not immune to mistakes, that is why I try to include a link to anything that is not in my personal experience – so you can check my work for yourself.

And I would like to publicly thank the commenter for bringing this error to my attention.

Cut ’em off at the pass!

May 30, 2010

It’s a common scene in old Western movies: The good guys are in hot pursuit, but the bank robbers are getting away. “Quick, let’s cut ’em off at the pass!” someone shouts, and all the good guys gallop off through a shortcut. A few moments later, they’ve managed to get ahead of the evildoers!

For ten years now, Children’s Hospital of Boston has been working on cutting Hypoplastic Left Heart Syndrome (HLHS) off at the pass – by doing heart surgery while the baby is still in the mother’s womb! One of the early signs of HLHS is Fetal Aortic Stenosis; a small Aorta present before birth. (This can be detected through a Fetal Echocardiogram.) Boston Children’s has developed a procedure in which a probe is inserted into the mother and passes through the womb, into the unborn child, and enlarges the Aortic Valve. This is known as Fetal Aortic Valve Dilation.

An article in the June 2010 issue of Congenital Cardiology Today details the procedure and reports on an analysis of 70 attempts to repair the defect. 52 of the procedures (70%) were “technically successful.” If the Left Ventricle is small (and it almost always is) when the operation is done, the Ventricle will not grow. However, it will support growth of other Left Ventricle structures and HLHS can usually be avoided. Aortic regurgitation (backward blood flow through the Aortic Valve) happened in about 40% of the cases. And unfortunately, death occurred 13% of the time.

But the good news is we are starting to be able to fight a severe Congenital Heart Defect before the child is even born – and stopping a severe Congenital Heart Defect almost before it starts!

The Hybrid Procedure

May 24, 2010

Until the 1980’s, most forms of Hypoplastic Left Heart Syndrome (HLHS) were fatal. That group of defects involve the left side of the heart, the side that pumps blood to the body, and surgeons had no procedure that could offer any hope. More often than not, all Pediatric Cardiologists could do was to offer Compassionate Care. This country has put men on the moon, many doctors would ask themselves. So why in the hell can’t we save these kids?

That changed with the development of the Norwood Procedure (a three operation sequence developed in 1981) and the first Neonatal Heart Transplant in the United States (1986) . But even though it was an answer, the Norwood Stage I is a difficult procedure.  It is a six-hour operation, a major re-plumbing of the heart and surrounding blood vessels performed when the child is less than one week old. Imagine, if you can, operating on a damaged heart the size of a walnut. And the delicate part isn’t the repair, it is balancing the blood flow correctly. Everything flows through the Tricuspid Valve, and you can increase the flow rate (which increases the pressure on the valve) some, but not a lot. Get the pressure too high and you damage the Tricuspid Valve, and we can’t have that. For a HLHS patient, the Norwood Stage I is the most important, the most difficult, and the most dangerous operation of the three.

What if it were possible for the Stage I to be an easier operation? Or better yet, what if you were able to eliminate it completely?

Well you can’t just disregard the Stage I – major HLHS requires some type of surgical intervention – but it can be an easier operation. Doctors in major heart hospitals around the US are refining what is known as the “Hybrid approach” and the early results are promising.

The Hybrid was developed at Nemours Cardiac Center in Orlando, Florida in 1999 and takes place not in the Operating Room, but in the Catheterization Lab. The chest is opened and both Pulmonary arteries are banded to restrict blood flow. Next, a stent is implanted in the Ductus Arteriosus to keep it open (creating a Patent Ductus Arteriosus, or PDA). And while the stent is placed via Catheter, it is not inserted through the groin. The Catheter is inserted directly into the Pulmonary Artery via the incision in the chest. At times, an Atrial Septal Defect (ASD) will also need to be created.

Once the stent is in place, the operation is over. Total time: 60 to 90 minutes, and the Heart/Lung bypass machine was not used. After the Hybrid, the rest of the Norwood is carried out as before. But the Hybrid Procedure allows the major surgery to be delayed, until the child is older and stronger.

A 2008 study showed that the results of this new operation were favorable. The University of Chicago started using the Hybrid for their high risk HLHS patients, but found that the results were so good that it is offered to all Norwood Procedure candidates. (Observant readers will notice that the “60 to 90 minutes” link and the “results were so good” link quote the same doctor, who worked at Chicago but later moved to Boston.)

Very few hospitals perform the Hybrid Procedure for HLHS; so if you get a prenatal diagnosis of Hypoplastic Left Heart Syndrome, be sure to ask about all treatment options – including (and especially) the Hybrid.

Becoming a Heart Warrior, Part III

December 31, 2009

The most important step to becoming a Heart Warrior is probably the most difficult: You will have to be able to discuss your heart defect intelligently.

This doesn’t mean that you’ll need to go to medical school and get an MD after your name. (Unless you want to – I know two CHDers who are physicians!) There are different ways to discuss something intelligently. You will need to know some medical terminology to discuss your CHD with a doctor, but you’ll use a different mindset when you discuss your heart with a potential boyfriend/girlfriend. And if a child asks you a question about your heart, you’ll answer their question in an entirely different way.

The first thing you should do is learn the anatomy of a normal heart. It doesn’t have to be a detailed knowledge, but you need to know the four chambers, the four valves,  and the major blood vessels. The heart has four chambers: two on the top and two on the bottom, and a wall (called the septum) right down the middle that divides them into left and right. The top chambers are the Left and Right Atrium, and the bottom chambers are the Left and Right Ventricle. The right side of the heart captures blood returning from the body and pumps it to the lungs. The left side of the heart takes blood coming from the lungs and pumps it back out to the body. So when people tell you that the heart is a pump, they’re wrong. It’s really two pumps in one case.

You need to understand the Cardiopulmonary cycle. Cardiopulmonary is a big word, but it’s really two smaller words: Cardio, from the word Cardiac, means anything having to do with the heart. Pulmonary means anything having to do with the lungs. And the Cardiopulmonary cycle is the path the blood follows as it moves through the heart and lungs.

A normal Cardiopulmonary cycle looks like this:

Vena Cava→Right Atrium→ Tricuspid Valve→ Right Ventricle→ Pulmonary Valve→Pulmonary Arteries→Lungs→Pulmonary Veins→Left Atrium→Mitral Valve→Left Ventricle→Aortic Valve→Aorta

Note that the first step is simply labeled “Vena Cava”, because there are two Vena Cavas: the Superior Vena Cava and the Inferior Vena Cava. The Superior Vena Cava brings blood from the upper half of the body and the Inferior Vena Cava brings blood from the lower half of the body. Both vessels send blood into the Right Atrium.

The Pulmonary Arteries and Veins seem to be backwards: The Pulmonary Arteries carry deoxygenated blood, while the Pulmonary Veins carry oxygenated blood. It doesn’t make any sense until you remember another definition of arteries and veins: arteries carry blood away from the heart, while the veins carry blood towards the heart. Since the Pulmonary Arteries carry blood from the heart to the lungs, and the Pulmonary veins carry blood from the lungs to the heart, this conundrum is solved!

Once you understand how a heart is supposed to work, you need to know how your heart works. Ask your parents, and ask your Cardiologist.

Your parents are going to be a great source of information, but remember something important: They were riding a wave of emotion when you were born, when you were diagnosed, and any time you went through an operation. Their memories are going to be clouded by that emotion. So ask your Cardiologist too, and use your critical thinking skills to combine the information you get from both sources.

Your doctor can also draw diagrams to help you understand, and you can test your knowledge of the medical terminology by talking with him or her. And if the doctor throws something at you that you don’t understand, you can always ask them to explain it in plain English!

If you have a Cyanotic heart defect, you should learn why it is called Cyanosis (From the word Cyan, which means blue) and what causes you to be Cyanotic.

You should learn what might happen to you down the road – any future operations that you may have to prepare for, and new medical advances. So read the medical literature. This is easier now than when I was trying to learn about my heart. Medical Journals are expensive, but a lot of information is available on the internet. Just enter your diagnosis into a search engine and click the enter button. Back in the old days I had to have a medical dictionary to figure out what some of the words meant, now you can just Google whatever you don’t understand. Be prepared to do a lot of Googling, especially at first! But don’t let that discourage you.

You will have to learn how to read carefully to see what an article is really saying. As I have mentioned before, if you do an internet search for Congestive Heart Failure, you’ll see that the  average time of survival after diagnosis is five years. That can be depressing… until you read further and learn that the study group included some very sick patients. So the “five years” isn’t true, a lot of it depends on how motivated the patient is. There’s really no way to teach this skill, you just have to read and learn.

And be prepared to find out some information that you may not want to know. You doctor may tell you that they have done all the surgical procedures that are possible, and from here on your life is going to be based on how well you take care of yourself. You may find a study that says CHDers don’t live as long as heart healthy people. That’s true – and almost every CHDer who has thought about it realizes that we might not be around as long as everyone else.

But remember that those medical journals can not account for the medical advances of the future. Seventy years ago, a kid with a heart defect lived a sad, miserable life and the vast majority of us died before our first birthday. And twenty five years ago, children born with Hypoplastic Left Heart Syndrome (HLHS) didn’t live a week. All that has changed! And Medicine makes new advances every day!

But it is not about length of life, but quality of life – and as long as you live your life to the fullest, figure out how to do what you want to do despite your health limitations – never give up and never give in! – then you will have become a true Heart Warrior!

Which HLHS surgery works better?

November 18, 2009

Update: My friend is out of surgery and doing well!

The three operation Norwood Procedure was developed in the 1980’s as an answer to Hypoplastic Left Heart Syndrome (HLHS). Since infant heart transplant was developed at about the same time, HLHS was 100% fatal before the invention of the Norwood.

But the results from those early Norwood Procedures varied wildly, especially in the early days when the Norwood consisted of only two surgeries. When the Sano Shunt – a direct Right Ventricle to Pulmonary Artery connection, using a “tube” made of Goretex – was introduced in early 2003, matters improved greatly. The Sano Shunt provided the heart with much more stability and increased survivability rates.

But while we know that the Sano works better, there hasn’t been much research into how much better it works…. until now.

The Pediatric Heart Network studied the cases of 555 infants with HLHS. Through random selection, roughly half of the infants received the Stage I Norwood with a Sano Shunt, the other half received the Stage I Norwood with a Modified Blalock-Taussig Shunt (MBTS).

Followup with the children showed that 26% of the Sano Shunt children required a heart transplant before the age of 1; 36% of the children with the MBTS version needed a transplant. But after two years, the number of children who needed a heart transplant was within four percentage points of each other. Good Pulmonary Artery growth helps in the success of Stage II and Stage III of the Norwood, and the MBTS seems to encourage Pulmonary Artery growth.

Meanwhile, the Sano Shunt had more complications that required a balloon or a stent to keep the shunt open. True, this is not as serious procedure as open heart surgery, but the fewer, the better. After all, minor surgery is any surgery that happens to someone I don’t know – when it is YOU, your child, or a family member, things quickly become very serious!

Overall, it seems that the Sano Shunt gives an HLHS patient better results over the first two years – after that, the odds even out. These children will have to be followed for years to determine the long term success rate of either the Sano Shunt or the Modified Blalock-Taussig.