Posts Tagged ‘Boston’

The Dirty Little Secret of HLHS

October 1, 2009

She didn’t even have a name for it.

When Dr. Helen Taussig wrote Congenital Malformations of the Heart in 1947, she described one malformation as “Atresia or marked hypoplasia of the aortic orifice prevents the expulsion of blood from the left ventricle in the normal manner.” Taussig described several variations of the defect – actually different defects, later grouped together under the same deadly name – but could offer no treatment suggestions. Her Tetralogy of Fallot (ToF) patients could at least squat and get some temporary relief; children cursed with this malady died in less than one week. The left side of the heart wasn’t damaged as much as it just wasn’t there.

It wasn’t until 1958 that Dr. Jacqueline Noonan and Dr. Alexander Nadas named the group of defects Hypoplastic Left Heart Syndrome (HLHS). Hypoplastic comes from the root word hypoplasia, which means “small”.

But having a name for it didn’t make it any less deadly as HLHS continued to claim 100% of its victims. This sad story continued until 1985, when the first successful infant heart transplant took place.

At about the same time the Norwood Procedure was developed. Originally designed as one operation (which consistently failed) the procedure was soon split into two heart operations – and eventually three – which seemed to work. HLHS children now had a chance. Obviously long term survival rates aren’t known yet, but approximately 70% survive the three surgery protocol.

And thats when the dirty little secret of HLHS showed itself: While survival rates for the three operation procedure may be in the 70% range, getting from Stage I to Stage II is the hardest step.

The goal of the Stage I operation is to make the Right Ventricle do the job of the defective Left Ventricle – getting the blood to the body. With all of the blood flowing through the right side of the heart, that side is subject to higher flow pressures than it usually receives. A lot higher. In a defect in which the entire left side of the heart is damaged, a successful outcome usually depends on the Tricuspid Valve – located on the right side of the heart.

The inital results were confusing. The Norwood worked – then it didn’t. There didn’t seem to be any rhyme or reason to it, no way to predict outcomes. The surgery itself worked, but too often the results  seemed to be as random as a roll of the dice. The answer was the Sano Shunt, invented by Japanese surgeon Shunji Sano – a direct connection from the Right Ventricle to the Pulmonary Artery through a Gortex conduit. (this sounds redundant, but the Pulmonary Artery is normally disconnected from the ventricle in the Stage I operation). Studies showed that survival chances with the Modified Norwood was 11 times greater than with the Classic Norwood.

And now it is time to turn the tables – we’ve got a nasty little trick of our own. Heart defects have to start somewhere… at some point in fetal development, there must be one isolated problem that seems to “snowball” into something bigger as time passes. If we could find that one little problem and repair it then, maybe we can stop the snowball before it gets too big. A pretty cool idea, and maybe one day…

Someday…

A study released Monday (September 28, 2009) revealed that for seven years, doctors at several hospitals in Boston and Harvard Medical School have been detecting Aortic Stenosis in fetuses. Fetal Aortic Stenosis is a snowball; it usually becomes HLHS as the fetus develops.

70 future HLHS patients underwent surgery while still in the womb – a needle was inserted into the mother’s abdomen, passed into the fetus, and into the heart of the unborn child. A small balloon was used to enlarge the abnormally small Aortic Valve. 51 of the procedures were considered successful… and 30% (17 children of the 51) were born with two functional ventricles. That’s 17 children who won’t have HLHS.

Mark September 28  on your calendar… that was the day that the CHD world changed forever.

Those People

August 19, 2009

Some things just **** you off.

Duncan Cross noted this on his blog, and somehow he didn’t go postal. And at first I was thinking that it couldn’t be true; somehow Duncan had misread, misinterpreted, or maybe was just having an officially certified 100% BAD DAY. So I clicked the link and checked it out for myself.

Thank goodness I don’t have any blood pressure problems.

There’s an “advice column” on Boston.com., the online presence of The Boston Globe. Seems fairly harmless; I mean, how many of us glance at Dear Abby while we’re reading the paper in the morning?

But the letter in the August 5th column torked me off – and a lot of other people, I bet. Here’s how it went:

My boyfriend has always had health problems, but a little over a year ago, things really got bad. There’s no need to go into the details, but suffice it to say, it isn’t going to go away, and it has impacted every aspect of our lives. Because it is often difficult for him to move around, we started going out less and less. Our home became less social as he didn’t like having people over as much. His interests narrowed, his mood soured (he’s being treated for depression). Our sex life dried up. I took it upon myself to do whatever I could to make life better for him; I have taken on more and more responsibilities; I physically take care of him; and I have been patient, accommodating and understanding as our lives changed…

…I feel isolated, stuck, and sad, and have been fighting the urge to flee. I think he may suspect my feelings, because he is reminding me more frequently how he loves me and couldn’t go on without me. But I just don’t know. On the one hand, I have all the responsibilities of the relationship, and none of the emotional or physical joy that should come with it. I don’t think he is either willing or capable of living beyond the lifestyle we currently live. I’m 24 and I am terrified at the prospect that this is it, that this is going to be my life. On the other hand, he’s still my best friend, and I love him and deeply care for him. He’s in pain, both physically and emotionally, and he needs me. If I left, it would break his heart, and when I think about the reality of that, it absolutely shakes me.

What should I do? It’s been over a year, and I feel I have tried everything, and things are not getting any better. Am I a horrible person to be thinking about leaving him?

Quite a mess is brewing – sick boyfriend isn’t doing so well and he is withdrawing into himself. In a way it is understandable, we’re all vain to some point. If we get an illness that really decimates us, it’s natural to think “I don’t want anyone to see me like this,” and retreat into ourselves. And that applies just as much to the person who gets blindsided by a bad case of the flu as it does to someone with a Chronic Illness whose health slowly deteriorates.

But this isn’t what irritates me. Our advice columnist answers in a flippant style that completely misses the mark:

You’re allowed to leave. You’re 24. You didn’t sign up for this. I fear the longer you stay, the worse it will be when you bolt.

He’s your friend. You owe it to him to be honest. Tell him you’re not up for this. You can’t commit to this life, at least not as a romantic partner. You can explain that part of your decision is about age and place in life. I truly believe it’s not just his illness. You want to discover more of the world. You want to get to know yourself better. That’s what unmarried people do at your age. You will feel like a jerk — but being a jerk is better than being a martyr.

This “advice” really grates on my nerves – You’re 24, it’s much too early for you to show any character! If the friend wants out, say so. And then she should grab her stuff and go. It will hurt for a little while, but it will be best in the long run… because the guy will know that she’ll cut and run at the first hint of trouble. At least if you’re up front with him he’ll know who isn’t on his side.

Duncan highlights the fact that the advice columnist and the young woman both miss important points: The young man is still working – a part of the letter that I did not highlight mentions the fact that both of them work – and he’s probably hanging on to his job by his fingernails in this terrible economy. He’s working when he really should be at home taking care of himself, and it is taking a lot out of him. Also if he’s taking medication for depression, his libido has probably dried up. He’s not in the mood, he’s exhausted, and although he’s working he knows that because of the economy or his health that job could vanish. It’s little wonder that things aren’t all Champagne and Roses right now.

But the best is yet to come! Our advice maven offers this gem:

Remind him of all of the other people in his life who care for him. Tell him to seek out as much support as possible. Dealing with a chronic is miserable. He needs to learn to cope without alienating everyone he loves.

A chronic. Not a person, but a chronic. A thing. With six words, she strips millions of people of their basic humanity and throws them into a nameless, faceless group. A group that makes you miserable when you have to deal with them.

Drop the word chronic and substitute nearly any word that identifies an ethnic or a social  group, and you’ll have a fight on your hands. Try using the word Italian, Jew, Republican, Democrat, Yankee, or Redneck in that sentence and people will start screaming. If you really want people to hate your guts, throw the N word in there.

But use the sentence as it is written and there is nary a peep. Apparently it’s still OK in certain circles to discriminate against those of us who are living our lives in spite of it all.

I’m not really upset at the female for wanting out of the situation, she’s young and probably doesn’t know any better. But our advice columnist – she has no compassion, no human decency, and I doubt she understands the inner strength that we sometimes have to draw from just to keep going. And she apparently doesn’t care.

After all, those people are just chronics.

When we all sing the same song

July 6, 2009

On Disability Awareness Day 2007 at Boston’s Fenway Park, a young man with Autism was chosen to sing the National Anthem. (He’s standing at the microphone behind the catcher, the camera will focus on him in just a moment.) Halfway through the song, his nerves got the better of him and he broke out in a case of nervous laughter. Rather than boo him back to his seat, the 30,000 fans join in and help him finish the song!

When we all pull together, we can move the world.