Archive for the ‘Glenn Shunt’ Category

Back to School

November 12, 2010

“Trouble is, there’s not enough of us to go around – we’re spread thin, so sometimes, important things get ignored or don’t get said.” – Judge Tolliver (John Goodman), The Jack Bull (1999)

Yesterday I had the chance to go back to school – I went back to my Alma Mater to our School of Nursing!

I asked for directions just to be sure – when I graduated the School of Nursing didn’t exist. And I didn’t want to assume I could find it only to discover that it was in a back corner of the campus. But it wasn’t a problem; as soon as I turned off of the main highway and scanned the campus, there it was. Everything was exactly as described, which usually doesn’t happen – more often than not, I get twisted up and turned around but everything went perfectly.

After meeting the instructor for the first time, we went upstairs and into a classroom where I met the students (about 30 of them) and talked about myself and my heart defect. If you’re a reader of this blog, you know I usually post the printed text of my presentation. Not the case this time – as I told the students, when I make a presentation to Heart Families, I’ve got a plan and am pretty sure what I will say. With them, I wasn’t sure what they wanted or needed to know, so I’d just talk about myself. If they had a question, feel free to break in.

Someone had a really good question about did I need oxygen. Technically no, I don’t need oxygen, but I sleep with a flow of four liters per hour. It was originally prescribed to keep my Hemoglobin down, and I can skip it for several days without problem. When I take a weekend trip, I don’t take it with me. But I’m like a rechargeable battery and the O2 is like my charger – after about 4 days of sleeping without oxygen, I feel run down.

After I talked about myself and the Question and Answer session, we moved over a larger room set up as a hospital ward. When I walked in there was a bed to my right with a medical mannequin in the bed, tucked under the covers neatly. I saw him/it out of the corner of my eye and for a moment there I thought it was a real person!

We didn’t bother Earl (or whatever the mannequin’s name was), he looked comfortable. I took another bed, and in pairs and threes the students came in and examined me. My heart was listened to more times than I can could count, and everyone took a close look at my blue fingernails. My right hand is a little more blue than usual because of the swelling associated with my wrist, but my left hand is better suited to observe Capillary Refill (press down on the fingernail until it turns white, then release. Observe how long it takes for the blood to flow back.)

More than one student seemed to be very interested in the fact that you can’t read my pulse in my left arm – a side effect of the Blalock-Taussig Shunt, the surgery I had in 1977. In the Blalock-Taussig, the Left Subclavian Artery is cut and sewn into the Pulmonary Artery. The Left Subclavian normally passes near the shoulderblade (the Clavicle), and down the left arm. Because it has been disconnected, you can’t get a pulse in my left arm, can’t take an accurate blood pressure reading, and blood draws and vaccine injections should be done in the right arm.

(NOTE: If you have the Modified Blalock-Taussig Shunt, a small artificial connection is used to connect the Subclavian Artery to the Pulmonary Artery and the Subclavian is left intact. You usually can feel the pulse bilaterally on a patient with the Modified Blalock-Taussig!)

When the students weren’t listening to my heart, the instructor was – with an electronic recording stethoscope. I’ve had this done before, back in 1977 at the University of Alabama at Birmingham (recounted in this post.) But back then it was a Stethoscope head connected to wires that ran to a machine the size of a toolbox, that recorded my heart on a cassette tape. This one looked like a regular Stethoscope, perhaps a little thicker around the head. It would record (sensitive enough that it recorded a cough!) and then transmit the recording to a laptop computer via Bluetooth! My Geek side started getting the best of me, and I was developing a very, very bad case of STEVE WANT! But I knew that if I asked how much it cost, the instructor would inject me with 1000 cc’s of reality. Reality is a difficult drug to take – it’s good for you, but can make you feel pretty lousy.

I enjoyed my visit to the Nursing School and I’d like to thank everyone for making me feel so welcome. Even if none of the students chose to work in Congenital Cardiology, they’ll bump into other patients like me – it’s estimated that in the United States, there are slightly more adults living with a Congenital Heart Defect than there are children. Adults with Heart Defects are living longer and better, and we’ll have “normal” medical problems in addition to our bad hearts. And today’s Cardiac Kids are growing into tomorrow’s Heart Warriors.

So its important for those of us with a heart defect to “meet the public” – and not just to raise awareness, but to educate. To guide new Heart Families through this scary world we never expected to enter, but also to give the professionals who will be taking care of us a chance to learn from us. it doesn’t matter if someone is the best Heart Surgeon, the best Cardiologist, or the very best Cardiac Care Nurse… sometime in the past, these people had no idea that Heart Defects even existed.

Someone had to teach them.

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Good Nurses

November 10, 2010

NOTE TO READERS: We’re off on a Secret Mission tomorrow, so there may not be a post on Thursday. You’ll hear all about it later, though!

I was looking through my 1967 Johns Hopkins records and especially looking over the nurse reports. The nurse reports make up over half of my total file – neat sheets with handwritten measurements gathered at certain times. This was long before electronic records and most monitoring equipment. Nursing was really a hands on profession, not that it isn’t now. I’ve been cared for by some awesome people over the years, and a lot of them are nurses. You see and interact with the nurse a lot more than you do with the doctor, so when you are assigned a really good nurse it is a Great Thing and a Big Deal.

A big part of the job is observation and reporting, and in the ’60’s you were doing this constantly. There weren’t many automatic alarms that would go off when something was wrong; it was the nurse’s job to almost see problems coming. You were looking, observing, and making notes on every patient, so a good nurse had her head on a swivel. The nurses reports in my file I’ve nicknamed my “pee and poop sheets” because that was a major part of what was recorded: My temperature, amount taken in (input), amount expelled (output), and what I was doing at the time. Remember I was five months old; at any time of day I might be “playing”, “sleeping”, or more than likely, “crying”. Hey, I was a baby, and you people had just cut my chest open and tinkered around in there. You’d cry, too!

The order of the day was “Observe the patient.” You can almost hear the nursing department answer OK… what are we looking for? Trouble is, Cardiac Surgery was only about 25 years old then – they knew they had to prevent Pneumonia but after that, no one was really sure what to expect. So the Surgical Department just told them to observe the patient closely.

I had a fever on my 3rd day post-op. Hopkins called in a doctor who specialized in combating fever. “We see this a lot,” he told my parents. “Many patients spike a fever on the third day after their surgery, we don’t know why. If this is a 3 day fever, it will last about 24 hours and then it will disappear. We don’t know why that happens, either.” And he was correct – it was a 3 day fever, and it was gone 24 hours later. But he also told the nurses to keep a close eye on me, just in case it wasn’t a 3 day fever.

How do I know? The nurses report for that 24 hour period is crammed with notes. The best I can figure, they were checking on me every fifteen minutes for almost 30 hours.

Good nurses are lifesavers – literally!

 

 

 

‘These wounds I had on Crispin’s day.’

October 24, 2010

Monday, October 25:  Saint Crispin’s Day


This day is called the feast of Crispian:
He that outlives this day, and comes safe home,
Will stand a tip-toe when the day is named,
And rouse him at the name of Crispian.
He that shall live this day, and see old age,
Will yearly on the vigil feast his neighbours,
And say ‘To-morrow is Saint Crispian:’
Then will he strip his sleeve and show his scars.
And say ‘These wounds I had on Crispin’s day.’
Old men forget: yet all shall be forgot,
But he’ll remember with advantages
What feats he did that day.

– from Henry V by William Shakespeare, 1599

…And say ‘To-morrow is Saint Crispian’

October 24, 2010

If you are a fellow heart surgery Survivor, a Heart Parent, or just want to help rid the world of Congenital Heart Defects, feel free to link to Monday’s post. It will appear very late on the night of Sunday, October 24.

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.”

What you need in a Surgeon

September 15, 2010

It had to happen. Consumer Reports is now rating Surgical groups that perform Bypass Surgery. Just pick up an issue; you’ll be able to find out who makes the best toasters, washing machines, and who can re-route your arteries.

If it were only that easy for Congenital Heart Surgery. There are 35 different heart defects – this is the “base” number, that doesn’t count any defect that may have elements of two or more defects – and a multitude of different surgical options. Consumer Reports only looks at Bypass Surgery, a Congenital Surgeon may repair an Atrial Septal Defect (ASD) in the morning and perform a Bidirectional Glenn Shunt in the afternoon. If he’s on call he may come to the hospital in the middle of the night not knowing what problem he’s facing.

So assuming you have time to pick a Congenital Heart Surgeon, what do you need to look for? Experience and skill are the two basic factors, with a little understanding of what the numbers mean.

Experience is easy – Doc, how many years have you been operating? How many heart surgeries have you done, total? The Cardiologist says my son needs to have the *** Procedure, how many of those have you done? Answers to these questions will help you decide.

Now comes the not so easy part – try to determine your surgeons skill level. Let’s invent a situation: You ask the surgeon how his patients do after the operation, and he tells you that 80% of them go on to lead full lives. Now we need to know something else – what kind of operations is he doing? If our fictitious surgeon is fixing ASDs and 80% his patients “go on to lead full lives”… RUN! That’s a pretty low number. But if he is an Hypoplastic Left Heart Syndrome specialist and he usually performs the Norwood Procedure, 80% is very successful.

This is where it helps to be familiar with the Aristotle Complexity Score, and like so much about heart defects, the general public doesn’t know it exists. Most of the CHD community is even unaware of its existence. The Aristotle Score is a “rating system” of congenital heart surgical procedures, based on the three factors. Each factor is assigned a score of 1 to 5 points, then the points are totaled to find the Aristotle Complexity Score.

The first factor considered is Mortality: Historically, what percentage of patients having this surgery perish during or immediately after the procedure? An operation with a low Mortality receives one point; the highest Mortality receives 5. The next concern is Morbidity. Morbidity is defined as “a diseased state or symptom”, but in this context it is a measure of how long the average patient spends in the ICU after the operation. Again, short stays earn one point and the longest earn a five. The third consideration is the perceived difficulty of the operation. All heart surgery is difficult and requires years of practice and training – if it wasn’t, there would be a surgical center on every corner. But closing an ASD is easier than performing the Arterial Switch.

So every heart surgery is rated, with the “easiest” (a straightforward ASD repair) being rated at 3.0 and the most difficult (A Biventricular repair) receiving a 15.0. For example, my 1967 Glenn Shunt would be scored a 7.0. The Blalock-Taussig Shunt is a 6.8, while the Modified Blalock-Taussig (which uses an artificial tube to make the Subclavian Artery to Pulmonary Artery connection) is considered not quite as difficult at 6.3. The Score does not exist as a “ranking system” of surgeons (almost like Consumer Reports)  but as information. If the OR calls Recovery and tells them that we’re sending down a patient with an Aristotle Score of 10, they know a very ill person is on the way.

So knowing the surgeon’s experience and skill level is really only half the question. If you were to be able to determine how difficult his work is, that could increase (or decrease) your confidence level.

Just in case…

August 26, 2010

I was very pleased – to say the least! – about my exam at the Emory Adult Congenital Heart Center yesterday. An Echocardiogram showed that my Left Ventricle is 6 Millimeters smaller than the original Echo done there in 2002. It was 82 Millimeters across in 2002; it is now 76. And in my case, a shrinking heart is a 100% Official Certified GOOD THING!

If there is trouble in the future, if my PulseOx numbers were to start dropping and I was feeling worn out all the time, there are a few options that we could try to help get me back on an even keel. A couple of them are invasive but do not involve heart surgery… surgery can be a risky (and quite possibly fatal) proposition for me.

First things first, remember that 1) I am not a doctor; I’m just trying to explain it to you as it was explained to me. 2) This applies only to my heart and my health situation. Every heart defect is different, and what works for me may not be such a good thing for you… and vice versa.

The general plan for me would be to increase the blood oxygenation… but when you do that, the heart is naturally going to work harder. The trick is to find a happy balance between a decently high PulseOx and the amount of work that the heart can do. Right now my PulseOx is in the low 80% range and I have that happy balance.

The first option I would have (and all this is way in the future, if at all!) is based on my unusual anatomy. Like many reading, I have the Glenn Shunt. But mine was done in 1967, and is a completely different operation. Let’s review the difference:

The Bi-directional Glenn Shunt, the operation usually performed today: The Superior Vena Cava is cut where it joins the heart and is sewn into the Pulmonary Artery. They usually try to sew it as close to the T formed by the Pulmonary Artery to deliver an equal amount of blood to both lungs.

The Classic Glenn Shunt, performed on me in 1967: The Superior Vena Cava stays where it is. Instead of being cut, it is sewn closed. The right branch of the Pulmonary Artery is cut and sewn into the side of the Superior Vena Cava, which means that all of the blood from the Superior Vena Cava is sent into the right lung.

Now in my case, the Vena Cava wasn’t sewn completely closed. I don’t know if that was an error or if a small opening was left to relieve pressure that got too high, but a small amount of blood gets through the chokepoint and into the Right Atrium. If I were to start having problems they could use a Catheter to plug that small hole. That would cause my PulseOx to climb but shouldn’t increase the heart’s workload too much, and would probably be my best option.

The second thing they could do would be to create a fistula in my right arm. Basically, they would “short-circuit” the circulatory system by connecting an artery directly to a vein. My blood would head down my right arm as usual, but would “turn around” and head back toward the heart before it normally would. (Don’t worry,there are lots of of arterial branches and veins…. my arm wouldn’t rot and fall off!) That would increase the PulseOx numbers… but would also increase the heart’s work load. It is probably my second best option.

The third option would be a combination of medications that could reduce the natural resistance inside my body. Part of the heart’s work comes from how far the blood travels – if you could take all of the blood vessels out of an average human child and place them end to end, you’d have about 60,000 miles of blood vessels! Part of the heart’s work is because of resistance – your blood also has to turn corners and flow through organs (“Scuse me! Comin’ through!”). The medication would “grease” my blood vessels and make the blood flow through them easier. This would cause my PulseOx to rise… but not as much as any other option. My heart would also work harder. With more effort but not as many benefits, this is my third and least attractive option.

But getting a good report now gives me something better than all three of these possibilities: time. Nothing has to be done now, nor for the foreseeable future. And if I do reach the point where something needs to be done, delaying it now means that another option  could be developed that might be even better than the three ideas currently on the table.

All part of the master plan to keep pushing that final day back!

UPDATE: See Heather’s comment below for a good laugh! 🙂  Thanks, Heather!

Let’s close this case!

July 9, 2010

Is there anyone here who is a fan of the TNT tv series The Closer?

At first I didn’t really understand the show, and then it hit me – even though she portrays the same person, star Kyra Sedgwick plays her role as if she has a split personality. Sedgwick plays the role of Deputy Chief of Police Brenda Lee Johnson, and in “normal life”, she’s just Brenda Lee – a hapless, helpless Southern Belle who is almost in above her head and barely makes it through the day. But when the Priority Homicide team gets a suspect into the Interrogation Room, Brenda Lee goes away. Highly trained police officer Deputy Chief Johnson takes over. The Deputy Chief remembers everything and she can’t be fooled. She has a mind like a computer, she’ll calmly tear an alibi apart, and before long someone’s headed to jail. And it seems that the moment the case is solved, she turns back into stumbling, fumbling Brenda Lee.

It is a good show and is very entertaining. Sedgwick is very good, playing the same role from two entirely different perspectives – that has to be difficult to do (I can’t play myself on some days!). The Closer is an hour-long drama – 48 minutes if you record it and zip past the commercials. In that hour you’ll see a good story, a very good actress, and have a laugh or two. And in that hour four children will be born with a Congenital Heart Defect.

Whoa – never think about an hour of TV quite the same, will you?

That’s one of the problems with educating the general public about Heart Defects: The statistics are just too big. The National Heart, Lung, and Blood Institute (NHLBI) reports that 8 in every 1000 American Children will be born with a heart defect. That’s accurate, but a pretty large number -much bigger than our circle of friends. And if the number is bigger than what we can easily comprehend, our brains just seem to “turn off”. That’s why your local politician can say this programs will “only” cost two billion dollars and no one says anything. You can’t logically think about a number that big.

So for the 8 in 1000 number to make sense, you have to personally know 1000 pregnant women. That’s the only way to get a frame of reference. Most people can’t even name 1000 people that they know, much less 1000 women (who may or may not be pregnant). The March of Dimes did a little math and they report the number as 1 out of 125 children (1000 divided by 8 equals 125; so 1 in 125 is also accurate). That’s better, but it is still a big number. So using a little more math gets us down to the 4 children every hour figure.

That’s understandable – even our youngest can count to four, and practically everyone can conceive of an hour. So now we have a figure that we can easily relate to.

Another problem we have in CHD education is that we tend to speak our own language. The letters CHD strike fear in the hearts of those of us who have lived it – but what if you have never been in our shoes? The letters mean nothing. The varieties of the defect have medical names that very few can pronounce and even fewer understand. What is Tricuspid Atresia to the uninitiated? Most of us would understand Tri and that’s about it – it has something to do with three, as far as they know.

We don’t even understand our own language – try telling the momma of a child with Dexiocardia what Scimitar Syndrome is. She doesn’t know, and quite honestly she doesn’t need to know – her child has their own set of problems that she has to worry about. And to be fair, tell the Scimitar Syndrome mom about Dexiocardia. She’ll probably know it is a heart defect, but that’s all. (I intentionally didn’t provide a link to either Dexiocardia or Scimitar Syndrome. It isn’t so easy to comprehend if you can’t just click a link, is it?)

Another part of our language we can’t understand is our repair options. Most of them are named after people, and usually we don’t know why they have that name, who the people are, or what they did. You usually have to be a “giant in the medical community” to have an operation named after you, but that gives us almost nothing to help us understand the person. The Glenn procedure? What good does that do? How will it help my child? And when you learn exactly what the operation does, we’re still using medical terms and it seems that we understand less than before we began. Instead of medical jargon, we need a good drawing of the heart and someone who can speak everyday English.

“You take this blood vessel, unplug it from the heart, and sew it together with this blood vessel. You sew them together as close to the middle as possible so blood can flow to both lungs.” would make so much more sense than what we usually hear: “The Glenn Procedure is a Superior Vena Cava to Pulmonary Artery anastomosis.”

You can’t really be educated about a subject if you can’t understand what is being said. And when you receive a diagnosis of a heart defect, there is rarely any time to take Cardiology 101 and get ready to speak the language. So we have to do our best to get it down to numbers we can all understand, and get our point across in plain, simple English.

KISS – Keep it Simple, Stupid. Yeah, we have to pull out that old line – and follow it, if need be. We owe that to the Cardiac Kids who aren’t here yet, and to the legion of young parents who will be thrown headfirst into this world.

Deputy Chief Johnson would agree – anything less than our best efforts would be a crime.

Experience

January 14, 2010

All the families – including mine – were gathered in a small conference room just down the hall from Surgical Waiting. Surgeon Albert Pacifico flipped through a handful of index cards; consulting them to refresh him memory. Pacifico was fast and he had a system; he often completed six heart operations or more in a single day.

“I will speak to you last,” he said to my parents. My mother gasped and began to cry.

“That’s not the way we handle that,” the surgeon’s assistant said, but there was no reassurance. Something was up, they could tell.

Finally it was their turn. There was scar tissue in my chest, a lot of scarring. More than had been anticipated. They had unintentionally torn it making the incision and suddenly blood was everywhere. Pacifico had used every trick he knew – and even made up a few new ones on the spot – and I had needed twenty units of blood, but things were stable at the moment. If we chose to go ahead, he would continue the operation.

It was what he didn’t say that hung in the room. If we go ahead, we could easily tear something else and it could happen again. And we may not be able to stop the bleeding this time.

Just because Daddy told him to call it off didn’t mean it would be any easier. Everything in my chest was being held together with baling wire and bubble gum; he had to make the repairs more permanent and leave the operative field in some type of order for the next surgeon, if anyone ever tried again. Although it would be a good 24 hours before I turned the corner and started getting better, I made it through.

The selection of a surgeon is the most important thing when going into heart surgery. Every surgeon is good, he or she wouldn’t be holding the knife if they weren’t. But when you are talking about operating on an infant with a heart the size of a plum and blood vessels measured in millimeters, “good” just doesn’t exist. This is working at a level that is well beyond the capacities of the average person.

So how do you pick the right surgeon? There’s a good rule of thumb that I often quote: The doctor you need does not practice in a town of 5,000 people.

That’s right – if you live in a smaller town (my hometown has less than 500 people!) you aren’t going to find the doctor that you need. You can make a quick determination just by observing the doctor on the first examination. If they are thrilled to see your child because “They are just so unusual!”… grab your kid’s arm and RUN. You’re in the wrong place. What you need is a doctor who has seen enough heart defects that they are almost boring. And those doctors work in prominent medical centers that see a lot of patients. Ask how many operations they do every year on children like yours, and the higher the number, the better. Post-operative care is just as important as the surgery itself, maybe more so.

(There’s always an exception: If you are at a hospital connected with a medical school, your physician may bring several of his students around to examine your child and learn about their bad heart. Medical students are a good thing; they may remember your child and use what they learned to save a life later in their career!)

Not only do you want a large hospital with lots of surgical experience and a surgeon who is experienced, you want the right type of surgeon. The doctor who did granny’s triple bypass is not who you are looking for. Rarely does a bypass specialist go digging around in the heart, you want a Congenital Heart Surgeon. Adults who need surgery for a Congenital Heart Defect may be surprised (and worried!) to learn that the best person to do their operation is a pediatric surgeon, but most Congenital Cardiac Surgery is performed on children.

You can’t control everything, and you may have to make some snap decisions, but following these simple rules (Major Medical Center, lots of operative experience, surgeon with lots of Congenital Heart Surgery experience) can tilt the odds in your favor.

BATTLE WOUNDS (NSFW)

October 24, 2009

October 25, 2009: Saint Crispin’s Day

“Then will he strip his sleeve and show his scars,

And say`These wounds I had on Crispin’s Day.’ “

Henry V, William Shakespeare

scars