Posts Tagged ‘ACHD’

Road Warriors

February 22, 2010

NEWS: Zeb has to have surgery (the Ross Procedure) on Friday, February 26. His Mom, Rhonda, is a friend of mine and reader of the Funky Heart!, so I would appreciate it if my readers could help me keep an eye on Zeb!

I am packed for the trip to Atlanta – it can be a pain traveling to Atlanta for my Cardiology appointments, but it’s all a part of having an unusual Chronic Illness. You need expert medical care, and I feel that the team at Emory are some of the best.  As I’ve said before, the expert you need will not be found in a town of 5,000 people. These folks get to be experts by using their skills, and there just isn’t enough medical traffic in smaller cities. So you have to find a big hospital that sees lots of patients, and has a lot of experience to draw on. (There are exceptions to every rule, and two of the best known exceptions in Medicine are The Mayo Clinic in Rochester, Minnesota and Duke University Hospital in Durham, North Carolina.)

A recent study reports that there are only 72 Adult Congenital Heart Defect programs in the United States. So it looks like Heart Warriors will be Road Warriors for some time to come.

We do what we must do to defeat the evil that lives within us.

The 50,000 mile tune up!

August 27, 2009

Things went great at my Adult Congenital Heart Defect examination at Emory University Hospital in Atlanta – they were very through. Pacemaker check, Echocardiogram, bloodwork, finger stick to check my anticoagulation (bloodthinner) level, and the Cardiology exam. Five different doctors were in the room with me, discussing my health at one point!

I’m doing well, my Cardiovascular system is “balanced.” The Echocardiogram shows no major changes from my last one – nothing different or alarming. My Mitral Valve has a small leak and my liver is slightly swollen; but I’ve had both of those problems for years. That’s pretty much my version of normal; the Cardiologist I’ve seen for 21 years didn’t even raise an eyebrow. The only “bad news” I got was that my pacemaker battery would probably need to be replaced in 16 to 21 months.

So I guess you’re just going to have to get used to having me around – I’m not going anywhere!

Fight for your rights

July 21, 2009

An article abstract I found this morning seems to fit in with our previous post. This report notes that from 1998 to 2005, the number of adults with a Congenital Heart Defect (CHD) being hospitalized rose 67%. Thankfully, the inpatient mortality is going down, but the cost of care is going up – to an estimated total of 3.1 Billion dollars in 2005.

$3,100,000,000… I think it might be time to take up a collection!

Obviously, medical technology is working – heart defects are not the death sentence that they once were. We can almost be considered a Chronic Illness: with proper management, we can live full lives.

In a way, it’s a good problem to have: Even though we’re still fragile, more of us are leaving the hospital and going back to our lives. But Heart Defects have never been recognized as a Chronic Illness. Even though CHDs are the number one birth defect in the United States, it’s still seen as “one of those sad things that happen every now and then.”

If you want something done right, you need to do it yourself. That’s why it’s up to those of us with Heart Defects to get the Congenital Heart Futures Act out of committee and passed. It has been laid aside as the Health Care Reform debate rages; so it’s up to us to get it back to the forefront. We may have to wait until the Reform debate plays out, which means that the bill could die in committee. If that happens, we have to reset, reload, and try again. Make your plans – we could be heading back to Washington. But if that is what it takes, we’ve got to be willing to get back in there and fight for it.

Getting there

April 2, 2009

Every six months or so I feel the need to mention this information again: Perhaps there are readers of Funky Heart who are new to the Congenital Heart Defect world and they don’t have their bearings yet. All of this came crashing down on them like a ton of bricks. In a few short weeks they’ve learned an entirely new language, met doctors they didn’t expect to meet, and learned what the phrase right now truly means. And you wonder if this madness will ever end.

The short answer is no. Your child will need specialized medical care all of his life. But we’re getting better at it. Today, 90% of all children who have open heart surgery survive to become adults. 75% of all children with Hypoplastic Left Heart Syndrome (HLHS) survive the three surgery procedure needed to live. That’s too low to be acceptable, but in 1980 there was no surgical option at all and the survival rate was close to 0% – a lucky few were able to get a heart transplant.

To educate yourself about your child’s heart defect, go to THIS LINK; it is excellent. The American Heart Association has some good information, and THIS ONE is probably the best.

If your Cardiac Kid is in his/her teens, it’s time to be thinking about moving them to Adult Cardiac Care. You can’t just pick an adult cardiologist out of a phone book, they aren’t trained to deal with patients who have CHD’s. You need someone who is trained to deal with Congenital Heart Defects but can see it from an adult perspective.  There aren’t that many of them (yet), so you need to go here and read the patient summary of the BETHESDA 32 REPORT. At the bottom of the page is a link to download the entire report as a .pdf file, you need to download it, print it (have plenty of paper for your printer; it is long) and read it carefully. It will give you an outline about how to move your child from a Pediatric Cardiologist to Adult Care. Also of interest is THIS REPORT (in .pdf format) from the National Heart, Lung, and Blood Institute (NHBLI). It’s only 9 pages long; print it and read it.

The ultimate goal is to enable Congenital Heart Defect survivors to live longer, better, and more fulfilling lives. Like the adults in this video produced by the Adult Congenital Heart Association (ACHA):

Don’t mess with a good thing

January 30, 2009

I was in my local cardiologist’s office, having problems. I was having dizzy spells and nausea, and each time I had those symptoms, I also had a heart flutter. This day was not shaping up to be one of the best.

Important Safety Tip: If you have a Congenital Heart Defect, you need the services of a very good cardiologist. Since my Adult Congenital Heart Defect Cardiologist is in Atlanta, I also have a local cardiologist that I see. He’s not an Adult Congenital specialist – I know, I’m breaking my own rule – but he’s a smart guy. If he’s faced with a situation he can’t resolve, he’ll call my Doc in Atlanta rather than insisting that he can figure it out himself.

The day had started pretty good. I was up early because it was a day that I was scheduled to work at the museum, and during breakfast my face got hot and I got dizzy. At the same moment I felt as if I was going to throw up, and I felt my heart skip a few beats. That’s unusual, especially considering that I have a pacemaker. But it passed in just a second, so I just mentally marked it as “One of those things”.

But then it happened again while I was getting a shower. Ok, once is a glitch, twice… twice is a problem. So now I was sitting in my local cardiologist’s office.

After an examination and X-Rays, the doctor’s next step was to interrogate the pacemaker. The word interrogate brings to mind memories of the TV show Homicide: Life of the Street. But that is almost the way it works: An electronic device communicates with the pacemaker and asks “Hey, what’s going on in there?”

Thankfully the cure was a simple one. A week earlier in I had stopped by the Pacemaker Clinic in Atlanta for my  yearly check. Your pacemaker clinic will check the pacer via telephone every few months, but at least once a year they want to see you in the home office. Not only do they get a better reading with you being monitored in person and not over a telephone line, but the best diagnostic tool ever invented is still the eyeball.

“I think we can turn your pacemaker down a little bit,” the pacemaker tech in Atlanta had told me. I was all in favor of that: Pacemaker batteries normally last 10 years or more, but because of my unusual heart, mine had worn out in less than three. So if they could do anything that would save the battery, I was all for it.

It turned out that my heart needed to be fully paced… tech-speak meaning my pacemaker needs to be running at full power all of the time. Turning the power down had set me up for the missed beats, the nausea, and the dizziness. Bummer!

But having a good local cardiologist kept me from making an emergency trip to Atlanta, so having a good local doctor is something you may want to think about.

Better Hospitals equal Better Care

September 3, 2008

I was already in the hospital when I had my stroke. I had been admitted to Emory University Hospital suffering from Congestive Heart Failure the day before, and a pacemaker had been enplanted to regulate my heartbeat. The nurses had been really good about letting me rest during the night, but now it was time for me to get up.

If you’ve had a major operation, you know that you just don’t “hop up.” Scratching my nose hurt, so why in God’s name should I even think about getting out of bed? But if you lay around too long fluid begins to collect in your lungs, and you’re on your way to pneumonia. So let’s get moving, young man!

It’s actually a bit easier than it used to be. When I was at the University of Alabama/Birmingham in 1977, they kept the fluid out of your lungs by coming to your room twice a day and slapping your back a couple of dozen times, then making you cough until you spit up into a cup. Today’s heart shaped pillows and cough bears are so much better!

So they sit me up, and slowly help me turn until my feet are hanging off the bed’s edge. Because of all the wires and tubes I have coming out of my body, this is more complicated than it sounds. There are two, and sometimes three, nurses involed in this operation: one to hold your arm and give you someone to brace against, and the others positioned behind you to keep all your wires and tubes from getting tangled. I ease myself up, take two steps, turn slightly, and settle into the chair.

The nurses turned on TV, so I wouldn’t have to just stare at the wall. The Montel Williams Show was on, so I blame him for what happened next. Apparently I am allergic to Montel Williams.

I was sitting there when my foot fell asleep. I didn’t even notice it at first, but when I did I didn’t give it much thought. My foot was under me at a funny angle, and I didn’t feel like wiggling and working it out. So I just let it ride.

A nurse walked by my cubicle and wished me a good morning, and I said good morning in return. She slammed on the brakes and looked at me and said “Smile for me.” I’ve never turned down a request for a smile from a young lady, so I gave her my best smile.

“We need to get you back in the bed right now,” she said, and rather than taking it slow and easy, that 100 pound nurse yanked me up, spun me on her finger, and slam dunked me in my bed. In a minute there was a doc leaning over me, flashing a little light into my eyes.

“So how we feeling?”

“I had a pacemaker put in yesterday, so I’m tired and sore. Other than that, pretty good.” Obviously, I wasn’t getting it. A few moments later, I was on my way to a CAT scan and my parents were being called. When they arrived, they were told that I had had a stroke, but it didn’t appear to be major. As for me, it seemed that my right side had just “turned off.” And within the hour, I was moved from recovery down to the ICU Unit.

There is a small community hospital near my home (not the one where I get my blood drawn). They seem to take a “weekends belong to us” approach: Just a few weeks ago a friend of mine became suddenly ill on a Friday afternoon. She was taken to the Emergency Room of this community hospital and admitted. Her doctor ordered some tests to determine the problem, but none of the tests were performed until Monday morning… when all of the regular staff got back from enjoying their weekend. It seemed to me that if something serious had happened to her over the weekend, she would have just had to cool her heels until 9:00 AM Monday morning.

My stroke happened early on a Sunday morning, yet the response to my needs was instantaneous. The nurses were on the ball, the CAT scanner was warmed up and ready to go, and the Neurologist was in the building. The patient (me) didn’t have to wait on anything.

When an ACHDer (when anyone, actually) begins thinking about what hospital to use as their primary care facility, they need to consider such things as weekend staffing. Do they seem to be “Open 24 Hours” or do they work bankers hours? Talk to your friends who have been a patient there over the weekend. If you happen to know someone who is on staff at the hospital, speak to them. Because health emergiencies don’t follow the clock.

MARK YOUR CALENDAR: ACHDer’s in the New York City area, there are two meetings coming up that will be on of interest to you: The first is an Educational Meeting on Heart Failure and Adult Congenital Heart Defects at 6:00 PM on September 9, 2008, at the New York Presbyterian Hospital/Columbia University Medical Center, in Room 213 on the 7th floor of the Milstein Building. The Milstein Building is located at 177 Fort Washington Avenue in New York City. If you’re coming, you are asked to bring a snack to share if possible.

Also, there will be an informal dinner with Maggie Lichtenberg, author of “The Open Heart Companion,” at the Viceroy Restaurant on September 17 at 6:30 PM. The Viceroy is located at 160 8th Avenue in New York City. Each attendee will pay for their own dinner.

For more information or to RSVP, e-mail Heather at

It’s my Party and I ain’t Crying!

August 31, 2008

My birthday is this week!

I used to hate the thought of a birthday, just basically ignoring the event. It’s just another year, I thought, and I’m probably never going to get the chance to be an old man. So why celebrate?

My attitude changed (somewhat) when my doc told me that my walking had probably added ten years to my life! That’s completely cool. Walking… now there was something I could do without feeling like I had just been hit in the chest with a baseball bat. It can get boring at times (especially when I forget to tape some music or a radio show) but you can do it almost anytime, anywhere. And if I do have my XM Radio ready to go, the walk flies by. The faster the music, the faster your pace, so I’ll tape a station all night long, then listen to it when I walk the next day. That gives me plenty of music, so I can zip by the slow love songs and find something to put some spring in my step. Thankfully good music doesn’t go stale, and day old music is just as fresh as when it was produced.

Another attitude changer was meeting Pam through the Adult Congenital Heart Association (ACHA). Pam has Tricuspid Atresia, just like I do, and she’s a year or two older than me! Pam’s defect must be perfectly balanced… she’s in her 40’s and has never had heart surgery! How in the world does that happen?!?!? I swear, if I ever go to Vegas on vacation, I’m taking Pam. That woman is so lucky we’ll bankrupt a casino or two!

My outlook improved even more when I went to the ACHA’s National Convention in Philadelphia this year. There were 300+ people in attendance and 80% or more had a heart defect. (There was also a ACHA sponsored research symposium, so a good number of Cardiologists were there , too.) None of the ACHDer’s I met had a bad attitude. All of us were lively, engaged people enjoying our lives despite whatever rocks had been tossed our way. As the old saying goes, “You play the hand you’re dealt.” Apparently we have some real cardsharks in the ACHA, because they had taken a poor hand and played it into a winner!

You’ve seen the photo of the “Tricuspid Atresia Gang” (it’s below). The thing about it, not every Tricuspid patient is in the photo. I met at least three more after that photo was taken! So there was at least eleven of us at the convention.

For so many years every one of us had heard we were “rare” and “unusual”, and I had never met anyone else with Tricuspid Atresia. I may not be the only one, I thought, but there sure can’t be many. There aren’t – Tricuspid Atresia occurs in 1 out of every 10,000 live births – but there are more of us than we think! And as a group, we seem to be doing pretty good!

So I’m having a birthday, and that’s awesome!

Best Care Anywhere!

August 11, 2008

In the CBS TV show M*A*S*H*, there was a banner hanging in Rosie’s Bar that read:



If you are an adult with a Congenital Heart Defect, this is what you need. No, not Hawkeye Pierce and B.J. Hunnicutt cracking jokes and curing you in 30 minutes; you need “the best care anywhere.” After all, you have an unusual heart that has probably been altered by at least one surgical procedure. You have reached adulthood and are beginning to ask questions that perhaps your pediatric cardiologist can’t answer. You are interested in starting a family, but aren’t sure if your heart is strong enough for pregnancy. You’ve got questions about your heart and you need better answers.

You very well may need an Adult Congenital Heart Defect Cardiologist. The way I look at it, my heart presents a set of unique problems that not everyone can deal with. It’s not that I want, but rather I need medical services that are hard to find.

So where does an adult CHD’er start? A good starting point is the American College of Cardiology’s 32nd Bethesda Conference Report. In 2001, a group of Cardiologists were called together to study the problems faced by adults with CHD’s and make recommendations on how to best serve this group. The ACHA provides a summary page of the meeting, but a better thing to do is to read the entire report. You can download the report as a PDF file here. Give it some time to download; it’s pretty detailed. At the same time, it’s pretty readable. And it is almost a must read for ACHD’ers.

Bethesda 32 groups heart defects into three groups: Simple, Moderate, and Complex. Simple defects — there are “simple” defects, the report cites several — can usually be monitored by the general medical community. As an example of a “simple” defect, consider the Patent ductus arteriosus I mentioned in an earlier post. The Ductus Arteriosus is designed to disconnect itself and close shortly after birth. Sometimes it doesn’t, and doctors have to disconnect it manually. Since it was supposed to disconnect anyway, once the connection is severed it shouldn’t be a problem anymore.

Those with Moderate or Complex heart defects need lifelong medical care and under ideal conditions they would be routed to regional Adult Congenital Heart Defect centers as they reached adulthood. Nothing is ideal; a Google search for “List of ACHD centers” causes Google to ask if I’m looking for ADHD (Attention-Deficit/Hyperactivity Disorder) centers!

This is one of the many benefits of joining the Adult Congenital Heart Association. Membership is free, and we maintain a list of Adult Congenital Heart Defect centers. If you feel you need a ACHD center (or you fit the profile in the Bethesda 32 Report) search the list and find one that appeals to you.

Be prepared to travel. There isn’t an ACHD center in every state, unfortunately; or perhaps the one 300 miles away seems to meet your needs better than the one 75 miles away. One of the best ways to make a quick evaluation of a center is to see how many patients they see: Experience, after all, makes you better at what you do. You wouldn’t expect to find an expert in hypothermia in Florida, and the National Weather Service isn’t going to put their best Hurricane expert in Montana.

If you are an adult with a Congenital Heart Defect, you need a more advanced level of care than most doctors/hospitals can provide. So download the Bethesda 32 report, read it, and find a good ACHD center. Make an appointment. And get ready to live… you life is not over, not by a long shot!

The Tallest Kid in the Room

July 28, 2008

They always have Highlights for Children. It’s a common staple of any Pediatrician’s office, but it never seemed that they made the magazine available for home delivery. I always wanted a subscription to Highlights, because I enjoyed all the puzzle pages. But that was then. Times have changed, and now I’d rather have a subscription to a good baseball magazine. Most children aren’t that interested in baseball, so I need to bring my own.

I walk into the doctor’s office and I can feel the eyes boring into my back. I can’t see them, but I know that everyone is looking at me. When someone my age comes into a pediatrician’s office without a child, everyone stares. What’s he doing here? If they are wondering now, just wait until the nurse checks me in and tells me to have a seat.

Usually all of the adult chairs are occupied. It’s impolite to stand in the waiting room – there’s no rule against it, but I’ve never seen it done unless every seat is taken – so I sit in one of the children’s chairs. That’s an adventure unto itself. You sit down as you normally do, but you just keep going down until your bottom hits the chair with a bone jarring THUMP and your knees are level with your eyes. Now I look stupid, so I stretch out my legs. Not only am I blocking the walkway, but now I really look stupid, so I pull my legs back in. Hopefully this won’t be as bad as the time that I had pneumonia: my doctor admitted me to the hospital, and after a 4 hour wait in the ER I learned the only available bed was on the Children’s wing. It was a loooooooooong way from my rear end to the child sized toilet, and I was too far down to stand up! I literally had to roll off the seat and then get to my feet. But there was no room in the inn, you take what you can get, and beggars can’t be choosers.

Until I was 30 years old I saw my local pediatrician for non-emergencies. It works the same way for kids with heart problems. “Adult” Cardiologists can’t deal with us – they are trained to deal with heart attacks, clogged arteries, and all the problems that your heart develops as you age. They usually don’t have experience dealing with Congenital Heart Defects (CHD). A friend of mine – also a Cardiologist – once said that if you chose to specialize in Adult Cardiology, you received about two hours of training in CHDs. Basically just enough to know that they exist. So no matter our age, Cardiac Kids are still patients of a children’s doctor.
But now there’s a new specialty, known as an Adult Congenital Heart Defect doctor. Adult Congenital Defect care is not taught at any medical school (yet); many of the ACHD docs are really Pediatric Cardiologists. Since their patients stay with them practically all their life, these “children’s doctors” found themselves dealing with questions about work, pregnancy, dating, insurance… questions that Adult Cardiologists usually have to answer. And so the field of ACHD Cardiology was born.

You won’t find one on every corner, but ACHD care can be found. Usually, you’ll need to go to a major medical center. I live in rural South Carolina; the ACHD centers in the South include Duke University, Emory University, and the University of Alabama at Birmingham (UAB). Other major centers include The Mayo Clinic, Stanford, the Philadelphia Adult Congenital Heart Center and Children’s Hospital of Boston. There are other clinics, of course, but those are the ones that spring immediately to mind. And now, the waiting rooms have people who are more in my own age range.

I miss the visits to the Pediatric Cardiologist, though. While I’m sitting there trying to get comfortable, absorbing all the stares, the kids don’t pay a bit of attention to me. Kids are kids, they are usually congregated in the play area, getting along famously and having a great time. For once, they aren’t being left behind on the playground – everyone is moving at about the same pace.

The parents are the ones sitting there with the worried look on their face, with good reason. I always hope that one of the parents will speak to me, perhaps ask if my child is sick. If they do, I’ll tell them that no, I’m the one with the heart defect. My parents were in the same boat you were. I’m 41 now, and still going strong. And hopefully someone will come to believe that with good medical care and a little good fortune, their child will do well.