Genetics And BAV Disease

I was talking with Scott in Austin, TX the other day. Scott is another gay guy who just had OHS to correct an aneurysm. He had AVR three years ago but they didn't address the aorta at that time...a serious mistake. So he had to have another surgery. He is (was) BAV. He told me about some genetic research that is going on to try to find the marker for BAV disease. This is research that really needs to be done. He gave me the name and telephone number of the person he has as a contact...called a Bicuspid Genetic Counselor. I think I'll telephone this person next week and see what they're up to. He seems to feel like they are interested in identifying others with BAV to include in their studies. BTW, Scott told me that none of his first degree relatives are BAV, but he has a couple of cousins that are!

Should I Scuba Dive Anymore?

I'm a certified diver..almost 30 years. I'm BAV with stenosis. Based upon the list of "do's and don'ts" that I was just given, I think I would still dive, but conservatively...i.e., certainly no decompression diving whatsoever, and strictly shallow sport diving in non-challenging environments (no currents to fight, etc.). Disclaimers big time: I am NOT a medical professional, just a guy trying to live life to the fullest. I give no advice, just relating what I'm doing.

Walking

Our dog, Bradley, takes me for two walks a day. Two miles in the AM and two miles in the PM...one hour each 7/365. I used to jog but I found that I couldn't do it anymore about four years ago. I can walk fine on level ground but hills are tough. Walking is recommended for people with heart valve issues.

Aspirin Therapy

I started tasking a daily coated aspirin on January 15th as suggested by my surgeon. I've never taken a daily aspirin before, only one a week, but I never had any reaction to aspirin like stomach discomfort. The coated aspirin is supposed to be easier on the stomach so I don't anticipate any problems.

I'm not exactly sure what taking a daily aspirin is supposed to do for me. The literature that came with the aspirin bottle shows a little graphic of blood flowing through an artery with platelets shown as less likely to clog a narrowing. So I suppose with a narrowed aortic valve, the aspirin would make clot formation there less likely. Or, perhaps the surgeon felt that I should be taking it just in case I have coronary artery disease. In any event, with a history of stroke on my mother's side of the family, it's probably a good idea for me to take aspirin.

Who knows where the time goes - Fairport Convention

Who, indeed?

Marfan Syndrome Similarities

My good friend Barbara L. has suggested looking at Marfan syndrome information for ideas on how to cope with BAV disease. Marfan is a much rarer but better understood genetic disease that manifests itself with some of the same symptoms that we see in BAV disease. In particular, since Marfan is also a connective tissue disease disorder, people who have it often have aortic aneurysms, and eyesight problems. I don't have Marfan, even though I do have some of the classic traits like being tall, relatively thin face with arched palate and crowded teeth, and of course, the aorta problem.

One of the best resources I've come across is a web site put up by a woman named Janis Cortese. It has a ton of information about Marfan. You can get to it here.

I Got What I Wanted

As the saying goes over at valvereplacement.com, only a surgeon can cure valve disease. A cardiologist cannot cure your valve problem. The reason I sought out a surgeon's opinion is that the conventional wisdom over at VR.com is that cardiologists tend to be more conservative in the management of valve disease, and prefer to delay surgical intervention until the onset of severe symptoms. On the other hand, it is felt that surgeons prefer to intervene at an earlier point, before any permanent damage is done to the heart. This may or may not be true depending on who the care providers are.

After sleeping on it, it occurs to me that I got what I wanted from the surgeon at Penn, i.e., an evaluation. In his opinion, it is not yet time for my surgery. I'm not thrilled to be walking around with an aortic aneurysm, but many people have one and many people don't even know that they have it. At least I'm aware of the problem, I have obtained an evaluation from someone who is capable of curing it, and I have a plan for management of my condition going forward. So, that's not all that bad a place to be, all things considered.

A Day Of Tests And The Aftermath

So, after a day of tests I met with my heart surgeon. I'm still processing all the information but I think where we're at for now is watching and waiting on my aortic valve, with management of my aortic aneurysm that primarily involves blood pressure control. I will be meeting with a new cardiologist soon to determine a course of medication(s) and perhaps other treatment options going forward.

This is not what I expected and I will need some time to process all the information. I do have concerns about what has taken place relative to the degree to which this particular surgeon is sensitive to the special needs of BAV patients as well as a seeming disconnect between what he told me when we first met vs. what he said yesterday. There also appears to be a discrepancy between what he told me and what I read over and over again in the literature about BAV and the severity of aneurysms.

For example, he seems to feel that my aorta is not a big concern at 4.3 cm. However, if you read the BAV literature, studies indicate that anything over 4.0 or 4.5 is serious enough to warrant replacement at the time of AV surgery in BAV patients. Ross, the administrator of VR.com is BAV and had his aorta rupture at 4.7 and it almost killed him.

Another disconnect has to do with the AV. When we first met he indicated that I was in the severe range factoring in my body area (my size.) However, yesterday he didn't seem much concerned about the condition of the valve.

I'm sorry to say that I came away with the feeling that they process so many sick people through HUP that they may not be properly responsive to someone like me. I felt as if yesterday's appointment had little relation to the previous appointment.

Well, at minimum I need to lower my systolic blood pressure in order to take stress off of the aorta. So I will make an appointment with a cardiologist at HUP. Hopefully this person will be up on the BAV literature so that I can run some of my concerns by him.

CT Scan Today

So in a couple of hours I'll be inside one of these CT scanners. They produce fascinating images and great detail which allows for excellent evaluations.

An Important Day

Well, today is an important day for me and those who care for and support me. I get a CT scan, EKG, chest X-Ray and then I meet with my surgeon, Dr. Bavaria, at the University Of Pennsylvania hospital. I expect that we'll have a game plan worked out by the end of the day and I'll have a much better idea about the condition of my coronary arteries and aorta, and a good idea in regard to the timing of surgery. Unfortunately, I'm battling a sinus cold that I've been unable to shake for weeks. Nevertheless, I'll make my way over to the hospital in West Philadelphia a little later this morning to start getting the testing done.

WHo Is Allowed To Visit In The ICU?

I'm just catching my breath from a busy weekend at the toy train exhibition,. I had fun but it was a bit exhausting.

My friend Jane has raied the issue of who can visit me in the ICU when I'm hospitalized. This has entered my mind, of course. Dan and I have Power Of Attorney, Medical Power Of Attorney, and Living Wills drawn up, but I will have to find out what the policy of the hospital is in regard to unmarried significant others.

Easy Does It!

So yesterday I spent the day as part of a team setting up my toy train club's display up at a big train show in Oaks, Pa. just outside of Philadelphia. This was the first time I found myself consciously making sure that I didn't lift anything that is too heavy or otherwise do anything too strenuous out of fear of doing damage to my aneurysm. In the past I've always been a big bull and not afraid to lift things or put my shoulder to something to move it, etc. Those days are gone now. I still had a lot of fun and managed to be useful without doing anything risky. It just calls for a little common sense.

New Reading Mateial

Member Shannon over at VR.com posted a link to a recent (December 2008) set of ACC/AHA guidelines for the management of congenital heart diseases. Bicuspid valve disease is a congenital condition. I haven't read it yet but the document in PDF format is here.

Gran Torino Trailer HD

Run Don't Walk to see this excellent movie. It will surprise you and it's good for the heart!

Symptoms: Who Me?

I used to pride myself on being able to leap tall buildings in a single bound. When we traveled I would always do the physical stuff like climb hundreds of steps to get to the top of the cathedral tower in Ulm, Germany. However. this past year we were in Spain and I couldn't even consider climbing to the top of the church tower in Santiago De Compostela for the view. On a cruise ship in October I couldn't walk up the several flights of stairs to get to my room at one point.

One day last summer I swam out to the sand bar off Fire Island. On the return I got a little tired and panicky but I made it. When I got to the beach I had major chest tightness down into my left arm. I put the dog on a leash and walked it off but it took 45 minutes.

These things are symptoms of my BAV disease to be sure. In the literature you find references to the subtle nature of some of the symptoms of valve disease, and warnings to cardios and surgeons that patients may not be aware that they actually do have symptoms. Last year in August when my cardio asked me about "chest pain, dizziness, shortness of breath, etc." I said no. That was a lie, but I was in denial.

What is clear in the literature is that people with BAV who are symptomatic should have surgery.