Educating Doctors – A Visit to My Vascular “Specialist”

I find that for the most part, doctors are more and more knowledgeable and “educated” about Familial Hypercholesterolemia (FH). They seem to understand how it works, what to prescribe, what tests to perform, why keeping the cholesterol numbers down is important and what to expect the aftermath to be if they can’t keep those numbers low enough.

I am also pleasantly surprised when a doctor understands that age has nothing to do with this disease. I have lots of respect for doctors who understand that in cases of more severe FH (like the homozygous kind) you must be as aggressive as possible with what the medical world has to offer (drug therapy and interventions) as early in the life of the patient as possible to get ahead of the disease and ensure the patient does have a life ahead of them.

I love and have huge respect for doctors who have the courage to act before a so-called symptom kills you. Cardiovascular disease is one of the main complications from FH and with this disease a symptom can often be fatal. I have had the luck to work with doctors who had the courage to act before a symptom was too late for me … I am here today because of such doctors.

But then there is this other crowd of doctors who are not covered in the “for the most part” crowd. Doctors who are so disconnected from the patient in front of them and so stuck in the letter-of-the-book (not sure what else; I want to give them the benefit of the doubt that they have read a book and not just simply call them ignorant) that they got taught in probably an old medical school course book from 1970’s when the research was done all on a 60+ year old male population, or something. Such is my current (till now) vascular specialist.

A while back, my cardiologist decided to refer me to one of his colleagues (who happens to be a former resident of his) that specializes in vascular health to manage my vascular disease, while he can focus on my heart health and my cholesterol management. I didn’t oppose him because I felt like I did need better management of my else-where vascular disease that does not necessarily live in the heart and he already has his hands full with my heart and cholesterol. I have seen this new specialist three times. So far, he is vague at best when speaking about my disease and how he plans to “manage” it.

On my latest appointment last week, we had spent about 45 minutes talking about the various stenoses that I have in the abdominal arteries:

• the main aortic trunk is more than 50% blocked (if I really do the math it is about 75% blocked based on what the normal lumen’s area is considered for me: the narrowest dimension of the aorta measures 0.6 x 0.4 cm when it’s supposed to be 1.2 x 1.2 cm); the transcript of the latest MRA notes “severe atherosclerosis of the abdominal aorta”;
• there is significant stenosis at the origin of the celiac trunk of more than 75%;
• there is mild proximal stenosis in the superior mesenteric of 50%;
• there is mild stenosis at the ostium of the right renal artery (~50%).

The entire time we’re seeing each other, as he also has said in previous appointments, he says “you have lots of disease.” He acknowledges this, which is somewhat reassuring. But then there is the part where I say “what do we about this?” And he comes back with “nothing. We watch it.” He confesses that "they (meaning specialists like him) are not trained to manage stenosis, really, but only aneurysms," and I don't have an aneurysm yet. I am a little taken aback and nervous because it seems to me like waiting for an aneurysm to happen might be too late. I want to be ahead of this. His uncertainty about how to deal with my (what seems to me) advanced stenoses is not very comforting. 

He explains that his biggest worry is not the celiac, mesenteric, or renal arteries, but it is the aorta. I am “happy” to hear this because my heart surgeon who also saw these results agrees that that’s very tight. Now, I had an endarterectomy of my aortic arch when I had my open-heart surgery, and the ascending aorta was so severely diseased that they replaced it with a Bentall graft. Based on my history, I ask him if we don’t need to be more aggressive about addressing at least the aorta if nothing else. He says no, because “there is not as much action through the aorta in your abdomen as it is in your heart”. So, we wait, because the disease in your heart aorta does not compare with the one in the abdominal aorta, so the threshold should not compare either. I am circumspect, but …  

He also offers that even with a bigger blockage he will be reluctant to have me see a vascular surgeon because I am “too young to have this kind of intervention.” He continues that “we really need to wait for more symptoms before we act. If you stop eating because of pain and you lose weight, then we can think about intervention.”

At this point, I sorta lose it. For full disclosure, I do not seek surgeries for the sake of surgeries. But in the past surgeries seemed to be the only option to save my life. And these findings scare me more than another bypass or graft. All I keep thinking about is my surgeon describing the amount of disease in my ascending aorta that he had never seen before. I was 40 when I had my heart surgery.  So, I remind him that age has absolutely nothing to do with the amount of disease in my arteries. I remind him that I have FH. That I have Homozygous FH and for the first half of my life my cholesterol was 600 mg/dl! I also remind him that my cholesterol now, although normal by healthy people’s standards – finally, just in the past few months!) is by far not “normal” for someone with a history of FH, heart attack and cardiovascular disease. So the disease in my arteries will likely continue. I also remind him that I am aging which will make the arteries even more stiff. So, shouldn’t we be a little more mindful about my particular situation and forget about what some book says about when such a surgery would be appropriate?

He back-tracks a little and he nods “yeah, that’s all true, but we also have to consider the risks of surgery. You realize that they would have to sever that aorta and by pass it with another blood vessel?” And I just chuckle: “You obviously don’t remember or don’t understand what I had done to my heart five years ago, if you think this scares me.” I also tell him that I do not want to “wait till I can’t eat anymore and start losing weight”. That seems more than irresponsible to me.

And then we talk about blood pressure. Usually, this is something I manage with my cardiologist and he has been trying to stabilize it for over a year with no success. But after doing some reading on my own and after talking with my surgeon, there are indications that stenosis particularly in the renal arteries can cause hard-to-manage blood pressure. So, I run this by him. He says, and this is an exact quote: “Well, your vascular disease has nothing to do with your blood pressure. You have just a regular, garden-variety blood pressure which is probably OK.”

I am almost speechless, but I find my voice, just barely to ask him to explain this “garden-variety blood pressure” of which I have not heard before. And he says “well, just a blood pressure that normally comes with age. You and I are the same age, so we normally get a bit of an elevated blood pressure as we age. That’s all. Easily managed with drugs.”

I remind him that mine is not easily managed with drugs. I also say, defeated, but wanted to give him one last “education” if I could, if not for me, for those people who will likely and unfortunately come after me: “So, we just got done talking about my history of severe FH, heart disease, massive surgery for 99% blockages in all main branches of the heart, and a trashed aorta; we got done talking about my abdominal stenoses and how my aortic trunk is so tight that, in your words, ‘scares you’, and you don’t think any of these findings have absolutely anything to do with my blood pressure? You think what I have is a ‘garden-variety’ blood pressure?!”

He pauses as he realizes the corner he painted himself into and says: “Well, no. That’s probably true. Those are some good points. All the stenoses probably have something to do with it, yes. But let’s leave it to the cardiologist for now.” Which is all fine and dandy, but ... is he considering these points in outlining his plan or is he not? He seems to me like he is not sure ... 

I was shaking so badly, I didn’t feel like I had the strength to argue anymore. We said our good-byes, I made the next appointment and he agreed to move my “watching tests” to 6 months rather than space them out to a year.

I am reconsidering ever going back, truly. I didn’t feel for one second that he actually saw me. I never felt like he really understood the risk of FH, the status of the vascular disease I have or whether he had a plan for it all. It would feel irresponsible of me to place my life in his hands. So, I probably won’t. Not anymore.

I wrote this blog mostly to share that you will need to be alert to ensure you are indeed getting the best care. I am probably a little bit too far on the end of educating my doctors rather than looking at them as the absolute authority for truth when it comes to this disease, but that is mostly because I have spent my life in this body and I have lived with this disease, I have seen it act in people in my family – most of the time watching doctors shrugging and not knowing where to find the answers. If you have just been diagnosed, you probably don’t have this perspective, so what I can tell you is: stay alert. Read, inform yourself, and yes, educate your doctor if you need to. It’s all part of your care.

I also wrote this blog for those doctors, medical professionals, medical journal writers who have told me in the past that “no, doctors don’t speak like this!” when I would quote doctors with some off-the-cuff statement that would render me more lost than when I first came to their offices. And I am telling you – yes, they do speak like this. To be in front of a clearly demonstrated cardiac and cardiovascular patient and to diagnose them with “garden-variety blood pressure” takes guts. And some doctors obviously go there …