Saturday, August 30, 2008

Fibromuscular Dysplasia

Well, it turns out I may not have had a kidney infarction after all. There had been continuous confusion amongst the many doctors involved as to whether I had an infarction (from a clot) or an infection (from unknown cause) in my right kidney. The key thing I understood at the beginning was that I had one small kidney artery that was completely blocked, killing 5% of that kidney, and another small artery right next to it that was partially blocked. The blockages were either from a clot of blood or cholesterol or from an infection. An angiogram showed no sign of an existing clot, so if that was the cause, it had dissolved by then. I was put on an antibiotic in case I had an infection, but that didn't change anything. The early prognosis was that I would be fine with such a small loss of kidney tissue, that I would take an anti-coagulant the rest of my life to ward off any more potential blood clots, continue to watch my cholesterol levels, and the pain should be gone in a few weeks.

Except the pain never left. It subsided for awhile, then got worse a while, then better, and so on, but never left. I was out of work that whole time, as the pain was generally more than enough to require medication that interfered with my ability to think, which is what I get paid for. Had I not taken the pain medication, the pain would have been too much to think, so either way, I wasn't much good for working. Last week the pain improved to the point that I thought was ready to resume working and I got a note from my kidney doctor allowing me to return. At that visit, my kidney doctor reviewed with me everything he knew from all the tests. That included something I hadn't been told about before: there were some partial blockages in the main artery feeding my right kidney, and it was determined (by their size, shape, location, etc.) that they were caused by Fibromuscular Dysplasia (FMD). The kidney doctor still didn't know what caused the full and partial blockages in the smaller arteries, as things didn't quite fit the normal characteristics of either infarction or infection.

Then I found out my company and the insurance company require a note from my general practitioner, so I had to wait until next week. Then a couple of nights ago, the pain got really bad again, and my blood pressure went high (around 160/110) and stayed there. Although the pain backed off a little, the BP stayed up, and my kidney doctor called in a prescription for Diovan to bring my BP down. After a day or so of that, my BP is back down. In the meantime, I've been studying FMD.

FMD is a rare disease. Really. There's actually a list of rare diseases, and FMD is one of them. And there's a support site on the Internet run by doctors and patients, and information from FMD doctors indicates that FMD is not well recognized or understood by doctors. Patient stories support the idea that most doctors are not familiar with it.

Basically FMD results in some cells in the walls of arteries growing larger than they should, constricting the flow of blood through the artery, eventually blocking it off altogether. No one knows what causes FMD, though genes and hormones both seems to be factors. There's no cure, but the symptoms can be treated with anti-coagulates and angioplasty. Most people who are familiar with angioplasty have heard of it because they know someone who had it to open up an artery around their heart. That common usage means someone gets an angioplasty once every few years or less often. Some people with FMD get angioplasty several times a year. FMD can effect any artery in the body, with the kidneys being the most common, and the carotid arteries being the second most common.

So, I've come up with a theory, which I wrote up and faxed to my kidney specialist yesterday. He called me late last night to tell me he had been in hospitals all day and had not had time to read the letter yet, so I'm hoping to hear from him next Tuesday. I have an appointment with my general practitioner on Wednesday, and I'll discuss this with her then. My theory is that I had neither an infarction nor an infection, but that both are the results of FMD. There is a form of FMD called intimal fibroplasia that comprises less than 10% of FMD cases, but it appears to me it fits perfectly with the full and partial blockages I have in my smaller kidney arteries. It seems to me to explain everything, including my continuing and fluctuating pain, and I really like understanding things. If I'm right, however, it's bad news, because this form of FMD is the most aggressive.

Well, as Bill Gates famously said, it's good when bad news travels fast. That's because the sooner you learn of something bad, the sooner you can do something about it. So, I have FMD. I may have the most aggressive kind. Since I know, my doctors and I can more aggressively monitor my arteries and intervene with angioplasty as often as necessary. And that could give me months or years more than I might have left if we hadn't figured it out.

Whenever I have more news, and feel like posting, without being to unintelligent due to pain medication, I'll post followups here.


megfrench said...

You'll be in my prayers on this John. I am so sorry you are having to go through this. I sure am glad you are such a researcher and can understand this. As Ron Burke says (quoting the Bible..."Grace, Grace to it!"
.....Blessings, Meg

Mimi said...

hi John:
I was googling FMD as I do often and came across your blog. Like you I have FMD in my kidneys. I have lost one kidney due to the disease, and another is in stage three kidney disease. I noted that you have visited a support group, probablly the yahoo group. I also wanted to pass on the website FMDSA.ORG. If you have not already visited this site, you will find it is extremely informative.
Not sure how far you are in your research on FMD, but I did want to share with you the importance of your spikes in B/P. As FMD can cause dissections, it is important not to have those high readings. If your B/P spikes high, there is a danger of dissecting your carotid arteries. The reason I share this with you, is having been diagnosed since 1996, I know how frustrating the lack of knowledge by local docs can be. It is important for you to get the most informed docs you can. If you contact FMDSA.ORG they will give you some doc's name in your area. I also wanted to let you know that arteriogram is the gold standard for diagnosing this disease, and I am hoping you have had one, so that you have a clear picture of the degree of your stenosis. Also you will want to have your carotids checked, they usually begin checking via ultrasound.
Okay, hope I don't sound to bossy, just want to help.

Take care
One Kidney Gal in Northern Cal
Take care.......

John said...

Hi Mimi, I've been to, and hadn't found the yahoo group, so I'll check that out also. High blood pressure has been a problem, and it looks like most blood pressure medications, along with many other kinds of meds, can't be used with compromised kidneys. My overall kidney function is still okay, but what do you use to control high BP?

I've had a angiogram that clearly shows FMD to the doctors, and I'm trying to learn to read the pictures myself. I've had an initial dopplar check of my carotid arteries, but Dr. Olin's lab is going to repeat, since most technicians don't know how to look for FMD correctly on the ultrasounds.

When you go on dialysis, try to get a home unit, results are much better overall than going to dialysis centers.

Hang in there, John

Anonymous said...

Hi John,
are you seeing Dr. Jeffrey Olin? If so you are in the best hands!
As far as B/p, well I have had it since I was 16 (now 51). My left kidney shrunk due to renals stenosi and started emitting a lot of renin which really raised by b/p. So I had to have it removed.
Thanks for the advise on dialysis, hopefully I will live long and the remaining one! But I do know that can change rapidly!
Take care........mimi

george ingraham said...

My girlfriend has been dealing with FMD for several years. Her whole body is plagued with artery beading/blockages.

Many surgeries.. Too many to list here.

She has tried several different pain medications with little or no success.

Her every day and night consists of so much pain her life is just miserable.

Last year a doctor put her on Methadone. It has been the only drug that has actually worked !

Apparently doctors are now being required to take patients off of it.

Her Doctor now has her back on pain medication that does not work.

Life is really extremely hard for her with so much pain.

Is there a pain medication out there for people with FMD !

She has more angio's scheduled.. Hopefully.... hopefully they will result in some relief..