Many of my OS friends have asked me about my medical condition which, because it has become such a change factor in my life, I often mention in my posts and comments, but never actually explain what is wrong. The last month I have had even more inquiries. This is a long post but that is unavoidable. It is primarily for those who have been concerned.
I just turned 70 years old and it is not uncommon for people my age to start having more medical issues than younger people. So, while some seem to have thought so, I have really not tried to hide my condition or anything like that. But some people are starting to guess what I have and perhaps to worry more about it simply because they don’t know what I have. That was never my intent. I come from a generation where medical issues are mostly kept to themselves.
So, while I do not wish to cause unnecessary concern, I also do not want to use my medical situation as a way to garner sympathy in public. Therefore, I have closed comments on this post. But please do not misunderstand my reason for doing this.
I welcome prayers and have no problem at all receiving private messages on the PM system here on in an email at my public email address that is printed at the end of this post. But there are many, many, people in the world, and some here on OS, who would gladly trade their medical troubles for mine. I am very aware of that and when I can I offer a word of comfort to them and always hold them in my prayers.
What I have is complex, and each part of it is rare and only partially understood. There is no magic pill cure for any of what I have, but pain can be somewhat relieved, the frequency of flare ups can be reduced by taking certain steps, and remissions are possible, but not common.
So, just like I had to retire from being a pastor before I wanted to, I have had to adjust to a radically different life style in general. That has not been easy and I still have a hard time adjusting to some things, most particularly cutting way back on motorcycle riding.
So, what is written below is where I stand as of now. I work with a doctor, a rheumatologist, at the Cleveland Clinic who coordinates my situation with other doctors there and with my primary care internist here.
There is no short way to describe my med condition, so I recognize this is a long post. It is very rare, especially as a syndrome of four things happening at once, but each one can be researched on Google individually, and some Google images are also available on each.
Here we go; this is essentially what I have sent to friends outside of OS that are concerned: I have four related rare problems that are presenting as a syndrome of all four occurring simultaneously. While there were some signs of these problems before, all of them got serious at once last March.
1. "Leukocytoclastic vasculitis" is also called "hypersensitivity" vasculitis. Vasculitis is irritation and inflammation of the blood vessels. This type is caused by an allergic reaction of my own body to some outside irritant. Over half the time the irritant is never identified but this type of vasculitis is usually caused by any number of drugs that the body reacts to adversely.
In my case we think, but cannot prove, that it was caused by an antibiotic or two antibiotics I was on simultaneously last March. In any case, this vasculitis involves the very smallest blood vessels and often presents by bleeding into the skin, causing small bleeds that look like red dots of 1 to 4 mm, and larger ones that look like bruises. The small bleeds are called petichiae and the larger ones are called purpura. This has been diagnosed by three biopsies.
The red spots started all over my body for about two months. They would clear and then something would trigger another round of them. But now the vasculitis is confined to my feet and shows up only when I have a flare up of erythromelalgia, which is described below. I am getting fewer and fewer of the bleeds as time goes on. I still get some micro red spots (less than 1 mm) on my feet, but not like before.
Since late summer I have had a different form of the same vasculitis, called nodular vasculitis. Bumps, or nodes, appear, mostly on my toes, that are red, elevated, up to about 4 mm in diameter. At first they were very bad and had blood trapped in them and were not just on the top of the toes but on the sides and bottom, making it hard to walk, and some were very painful that involved messing up a nerve. I am on prednisone for this.
Mostly now they are confined to the tops of the toes, with no trapped blood and only a few new ones. But the old ones seem to take months to go away, though; and can flare up with each new flare up of the ethromelalgia. Working with my doctor, I have been reducing the amount of prednisone lately but think I may have gone too low and may have to increase it again. I hate taking prednisone but it does help keep the nodes from being inflamed all the time.
2. "Small fiber neuropathy" is a problem with the very smallest nerves in your body. It often is confined to the feet, as mine is. I have had it since 2003 and it has gotten a little worse each year. Symptoms are numbness, tingling, burning, stinging, shooting electrical shock type pain, and “banding,” a feeling like your feet are wrapped tightly with tape or Saran wrap. In spite of the overall numbness, the surface of my feet are ultra sensitive to pain, which is also a common trait of this type of neuropathy.
This form of neuropathy usually manifests itself in diabetics, but I am not diabetic. Small fiber neuropathy is diagnosed by an EMG test, which was the most painful test I have ever endured in my life. It takes over 2 hours. They first place two electrodes on you and then run shocks between the electrodes, increasing the level in each area they test. After that the doctor administering the test sticks needle probes into your muscles and then runs electrical currents between the probe and a ground electrode elsewhere on your body to see what you can feel and what not, and how your muscles react, increasing the voltage as they test each muscle. Never again will I take that test.
And, ironically, the way they diagnosis small fiber neuropathy is because the EMG test, which checks for large fiber neuropathy, comes back "normal." The "diagnosis" of my neuropathy is by exclusion. I could have told them that before I endured the test.
3. The most debilitating and painful thing I have is "Erythromelalgia." This is the sporadic reddening, bright beet red, and swelling of blood vessels and feet or lower limbs, and/or sometimes the hands, the ears and face can be involved. I have some involvement of my hands and wrists, and my face and ears, but not often and not worth worrying about yet.
My feet get bright red and swell, about once a day, usually over night and essentially every night, like clockwork. It can be very painful. And I am hopefully asleep having taken some strong pain pills before the flare up starts. If not, I am up the rest of the night. The feet get red because the blood vessels dilate and over 10 times the amount of blood flows through the vessels of the feet than normal, but, the blood is not oxegonated so it does not feed the cells like it should. It’s hard to believe but the blood flow and lack of oxegon have been measured in tests on typical erythromelalgia patients. When this happens my feet feel like the nerves are setting off needle pricks and stinging electrical shocks, and the feet sting, burn, get very hot, red, and extremely sensitive to the touch. IE: they hurt like hell, and it is hard to describe the pain. It is next to impossible, but not quite, to walk during a flare.
When I get an overnight flare the actual flare up goes away after two or three hours but the feet stay very red and swollen until I get up and get them elevated and blow a fan on them. Why this happens and they don’t just go back to normal since my feet are at the level of the heart at night, nobody knows.
I usually get up around 10 am. So once I get them elevated with a fan on them they will then cool down and the blood will flow out of them in a couple of hours. Until then I can hardly walk from the pain. By mid afternoon much of the pain and swelling is gone, so the best time of day is from then until I go to bed, and that is one of the reasons I stay up so late, until 2 or 3 am, so I can have time each day with little pain.
Heat triggers a flare up, as does any extensive exercise including walking; as does leaving them down in what docs call "a dependent position." For example, sitting at a table with my feet on the floor. Day time flares, that in my case last from 2 to 3 hours even when elevated and with a fan blowing on them, can be resolved by putting the feet up to level with the hips or higher and cooling them. Putting them above the level of my heart helps drain the blood quicker so I usually do that first.
I sit in a LazyBoy almost constantly with the laptop computer, and the TV and my stereo system in front of me. I prop up my feet and have a fan blowing on them much of the time, even when they are not flaring. I go barefoot in the house. We keep the house at about 68 degrees because cooler temps reduce the odds of a flare.
It is better for me now because it is cold outside, the house is cooler and I can go out on the porch bare footed for a few minutes to cool them if they get really bad. That sounds strange when the temp is below freezing but it is a whole lot better than just doing nothing when a flare happens. I have had only one day flare in over 2 months by living like this. But this way of living is a radical change in life style.
In the summer I really can't spend more than minutes outside when the temp is over 80 degrees or that alone will trigger a flare up. I can ride my motorcycle for up to two hours when the temp is between 40 and 65, but for no more than an hour when it is between 65 and 80 without causing a flare up. If the temp is over 80 my feet will flare in less than an hour on the motorcycle.
The general effect of this is that I hesitate to go anywhere, like out to dinner or to a movie, because the odds are that the cost of doing that will be a painful flare up and it just isn't worth it.
4. "Renaud's Phenomena" is the exact opposite of erythromelalgia. The blood vessels in the feet constrict to the point where they become ice cold. My feet are usually either ice cold and very pale and white or purplish looking or they are red and hot. There is no middle ground. There is no normal. Or I guess this is my "new normal."
Since I have the neuropathy and don't feel all that much in my feet anyway except at the skin surface and during a hot flare, I really don't mind the cold feet part of the problem. In fact when my feet are cold I feel pretty good knowing that a flare up is less likely to occur.
Sometimes however, even when they are cold you can actually feel and watch a flare start, in the heel or arch or a toe and within a minute that part will get hot and then you can watch it spread, first to the one foot, maybe from toe to toe, or from arch to toes, but inevitably some signal will also start the other foot to flaring. It is weird and if I hadn't watched it happen many times, even trying to stop it from spreading to both feet, I would not believe it could happen like that. Most of the time it is impossible to stop the hot flare even using an ice pack. Sometimes if I feel and see it starting I can delay it for an hour or two, but as soon as I remove the ice it starts back again in the same place.
So, my body has lost its ability to control the blood vessels in my feet and to a far lesser extent my hands. My hands just get red, swell a little, but never hurt, and that is not often enough to worry about. Flares in the hands will come and go on their own after a couple of hours. I occasionally will get a red flushing of my face and ears which feel very hot but that goes away on its own also.
The doctor at the Cleveland Clinic is trying different drugs. So far two of the drugs have had a helpful effect on reducing the pain, for which I am grateful, but those new pills have affected my liver enzymes and I now have enzymes that are a bit higher than normal. The pills appear to have been the tipping point for the higher than normal enzyme lever because I always have had normal enzyme levels when tested twice a year which they do when you take blood pressure meds and lipid lowering meds like I have for years. In fact, when I first went to the Clinic my blood test indicated some of the best enzyme readings I had ever had.
But, when my enzyme levels went up the Doc at the Cleveland Clinic had me get a liver ultrasound which shows fatty liver deposits, which is quite common for overweight older people like me.
Even though the higher than normal liver enzymes started only after I started on the new drugs, the Cleveland Clinic doc I go to, who is a rheumatologist, wants me to see a liver specialist up there anyway. I haven't decided on that and want to talk to my primary care internist before I do it. I am convinced that the drugs were the tipping point and do not want to go through the pain of a liver biopsy, which the rheumatologist mentioned I may need, and which is not without complications itself. Fatty liver is common in my age group and usually amounts to nothing serious.
But I have a feeling that in a world renowned research hospital like the Cleveland Clinic their answer to everything is to do test after test after test, even if they don't expect to find anything more than they already know. And even if a biopsy were to confirm the type of fatty liver I have the treatment is identical for all types of fatty liver, with is simply to remove drugs that elevate enzymes, slowly lose weight, reduce triglycerides, and watch what you eat. There is no “cure” for it but it can be stopped from progressing and even can be reversed somewhat with lifestyle changes.
So that is where this whole mess is right now. As one who has been blessed until the past year with pretty good health, or controllable issues like high blood pressure, high triglycerides, etc. it is hard to change gears so radically. Giving up motorcycle touring or even long day trips has been very hard on me, a guy that has over half a million miles on motorcycles and has been riding for 55 years.
I can use your prayers and invite private coorespondence from you by PM here on this system or by email at firstname.lastname@example.org .
I thank each of you who have expressed concerns and will be glad to hear privately from anyone.
Blessings and Peace to each of you.