According to the Merck Manual “Eosinophilic Fasciitis is a disorder in which the skin of the arms and legs becomes painfully inflamed and swollen and gradually hardens.” Following is my story and how a 53 year old female became acquainted with this extremely rare disease.
In February, 2004, a blood test showed I was moderately hyperthyroid. My PCP ordered a thyroid scan which showed a toxic multi-nodular goiter. I was advised to seek treatment since hyperthyroidism was very hard on my heart and the best treatment was to simply swallow a radioactive iodine pill. Despite sounding scary, the radioactive iodine would only be absorbed by my thyroid gland with the desired result of destroying it. Then I would take Synthroid the rest of my life to replace the hormone produced by my thyroid. Other options included anti-thyroid medication or surgery to remove my thyroid but the radioactive iodine pill produced fewer side effects and was less risky than surgery. I researched the Internet and found that I-131 Radioactive Iodine Therapy was the cheapest, the fastest and the best treatment of hyperthyroidism.
On March 5, 2004, I had an appointment at the hospital to receive my pill. The technician gave me a sheet of warnings to heed for the next few days - don’t sleep with anyone, stay away from small children, use separate dishes and silverware and flush the toilet several times after using. I was also advised to carry a letter with me for the next 30 days that stated the date I received the pill, how much I was given and who to call with questions. It seems with today’s sophisticated equipment it was possible for me to set off alarms in airplanes, federal buildings and some tunnels, but I was assured it was perfectly safe for me to swallow the pill. I asked the attending physician if there were any side effects I needed to watch for. He said there would be no side effects, although the sheet given to me did state there might be some nausea and tenderness in the neck area. Then they brought in the small lead cylinder. As the technician and doctor watched, I was instructed to remove the container inside and just tip it up and take the 29 milliculeter capsule inside. Thus began a year long roller coaster ride for both my family and me.
I didn’t really feel any different for the first two weeks. Then I started having pain in my calves, thighs, forearms and upperarms when I moved them. I also noticed stiffness when I got up from a chair but it went away quickly as I walked around. I went back to the Internet and did a little more research and discovered it wasn’t uncommon to have some pain after the I-131 Therapy as large amounts of stored hormone were released from the thyroid. So I didn’t pay much attention to the pain but mentioned it to my PCP on my first visit after the treatment. He tested my thyroid levels but said nothing had changed so far.
By the middle of April my lower legs and forearms were swelling and the pain and stiffness in my knees continued. I was surprised one day when I went to kneel in the garden and almost landed on my face. It felt like I had lumps in my knees and the skin on my legs wouldn’t stretch far enough to allow me to kneel. And I was having trouble getting my arms over my head to use my curling iron or to shampoo my hair. I didn’t have pain while I was sitting or laying still, only when I moved my limbs. The swelling in my legs didn’t seem to go down at night either. You could press on my calves and leave an indentation with your fingers and the elastic in my socks left a deep ring around my legs. I jokingly referred to myself as "Sponge Barb". I called my PCP about the swelling and he checked my thyroid levels again and said the bottom had fallen out. He assumed that was why my legs were swelling, and he started me on Synthroid in early May; 25 mcg first week, 50 mcg second week, and 75 mcg the third week. Then I was to come back in 6 weeks for the levels to be checked again.
The swelling, stiffness and pain didn’t get any better, though. In fact, it was gradually getting worse. I had never had problems with my knees before but they hurt now when I tried to bend them. The muscles in my arms and legs hurt even when I rolled over in bed. It wasn’t excruciating pain, but it hurt. And the skin was very tight on both my arms and legs. It felt like my skin just wouldn’t stretch anymore. It was hard to wash my lower legs and feet in the shower because I couldn’t bend them to get at them. In the mornings my calves were so stiff and painful I sort of shuffled out to the kitchen. I had difficulty walking down stairs, too. I had to take the steps one at a time. Sometimes I stubbed my toe going up the stairs and I would get a shooting pain up my leg. My PCP ran more blood tests including a test for lyme disease. The lyme disease was negative but my protein level was low and my Eosinophil level was very high. He did a 24 hour urinalysis. The urinalysis was fine. My Eosinophil level, however, was elevated to 40%; 3% is normal. He thought I might be having some type of allergic reaction or serum sickness. He prescribed a week of Zyrtec. It didn’t seem to make any difference. On May 28, he gave me an 8 day course of Prednisone; 40 mg for 2 days, 30 mg for 2 days, 20 mg for 2 days and 10 mg for 2 days. The swelling went down immediately and I lost about 10 pounds the first couple days. The pain went away but the stiffness didn’t. The Prednisone gave me a real buzz. The first couple nights I hardly slept and I was busy doing everything I had neglected while I wasn’t feeling well, but by the time I was down to 10 mg the pain started coming back again. I rolled over in bed one night and noticed the pain was back. However, blood tests showed my Eosinophil level went down while I was taking the Prednisone so we thought maybe it did the trick.
Several days after ending the Prednisone therapy the swelling started coming back along with more pain. I quickly gained 10-12 pounds. I had an inflamed band about 3 inches wide around my lower legs. My knees and ankles were very stiff. I couldn’t point my toes and it was hard to bend my ankle when I put my shoes on. My wrists felt stiff and I was starting to have trouble opening my hands all the way. The skin on my forearms and lower legs was so tight it seemed like it couldn’t stretch any further. My armpits were sunken and I couldn’t raise my arms very high or bend my arms behind me. I had pain down my left side making it difficult to stand up straight. I took a couple ibuprofen in the morning and 1 or 2 at night and that took most of the pain away, but not the stiffness. And I was starting to feel tired and kind of sick, like I had a mild case of flu.
My PCP was stumped as to what was wrong with me but he wanted to send me to a rheumatologist and keep me on a low dose of Prednisone until I could get in. I agreed to see a rheumatologist but I declined the Prednisone. I knew Prednisone had a lot of bad side effects. Anyway, I thought I needed to see an endocrinologist not a rheumatologist. I was sure once my thyroid levels were straightened out all my problems would go away. Once more I had been spending my early morning’s doing research on the Internet and reading about different myopathy’s and neuropathy’s associated with I-131 Therapy. My sister-in-law had a neighbor who had the same treatment and went through similar pain in her limbs but did not have the associated elevated Eosinophil count. After she saw an endocrinologist and her thyroid levels were straightened out, she felt better. My PCP didn't think I needed to see an endocrinologist, but finally he agreed to make me an appointment with one. But the earliest appointment wasn't until the end of September.
June was not a good month. By now I was feeling sick at the end of each day and ran a very low grade fever of 99 - 100. My legs were swollen and sore all the time so I elevated them in my recliner with pillows underneath to feel more comfortable. I slept with soft pillows under them at night and my arms extended with pillows under them, too. If I bent my arms or legs at night it was painful to straighten them out when I woke up. I would go to bed freezing then wake up a few hours later sweating. I couldn’t cross my legs without pain and it hurt along the shin bone and calf muscle. I cut telephone conversations short because it hurt to keep my arm bent. I took ibuprofen and that helped the pain but the swelling and stiffness was always there. Then my hair started falling out. Every time I took a shower there would be a big wad in the drain. I started to think maybe there was more going on than just getting my thyroid levels straightened out.
My PCP wanted me to quit taking ibuprofen because he thought that might be causing the swelling. He gave me Tramadol to take with 2 Extra Strength Tylenol. I took it in the morning and then again at night. I felt soooo good when I took them. I had never taken any kind of pain pill before.
I continued with my early morning research and discovered something called Eosinophilia Myalgia. A group of people had gotten this from a contaminated batch of L-Tryptophan, a diet pill purchased from a health food store. Wow, their symptoms were sure similar to mine. I even mentioned this to the nurse when she made her weekly call with blood test results. And I bought a Merck Manual to supplement my research. Lupus looked like it could be a possibility. I mentioned it to my PCP. He did an ANA(Antinucleur antibody) test along with another CBC. My Eosinophils were back up to 37% and the ANA test was positive. Since the ANA was positive, follow-up tests were being completed but it would take several days to get the results. These tests turned out to be negative. But this time my PCP called instead of the nurse and talked to me on the phone. He said he wanted me to see an Oncologist-Hematologist, too. He mentioned Myelodysplasia Syndrome and Lymphoma and said I might have to have a bone marrow test. I said okay and hung up the phone and cried.
Things started happening quickly. My appointment with the hematologist was a week away on Monday and the rheumatologist called and said they had a cancellation and could get me in on the Friday before the hematologist visit.
The rheumatologist had a resident do my initial exam. I had everything written on a piece of paper so I could remember the progression of events. I tried to tell her everything. She was so gentle when she touched my arms and legs. After she examined me she said she would confer with the rheumatologist and then they would both be in. When the rheumatologist came in he examined my arms and legs and pointed to the inflamed band around the bottom my legs and said that was the orange peel effect. I had read about that in conjunction with an autoimmune disease but couldn’t remember which one. Then he said my arms and legs felt “woody”. He mentioned Eosinophilic Fasciitis. I recognized that name from my research but I couldn’t remember the particulars. I had read about so many diseases. They looked at my blood tests. The ANA was positive but the ESR (erythrocyte sedimentation rate) detecting inflammation wasn’t elevated. The resident asked if that would have to be elevated for an EF diagnosis. The rheumatologist said he wasn’t sure but he would investigate. He didn’t think I had Scleroderma because my fingers and toes weren’t involved. He said he had only seen one case of Eosinophilic Fasciitis and if that wasn’t what I had then it would take a group of specialists to figure out what I did have. I said I was seeing an Oncologist-Hematologist on Monday and he said that was good. I also told him about the appointment with the Endocrinologist. He said that was good, too. He said I needed a full skin biopsy and I needed to have it done as soon as possible. He also said the biopsy had to be sent to a good laboratory. Thankfully, my insurance paid for a good laboratory. I asked him about the prognosis for EF. He didn't want to talk about that until after the biopsy. He also said we needed to start aggressive treatment right away - 60 mg of Prednisone a day.
I went home and looked up EF (Eosinophilic Fasciitis) on the Internet and in the Merck Manual. I found it was an autoimmune disease possibly related to Scleroderma. It said 60 mg Prednisone was usually prescribed for a couple of months; then gradually dropped to 10 mg for 2-5 years. There were very few documented cases. It said the inflammation eventually went away but most people were left with the scars and contractures it caused. Sometimes limbs stiffened in odd positions. Rarely internal organs were involved. Prognosis was poor if bone marrow was involved. Myelodysplasia and aplastic anemia were possible complications.
I started 60 mg Prednisone on June 25, 2004. My legs felt better after the first dose. The swelling started going down immediately. Once again I lost about 12 pounds in 3 days. I waited for the buzz the Prednisone gave me the first time I took it, but the buzz never came. At night I only slept about an hour or so at a time and then I had to get up and go to the bathroom. I guess it was from the Prednisone. I would watch television or listen to the radio until I fell asleep again. I probably only got 4 hours of sleep each night. I started feeling physically tired and extremely weak after about a week on the Prednisone. By the 10th day I was so tired and weak it scared me. I had never been that weak before. I called the rheumatologist. He said as long as the swelling was down I could drop the Prednisone to 40 mg a day. I remained on 40 mg a day for the next two months.
On Monday I saw the Oncologist-Hematologist. I told him my whole story. I asked him if he had ever seen EF before. He said he had seen it once and that was when he was at the Mayo Clinic. He didn’t want to offer a prognosis. He looked at my legs and said he couldn’t see any of the orange peel effect. He called my rheumatologist and conferred with him while he was still in the room with me. He told him I must have had a quick response to the Prednisone because he didn’t see the orange peel effect on my legs anymore. My rheumatologist told him I looked like a classic case of EF. The hematologist told me he thought my rheumatologist had "hit the nail on the head" with EF so he didn’t proceed with a bone marrow test at this time. He said he would close my file until such time as my rheumatologist called him.
My daughter did some research through the university she was attending and found some cases where patients with EF were getting good results with Cimetidine (Tagamet) along with Prednisone. I showed the information to my rheumatologist and he said he didn’t have a problem with me taking it. He started me on 800 mg a day. He also prescribed 70 mg of Fosomax once a week because Prednisone depletes calcium from your bones very quickly.
I saw the surgeon in early July for the biopsy. I asked him if he had ever seen a case of EF before. He said he had heard of it but had not seen a case. My rheumatologist had requested a biopsy of my arm and ankle area. The surgeon said he didn’t want to do a biopsy around my ankle because it wouldn’t heal very well. After I started the Prednisone and the swelling went down, my arms looked pitted with grooves or “furrowing” along the veins in the upper arm area. There were also little lumps all over my arms. The surgeon picked one of these lumps on my left forearm and marked it. He deadened the area, took several deep samples and stitched me up. He said he would notify the lab right away that the samples were coming. It took about a week after the biopsy for the site to quit oozing. My arm remained numb from the incision site down to my wrist for the rest of the summer. It didn’t really heal nicely until a couple months later when my Prednisone level was down to 30 mg.
The biopsy confirmed EF. My rheumatologist said the disease was so rare there was very little information on it. He offered to print some information from the Internet but I said I had already read everything I could find. He told me it rarely affects the internal organs and 75% of people end up with permanent contractures. He said he couldn’t find any research studies going on but if I found any to let him know. He also said if my Eosinophil level didn’t drop to the normal range I would have to have the bone marrow test. Thankfully, it dropped to 3% and has stayed there for over a year.
I was able to get the endocrinologist appointment moved up to July. He also had a resident do my initial exam. I told my story again. I took the pictures of my thyroid scan and the resident looked at the scan and the stack of test results faxed to his office from my PCP and said, “Wow!” He read through them and then went out to confer with the endocrinologist. They both came back in and we went over everything again. The endocrinologist said he wasn't confident of the original diagnosis of Toxic Multi-Nodular Goiter because my initial blood tests for hyperthyroidism looked more typical of Graves' Disease. He did say the I-131 Iodine Treatment would have been the same with either Graves' Disease or Toxic Multi-Nodular Goiter. So he ran a blood test for Graves' Disease and another TSH level. The test for Graves' Disease turned out to be negative. I asked him if he had ever seen anyone have my problems after I-131 Therapy. He said not exactly. People who had problems with joint and muscle pain afterwards generally were a lot more hyperthyroid to begin with than my test results showed. And he said the pain and stiffness I was describing was not typical of what he had seen. He said the amount of Synthroid I was receiving appeared to be appropriate and he wouldn’t change anything at this time. He said he was sorry but he could not really shed any new light on my situation and would close my file.
Most of July and August I spent on 40 mg Prednisone. I still had a fair amount of pain and stiffness through the summer. My knees were the worst. It was difficult to bend my knees or to stand in one place. The skin on my arms and legs was still very tight and the skin on the top of my shins was very shiny and had a waxy feel. The hair on my legs grew very slowly and hardly at all on my shins. Sometimes my legs were a splotchy red or purple and sometimes they looked normal. My arms were hard as a rock, especially after I tried to do anything physical. Sometimes it felt like they were being “shrink wrapped”. The tightness extended down my left side and it was hard to stand straight. I had problems walking for any length of time. A trip to the grocery store was about all I could handle. I pushed the cart because I could support myself on it. When we came home I would lay down and rest and my husband would put the groceries away. I also got cramps in my fingers. I liked to sew and play the piano but the cramps made it difficult.
My Eosinophil levels remained low throughout the summer so in August my rheumatologist lowered my Prednisone to 30 mg. I was to stay at that level for 2 weeks then drop to 20 mg.
30 mg went okay so I dropped to 20 mg two weeks later. After about 3 days I started having a lot of pain in my legs and arms. They didn’t swell but I had a lot of pain. My rheumatologist thought maybe he had dropped the level too fast so he wanted me to go back up to 30mg again for 2 weeks then drop to 25 mg. It went okay this time. I started to gain more strength and had a little less stiffness. I started mowing the grass again...slowly, and did housework without getting hand cramps. And I started sleeping better at night. I also started gaining weight again.
My brother and sister-in-law came for a visit in September. We visited his old college campus and did a lot of walking. It was easier to handle the walking now even though I walked slowly. It seemed like my muscles locked up if I tried to go too fast. But I was feeling a lot better.
The first of October I started having mild diarrhea. I figured it was just from the medications. My rheumatologist thought I might be getting lactose intolerant because of the extra dairy products I was consuming. I gave up milk, cheese and yogurt for about 5 days but didn’t see any difference. I didn’t really pay a lot of attention to the diarrhea because it wasn’t that much of a bother and I was starting to feel so much better.
I was doing stretching exercises everyday and I started seeing a physical therapist. He gave me some specific exercises for my knees and arms. They were simple exercises but they made a big difference. My knees improved a lot and so did my arms.
All in all, everything was starting to go much better. Then I remember a conversation that went something like this:
“Barbara, do you know where you are?”
“No.”
“You’re in the hospital. Do you know how you got here?”
“No.”
“You were brought here by ambulance. Do you know why you’re here?”
“No.”
“You had a seizure. Do you hurt anywhere?”
“No.
I don’t remember being upset about the conversation. I must have drifted off to sleep again. I had a similar conversation a couple more times with hospital personnel. The second time I was asked this set of questions I answered with, “Somebody said I....”
I guess I had gotten out of bed about 3:00 AM on Tuesday, October 19, and had a grand mal seizure. My husband found me on the bedroom floor bleeding profusely from my nose. He didn’t know it at the time but I had broken my nose and bitten my tongue.
My husband was with me in the emergency room. He said I talked to him and all the hospital personnel. I said I felt fine but I was confused about what time it was. I remember very little of it. Then about 8:00 AM, while still in the emergency room, he said we were talking and I started having another seizure. I vaguely remember that one. I felt everything get very fuzzy around my head. I remember a nurse asking me, “Do you remember anything? Did you see an aura?” After that seizure they started me on Dilantin. My husband said I went through a full day of blood tests, CT Scan, MRI, EEG, etc. I don’t remember any of it. I was in the hospital for three days but only remember brief moments of time. I know my son was there but I don’t really remember talking to him. I remember seeing doctors but recall very little of what they said. My husband said the neurologist couldn’t find a reason for the seizures, which I guess happens in a lot of new onset seizure cases.
After I got home and my husband started telling me what had happened, I couldn’t believe I had no recollection of it. It still bothers me that I don't remember the ambulance trip, any of the tests at the hospital and very little of my conversations with family or hospital personnel. I must have slept for most of 3 days. It was unsettling when I saw the blood stain still on the carpet of my bedroom floor from my broken nose. I was so thankful the seizure hadn’t happened while I was driving. What if I had hurt someone else? And I was very thankful for my husband and family, the rescue squad, the doctors and everyone in the hospital that took care of me.
I saw my PCP on a follow-up visit a week after the seizure. By then I had a rash on my stomach and arms. And I had a dry cough and a tender mouth. He said I should call my neurologist because a lot of people get a rash from Dilantin. I called and he took me off of the Dilantin right away and gave me Carbamazepine, 1000 mg’s a day. I took 2 pills in the morning, 1 at midday and 2 at night. At first I would occasionally lose my balance but that went away after a couple weeks. But the 2 pills in the morning made me feel very drowsy. I had trouble even thinking straight until about midday. I constantly battled fatigue and usually took 1 or 2 short naps a day. I had to quit taking the Cimetidine because it wasn’t compatible with Carbamazepine. I wasn’t to drive, swim alone or climb ladders for six months. The neurologist told me not to get overly tired and to stay away from caffeine since those two things can trigger seizures. And I wasn’t to drink any alcohol. That wasn’t a problem for me since I don’t drink anyway. I was to have a follow-up EEG in six months and if that one looked okay he said we might talk about reducing the medicine.
I was a little worried that after spending three days in bed I would be very stiff and sore. But actually it was just the opposite. My arms and legs felt pretty good. And I didn’t notice any difference in the EF after discontinuing the Cimetidine.
I read what the Merck Manual had to say about seizures but didn’t do a lot of research on the Internet. It gets pretty complicated when you’re talking about your brain and I was tired of looking things up. My rheumatologist said he couldn’t find any information that linked seizures with EF. Since then, I found one entry on PubMed where a woman had a grand mal seizure at the same time as she was being diagnosed with EF. I’m happy I didn’t have a stroke or a heart attack or they didn’t find a brain tumor but at the same time it’s disturbing when you don’t know what caused the seizures.
At first after the seizures my husband was afraid to let me out of his sight. The first few nights he held onto my nightgown so I wouldn’t get out of bed without him knowing it. During the day we stuck together like glue. It was scary for him because he saw what happened to me and it was scary for me because I had no memory of anything happening. Gradually we both got used to going places again and I didn’t think about it all the time.
My diarrhea went away while I was in the hospital but came back the first week of December. My PCP sent me to a GI doctor. I had a Colonoscopy with biopsies, Upper Endoscopy with biopsies, and blood tests for Celiac disease and Lactose Intolerance. Everything was okay except one of the biopsies of the colon showed mild Lymphocytic Colitis. He prescribed Asacol. That made it worse. Metronidazole, an antibiotic, helped for awhile, but then it came back. Finally he said to try 2 tablets of Metamucil everyday. That helped more than anything, although I still have the problem off and on. Altogether, I had diarrhea for nine months before getting relief from the Metamucil. I'm not sure why he didn't have me try the Metamucil first rather than prescription drugs, but it's too late to worry about that now. My rheumatologist said his research showed EF didn’t affect the intestinal tract. That was good to know. I lost about 10 pounds after the diarrhea started and have stayed at that weight ever since. Thank goodness for elastic and spandex in clothes. I began to lose track how many times my weight went up and down.
Starting in December my PCP started raising my synthroid levels. First he went to 88 mcg from 75 mcg. Six weeks later he raised it to 100 mcg and six weeks after that raised it to 112 mcg. Once I reached 100 mcg I started noticing a big improvement in how I felt mentally as well as physically. I had more energy and my muscles started feeling better. I don’t know if it was from the increase in Synthroid, the decrease in Prednisone or the improvement in my EF. Probably a combination of all three. And my hair stopped falling out sometime in December. I am currently at 125 mcg of synthroid.
Just to add a little more interest to the scenario my cholesterol levels doubled over the past year. My LDL went as high as 235 from 111 a year ago and my total cholesterol went to 320 from 167 a year ago. Uggh. More medication. I started on 10 mg Zetia the first month. The LDL went down to 186. Then my PCP added 10 mg Pravacal with the 10 mg Zetia. I’m going to continue with this medication until December, 2005 when I will be re-evaluated again. Hopefully things will all even out soon.
I didn’t have any seizures from October 20, 2004 through April 1, 2005. I had my follow-up EEG on April Fool’s Day. Since it was a sleep deprived EEG, I didn’t nap the day before and I stayed up until 12:30 AM that night. My daughter came home to help me stay awake since I was usually out like a light by 8:30 PM. Unlike when I was on 40 mg Prednisone, the Carbamazepine made me sleep up to 10 hours a night. We colored her hair and generally goofed around to pass the time. She brought an air horn and tooted it if I started to drift off. Since I was only supposed to get 3 hours of sleep, I went to sleep at 12:30 AM and awoke at 3:30 AM. I was to be at the hospital at 6:45 AM. The technician went over my history of illnesses and medications. My husband and I always kept a list with us because it had become a sizable list. When I told her I had Eosinophilic Fasciitis she said, “You have what? Can you spell that for me?” I was used to that question so I closed my eyes and sounded out the words in my head and spelled it. Then they measured my head and hooked me up to all the electrodes. They started by having me open my eyes and shut my eyes several times. Then they had me close my eyes and flashed a series of lights at me and had me hyperventilate for 3 minutes. Afterwards they said, “Okay, just fall asleep for us now.” If I was at home I would have been out in a few minutes but I could not fall asleep for the test.
Unfortunately, I did not get a passing grade on the EEG; bilateral independent temporal slowing and bilateral independent temporal epileptiform abnormalities. My neurologist said the results weren’t really wrong but they weren’t right either. He said they could be more specific if I had fallen asleep. So I am continuing with the Carbamazepine and have another EEG scheduled for September 1, 2005. But he did drop the morning dose of pills from 2 to 1. That has helped a lot to eliminate my fatigue during the day.
My rheumatologist continued to reduce my Prednisone levels until March, 2005 when I reached 5 mg a day. He said he wasn’t in a hurry to go lower since I was at a very low level and I was making good improvement. The furrowing in my upper arms has disappeared and most of the pitting isn’t visible anymore. My skin has softened quite a bit and I no longer feel like I’m being squeezed to death. I still have some stiffness in the ankle, knee, wrist and elbow joints but I can get into the bathtub, fasten my own necklace and lift my beautiful granddaughter. And I can cross my legs now without pain; something I couldn't do for a year. I have continued with the stretching exercises and started doing some light aerobic exercise. I can walk fairly briskly and can stand from sitting without groaning. My hair has grown back and I now have a whole head of curls instead of straight hair. What a pleasant side effect! As of May 19, 2005 I started alternate day therapy with 5 mg of Prednisone. My rheumatologist said our goal this year is to get completely off the Prednisone.
In late May I caught a mild cold that progressed into a pretty decent cough. After a few weeks, the cough tapered off only to return again a few weeks later. I didn't run a fever and didn't feel sick, but just to be on the safe side I visited my PCP. He said everything in my chest sounded okay but with my history he decided to do a chest X-ray just to make sure there wasn't anything going on. The chest X-ray was normal. My cough never really went away and since May I have continued to have an intermittent dry cough until September.
The results of the EEG on September 1 were better than the April test but still considered abnormal. This time there was persistent theta slowing focally in the left temporal lobe. My neurologist said he didn't see the same type of slowing on this EEG that was on the previous one, though, so he asked me how I felt about discontinuing the Carbamazepine. I told him that as much as I would like to stop taking it, I did feel a certain sense of security while on it. He said it was good that I hadn't had a seizure in the past year but it would be even better if I went another year seizure free. He dropped my dose to one pill in the morning, afternoon and evening and said if nothing happened in the next year he would discontinue it completely.
In August my rheumatologist felt I was in a remission with the EF. He said my blood levels have been normal for a year and there has been no signs of inflammation. My skin has softened and doesn't show the pitting and furrowing anymore. I am continuing with alternate day therapy with 5 mg Prednisone. I have developed a blueish red, shiny spot on my right hand between my thumb and first finger. There is also a red rash that has developed along my knuckles on both hands and extends a little down my fingers. I showed it to my rheumatologist but he didn't really say much about it. He stretched my next visit out until December.
In December my PCP did another cholesterol check. My LDL was down to 162. Although it is still high he said he would be satisfied to see it below 160 since my HDL is 67. He told me to continue with the Zetia and Pravacol and to watch my diet and have it check in another 6 months.
I also saw my rheumatologist in December. The purplish spot on my hand had grown from about a quarter size to about a 2 inch diameter. He was also concerned about obvious Raynaud's Phenomenon in my hands. There was no swelling of my fingers or hardness of my skin, however. And my blood tests remained fine. He mentioned possibly sending me to a dermatologist since they handle the skin portion of auto-immune diseases. He was concerned I might be developing scleroderma. He said we might have to treat the Raynaud's but in the meantime to make sure I kept my hands as warm as possible and didn't get frostbite. He gave me a brochure to read about it. He also said I could quit taking the prednisone now but advised I wait until after the holiday season just in case something went wrong. He made my next appointment for February 28, 2006.
As of January 2, 2006 I quit taking prednisone. I feel fine and have noticed no changes. I still have tightness in my ankles and knees but I'm not sure that will ever go away. Sometimes I have pain in my left leg joint where it connects to my hip and in both elbows extending down towards my wrist. But I'm not complaining. I would say my flexibility is about 90 - 95% of what it was before I contracted EF. I do stretching exercises and walk as much as I can. I also have a very dry mouth (something I think I forgot to mention until this point). I have pain along the sides of my tongue and the inside of my cheeks a lot, too. Some days are better than others. If it's really bad I just take a Tylenol. My weight has stabilized and I am about 12-14 pounds lighter than before EF. I have had 3 colds in the last 8 months, something that is unusual for me. All 3 developed into prolonged coughs. I coughed for about 5 months with the first cold and 3-4 weeks with the other two colds. I think I must develope bronchitis.
I saw my PCP in February. It was really cold in his office and my fingers turned white and spotchy red with the Raynaud's. He wanted to put me on Procardia XL 30 mg since it would be good for the Raynaud's. He said the Atenolol I was taking for high blood pressure was just making it worse. He also wanted to switch my cholesterol medication to Zetia and Zocor. I asked him if Zocor was simvastatin and he said he thought it was. I didn't want to switch since I had read of connections between simvastatin and EF. He said that was okay. I took the prescription for the Procardia XL but didn't have it filled until I talked to my rheumatologist. Since he is more of an expert on Raynaud's I wanted to make sure he agreed it was a good medicine to take for that condition. He agreed it was fine.
My February appointment with my rheumatologist went very well. He said the spots on my hands hadn't spread and hadn't hardened so he didn't think I had scleroderma. He said it could have gone either way. He was pleased with the way the skin on my legs and arms felt. The woody feeling was gone and I had good flexibility. He said clinically I was fine and he wanted to take one more blood test and see me in 4 months before releasing me.
Today is March 5, 2006; exactly 2 years since I swallowed that radioactive iodine pill. I feel very fortunate to be in a remission from EF. I also feel very fortunate to have been treated by a rheumatologist who diagnosed my disease quickly and then kept a good eye on my progress. Every visit has ended with, "You know you can always call me if you need anything before your next visit." I have trusted him completely along the way.
In April, 2006, I changed my Primary Care Physician for reasons I won't go into here. I had my records transferred but my first appointment was before my new PCP had a chance to receive them. I reread this blog before I went to refresh my memory so I would be able to summarize my medical history as succinctly as possible. I knew I would be giving him a lot of information to absorb at one time. Fortunately, he was a good listener and commented that I did a good job telling my story. He decided to do a blood workup including Hemoglobin A1C, CBC, CMP, CPK, ESR, T3, Free T4 and TSH. I was also having bloodwork done for my rheumatologist so we just combined the two and I had six vials drawn in late May. Then I was to see my new PCP and my rheumatologist in June. My PCP sent a copy of my results in the mail before my visit. Nobody had ever given me a copy before, they just read the results over the phone. Right away I noticed my white blood cell count was low - 3.3 with normal being 4.0 - 12.0. It took me by surprise. Except at the beginning when my eosinophil count was too high, everything had been okay until now. My PCP asked if I had been sick. I didn't remember anything. He said we needed to keep an eye on it because it might be from the Carbamazepine. I showed him my hands where I had the red spots before. I had lost the pigment in those spots. At first I thought the spots had just faded, but as I got a little tan on my arm and hands it became obvious I had no pigment in those areas. He said it was vitiligo. He checked his palm pilot and it said vitiligo runs with autoimmune diseases and thyroid disorders, among other things. I also lost 5 pounds in the last 2 months. We decided I would have another CBC in 3 months and come in for another appointment.
I also saw my rheumatologist in June for what was suppose to be my last visit. He was intered in my hands and my low white blood cell count. He agreed the low wbc could be from the carbamazepine. I told him I was to see my neurologist in September and he was suppose to withdraw the carbamazepine at that time. He said the spots on my hands were "a vitiligo like condition". He said it showed there was still an inflammatory process going on. He asked me the standard questions regarding hard spots on my skin, swallowing difficulties, stiffness and pain in my joints. He listened to my heart and lungs and felt my neck for swollen glands. He asked if I had had a mammogram recently. I had, and it was normal. I admitted I had trouble swallowing my calcium pill a few times. It just seemed to get lodged half way down my throat and I couldn't get it to go down. My elbows were my biggest joint complaint. I can bend and straighten them but it hurts to do it. Sometimes at night they can get pretty sore and now I notice my left wrist is getting sore, too. Needless to say, it wasn't my last visit and I will see him again in September.
My white blood cell count in September went up to 4.1. My neurologist said my previous low count could either be from the EF or from the Carbamazepine. He wants my PCP to do a CBC every 3 months and if it goes down again he said he would probably change my medication. I had hoped he would take me off the seizure medication but he said he didn't want to. I asked him what the "persistent theta slowing" meant on my last EEG. He said it meant there was a problem in my brain. He said he sees that type of slowing with migraines (which I don't have), epilepsy and TIA's. He also said I may have more seizures. He said he wasn't as concerned about what the problem was as how I was doing. He asked me if I was depressed and how good my memory was. He gave me some cognitive tests and said I appeared to be doing fine.
There has been nothing new with the EF. My left elbow still gets pretty sore and my ankles still get tight but nothing has really changed for a long time. My PCP raised my Nifedical from 30 mg to 60 mg because my blood pressure and pulse were too high. I do notice my hands are warmer on the 60 mg. I was cold all the time before, even after exercising; now I'm cold but I warm up when I move around. I can feel my finger tips start to tingle as they warm. But I don't think this medicine is controlling my blood pressure. I take it at the machines in the grocery store and drug store and it is still quite a bit higher than it was on the Atenolol.
It's funny how your worry list changes. Two and a half years ago my thyroid problem and the radioactive iodine pill were my main concern. Then I developed the EF and I pretty much forgot about the thyroid. Next I had the seizures and they jumped to the top of my list. Now it appears they are going to stay at the top of the list.
In February, 2004, a blood test showed I was moderately hyperthyroid. My PCP ordered a thyroid scan which showed a toxic multi-nodular goiter. I was advised to seek treatment since hyperthyroidism was very hard on my heart and the best treatment was to simply swallow a radioactive iodine pill. Despite sounding scary, the radioactive iodine would only be absorbed by my thyroid gland with the desired result of destroying it. Then I would take Synthroid the rest of my life to replace the hormone produced by my thyroid. Other options included anti-thyroid medication or surgery to remove my thyroid but the radioactive iodine pill produced fewer side effects and was less risky than surgery. I researched the Internet and found that I-131 Radioactive Iodine Therapy was the cheapest, the fastest and the best treatment of hyperthyroidism.
On March 5, 2004, I had an appointment at the hospital to receive my pill. The technician gave me a sheet of warnings to heed for the next few days - don’t sleep with anyone, stay away from small children, use separate dishes and silverware and flush the toilet several times after using. I was also advised to carry a letter with me for the next 30 days that stated the date I received the pill, how much I was given and who to call with questions. It seems with today’s sophisticated equipment it was possible for me to set off alarms in airplanes, federal buildings and some tunnels, but I was assured it was perfectly safe for me to swallow the pill. I asked the attending physician if there were any side effects I needed to watch for. He said there would be no side effects, although the sheet given to me did state there might be some nausea and tenderness in the neck area. Then they brought in the small lead cylinder. As the technician and doctor watched, I was instructed to remove the container inside and just tip it up and take the 29 milliculeter capsule inside. Thus began a year long roller coaster ride for both my family and me.
I didn’t really feel any different for the first two weeks. Then I started having pain in my calves, thighs, forearms and upperarms when I moved them. I also noticed stiffness when I got up from a chair but it went away quickly as I walked around. I went back to the Internet and did a little more research and discovered it wasn’t uncommon to have some pain after the I-131 Therapy as large amounts of stored hormone were released from the thyroid. So I didn’t pay much attention to the pain but mentioned it to my PCP on my first visit after the treatment. He tested my thyroid levels but said nothing had changed so far.
By the middle of April my lower legs and forearms were swelling and the pain and stiffness in my knees continued. I was surprised one day when I went to kneel in the garden and almost landed on my face. It felt like I had lumps in my knees and the skin on my legs wouldn’t stretch far enough to allow me to kneel. And I was having trouble getting my arms over my head to use my curling iron or to shampoo my hair. I didn’t have pain while I was sitting or laying still, only when I moved my limbs. The swelling in my legs didn’t seem to go down at night either. You could press on my calves and leave an indentation with your fingers and the elastic in my socks left a deep ring around my legs. I jokingly referred to myself as "Sponge Barb". I called my PCP about the swelling and he checked my thyroid levels again and said the bottom had fallen out. He assumed that was why my legs were swelling, and he started me on Synthroid in early May; 25 mcg first week, 50 mcg second week, and 75 mcg the third week. Then I was to come back in 6 weeks for the levels to be checked again.
The swelling, stiffness and pain didn’t get any better, though. In fact, it was gradually getting worse. I had never had problems with my knees before but they hurt now when I tried to bend them. The muscles in my arms and legs hurt even when I rolled over in bed. It wasn’t excruciating pain, but it hurt. And the skin was very tight on both my arms and legs. It felt like my skin just wouldn’t stretch anymore. It was hard to wash my lower legs and feet in the shower because I couldn’t bend them to get at them. In the mornings my calves were so stiff and painful I sort of shuffled out to the kitchen. I had difficulty walking down stairs, too. I had to take the steps one at a time. Sometimes I stubbed my toe going up the stairs and I would get a shooting pain up my leg. My PCP ran more blood tests including a test for lyme disease. The lyme disease was negative but my protein level was low and my Eosinophil level was very high. He did a 24 hour urinalysis. The urinalysis was fine. My Eosinophil level, however, was elevated to 40%; 3% is normal. He thought I might be having some type of allergic reaction or serum sickness. He prescribed a week of Zyrtec. It didn’t seem to make any difference. On May 28, he gave me an 8 day course of Prednisone; 40 mg for 2 days, 30 mg for 2 days, 20 mg for 2 days and 10 mg for 2 days. The swelling went down immediately and I lost about 10 pounds the first couple days. The pain went away but the stiffness didn’t. The Prednisone gave me a real buzz. The first couple nights I hardly slept and I was busy doing everything I had neglected while I wasn’t feeling well, but by the time I was down to 10 mg the pain started coming back again. I rolled over in bed one night and noticed the pain was back. However, blood tests showed my Eosinophil level went down while I was taking the Prednisone so we thought maybe it did the trick.
Several days after ending the Prednisone therapy the swelling started coming back along with more pain. I quickly gained 10-12 pounds. I had an inflamed band about 3 inches wide around my lower legs. My knees and ankles were very stiff. I couldn’t point my toes and it was hard to bend my ankle when I put my shoes on. My wrists felt stiff and I was starting to have trouble opening my hands all the way. The skin on my forearms and lower legs was so tight it seemed like it couldn’t stretch any further. My armpits were sunken and I couldn’t raise my arms very high or bend my arms behind me. I had pain down my left side making it difficult to stand up straight. I took a couple ibuprofen in the morning and 1 or 2 at night and that took most of the pain away, but not the stiffness. And I was starting to feel tired and kind of sick, like I had a mild case of flu.
My PCP was stumped as to what was wrong with me but he wanted to send me to a rheumatologist and keep me on a low dose of Prednisone until I could get in. I agreed to see a rheumatologist but I declined the Prednisone. I knew Prednisone had a lot of bad side effects. Anyway, I thought I needed to see an endocrinologist not a rheumatologist. I was sure once my thyroid levels were straightened out all my problems would go away. Once more I had been spending my early morning’s doing research on the Internet and reading about different myopathy’s and neuropathy’s associated with I-131 Therapy. My sister-in-law had a neighbor who had the same treatment and went through similar pain in her limbs but did not have the associated elevated Eosinophil count. After she saw an endocrinologist and her thyroid levels were straightened out, she felt better. My PCP didn't think I needed to see an endocrinologist, but finally he agreed to make me an appointment with one. But the earliest appointment wasn't until the end of September.
June was not a good month. By now I was feeling sick at the end of each day and ran a very low grade fever of 99 - 100. My legs were swollen and sore all the time so I elevated them in my recliner with pillows underneath to feel more comfortable. I slept with soft pillows under them at night and my arms extended with pillows under them, too. If I bent my arms or legs at night it was painful to straighten them out when I woke up. I would go to bed freezing then wake up a few hours later sweating. I couldn’t cross my legs without pain and it hurt along the shin bone and calf muscle. I cut telephone conversations short because it hurt to keep my arm bent. I took ibuprofen and that helped the pain but the swelling and stiffness was always there. Then my hair started falling out. Every time I took a shower there would be a big wad in the drain. I started to think maybe there was more going on than just getting my thyroid levels straightened out.
My PCP wanted me to quit taking ibuprofen because he thought that might be causing the swelling. He gave me Tramadol to take with 2 Extra Strength Tylenol. I took it in the morning and then again at night. I felt soooo good when I took them. I had never taken any kind of pain pill before.
I continued with my early morning research and discovered something called Eosinophilia Myalgia. A group of people had gotten this from a contaminated batch of L-Tryptophan, a diet pill purchased from a health food store. Wow, their symptoms were sure similar to mine. I even mentioned this to the nurse when she made her weekly call with blood test results. And I bought a Merck Manual to supplement my research. Lupus looked like it could be a possibility. I mentioned it to my PCP. He did an ANA(Antinucleur antibody) test along with another CBC. My Eosinophils were back up to 37% and the ANA test was positive. Since the ANA was positive, follow-up tests were being completed but it would take several days to get the results. These tests turned out to be negative. But this time my PCP called instead of the nurse and talked to me on the phone. He said he wanted me to see an Oncologist-Hematologist, too. He mentioned Myelodysplasia Syndrome and Lymphoma and said I might have to have a bone marrow test. I said okay and hung up the phone and cried.
Things started happening quickly. My appointment with the hematologist was a week away on Monday and the rheumatologist called and said they had a cancellation and could get me in on the Friday before the hematologist visit.
The rheumatologist had a resident do my initial exam. I had everything written on a piece of paper so I could remember the progression of events. I tried to tell her everything. She was so gentle when she touched my arms and legs. After she examined me she said she would confer with the rheumatologist and then they would both be in. When the rheumatologist came in he examined my arms and legs and pointed to the inflamed band around the bottom my legs and said that was the orange peel effect. I had read about that in conjunction with an autoimmune disease but couldn’t remember which one. Then he said my arms and legs felt “woody”. He mentioned Eosinophilic Fasciitis. I recognized that name from my research but I couldn’t remember the particulars. I had read about so many diseases. They looked at my blood tests. The ANA was positive but the ESR (erythrocyte sedimentation rate) detecting inflammation wasn’t elevated. The resident asked if that would have to be elevated for an EF diagnosis. The rheumatologist said he wasn’t sure but he would investigate. He didn’t think I had Scleroderma because my fingers and toes weren’t involved. He said he had only seen one case of Eosinophilic Fasciitis and if that wasn’t what I had then it would take a group of specialists to figure out what I did have. I said I was seeing an Oncologist-Hematologist on Monday and he said that was good. I also told him about the appointment with the Endocrinologist. He said that was good, too. He said I needed a full skin biopsy and I needed to have it done as soon as possible. He also said the biopsy had to be sent to a good laboratory. Thankfully, my insurance paid for a good laboratory. I asked him about the prognosis for EF. He didn't want to talk about that until after the biopsy. He also said we needed to start aggressive treatment right away - 60 mg of Prednisone a day.
I went home and looked up EF (Eosinophilic Fasciitis) on the Internet and in the Merck Manual. I found it was an autoimmune disease possibly related to Scleroderma. It said 60 mg Prednisone was usually prescribed for a couple of months; then gradually dropped to 10 mg for 2-5 years. There were very few documented cases. It said the inflammation eventually went away but most people were left with the scars and contractures it caused. Sometimes limbs stiffened in odd positions. Rarely internal organs were involved. Prognosis was poor if bone marrow was involved. Myelodysplasia and aplastic anemia were possible complications.
I started 60 mg Prednisone on June 25, 2004. My legs felt better after the first dose. The swelling started going down immediately. Once again I lost about 12 pounds in 3 days. I waited for the buzz the Prednisone gave me the first time I took it, but the buzz never came. At night I only slept about an hour or so at a time and then I had to get up and go to the bathroom. I guess it was from the Prednisone. I would watch television or listen to the radio until I fell asleep again. I probably only got 4 hours of sleep each night. I started feeling physically tired and extremely weak after about a week on the Prednisone. By the 10th day I was so tired and weak it scared me. I had never been that weak before. I called the rheumatologist. He said as long as the swelling was down I could drop the Prednisone to 40 mg a day. I remained on 40 mg a day for the next two months.
On Monday I saw the Oncologist-Hematologist. I told him my whole story. I asked him if he had ever seen EF before. He said he had seen it once and that was when he was at the Mayo Clinic. He didn’t want to offer a prognosis. He looked at my legs and said he couldn’t see any of the orange peel effect. He called my rheumatologist and conferred with him while he was still in the room with me. He told him I must have had a quick response to the Prednisone because he didn’t see the orange peel effect on my legs anymore. My rheumatologist told him I looked like a classic case of EF. The hematologist told me he thought my rheumatologist had "hit the nail on the head" with EF so he didn’t proceed with a bone marrow test at this time. He said he would close my file until such time as my rheumatologist called him.
My daughter did some research through the university she was attending and found some cases where patients with EF were getting good results with Cimetidine (Tagamet) along with Prednisone. I showed the information to my rheumatologist and he said he didn’t have a problem with me taking it. He started me on 800 mg a day. He also prescribed 70 mg of Fosomax once a week because Prednisone depletes calcium from your bones very quickly.
I saw the surgeon in early July for the biopsy. I asked him if he had ever seen a case of EF before. He said he had heard of it but had not seen a case. My rheumatologist had requested a biopsy of my arm and ankle area. The surgeon said he didn’t want to do a biopsy around my ankle because it wouldn’t heal very well. After I started the Prednisone and the swelling went down, my arms looked pitted with grooves or “furrowing” along the veins in the upper arm area. There were also little lumps all over my arms. The surgeon picked one of these lumps on my left forearm and marked it. He deadened the area, took several deep samples and stitched me up. He said he would notify the lab right away that the samples were coming. It took about a week after the biopsy for the site to quit oozing. My arm remained numb from the incision site down to my wrist for the rest of the summer. It didn’t really heal nicely until a couple months later when my Prednisone level was down to 30 mg.
The biopsy confirmed EF. My rheumatologist said the disease was so rare there was very little information on it. He offered to print some information from the Internet but I said I had already read everything I could find. He told me it rarely affects the internal organs and 75% of people end up with permanent contractures. He said he couldn’t find any research studies going on but if I found any to let him know. He also said if my Eosinophil level didn’t drop to the normal range I would have to have the bone marrow test. Thankfully, it dropped to 3% and has stayed there for over a year.
I was able to get the endocrinologist appointment moved up to July. He also had a resident do my initial exam. I told my story again. I took the pictures of my thyroid scan and the resident looked at the scan and the stack of test results faxed to his office from my PCP and said, “Wow!” He read through them and then went out to confer with the endocrinologist. They both came back in and we went over everything again. The endocrinologist said he wasn't confident of the original diagnosis of Toxic Multi-Nodular Goiter because my initial blood tests for hyperthyroidism looked more typical of Graves' Disease. He did say the I-131 Iodine Treatment would have been the same with either Graves' Disease or Toxic Multi-Nodular Goiter. So he ran a blood test for Graves' Disease and another TSH level. The test for Graves' Disease turned out to be negative. I asked him if he had ever seen anyone have my problems after I-131 Therapy. He said not exactly. People who had problems with joint and muscle pain afterwards generally were a lot more hyperthyroid to begin with than my test results showed. And he said the pain and stiffness I was describing was not typical of what he had seen. He said the amount of Synthroid I was receiving appeared to be appropriate and he wouldn’t change anything at this time. He said he was sorry but he could not really shed any new light on my situation and would close my file.
Most of July and August I spent on 40 mg Prednisone. I still had a fair amount of pain and stiffness through the summer. My knees were the worst. It was difficult to bend my knees or to stand in one place. The skin on my arms and legs was still very tight and the skin on the top of my shins was very shiny and had a waxy feel. The hair on my legs grew very slowly and hardly at all on my shins. Sometimes my legs were a splotchy red or purple and sometimes they looked normal. My arms were hard as a rock, especially after I tried to do anything physical. Sometimes it felt like they were being “shrink wrapped”. The tightness extended down my left side and it was hard to stand straight. I had problems walking for any length of time. A trip to the grocery store was about all I could handle. I pushed the cart because I could support myself on it. When we came home I would lay down and rest and my husband would put the groceries away. I also got cramps in my fingers. I liked to sew and play the piano but the cramps made it difficult.
My Eosinophil levels remained low throughout the summer so in August my rheumatologist lowered my Prednisone to 30 mg. I was to stay at that level for 2 weeks then drop to 20 mg.
30 mg went okay so I dropped to 20 mg two weeks later. After about 3 days I started having a lot of pain in my legs and arms. They didn’t swell but I had a lot of pain. My rheumatologist thought maybe he had dropped the level too fast so he wanted me to go back up to 30mg again for 2 weeks then drop to 25 mg. It went okay this time. I started to gain more strength and had a little less stiffness. I started mowing the grass again...slowly, and did housework without getting hand cramps. And I started sleeping better at night. I also started gaining weight again.
My brother and sister-in-law came for a visit in September. We visited his old college campus and did a lot of walking. It was easier to handle the walking now even though I walked slowly. It seemed like my muscles locked up if I tried to go too fast. But I was feeling a lot better.
The first of October I started having mild diarrhea. I figured it was just from the medications. My rheumatologist thought I might be getting lactose intolerant because of the extra dairy products I was consuming. I gave up milk, cheese and yogurt for about 5 days but didn’t see any difference. I didn’t really pay a lot of attention to the diarrhea because it wasn’t that much of a bother and I was starting to feel so much better.
I was doing stretching exercises everyday and I started seeing a physical therapist. He gave me some specific exercises for my knees and arms. They were simple exercises but they made a big difference. My knees improved a lot and so did my arms.
All in all, everything was starting to go much better. Then I remember a conversation that went something like this:
“Barbara, do you know where you are?”
“No.”
“You’re in the hospital. Do you know how you got here?”
“No.”
“You were brought here by ambulance. Do you know why you’re here?”
“No.”
“You had a seizure. Do you hurt anywhere?”
“No.
I don’t remember being upset about the conversation. I must have drifted off to sleep again. I had a similar conversation a couple more times with hospital personnel. The second time I was asked this set of questions I answered with, “Somebody said I....”
I guess I had gotten out of bed about 3:00 AM on Tuesday, October 19, and had a grand mal seizure. My husband found me on the bedroom floor bleeding profusely from my nose. He didn’t know it at the time but I had broken my nose and bitten my tongue.
My husband was with me in the emergency room. He said I talked to him and all the hospital personnel. I said I felt fine but I was confused about what time it was. I remember very little of it. Then about 8:00 AM, while still in the emergency room, he said we were talking and I started having another seizure. I vaguely remember that one. I felt everything get very fuzzy around my head. I remember a nurse asking me, “Do you remember anything? Did you see an aura?” After that seizure they started me on Dilantin. My husband said I went through a full day of blood tests, CT Scan, MRI, EEG, etc. I don’t remember any of it. I was in the hospital for three days but only remember brief moments of time. I know my son was there but I don’t really remember talking to him. I remember seeing doctors but recall very little of what they said. My husband said the neurologist couldn’t find a reason for the seizures, which I guess happens in a lot of new onset seizure cases.
After I got home and my husband started telling me what had happened, I couldn’t believe I had no recollection of it. It still bothers me that I don't remember the ambulance trip, any of the tests at the hospital and very little of my conversations with family or hospital personnel. I must have slept for most of 3 days. It was unsettling when I saw the blood stain still on the carpet of my bedroom floor from my broken nose. I was so thankful the seizure hadn’t happened while I was driving. What if I had hurt someone else? And I was very thankful for my husband and family, the rescue squad, the doctors and everyone in the hospital that took care of me.
I saw my PCP on a follow-up visit a week after the seizure. By then I had a rash on my stomach and arms. And I had a dry cough and a tender mouth. He said I should call my neurologist because a lot of people get a rash from Dilantin. I called and he took me off of the Dilantin right away and gave me Carbamazepine, 1000 mg’s a day. I took 2 pills in the morning, 1 at midday and 2 at night. At first I would occasionally lose my balance but that went away after a couple weeks. But the 2 pills in the morning made me feel very drowsy. I had trouble even thinking straight until about midday. I constantly battled fatigue and usually took 1 or 2 short naps a day. I had to quit taking the Cimetidine because it wasn’t compatible with Carbamazepine. I wasn’t to drive, swim alone or climb ladders for six months. The neurologist told me not to get overly tired and to stay away from caffeine since those two things can trigger seizures. And I wasn’t to drink any alcohol. That wasn’t a problem for me since I don’t drink anyway. I was to have a follow-up EEG in six months and if that one looked okay he said we might talk about reducing the medicine.
I was a little worried that after spending three days in bed I would be very stiff and sore. But actually it was just the opposite. My arms and legs felt pretty good. And I didn’t notice any difference in the EF after discontinuing the Cimetidine.
I read what the Merck Manual had to say about seizures but didn’t do a lot of research on the Internet. It gets pretty complicated when you’re talking about your brain and I was tired of looking things up. My rheumatologist said he couldn’t find any information that linked seizures with EF. Since then, I found one entry on PubMed where a woman had a grand mal seizure at the same time as she was being diagnosed with EF. I’m happy I didn’t have a stroke or a heart attack or they didn’t find a brain tumor but at the same time it’s disturbing when you don’t know what caused the seizures.
At first after the seizures my husband was afraid to let me out of his sight. The first few nights he held onto my nightgown so I wouldn’t get out of bed without him knowing it. During the day we stuck together like glue. It was scary for him because he saw what happened to me and it was scary for me because I had no memory of anything happening. Gradually we both got used to going places again and I didn’t think about it all the time.
My diarrhea went away while I was in the hospital but came back the first week of December. My PCP sent me to a GI doctor. I had a Colonoscopy with biopsies, Upper Endoscopy with biopsies, and blood tests for Celiac disease and Lactose Intolerance. Everything was okay except one of the biopsies of the colon showed mild Lymphocytic Colitis. He prescribed Asacol. That made it worse. Metronidazole, an antibiotic, helped for awhile, but then it came back. Finally he said to try 2 tablets of Metamucil everyday. That helped more than anything, although I still have the problem off and on. Altogether, I had diarrhea for nine months before getting relief from the Metamucil. I'm not sure why he didn't have me try the Metamucil first rather than prescription drugs, but it's too late to worry about that now. My rheumatologist said his research showed EF didn’t affect the intestinal tract. That was good to know. I lost about 10 pounds after the diarrhea started and have stayed at that weight ever since. Thank goodness for elastic and spandex in clothes. I began to lose track how many times my weight went up and down.
Starting in December my PCP started raising my synthroid levels. First he went to 88 mcg from 75 mcg. Six weeks later he raised it to 100 mcg and six weeks after that raised it to 112 mcg. Once I reached 100 mcg I started noticing a big improvement in how I felt mentally as well as physically. I had more energy and my muscles started feeling better. I don’t know if it was from the increase in Synthroid, the decrease in Prednisone or the improvement in my EF. Probably a combination of all three. And my hair stopped falling out sometime in December. I am currently at 125 mcg of synthroid.
Just to add a little more interest to the scenario my cholesterol levels doubled over the past year. My LDL went as high as 235 from 111 a year ago and my total cholesterol went to 320 from 167 a year ago. Uggh. More medication. I started on 10 mg Zetia the first month. The LDL went down to 186. Then my PCP added 10 mg Pravacal with the 10 mg Zetia. I’m going to continue with this medication until December, 2005 when I will be re-evaluated again. Hopefully things will all even out soon.
I didn’t have any seizures from October 20, 2004 through April 1, 2005. I had my follow-up EEG on April Fool’s Day. Since it was a sleep deprived EEG, I didn’t nap the day before and I stayed up until 12:30 AM that night. My daughter came home to help me stay awake since I was usually out like a light by 8:30 PM. Unlike when I was on 40 mg Prednisone, the Carbamazepine made me sleep up to 10 hours a night. We colored her hair and generally goofed around to pass the time. She brought an air horn and tooted it if I started to drift off. Since I was only supposed to get 3 hours of sleep, I went to sleep at 12:30 AM and awoke at 3:30 AM. I was to be at the hospital at 6:45 AM. The technician went over my history of illnesses and medications. My husband and I always kept a list with us because it had become a sizable list. When I told her I had Eosinophilic Fasciitis she said, “You have what? Can you spell that for me?” I was used to that question so I closed my eyes and sounded out the words in my head and spelled it. Then they measured my head and hooked me up to all the electrodes. They started by having me open my eyes and shut my eyes several times. Then they had me close my eyes and flashed a series of lights at me and had me hyperventilate for 3 minutes. Afterwards they said, “Okay, just fall asleep for us now.” If I was at home I would have been out in a few minutes but I could not fall asleep for the test.
Unfortunately, I did not get a passing grade on the EEG; bilateral independent temporal slowing and bilateral independent temporal epileptiform abnormalities. My neurologist said the results weren’t really wrong but they weren’t right either. He said they could be more specific if I had fallen asleep. So I am continuing with the Carbamazepine and have another EEG scheduled for September 1, 2005. But he did drop the morning dose of pills from 2 to 1. That has helped a lot to eliminate my fatigue during the day.
My rheumatologist continued to reduce my Prednisone levels until March, 2005 when I reached 5 mg a day. He said he wasn’t in a hurry to go lower since I was at a very low level and I was making good improvement. The furrowing in my upper arms has disappeared and most of the pitting isn’t visible anymore. My skin has softened quite a bit and I no longer feel like I’m being squeezed to death. I still have some stiffness in the ankle, knee, wrist and elbow joints but I can get into the bathtub, fasten my own necklace and lift my beautiful granddaughter. And I can cross my legs now without pain; something I couldn't do for a year. I have continued with the stretching exercises and started doing some light aerobic exercise. I can walk fairly briskly and can stand from sitting without groaning. My hair has grown back and I now have a whole head of curls instead of straight hair. What a pleasant side effect! As of May 19, 2005 I started alternate day therapy with 5 mg of Prednisone. My rheumatologist said our goal this year is to get completely off the Prednisone.
In late May I caught a mild cold that progressed into a pretty decent cough. After a few weeks, the cough tapered off only to return again a few weeks later. I didn't run a fever and didn't feel sick, but just to be on the safe side I visited my PCP. He said everything in my chest sounded okay but with my history he decided to do a chest X-ray just to make sure there wasn't anything going on. The chest X-ray was normal. My cough never really went away and since May I have continued to have an intermittent dry cough until September.
The results of the EEG on September 1 were better than the April test but still considered abnormal. This time there was persistent theta slowing focally in the left temporal lobe. My neurologist said he didn't see the same type of slowing on this EEG that was on the previous one, though, so he asked me how I felt about discontinuing the Carbamazepine. I told him that as much as I would like to stop taking it, I did feel a certain sense of security while on it. He said it was good that I hadn't had a seizure in the past year but it would be even better if I went another year seizure free. He dropped my dose to one pill in the morning, afternoon and evening and said if nothing happened in the next year he would discontinue it completely.
In August my rheumatologist felt I was in a remission with the EF. He said my blood levels have been normal for a year and there has been no signs of inflammation. My skin has softened and doesn't show the pitting and furrowing anymore. I am continuing with alternate day therapy with 5 mg Prednisone. I have developed a blueish red, shiny spot on my right hand between my thumb and first finger. There is also a red rash that has developed along my knuckles on both hands and extends a little down my fingers. I showed it to my rheumatologist but he didn't really say much about it. He stretched my next visit out until December.
In December my PCP did another cholesterol check. My LDL was down to 162. Although it is still high he said he would be satisfied to see it below 160 since my HDL is 67. He told me to continue with the Zetia and Pravacol and to watch my diet and have it check in another 6 months.
I also saw my rheumatologist in December. The purplish spot on my hand had grown from about a quarter size to about a 2 inch diameter. He was also concerned about obvious Raynaud's Phenomenon in my hands. There was no swelling of my fingers or hardness of my skin, however. And my blood tests remained fine. He mentioned possibly sending me to a dermatologist since they handle the skin portion of auto-immune diseases. He was concerned I might be developing scleroderma. He said we might have to treat the Raynaud's but in the meantime to make sure I kept my hands as warm as possible and didn't get frostbite. He gave me a brochure to read about it. He also said I could quit taking the prednisone now but advised I wait until after the holiday season just in case something went wrong. He made my next appointment for February 28, 2006.
As of January 2, 2006 I quit taking prednisone. I feel fine and have noticed no changes. I still have tightness in my ankles and knees but I'm not sure that will ever go away. Sometimes I have pain in my left leg joint where it connects to my hip and in both elbows extending down towards my wrist. But I'm not complaining. I would say my flexibility is about 90 - 95% of what it was before I contracted EF. I do stretching exercises and walk as much as I can. I also have a very dry mouth (something I think I forgot to mention until this point). I have pain along the sides of my tongue and the inside of my cheeks a lot, too. Some days are better than others. If it's really bad I just take a Tylenol. My weight has stabilized and I am about 12-14 pounds lighter than before EF. I have had 3 colds in the last 8 months, something that is unusual for me. All 3 developed into prolonged coughs. I coughed for about 5 months with the first cold and 3-4 weeks with the other two colds. I think I must develope bronchitis.
I saw my PCP in February. It was really cold in his office and my fingers turned white and spotchy red with the Raynaud's. He wanted to put me on Procardia XL 30 mg since it would be good for the Raynaud's. He said the Atenolol I was taking for high blood pressure was just making it worse. He also wanted to switch my cholesterol medication to Zetia and Zocor. I asked him if Zocor was simvastatin and he said he thought it was. I didn't want to switch since I had read of connections between simvastatin and EF. He said that was okay. I took the prescription for the Procardia XL but didn't have it filled until I talked to my rheumatologist. Since he is more of an expert on Raynaud's I wanted to make sure he agreed it was a good medicine to take for that condition. He agreed it was fine.
My February appointment with my rheumatologist went very well. He said the spots on my hands hadn't spread and hadn't hardened so he didn't think I had scleroderma. He said it could have gone either way. He was pleased with the way the skin on my legs and arms felt. The woody feeling was gone and I had good flexibility. He said clinically I was fine and he wanted to take one more blood test and see me in 4 months before releasing me.
Today is March 5, 2006; exactly 2 years since I swallowed that radioactive iodine pill. I feel very fortunate to be in a remission from EF. I also feel very fortunate to have been treated by a rheumatologist who diagnosed my disease quickly and then kept a good eye on my progress. Every visit has ended with, "You know you can always call me if you need anything before your next visit." I have trusted him completely along the way.
In April, 2006, I changed my Primary Care Physician for reasons I won't go into here. I had my records transferred but my first appointment was before my new PCP had a chance to receive them. I reread this blog before I went to refresh my memory so I would be able to summarize my medical history as succinctly as possible. I knew I would be giving him a lot of information to absorb at one time. Fortunately, he was a good listener and commented that I did a good job telling my story. He decided to do a blood workup including Hemoglobin A1C, CBC, CMP, CPK, ESR, T3, Free T4 and TSH. I was also having bloodwork done for my rheumatologist so we just combined the two and I had six vials drawn in late May. Then I was to see my new PCP and my rheumatologist in June. My PCP sent a copy of my results in the mail before my visit. Nobody had ever given me a copy before, they just read the results over the phone. Right away I noticed my white blood cell count was low - 3.3 with normal being 4.0 - 12.0. It took me by surprise. Except at the beginning when my eosinophil count was too high, everything had been okay until now. My PCP asked if I had been sick. I didn't remember anything. He said we needed to keep an eye on it because it might be from the Carbamazepine. I showed him my hands where I had the red spots before. I had lost the pigment in those spots. At first I thought the spots had just faded, but as I got a little tan on my arm and hands it became obvious I had no pigment in those areas. He said it was vitiligo. He checked his palm pilot and it said vitiligo runs with autoimmune diseases and thyroid disorders, among other things. I also lost 5 pounds in the last 2 months. We decided I would have another CBC in 3 months and come in for another appointment.
I also saw my rheumatologist in June for what was suppose to be my last visit. He was intered in my hands and my low white blood cell count. He agreed the low wbc could be from the carbamazepine. I told him I was to see my neurologist in September and he was suppose to withdraw the carbamazepine at that time. He said the spots on my hands were "a vitiligo like condition". He said it showed there was still an inflammatory process going on. He asked me the standard questions regarding hard spots on my skin, swallowing difficulties, stiffness and pain in my joints. He listened to my heart and lungs and felt my neck for swollen glands. He asked if I had had a mammogram recently. I had, and it was normal. I admitted I had trouble swallowing my calcium pill a few times. It just seemed to get lodged half way down my throat and I couldn't get it to go down. My elbows were my biggest joint complaint. I can bend and straighten them but it hurts to do it. Sometimes at night they can get pretty sore and now I notice my left wrist is getting sore, too. Needless to say, it wasn't my last visit and I will see him again in September.
My white blood cell count in September went up to 4.1. My neurologist said my previous low count could either be from the EF or from the Carbamazepine. He wants my PCP to do a CBC every 3 months and if it goes down again he said he would probably change my medication. I had hoped he would take me off the seizure medication but he said he didn't want to. I asked him what the "persistent theta slowing" meant on my last EEG. He said it meant there was a problem in my brain. He said he sees that type of slowing with migraines (which I don't have), epilepsy and TIA's. He also said I may have more seizures. He said he wasn't as concerned about what the problem was as how I was doing. He asked me if I was depressed and how good my memory was. He gave me some cognitive tests and said I appeared to be doing fine.
There has been nothing new with the EF. My left elbow still gets pretty sore and my ankles still get tight but nothing has really changed for a long time. My PCP raised my Nifedical from 30 mg to 60 mg because my blood pressure and pulse were too high. I do notice my hands are warmer on the 60 mg. I was cold all the time before, even after exercising; now I'm cold but I warm up when I move around. I can feel my finger tips start to tingle as they warm. But I don't think this medicine is controlling my blood pressure. I take it at the machines in the grocery store and drug store and it is still quite a bit higher than it was on the Atenolol.
It's funny how your worry list changes. Two and a half years ago my thyroid problem and the radioactive iodine pill were my main concern. Then I developed the EF and I pretty much forgot about the thyroid. Next I had the seizures and they jumped to the top of my list. Now it appears they are going to stay at the top of the list.
posted by efasciitis | 5:16 PM
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