Scleroedema

[Margaret Mueller]

July 14 & 15, 2009: Met with AP rhemy docs. Took my history, more x-rays, more blood. The best doctors I've ever met with. Precise, probing questions, specific interests. Physically examined skin, symptoms. It seemed like each question narrowed the field, until he told me I have scleroedema. I hadn't heard of that one. My skin has no involvement. I will be starting AP on Friday. When we leave Bill says, “this wasn't a medical journey.” “No, it has been a spiritual journey.” We both feel that this is absolutely the right treatment.

Friday, July 17, 2009: First day of my meds. Oh, the anticipation. A little nausea with some, but I got past it. Looked up scleroedema on line, and in the first page an article about whooping cough and scleroedema — whooping cough (a bacterial infection) is what I had when the hyperpigmentation and muscle weakness started in June 2005. CDC says pertussis was a pandemic that year. This ties the threads of my history together. Adults need boosters for Pertussis.

Monday, July 20, 2009. Our anniversary. My second day on antibiotic protocol. I think I'm noticing some subtle changes. My finger tips seem to have faint “puckering” for the first time in years. I didn't have pain in my hands when I used the stairstepper at Physical Therapy today. I use my fingers to measure how wide I can open my mouth, they fit without binding today which means it opens slightly wider. My digits, fingers and toes, which feel 'thick' are loosening. Only time will tell, are these changes real? Permanent?
I also have adult on-set (at 55!) FSH muscular dystrophy, the doc says the AP will have no impact on my FSHMD. However, I noticed that it required less effort to lift my right arm when I changed positions during my workout. My right arm has felt thick and bound up in the shoulder and upper arm/tricep area.
Bill ordered champagne for dinner at Piatti, I didn't partake. I want to get well.

Tuesday, July 21, 2009: My hands didn't hurt this morning when I used the stairstepper at PT. My toes cracked when I got out of bed this morning, and they haven't done that in years — my swelling in my feet must have gone down. I have more agility walking, and instinctively I've started walking faster. My right arm has less restriction, more mobility. My left hand makes a tight fist. I have more dexterity in my fingers, I could pick up things I spilled today — easily. The tissue in my fingers is loosening up. No aching in my knees, elbows or ankles today.

Wednesday, July 23, 2009: The rhemy I saw a week ago has been seeing scl patients for forty years, and is a member of the college of Rhematologists, so he's not a “light-weight” in experience or knowledge about scl patients. His knowledge of scleroderma and its diverse presentations was evident as he meticulously questioned my history, labs, and physical condition during the three hours he spent with my husband and I. When he addressed my specific condition, scleroedema, he explained the diagnosis completely, was not condescending or impatient with any of our questions. He prescribed 150 mg of Doryx 2X/da M-W-F with a probiotic and supplements. This is different than I have read scleroderma AP patients receive. That is because my rheumy does not do a one-size-fits-all treatment of patients; this AP is for me and my specific condition. He made no promises about the progress of my treatment which is why am keeping this journal. Regardless of outcome — which let me make it clear I am an optimist by nature — I hope to give other people access to my experience of my disease.

Thursday, July 23: I got up this morning and started cleaning the kitchen. That may not seem like a milestone, but to have the energy to do that is huge for me. My lips aren't as dry; it almost feels like some kind of natural lubricant, or oil is being produced again to make them soft, and slidey rather than like two pieces of paper. Between my thumb and forefinger, their has been a mass of hard tissue. It is starting to recede on my left hand. The hands are less swollen. I wonder if I will be able to wear my gloves again? I can pinch twice as much skin (which isn't much in the first place) as I could a week ago. I showed Bill, he was so surprised, and happy. It is the little things, ya know? I can pinch my left check near my eye, but not my right at all.

Made some more incremental improvement today, less swelling, less thickness between the thumbs and forefingers, and I can clasp my hands together. wow. I also was brushing my teeth, and suddenly thought, “where'd all this saliva come from?” I guess it used to be in that quantity, but its been a while.

Friday, July 24, 2009: It is D's 16th Birthday. I haven't felt like baking in a long time, but I tried. I made cupcakes from scratch. It wasn't easy, but I did it, and they turned out. D. had a meltdown this evening. We got it worked out; it's tough being a teenager. Took the family out to dinner. I didn't feel too tired today.

Saturday, July 25, 2009: Big day at church, visiting clergy and a baptism. Everyone came up and said how good my face looks. I hadn't noticed, but I look more relaxed, there is less tightness, and the skin doesn't feel stiff, but supple. Wow.

Sunday, July 26, 2009: It's B's 14th birthday. I'm baking again! and I feel good. I haven't done this much cooking in one week in a long time! Brian had a good birthday. Another big day with lots of people noticing I'm doing better.

Monday, July 27, 2009: I had two girls come over and help me sort out my closet — things I can't wear anymore because of the FSH. Suit jackets, silk shells, any shoe with a heel had to go, because I just can't balance. I am exhausted after two hours, and they did the work.

Tuesday, July 28, 2009: My primary wanted me to see the rheumy at the local med ctr. I took my history. He was impressed. He was curious about the AP. He told me he doesn't like to prescribe the usual meds, because he says they don't work, except, he says, the lung med. does. He says scl patients he has seen doing antibiotics are doing better. He examined me, and says he thinks I have localized scleroderma, but he couldn't find any hard skin (probaby because I've never had any). My skin has felt thicker — he notice particularly the skin on my chest which has the whiteish, shiny skin and has been thick. But, he commented that it was soft, and he didn't expect it to be because of the whiteish marks. I didn't know it had softened up! He also commented on the lacey pattern and hyperpigmentation on my legs.
Because my scleroedema came on at the same time as my FSHMD, he thinks the cause is the same, and says my FSH may reverse because of AP. That doesn't seem to make medical sense to me, but I think it's interesting. He says that he has had other patients with neck weakness reverse symptoms.
At the end, I asked if I should get flu, pneumonia and shingles shots. He said not to get the shingles, because it's a live virus, and because all this came on after my illness in 2005, he thinks live virus would accelerate my sclero symptoms. He said I should keep doing what I'm doing.
He asked if he could keep the records I brought with me, and I said sure, but when I went through what was left, he only kept what I wrote about my AP.

Thursday, July 30, 2009: I had my regular appt. with my PT. I showed her all my AP progress, she's been excited to see what would happen after I started the meds. I told her my right arm, which I've been working out since January (seven months) and has been static, now feels less thick, has greater range of motion, and has become stronger than the left arm, which has been dominant until now. I also told her what the med ctr. doc said about possibly recovering from the FSHMD, and that I would like to try to work on my scapular (shoulder blade muscles) She gave me three new exercises, and touched the muscles as I tried to work them — oh, and they were working! I am sore already! She says my face has really changed a lot. This is a very happy day.

Friday, July 31, 2009: Made a giant batch of spaghetti sauce, went to PT, grocery shopped, gassed the van, packed food and clothes for family reunion this weekend. Where'd all this energy come from? Mom hadn't seen me in two weeks, Sis since January — they could see a big difference. They mostly noticed my face, but I showed them my hands, too. They could tell a big difference in the way I move and walk, too. More family tomorrow.

Saturday, August 1, 2009: My collar bones are parallel to the floor; they've been tipped up because of my weak shoulders. Yesterdays exercises are working. Big day all day. I made two salads for dinner, played cards and dominos with various family members. Watched them play games on the lawn. I was careful to stay in the shade. I gave up when the family was roasting marshmellows with the kids around the fire pit at 10:00 p.m. I just got tired. I didn't try the lawn games, maybe I should have.

Sunday, August 2, 2009: I woke up this morning, and my hands were flat, not clenched at my chest. They felt L-O-N-G. I haven't woken up with flat hands since I can't remember when! Then, I checked my legs. I still have “brown socks” starting at my ankles, but the hyperpigmentation has faded this weekend, not completely, but it is really, really faint, and when I press my leg I don't see the imprint of my fingers — the inflammation seems gone. I don't see the “lacey” pattern on my legs at all. I wonder if that will change with the temperature? My hands and face seem lighter. Skin is even looser on the backs of my hands. I got tired earlier today, and a little cranky. We got home and cooked dinner for the family. I've cooked a lot this week, and not just simple meals.

Friday, August 14: All symptoms are improving. I am continuing to see more flexibility in my hands, and regaining some fine motor skills I had lost. I am starting to realize that my arms and legs, shoulder joint and back of my neck was swollen. My PT noticed that the dark spots on my neck and back are fading. My legs are fading dramatically. I have knuckles on the backs of my hands again — they were too swollen to see before. I've never been vain, not a stand in front of the mirror sort of girl, but I am just amazed at how supple my face has become. Four years ago this fall, I told my husband that my legs felt like I was slogging through molasses when we went for a walk. I think that they were impacted by the scleroedema. I am moving much more freely. I've talked to my PT about working up to a 5k by Thanksgiving. I don't know if I can run, but I definitely think walking it is in the cards. My PT has started me on one pound weights for my should joint muscles which are responding. I am also starting squats and modified lunges to develop more strength and balance. The work is starting to strengthen my neck. It feels less strained. And, energy, I have so much energy. I'm cooking every night, and it isn't horribly difficult anymore. It is getting to be fun again. My oldest wants to bring his college roomies home next week for his 21st. I think I can. I think I can. I think I can.

August 29, 2009: Got the house clean and gave a great bbq for my son and ten friends — what great young people! And, it didn't wear me out! Wow. Since the fifteenth, I have started new Physical Therapy for my shoulders, and have they responded! It seems to me that there is reduced inflamation in my muscle, that the AP is knocking down the bacteria, and my muscles are responding with less swelling, less fatigue, and they are retaining the fitness from the exercise. Less swelling also means that my joints can exercise in the right position (my shoulders have been out of joint), and the fit muscles are holding the bones in place, so they both have no pain, and they work better. This is what she's having me do: I lie on a table with my arm perpendicular to the floor, one pound weight in hand. I lift my arm horizontal to the floor and hold it for ten seconds; ten times. Then, with my elbow ninety degrees to my arm, I lift my forearm horizontal to the floor, hold it and repeat ten times. Then out to the side ten times & hold….I'm doing thirty reps each exercise, each arm. My shoulder blades have tightened up and my right arm is staying in the joint.

Oh, man, the leg exercises I've been given are excruciating. I have a lot of work to do to get these back in shape. I am walking better, but working on my legs makes me realize that the edema is greater in them than in my arms at this point. I wonder if the physical therapy is going to loosen them up. I guess I'll know if I retain the fitness the way I do with my arms.

Another nice thing, I have a lot, lot less fatigue. I do a lot of work for my church, and in the last few weeks I've once again been able to chair meetings, organize effort and complete projects. This is really gratifying.
Visually inspecting the change in my arms, I realize that the edema is not completely gone, so I know there are more changes to come. It is something to look forward to.

Sept 17, 2009
Today is my two-month marker for starting AP. I see Dr. F. Monday. I think he'll be surprised at how much progress I've made. I feel as though the scleroedema has invaded the muscles all over my body. As the AP began to take effect, the muscles have shrunk from being bloated, have regained mobility, flexibility and increased neural responses. No, no one has tested this, but two nights ago I dropped a jar while I was cooking, and caught it on the way down. I couldn't do that a month ago. My fine motor skills are also getting a lot better; I had no sensitivity in my finger tips two months ago, and now I can feel again. I can pick things up with my fingers again. I may start needle work again.

I think the scleroedema was forcing my joints apart. Since I started AP I can feel that the density of the muscle tissue surrounding the joints has diminished, and as the tissue gets smaller, my arms, legs, fingers all work better. My right shoulder joint was essentially dislocated, but thanks to shrinking edema and a great physical therapist, it now stays in joint all the time. Of course, with that change the pain has gone away. Visually, my forearms, calves and hands are transformed from bloated to slim again — hey, I always knew i was skinnier underneath it all! I can still feel edema in my shoulders, neck, arms and legs, but it is greatly, greatly diminished.

My energy and clarity of thought has really, really improved in the last few weeks. I've been sick for four years. Last night, I attacked boxes that have been sitting in the same spot since I got sick, I sorted, threw away, and dusted. Wow. I couldn't ever get up the fortitude to tackle it before now. When I tried, I just ended up collapsing after an hour. The pain and fatigue would just overwhelm me. Last night, I just did it. I just did it.

My neck has been a big problem, but this week the muscles at the front of my neck started to work to support the weight of my head. I can now eat at least part of my meals without holding my head up.

Sixty two days, and a world of change. Thank you everyone for your generous support. I can't wait to see where I'm at after one year.

Wednesday, September 23, 2009: I had my first return visit with Dr.F on Monday. He was so much fun! He started out pinching my cheeks to check the change in my skin — which is considerable. He looked at my hands, and I ran through my laundry list of improvements. He said he was very proud of me, went over my labs, and upped my doryx to Monday thru Friday, instead of M-W-F. He is monitoring my liver function and noted other indicators of inflammation. We saw him for about a half hour to forty-five minutes. He also added DHEA to my regimen.

The last weekend was a great time — we drove to see our son in Berkeley and hiked in Tilden Park from Anza Lake to Berlin House, about four miles up hill. I did it without a problem, not winded, not aching, it wasn't too much for me at all. The best moment on this beautiful fall day came when Nick, who is 21, put his arms around me and said, “Mom, I am so proud of you for researching what you needed to do to get well, and that you've stuck with it and done it, so that our family can have times like these together.” There is nothing better than to have your family understand and appreciate what you are doing to recover. We went back to his apartment and I cooked dinner for him and his friends. I could do all that — and I couldn't begin to do that two months, let alone twelve months ago.

Sunday, we went to church and then drove an hour to my mother-in-law's for her birthday, another long day. I drove her car back so she could stay with the boys while we made our day trip to see Dr. F. And, still, I could do it. I have the energy, the alertness, everything to live theses days to the fullest.

I was asked by a friend what symptoms I still have. That is hard to answer because, as I improve, I'm suddenly realizing that there are things wrong that I hadn't noticed, or attributed to other things. I know I still have swelling in my hands, a twinge in my left knee, and weakness in my neck and shoulders, but I've also notice improvement in all those areas. My hyperpigmentation has almost disappeared except my feet seem to be the last area to fade out, and I still have brown 'socks.'

The great thing is, nine weeks after AP, I've gotten a great deal of my life back, and that is huge.

Sunday morning, October 18
When you start to feel 'normal' I guess you aren't dazzled by improvement anymore, and you don't post as often. Shame on me, it's like not being grateful for AP, and for your very life.

There have been more positive changes in the last three weeks. My fingers on both hands have loosened up a lot. I can pinch skin on all sections of my fingers on both hands now. I noticed that two nights ago. Even the hardest section on my left hand is loose now.

The other big gain is that my right shoulder is no longer dislocated. Since being on AP the steady recovery of my right shoulder has been evidence that the neurologist is wrong, I may have the genetic marker for FSH muscular dystrophy, but that is not what is happening right now to my shoulder. Within ten days of starting AP the shoulder blade muscles started coming back, and with the instruction of my remarkable physical therapist, Rachel W., I have been strengthening the shoulder until it is now in place and not popping out since last week. Last night i was finally able to do exercises to stretch my right arm forward — Rachel initially tried those with me in March. I've come a long way. I think I have many more months of therapy to get my arms fully functional, but I know its coming.

My neck is still impacted, but I don't feel and hear 'threads' snapping when I move my head anymore. It seems like only the vertebrate at the very top of my neck are stiff, so I guess I have to wait for that to dissipate, and I'll be able to re-gain lifting my head, and being able to hold my head up at meals. On the plus side, the 'interior neck flexors' on the front of my neck are coming back, and the strength they have is visible because that portion of my neck is acquiring muscle tone. I have the same 'philadelphia collar' Rachel gave me January 13, and I am hoping its the only one I ever have — that in a few months I won't need it any more.

I saw a guy giving salsa dancing instruction last month, and thought that was a good idea. Well, Rachel said dance was o.k. with her. On Thursday, they gave me a referral to a dance instructor who works with the physical therapists. They think I should start with cha-cha and rumba! Salsa involves too much hips, and I'm not stable enough for that!! I''m supposed to tell the instructor that I need to work on 'control.' I'm so looking forward to this! I think its really going to help my coordination and agility.

Recovery isn't all probiotics and skin softening.

Tuesday, November 3, 2009

I had my six month follow up with my neurologist yesterday.

He was prepared for a routine follow up on FSH muscular dystrophy, which was his 'definitive diagnosis' for me earlier this year. He asked how I was doing and I said fabulous. My physical condition, attitude and demeanor were all shouting, “this isn't the woman you saw months ago.”

I told him I wanted to tell him what I'd been doing for the previous six months. I started out by telling him that I had decided to see a rheumatologist about the scleroderma. He interupted, “Who told you you had scleroderma? Who ran the test?” I reminded him that he did.
I didn't remind him that when he gave me a diagnosis of FSH muscular dystrophy, that I had asked him about my positive scl-70, and whether he thought I should see a rheumatologist. For the record, he said he didn't think so. For the rest of the record, it was not he, but my primary care doctor who brought the scl-70 to my attention, which is why I asked him about it. Actually, I was setting him up to see if he really had paid any attention to an alternative diagnosis. There, I admitted it, I didn't think he was looking at the whole picture and that scleroderma wasn't even on his radar screen; yesterday I found out I was right.

Then, I told him that I have seen four rheumatologists and they all say I have scleroderma, but each one has a different diagnosis. I explained that I had researched the options, and opted for antibiotic protocol, and that the doctor who is treating me diagnosed 'scleroedema' and was treating me with 'doryx.' At that point he apologized that rheumatology was not his field, and what is 'doryx', so I explained it to him. Then, I told him how I had researched scleroedema on the internet, and that a 1954 study linked five cases of scleroedema to whooping cough, and that I had had whooping cough four years ago. I also shared with him that the lump he had noticed when he examined me — the one at the base of my neck/top of my spin was a symptom of scleroedema. I went on to explain that the doctor had said specifically that the ap would not have any effect on my fsh muscular dystrophy. He said that was right.

Then, I explained that my primary care, being unfamiliar with ap, had wanted me to see the head of rheumatology at the UC med center where I live. Dr. C, I told him, walked in the room and asked how long I had had FSH, he was seeing another patient with autoimmune disease with adult on-set muscular dystrophy, and he had been reading up on it, “fpr example, did you know that the genetic marker is dormant in one-third of people their entire lifetimes?” Heexamined me, and at the end of the exam made this speech, “I think you have localized scleroderma, None of the medications for scleroderma work, so I wouldn't prescribe any of them for you, the only people I see getting well are those on ap, so I would advise you to continue doing it, and I predict that as your scleroderma gets better, that your FSH will reverse.” I could see that the neurologist was ready to burst, so I quickly added that I had responded to Dr. C's prediction by stating that his prediction did not make scientific sense, so what was he basing that on? Dr. C. said the thought it would reverse because that is what he was seeing in his other FSH patient with autoimmune disease. (Yes, I consciously set him up) The neurologist was clearly flustered, 'this would only make sense if the fsh caused the scleroderma, did he say that?' No, I explained how autoimmune diseases are bacteria in our white blood cells, and that the two are separate issues.

I told the neurologist how my symptoms of scleroedema had improved, and as they improved, my muscles that he said were impacted by FSH were coming back. He actually examined my shoulders at that point, and found them to be healthy. He asked me to raise my arms. My left arm was normal, my right impaired. I explained to him how I perceived that as the antibiotics were working, the muscles in my right shoulder were changing. I felt that the scleroedema was actually forcing my shoulder joint, and the joints in other parts of my body apart. As the antibiotics work, the swelling goes away, and as it does, I regain use of my muscles. He pondered this. I explained that my physical therapist and husband had noticed that those brown spots he had observed on my back and neck were going away, and as they did, I was feeling my muscles come back to life. I told him how I would feel them working, ask for exercises to strengthen them, and then work them. I demonstrated that my right shoulder blade still pops up, but that I can think it back into place.

The neurologist asked how often I go to physical therapy. Every day, I told him. He was aghast, that's too much exercise, do you do the same exercises every day? No, I told him, I joined the cardio class on Tuesdays and Thursdays two months ago, and on Mondays, Wednesdays and Fridays I do my regular exercises. He asked how long I exercise, and I told him two hours a day. He thought that was too much; I told him I didn't think so. I told him that I really don't expect to get full recovery from the fsh, but that I have absolutely nothing to lose by trying. He conceded that, and I looked him in the eye, and said, “Dr. S., I'm a fighter.” He chuckled and said, “Yes, you are.”

He suggested I continue doing what I'm doing. He said he needs to do some research about this, and he'd really like to see me again in six months.

This is the most fun I've had for a $20. co-pay ever.

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