A member of the Fantastic Four?

I was discussing scleroderma with someone over the weekend and they described what it must be like as being The Thing from the Fantastic Four. I think she was a bit accurate.

I then saw this newspaper article titled Disease is `like turning to stone' about a young lady with scleroderma and thought I'd post it here.

A so-so weekend for me. Didn't feel too bad, didn't feel great. I really wish the fibrosis in my lower legs would go away. I'm definitely walking slower because of it. I had it in my right forearm on Saturday and a bunch of people were creeped out by it when they could feel it, but it was almost non-existent in my arm yesterday.

My first checkup at Johns Hopkins

My first follow-up visit was this morning, 7 weeks from my initial visit to Johns Hopkins. My wife and the girls were on vacation and met me at the hotel to come along. If the darn valet guy didn't take 25 mins to bring the cars this morning we wouldn't have needed to rush. I need to find a better hotel! Below are the highlights of the visit.

After the quick weight/blood pressure/temperature, Dr Wigley came in and we reviewed my status over the past 7 weeks. "Good", in my case, doesn't necessarily mean things are getting better. "Good" can also mean it's not getting worse. Other then some additional skin tightening on the upper half of my torso, he was very pleased to see no additional hardening of skin elsewhere on my body. That's "good"!

A change that has occurred since I was last there was about 2 weeks ago. I emailed the Dr to say I feel like I have shin splints. He immediately replied back saying that he can't be sure since he's not physically seeing it, but it sounds like fibrosis of the tendon. Well today, we confirmed that it indeed is fibrosis of the tendon. With scleroderma, my skin is getting hard because of excessive collagen being produced. Fibrosis is the generation of excessive fibrosis connective tissue. I have it in both my lower legs, though the left leg hurts more and also in my right forearm. To give you an idea of how odd it is, if you put your hand around my lower forearm and I bend my wrist back and forth, it would feel and sound to you like a creaky door moving inside. This fibrosis is also typical in scleroderma patients and indicates that it is still active. That's not so good.

What he suggested between now and the next visit is to up the dosage a bit on the Cell Cept medication. Cell Cept is the medication they give to transplant patients to prevent rejection of the transplanted organ. In my case, it is being used to try and get my out of whack immune system to stop attacking me. If the fibrosis is still a problem at the next visit, he spoke to us about medication from Novartis called Gleevec that is used in Leukemia patients and has been shown to address fibrosis. They haven't used it enough to get conclusive evidence, but in their initial findings they are seeing positive results. I'm so lucky to be going to a Dr that has dedicated his career to this disease and is aware of all the existing and bleeding edge treatments. We even discussed some of the stem cell research going on with scleroderma patients.

This past Monday (President's Day) I felt better then I had in months. I had no pain in my legs and the fibrosis wasn't bothering me. My wife attributes it to me sitting on my butt over the long weekend - I'm sure she's at least partially correct. As I mentioned previously, "normal" is just the constant discomfort I feel. On Monday it just felt so odd to be able to stand up from sitting in a chair, and it not hurt. If this fibrosis goes away, I'd feel so much better then I felt back in December. I'll keep my fingers crossed (well I would if I could physically cross them) for some more days like Monday.

My next appointment at Johns Hopkins is April 5th. We are still optimistic that the treatment will yield positive results. The next month or so should be a good indication or if we need to consider further adjusting the treatment.

Johns Hopkins Scleroderma Center Take 2

Later today I'll head back down to Johns Hopkins Scleroderma Center for my first follow-up visit tomorrow since my initial visit the beginning of January. It will be 7 weeks tomorrow since my visit and 6.5 weeks since I've been on the medication.

This past Monday (Presidents Day) was the best I felt overall in a long time. Yesterday and today are more "normal" with some pain back in my shins and hips.

I'll work from home today and head to Baltimore late this afternoon.

My aching shins!

Starting about 5 days ago, out of nowhere, my shins started to hurt. Whether it's actually shin splints or not, I don't know, but when I walk, my shins are killing me. I looked up shin splints online and I'm guessing if it has anything to do with scleroderma, it has to do with the connective tissue, one of the causes of shin splints. It's not like I've been jogging lately so I'd lean towards connective tissue as the culprit. Whatever the cause, it hurts like hell!

Scleroderma vs Terrorism

I received an email overnight from a co-worker at my previous job that found out I have scleroderma. I've received many over the past couple months as word spreads, but what was different was this person is in Iraq, fighting the war on terrorism. She wished me good luck and she hopes things turn out well.

I thanked her, told her how ironic it was that someone in Iraq is wishing me good luck with my fight. I wished her the best and for her to return home quickly and most importantly to return home safely.

We're both in the fight for our lives.

How I stay current with Scleroderma

I never heard of scleroderma until I was diagnosed with it. Now that I have it, I'm interested in any news related to scleroderma. So here's the methods I use. If anyone has other methods, please post them.

Yahoo Groups is a community platform where members of individual groups can post/email messages and every other member of the group can view them as individual emails, daily digest emails, or via a web interface. You can go to http://groups.yahoo.com and search on scleroderma. I currently joined the following groups - sclerodermasupport2, sclerodigest & SDWorld.

Another way to stay informed is to sign up for the Scleroderma Foundation weekly newsletter at http://www.scleroderma.org/e_letter_signup.shtm

I also created a Google Alert at http://www.google.com/alerts on scleroderma and had Google send me the alert once a day. It contains the title of news articles and other blog entries that mentioned scleroderma along with a short blurb and link to the actual content on the web.

What a difference a night makes!

I wrote yesterday's Scleroderma Blog entry and my legs were sore as they have been for the past 2 weeks. Went to bed last night, had a great nights sleep unlike the tossing and turning of the previous 2 weeks, and woke up immediately noticing my legs weren't hurting. All day, no issues despite a day where the thermometer didn't rise about 20 degrees. As a friend says, "pleasure is the absence of pain", and today was a pleasure!

So what hurts today?

Before I was diagnosed with Scleroderma, nothing hurt. I was in great shape. Today, most things hurt and that hurt has become my "normal" feeling. When someone asks he how I feel, it's never "good", just "the same".

Other then what I recall being about two weeks soon after visiting Johns Hopkins a month ago, my knees, hips and legs have bothered me. Even sleeping is uncomfortable as I need to constantly change positions. I was hopeful when it didn't bother me for those 2 weeks that either the medication or the disease itself was going to cut me some slack, but it was short lived. But, for those 2 weeks, I felt noticeably better.

I'm hoping another feeling that won't become normal is the tightness of the skin on my upper chest and neck. It doesn't feel as bad today, but for the last week or so, it's been real tight with me only able to life my chin up a little before it pulls the skin on my chest.

My hands have been the same for the past month or so. Not too bad and not too good. Always stiffest in the morning and stiff throughout the day. My wrist bother me for a couple of weeks and hasn't for the past 2.

This scleroderma is a pain.

Johns Hopkins Scleroderma Center

I was very lucky to get an appointment with a top scleroderma specialist, Dr Wigley, who was, in one word, AMAZING!

What I report below is my understanding/interpretation of what occurred. Even if it's 100% accurate, it's what I was told and it relates to my involvement with disease which may be completely different then another individuals.

I arrived at the center in Baltimore, MD 30 mins before my appointment. I had sent in a bunch of paperwork they had sent me a month before so there was minimal paperwork to do when I got there. Some of the paperwork was providing permission for participating in clinical studies.

They weighed me, took my blood pressure and I went into an exam room with my wife. The first person to come in was a woman to discuss the various clinical studies underway and if I had any questions. She gave me her business card which included her phone number and email address.

After a few minutes the Dr came in. He introduced himself, gave me his card with phone number and email and reviewed my medical history, tests I had taken, and my experience so far with the symptoms of scleroderma. I was given an exam by the Dr, checking my extremities, skin, etc.

What happened next was my wife and I were given a top not education in scleroderma. He started out stating that each and every patient was different and there's no magic formula.

The Dr explained that there's an active state of the disease which typically (not 100% but typically) lasts 1.5-2 years and that's where most of the damage is done. Then the disease plateaus and there's even instances where the skin starts to recover and regain some of its elasticity. Amazing that in all the sites describing the disease none of this was mentioned.

With the above info, the goal is to get the disease to plateau as soon as possible. The Dr then reviewed what they know about the disease and the interaction between the various internal anti-bodies, fibroblasters, etc. He described what part they current target and what the clinical studies are trying to do in determining other areas to target. He also explained autoimmune diseases and the trouble in coming up with cures/treatments.

The Dr then reviewed the various organs that are typically impacted and what tests should be done to monitor them. He started with the lungs and explained what scleroderma can do. In my case there's no detectable lung involvement and he recommended follow-up PFTs every 4 months.

Next was the GI system - esophagus, stomach & intestines. Smart eating is important and an anti-reflux medication like I'm on is used to prevent damage.

About 10% of patients experience a problem with their kidneys. He suggested I monitor my blood pressure to make sure it remains at an acceptable level. If it spikes, it could be a sign of my kidney having an issue.

He reviewed what my rheumatologist explained regarding raynauds. He suggested I take a baby aspirin every day to go along with the raynauds medication.

We discussed the muscle/joints/skin and this is where we reviewed the options for treatment. The first option is just to watch, which I wasn't interested in. Next he reviewed 3 treatments of available medication. We selected using CellCept. CellCept is a medication that transplant patients use to help the body not reject the transplanted organ. What it does for me is try to convince the immune system, which is confused, into not thinking it needs to "help" me, which in turn, because I don't need any help, hurts me. The third option was more radical treatment and not recommended for someone with my current involvement of the disease.

Lastly we talked about pain and for now prescribed some strong ibuprofen.

His nurse came in, introduced herself, gave me her card with phone number and email. Whenever I have tests done, I need to email her so she is on the lookout for the results.

After the exam, I gave 12 viles of blood. Some were tests for me and some were for use in the clinical studies. I finished by scheduling the next follow up, about 6 weeks from the initial exam. Everyone in the scleroderma center couldn't have been nicer. It was my first visit and I feel as if I've been there 10 times. My wife and I left feeling there was hope in getting it under control and not having it be the focus of our daily life. The Dr spent 2.5 hours with us, answering

I'm sure not everyone can get appointment with a top specialist, but if you can get to the Scleroderma Center at Johns Hopkins, you will receive top notch care, from caring individuals that want to help you.