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.