Thursday, February 1, 2007

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.


Teresa said...

Like the blog - how long did it take for you to get the appt with the JHU clinic?

Scleroderma Blog said...

I emailed JH on 10/16 and received a reply on 10/17. The doctor I wanted to see was unavailable for a couple months and I was able to get one of the earliest appointments when he returned - 1/4.

I don't know if that is typical or just lucky timing on my part. My parents happen to know someone who's wife also has scleroderma and is treated at JH so that might have had something to do with it, but I can't be sure.