Tuesday, January 6, 2009

2009 update - Johns Hopkins visit

Happy New Year all !!! I hope you all had a happy holiday too. Today was my latest visit to the Johns Hopkins Scleroderma Center in Baltimore, MD. I left early this morning and got there in plenty of time for my 10am appointment. My blood pressure and weight are all normal. Too bad I’m sick or I’d be a heck of a healthy 45 year old guy. Dr Wigley came in and we chatted about the past 6 months since my last visit. We reviewed the medication I’m on, any changes in my condition (skin, breathing, pain, etc). For everything he asked, he checked off “normal” on his long checklist.

Dr Wigley then examined me from head to toe checking the skin on my fingers, hands, arms, face, chest, abdomen, legs and feet. He said everything looks fabulous. After I got dressed, he came back in and his first question to me was “What do you think about stopping the CellCept?”. That’s the medication that is suppressing my immune system so it doesn’t over produce collagen, which is what was happening and the cause of all the damage. I didn’t answer right away and he quickly jumped in with “I know it’s a pretty tough decision to stop taking something which has helped so much.” I asked what is the downside of continuing taking it and he indicated there’s a higher risk of some cancers. The immune system, which we’re suppressing with the CellCept is also responsible for good things, like preventing cancer. He indicated that about 80% of the folks that stop taking the medication do not see any additional flare ups, while 20% that stop the medication do see some flare up. To put it differently, from this patient’s viewpoint, there’s a 1 in 5 chance I could get extremely sick again. So what we decided was to get a Pulmonary Function Test (PFT) to check my lungs as well as a catscan to check for pulmonary hypertension, both which I do at least once a year. If they come back normal (and all previous times they have), we’ll discuss either stopping the medication or going to ½ the dosage I have now, which is 1 pill twice a day (down from 3 pills twice a day at peak). He indicated that we’ll make the decision after the winter is over.

After we finished up, I had to walk for 6 minutes for one of the research studies they are doing. They check your breathing after the 6 minutes. The woman indicated I walk the farthest of all her patients. She tracks how many “laps” I do. I asked her how far and she told me the number of laps. So then I said it was about 10 yards (golf paces) and each lap is a back and forth for 20 yards. She looked at me oddly (nothing new there) that I would be able to measure distance in yards by taking steps. I guess she never played golf.

So physically, it seems I’m doing great. Of course I still have the damage done during the first year before things started to calm down, and I’m hopeful that over time that might improve on its own or some medication is found to help the body repair itself. We’ll just have to see. I’m not thrilled with stopping the medication, and that’s coming from someone that never took medication for anything before all this started. So we’ll see how that goes. I head back there on July 15th for my next appointment. Even the receptionist commented on how well everything is going and they don’t get to see me again for 6 months.

As for the family, we had a tremendous holiday. My wife's parents were up from NC for Christmas and we spent a lot of fun time with them and her brother’s and sister’s families. From December 29th through January 3rd, we were up at Mount Snow in Vermont. My wife, my girls and I and our good friends had a blast skiing for 4 days. The temps were pretty normal (teens or twenties) except for New Years Day when it was -32 degrees Fahrenheit at the summit!!! It was the coldest I think I was ever outside for. I only skied two runs before calling it quits that day. The other days we skied for a much longer time. Hopefully the Giants can win 3 more games to finish out the season. That will hopefully mean 2 more home games to sit through in the cold, but when they win, it always seems to be a bit warmer.

Stay well all and Go Giants!


bouy said...

I am so glad that you are still doing so well all things considered. If there is such a thing as luck with having a disease, then I hope your's is good and the upcoming medication reduction will work out.
Thanks for the update!

Scleroderma Blog said...

Someone asked if I was ever on minocycline.

The answer is I was not. All medication I've taken specific to addressing the disease is CellCept. Everything else was either for pain/inflamation, reynauds, acid reflux, etc.

Anonymous said...

Did you decide to go off the Cellcept? I see Wiggles tomorrow, and I'm wondering if he is going to suggest me going off. I'm currently on 1,000 mg (guess I'll probably have to go down to 500 mg first). Cellcept saved my life, but I would like to go off it.

Scleroderma Blog said...

Last week I had a Pulmonary Function Test (PFT) and echo cardiogram. I can't tell much from watching the echo but there wasn't any problem in the past. The PFT is pretty easy to compare to previous tests and the "reference" you are shooting for which is 100%. I think all the results were the same or better with this latest PFT.

I emailed the team at Johns Hopkins to be on the lookout for my results. Once they review them they will let me know if I'm off. At this point I think the answer will be yes.

I'm on one 500mg table twice a day. Dr Wigley indicated that going down to 500mg was essentially doing nothing. But I'll wait for the expert to weigh in after they review my latest tests.

Good luck on your visit!

Megan said...

Hello there! Just found your blog. Great information. My husband also sees Wiggy (as we call him) at JHU. We're currently researching starting the cellcept.

Anonymous said...

Just found your blog. I also see Dr. Wigley and have been for 16 years going on 17 years.
I just read the Fiscal Report for 2009 and there are some impressive things happening in search for a cure. My next appt. is in March 2010.
I was diagnosed in 1993 with Scleroderma and in Novemeber with Pulmonary Artery Hypertension-

kathleen said...

Hi - It was encouraging to read your entry and how well you are doing. After a 3 year search, my 24 year old daughter has been diagnosed with scleroderma. She is very ill ( failed esophagus, 70 LB. weight loss and too much pain to use her feeding tube)and we are looking for the best place to take her. It sounds as if JH is one of the top choices Would you concur? Her depression is really scarry and she (we)need to find some hope. Is psych. support part of the care offered? Any info you could send my way would be greatly apreciated. Continued good luck.

Scleroderma Blog said...

All - Happy New Year everyone! May 2010 be better for all of us then 2009!

I have my 6 month visit coming up mid January and I'll post back after that.

Kathleen - I don't know where you are, but if you can get to Scleroderma Center @ JH and they can take you quickly, then sure! If they can't, and some place else can, I'd suggest going to any scleroderma facility that can address your daughter's situation as quickly as possible. I know I went from feeling "odd" to feeling horrible very quickly (over the course of a few months) and though medication did kick in, it took a few months after treatment started for there to be any indication things might stop getting worse.

Do anything you can to get her treatment from someone specializing in the disease. Good luck!!!!