Science and the Scleroderma Girl: Hard Choices (Part 2)

Last week I wrote a post about trying to make a good decision about what drug I should be treated with for my systemic sclerosis. My rheumatologist had offered me methotrexate and CellCept; after trying to gather info about the drugs and their symptoms I unhappily picked methotrexate.

Cat
the Mother of Cats chose badly…

The first weeks of the drug seemed okay. I had a couple of hard days after taking my dose on Monday, but then I would feel much better for the rest of the week as the pain and brain fog receded for several days. The crazy thing was, my knees hurt REALLY badly during those two bad days. I checked online and other people had experience a similar phenomenon, so I soldiered on. Then one week the pain was pretty bad in my lower chest and right side and I was having trouble walking and breathing…

IV in arm.
Off to the ER I went where they hunted for lung damage and blood clots… 

It was an inflammation of the cartilage of my ribs, a condition called costochondritis. I called the rheumatologist’s office to see what I should do next, but didn’t hear back for a couple of days. I then emailed, and called again.

Finally the call came back; I was having a rare bone reaction and needed to stop the methotrexate. He was starting me on CellCept immediately.

Oh, that was an adventure. So much stomach pain… scratch that… stomach fire! Not just my stomach… my intestines were on fire too!! I gulped down spoonfuls of coconut oil trying to baby my stomach lining. I added food with the pills. I started vomiting in the middle of the night. It didn’t matter what I did, my stomach was going to be very upset. I stopped the drug and shot off an email to the rheumatologist again.

Hence began the two month battle to get me onto another form of drug in CellCept, which is mycophenolate mofetil. A stomach gentle version called Myfortic did exist, but it was not approved for systemic sclerosis, so the pharmacy refused to fill it. My rheumatologist filed an appeal. It was denied. My rheumatologist doubled down. Another rejection. Eventually I drove down to the Kaiser pharmacy and talked to the pharmacist as calmly as I could. I reminded him that Myfortic as just another salt of the approved drug, I had failed the approved form, my rheumatologist had appealed for this drug, and that I had a letter on file from another doctor in the Kaiser system stating that my gastritis prevented me from taking NSAIDS or anything else that would damage my stomach lining. I must have looked pathetic, because he gave me the pills.

Three years later I have greatly improved because of this drug that we had to fight for. In the meantime I have discovered (by hunting for info on PubMed Health) that while methotrexate helps with symptoms like inflammation and fatigue, the better choice in the long run is the drug that I am now receiving as it is associated with skin, lung, and heart improvements and better survival rates. I do have a higher lymphoma risk with this drug, and infections are a constant concern, but I think that I’m with the best drug available for me right now.

There is a take home lesson here. If you aren’t happy with your treatment, speak up! I should have contacted that doctor about those hurting knees long before I ended up in the ER. It’s easy to take a passive course when you are dealing with busy doctors and unhelpful pharmacists, especially when you aren’t sure if your symptoms are significant, but it is worth the time to shoot off a fast email anyway.

And let’s be honest. There are no easy choices, only hard ones. But even a bad choice can be corrected down the road with some luck,  persistence, and a dash of science.

Rose
I bet you wondered where the rose was, didn’t you. Here it is, a little beat up by the hot weather, but still looking great!
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The Scleroderma Chronicles: The lung results are in.

Spoiler Alert: More good news!!

Last week I drove across town to a hospital that is connected to my pulmonologist’s Kaiser office building for my pulmonary function test. If you’ve never had one of these, there is a machine that you breathe into, a computer that is calling the shots and a sealed glass booth that isolates you from the outer world. Into the booth I went. Time to get some answers!

As part of the test I used an inhaler to get a big slug of the drug albuterol. Wow. That really helped. I’ve been avoiding my inhaler for months as I would feel just HORRIBLE after using it due to dropping blood pressure.

But I just quit using my blood pressure medications two weeks ago and now it was really obvious that my breathing was much better after using the drug. “Here, you’d better take this diffuser”, said the technician giving me the tests. “You’re going to be using this!”

Wednesday my pulmonologist called me with the results. My lungs are better than they were two years ago! More to the point, my pulmonary arterial pressure is down into normal ranges and there is less leakage (okay, they call it regurgitation… what an ugly thing to say about my heart!) at that heart valve. Woohoo!! The fatal complication that we all thought I was going to have to face down is suddenly off the table. I’m not going onto oxygen. My doctor and I virtually hugged over the phone.

Here’s the deal. I have a second autoimmune disease called Sjogren’s Syndrome that can cause small airway disease in the lungs. My Sjogren’s has been pretty bad this year, and since I responded to the inhaler drug really well it looks like that is what is going on. My doctors focus on my bad boy systemic sclerosis so much that they tend to forget about this other life-altering, but not fatal, condition. This year Sjogren’s has been stabbing me in the back.

“Time to start giving your inhaler a workout!”, my pulmonologist told me. “Then try to get more exercise. Your lungs need to continue their recovery, and we are keeping you on the high dose of your immunosuppressant drug.”

Inhaler and fitbit.
I bought a fitbit yesterday and I plan to use the inhaler daily while I steadily increase my exercise. Next week I’m going gym shopping…

It’s the drug. It absolutely is the new drug that I’ve been taking for the last 2.5 years. It is a new drug for the systemic sclerosis community, one originally developed for organ transplant patients, that is now collecting a body of evidence that shows that it not only slows down the rate of disease, but also allows some reversal and healing to occur by impairing the immune system attack on the lungs.  My heart is better because my lungs are better. In a time when I have been experiencing chest pain and shortness of breath, it was because I was getting better and needed to come off some of my drugs, not because I was getting worse. I am completely blindsided and gob smacked by the unexpected turn of events.

Next week is the Estes Park Wool Market in Estes Park, Colorado. I’m off to the mountains and boy will I be running wild with my BKB Deb. I’m going to pet the alpacas, eat lamb kabobs, and then I am going to buy a boat load of yarn. My new fitbit will be getting a workout!

After that I’m going to see my internist so she can look at the big picture with me to see what else we’re missing. There’s another drug that I want to drop…

That will be another post.