ME AND SCLERODERMA
MY LIFE IN A NUTSHELL, OR IS THAT AS A NUTSHELL
I forgot, this isn't just my family and close friends who read this. There are others out there that don't know me that are reading this blog, and don't know what I've gone thru the past few years, and what I'm still dealing with today.
WARNING: This post is long, and it may be a little long winded... I'm hoping i got the gist of what's going on help those of you that may be confused.
I have a very boring life. I'm sorry, but I do. I don't work, i don't go to school, i sit around on my computer all day long playing on the internet.
I was diagnosed with an autoimmune disease called Scleroderma August 16, 2001. Scleroderma means "Hard Skin". My body produces too much collagen. Collagen is that lovely stuff that is in your skin that makes you pretty and not have wrinkles. Well when the collagen is in your skin, and your body overproduces it, and has no where else to go, it will eventually go to other parts of your body. It will go into your muscles, tissue, and then finally your internal organs.
When I was initially diagnosed I had progressed pretty fast. I had lost about 60-80 lbs in the matter of months. My hands were doing funny things. When I got cold they would turn white, and then blue, and take forever to heat up. I felt like an 80 year old woman, and was tired all the time. I walked into the doctor in May and they didn't have a clue on what was going wrong. They did a whole lot of blood work and finally came back to me with. "we don't know what's wrong, but your blood work showed a high ANA, so we set you up with a Rheumatologist, however the first available appointment is August."
I went about my life and finally when August came i went to see the new doctor. He took one look at me and told me what was wrong with me. In all of like the first 2 minutes I was there. He told me that I had Scleroderma. I was amazed. He told me that he didn't know much about the disease, but to go home and research it on the internet. He also told me that there was no treatment and no cure.
For a while there I was just in limbo... I had my good days and my bad, and it continued this way for about a year, then i went into a type of remission. I stayed in remission for almost 5 years.
In about January of 2007 I started having problems again. I was having a hard time breathing, I was getting finger ulcers (sores on my fingers that don't heal and hurt soooo bad) and i was having weird problems with my stomach.
I hadn't seen a Rheumatologist in just over a year. I had moved to Albuquerque in May of 2005. I saw doctor down there when i established myself, but then moved back home to Utah in June of 2006. When I came home I was feeling good. But then winter came, and left, and I wasn't doing so good anymore.
I went to my PCP (personal care physician) and we started running tests on my stomach and blood work and he kept telling me i really needed to see my Rheumatologist, and then when the ulcers started appearing he really wanted me to see my Rheumatologist again. I called the one that I had been seeing before I left Utah, but there was a wait of about 4 months to get into see Him. Dr. Weeks wanted me to see someone sooner. His office made me an appointment to see a new doctor over at McKay Dee. They fit me in within the next week, and I'm happy he did.
I had my first appointment with Dr. Dames in June. She set me up with a Pulmonologist that she works with and I started seeing him in August. After CT scans and a bronchial scope I was diagnosed with Pulmonary Fibrosis, related to the scleroderma on August 15, 2007.
Dr. Dames had all these ideas for me. We went thru testing to try to get into the SCOT trial, which was a stem cell transplant, or strong dosages of chemotherapy. We did all of that in September and in October I came down to Arizona to go to the Mayo Clinic in Scottsdale to do more testing to see if I could become part of the trial. During all of this we found out that in the 2 months since I was diagnosed with PF, my lungs had actually gotten worse, and I was no longer eligible for the trial.
I got home to Utah, from Arizona on the 30th or 31st of October. I woke up the morning we left Arizona with a cold. We had stayed with my brother and his family while we were here and the kids had a little bit of congestion while we were there, so I wasn't worried about it. It would last a few days and I'd be good as new. On November 4th I went into the ER not being able to breathe. They took my pulse ox levels and they were really low. I was put on Oxygen there. I was admitted into the hospital for a few days for Viral Pneumonia.
When I left the hospital I left with a tank of Oxygen and a script to be on oxygen 24 hours a day 7 days a week. I wasn't happy about this. Neither were my doctors.
After not being accepted into the trial Dr. Dames and Dr. Ivester decided we needed to do something else, and I had one of 2 choices. We could do high dosages of Cytoxan (a chemotherapy drug that is in experimental stages for treatment of Auto Immune Diseases) or I could go the route of Cell-Cept (an immuno-suppresant used on patients after kidney transplants).
After discussing it we decided to go the way of Cytoxan for 6 months, then re-evaluate. If it was working we would go another 6 months. If it wasn't we'd start on Cell-Cept.
January of 2008 I started Chemo. It wasn't fun. I just had to go once a month, but that was enough. I would start the day before the treatment with drinking water, lots and lots of water, then I would go to the hospital for my treatment the next morning, and spend 6 hours in a chair with an IV. They would first take blood for tests that needed to be run, checking liver functions, and kidney functions, as well as a white blood count along with other stuff. Then I would have a bag of saline for 15 min, then they would run a drug to protect my kidneys and bladder from the cytoxan for an hour. after that hour I would get the cytoxan for 3 hours, then again with the drug the protection drug (i can't think of the name right now) and then 15 min of saline again. 7 to 10 days later I would go back to the hospital to have my white blood count ran.
I did this in January, then again in February. My white blood count didn't go down with the last dosage, so they upped it. Went thru the 6 hour cycle, 7 to 10 days later with the CBC, and then return again in March.
March my count didn't go down again, so the dosage was upped again. Went back in April, count went down, but not as low as they want. Dosage was upped again. May my count didnt' go down, but I had basically topped out on what they wanted to give me dosage wise, so I stayed the same as April. June my count went up... yes, you heard me right. My count went UP! It isn't supposed to do that when your on a drug that is trying to kill your immune system. End of June we did the tests all over again and such... did my PFT's (pulmonary function tests) had a echo done on my heart (which i have to have done every 6 months to make sure I don't develop Pulmonary Hypertension), blood tests... The works...
I got worse on 6 months of chemo. My lungs got worse, I felt like crap, I felt sick, I was constantly green, and i just didn't want to do anything. Oh, and I had gained 20 lbs. How you do that when your not eating I don't know. however... after June's treatment, my white cell count finally dropped. Yeah... funny thing.
July I started Cell-Cept. I don't remember what the dosage was when I started. I know that within 6 weeks I was on the current dosage I am on.
2 months I got all the tests re-done just. Everything was about a month or 2 earlier than it should have been, but Dr. Dames wanted a new base line before I moved, so that my new Doctors would know where I was.
My Pulmonary Levels on my heart went down (i was borderline pulmonary hypertension)
My Pulmonary Function Tests went up... Meaning my lungs got better.
I have wrinkles on my hand. Because of the problems with my hands, and this disease and with my skin being thicker, and especially noticeable on my hands I haven't had wrinkles on my hands... you know when you bend your wrist to give a high 5 or when you lay your hands flat on something and your fingers have wrinkles... I didn't have those. I do now.
Since we've moved to Arizona I haven't had to use my Oxygen. I can breathe down here. The machine is still in my closet in case I ever need to use it, but I don't HAVE to use it.
I continue to take 4 little pills a day, okay not so little, and they are basically saving my life. I am hoping to one day go back into remission again and not have to deal with all these health issues anymore. But at least I'm doing okay right now. I still have to be careful because I am on an immuno-suppresant and my immune system isn't as strong as it once was. I catch colds much easier, and they last longer, and there is a chance that I could catch something and get very sick. But that's not something that I really worry about these days.
So... There's my story. If you have any questions feel free to ask me. I'll answer them to the best of my ability. But that's my life in a nutshell.
So now that you've read my story let me update you on the last 2 years...
I'm no longer on Cellcept. My insurance would no longer cover it. I'm now on Imuran. It hasn't helped as much as Cellcept, but it's at least keeping things at bay. I was only on 50 mg a day, but I'm now up to 150 mg a day. This is more because the arthritis is getting worse, not my lungs or my heart. My doctor doesn't want to up my steroids (prednisone) so upping the Imuran must be better?
I've been put back on Oxygen, 16 hours a day. So basically from 7 pm to 12 pm I'm on the oxygen tank. HATE IT!
I've been having lots of problems with really dry skin, and dry patches. We are looking into lots of different things for that, so if you have suggestions they are greatly appreciated.
Health wise that's all that's new. *shrugs*
As of August 2011 I was put on oxygen full time. 24 hours a day, 7 days a week. I'm back to hauling that little silver and green tank with me everywhere I go, and using a backpack instead of my cute purses to carry all my junk. *sigh* I absolutely HATE it... I feel like I'm chained to something that will never leave me.
We are currently running LOTS of tests to see what we can do about all of this. The words Lung Transplant have even been thrown out there. But that would still do nothing for the Scleroderma. Just make it easier for me to live a more productive life. There is nothing they can do about the Scleroderma but help with all the symptoms.
With Olive (my cousin E) here in Arizona with us my life has gotten a little better. I do go out a little more, and that's because I have someone to tag along with, and have someone to go with me to the store and such. And she sure does make me smile.
back in October of last year my pulmonologist (Dr. Ronn) was worried about my heart and the chance of Pulmonary Hypertension. He sent me to a PH expert in the Valley, Dr. Jeremy Feldman.
I had an appointment with him the next week. He looked at my tests, and set me up with a right heart catheter to check out the hypertension issue (which turned out to be borderline and something we will just have to watch), and he mentioned the words LUNG TRANSPLANT. Scared me half to death, still scares me. None of my other doctors have ever talked about this, and those words just meant that I am worse off than I ever thought I was.
He explained it to me this way. I'm not really bad off right now, but I'm bad enough to be put on a transplant list, and hopefully I'll get one before I get really bad. But if I ever do make it to that place where I'm really sick, then it is better to be on the list now than to wait the time it would take to get on the transplant list when I'm in serious need of one.
So, this is not an emergency situation. I'm still doing good. I'm not perfect, and my lungs are getting worse, but I'm still able to function and do things on my own. My diffusion rate is falling fast (lost a whole point in a six month time period), but my lung capacity is still above 70%. That's better off than a lot of people I know.
After a lot of discussion and many appointments with my doctors I've been in contact with the University of Utah Medical Center's Lung Transplant team. I'm waiting on some information and then we are going to set up the first of many appointments in Salt Lake. After the first appointment they will decide then if they think I would do well with a transplant. Then I take a week and do lots of preliminary tests. After I take the tests an I have nothing else wrong with me (like cancer) then all my information will be presented to the Transplant Board. If they say yes, that I'm a good candidate, then they will put me on the transplant list.
UPDATE - 07/08/13
Wow... It's been over a year since I last updated.
We've decided that SLC is not a good fit for me. I'm going to stay in Arizona and I am currently working with St. Joes Hospital to get on the transplant list here. It is just better for me physically, mentally, and economically. I'd also have a lot more people here willing to help me out than if I was in Utah.
Currently we're working with the doctors on my esophageal problems. I go in on Monday for some tests, and again on Tuesday.
I'm still on oxygen 24/7 even tho I hate it. I'm still not in an emergency situation so a transplant is not a must have now thing, but I am going to need one.
My last PFT's are up from the previous one's. I'm actually doing a lot better than I was a year ago.
I'm also more social. I've got a Crochet group that I go to at least once a week. I love it! and I love them. They have become a great group of friends. I've also made a few friends here in Buckeye. I'm doing really well. And enjoying life!