Approval!

Well, we're all looking for a little approval in life.  And while I may not get much--mostly groans at the dinner table (yes, you have to eat this), funny looks from strangers when I dress like a bag lady (sorry--but even in Southern California I've gotta protect these fingers from the cold), and a distinct lack of credit card offers of late (I can't even get pre-approved anymore)--I got my big approval.

My insurance company has approved the stem cell transplant, and I have tentative dates for my time in Chicago this summer.  We're working out the details, but it looks like I will head back in June for pre-testing, undergo mobilization and cell harvesting in July, and receive the transplant in August.

Hoping all goes well this week--I'm off to UCLA and to a San Diego pulmonologist for more approvals.

Oh, and can you believe how big the boys are getting??  That's us in Palm Desert for a tennis tournament a few weeks back.  The boys both played great and both made the semi-finals!

Crossover

After a quick trip to Chicago, the preliminary results are back.  The researcher, Dr. Burt, who is conducting the ASSIST trial has evaluated my pulmonary function testing and skin score testing and, under study protocol, I am moving into the crossover group.  

As I mentioned a few days back, the study protocol are clear for a member of the control Cytoxan group to move into the experimental stem cell transplant group.  Skin score must worsen by at least 25% or lung function must decline by an additional 10%.  Unfortunately, since my last visit in late August, my skin score has more than doubled--a worsening of 140%.  My lung function has declined about 20% since my initial Chicago evaluation.  Sadly, I more than meet criteria for the crossover to the stem cell transplant arm of the study.

So, what happens next?  I did have other testing done while I was in Chicago and I do not yet have these results.  If my cardiac function declines, I may not be able to receive the stem cell transplant.  The study is quite strict about cardiac function, and I have a heart defect (atrial septal defect) and some questionable heart thickening which could keep me from the transplant.  That will remain in question until just prior to transplant, as I will need to return to Chicago for another heart catheterization, a cardiac MRI, and additional heart stress testing before I could be cleared.  

My insurance will need to re-approve the procedure.  This takes time.  I was approved last year, but did not have the transplant, so we may have to start the process over.

I will see my local rheumatologist and my specialist at UCLA for additional opinions about my options.  The key, I think, is to make sure that reasonable options are exhausted but not wait too long.  Dr. Burt admonished me about my personal risk in waiting too long, as my heart is the big question to him.  The greater the damage to my lungs, the greater the strain on my heart, and the greater likelihood that I will not be able to receive the transplant.

He and his nurse also reviewed some of their latest transplant data, which were more mixed than the review of a year ago.  Some patients are having recurrent scleroderma problems about 2 years after transplant.  Some continue to do well.  Patients who are still "high functioning" like me are less likely to see dramatic improvement, while those who are, for example, already wheelchair bound might find themselves able to walk and drive again.  This certainly is a more dramatic response than what I can expect, which would be to hope for stabilization of lung and musculoskeletal function.

My greatest sadness at the visit was a discussion about my horseback riding.  I feel slighted still that nearly 3 years ago I was forbidden to play tennis.  Now, my doctor has said that for a period of 4-6 months at minimum after transplant I will not be able to visit a stable.  Apparently, there is a fair amount of fungus in hay, soil, and airborne at stables, which can be inhaled and create significant health problems for those with brand new immune systems.  Hence, I will be restricted from riding and visiting my horse.  I have thought a great deal about this issue today, and I think I will put Atlas up for sale.  I hate to think of him missing another year of the show circuit--he's not getting younger and he's an amazing athlete.

More than ever, I would love to hear back from you.  Let me know what you think about my options.  Let me know what you think I should do about my beautiful horse.  I'd love to hear from you.

Rituximab Shows Promise in Scleroderma!

Exciting news in Scleroderma land! :)  A small "proof-of-principle" study published in the February issue of Rheumatology showed exciting preliminary results.  Patients who received one year of rituximab treatment (a monoclonal antibody used for some cancer and autoimmune disease treatments) had significantly better lung function than control patients.  Check out this link for more information:
http://www.medpagetoday.com/Rheumatology/GeneralRheumatology/18352

It's always exciting to see that research is being done on this nasty ol' disease, and it's because of people like you who support research that we see progress being made.  Thanks!

Speaking of progress, I am heading to Chicago for my check-up tomorrow.  My pain has been somewhat less this week, and I'm excited to get more good news.  Wish me luck!

"To the pain"

I'm not a movie buff, but I have to say that there are some great movies out there.  Steve and I seem to be drawn to comedies, especially when quoting flicks.  "Looks good on you."  "But does it go to 11?"  It's pretty sad when Caddy Shack and Spinal Tap pass for culture, but heck... we're Californians!

Lately, I've been thinking about one of my favorite movies, The Princess Bride.  When Humperdinck suggests a duel to the death, Westley declines.  He describes a far worse fate... a duel "to the pain."

After my experiences of the past few weeks, I have a new appreciation for pain.  Fortunately, my pain subsided significantly for several days since my last posting.  It was a welcome relief.  For the past week or so, I have once again experienced increasing pain in the mornings and evenings, but it has not been quite as dramatic as early February.  Nonetheless, I have trouble with mundane tasks which didn't bother me even a month or two ago.

I will be heading back to Chicago next week for my 6 month reevaluation in the stem cell transplant trial.  I have been assigned to the control arm, and received 8 months of high dose IV Cytoxan.  I chose this trial because, unlike most trials, there is an opportunity to "cross over" to the experimental arm if my condition significantly worsens.  I find myself in a bit of a spot.  What I really want is to be well, and what I don't want is to be worse.  But, given that I feel so much worse, I occasionally find myself wondering about and even wishing for the stem cell transplant.

My pain, my joints, and my other symptoms won't have any impact on the cross over.  The study criteria are quite clear and quite strict.  I would need to have a 25% worsening of my skin score or a persistent and further 10% decline in lung function.  Skin score and lung function are good indicators of prognosis in scleroderma, while arthritis, tendonitis, bursitis--while inconvenient--do not impact my life expectancy.  Because stem cell transplant carries a high risk, and a real (although modest) chance of death, only these life threatening complications will impact the researcher's decision on whether I stay on traditional therapy or move into the stem cell arm of the trial.

My local doctors are working to tweak my treatment to help my symptoms in the meantime.  Wish us luck in the never ending battle for the prior authorization!

"Hold it, hold it! What is this? Are you tryin' to trick me? Where's the sports? Is this a kissing book?"  TPB

Progress and Pain

Both of my boys have birthdays in January, so it has been a very busy month!  Jake chose to have his party at a Laser Tag spot, and Luke chose to have his party at home.

For Luke's event, the kids participated in a photo scavenger hunt, wandering the neighborhood with cameras (and adult supervision) to try to find a number of unusual items on a long list.  :)  The photo here is one of my favorties.

Progress: the kids are another year older, and they're doing well.

Sadly, I was not able to walk along with the kids.  This week in particular, my pain has been quite brutal, especially in the afternoons and at night.  I am having trouble walking, navigating stairs, brushing my hair or teeth, opening my pill bottles, pushing buttons, and so on.  It's kept me from writing in my blog (although I have voice recognition software, just using the mouse has been trouble).  I feel particularly hypochondriacal when I see how much I can do between about 9 am and noon.  I am still able to exercise, type a bit, shower and wash my hair, and so forth without much difficulty.  My joints are stiff and sore in the mornings, but I feel like a cripple later in the day.

My doctors are on the case.  They plan to change my meds and I am going to try a new rub-in joint cream. I head to Chicago on March 2 for a review in the trial, and to see where I stand with respect to the lung and skin issues.  I had hoped to gain at least a couple of years of trouble-free existence after the Cytoxan infusions, but my optimism is fading in that respect.  Dr. Burt (who runs the Chicago trial) was kind enough to call me on Friday, however, and discuss my situation.  He is open to allowing me to take just about any immunosuppressant medication that my local doctors recommend, so that is a relief and my local rheumatologist is already seeking approval from the insurance company for a new injectable.

Progress:  Dr. Burt visited San Diego this week and gave the Grand Rounds lecture at Scripps Clinic.  I am told he was very well-received, and generated a great deal of enthusiasm for his work doing stem cell transplants in autoimmune diseases like MS and diabetes.  Let's hope we see equally good results in systemic scleroderma.

*This is my second post on the new blog, so be sure to check out January's news!  I just got everything up and running, though... sorry that you didn't get a chance to see it earlier.