Introduction

If all goes as planned, this blog will keep everyone informed of my wife Jen's successful treatment for Myositis. Myositis is an autoimmune disease where Jen's immune system mistakenly thinks her muscles are an infection. The symptoms are extreme fatigue and muscle loss. Untreated, Myositis would continue to weaken Jen to an extent we don't even want to talk about. For more on Myositis I recommend the Myositis Association's website and related links: http://www.myositis.org/

Jen's treatment started almost 2 years ago with prednisone, which continues today. She has gone through all the "standard" and "slightly more rare" drug options and has received the best care in the world from Dr. Christopher-Stine at Johns Hopkins Medical Center in Baltimore, MD. I can't go through all the details right now, but rest assured that we have tried everything. Jen's doctors are all great; Jen has just been resistant to every treatment. Her immune system continues to attack her muscles at almost the same rate it was 2 years ago. Her CPK numbers (Creatine kinase; a measure of muscle enzyme levels in her blood that indicate the level of ongoing muscle damage) have fluctuated some, but today are only about 50% of where they were 2 years ago. This despite some very strong immunosuppressant drugs. Jen can still walk, but watching her I am continually amazed at how little strength you need to walk once you are standing upright!

Earlier this summer we learned that Jen tested positive for the antibody SRP (Single Recognition Particle), which means she has "anti-SRP positive" Myositis. Folks with this usually get hit hard and fast and are usually much worse off than Jen was at the start. Anti-SRP positive Myositis patients usually respond well to steroids in the beginning and then nothing but Rituxan. Jen took Rituxan (even before the antibody test) but it hasn't done much good. Rituxan was supposed to be the magic drug, but Jen's immune system continues to be in high gear and the muscle weakness continues to progress.

So this brings us (very quickly) to the present. On Tuesday of next week we are going to Johns Hopkins to visit the famous Dr. Brodsky. Dr. Brodsky is famous for developing (with colleagues) a technique called high-dose cyclophosphamide (HiCy). This is a chemotherapy whereby the drug eliminates your entire immune system but leaves stem cells in your bone marrow and blood intact. Stem cells are apparently resistant to cyclophosphamide due to an enzyme that breaks the drug down before it can destroy the cell. These stem cells eventually (beginning about 7 days or so after treatment) "reboot" your immune system. The "new" immune system will hopefully forget that it used to think Jen's muscles were an enemy.

So, on Tuesday we hope to convince Dr. Brodsky that he should try this technique on Jen. As far as we know, no Myositis patient has ever received this treatment. However, Myositis patients have received bone marrow transplants and this is only slightly different (and possibly better.) I think the main reason no one has tried this treatment is that Myositis patients are usually responsive to one of the other treatment options. There are several blogs out there of MS patients who have received HiCy treatment from Dr. Brodsky. Please see Got HiCy? for more information. Also, Chris had MS (and links from there) has been a big help figuring this all out.