Open letter to my Parents

realize there’s some confusion right now about my test results and what everything means. I’ll try to break it down for you as best I can, as I understand it.

Myelofibrosis is broken down into two types: Primary and Secondary.

Originally, Dr. Carroll thought I had primary idiopathic myelofibrosis, which is a pre-malignancy blood cancer. This illness progresses by the bone marrow becoming scarred and fibrosed – essentially turning from a liquid (normal) to a solid (not normal). Bone marrow produces your red and white blood cells and the scarring stops/lowers the production. The only known “cure” is a bone marrow transplant. There is a way to keep the red blood cells alive longer, but no one really knows why it works. This treatment includes thalidomide-type drugs. That is what we were going to do.

However, when I told him about my autoimmune illnesses, Dr. Carroll made a leap of faith and gave me an immune suppressant called cyclosporine.  In his mind, if i had primary, it would keep my blood alive longer. If i had secondary, it would heal my marrow and keep my blood alive longer. As you know, it healed my marrow, which means I do NOT HAVE primary myelofibrosis because primary would NOT EVER have gotten better.

Therefore, I have secondary myelofibrosis. Secondary myelofibrosis is secondary to another illness. Originally, he had ruled this out because usually, it is secondary to another cancer such as myeloma or lymphoma. He had tested these for me and I did not have them. That is because in this case it is secondary to an autoimmune connective tissue disorder. More on that below. Anyway, when myelofibrosis is secondary, it means it is brought on BY that other illness. Therefore, if you treat the illness that brings it on, it actually gets better and your bone marrow becomes liquid again. This could not happen in primary myelofibrosis.  Does this mean I don’t need the bone marrow transplant and I have been cured? No – but it is indefinately on hold.  The plan is that we keep me on cyclosporine for as long as we can. This treatment cannot last forever – it can cause severe kidney damage and also it suppresses my white blood cells which makes it harder for me to fight off infection. In time, i will need to get off this drug. We have no idea how long. The bone marrow transplant option still has to be a very last resort option to reset my immune system and save my life should there become worse complications down the line either because of the drug treatment or because of other unforeseen problems.

So what else can we do other than continue this cyclosporine? We need to find out what the underlying illness is to determine that. We now know it is a “connective tissue disorder.”

There are five known connective tissue disorders.
1- lupus
2 – osteoarthritis
3 – scleroderma
4 – sjogens syndrome
5 – a mixture of these known as mctd

I do not have 3/4/5. Scleroderma is a hardening of the skin and sjogens involves tear ducts and saliva. Due to the fact that Dr. Friedman of Cedar-Sinai found positive testing for systemic lupus back 5-6 years ago in collagen testing, that is likely the culprit. I meet with Dr. Snyder on July 17 and then Dr. Carroll wants to see me again. The two of them are going to float thoughts between each other as to how to proceed diagnostically to see what it is I have, how it can be treated, and what are my options for long-term care and treatment.

Altogether this is actually great news because it means less likelihood of a bone marrow transplant and of death. However, my gut instinct is that lupus is not the cause of all my problems but likely yet another symptom of a greater cause. At some point in my life the autoimmune illness just “switched on” in me and my body turned on itself. Why did that happen? Likely my mononucleosis and monohepatitis in college was the traumatic experience that set this switch on. Before that, I was genetically predisposed for it. I have been told this was due to a deformity in one of my chromosomes, #21, and that it was not due to my parents but an actual genetic defect. It is possible you folks have it too, but obviously I am apparently more susceptible or my body simply is more actively destroying itself. In any case, the cyclosporine is working and I am healthier (ironically) than I have been in years.

Anyway, hope that explains it.