Houston Trip to See My Hero - Dr. McClain

The trip to Houston was well worth it. I finally sat face to face with a man who not only knows about this disease but has 500 active patients with Histiocytosis (LCH). Most of those are children and I am still in the rare category. I am only 1 of 10 adults with this exact problem that he is treating. But, I have always been the "only one" my doctor's had so, this is a refreshing change. I am not alone in this anymore. As for managing that many patients, he did admit it can be over-whelming at times and could use more help but he already has Dr. Allen, who is 20 years younger than him and instrumental in the research of this disease because his background is a little different than Dr. McClain's.

So, we arrived at the Doctor's office a little early but at 2:33, 3 minutes passed my appointment time, I was called back. A nurse checked my vitals while the famous Dr. McClain was hovering at the door waiting for her to finish so that he could come in. He's nothing if he isn't prompt. He even told us later that he doesn't like to keep his patients waiting. Finally, here I was, starring at the man who can give me all the answers - if they exist. He seemed just as privileged to meet me as I him. He added that this is the reaction he gets from most of his patients. He had a large folder in his hands with all my records (from mailing them, I know they weighed 1 pound.) He said, I have read through your history, but I would like to hear about it from you. So, I started at the scary beginning when I was 21 years old and woke up from a reconstructive knee surgery with a severe case of Diabetes Insipidus (DI) and talked through the next 6 months of symptoms that pointed to other pituitary damage and finally the removal of a skull lesion that was biopsied and confirmed to be LCH. Then the highlight of my life, the conception of Twins, a daughter and signs that the DI had improved. "Spontaneous Remission" they call this. (FYI - he doesn't think the disease was caused by my knee surgery; just a coincidence)

I then jumped to what I now know as my first sign of this disease being active again, 2006, when my right arm when numb and the hand was not functioning normally. I saw several doctors, did nerve testing and an MRI of my arm, they couldn't figure out what caused it and I got tired of going to doctors. It gradually improved and after a year, the feeling started coming back. After that, it was little things I started noticing, like balance, memory, speech, fine motor skills...all of these things declining. Surfacing with irregular occurrences but still a little more than what I thought was normal for my age. I was still able to conceal it but observed myself more carefully. Fast forward to the fall of 2009. I began to get headaches that became migraines and my fine motor skills in my right hand...became noticeably worse. I started having trouble with things like putting on eye-liner and lipstick. Fatigue was also more frequent. I just didn't feel the same. So, I mentioned these things and a few other details to my Oncologist at my annual check up in January 2010and she ordered the MRI which revealed "hyper-intensive activity on the brain stem".

I was told by a neurologist in February that I have lesions on my brain, these are not tumors, they are lesions. It is a concentration of these histiocytes damaging the nerves in my brain. But I was also told, that we couldn't do anything about it - we would just watch it. This scared me more than anything, because I didn't want to just "watch" this thing shut my body down piece by piece. This is when I started looking for answers and found Dr. McClain.

Dr. McClain knows exactly what has happened. One percent of the patients who have lesions on their skull, get this on their Central Nervous System (CNS). This is a result of the bone tumor I had when I was 21. I am now in that 1 percent. He only has 9 other adults like me. He has had more children with this and has only been treating adults for 4 years. The longest he has followed up with a patient with this (LCH-CNS) treatment is 8 years. Specifically, a child he started treating at 8 years old. That child is now 16, his fine motor skills have improved and so far, the disease has not returned. He said that CNS involvement seems to occur 3 to 7 years after the presence of the skull lesions but there is no way to tell exactly how long mine has been active. We just have to go with what we know and that is at least 5 years, according to my 2005 MRI. You didn't miss anything. I didn't mention that the retrospective look at my 2005 MRI reveals some of these lesions but they weren't noted then. David asked for clarification on his diagnosis and he said "without a doubt, this is a classic case of Histiocytosis and the MRI of the brain and his own physical exam are conclusive for this now being active in my brain." He described it as an over-production of the "BAD GUYS" in the white blood cells. These destructive cells can attack anything in the body. He also said I am unique because my DI seems to have improved and I was still able to conceive. Most people with DI have to be on medicine their whole lives and I haven't been on mine for years...so this is a sign that the body can repair itself. And it seems my body keeps repairing itself.

So, what now? He is the doctor who has developed a treatment protocol that has been used enough to be proven effective. He will advise my doctor in Orlando and over-see my treatment from Texas. It is a mild chemo - like cancer is treated but, this is NOT cancer. One week on, 3 weeks off, for 12 months. The 1 week on will be out patient, once a day, for 5 days, 1 hour a day. There are some side effects but we will know more once I actually start treatment. The side effects he is aware of are mostly nausea and fatigue. Like any other chemo treatment, my oncologist will monitor my blood levels and advise me as to the status of my immune system throughout the treatment. He plans to start a low dose and dial up from there, pending my tolerance. To his surprise, adults can't handle the same dosage as kids. I will have a spinal tap before I start treatment and one at the end of treatment. This will help them study the histiocytes in the blood. He also recommended that I have an endoscopy to help determine what is now happening with my esophagus and constant acid reflux problem. I have to be honest, I don't expect this year to be easy, but I am ready. I have lots of support!!!

Finally, he feels we have caught this early and said it is good that I took the proactive approach to finding answers. He also believes this treatment will stop it anywhere in my body. So, if it is active somewhere else that we haven't detected yet, this will stop it. He agreed that with physical therapy, it is possible to repair any physical damage. He even approved my use of the Wii Fit to help with the process. Look out kids! Mommy has to go to physical Wii-therapy! :)

Over-all, I have been validated. I feel like I am in good hands now and though this year might be a little tough on me and the family, it is with a good purpose. I refuse to stand by passively and let some silly disease have the best of me...I will stand up against it and fight it off will all my might!