My mother's name is Angelica Ramirez Zuniga. To quote my father, "She doesn't have a bad bone in her body." I completely agree.
Her symptoms began to appear toward the end of my senior year of high school (May of 1992) and grew progressively worse through the summer. My first visit home from college (for Christmas) consisted of a three hour drive to a hospital in San Antonio with my mother in the back seat. I can't find the words to describe the experience-- I imagine it might be akin to sitting in a car with a woman who is giving birth. Only in this case, we did not know why my mother was in so much pain nor did we know if there would ever be an end to it. We were helpless as we watched her convulse from the torture of her muscle spasms, beads of sweat soaking through her clothes.
Doctors had no idea what was wrong with her. Specialists of all sorts worked to treat her symptoms-- muscles cramping so strongly that her knee had fractured, an inability to bend her limbs on demand, pain...
Although the treatment her doctors were able to fashion together reduced the pain she experienced, it was not able to restore any independence. My mother could not bathe herself, take herself to the restroom, or even turn herself in bed.
After my Freshman finals (June 1993), I got a call from my father. "Mija-- You have to come home quick." My mother was in the hospital and had been given only a 10-12% chance of recovery. He related what the doctors had said-- One of the medications that had been prescribed to handle her symptoms required liver function monitoring; Unfortunately, my parents had not been told and my mother's liver had begun to deteriorate and was dangerously close to failing.
With a removal of the medication, great hospital care, and liver transplant teams at the ready, my mother's own liver stopped deteriorating and fully recovered. But this meant that treating the symptoms as completely separate entities was no longer an option.
In 1997, a local clinic began hosting a neurologist who would visit our small home town a few days out of the week. Although this condition is very rare and he had never seen it before, he was able to diagnose my mother and we finally had a name to work with-- Stiff Man Syndrome.
Her diagnosis, Stiff Man Syndrome
To quote the National Institute of Neurological Disorders and Stroke,
Stiff Man Syndrome (Stiff Person Syndrome) is a rare neurological disorder ... that is characterized by fluctuating muscle rigidity in the trunk and limbs and a heightened sensitivity to stimuli such as noise, touch, and emotional distress which can set off muscle spasms.Newly armed with a correct diagnosis, we were able to find a neurologist who had experience with her illness. Although the doctor we found lives and works 7 hours away from my parents' home town, the visits made to her office have been worth the long trek.
Her Treatment, IVIg
The treatment my mother receives is called Immunotherapy. According to NINDS,
An immunologic therapy, intravenous immunoglobulin (IVIg), administered to patients suffering from stiff person syndrome (SPS), provides dramatic relief from disabling symptoms, according to a study appearing in the December 27, 2001, issue of The New England Journal of Medicine .
Over time, the treatments administered have given my mother a new lease on life; The improvements brought about have been substantial. Her balance has improved and her stiffness has waned more and more each year. Now she has the ability to participate in many regular daily activities that many of us take for granted. I'm sure you can imagine how grateful she is to again have the ability to dress without assistance, to use the ladies facilities in complete privacy, to bend her legs on demand, .... Can you imagine the joy she experienced recently, when for the first time in 15 years, she was able to drive again? (Pictured above is a "first in 15 years" I was able to enjoy with her over a summer visit in 2006. I still get sappy thinking about excited and happy she was to be able to join me for a simple swim.)
Her Predicament, MediCare
In 2005, the Centers for Medicare & Medicaid Services (CMS) lowered the payment rate for IVIg. This new payment rate makes it financially prohibitive for health care providers to administer IVIg since the costs for purchasing IVIg exceed what CMS will reimburse.
Since it is illegal for a health care provider that accepts MediCare to charge more for a treatment than what CMS has agreed to pay, there is no way that a health care provider can recoup the uncovered costs associated with treating MediCare IVIg patients. Understandably as a result, many treatment centers that administer IVIg will not accept MediCare.
On December 1st of 2007, my mother will turn 65. Whether she wants it or not, MediCare will become her primary insurance provider. IVIg transfusion centers that accept her current insurance will not (can not) accept MediCare.
Any CMS cost savings generated by patients not having access to IVIg treatment would transform into different and possibly more costs down the road. For example, anyone in my mother's position could need up to 24-hour assistance. And who's to know the health toll that the physical requirements of assisting another and the emotional strain involved in watching a loved one suffer can have on one's life partner?
Personally -- after so many years of witnessing my mother's progressive improvement, I cannot stomach the idea of her losing access to this treatment. I am worried not only for the health of my mother, but for my entire family.
A Possible Solution, petition to Congress
The Immune Deficiency Foundation (IDF) is advocating for people who require access to IVIg. According to a recent release, IDF has so far been able to temporarily stop further reductions for IVIg MediCare payments.
IDF (and now I) request that if you care about patients receiving required treatment, please write to your representative about this issue. IDF has put together a site to facilitate this process-- with a few clicks, you can find your representatives and generate a letter to send either via e-mail or snail mail.
Thank you for taking the time to read this. -elsa