Sixteen years ago on this day I endured a grueling 14-hour surgery to remove a tumor from between the top two vertebrae of my spine. The cause? A condition called Neurofibromatosis, or NF for short.
Because May is NF awareness month, I thought I’d share with you, dear reader, about something I consider highly under-publicized. It’s not a disease in the truest sense of the word because it doesn’t spread. It’s a genetic disorder where tumors can grow anywhere along the central nervous system. These tumors are nearly always benign (as mine was), but they can have devastating effects on your body and, as a result, your life. In my case, prior to my surgery, my left-side extremities had lost much feeling and function (I’m a professional musician, so this was noticeably detrimental to my vocation). Also, the nerve which controls my diaphragm was being pinched, so I was beginning to have difficulty breathing.
As I said, NF is genetic; there is a 50% chance of this being passed along to one’s offspring (I and one of my three sisters have this condition; our one child has it). So why don’t more people know about Neurofibromatosis? Perhaps it’s because NF isn’t as fatal as similar afflictions such as Multiple Sclerosis or Cystic Fibrosis, although the genetic transmission rate is lower for those two. Happily, I have been seeing references to NF and the Children’s Tumor Foundation on recent television medical dramas such as Bones and House.
NF is what I have termed a snowflake disorder, in that it doesn’t present in any two persons exactly the same way. Traits that nearly all NF patients have in common include the presence of small skin discolorations called “cafe-au-lait” spots (you usually need six or more of these for a diagnosis), skin tags of two different varieties – one is red that bleeds profusely when nicked – which can cover little or much of the body, and an abnormally large head (which is why I require a size-8 cap; most OSFA hats only expand to 7 6/8). Tumors can form anywhere. External ones are often plexiform; they can become very large and debilitating, like for this young man in England.
Internally, tumors can form along the spinal column, in the abdominal cavity, or in the brain. They may appear in the ocular cavity, on optic nerves, or in the case of NF 2, acoustic nerves. Half of NF patients have learning disabilities. Some have mobility issues due to softening bones, unusual leg curvature, and scoliosis. My son and I are fortunate: we have no learning problems and to look at us, without some knowledge of characteristics, you wouldn’t know we actually have NF.
Although (depending on the type and patient) symptoms may not appear until after puberty, Neurofobromatosis can be diagnosed at a very early age. In fact, I first learned I had NF when our son was diagnosed before his first birthday; it was because so little was known about NF in the late 60’s and 70’s that I was unaware of my condition until then. Our son is now 20 and remains issue-free; the problem which necessitated my surgery surfaced when I was in my mid-30’s.
I do not wish to bore you with too much information, so I’ll not elaborate further. What can YOU do? Start by going to ctf.org, official website for the Children’s Tumor Foundation, to learn more about the different types of NF. Because May is NF awareness month, there are several benefit walk-a-thons being planned around the country; for example, I know of one in Boston, where an acquaintance of mine named Heather (who also suffers with NF) is participating. Click here join.ctf.org/HNiles if you would like to donate in support of her walk. Much more is being learned about NF, including genetic mapping, and we hope to get closer to a cure.
Thank you for indulging me on this topic. Do me a favor, though? Sure, you can just say NF for short, but, since learning is fun, teach yourself to say the entire word. Break it down into its sub-components: Neuro (meaning of the nerves) – Fibroma (a type of tumor) – Tosis (common ending of medical conditions). Neuro-fibroma-tosis; got it? Good!
Have a wonderful day, my friends! Support arts education, study to show yourself approved, and always be kind and helpful to others.