NF: A Life Sentence

Pet therapy 2 days after my NF surgery

Hi everybody! My name is Jen and I have NF1. My mom and brother also have NF1.  I never thought anything of it when I was told I had NF.  Other than 'spots' and outer tumors, etc, I thought that was all NF was about. I never had pain or complications, etc. Then in 8th grade (around 1996) I came down with migraines as well as  the most terrible facial pain. I can't describe it. The pain would then extend to my neck. I woke up one morning with my neck all twisted, and I had to stay home from school for a few days. The facial pain got worse so I thought it was my wisdom teeth. Had an x-ray and from there he saw something strange (and it had nothing to do with my wisdom teeth) so I had 2 more x-rays, a CT scan and an MRI I guess to really make sure what they saw. The revelation: a tumor in my skull, and an optical glioma. Had a follow up MRI a year later, and nothing changed, so I didn't see anymore doctors for at least 10 years (when I moved to Boston and learned about the NF clinic at MGH). I actually didn't know the extent to my facial tumor until I moved to Boston. Imagine a flat jellyfish and that's what the tumor in my skull looks like. It wraps around my sinus cavity, my nose bone, my upper jaw (I have a lump in my jaw that dentists keep thinking it's some gum disorder), around my temple (part of my skill is actually lifted at my temple) and somewhat behind my ear. so that explained the facial pain and frequent migraines! When I was first given the MRI, my doctors were not very clear about where the tumor was, how big it was, etc. So I am REALLY glad I am in Boston, and that my doctor is cool enough to show me pictures. Dr. Scott Plotkin is a good guy. :)

Thanks to NF I am short. I also have mild scoliosis and thanks to my optical glioma, I am partially color blind in my right eye. I wear glasses. When I was in school, of course, people made fun of my height and often made fun of my spots. One day people in my drama class passed a note around with a picture of a face and neck with spots everywhere (though I don't have spots on my face). I knew it was about me. I wonder what kind of pleasure that gave them to make fun of my cafe au lait spots.... silly.

Anyway, my face doesn't hurt as much anymore, and I rarely get migraines, but in addition to my facial pain, I developed awful lower abdomen pains for years that was worse than my facial pain. When the pain got worse, I thought it was a gynecological issue. Had ultrasounds, etc, nothing. Then I thought it was a  gastrointestinal issue. So I had an upper GI, endoscopy, colonoscopy and guess what? They randomly found cancer in my stomach- totally unrelated to my pain in my abdomen! This was in 2009. I am still dealing with repercussions from THAT surgery (mainly esophageal problems).

My NF survival wound. You can sort of see my scar
from my cancer surgery, which extends from my belly button
to my sternum. They recut a good portion of my old scar!

 During the summer of 2010, my abdominal pain became excruciating. I had my gastroenterologist run many more MRIs, etc, and turns out I had an NF tumor where I had pain. I didn't find out about this until February 2011. I then couldn't get a hold of my NF doctor until this past JULY to talk to him about dealing with it. Had a biopsy to make sure it wasn't a sarcoma (needle biopsies are terrible by the way! I felt EVERYTHING). The only thing that could be done for me (since pain meds did nothing even oxycodone for a <100 lb person did nothing!) was surgery. So on Aug 23 I had my first NF surgery.  Of course now I have some sensory nerve damage (that's slowly going away). I take 2400 mg of gabapentin a day and that helps tons! I recommend that for any tingling types of pain you might have! And visit a pain clinic. I see a pain psychologist and she is AWESOME. My primary care doctor is also awesome, gotta add that in there too :)

I still have pains in my left and right side. Before my surgery, my surgeon told me that my right side pain was just referred pain, and that it would go away after my surgery. Well, it hasn't. It took 7 years for doctors to finally find the answer for my left side pain, let's see how many years they will take to find something on my right side.

I additionally worry about new tumors growing. I never knew tumors can just randomly GROW with NF, but they do. That scares me. What if more grow back? What if I become in pain again? I seriously could not function from 2010-now. I don't know how I survived my first semester of grad school but I did. What keeps me going is that I know there are people who go through worse.

I call NF a life sentence because those with it have to ENDURE IT. They have to worry about tumors growing randomly, tumors pressing on nerves, surgeries, pain meds, etc. It is baffling to me how NF is so misunderstood, yet it is one of the most common genetic disorders. I am not downplaying other genetic disorders, but NF deserves more attention. Some people have no problems with their condition, but many DO, and when people DO, their problems are awful. And there's usually nothing that can be done about it.  Some people go deaf, some people become paralyzed, some people have disfigured faces, some people can't function because they are in so much pain (back pain, stomach pain, leg pain, etc). I really love how there have been more walks, more races and more fundraisers about NF.

This is Bob :) I have named
all of my tumors

There are several NF non-profit centers around the country that help put on races, etc, so you should check it out. Here is the Facebook link for NF Northeast. This is for Michigan This is for Arizona This is for California, and this is for Louisiana. ... that's about all I can find on Facebook!

I have a personal blog which chronicles my personal battles (and all the drama that occurs between me and doctors). Feel free to follow me and if you have a blog let me know so I can follow you!

Thank you for reading my story.