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For those who don’t know how this all developed, here’s a little bit of background: I was born with a Neurological disorder called Neurofibromatosis type 1 (NF1 for short). NF1 causes benign fibrous tumors, mostly on the skin, all over the body. The number and size vary from person to person. You also see birthmarks called “cafe au lait spots.” Sometimes larger, diffuse tumors called Plexiform Neuromas grow deeper inside the body. These only cause problems if they grow too close to organs or structures and interfere with their functions. (This will be important later). While it is genetic, in my case it was a 1:13000 “spontaneous mutation.” Fortune was in our favor as both our sons tested negative pre-nataly and will not develop the condition themselves.
In more serious cases NF1 can cause skeletal problems or tumors in the eyes called gliomas. As I recently learned myself, in 5-10% of cases, a plexiform neuroma that started out benign will turn into a malignant tumor or sarcoma. This is what happened to me. Typically it happens between age 18 and 35; my occurrence at 49 is on the high end of the scale, and is rarely seen.
And here’s a brief timeline of how my current condition unfolded:
Weekend of 10/12/2018: camping with my Cub Scout pack. I began having some stiffness in my back, but nothing out of the ordinary. I thought I pulled something, and sleeping on a camp cot wasn’t helping.
10/14 through 11/18: the stiffness worsened, with spasms spreading down my legs. I started having trouble walking around. I started using a cane. All signs pointed to sciatica or a pinched nerve. An MRI of my lower back revealed two minor herniated discs in my lumbar region. I was trying to get an appointment with a pain management specialist to discuss a steroid shot, but began having trouble with bathroom functions (starting them, not with controlling them) so my doctor said “go to the ER, don’t wait for the specialist.”
11/19/2018, 6pm: I hobbled into the Cooper University Hospital ER. The doctors there told me that the nerves showing problems wouldn’t be impacted by the lumbar disc herniations (which were also described as minor anyway). A new MRI was performed on my thoracic spine. This revealed a likely fibroma — a peripheral nerve sheath tumor— on my T4-T6 vertebrae, which (along with some hemorrhaging) that was causing compression of the spinal cord and nerve roots. This disrupted the signals to my legs and everything else below the waist.
11/20/2018, morning: I had surgery to remove the tumor and relieve the pressure on my spine. They performed a laminectomy and resection which resulted in over half of the tumor being removed. The other half required a different “approach” (physically) and was left for a future procedure. The initial impression of the the surgeon was that the tumor was benign. The way it behaved with the vertebrae for example... it grew through the opening in the foramen rather than aggressively attacking the bones. Still, as a matter of protocol the tumor was set to pathology.
11/24:/2018: I was released and told to begin physical therapy. I’d be in PT for 6 months or so and eventually need a second operation to finish removing the fibroma.
11/29/2018: a post op appointment with the nurse practitioner becomes an appointment with the neurosurgeon who operated on me. The pathology results surprised him. I had a Malignant Peripheral Nerve Sheath Tumor. Cancer. I needed to talk with oncology as soon as possible. My own research into spinal tumors with NF1 over the week since the surgery suggested that NF sarcomas might require different treatment than other cancers. So I informed the team at Cooper that my next stop was to see an NF specialist to get more information and expert advice on the best approach. I had reached out to the foremost support organization for NF (type 1 and type 2): The Children’s Tumor Foundation. They referred me to Dr. Yohay at NYU Langone.
December 2018: I saw the team at NYU, and on their advice had a PET-CT to confirm that I did not have any metastatic issues. (That scan came back as we hoped it would— the spinal tumor was the only cancer found.) Dr. Yohay (the NF specialist) and the oncology team all agreed that the best option would be Proton Beam. And since Penn is both highly regarded with the most experience with the proton beam treatment in the region, AND is much closer to home than NYU, they referred me to Dr. Shabasson at U of P.
January and early February 2018: I consulted with doctors at Penn. Out of an abundance of caution, a pair of lymph node biopsies were performed and both came back negative. A consult with medical oncology confirmed that the NF1 involvement rules out chemotherapy. After a few minor delays I will finally begin the radiation regimen 2/18.
Radiation treatment will be 25 sessions, 5 days a week for 5 weeks. Barring complications or other negative developments, by 3/22 I will be finished with the proton beam.
Sometime in late April or early May, I’ll get that final operation to remove the rest of the tumor. The key going forward will be monitoring my spine with periodic MRIs to look for recurring growth.
That’s pretty much the beginning of the story. What comes next will unfold in the journal below.
Thanks for stopping by and caring enough to read my rambling account.