Post by terri on Sept 26, 2014 9:34:17 GMT -6
Hello,
I am finally writing my first post. Reading this forum truly helped me navigate the frightening waters of uncertainty since May of this year. Thank you to all of you who have shared your stories. I feel that I have learned enough through our experience to be able to help others and can finally reach out for some support as well.
My 12-year-old presented with leg pain that began about a year ago. It took until May of this year (and of course an MRI) to discover his tumor which was a dumbbell-shaped intradural extramedullary soft tissue mass in the neighborhood of 2-3cm between L1 and L2. In other words, it began inside of the sheath covering the spinal cord on the dorsal nerve root, was displacing the nerves inside of the spinal canal and extended out through the neural foramina causing the dumbbell shape.
Surgery was scheduled 5 weeks later with Dr. George Jallo at the Johns Hopkins Hospital. In our case, despite the risks, holding off on surgery was not an option that made any sense. When you are already symptomatic with symptoms worsening, it is my understanding that you are only risking permanent damage to those nerves as the tumor grows and compresses them. My son is an ice hockey athlete and we felt that the sooner we relieved the pressure the better.
Something to keep in mind that I did not know before surgery is that benign nerve sheath tumors typically arise from the dorsal nerve root which is the sensory nerve as opposed to the nerve branch responsible for motor function. Unfortunately for my son, the nerve did have to be sacrificed. I believe this was because it was a neurofibroma which typically engulfs the nerve root and is therefore difficult or impossible to separate from the nerve. As a result, my son does not have a great improvement of symptoms yet since surgery but he is back to being a very active teenager and has zero loss of any motor function. He will probably always have a right thigh that feels different but we are hoping that he will get used to that especially being so young.
More on surgery and recovery: He had a laminoplasty which is less severe than a laminectomy. The tumor was, we think, 100% removed. He is still on gabapentin/neurotin and says that the pain in his leg is much worse when he forgets to take it. We were in the hospital for 6 1/2 days mainly because he did experience a severe headache for a couple of days that could have been due to cerebrospinal fluid (CSF) loss or simply inflammation. The incision was at first terrifying to me but has morphed quite a bit from those early days and I can now see that it will be a minor line (approx. 5") nicely tucked into the natural dip in his back between the muscles. He does have lower back pain now that he did not experience before surgery and sometimes takes Ibuprofen. Given the hand that we have been dealt, we really could not have asked for a better recovery overall. He even flew to Colorado with grandparents less than 4 weeks after surgery and began playing ice hockey after 9 weeks!
We are now in the midst of working with the genetic counselors at Johns Hopkins to determine if he has a segmental form of neurofibromatosis. He mostly lacks signs of NF1 but does have a birthmark on his lower back which has raised concern. We will be getting a biopsy of the birthmark and a blood test for genetic testing. What I've discovered in my mostly on-line education on the subject is that spinal cord tumors are rare, nf1 is not that rare in the world of genetic disorders, segmental nf is rare and also under diagnosed, and having a segmental form of nf with a spinal cord neurofibroma is really rare! Neurofibromas are often cutaneous.
Navigating all of these medical intricacies is something that many of us are familiar with. For me, knowledge is power and we are figuring this out using a variety of resources and medical professionals. I would not advise simply relying on your surgeon for all of the answers. Genetic counselors, for example, look at a much bigger picture and we may find that an nf specialist will be our greatest source of support. It has been and will be a journey for sure.
As lucky as we have been, my son still had a major surgery to remove a tumor and will never quite be the same. Hopefully, he will get a strengthening of character out of it:)
We also return this Tuesday for a follow-up MRI and appointment with Dr. Jallo.
Thanks for listening. I am hoping to provide some comfort or answers to anybody out there needing it!
I am finally writing my first post. Reading this forum truly helped me navigate the frightening waters of uncertainty since May of this year. Thank you to all of you who have shared your stories. I feel that I have learned enough through our experience to be able to help others and can finally reach out for some support as well.
My 12-year-old presented with leg pain that began about a year ago. It took until May of this year (and of course an MRI) to discover his tumor which was a dumbbell-shaped intradural extramedullary soft tissue mass in the neighborhood of 2-3cm between L1 and L2. In other words, it began inside of the sheath covering the spinal cord on the dorsal nerve root, was displacing the nerves inside of the spinal canal and extended out through the neural foramina causing the dumbbell shape.
Surgery was scheduled 5 weeks later with Dr. George Jallo at the Johns Hopkins Hospital. In our case, despite the risks, holding off on surgery was not an option that made any sense. When you are already symptomatic with symptoms worsening, it is my understanding that you are only risking permanent damage to those nerves as the tumor grows and compresses them. My son is an ice hockey athlete and we felt that the sooner we relieved the pressure the better.
Something to keep in mind that I did not know before surgery is that benign nerve sheath tumors typically arise from the dorsal nerve root which is the sensory nerve as opposed to the nerve branch responsible for motor function. Unfortunately for my son, the nerve did have to be sacrificed. I believe this was because it was a neurofibroma which typically engulfs the nerve root and is therefore difficult or impossible to separate from the nerve. As a result, my son does not have a great improvement of symptoms yet since surgery but he is back to being a very active teenager and has zero loss of any motor function. He will probably always have a right thigh that feels different but we are hoping that he will get used to that especially being so young.
More on surgery and recovery: He had a laminoplasty which is less severe than a laminectomy. The tumor was, we think, 100% removed. He is still on gabapentin/neurotin and says that the pain in his leg is much worse when he forgets to take it. We were in the hospital for 6 1/2 days mainly because he did experience a severe headache for a couple of days that could have been due to cerebrospinal fluid (CSF) loss or simply inflammation. The incision was at first terrifying to me but has morphed quite a bit from those early days and I can now see that it will be a minor line (approx. 5") nicely tucked into the natural dip in his back between the muscles. He does have lower back pain now that he did not experience before surgery and sometimes takes Ibuprofen. Given the hand that we have been dealt, we really could not have asked for a better recovery overall. He even flew to Colorado with grandparents less than 4 weeks after surgery and began playing ice hockey after 9 weeks!
We are now in the midst of working with the genetic counselors at Johns Hopkins to determine if he has a segmental form of neurofibromatosis. He mostly lacks signs of NF1 but does have a birthmark on his lower back which has raised concern. We will be getting a biopsy of the birthmark and a blood test for genetic testing. What I've discovered in my mostly on-line education on the subject is that spinal cord tumors are rare, nf1 is not that rare in the world of genetic disorders, segmental nf is rare and also under diagnosed, and having a segmental form of nf with a spinal cord neurofibroma is really rare! Neurofibromas are often cutaneous.
Navigating all of these medical intricacies is something that many of us are familiar with. For me, knowledge is power and we are figuring this out using a variety of resources and medical professionals. I would not advise simply relying on your surgeon for all of the answers. Genetic counselors, for example, look at a much bigger picture and we may find that an nf specialist will be our greatest source of support. It has been and will be a journey for sure.
As lucky as we have been, my son still had a major surgery to remove a tumor and will never quite be the same. Hopefully, he will get a strengthening of character out of it:)
We also return this Tuesday for a follow-up MRI and appointment with Dr. Jallo.
Thanks for listening. I am hoping to provide some comfort or answers to anybody out there needing it!