Monday, November 17, 2014

Rare and Unique NF Complications

Thanks to everyone who participated in my pre-post discussion! For those of you who want to participate in conversations about Neurofibromatosis, like the "MBNF, Manitoba Neurofibromatosis Support Group" on Facebook!

It really wasn't until I decided to write this blog post that I realized that I didn't like the word "rare". I mean in some cases its great, like "Hey I have a rare coin from the 1800's that's worth a lot of money", then sure, rare is a great word to use! But when it comes to medicine and complications from different illnesses, "rare" isn't such a great word. To me, it almost alienates people. It puts a label on them and makes them appear different from everyone else. I mean, a lot of people already struggle with being "different" because of their diagnosis of Neurofibromatosis, so why further alienate them from normalcy? Why place an unneeded label on someone who is just trying to find a place to fit in? So throughout this post you will see me use the word "unique" instead of rare, and maybe we can start changing some of our language!

Like all other illnesses, Neurofibromatosis has a set of  unique complications. My Mom and I always used to laugh at how I was the exception to every rule, and how majority of the complications I have are unique, which at times can be very frustrating. So here, I'm going to explore some of these complications with you! Keep in mind that these occur in less than 1% of people living with NF:

1. Early or Late Onset Puberty -- there really is no known cause for this complication at this point in time. Typically changes in hormones such as in puberty and in menopause can cause either changes in your current tumors, or cause new tumors to develop. Therefore, current research indicates that the growth of neurofibromas could be due to hormonal changes and imbalances.
In terms of my own health, I had to deal with extremely late onset of puberty. While everyone was going through puberty between the ages of 10-16,  I didn't hit that stage until I was almost 18 years old, which actually was a very stressful process for me.

2. Epilepsy -- generally, epilepsy in Neurofibromatosis occurs secondary to a brain tumor. Epilepsy seems to be able to be well controlled with medication, and usually  people with NF have a mild cases. Statistically speaking, partial onset seizures (seizures that impact only one hemisphere of the brain) are the most common type of seizure seen in NF patients.   

3. Malignant Peripheral Nerve Sheath Tumors -- these tumors arise from the peripheral nerves or from cells associated with the nerve sheath (such as Schwann cells and fibroblasts). The cause of this kind of tumor is unknown. These tumors are cancerous, and often require treatments such as radiation and chemotherapy.

4. Brain Tumors (other than optic glioma)-- one of the trademark symptoms of Neurofibromatosis are tumors of the optic nerves (optic gliomas). In some cases, optic gliomas can cause involuntary eyeball movement, and sometimes blindness. It is generally recommended that children are observed yearly by an ophthalmologist who performs tests that can screen for these types of tumors. Any other kind of tumor in the brain is unique!
When I was 13 years old,  I was diagnosed with a benign astrocytoma of the hypothalmic/pituitary region. Astrocytomas are tumors that arise from astrocytes, which are star-shaped cells that make up the "glue-like" supportive tissue of the brain. The hypothamic/pituiary region of the brain, is located right in the middle of the brain (think of a pit in a peach, that is where the tumor is), thereby making the tumor inoperable. Thankfully, my tumor hasn't grown in over 7 years!

5. Severe Mental Retardation -- this is one of the most severe behavioural and cognitive abnormality seen in Neurofibromatosis. Although it is common for individuals with NF to have learning disabilities, it is very uncommon that they are seriously impacted. This is usually evident in the first few years of a child's life.

6. Hormonal Complications -- hormonal conditions related to NF are typically a complication of a brain tumor, but research has also showed that individuals born with NF who do not have brain tumors brain tumors can still suffer from a variety of different hormonal conditions. This is something that is still being researched...
I was diagnosed with hypopituitarism in 2010, which funny enough is extremely uncommon worldwide (not just in NF). Basically, this  means my body doesn't make nearly enough hormones. The hormones that have been impacted the most since my diagnosis are:  growth hormone, cortisol, estrogen, progesterone, and prolactin.

7. Problems with Growth (too short or too tall) -- once again, there is no definitive cause for problems with growth in individuals living with Neurofibromatosis. Sometimes, the brain makes too much growth hormone, which results in a child being overly tall, and it can also lead to the brain not making enough, thus resulting in an abnormally short stature. If caught in time, children who are growth hormone deficient can be given growth hormone injections to stimulate their growth, while children who make too much of this hormone typically have to undergo surgery.
Along with the hypopituitarism, I was diagnosed with growth hormone deficiency in 2010. However, since my growth plates had shut a year or so before the diagnosis, I was not a candidate to receive growth hormone injections to stimulate growth.

8. Stroke -- in Neurofibromatosis, stroke is most often caused by blockages in the blood vessels going to the brain, leading to a reduction or complete blockage of blood to the brain.

Well, I hope everyone has a wonderful week! Thanks for tuning in!


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