Rare Disease Day & Surgery
- mycorneroftheworld
- Mar 1, 2019
- 3 min read
This Rare Disease Day has been a very strange one for me.
Today, I met with a new neurosurgeon who decided that the best option was to try a shunt again.
First, if you haven't been here before, here is some info about Intracranial Hypertenion from NORD's (National Organization for Rare Disorders) Rare Disease Database:
"Intracranial Hypertension (IH) is characterized by increased pressure inside the skull. Intracranial means inside the skull and hypertension means high fluid pressure. Intracranial hypertension means that the pressure of the fluid that surrounds the brain (cerebrospinal fluid or CSF) is too high. Elevated CSF pressure can cause two problems, severe headache and visual loss. If the elevated CSF pressure remains untreated, permanent visual loss or blindness may result. Pseudotumor cerebri and benign intracranial hypertension are both former names for IH, which are now considered inaccurate. These names do not adequately describe the disorder and downplay the seriousness of IH.
There are two categories of IH: primary intracranial hypertension and secondary intracranial hypertension. Primary intracranial hypertension, now known as idiopathic intracranial hypertension (IIH), occurs without known cause. This form most often occurs in young, overweight, females in their reproductive years (ages 20-45). Secondary intracranial hypertension has an identifiable, causative agent, including drugs (such as tetracycline, lithium, Vitamin A-derived oral acne medications or excessive ingestion of Vitamin A, and oral or intrathecal steroids, growth hormone treatments), sleep apnea and certain systemic diseases such as lupus, leukemia, kidney failure (uremia), meningitis and dural venous sinus thrombosis. There is an association of IH and Chiari type I malformation. Many other causes have been suggested in the medical literature but have not yet been confirmed as true causes. It is critical in these patients to rule out an intracranial space occupying mass by neuro-imaging (CT or MRI). Although many factors are known to trigger the disease, the mechanism by which IH occurs, in either primary or secondary forms, is not known. In many cases, either type of IH may be chronic." -NORD, Idiopathic Intracranial Hypertension*
I've been down this road with Intracranial Hypertension already. I had a VP shunt installed in September of 2017. If was revised twice in November of 2017, then removed in July 2018. There's a whole story about the mess of that 10 months but it would be a lot to recount here.
This time, I have a new neurosurgeon. I told my friend last night that she would like this neurosurgeon because he is a male version of us and his our sense of humor (she met my last one on my final visit with him so she gets how that one was). And I do like him. But I'm also nervous because we are starting over again. And I'm in a new health system. My old health system only had my neurosurgeon and another one who is further away to deal with IH. I get that surgery is surgery but I had all 6 of my previous brain surgeries at one hospital and now I'm starting over with a place I don't really know. It's an amazing hospital system but it's different and I don't know things there. The hospital is close to me and had to have someone help me to the elevators because despite the fact that it was remodeled 5 or 6 years ago, I haven't been there since way before that.
So I have nerves this time. I'm having another VP shunt installed (or possibly switched to LP depending on what is happening with my ventricles at the time). I know what to expect but it's a different world being in a different hospital setting.
* NORD - Idiopathic Intracranial Hypertension http://rarediseases.org/rare-diseases/idiopathic-intracranial-hypertension/
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