Wednesday, April 23, 2014

Idiopathic Intracranial Hypertension

Since my last post, we actually got her official diagnosis. She has idiopathic intracranial hypertension (IIH), also called Pseudotumor cerebri (PTC). Kylee had her spinal tap(lumbar puncture) on march 10th. She did really well with it. Of course she was sedated for this procedure. She has become well aware of what goes on now when we go anywhere that she has to have anesthesia and does not like to be bothered with vitals being taken, the gas mask, or bandages. Thankfully, she was the only child there so the child life specialist was able to entertain her. She came out of anesthesia the same as always -cranky, but fine as soon as we leave. She seemed to be sore in her lower back area for about a week. This is probably due to the fact that it looks like they had to poke her 3 times to get the cerebrospinal fluid. They do two things with the CSF. 1. They measure the opening pressure of the CSF. Normal in a child Kylee's age is about up to 20. Kylee's was slightly elevated at 24. They also send out the CSF to test for different things that may contribute to the IH. If there is a likely cause, then it is just referred to as intracranial hypertension. If there is no known cause, it is idiopathic. Anyways, they collected tubes of blood and with the blood samples and CSF, they tested to see if there were any viral infections, Lupus, certain vitamin deficiencies or excess vitamins and a few other things that can be linked to IH. The only abnormality that showed up was elevated parathyroid hormone. A visit to the endocrinologist the next day resulted in another blood draw and urine testing of calcium, vitamin d, phosphorus and kidney function. With elevated parathyroid hormone, it can be a result of different reasons. Kylee's vitamin d showed to be low, so we started her on vitamin d supplements. There doesn't seem to be any link of low vitamin d and IH unless it has to do with abnormal calcium levels which Kylee's calcium levels were within normal limits. We are waiting on having another sleep study to check for sleep apnea. Sleep apnea is very common in the Ds population and there has shown to be a link with IH and sleep apnea. Kylee was diagnosed with sleep apnea over a year ago and at that time her tonsils and adenoids were removed which resolved the apnea. She has been sleeping terribly for a few months now, which could be apnea or it could be from the IH. In a way, it would be nice if she had apnea. Take care of the apnea and if that is possibly contributing to the IH, then hopefully it would put her into remission and lessen her chances of it reoccurring. She started on a medication called diamox on march 19th. That is supposed to reduce the amount of CSF produced. Since it is a type of diuretic, it can affect your kidneys so staying well hydrated is essential. This has been quite the task in getting her to drink enough. She gets easily frustrated with us constantly pushing a drink at her. Thankfully, I found a support group for IH and this has been very helpful in learning about IH. Unfortunately, there isn't a known cure, just medications to try to lower pressures or the options of placing a shunt. Medications or shunts can put the person into remission, but not always. While the support group has been very helpful,it has also made me aware of how little research has been conducted for IH. Also, I have seen stories from others of children having to endure pressure headaches that medications have a hard time relieving, nausea, vision disturbances, etc. It is one thing to have a headache, but when it is a child and medications don't relieve it, it is worse. And it seems that since IH is misunderstood, patients often aren't treated properly. Anyways, Kylee was seeing a neurologist that we weren't very happy with for many reasons. The hard thing with Kylee not being able to tell me how she feels and being on a medication that can affect electrolyte balance, kidneys, etc is that I have to rely on her behaviors and her physicians. When the neurologist doesn't get that Kylee is unable to communicate how she is tolerating IH and the meds, it is beyond frustrating. The medicine can lower your potassium levels since it is a type of diuretic and some of the ways to tell would be tingling in extremities. When I asked the neurologist about drawing labs to see how she was tolerating the med, her response was just that(she would have side effects such as tingling) to which I had to remind her again, she can't tell me if she is tingly, etc. If you were to google IH, you would know that a high opening pressure would be a reason to start medicine. When you have an LP, the Dr will lower the pressure back down to normal (closing pressure). CSF is constantly being replenished so just because it is brought down to"normal" it will go back up and if you have IH, it will likely go back up to being elevated. The neurologist stated in her notes that she felt strongly on not starting Kylee on medicine since her pressures were brought back down to 14. Also, if you were to google optic nerve swelling (papilledema) and IH, it doesn't always occur in IH. So, for her to talk about doing better if her papilledema is resolved is crazy. There are plenty of patients who do not have papilledema but have extreme pressure headaches and high opening pressures. Ok, rant done on this neurologist. I had a hard time getting an appointment with a different neurologist, but was able to get in with a neurologist new to a different practice. Kylee saw Dr. Wallace last Thursday and she was great. Was very thorough and explained a lot and also went over Kylee's MRI images. We increased her nighttime dose of the diamox and also started her on an iron supplement because of her low ferritin levels and that fact that she has periodic limb movements(which can be caused by low ferritin levels.) Dr Wallace referred us for a sleep study to check if the iron may be helping the plmd and check for apnea. We follow up with her in 2 months. She also gave me a slip to check her labs before her endocrinology appointment on the 12th, but if I felt she needed them sooner to do them sooner(didn't have to bug her about it like the last neurologist.) While trying to get an appointment with a different neurologist, I also made an appointment with a neuro-ophthamologist at Duke. We have a wonderful ophthamologist that Kylee has been seeing, but wanted Dr. El-Diari(neuro-ophthamologist) to check her out since she specializes in IH. An IH patient doesn't necessarily need an ophthamologist, neuro-ophthamologist and a neurologist, but considering not a lot is known about IH, I want to make sure she is getting the best care possible. Her appointment wasn't until June, but the ophthamologist that sees Kylee sent Dr El-Diari an email to see if we can get in sooner. At Kylee's last ophthalmology appointment, which is never fun because it takes 3 of us to restrain her to get a good look at her eyes, she said her papilledema is still there but is slightly better. Considering she was only on a very low dose of her meds for 3 weeks, that is at least positive. We are scheduled to see the Dr. El-Diari at duke tomorrow. Thankfully, it is spring break so Kate can stay with grandma. It takes 2hrs to get there and we were told to expect a 3-4 hr appointment! Hopefully, it won't be too traumatizing for Kylee and it is worth the drive. If you read this far, bless you cause I'm sure it is a lot of information that doesn't make much sense, but my reason for writing this is to keep track for myself as well as anyone who may be searching for IH. I'm hoping to update her outcome of tomorrows appointment sooner as well as do a post about Kylee's hip dysplasia and her hip surgery that she will be having in August. I'm not looking forward to it, but am looking forward to being able to sit next to her without feeling her hip going in and out of socket!


  1. Thank you for posting! I love the idea of getting more information on the web because you are so right - there is so little known and very little on the web. I love the truth you put to the condition. I have IH as well and am studying to become a neurologist. Goal: Cure IH!

    1. Awesome on studying to become a neurologist. I think if more doctors listened to patients better they would learn more. Instead of following textbook studies.