Yesterday, we saw the neuro-ophthamologist at Duke. It was a long 3 hrs there and that didn't even include everything they had intended to do. That being said, Kylee did fairly well all things considered. It takes 3 of us holding her down just to get a good look at her eyes. And bless her, she squeezes her eyes shut as tight as possible.
Dr El-Dairi is supposed to be well knowledgeable about IIH and she was very nice, but I think I came away from the appointment more frustrated and confused. Mostly, I've been having curse Down syndrome moments. Don't take it the wrong way, but because of Kylee's inability to fully describe how she is feeling, it makes the whole IIH and treatment more difficult. I wouldn't change her because I feel her Ds adds something so heartwarming to her personality. Everywhere we go, she has to interact with others, saying hi, giving hugs, etc. There are plenty other reasons why as well. Typical IHers have headaches which makes knowing how the medication is working a lot easier. Since her first visit to the ophthamologist when the optic nerve swelling was noticed, it has always been said that Kylee doesn't display typical IH symptoms such as headaches, nausea, etc. I have always thought that Kylee and others with Ds have a much higher pain tolerance and this would make sense as to why Kylee doesn't act in pain lately.Going to the first neurologist, it was noted just that-no headaches, nausea or anything. Nothing was taken into the consideration some of the changes I had noted like a lot of ear rubbing (with no ear infections diagnosed). People with IIH will tell you they often experience "whoosing" noises in ears. Kylee has also had her swallowing difficulties of thin liquids reoccur. She also has reflux to go with it as well. Whether or not it goes with the IH, I don't know, but it started around the same time as her eye change was noticed. Also, Kylee has dark circles under her eyes. It has always been chalked up to allergies. I don't blame her pediatrician or ENT for thinking that because that is what it looks like. However, bringing it up to the neurologist and neuro-ophthamologist, as well as trying allergy meds and no signs of allergies(congestion, sneezing, watery eyes, nothing) it should be noted. Especially, when I have said others with IH have noticed it as well when there pressures were up and when Kylee had her spinal tap, it had went away for a week. Also, Kylee grinds her teeth a lot more and will pull at her hair at night. Kylee has always been a teeth grinder, but it seems to be worse at certain times. She also has low iron and there may be a correlation to that. All these things and given the fact that her optic nerves were swollen and she had higher pressures with her spinal tap would make sense to me that there is a connection of some. Ok, back to her appointment yesterday. The Dr felt that Kylee has some features of false appearance of swollen optic nerves, but also had features of true swollen optic nerves. False swollen optic nerves can be more common in the Ds population in general. Typically, the Ds population have different looking optic nerves anyways. Also, she said new criteria defines high opening pressures in pediatrics is now 25(Kylee's was 24), which is one reason for thinking false swollen optic nerves. But she also said given the fact that the ophthamologist previously noted the appearance of swollen optic nerves and the fact that her protein levels were low in her CSF(can be a factor in IIH,) she would be noted to have features of swollen optic nerves. Basically, she is seeing Kylee mid-treatment and she doesn't know what Kylee's optic nerves looked like before so she can't say definitively if she has true papilledema (swollen optic nerves). She wanted to take photographs of her eyes, but Kylee won't even sit still to have them just examined so we will have to wait until she goes under anesthesia in August for hip surgery to get pictures then. Her plan is to look at the photos, take her off of diamox, then see her in her office to see what her optic nerves look like. The only thing I don't like about this situation is if the diamox truly helps her and take her off of it while after she has hip surgery and is in a full body cast, not having full movement, she may be more irritated. Dr El-Dairi did feel she wasn't on a true therapeutic dose of diamox at this point if she does have IIH. Going back to the whole no complaints of headaches, nausea, it seems that her previous neurologist and this neuro-ophthamologist want to base how well Kylee is doing on what her optic nerves look like. While I understand she isn't your typical IIH patient outwardly, papilledema isn't always present in IIH. In fact, it was noted in the Dr notes yesterday that headaches, nausea and vomiting aren't exclusion criteria in saying she doesn't have IIH. Confused yet? She did at least say that she hasn't had any patients complain about dark circles and she hasn't seen it in literature, but that doesn't mean it isn't possible. My thinking is most patients can describe their symptoms so they wouldn't really need to mention much about the dark circles. One other thing she did mention that was confusing to me is the eye crossing Kylee had at the start of this is not neurological. I thought I read different. See why I am confused:) Anyways, we will just continue seeing the new neurologist(which we would anyways), continue giving diamox and check labs to make sure she is tolerating it ok, wait on our sleep study, follow back up with neurologist in 2 months and follow up with either ophthamologist or neuro-ophthamologist after photographs are taken in August. We have an appointment with the gi doctor and she seems to be doing well on the prevacid. She had some reflux symptoms when everything started, but we limited certain foods. Now with taking the diamox, it can cause reflux and as well as the increased fluid intake ( which has been everything possible we can give her to drink,) but it has been mostly orange juice, so we started her on the prevacid. Then we also follow up with endocrinologist to see where she is at with her vitamin d levels, etc. Again, if you read all this jumbled mess, thanks!