Annie's IEP, CPAP, Ankle & Knee, G-button, and Eye Appointment Update--Whew!

Annie's IEP School Meeting
It's been another busy couple of weeks for Annie.  We had her IEP school meeting last week which actually went very smoothly.  We're excited about the things Annie is doing in school and the ways she's learning to interact with others more.  Unfortunately Annie won't qualify for the extended school year (ESY) summer program for children with disabilities.  We have been frustrated by that since she was able to attend ESY in Texas, and also because we have such limited in-home nursing hours here in Nebraska.  (In Texas we got 15 hours a week, and here we get 4.)

So that means we have very limited respite available over the summer while Annie is at home all day which makes errands and outings, meeting all of Annie's needs, and caring for two other small children very difficult. Nebraska's state disability programs are so great at helping with all of Annie's other needs, we just can't figure out why they limit in-home nursing hours so dramatically.  However, we somehow managed this same situation last summer (our first in Nebraska) when Annie didn't qualify for ESY then either.  I have learned how to pull a grocery cart full of groceries and two children while pushing Annie in her wheelchair with one hand.  Not easy, but possible.  We'll definitely need to stick to a strict schedule this summer.

Annie's CPAP Follow-up
We took Annie to the pulmonology clinic yesterday to review her CPAP titration sleep study and CPAP memory stick data.  The sleep study started with her CPAP at pressure level 4, then watched how she responded.  After still seeing lots of obstructions they increased the pressure by one level and watched again.  They got her to level 8 when they first started noticing a decrease in the number of her obstructions, but since the sleep study was almost over by then, they increased her to level 10, but didn't have enough time to collect data on how she tolerated a level 10.  So they are prescribing a level 9 for her and instructing us to watch how she does and if we don't see an improvement in her sleep and alertness during the day, they will consider increasing the pressure further.  I was surprised to hear that she required a level 9 and would still have some obstructions at that level.  It reminds us of really how terrible her sleep apnea is, even with her tonsils removed. So she'll sleep with a pressure of 9 tonight and we'll see how she does.  Hopefully it will improve her sleep, decrease her seizures, and help her be more alert and happy during the day!

Her CPAP machine has a memory stick in it that records how much Annie is using it and at what times she's pulling it off her face or having leakage around her nose.  We were pleasantly surprised to see data for the first time yesterday showing her use of CPAP.  Most mornings when we go into Annie's room, her mask has slipped off her nose onto her cheek, but we've never been sure at what point that is happening during the night.  The data we got yesterday showed that Annie is actually keeping the CPAP on most of the night, usually pulling it off around 5:00am or so.  That was incredibly encouraging to us!  Way to go Annie!  It's nice to come away from an appointment feeling excited and hopeful rather than discouraged for once!

Ankle and Knee Concerns
Tomorrow is Annie's orthopedist follow-up for her heel cord lengthening that happened last December.  We are nervous because she doesn't move that foot or ankle anymore.  We're thinking that her ankle is still just so stiff from being in a cast for 6 weeks and now needing to be in her AFOs (ankle and foot orthotics) all day.  So we're expecting the orthopedist to suggest some concentrated physical therapy on her ankle to get it to move and improve her range of motion.  The area around her 5-6" long incision is also very lumpy under her skin which I didn't expect, and the skin itself goes rather blue anytime Annie's foot or leg is cold.  We doubt there is anything seriously wrong, but it will be nice to get some answers tomorrow.

We are definitely more nervous about Annie's knee, however.  Last December she had knee surgery to correct a dislocating knee cap that was extremely painful.  She has been wearing a flexible knee brace since mid-January, but we have still been trying not to bend it much at all while it continues to heal.  This past week as we've been starting to slowly bend her knee more (with the brace on) we've been noticing some popping.  With the brace on it has been hard to tell if it has been actually dislocating or just making a popping sound.

However, this morning as we began getting her ready for her shower, we took her knee brace off.  She was having her myoclonic seizures (spasm-like jerks) and we noticed that with each spasm, her kneecap was being dislocated.  We would slide it back in place after each dislocation, but it continued to dislocate as her knee bent during her shower and afterwards.  None of the dislocations were causing her pain, but with the extensive work they did on her knee, we know it should not be dislocating at all.  Her next follow-up with her sports medicine doctor is next Tuesday, so we'll talk to him all about it then.  I'm really hoping we aren't facing yet another surgery on her knee--one has been more than enough!  But we also don't want to let her knee to continue dislocating because we know of the damage that causes to her joint and the pain and swelling that will inevitably follow.  It has been about 9 months since her kneecap started dislocating and we're ready to finally get this issue resolved.

G-Button Update
Several weeks ago Annie was having pain around her G-tube stoma.  There was no sign of infection so the doctor ordered a full abdominal sonogram which showed no signs of problems either.  I did, however, feel inspired looking at the images around her stoma that her G-button size might be too small since we have never gotten her a larger size since she was 2 years old.  After mentioning that to the doctor, he was surprised she was still wearing the same size button from 5 years ago and suggested we see a surgeon to help us determine if that was the real issue and to determine the correct size of button she would need.  Since then her pain has subsided, so we have delayed contacting the surgeon because of all the other appointments we've had lately.

However, Annie has been having incredibly large amounts of air in her tummy the last few days causing her to vomit several times a day.  We can't figure out what it would be due to, but decided to change out her G-button to see if that helped.  It probably won't affect her vomiting--we'll just continue to vent her frequently until it subsides or we decide she needs to see a doctor--but the new G-button I put in her today seems to fit well, so we'll watch to see if this new one starts causing her pain, and if so, then we'll take her to the surgeon.  Otherwise, with so many other concerns right now, I just can't manage to make it a priority unless it starts getting worse.

Ophthalmology Appointment
Because Annie's anticonvulsant (Sabril or vigabatrin) was recently FDA approved, and because it can cause vision loss, the FDA is now mandating that patients get an eye exam every 3 months in order to get refills of their Sabril.  For the past 7 years we have been getting this drug from Canada and paying for it fully out of pocket.  (Insurance companies won't cover drugs that are not FDA approved.)  We paid about $120 a month for this drug from Canada, but now that it is FDA approved and available in the US, our assigned one of four pharmacies in the U.S. that carries Sabril is charging us $3500 for a month's supply!  Fortunately our insurance now covers it 100%.  So it's a huge blessing that we no longer have to pay $120 a month to get this drug from Canada, but now the FDA has an incredible amount of paperwork for us, the ophthalmologist, and the neurologist, and eye exams that have to occur every 3 months which makes for more appointments for us.  Fortunately, the appointments are short, but we still have to drive 30 minutes away.  I guess it's a small price to pay to now have her anticonvulsant covered by insurance.  We'll definitely get to know our ophthalmologist well!