MD clinic is where we meet with Pulm, Rehab, neuromuscular and the wheelchair company all in one clinic. While the girls genetically don't have muscular dystrophy, whatever they do have, acts like MD so the choice was made to move the girls into this clinic to get more comprehensive care. At fitst they just put Jilli into this clinic but at our special need appointment at the beginning of the month I asked if Lydia could be moved into the clinic to and they agreed to move Lydia into the clinic however they said for her first visit she would only see neuromuscular and they would then decide in clinic if they would take her full case at the next appointment. We got into clinic and started talking with Pulm and she went in and added Lydia to this appointment, she said it is silly not to and she was transferring care over to herself. I am so grateful they stepped up and just did the right thing.
Pulm:
J- She asked about how well Jilli sleeps at night and I told her most nights it takes her 2hours to fall asleep. We know Jilli has two cysts in her brain in the part that makes melatonin so the doctor said to try her on some melatonin to see if maybe because of the cysts her body isn't able to make it like she needs. So far it is really helping. I am hopeful for her sake that this helps because she gets so frustrated some nights that she just can't fall asleep. She also said that if we need to use up to 2lpm at night of oxygen that is fine, that there is no harm in that. This doctor seems much more flexible in trusting us to make choices for her. We also talked about medical studies that have been done with long acting asthma inhailers and muscle disorders, the studies show they don't do anything. These meds do have side effects. We used them with the girls to try to help with their breathing but being on oxygen has done a ton more for that then the inhailers ever have. She said come spring we can try that.
L-We talked about her oxygen use. She said that she thinks Lydia likely needs oxygen at night like Jilli. The hard thing is that she probably doesn't need oxygen every night but many nights so she worries about doing the testing for overnight oxygen because she might pass it the night that we do the test but the next night she would fail it. Because of that she made the call to just put her on oxygen full time. Lydia has never had her own nebulizer because they said Jilli had one, however the one Jilli has is almost 5 years old and has been used a lot by both girls and it is starting to not work well. It is taking twice as long to do a neb at this point. The doctor said she was going to send in scripts for both girls to get new nebs.
Rehab:
J- Our normal rehab dr was out so we had a different doctor, but I really liked her! She was really good at asking questions which I like. I always come into appointments with a list but it is also nice when they ask questions that help lead us to new ideas. Jilli is old enough now that logically you would think she would be potty trained but she does't feel when she peeps or poops. Rehab said that its time to look into why. She might need diapers forever and if that is how it is thats fine, but we need to at least look into why she doesn't feel it. Rehab had some ideas but we will see what urology has to say. They are also going to send a script in for diapers.
She said that at this age she likes to get hip and spine x rays because its good to get a good baseline to see how the muscle weakness is effecting the bones.
Jilli's leg braces are starting to get small and they said if she grows any more to call Shriners to get her in for new braces.
*rehab didn't see Lydia this time but will next time
Neuromuscular:
J- We talked about the urine test and how it id not definitive at this point but we are going to run it at least once more and if Jilli ends up in the ER they are going to run it as soon as she starts a crash. She was having a pretty good muscle day although everyone agrees that she is showing signs that another crash might not be far away. We talked about how the best muscles for a muscle biopsy for Jilli would likely be the diaphram but no one would even try doing that because it is not safe even though it would likely give us better results. We still plan on doing another muscle biopsy at some point but we need to wait a few more years.
L- We talked about how the girls are very similar but different in little ways and so we want to be smart about what testing we do with Lydia but we also don't want to miss anything. Because of that he would like t do a full exome sequence on Lydia. I think our current insurance might pay for it so now is the time. We talked about the curve in her legs and he agreed that its time for x-rays to see how everything is looking. We also talked about how we watch Jilli's heart because it is a big muscle and that we need to do the same for Lydia especially since she now has a heart murmur that no one has head of before. Cardiology got us in on Monday. We talked about how in some ways whatever this is, it is likely effecting Lydia more then Jilli. Thats a bit of a hard pill to swallow but we need to have true eyes when looking at things.
Wheelchair:
We were told when we got the smart drive that we can't use it when their is salt on the ground. It also says that on the manual. It is been a winter where we have had salt on the ground since November so we have not been using the smart drive. The wheelchair guy that was there that day said it is fine to use it in salt but this is also the same guy who didn't know how to use the smart drive and drove Jilli into a table. I am trying to reach out to other user of the smart drive to see if they use it in the winter. I feel like the no salt thing wasn't mentioned to us before ordering and maybe we would hae thought about a power chair more if we knew that. But for now we will figure out how to make it work!
It was a very productive appointment. We got a lot done! Because of the added things that now need to get done (heart, x-rays, ect) We will be at rmh until next Thursday. Today Jilli has a feeding tube change out. Its been a busy week here and next week will be too but I am grateful to be getting so much done and for rmh for providing a bed so close, amazing staff and therapies that pour into my kids. Today is gardening and Jilli is SO excited!
Also, even though we are not home, we are still doing the Band-aid drive! We plan to make it to Church on Sunday and if you mail them to our house I promise they will get in the house safely. We have filled about half the box... who is going to help us finish!
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| Lydia helping to unpack |
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| Jilli's special gift from the Magic room |
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| RMH got a new surface table, it is so cool |
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| Caroline and Stacy were here Monday and Tuesday so we enjoyed time with them |
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| WE got a second card for RMH because they are so helpful for carrying oxygen in the play room |
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| When you buy a brand new pair of LLR and are so excited to wear them to be comfy in appointments but still look put together and you sit down at the appointment and something feels strange and you realize the back of your leggins are suddenly full of holes. There are the two big hold and tons of tiny ones. I have contacted LLR about it... I know they are the right size because its not the first pair of LLR leggins I have, and I know I got these on a sale, but they shouldn't fall apart. |
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