Wednesday, July 13, 2011

This Squeaky Wheel Got Greased

Tuesday morning I called the MD clinic to see if they had any openings come up. The scheduler did not have any openings, but assured me that I was on the list to call when something did come up. Less than 5 hours later, my new best friend called to tell me that they just happened to have an opening come up in the schedule for this morning. Praise God! We had SO many people praying that we would get in sooner and it was very apparent that God answered this prayer. I was seriously giddy the rest of the day. I don't know that I've ever felt that type of excitement, literally to the point of being nauseous. Leah was less than thrilled that she would be missing her 3rd day of dance camp and was even less impressed to find out it was for a doctor's appointment. None the less, we were going.

Leah has definitely been sensing our anxiety the last few weeks and has asked a lot of questions regarding why she was going, did she have to and the like, usually accompanied by lots of tears. When we got to the clinic we found out we were actually seeing the MD specialist's coworker, who was more of a general pediatric neurologist. We were told that they had talked about Leah's case and that this visit was not to replace the appointment in February, but rather to tide us over. We went over all of Leah's history and she did a full physical exam. There was a lot of pushing and pulling of hands, feet, legs, head, etc. The doctor was very friendly and thorough. When she finished the exam, she explained that she noted some general weakness, but that it wasn't too bad.

The area she found the most muscle weakness in was surprising to us all. Her neck, chest, shoulders and upper arms seem to have been affected the most. This is obviously an odd area for weakness and lead the doctor to believe that we probably are dealing with some form of Muscular Dystrophy. In addition to the weakness, Leah's tone is low. I asked for clarification on this and learned that this means she is "loose, overly flexible and floppy". Her muscles, ligaments and tendons don't do their job holding stuff in it's place. It surprised me because one of the things we've been working hard on, both in PT and at home, is her tightness. Her Achilles tendons and the ligaments/tendons behind her knees are super tight. The doctor showed us examples of her low tone (her fingers when spread can go much further than normal, her ankles roll inward, etc) and explained that ligaments and tendons that are tight are probably trying to compensate for the low tone and weakness. They try to counteract each other's problems. While it's neat that the body tries to fix itself, this is one of those cases that one problem leads to the next.

She then noted that while her reflexes are spastic, they are not pathological (I think that's what she said at least). Basically when they check her reflexes (rubber hammer to the knee), her feet spasm and she has what looks like an exaggerated response. My understanding is that her reflexes are still in the normal range, showing that her issues are most likely not nerve related. This is good news, especially since it means we don't have to have the elctromyography done, for now. She showed us some ways to check for muscle spasms when Leah is in pain. If she were having true spasms, then they would further evaluate for nerve problems.

We also discussed my grandpa, who was discharged from the Navy in the 40's with a muscle disease that they called MD, but were never able to pinpoint what was going on. They obviously didn't have the same technology and capabilities as they do now, but I did have a whole stack of papers from my grandpa to take with us. I spent sometime talking with my dad tonight and apparently grandpa had severe weakness in his neck, chest and upper body, as well. He was a part of several research studies and they told him he wouldn't live past 30 due to the severity of it. Despite this prognosis, he lived into his 60's, when cancer eventually took him. He was very successful and often hid his pain and disability. He wound up being one of the scientists working on the Manhattan project and the atomic bomb. He was a fascinating character, just like my dad. Dad told me that often grandma had to physically push him out of bed because he couldn't lift his head off of his pillow. All of this info would have been great to have today and I plan on conference calling him during our next appointment. :)

The doctor also reviewed all of these reports and labs. It's a huge blessing to have that history and information. Based off of the physical findings, Leah's history and our family history, the doctor feels it is quite likely that Leah does have a form of MD. She said that her symptoms don't fit any of the classic, well known MD's completely and suspects it will be a rare form or even possibly a new form. Looking at my grandpa's life, my dad (who also has had similar symptoms as my grandpa did) and Leah as of today, the doctor assured us that her prognosis was very good.

We had some blood drawn for some testing that the doctor didn't really specify and should have results on those next week. I am not expecting any big answers from it though. She did order a sleep study since Leah's legs often bother her at night (common in muscle disease), she still snores and she is still so tired despite getting 12+ hours of sleep a night. There is a small chance that she is having trouble supporting her airway while she sleeps, thus causing the snoring, possible apnea and tiredness. This probably wont happen for a few months. In February we will see the MD doctor and he will probably order a muscle biopsy, as well as some more tests.


Until then, we are to let Leah set the pace. The goal is to get her to the point that she can A) know when her body is tired or is on the verge of being in pain and B) voice this appropriately (see: without tears, screaming, drama, fits, etc.). This is going to be VERY important because there isn't much we can do for the pain when it gets bad other than heat, tylenol and massaging the afflicted area. Also, by the time she is in pain, she has already possibly done damage. If we can get her to recognize how she feels before she is in pain we can hopefully avoid the pain and damage all together. It's going to be imperative that she feels comfortable talking to someone at school about this so that they can help us avoid the afternoon meltdowns, pain and exhaustion that we dealt with all last year. This may mean she has to leave early or come late when she's having trouble. She will probably need to modify her PE time too. Hopefully they will work with us!

In addition to teaching her to recognize these warnings, she instructed us to be very careful about extraneous activities or anything that might damage her muscles. Normally when you attempt to get stronger, you exercise causing your muscles to break down so they can grow back stronger. In MD, the muscles do not regrow properly or at all. If she does indeed have MD and works on building her strength, she could do irreversible damage to her muscles, wasting away what good muscle is still there. This fact alone was so worth the visit and pushing to get in before February. We have been working hard on her strength in therapy and it scares me to think we could have done damage without knowing it. Our focus will now be strictly working on stretching out those tight areas and working on skills like balancing.

Leah has begged us to take ballet for a long time now and has been so cute going to dance camp this week. She LOVES it. I asked the doctor about continuing with a dance class and she said she could try it this fall as long as it didn't cause more issues. Unless it causes her pain, we will let her enjoy every minute of it. I'd hate to hold off on it only to find out she can't do it later. We've been blessed with the opportunity to have her take lessons at a small studio that a friend from church owns. It couldn't be a better fit for Leah.

So for now we will wait for the blood work to get back, get in for a sleep study, let Leah call the shots and take it a day at a time. We know that Leah is God's child and that He holds her in His hands. He loves her more than we do and His goals, dreams and aspirations far outweigh anything we could wish for her. We are so thankful that we got in early and that we have some answers to grasp and wrestle with.

We did talk to Leah on the way home and explained some of what is going on. We need her to be aware, but still want to protect her. We also wanted to be certain to get a clear diagnosis so that we don't mislead her. We have avoided using terms like Muscular Dystrophy and disease, as she is aware of what those are. After all, she knows Uncle Jorge and has "worked" at the MDA telethon almost every year since she was born. For now she knows what to watch for, that God made her exactly how He wanted her and that we love her. For now, we think that's enough.

I know this post is long, but here are just a few ways we've seen God at work in this:
- We had so many praying for a sooner appointment. Our therapist on Monday told me to call the MD clinic everyday if that's what it took. It only took one day. :) With appointments being scheduled 8 months out, I can't help but think we weren't the only ones on the wait list, nor were we first on the list. Also, my brother Jorge's, who passed away from MD (he was adopted), doctor is now the Chief of the clinic. So many little connections.
- The fact that we are already established with two therapies is awesome. Through recommendations from friends, we found two amazing, Christian therapy centers that truly love Leah and care about her best interest. When I called to tell them we got a sooner appointment, they were as excited as I was and sharing the news with each other before we hung up. They truly care. Both of these places have wait lists, often as long as a year, for therapy.
- While our insurance is not that great, they cover most all of the testing under our specialist copay. I can't tell you what a blessing this has been to have this insurance, at THIS exact time, with all we are having done. So much easier than our other 80/20 policy.
- As soon as we know for sure what Leah is dealing with, we will have Kate tested too. Obviously we need to know, but especially since Kate has had lung and heart issues. MD affects all muscles, including the heart and lungs. Praise God that we have a great cardiologist and pulmonologist already should we need them again!

1 comment:

Unknown said...

Praying for you guys!!!