Sunday, August 25, 2013

Hear Ye! Hear Ye!

So, some of you know that Hannah has a fluctuating hearing loss that's recently gotten worse.  And I've had some interesting questions and comments about it. "Can she not hear me when I talk?", "She seems to hear fine", "I've never noticed her not hearing".  So, those are fair comments because really, she does so well most of the time. So here's my understanding of all this with what I'm sure is probably an oversimplified and maybe not totally accurate biology / anatomy lesson thrown in.  If you want to skip the anatomy and personal history - just go the last two paragraphs. :)

First, many young children get lots of ear infections. That's why tubes in ears are so prevalent. And besides true "infections", many young kids have fluid collect behind their eardrums.  That fluid can cause a temporary hearing loss until it drains out. It drains out through our eustachian tubes.  Most of our Eustachian tubes angle downwards to help the fluid drain out. But in very young kids, the tubes are closer to horizontal. That's why they tend to get fluid "stuck" behind their eardrums and sometimes that fluid gets infected.  So sometimes, doctors put tubes in their eardrums. Then when the fluid can't drain down the eustachian tube, it drains out the tube. As kids grow older (and bigger), their eustachian tubes begin sloping more downwards and fluid can drain out the normal way. And while anyone of any age *can* have an ear infection, this all explains why it's more common in younger kids and why many kids seem to outgrow the problem as they get older.

Remember this diagram from school? (except we called those 3 bones in the middle ear the hammer, anvil, and stirrup)

Now, kids with cleft palate have some anatomy differences besides the obvious hole in lip / roof of mouth. Their eustachian tubes are also more horizontal at a young age. And they also begin to slope downwards as they grow. But it takes much longer on average for them to slope downwards enough to outgrow the collection of fluid behind their ears.  Besides that, their eustachian tubes don't always work as well. It's called "eustachian tube dysfunction" and it happens to other kids too. It's just fairly prevalent in kids with clefts.  Think about how you may "pop" your ears when they feel stopped up. Hannah can't do that and it's because of the eustachian tube dysfunction.  Interestingly, I've recently heard that sometimes Asian kids also take longer to grow out of this eustachian tube dysfunction.  If so, then Hannah has 2 factors coming into play there.

Most kids with clefts see ENT regularly, especially at younger ages. All our visits to the cleft team include a check by ENT and audiology and that's pretty standard.  But we also see them a lot more often than the whole team. So - to make a really long story short, here's the basics of what happened. First, Hannah had fluid in her ears due to the eustachian tube dysfunction. Tubes were placed in her ears. They lasted 3 years. They never fell out like they were supposed to. They worked mostly well and hearing was normal / borderline normal during those 3 years. Sometimes they got blocked with wax and then her hearing would temporarily decrease until the dr. cleaned the wax out. But mostly she was fine. After 3 years, her ENT removed the tubes surgically because when left in that long, they can start to cause more problems. When they were removed she was left with a hole in each eardrum that never healed - something that happens to 2-4% of kids with ear tubes.  BUT, the holes were a blessing in disguise and acted as "natural tubes".  Hannah kept those holes for 2 years and during that time she had a fluctuating hearing loss that fluctuated between normal, borderline, and mild loss.  A year and a half ago (at 8 years old), her ENT repaired the hole in her left eardrum.  The repair went beautifully and Hannah has a perfect, complete eardrum. BUT, it immediately developed fluid again.  And slowly over the last year, her hearing has decreased, in both ears. It's been fluctuating between borderline, mild, and dipping occassionally to "moderate" loss.

Well, last month she had what seemed to be a pretty bad double ear infection. And I knew her hearing wasn't good then. But after several rounds of antibiotics (oral and drops), and having her ears cleaned and suctioned (as fun as it sounds), they finally cleared up. And interestingly, the final word was that as bad as her ears were, neither one was a true middle-ear infection (which is what she's gotten in the past). The right ear had a piece of an ear plug that had broken off and gotten stuck inside (no wonder she couldn't hear!). And the left ear was an outer ear infection / swimmer's ear. And it seemed to me that her hearing improved when all that was cleared up. So, we went back to the ENT Tuesday just for the purpose of checking that this ear infection was in fact gone. And it was completely cleared up. That's the good news.  Hannah will be 10 this week and is pretty much past the age where many kids with clefts are able to have workable eustachian tubes. So, the ENT went ahead and sent us to audiology to check her hearing and ear pressures. If all was good in the left ear (the one that was repaired), he would schedule her to repair the right eardrum.

But, all was not good.  She still had a flat tympanogram (fluid) for the left ear. And her hearing test for *both* ears was the worst they have been. Now, the good thing is her "bone conduction" tests are normal.  That means that when a device is placed against the bone behind her ear and sound is delivered that way (bypassing her middle ear), her hearing is normal.  That indicates that her hearing loss is "conductive" which means it's a problem in the middle ear, not a problem with her inner ear and nerves.   The good news is most conductive hearing losses are not permanent and many can be fixed medically. So..back to the ENT (just across the hall) who of course said he would *not* be scheduling Hannah for repair of her right eardrum and instead, it was time to put a tube in her left eardrum again. There are risks with that because he's going into an already repaired eardrum. But at this point, the risks of not doing it are greater. He thinks her hearing may go back to almost-normal in that ear with the tube.  I asked why her right ear had just as much of a hearing loss. He said it might still be a little inflamed from the infection last month but also the longer she had a hole, the more likely her hearing could get worse. But the solution to that is to repair the hole, which he can't do because she still hasn't outgrown the eustachian tube dysfunction. If he repaired her right eardrum now, he'd be in the same position of having to go back in and put a tube in again.  The hope is she might be nearing a growth spurt sometime in the not-so-distant future and the increased growth could finally help her eustachian tubes. But we will wait a good while before attempting another repair.

What this means is that her hearing will likely continue to fluctuate for quite some time. And as it's been fluctuating at a greater level lately, it's very likely that will continue at least for the right ear. The left ear may fluctuate more mildly after the tube, but that remains to be seen for sure. So, how bad is her hearing? Her hearing at the lower frequencies went from "moderately severe" at the lowest to "moderate". At the higher frequencies it was "mild" to "borderline normal".  This means she has a harder time with lower frequency sounds.  Interestingly, people tend to whisper at higher frequencies. So sometimes, Hannah might hear something as soft as a whisper. But other times, she doesn't hear something that seems so clear to others. There are certain speech sounds she hears fine. But she has trouble with other speech sounds.  She misses word endings often (and has even when she had a milder loss). So for example, she may not know if you said "she walks" or "she walked". When you have a face-to-face conversation with her in a somewhat quiet environment, she may hear everything you say. But the farther away from you she is, the more she will struggle. The more background noise there is, the more she will struggle.  Also, she may struggle more later in the day. Sometimes, she *can* hear things but it takes a lot more focus and attention than it might for others. So that could explain why she's often tired at the end of the day. Even last spring, she'd get in the car after school and need some "down time" before she could engage in conversation again. She does well, but it takes effort. She's also pretty good at using context to figure out what she might have missed. She does this naturally without always realizing she missed something. For example, if I say "Hannah go upstairs and brush your teeth" - she may hear most but not every sound in those words. But because I've said that to her often, her mind "fills in" what her ears might have missed.  This works pretty well for topics that are familiar. But for novel topics, it becomes difficult.  If you took your favorite book that you've read several times before and this time you could only see 4 out of every 5 words, it would be a little of a challenge. You might have to put some effort into reading it, but you could probably get through it and understand it well because it's so familiar to you. But then imagine taking an economics text book (unless you're an economics person) and being able to only read 4 out of every 5 words. For that 5th word, you might even see half the letters, but because the word is a new, unfamiliar term, you can't quite figure out what the word is. It would be much harder to comprehend. Then imagine that there were lines and arrows drawn all over that book that pointed to phrases written in the margin that had nothing to do with the book.  It would be distracting.  That's kind of like Hannah trying to listen to her teacher in class teach new information while hearing the kid behind her shuffling papers, the air conditioning, the kid giggling beside her, etc. It's a lot to sort out and takes a lot of effort. Add to that that her hearing loss if a "fluctuating loss". So some days she really might hear "just fine" (relatively). But other days, it only appears that she is hearing fine, when in fact she is not. So that pretty much sums up Hannah's hearing. She will have the tube placed in her left ear mid-September and I hope that will help. Meanwhile, we're working on some other strategies to help compensate in the meantime.

And how has Hannah handled on this? Well, it depends. She's highly irritated over the fact that she needs a tube in her left ear.  On day 1, "irritated" wasn't really the right word. "Furious" might be more accurate. She says it makes no sense because she had a perfect eardrum, then "we" (because you know, I was totally "helping" and it's my fault according to her!) created a hole when we put a tube in, then we took the tube out, then we fixed the hole, and now we want to PUT A HOLE BACK IN THE EARDRUM?? Yeah, I kind of agree. Makes no sense when you look at it that way. But it is reality. And she's better with it now. (i.e. "irritated" rather than "furious".)  But the news that she has a more significant hearing loss? Doesn't bother her at all. I mean, her hearing is no different after being told than it was before being told.  It all just seems normal to her.  And in the grand scheme of things, this is not a major problem. It really isn't. She will need a little help especially in not missing instruction at school. That's all. But it's totally "doable" and she will go on with her singing, laughing, flipping, dancing "tween" self.  Her birthday coming up this week is a much bigger deal. As it should be. :)

Sunday, August 11, 2013

Update on Sadie

Sadie's surgery went well overall.  We arrived at the hospital and she quickly got her vitals done and was in a room.  Surgery was delayed just a bit because the anesthesiologist had some concerns.  You may remember that Sadie has some mild kidney issues. Her kidneys are located in slightly different places than is typical. The left kidney shrunk in size significantly between the time she was 4 and 6 years old.  It is likely not contributing much in terms of function. But her right kidney is growing well and compensating well for the lack of the other one. She takes 1 medication for mild metabolic acidosis (a kidney-related issue) but her actual kidney function is normal. There are no side effects and according to her dr - no precautions or restrictions for her. (Except we avoid NSAID's such as ibuprofen because they can be hard on kidneys). Well, when I filled out all the pre-anesthesia forms I put all that information in the appropriate places and listed the medicine, dose, etc. I included her nephrologist's contact info. Why do we fill those forms out? They certainly weren't read (or if they were, they weren't read by the anesthesiologist). He seemed genuinely surprised and concerned to find out she took this medication.  So he wanted to do lab work before the surgery to check that all was good. While I was confident (based on knowledge from her other doctors) that all was fine, I still appreciated that he wanted to be careful. They did the lab work and it all came back great.

Sadie was quite the charmer the whole morning.  She refused to lie down for the blood draw and said she wanted to watch. She held out her right arm and pointed and said "here's where you need to stick me.".  When the anesthesiologist left me and asked if I had any further questions, Sadie piped up and said "I do".  She then proceeded to ask him "Are you going to flavor the gas that makes me go to sleep? Because last time you rubbed chapstick on it?". He asked her if she wanted him to do that. She replied "no. It doesn't really help. It just makes it smell like stinky chapstick. I think it's better if you just forget that." lol!

So - surgery went well and she did great.  But then in recovery and post-op, the decision was made to limit her IV fluids.  Now, in the past Sadie has had a tendency to get nausea after surgery. It's generally mild though. And what her nurses have done in the past is give her extra IV fluids before discharge and that really seems to help prevent or minimize the nausea. (Which is much appreciated as we have a 3 hour drive home!). But this time, they not only wouldn't give her extra fluid, they wouldn't even give her the normal amount. They limited it pretty significantly and said they were ordered to do that because she takes sodium bicarb (her medicine). Now, they perhaps had a very valid reason for this..but they couldn't say what it was and I really don't think so. From everything I know..the risk of dehydration is a bigger risk than giving her too much fluid. With normal kidney function, she has absolutely no fluid restrictions in normal daily life.  When Sadie first woke up, she was okay. In fact, she seemed to be doing really well. She drank just a couple sips of juice and then they started preparing discharge papers.  As soon as she got in the car, she threw up. Then she seemed okay for a while. She had a drink in the back with her, but she didn't drink but a couple sips. She mostly slept all the way home.  But once home, I started pushing her to drink and she couldn't keep anything down.  She was also throwing up her pain medicine which later in the evening meant she was in pain.  I called her surgeon's office and got the on-call doctor, who happened to be her surgeon's resident. He had been in surgery with Sadie that day so he knew her and her situation. I asked him to call in some anti-nausea medicine for her and he did. He was very nice and left his cell phone number and told me to call him back if we had any other problems. The medicine did the trick. She was able to finally start drinking a little and keep her pain medicine down. She slept most of the night and the next morning, she was totally acting like herself again.

My concern is that her next surgery (in 2-3 months) will be longer. I really don't want them limiting her fluid again because I'm convinced that's what made the day so rough for her. So I will be calling her nephrologist this week and asking him to send something both to her surgeon and a copy to me indicating what, if any, precautions she needs and indicating that it is okay and even needed for her to have the extra fluid. (If he agrees and I'm not missing anything. But I think he will agree). I spoke with her surgeon about it as well and he said yes, have the neph send him the info but also he can plan to keep her overnight next time to make sure she gets what she needs.  So, we have a plan. :)

Meanwhile, Sadie has this lovely metal bar attached to her arm.  For the first 5 days it was bandaged.  She did well remaining mostly inactive, except for the fact that the night before we went to get the bandage removed, she fell down the stairs. Down 9 stairs to be exact and crashed into the shelves at the bottom! Um, that's not really a good thing. But her instincts kicked in and she fell protecting the surgery arm. It scared her more than anything and she wasn't hurt. But still..I'd appreciate it if she didn't do that anymore!

So, we got the bandage off and I got instructions for her surgeon on activity level, cleaning the pin sites, and how and when to turn the fixator for the next week.  Sadie of course continued to charm everyone there and somehow left with a pile of candy! Then we went next door to see the OT's who normally make her splints. This time they were charged with making her a splint / protective cover for the fixator.  The lady we were working with was not thrilled to have this task and spent the first 10 minutes telling me she didn't have any idea how to do that.  But, I had some ideas to throw out. And then she finally got a co-worker to come brainstorm with her and the co-worker had some ideas.  Finally a plan was created and she started creating this contraption, um, I mean splint. She did it while mumbling and muttering under her breath at first, but slowly became more personable as a workable solution started to take shape. So Sadie now has a VERY custom splint - one of a kind! The OT was in such a foul mood in the beginning that she didn't even give Sadie a choice of colors like they normally do. But that's okay, because we took the plain white splint and added some of our own "bling" to it.  I have to appreciate them though because the OT's there are really good at what they do AND they know how to bill insurance the right way to get it covered. (A problem I ran into locally). And they work with the surgeon next door (who I love!). But they are not always the most thrilled to see us.  I figure they should be happy to see Sadie - her needs dictate they learn new skills and expand their creativity! ha! (Of course, when it comes to OT's, I'm kind of spoiled because in my job I work with the best OT's and they manage to be good at their jobs AND be friendly!).

So, the very next day - Sadie woke up and I could tell her hand had been bleeding at the pin sites. Not a lot, but that's not really a good thing. But being so early in the process I wasn't sure if it might be normal. So I took photos and e-mailed them to her surgeon. He responded back rather quickly and said to see how it did in a day or two. If it continued, we could stop the turning of the fixator to give it more time to "heal". But regardless he wanted to start her on antibiotics. They are (as they admit) very paranoid about infections with the pin sites of these fixators. Better to be safe than sorry. So, his assistant called in some antibiotics and she started it that night.

Since then, no problems. No more bleeding or signs of infection. Everything is going well. Now the biggest challenge is reminding her not to carry or lift things with that arm and keeping her from falling down the stairs again! That keeping my very active tumbling, flipping, dancing, leaping older child from doing those things while standing in the same room as Sadie! (Which is actually a challenge her grandparents have for a few days as the girls are there having fun and being spoiled while I return to work tomorrow!). We will return to Atlanta on Wednesday for an x-ray and further instructions on when and how to turn the fixator. (There's multiple places you can turn it - we are going one direction this week..will do a slightly different direction next week). I also hope to find out a little more on how long he expects this progress might last (he can't give me an exact depends how she responds).

Sadie amazes me though with her resilience, her confidence, and her bravery!

Meanwhile, Hannah's had her own little medical issue going on. She basically got ear infections in both ears the first of July. And they are just now clearing up. She had three rounds of  antibiotics and then the day before Sadie's surgery, Hannah had an ENT appointment to try to get to the bottom of it. Both ears were red and draining. yuck! She got her ears suctioned and cleaned (a process she did not enjoy) and they found the problem in the right ear: a piece of an earplug had broken off and got stuck against her eardrum.  I had said her hearing had decreased - now we know why! It was removed rather easily. Nothing unusual found in the left ear, but that eardrum was red, infected, and draining too. She started antibiotic ear drops which she hates! But she got through it and now her ears look better, are no longer draining, and she is definitely hearing better.  We go back in a couple weeks just to double check, but hopefully she finally over all that.
(just for fun - happy girl!)


 (day after surgery)

 (So adaptable - playing cards!)

 (a blinged-out splint / cover)

Summer Recap

Well, it's official. Summer is over. I start back to work Monday and the girls start the following Monday. We had lots of fun things, and some not-so-fun things. I completed some projects (well, okay, maybe 1 project). Other projects that were planned are now getting pushed back to the next break. :) But overall, it was a good summer. So here's our summer 2013 recap. Mostly in pictures.


Notable things in June: We had lots of free time to swim, play, play games, do crafts, hang out with cousins, and just enjoy being together. I loved June! We also spent a weekend at grandma and grandpa's house. Sadie took 5 days of swim lessons from an incredible instructor. She went from not swimming at all to being able to swim the length of the pool freestyle, the width of the pool backstroke ,floating on her back for 1 minute and treading water for 30 seconds. So proud!! She still needs some practice before I'm confident with her swimming away from me, but she made HUGE progress.


July brought more fun. It was the busier of the two months though with girls and I going every which way. There was a family trip to Mobile Bay which was lots of fun! The girls had great experiences at camp. They had VBS. I had a workshop in Huntsville.  And Hannah got her "new room" she's been wanting.  I redid her room while she was at camp as an early birthday present for her.