Sorry it has taken forever for me to get to the summary of the actual appointments. I will try to be concise!
1) Audiology. This was a brief appointment to dispense new ear molds for Evan's hearing aids. Unfortunately, when the audiologist inspected the molds right before our appointment, she discovered that the lab had sent molds made with "short" extensions into the ear canal ("long" extensions were ordered). Evan's left side has enough twists and turns in the external ear to support the mold without extending much into the actual ear canal. The cartilage is much stronger and can actually support the aid. His right side (the more "CHARGE-y" ear) is another story--the cartilage is very floppy and the external ear has very few folds. There isn't much for the ear hearing aid to cling to unless the mold extends deeper into the canal. We have always had issues getting the right hearing aid to stay in (or not constantly chirp), which is unfortunate because that side actually tests with slightly better hearing. Because of more feedback on that side, the aid has been programmed with a lower volume setting to reduce feedback. The most recent set of molds did not hold the right aid in at all, so Evan hasn't worn it much.
The audiologist attached the new molds (the "short" ones). The left one fit fine. The right one appeared to be staying in OK, with minimal chirping, so the audiologist reprogrammed it to the target volume setting. That made it chirp more, but she left it because the "long" ear mold was on it's way. She also wanted us to try hearing aid tape (basically toupee tape) to help hold the aid on the side of Evan's head, thinking the mold might stay in better if the weight of the aid wasn't pulling on it. This lasted maybe an hour--so I would say not so much.
The new right mold came in the mail on Friday. As Jeremy was attaching it, he discovered a possible other reason for the feedback: there is a crack in the plastic tubing. The ear mold comes with a piece of flexible plastic tubing embedded in it, that you cut to fit. There is a tubing made out of a harder plastic that connects the hearing aid speaker to the mold tubing. This plastic tube doesn't get changed with the molds, and it is what is cracked. Jeremy covered the crack with a little piece of tape, and that helped with the feedback when Evan wasn't wearing it. Ironically, the "long" mold is maybe too long. It must be hurting, because Evan cried and cried when we put the right aid in. We will try again this week (maybe he just isn't used to the aid on that side).
I have been resistant to any surgical procedures to alter Evan's external ear. Mostly because Evan has been through so many surgeries already...surgeries that he hasn't had any choice. I want him to participate in the decision to pursue any "cosmetic" surgery. Partly also because I want Evan to know we think his ears are beautiful and we love them (and him) just the way they are. Partly too because I know surgery can only do so much to make the ears look symmetric, and it's still going to look like there has been surgery done. If that's the result, then I don't see the point. The ENT mentioned that a fairly minor procedure can be done to make the ear hold the hearing aid a little better. This we may consider.
2) Urology. Basically, things are stable.
3) Craniofacial. I had concerns that Evan's shoulder joints seemed weak to me (when you pick him up by holding his chest under his armpits, it feels like the shoulders are trying to dislocate...like there isn't enough muscle or ligaments holding them in). I wanted to know if an ortho consult might be a good idea (aside from the fact that CHARGE is known for scoliosis and other bone issues down the road). I was reassured that Evan's shoulders are OK...they just feel like that because he has "low tone." That assessment doesn't really sit well with me, but I have learned that pushing an issue with this particular provider doesn't yield the desired results. I'll just discuss it with our PT and Dr. Oldham. And other CHARGE mom's--you have seen photos and videos of what Evan can do. Does he look like he has any "low tone" to you??? He is very strong--legs and arms--he can pull himself up to standing and push things. He may have had tone issues in the past, but I don't think that is the problem right now. We briefly touched on the ear infection issues ("Are they draining???" Arrrggg...more on why this makes me cringe in the ENT section). I asked about testing to rule out choanal stenosis. Dr. Lewis thought it was not possible to have stenosis in the presence of a cleft, but she would leave that to ENT. Possibly Evan could have some underlying immune deficiency that makes him more prone to them. Dr. Lewis thought we should monitor things for a few months and then consider an immunology consult. She ordered some basic blood work to screen for an immune-system issue after I pushed ("He can't wear his hearing aids. This is the critical period for language development.").
4) Dietician. Cam was very pleased with Evan's "numbers." He grew another inch since age 2--at 2 1/2 he is 34 inches (50th percentile). He has actually lost weight at 25 lbs (he had been up to 27), but we think that was from when he was sick. With his weight and weight vs. height he is also at the 50th percentile. Evan has really thinned out in the past 6 months--from about age 1-2 years he was pretty chubby. Cam was happy to hear the blendarized diet has worked so well for Evan, and the only changes we are making is to add half a Flintstone's vitamin to each day's blend. We will also be gradually taking out the 1 can of Pediasure he gets with the day BD and replacing it with 8 oz whole milk plus 1 teaspoon flax seed oil. At that point, he will only be getting one can of Pediasure plus his water at night. His day feedings will be all "real" food!
5) ENT. This appointment was a little bit frustrating, to say the least. To start with, we waited for an hour to see Dr. Perkins (this was after we had already spent an hour waiting and seeing the other two providers)...long enough that we had to "reschedule" our nephrology appointment to the end of the day. We explained to Dr. Perkins our concerns about Evan's recurrent ear infections, and how it is really having an impact on him being able to wear his hearing aids. We got the by now very familiar "How do you know it's an ear infection? If it isn't draining [pus], then it can't be an ear infection?" By this point, I am getting a little ticked off because, we've been waiting so long to hear the same song and dance. I just said "Well, I don't know what to tell you. His pediatrician (who Dr. Perkins knows is a seasoned Dr., and knows what an ear infection looks like) says the ears are infected. When he is on the antibiotics, the infection clears up." Dr. Perkins feels that there is a myriad of possible infections a kid like Evan could get because his palate muscles don't work properly (because of the soft cleft--even though it's been repaired, the muscle fibers still don't function normally), because of his poor swallow there is probably aspiration. He reviewed a CT Evan had taken about a year ago and said that although he did show some narrowing on one side, it wasn't narrow enough to be stenosis. We talked about VitalStim and oral feeding (is there a connection between feeding and the "infections")--Dr. Perkins thought that the improvement Evan showed in being able to handle his secretions alone meant VitalStim was very successful with him. I almost got a hint of "this is sooo much better--what more do you want?." His recommendation was to keep Evan on the prophylactic antibiotics for 6 months, not feed him orally during this time and see how he does. He also wanted to repeat a BAER under anesthesia (the most recent BAER showed improvement with bone conduction, but Dr. Perkins thinks this is just "noise" in the test, not an actual improvement). He also wanted us to keep Evan as healthy as possible for the swallow study already scheduled for January 30th. He predicts Evan will still show some silent aspiration--it will be better than his previous study--but he still will not be "cleared" for oral feeding (the last study showed enough aspiration, we were told not to give any food by mouth...we've been bad).
6) Nephrology. Evan has been assigned a new provider--Dr. Symmons (Dr. Eddy, his previous Dr., got a big research grant and had to cut back her clinic time). In a nut-shell, everything looked stable...but the most recent kidney U/S was 6 months ago and they didn't have any lab results yet. We were advised to be very careful about the possibility of kidney infection (always get a catheter urine culture with any fever...which to be honest, if the ear looks obviously infected Dr. Oldham hasn't been doing), and told that Evan is 100% guaranteed to develop hypertension at some point during his life because the kidney is critical in regulating blood pressure and Evan has chronic renal insufficiency by default because only one kidney is present. Elevated blood pressure damages the kidney, so we want to keep an eye on this. Evan's blood pressure right now is normal. The fellow working with Dr. Symmons did most of the exam, and saw Evan was on prophylactic antibiotics when we reviewed the med list. He said, "That's between you and your primary care pediatrician," not without an air of you-know-you-are-promoting-antibiotic-resistance. (As an aside, we certainly do not have wide-spread antibiotic resistance because Evan is on them prophylactically, and it's not even because pediatricians over-prescribe them for pushy parents. It's because for years the livestock industry dumped antibiotics into cattle feed. That's where most of the resistance has come from. A little less soap-boxing would be appreciated.)
We got some lab work done (blood draws are never a good time for Evan, but he only needed 2 sticks to fill 4 tubes) and got some dinner. We left Children's around 6 pm. It was snowing at the hospital...not a good sign for the pass. It was snowing pretty hard on top (traction tires required for cars, trucks had to chain up), and it took longer but we made it home safely around 9:30. Evan was a very good boy. He happily clanked his puzzle pieces together all the way home.