I keep telling myself, it could have gone much worse!! Thank goodness I had only Connor with me, because if I had had Ian and/or Gavin with me also, I might have lost my mind!!
Connor's ENT is at Cinci Children's, which is about 90 minutes from us (we're in North Dayton). He goes to one of the outpatient clinics about 70 minutes from us, so we try to get our appts there whenever possible. Yesterday Connor's appt was supposed to be at 3:45, but he has to see audiology first, so we were told to get there at 3:30. We got there at 3:26. Perfect, right?
It was busy in the waiting room, but there are several clinics that share a waiting area, so I didn't think anything of it. Connor nursed for a while, then played for a while, then I got to chatting with another mom, so I didn't even notice how much time was passing. Then I looked at the clock and it was 4:45 already!!! An HOUR after our appt time with the ENT, and we hadn't even been to audiology yet!
So I asked at the desk what was going on, they said they were running behind (no kidding) and would get to me soon. Okay... It was starting to get hard to keep Connor occupied, I was getting hungry, I hadn't brought my pump with me (Connor only nurses from one side, so my other side was starting to get full)...another whole hour passed before we got in to audiology!
The good news here is that Connor is getting old enough to really cooperate with hearing tests, and I'm trusting the results more. Instead of subjectively watching his response to sound (did he look, did he blink?) this time he was able to be "trained" to throw a ball into a bucket when he heard a sound. And if he hesitated, they didn't count it as a response. A few times he started to throw the ball in, stopped, looked to the tester for guidance...that wasn't counted as a response.
Finally we finished the hearing test and went to see the ENT. His left ear tube is completely out, but the hole is still open. His right ear tube is half out, and is crooked such that he got a flat reading on his tympanogram (the air didn't flow through it). His right ear canal is a little bigger than a newborn's (finally!) his left is still smaller than a newborn's, but with the special speculum otoscope the ENT could see his ear drum (what ear drum there is to see...his left ear drum is malformed). His hearing test showed 40 dB loss at low frequencies (250, 500, 1000) and 25 dB loss at 1500. The rest of the frequencies were at 20 dB.
Here's where it gets really frustrating... This kind of loss can't easily be aided, the aids can't be programmed easily for only those low frequencies without potentially causing loss at the frequencies that he has normal hearing. AND the shape of his canals (he has atresia/stenosis) would make it very hard to fit an aid without getting significant feedback. And his external ear has grade 1 microtia, so fitting a mold would be really hard, and his ear might not hold up the weight of an aid anyway (he just doesn't have the cartilage). So hearing aids still aren't a good answer for him.
Then we started talking about his tubes. Obviously the tubes have REALLY helped him, with his palate still not corrected (he has a submucous soft palate cleft and VPI, neither have been corrected yet) and with all the malformations in his ears, the tubes are a must. BUT...putting him under anesthesia is never easy because of his airway issues (laryngotracheobroncho malacia). So he requires a 23 hour hospital stay for every sedation. And with his immune deficiency (PID, t-cell deficit, b-cell dysfunction), having him in the hospital in the winter is not a good idea. But then again, because of his immune deficiency we don't want to risk him getting more infections because his tubes are out.
SO...see how we're stuck in the middle of a huge Catch 22??? (which is ironic, because CATCH-22 is actually another name for his syndrome, CATCH being an acronym for the more common symptoms, 22 referring to the deleted chromosome).
He has hearing loss...we can't aid him. His tubes are both out, it's not easy to replace them.
I actually have begun polling his other specialists to see if anyone else wants him under anesthesia for any other reason, thinking that if we have any other procedures that need doing, then it would justify the risk more. But so far no one else does (which is a good thing!).
So the decision right now is to wait until March to make a decision. If he has even one more ear infection between now and then, the decision will be made for us, tubes immediately. If he loses any more hearing between now and then, the decision will be made for us, tubes immediately. If he manages to have no infections and his hearing stays the same, then we'll keep putting it off two months at a time.
So I left the dr after 6pm and started the hour plus drive home. Both of us tired and hungry. Then it started snowing, and here in south Ohio people jsut aren't used to snow. There's a long stretch of the main high way under big time construction, so it was a nightmare!!! I finally got off the high way and just took surface streets home, which took longer but at least I got away from crazy drivers and construction. We got home at 7:30pm. I was in such a crummy mood by the time we got there, I had to go hide in the bedroom for a while to stop from snapping at everyone.
I just hate being in this position where he doesn't fit any conventional treatment plan. All things considered, he's doing SO well, REALLY well, and I have to remind myself of that. But appts like this are a stark reminder of how "abnormal" he is in so many ways!!!! He sees his pulmonologist soon, and I expect that appt to be similar...we know what's wrong, but it isn't fixable, and it's all complicated because of his underlying syndrome. All we can do is keep chugging along. Same with gastro...we know he's not normal, but it really isn't fixable, so we just keep going. Heck, even his palate isn't quite a "normal" cleft!!! It took two years for even the palate specialists to find the darn cleft, and we haven't even started talking about how to go about fixing it!!
SIGH.
Connor's ENT is at Cinci Children's, which is about 90 minutes from us (we're in North Dayton). He goes to one of the outpatient clinics about 70 minutes from us, so we try to get our appts there whenever possible. Yesterday Connor's appt was supposed to be at 3:45, but he has to see audiology first, so we were told to get there at 3:30. We got there at 3:26. Perfect, right?
It was busy in the waiting room, but there are several clinics that share a waiting area, so I didn't think anything of it. Connor nursed for a while, then played for a while, then I got to chatting with another mom, so I didn't even notice how much time was passing. Then I looked at the clock and it was 4:45 already!!! An HOUR after our appt time with the ENT, and we hadn't even been to audiology yet!
So I asked at the desk what was going on, they said they were running behind (no kidding) and would get to me soon. Okay... It was starting to get hard to keep Connor occupied, I was getting hungry, I hadn't brought my pump with me (Connor only nurses from one side, so my other side was starting to get full)...another whole hour passed before we got in to audiology!
The good news here is that Connor is getting old enough to really cooperate with hearing tests, and I'm trusting the results more. Instead of subjectively watching his response to sound (did he look, did he blink?) this time he was able to be "trained" to throw a ball into a bucket when he heard a sound. And if he hesitated, they didn't count it as a response. A few times he started to throw the ball in, stopped, looked to the tester for guidance...that wasn't counted as a response.
Finally we finished the hearing test and went to see the ENT. His left ear tube is completely out, but the hole is still open. His right ear tube is half out, and is crooked such that he got a flat reading on his tympanogram (the air didn't flow through it). His right ear canal is a little bigger than a newborn's (finally!) his left is still smaller than a newborn's, but with the special speculum otoscope the ENT could see his ear drum (what ear drum there is to see...his left ear drum is malformed). His hearing test showed 40 dB loss at low frequencies (250, 500, 1000) and 25 dB loss at 1500. The rest of the frequencies were at 20 dB.
Here's where it gets really frustrating... This kind of loss can't easily be aided, the aids can't be programmed easily for only those low frequencies without potentially causing loss at the frequencies that he has normal hearing. AND the shape of his canals (he has atresia/stenosis) would make it very hard to fit an aid without getting significant feedback. And his external ear has grade 1 microtia, so fitting a mold would be really hard, and his ear might not hold up the weight of an aid anyway (he just doesn't have the cartilage). So hearing aids still aren't a good answer for him.
Then we started talking about his tubes. Obviously the tubes have REALLY helped him, with his palate still not corrected (he has a submucous soft palate cleft and VPI, neither have been corrected yet) and with all the malformations in his ears, the tubes are a must. BUT...putting him under anesthesia is never easy because of his airway issues (laryngotracheobroncho malacia). So he requires a 23 hour hospital stay for every sedation. And with his immune deficiency (PID, t-cell deficit, b-cell dysfunction), having him in the hospital in the winter is not a good idea. But then again, because of his immune deficiency we don't want to risk him getting more infections because his tubes are out.
SO...see how we're stuck in the middle of a huge Catch 22??? (which is ironic, because CATCH-22 is actually another name for his syndrome, CATCH being an acronym for the more common symptoms, 22 referring to the deleted chromosome).
He has hearing loss...we can't aid him. His tubes are both out, it's not easy to replace them.
I actually have begun polling his other specialists to see if anyone else wants him under anesthesia for any other reason, thinking that if we have any other procedures that need doing, then it would justify the risk more. But so far no one else does (which is a good thing!).
So the decision right now is to wait until March to make a decision. If he has even one more ear infection between now and then, the decision will be made for us, tubes immediately. If he loses any more hearing between now and then, the decision will be made for us, tubes immediately. If he manages to have no infections and his hearing stays the same, then we'll keep putting it off two months at a time.
So I left the dr after 6pm and started the hour plus drive home. Both of us tired and hungry. Then it started snowing, and here in south Ohio people jsut aren't used to snow. There's a long stretch of the main high way under big time construction, so it was a nightmare!!! I finally got off the high way and just took surface streets home, which took longer but at least I got away from crazy drivers and construction. We got home at 7:30pm. I was in such a crummy mood by the time we got there, I had to go hide in the bedroom for a while to stop from snapping at everyone.
I just hate being in this position where he doesn't fit any conventional treatment plan. All things considered, he's doing SO well, REALLY well, and I have to remind myself of that. But appts like this are a stark reminder of how "abnormal" he is in so many ways!!!! He sees his pulmonologist soon, and I expect that appt to be similar...we know what's wrong, but it isn't fixable, and it's all complicated because of his underlying syndrome. All we can do is keep chugging along. Same with gastro...we know he's not normal, but it really isn't fixable, so we just keep going. Heck, even his palate isn't quite a "normal" cleft!!! It took two years for even the palate specialists to find the darn cleft, and we haven't even started talking about how to go about fixing it!!
SIGH.
