Eleven Hours

That is total amount of time we spent @ CHLA this past week, not including any travel time.

Settle in with a cup of coffee this is going to be long!!!

If Monday was the quickest appointment ever, then let's call Tuesday loooooong. We had a 5 hour ordeal with back-to-back ENT & Pulmonology appointments. Let's just say it was a good thing someone called to remind me of the times because I had them switched in my calendar and that could have been VERY messy! I am going to purposely be vague on some of the details because there is still so much to work out between the two of them (insert rolling eyes).

Here is the Tuesday review:

ENT: Our appointment was for 9:15. We saw Dr.ENT1 @ 10:40!! Now I understand there are emergency situations which sometimes cause the schedule to go haywire BUT that was not the case today. He was late because it was "picture day". Yeah that's right, there was a photographer there taking pictures of the ENT staff. HELLO!!!! People are waiting, people are double booked for appointments, Austin needs a nap, Nurse M & I need some food before the next looooooooong wait. Have some freakin' consideration!!! Prior to him gracing us with his presence I notified the Trach Nurse that Austin had had a sleep study done in April and I would like to review the results. Finally Dr. ENT1, who apparently is very photogenic, came to see us. So let's just say I was not as happy to see him as I otherwise would have been. To top it all off we did not seem to agree (to put it mildly) on surgical outcomes for Austin's Trach removal and/or ear reconstruction. (Ps: I just got an email today from Dr. ENT1 thanking me for the research I had done on this subject. He will keep it in consideration for other patients. -- insert gigantic I TOLD YOU SO!! smiley) We go back in 6 months to see him again.

The Bottom Line: Austin's sleep study was not good. It indicated that he needs 3L of O2 to sleep at night. Personally since I put him to sleep every night with 2L I know this is bunk! But there it is in B&W, he needs more O2. Dr. ENT1 will NOT consider removing his Trach with O2 requirements so high. He might consider it if the O2 requirement was less and then Austin could just get O2 with a nasal cannula, but that is a big MAYBE! This is VERY bad news because all signs point to Chronic Lung Disease. These are three little words that no one wants to hear, especially not the guy who is in charge of determining if & when Austin's Trach goes.

Pulmonary Care Center: This appointment consists of seeing four people in one visit - The Pulmonologist, The Nurse Practitioner, The Dietitian and The Social Worker. All things considered this appointment actually went fairly smoothly especially in comparison to the ENT visit, that is not usually the case. Austin weighed in @ 24lbs. This means he had a weight loss but is perfectly proportioned @ 25%ile for both height & weight. I put on my boxing gloves and convinced Dr.Pulmo1 that Austin needs prophylactic TOBI nebs to help him keep his lungs clear of funky stuff. We had this discussion last October before flu season started. Since that visit Austin has had pneumonia diagnosed 3 times and misdiagnosed once because I did not push for it. I will never make that mistake again! So we compromised and he will begin 3 months of TOBI nebs the very next time he gets a "cold" or anything that could remotely turn into pneumonia. Our hope is that he will stay clear all summer, but regardless he will get them next time he is sick without a fight. The dietitian added a few more calories to his feedings to compensate for his new active lifestyle. The Nurse Practitioner came in and scared the beejeezus out of us about the Swine flu coming back in the fall. Niice! The Social Worker just wanted to make sure all was well at home. It is. We go back in 3 months to see them all again.

On Thursday we had a 6-hour Craniofacial Team appointment. Basically this consists of us traveling from department to department within the hospital to visit 8 different specialists. This appointment typically happens once a year.

Here is the abridged version (believe it or not!) of Thursday's appointment:



Dentistry: Austin had his first dental visit. This consisted of him sitting on my lap facing me with his head on a pillow on Dr.Dentist's lap. With all the screaming & crying she was able to get a great view of his teeth! Once she gave him a Tigger toothbrush all was well with the world.

Hearing: Started off tense as I had to convince the snooty busy Audiologist to give him a hearing test with his BAHA. This was supposed to be a regularly scheduled test, but she acted like she couldn't be bothered...guess who won? I will go into more details about his BAHA, but it went very well. (Thanks to John Tracy Clinic, Austin is the King of Behavioral/Play Audiometry Hearing Tests!)

Speech: This was probably the some of the BEST news of the day, mostly because it was unexpected (and a little too easy.) I was able to get the Speech Language Pathologists to agree to TWO new therapies that I have been harrassing asking Regional Center for. Woohoo!! Austin will attend Speech therapy once a week @ CHLA to work with his Passy-Muir Valve tolerance. And they have also agreed to look into getting him VitalStim therapy for his feeding issues. If he can overcome these hurdles he could be talking & eating like an average 2 year old soon. No guarantees, but it is a HUGE step in the right direction to get these therapies going soon. Keep your fingers crossed!!

ENT: This appointment piggy backed on the previous appointment on Tuesday, but with Dr.ENT2 (she was essentially singing the same tune and frankly I was getting tired of the song!) But I did my best however to insist convince Dr.ENT2 that Austin would benefit from another sleep study with capping.

According to Austin's Nurse J, the conversation sounded something like this:

Me: This is what I want...

Dr: Well, I am not sure...

Me: This is what I want...

Dr: Um, no I don't think we can...

Me: This is what I want...

Dr: I am not sure I want to get involved...

Me: You already are and by the way, This is what I want...

So in the end the squeaky wheel (me) got her to sign off on the PMV therapy and a follow up sleep study with capping & nasal cannula. (insert GIANT grin!)

Stay with me here we are half done!

Plastics: Dr.Craniofacial is no longer recommending Jaw Distraction surgery. This is good & bad. Good because the surgery scares me quite frankly. Bad because it means the Trach stays in longer. There are lots of other factors which contribute to this new revelation, but those are all wrapped up the craziness of Tuesday's ENT & Pulmo appointments so I will leave it at that for now. From a Craniofacial (Plastic Surgeon) perspective the ONLY surgery he is recommending for Austin is ear reconstruction when he is ~4 years old. It has yet to be determined if Austin is a candidate for ear canal surgery at the same time. I dazzled him with my research and he agreed that IF Austin was a candidate going to Palo Alto would be worth a shot! This is BIG maybe, but MIGHT make it possible for Austin to use traditional behind the ear hearing aids instead of the BAHA. Once again fingers crossed!!

Pulmonology: This meeting also piggy backed our Tuesday appt. Dr.Pulmo2 agrees we can do another sleep study with capping and promises only a 3 month wait instead of a 9 month wait like last time. She believes that Austin was getting sick during the last sleep study and that contributed to his crappy O2 needs. (I'll buy that!) She was impressed with Austin's use of the PMV and happy with his O2 saturation and vocalizations. She likes that Austin is able to communicate with some verbal words and so many signs. Oddly, she was further impressed that we have learned sign language right along with him. My response was 'of course, otherwise how would we know what he wanted?' She said, you would be surprised at how many people don't learn along with their hearing impaired kids. She gave me double brownie points for learning. Since she is going to be the final word on any and all surgeries Austin has I am glad to have her on my side. Her main focus is on children who have Craniofacial issues with lung issues. I was very impressed with her.

Genetics: This meeting with Dr.Genetics & his staff almost didn't happen. But once I got the receptionist on the right track we saw them. Since we already know Austin's Dx of Branchio-Oto-Renal syndrome this evaluation was just to see if there might be any other concerns. Dr.Genetics mentioned something that will be followed up on in the near future, otherwise he will just consult on Austin's case as needed.

Finally, one more visit with the Social worker just to tie up loose ends. Austin left with another coloring book and two crayons. Poor kid fell asleep in the car on the way home. Officially we will have the written recommendations of the Team in about 3 weeks, but after speaking on the phone today with our Case Manager they believe Austin is already seeing everyone he needs to be seeing. (Ya think?)

PS: Since I am updating I figure I might as well include his last two appointments with GI & Neurosurgery in late May. Our appointment with Dr.GI was quick & painless. She ordered the Modified Barium Swallow Study (Thank you, finally!!) so we can try assess his swallowing capabilities and trial more foods, as approved. We also discussed trialing Austin off his Reflux meds, Periactin & Prevacid. For now his Rx for Periactin is now gone, but the Prevacid trial did not go well so we will continue until further notice. One down, one to go. We go back in 6 months for a follow up.

Our appointment with Dr. Neurosugery was the same day and pretty quick & painless as well. He reviewed the MRI and there have been no changes since our last visit 4 months ago. He will see Austin again in 6 months to assess his head growth & closure of his fontanel. We are hoping to be off his patient list in November!

Okay that's is about it. Sorry if your head hurts now.

Thanks for peeking,