Our SARPE Saga - The (Dead End) Road to Surgery

About a year ago I broke down after trying to get Amanda seen by the Craniofacial Team at Children's Healthcare of Atlanta (CHOA). I sobbed on the phone,"Please just look at my child."

 

After her lung collapsed in 2011 her pulmonologist suggested that she be seen by the craniofacial team at CHOA. I was beginning to feel a desperate need to at least have her looked at. I'd been trying to get Amanda in to see the team of specialists for most of her life. However we were always turned down - not by our insurance - but by the practice. It seemed that she somehow didn't meet their threshold for care, even though they had never seen her. So for 14 years we had struggled with breathing issues and feeding issues on our own.

 

Kevin realized how important this was to me and stepped in. He has an amazing ability to logically persuade people to do what they should do. He was able to determine what I call "the magic words" to get Amanda admitted for an assessment.

 

Once we were in we were seen by three specialists: a plastic surgeon, an orthodontist and a dentist. It was an oddly cryptic appointment. They all looked at her. They took photos of her face from several angles. They left us alone in the room while the conferred.

 

The plastic surgeon came back into the room and announced an extensive surgical plan for correcting Amanda's mouth and nose. Honestly, I was surprised. I thought they would just give her some sort of braces - especially after the orthodontist looked at her.

 



She has a very high, arched palate. I can't fit my pinkie into the roof of her mouth. It obstructs her breathing. It prevents her from being able to retract her tongue into her mouth, which creates another set of issues. For instance she is on a semi soft diet because she never developed rotary chewing or the ability to swallow completely.

I didn't realize the extent of what was planned until I read the follow up report from the team. I was surprised to read the words "complex set of issues."

First Amanda was scheduled for a surgically assisted rapid palate expansion and an uvulopalatopharyngoplasty (no wonder they shorten that to "UPPP")

A plastic surgeon was going to cut the roof of her mouth and an orthodontist would insert a palate expander opened to its maximum width. At the same time the surgeon would remove her tonsils and some of the back of her palate that is blocking her airway.

While this sounds horrific I could see the need for it. So we began our journey to craniofacial surgery.

It seemed fairly straightforward in April, 2012. Kevin tried to take Amanda for a sleep study to document her sleep apnea, but she kept pulling the electrodes off in her sleep. After a few hours they were sent home.

Next I took Amanda to the craniofacial office and they took an imprint of her mouth to build the palate expander. To do this they made up some pink plastic material and stuffed it into her mouth. She gagged horribly, but they got it done.

This is a palate expander

All we had to do then was wait for our scheduled surgery date in late May.  However, the night before our surgery we received a call that the surgery had been denied by Kaiser HMO.  Kaiser required a CT scan before approving the procedure.

Of course, Amanda won't hold still for a CT scan so it had to be scheduled under general anesthesia. Luckily, we got an appointment within a week. So we got up at 5 AM and headed to the hospital. After 5 hours at the hospital the anesthesiologist decided that she couldn't take the risk of putting Amanda under for the CT scan because there was an order for an MRI from the previous fall that had never been completed.

The irony is that the MRI was supposed to be performed under general anesthesia so the risks were the same, regardless of which test came first. Still, the doctor refused and sent us home. 

Then we had to go back to the neurologist who ordered the original MRI, get a new MRI ordered, approved and scheduled. Luckily the CT order was still good and I was able to schedule both tests at the same time. Amanda only needed to go under GA once that way. The first available date was at the end of August 2012.

The tests went well, but Amanda came back to the recovery room cyanotic.* Her lips, nails and the area around her eyes were all bluish gray due to lack of oxygen. She remained on oxygen for several hours until her pulse oxygen level returned to normal.

The surgeon was confident that the procedure would be approved. Amanda had to go through the awful molding process to create a new palate expander since the first one was either missing or they thought it wouldn't fit. (I'm not sure which was the actual reason.) This time Amanda knew what was about to happen and wanted no part of it.

The first time they tried to ram the molding material into her mouth she bit the technician.  The tech brought in back up to hold Amanda's mouth open and the two of them tried again. It was horrible to watch Amanda try to writhe away from them, choking and gagging. Finally they got it done.

Then, we waited for the approval from Kaiser HMO. We waited nearly 2 months for a decision.

Surprisingly, in late October 2012 the surgery was denied a second time. Kaiser HMO deemed it "cosmetic." Personally, I believe this was based solely on the fact that the doctor scheduled to perform the surgery is a board certified plastic surgeon and Kaiser unilaterally denies coverage for plastic surgery.

Now were are waiting the results of our second appeal. I've sent Kaiser the photos above as well as a video demonstrating her obstructed breathing. I have no idea if it will be denied again - or what we will do if it is.  
Note: the day I wrote this I got the final denial from Kaiser HMO in the mail. I'm not sure what we'll do now....I suppose that we will continue to watch our child struggle to breathe and eat  for the rest of her life.

*this link connects to a 38 second video of Amanda post-MRI - you can hear her obstructed breathing

For more on the proposed surgeries please read my earlier post, My Informal Medical Training.