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.
 

"Oh, no! My Child Has to Have Surgery!"

I've been thinking a lot about surgery lately. So much that I actually thought I'd written about this already....

Years ago I was waiting while Amanda had leg surgery. We were sitting in the Day Surgery Lounge of Children's Healthcare. Near us was another family, the mother sobbing hysterically in her husband's arms. I wondered what their child could possibly be going through to have her is such a state. After about an hour she collected herself and sat quietly waiting.

When the nurse brought them back to see their child I overheard her husband say, "There, I told you a tonsillectomy was nothing to be scared of." It was all I could do not to laugh! All that drama for tonsils? Seriously?

Then I remembered Amanda's first surgery. The first surgery is terrifying for parents, whether it is open heart surgery or a tonsillectomy.

In February of 2006 when Amanda was 6 years old she had strabismus surgery.  I was scared and frustrated. We'd had her wear a patch for ages in order to avoid surgery and now we still had to go through with it.

However, it was obvious to us that she had little or no depth perception. When doing a board puzzle she would put the correct piece near the space and the randomly slide it around until it clicked into place. We had her using sippy cups because she would try to put her cup on the table and miss it by an inch, the cup crashing to the floor. We could see it when she was walking with us. If she got to a change in surface, even a different color tile, she would pause and test it with one foot to see if she could contiue or needed to step up or down. We hated the idea of surgery, but we could see how it would improve her life if it worked.

See how her left eye is drifting?

In retrospect it was a relatively minor procedure, but at the time it was very scary. I remember waking up at 5AM to get her ready. I was worried that she would be hungry, but she was so sleepy that it wasn't an issue. I bundled her up in her favorite fuzzy blanket, put her in the car in her pajamas and drove the hour to the surgical suite.

Now I know that surgery that doesn't require hospital services is generally considered safe surgery. If the doctors can perform the operation in an outpatient surgical suite they have all of the necessary safety equipment and an anesthesiologist present, but don't expect any complications or an overnight stay.  But I didn't know that then.

We checked in to the office at 6 AM. After waiting a few minutes we were brought back to a pre-op area. We waited in our own space with 3 walls and a curtain for privacy. There was a gurney for Amanda to lie on and a chairs for us. They let Amanda stay in her own pajamas. They gave her a little "loopy juice" (versed) to make her sleepy and help her forget the procedure, then they covered her with a warm blanket and wheeled her through the curtain.

I stayed with her as long as they would let me, holding her hand all the way to the doors of the surgical suite. When the doors closed behind her I realized that tears were streaming down my face. Amanda had only been away from me while she was at school. I felt so helpless with her out of my sight, out of my control.

My husband was surprised that I was crying. He knew logically that it was a short, safe procedure. All I knew was that my baby was being hurt. In that moment it didn't matter that it was the right thing to do.

It really was a quick surgery. It think the actual procedure lasted about 30 minutes. After that she was in recovery for another half hour or so. We left the surgeon's office by 10AM.

At first Amanda's eyes were blood red. It was kind of creepy, but the blood cleared in a few days. Best of all her eyes really weren't drifting anymore. We could see her really looking at things - both eyes focused together. It was surprisingly cool. It made me wonder what the world had looked like to her before the surgery.

Amanda still wears glasses. She has progressive myopia that had nothing to do with the amblyopia and strabismus. Happily, her depth perception is much better thanks to that surgery.

No parent wants to put their child though a surgery. However we've learned that if there is need for surgery, there is no point in waiting. I don't think doctors lightly suggest surgery. I'm fairly confident based on our experiences that they will always try other, non-invasive methods first. Before eye surgery Amanda's doctors tried glasses and patching. Before back surgery her doctors had her go to physical therapy and wear a brace. Only in an emergency will most doctors suggest immediate surgery. When Amanda broke her hip and the doctors said it needed to be surgically pinned as soon as possible we didn't hesitate. She had surgery within hours of arriving at the Emergency Room.

If you are facing non-emergency surgery for your child,  you have the opporunity to alleviate some of your fears beforehand. Sit down with your child’s doctors and ask the questions you need answered to make an informed decision. Ask about the risks of surgery, the surgery itself, how the doctor thinks the surgery will help and what to expect to after the surgery. Ask for an email address to contact them if you have additional questions. I often find myself with questions after I've left the office. If you are still unsure, get a second opinion from another medical professional. It can only help in your decision-making process.

However, if your child needs surgery don't be surprised if the doctors tell you the worst case scenario. For liability reasons they have to warn you about the risk of blood transfusions, comas, even death. THAT DOESN'T MEAN THAT WILL HAPPEN! It just means that there is a risk to any procedure. It may mean that one or more of those things has happened to a patient somewhere, at some time during this specific procedure. In this age of litigation doctors have to warn you of any and all possible risks or they may be sued for malpractice.

And, speaking from experience, if something does go wrong you are in the best place to handle an emergency. Amanda has had a blood transfusion (so have I) without any complications even though it was unplanned. Following hip surgery her lung collapsed. She was treated immediately and although it resulted in an extended hospital stay she has had no long term complications.

I've found that other parents are often the best resources. They will tell you truthfully about their experiences, good and bad.We are lucky to have a resource in the U.S. called Parent to Parent. They have a nationwide network. They will try to connect you with another parent whose child has undergone the same procedure.  We were very lucky to have a family in Atlanta to talk to before Amanda had her spinal fusion. In fact, the mom was so kind that she showed up at the hospital with magazines and snacks.  She even moved a more comfortable chair into the room for me. Talk about supportive!

Now we have been through nine surgeries with several more on the horizon. I say "we" because although Amanda has the surgery it impacts our entire family. My advice is simple. Do it if it is medically necessary to improve your child's health or quality of life. Don't do it casually. Research the proposed procedure online. Talk to the surgeon and other parents. Find out what the recovery will be like and what the potential downsides may be. If you are concerned about blood transfusions donate blood in advance of your child's surgery to be used in an emergency. Do your best to prepare yourself and your child,  then be confident in your decision knowing that you have taken a careful approach. But expect to be scared - after all, you're a parent. Its part of your job to worry about your child.