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Zealous Advocate

From Mark:

I am accustomed to being a zealous advocate in my normal everyday life. That term takes on a whole new meaning when you are fighting for your sick child. The battles with our doctors continue, and I am proud to be my daughter’s zealous advocate.

We weighed the pros and cons, and we decided to get Getty a G-Tube and also a Nissen procedure. The G-Tube is an easy decision, as Getty will lose swallow function some day and the G-Tube will allow her to eat.

The Nissen is designed to prevent future reflux and potential reflux aspiration pneumonia. The procedure is a bit more controversial, but as Kate and I see it, the cost-benefit analysis leads us to the conclusion the Nissen is the way to go. Not getting the Nissen means there is a risk of future reflux aspiration pneumonia, and the risks of getting the Nissen don’t seem so bad in comparison.

The majority of SMA1 kids need a Nissen at some point and SMA1 kids lose strength with each passing day. So it is best to do these procedures now, as a preventative measure, while Getty has the strength to endure the surgery rather than reacting to some potentially horrible situation later, when she may lack the strength to undergo surgery. We scheduled the procedure for mid-November.

The issue is now how to properly do the procedures. An SMA1 child undergoing a surgical operation must have proper nutrition and must have proper ventilation support post-op.

Our gastro thinks a normal glucose IV should be enough. However, the SMA community tells me Getty needs a PPN feed instead. PPN is essentially an IV with additional nutrients in it, not just sugar water.

Our pulmo thinks it is good enough to have a BiPAP breathing machine on stand-by if it appears Getty needs it post-op. However, the SMA community tells me Kaiser needs to put Getty on BiPAP automatically post-op, whether it appears she needs it or not.

I have been studying these issues for a couple weeks, communicating with the SMA community, and communicating with my doctors. I have been trying to push our doctors into following protocols as provided to me by the SMA community.

We met our Kaiser surgeon this week, and he’s a jerk. We were asking him all kinds of questions about proper surgical protocol, and he was offended that we were challenging his opinions based upon information from the SMA community. We told him the information we are getting from the SMA community is based upon input from the handful of leading SMA physicians in the world. He wrote them off as “academics” who publish impractical papers to further their own careers. He told us he agrees with our gastro that there will be no PPN, and he agrees with pulmo that there will be no BiPAP involved. We were shocked to hear for the first time the idea that there will be no BiPAP involved at all.

The next day, I continued to do all I could to advocate for our girl. I got hold of  and spoke extensively with the office of Dr. Kathryn Swoboda, one of the top SMA doctors in the world. Her office assured me she was a practicing physician and not just an “academic.” She also told me when their office performs any surgical procedure on an SMA child, they use  a PPN feed and they put all SMA kids on BiPAP post-op. In other words, my doctors, who maybe see a handful of SMA cases in a decade, are not following guidelines established by the top SMA doctors in the world, who deal with SMA kids routinely.

I immediately put in calls to our gastro and pulmo to challenge their practices based upon input from Dr. Swoboda’s office. Thankfully, Dr. Swoboda’s office even said they were willing to talk to our Kaiser doctors directly to convince them to change their practices.

I then wrote a cold email to our surgeon to challenge his practices and to inform him he is mistaken to write off Dr. Swoboda and other top SMA doctors as merely “academic.” He wrote me a quick note back saying he simply agreed to do the surgery, and if I had a problem with how it’s recommended then I should take it up with gastro and pulmo. He will follow their instructions.

After leaving messages for gastro and pulmo, I called our pediatrician for help. I told her the problem, and she offered to help. She got a hold of our pulmo, and that led to a conversation between pulmo and me. Pulmo said there must have been be a miscommunication. He was not recommending no BiPAP at all; instead, he was recommending BiPAP as needed based upon post-op observations. This put my mind at ease, somewhat.

The SMA community has been advising me to get Getty on BiPAP pre-op as well as post-op because when you put a kid on BiPAP for the first time, they struggle with the machine and cry a lot. They just are not used to a machine blowing air into their little face. So, if you try to start BiPAP for the first time post-op, then the patient will waste a lot of energy struggling and crying when they should be devoting all their energy to recovery. Pulmo said there is pretty much no way to start BiPAP pre-op, as I had asked.

I was unable to get a hold of gastro to talk about the PPN feed.

The next day, I spoke to another SMA father*. He told me post-op BiPAP should be mandatory and not discretionary based upon post-op observations. An SMA child inherently needs breathing support post-op so they can recover from surgery without having to struggle to breathe. Even if she looks like she’s breathing fine post-op without BiPAP, she is wasting her energy trying to breathe when she should be using her energy trying to recover.

He also confirmed the need for pre-op BiPAP acclimation, for the reasons stated above. He also said we need to get Getty used to a BiPAP machine at home right now so she will be accustomed to it by the time the surgery is done.

This SMA father also told me we need to do a PPN feed post-op because using straight glucose IV does not give SMA kids enough fuel to properly recover.

When his child went to see Dr. Mary Schroth, one of the top SMA doctors in the world, they walked out of her office with a prescription for an at-home BiPAP machine right away. He said an SMA1 diagnosis alone inherently justifies an at-home BiPAP machine prescription.

The bottom line is … our doctors are doing it wrong and they need to be corrected.

What we want is (1) pre-op home BiPAP acclimation; (2) automatic mandatory post-op BiPAP; and (3) a post-op PPN feed. To make matters worse, it is my understanding that there is no harm in doing it this way. Our doctors just don’t see the need and are not doing it based upon our request alone.

This SMA father also told me he would try to get Dr. Schroth involved in our case. I truly hope Dr. Schroth is willing to help set our Kaiser doctors straight with a couple of conference calls. I am honored that this SMA father is helping us and is willing to try to get one of the leading SMA physicians in the world involved on our behalf.

Not only do we have to fight SMA while working full time, we have to fight our doctors. It’s so sad and exhausting. I have spent so much time and energy on these battles, advocating for our Little Owl. If we didn’t have the SMA community in our corner, we would just quietly accept whatever our doctors told us. I feel sorry for SMA parents for having to grapple with SMA in general, but I especially feel sorry for those SMA parents who never get connected to the SMA community. I can only image all the bad medical advice they follow unknowingly.

*Name withheld for privacy.