Getty Owl Foundation

Why we fight?

From Kate:

This is why we fight. Getty is the most amazing gift that Mark and I could have ever asked for. She is our world. She is always happy and content. As she gets older we see glimpses of her special personality. She is silly and curious and she is just well, she is just Getty!

Good night sweet owl. Your mommy and daddy can’t wait to snuggle next to you.

Getty Couldn’t Ask for a Better Mother

From Mark:

Thanks to our craptastic medical experience, we have not been able to go on any evening walks as a family in weeks. Since we got the diagnosis, those walks have been a big part of what holds us together. It has been a time to get out, relax, and just enjoy our family. When you have a child with a terminal disorder, every walk in the park really counts.

I know our walks together are particularly important to Kate. We were both floored by the SMA diagnosis, and yet Kate was a real trooper, immediately launching into the fight to do whatever she has to do for her little girl.

In order to do what was best for Getty, Kate wanted to stay home and provide for her daughter. However, Kate did not blindly decide to just stay home, she did extensive research into work and other benefits we may be able to use.

Unfortunately, the reality Kate found was that the best option for the family was for Kate to go back to work full time to maintain full income and health care benefits for Getty and to keep a roof over her head. It is a shame that the best option for a family with an infant with a terminal degenerative condition who is susceptible to illness is to send the child’s mother off to work full time in a school, which is a germ cesspool.

Despite the seeming injustice of it all, Kate gets up every day, takes care of Getty, gets ready for work, and heads out the door, voluntarily leaving her Little Owl behind. I cannot imagine the knot in her stomach every day she leaves the house.

When Kate is at work, I know she gives her students her all. She is a wonderful teacher and I know she would never compromise her own teaching standards due to a personal setback.

After a hard day, she is always quick to come home to be with Getty. Similar to her teaching style, she takes great care to provide Getty only her best.

When I wrap up my work day, and we have some time as a family, I see Kate feeding, bathing, changing, and loving little Getty. Kate reads her stories, sings her songs, and tries to teach her to say, “who, who, who.”

It is a beautiful sight to witness the bond between them. Getty may have SMA, but she may never know it with all the love, care, and attention showered upon her.

When we got the diagnosis, Kate and I quickly came to the conclusion that, for as long as we may have her, Getty deserves only the best; and when it comes to motherhood, Getty certainly has it.

We face a hard reality so far from what we imagined, but you handle it with grace and strength. Thank you, Kate, for all your love, work, dedication. I look forward to many more walks with you.

The McDonald’s of Health Care

From Mark:

Instead of putting in a normal workday, I spent a good chunk of yet another day in a Kaiser fight.

As I anticipated, Kaiser denied our request for expedited review to go see Dr. Wang, an SMA expert at Stanford.

I also had a lengthy phone consult with our gastro. She was willing to walk me through her rationale for recommending against a PPN feed. Essentially, her reasons were as follows:

1. Some major children’s hospitals use normal IV as standard procedure and not PPN;
2. Based upon what these hospitals do, our gastro would need to see strong evidence to go against what she sees as the standard of care;
3. Dr. Swoboda’s nutritional guidelines calling for PPN are only guidelines and are not a formal study sufficient to convince gastro to recommend PPN.

I asked, “Why NOT recommend PPN? What’s the risk?”

She responded with the following two reasons:

1. PPN creates a risk of severely burning flesh where the probability of that is not remote; and
2. PPN can cause an electrolyte imbalance, creating a need for frequent monitoring and blood draws to ensure constant proper blood chemistry.

I immediately emailed the gastro’s full reasoning to Dr. Swoboda’s office. Shortly thereafter, I got input from Dr. Swoboda herself. She wrote that for the past several years, she has routinely used PPN which contains no more than 10% dextrose and, thus, will not cause burning flesh. Increased survival using her proactive protocols has been statistically significant compared to other groups. She’s in the process of publishing on it, but until her work is in print, it will be difficult to consider it “evidence” unfortunately.

In sum, what I’m gathering from our gastro is that she recommends against PPN simply because other people are doing it that way. Looking again at the arguments against PPN we see…

1. PPN creates a risk of burning flesh. Well, Dr. Swoboda shot this argument down with the 10% dextrose PPN; and
2. PPN can cause need for frequent monitoring and blood draws to ensure constant proper blood chemistry. In my mind, this argument reminds me of when I was a kid doing math homework, whining, “I don’t waaaannnnnnaaaaaa coz it’s toooooooo haaaaaarrrrrrd!”

I forwarded Dr. Swoboda’s rebuttal to our gastro. I also wrote another email to Dr. Schroth at University of Wisconsin’s American Family Children’s Hospital, asking for help.

I spoke to one of the SMA dads, who is well-versed in all of this stuff. He was very apologetic that we had to go through all this and very confident in his belief that our gastro was not giving us the best possible care. He is certain that if we could just get help from Dr. Schroth, we would be so much better off. He told our story to Dr. Schroth recently, and she said she would be willing to help us. That tells me she disagrees with our Kaiser team.

All I know is that the entire SMA community praises Dr. Schroth to no end. Her knowledge and expertise with SMA is considered top notch. Her word on SMA is golden.

This SMA dad also told us that SMA kids who don’t go directly on BiPAP post-op tend to expend all their energy trying to breathe when they should be simply trying to recover post-op. As a result, these kids may look just fine when they are recovering in the hospital, but once they get home, they have a serious crash and may never regain their previous strength.

Kate spoke to a local SMA mom today who told us her child did the G-Tube/Nissen procedure, and her pulmo did not put her child on BiPAP post-op. The child went home and crashed soon after. She never regained her prior strength and passed away not too much longer after that. She believes the crash was caused by the failure to go on BiPAP post-op. Who was her pulmo? The same one we have.

Kate also mentioned to this SMA mom that we met our pediatric surgeon who is scheduled to do Getty’s G-tube/Nissen. When we were in his office, we asked him a lot of questions about the procedure and he was irritated that we were challenging his ideas. We asked him how many SMA G-Tube/Nissen procedures he has done. He told us he had done a handful in the past decade, and he told us about the last one he did. It turns out the last one he said he did was the case of the SMA mom Kate was talking to today. That SMA mom told us that our surgeon  was NOT the surgeon doing the procedure on her child and that their surgeon was the OTHER Kaiser surgeon. In other words, our surgeon was apparently taking credit for some other surgeon’s work. We wonder how many other SMA G-Tubes he claims to have done were really his or if he just happened to be in the same room as whoever was actually doing the surgery.

Kate and I are getting the feeling Kaiser is the McDonald’s of health care. If you want a Bic Mac and fries, Kaiser will fill you up just fine. If you need extra pickles, you run the risk they’ll screw up your entire order. If you need special service and a quality product, go somewhere else.

I’ve never been to Wisconsin. Maybe it’s nice this time of year.

Battle & Soup du jour

From Mark:

Kaiser denied or request for “expedited review” in which we asked Kaiser to pay for us to go see one of the top SMA experts in the world at Stanford. Kaiser thinks it has all the expertise it needs in-house, despite the fact that the leading SMA experts in the world, who see SMA kids all the time, recommend one thing while Kaiser, who sees maybe a handful of SMA kids in a decade, recommends something else.

Today, I appealed the denial and argued our case before the Kaiser expedited review panel. I presented the details of our case, but my argument essentially boiled down to the notion that we need to see an outside expert at Kaiser’s expense because Kaiser lacks SMA expertise in-house. My hopes of successful appeal are low because if Kaiser grants the appeal, then in doing so, Kaiser is tacitly admitting its physicians lack sufficient expertise in handling SMA cases. I can see where Kaiser might find making such an admission potentially problematic.

I gave it a good shot. We’ll get the decision in a few days.

I also arranged a phone consult with gastro for tomorrow to review the post-op PPN feed issue I discussed in previous posts. I tried to get Dr. Swoboda’s office to join in the call to give me some ammunition to fire at our Kaiser gastro in our quest to get the PPN feed. Unfortunately, Abby at Dr. Swoboda’s office isn’t available, so I’m on my own to find out why our gastro disagrees with one of the top SMA doctors in the world. If our gastro still disagrees with PPN, I will be taking diligent notes and hopefully running our gastro’s reasoning through Dr. Swoboda’s or Dr. Schroth’s office later.

In happier news, we really enjoyed having a wonderful dinner at Stacey Ball’s home. She made a wonderful soup. It was more of a stew than a soup, but I liked the title of this post.

Despite being a new mom, Stacey has volunteered her time and energy to put the entire GETty Crafty Craft Fair on pretty much all by herself. We cannot thank you enough, Stacey, and everyone who has contributed in any way to our Little Owl’s cause!

THANK YOU!

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.