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Fortress Around Your Lungs

From Mark:

The defense of Getty’s lungs is coming along nicely. One of the biggest threats to an SMA baby is a lung problem, such as pneumonia. One of the biggest risks of pneumonia comes from aspiration of something. These kids can aspirate their own secretions, like saliva and mucous. They can aspirate their food, either on the way down the hatch or on the way back up the hatch via vomiting or reflux. They can aspirate anything that goes down the throat. Common colds and flu are especially problematic due to the increased secretions and increased likelihood of an aspiration event.

The lungs are at risk mainly due to a few of factors:

  1. Lack of swallow function;
  2. Lack of effective cough function; and
  3. Increased risk of reflux

SMA robs these babies of necessary proteins to promote adequate muscle tone, and the problems just get worse every day as their strength diminishes. The lack of tone affects the swallow muscles, robbing them of their ability to send food down the esophagus instead of into the lungs. The lack of tone makes the cough function ineffective in that it is not strong enough to rattle around the secretions and bring them up to the throat for proper disposal, usually via swallowing. These kids also often have a weakened esophageal sphincter, which keeps swallowed food in the stomach, preventing reflux.

Much of what we are doing boils down to protecting the lungs. Even though Getty had been swallowing properly, we scheduled a G-tube placement surgery to allow feeding in a manner that bypasses the swallow function when that time comes. Over the past few weeks, however, Getty has been coughing, choking, and gurgling more and more during bottle feeds. It got to the point where we decided to get a nasogastric tube (NG-Tube) placed.

The NG-Tube is very narrow. It has a rubber plug at one end. The other end goes into her nose and down into her stomach. The whole thing is held in place by taping it to her cheek. The capped end of the tube just dangles freely, but we can tuck into her onesie. The initial placement of the NG-Tube is obviously uncomfortable, having a tube jammed up your nose and all, but once in place, Getty doesn’t notice it. Once the G-Tube is placed, the NG-Tube will be removed.

G-Tube placement alters the stomach and increases the risk of reflux. The upcoming Nissen fundoplication surgical procedure is also a lung protector. They wrap the upper part of the stomach around the base of the esophagus and tie it there. That allows food to pass through, but helps prevent reflux and the accompanying risk of reflux aspiration pneumonia. Although Getty is not currently presenting reflux, we think the Nissen is a good lung protection measure to take. Preventative Nissens in SMA cases are controversial, and doctors are split as to whether to do them or not. They are not without risk.

Even though we currently have the NG-Tube, we continue to bottle feed Getty in an effort to keep her mouth and throat as strong as possible. If they are strong, then she will be better able to manage her own secretions. If she coughs through a feed, gets formula gurgling in her throat, or if she just gets too tired to finish a bottle on her own, we feed to the tube. We do this via bolus feed. This means we attach a big syringe to the end of the NG-Tube, pour the remaining formula in, and let it gravity feed through the tube to her stomach. If we know she’s really tired or if we are out in public, we can skip the oral feed and feed straight to the tube. Then, we just flush the tube with a dash of water in a smaller syringe.

We found out today Getty needs more calories than what she is getting. So now we have another piece of equipment to add to our inventory: a feeding pump. We fill a plastic bag with formula, which then gets pumped into her tummy through the NG-Tube at about 1 ounce per hour while she sleeps.

On top of all that, we are using a suction machine to suction her secretions from her nose and mouth. We are using the cough assist machine a couple of times per day to generate a simulated cough to get any gunk out of her airways. She is really quite cooperative with all the machines. As soon as I turn on the suction machine, she opens her mouth and waits for me to begin.

All of this is an exhausting and time-consuming pain. The bigger pain is the emotional pain watching your little girl degenerate and lose strength and function before your very eyes. When I see a child on TV or in public, I am amazed that they can do something as simple as hold their own heads up and move their legs. On the flip side, I know our little girl has needs, and I am happy that we have been able to meet them. I get pleasure knowing we are providing her the fortress she needs, and I melt when I see her little smile.

Kaiser has been a big part of her success, and I thank our Kaiser team. We went a few rounds with Kaiser trying to get a BiPAP breathing machine in connection with the upcoming surgery and also trying to get a PPN feed instead of a normal glucose feed to get Getty through the surgery. With the help of some of our loving SMA families, we were able to get renowned SMA expert, Dr. Schroth of the University of Wisconsin, to personally speak to our Kaiser team by phone. As a result of those conversations, we now have the pre-op and post-op BiPAP plan in place.

On the other hand, our Kaiser gastroenterologist was not sold on the PPN feed even after speaking directly to Dr. Schroth. Dr. Schroth routinely recommends PPN feeds for her SMA surgical patients. However, through these conversations, Dr. Schroth told us that, although she recommends PPN feeds for her patients, there is no scientific evidence to support PPN use. Kate and I have come to accept our gastroenterologist’s conclusion that a PPN feed is not necessary.

Although it is emotionally and physically draining, and I spend the bulk of my days lately on medical issues instead of work, I am confident that Getty’s current medical needs are being met.

Again, we thank Dr. Schroth, the SMA community, our personal SMA friends, and our other friends and family for all your love, kindness, generosity, and support.

In other news, our Kaiser team has plugged us into a Neuromuscular Clinic lead by a neuromuscular specialist at UC Davis who is apparently quite knowledgeable about SMA and is fairly well-connected to other renowned SMA experts such as Dr. Swoboda of the University of Utah, Dr. Wang of Stanford, and others. We look forward to learning more about the Neuromuscular Clinic.

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  • Brittany says:

    Hello Mark, I was one of Kate’s students. I just wanted to share some with you on the G-tube experience. My niece, technically second cousin, Ashlynn has had a G-tube from day43 about. She is a miracle. The doctors told the family to take her home so we can enjoy her and let her expire. She will be two November 8. We were told she is blind, deaf and will have no muscle movement or coordination. She is conversating, crawling and beginning to walk. The G-tube has allowed her to stay healthy but with great patience and caution she is not able to eat solid foods regularly. She loves to feed herself and mingle with the family during meals. Her G-tube had been tugged, pulled out, slobbered all over, but she enjoys her tube just like a stufty. It was heart wrenching at first to see the site her stomach right after it was in place, but it was more heart wrenching to watch her be devastated every time someone would eat in front of her. The G-tube was and is remarkable for her in growth and understanding. She understands that she is ill, but nothing will stop her, not even her little big brother.

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