Getty Owl Foundation

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.

New Upcoming Event Added

We have added a Pampered Chef Party as a new upcoming event!

Click here to check it out!

Thanks!

Getty’s 1st Halloween

From Kate:

I think about this time last year I was just starting to show my pregnancy. I remember Halloween last year and thinking about how fun it was going to be to have little Getty with us. I remember walking the aisles in Target to see what kind of selection was out there for our new addition. I settled on a ladybug costume that had a big puffy body and a equally puffy hat. It was going to be perfect. So should I buy it now or maybe wait until she is born? I decided to wait, and I am so glad I did because Getty isn’t a ladybug, she is an owl.

When Getty was born she instantly looked wise like an owl. Her old soul allowed her not to stress much about anything. Still to this day if Getty does get fussy, which is rare, she has a clear reason for it and we can usually figure it out pretty easily.  Her deep blue eyes are always focused on the nearest face and she always seems to be looking into your soul.

Her cheeks contribute to her profile of an owl. Her soft puffy cheeks are so kissable and become pink when she smiles. Her round belly solidifies the persona of an owl. Now if we can just get her to “hoot, hoot” we should be good. 🙂

We got a chance to meet a terrific couple about a month ago, Robert and Meri Phelps. We had been corresponding with them for months, but only just lately got a chance to meet personally. They welcomed us to their home and we got to meet their sweet little son, Aaron. We felt so comforted to be able to ask lots of questions about SMA and to get a chance to gauge where our journey is possibly going. After lots of conversation, Meri brought out a costume that she thought would be very appropriate for Miss Getty. It was an owl costume that Aaron wore the year before. It was simply perfect. What are odds I thought to myself? I didn’t question it and took it home for Getty to wear.

So the big day came yesterday. I have been waiting to share this with Getty for an entire year. I dressed her in the costume and Mark and I took some everlasting pictures of our little girl.

My thinking, at least right now, is that Getty might have to be an owl every year. It just doesn’t seem right for her to wear anything else. Well we have a year to think about it at least. I think next Halloween, I will let her choose. 🙂

I hope everyone had a wonderful Halloween. We had a great time handing out candy and checking out all of the Spider-men, ghosts, and pirates.

It was wonderful to spend time as a family after our great trip to Santa Rosa. Getty was very tired but in good spirits. She was a real trooper allowing us to dress her up and to take lots of pictures.

Our loyal steed, Cooper, took part in the Halloween festivities as well. He, too, gets an annual costume. He has been a lobster, a ladybug, and this year, he was a pink fairy. He truly puts up with a lot. But I think he was happy that he wasn’t the only one dressed up this year. His sweet sissy was right there with him.

Relaxing in Santa Rosa

From Kate:

It has been very difficult to get out of town in the last few months. Mark and I have always enjoyed sneaking away for a weekend here and there in order to just break from the routine of life, see new things, and relax in a different environment. We were able to get away this summer briefly for a few days to take Getty and Cooper to San Fransisco. It was wonderful to find a dog-friendly beach for Coop to run his legs off and for Getty to see the ocean for the first time.

We have been so incredibly consumed with Getty’s SMA that we simply didn’t know when would be a good time to sneak away. We hadn’t given it much thought and we also were not sure if Getty’s health would be compromised if we did. So last week I looked at Mark and said, “we need to get out of here!” For both of our sanity we need to breathe and relax. So here we are in the Railroad District of old Santa Rosa.

The moment we got to the room, we laid sweet Getty on the bed. She just giggled. It is far fluffier and bigger than our bed at home. She looked at us as if to say, “where are you guys going to sleep?”

The Railroad District is adorned with Peanuts character statues. Charles Schulz, in his later years, lived in Santa Rosa and there is a museum in his honor we will be checking out tomorrow. I have always been a huge fan of all the Peanuts characters. Snoopy and Woodstock have always been my favorite cartoon characters. I was hoping to get out this way so Getty could see all of them and perhaps enjoy them as much as I do.

This trip was made possible thanks to a couple of things: a car bed and a stroller. The car bed was loaned to us from Families of SMA (FSMA). Getty is no longer able to safely use a regular infant car seat. The contour shape of a regular car seat cuts off her airway and causes her to gurgle on her saliva. The car bed allows Getty to safely and comfortably lay down in the car. I rode in the back with her on our way to Santa Rosa and I felt a bit envious as she looked so comfy and snuggly in her car bed. She fell asleep almost immediately and remained snug as a bug until we got to the hotel. Thank you FSMA for being so thoughtful, we certainly appreciate it.

We were also given an unbelievable gift from one of Getty’s guardian angels. An anonymous person gifted us a new stroller. This stroller has the ability to recline and fits the shape of Getty’s little body. She is so comfortable and she is so much more happy when we get out. Earlier we were getting to the point where we were reluctant to go out in the old stroller because we knew she felt restricted. Since the previous stroller was actually just a frame to hold the removable car seat, it restricted her breathing, contributed to her gurgling, and she just looked miserable as it was hard on her posture. For the person who purchased this stroller we can’t thank you enough. You have given us peace of mind and that, in itself, is so precious to us. Knowing that Getty is protected from the elements and at the same time surrounded in comfort and safety is just amazing.

We have really enjoyed catching up on some great family time and on some much needed sleep. We have enjoyed just being in the moment and enjoying each others company and making memories.

PS: The plastic cover on the stroller is a rain shield.

Make it Through

From Kate:

We wanted to share an absolutely beautiful song that was written and sung by Robbie Rosen. “Make it Through” is a fitting tribute to a little princess, Miss Sophia, who shares the same SMA Type I diagnosis as Getty. Her family is such an inspiration to Mark and me. We continue to lean on them for advice and strength.

We encourage you to watch it and share with others.

Thank you to Vincent and Catherine Gaynor for incorporating other sweet babies and their angels in the video.

http://www.youtube.com/user/robbierosensings#p/a/f/0/mZ51wEIWrNk

Here is a link to their Sophia’s Cure Foundation in honor of their sweet Sophia:

http://www.sophiascure.com/