I'm sooooooo glad we were discharged on a Friday and had the weekend where we could just—well—I was going to say recharge, but honestly, it was anything but relaxing. Not having our usual weekday responsibilities did allow me to have more time to adjust to our new schedule though, so I'm very grateful we got two whole days before we jump back in to the thick of it!
Two weeks ago, Damian's GI (liver/digestion) doctor called me and gently urged that she really thought it was in Damian's best interest to finally get a feeding tube. Damian's latest swallow study, showed that Damian's swallowing had become really "risky" and nearly unsafe. So I agreed.
Damian's doctor said if we were to go the standard route of making appointments to see the surgeon, feeding therapists, etc. before we scheduled an outpatient surgery, it would be months before Damian would get a feeding tube. Instead, she said she would request Damian be an emergency admission as an inpatient, and we would just stay at the hospital while we had all the consultations, exams, and ultimately the surgery done. As an emergency admission, she said we would be called to come in to the hospital just as soon as a bedroom was available.
That call came just a day or two later (on Wednesday), and we were admitted to Children's Hospital that night. It was a week and a half before the surgery finally happened as there was a lot Damian had to do to prepare for it. I took plenty of video footage of our entire experience and will be starting to edit a video this week for our YouTube channel, so I'll skip a lot of the details and instead answer my most frequently asked questions about this very new chapter of our lives:
What is a G-Tube and how is it placed?
I'll definitely be able to explain this better in our upcoming YouTube video, but here's the gist of it: there are a few different types of feeding tubes, each named to describe where the tube itself starts and ends. Damian had an NG (Nasogastric) tube at first when we went to the hospital. The N (Naso) means it starts by going through the nose and the G (gastric) means it ends in the stomach.
"G tube" means it just goes directly into the stomach. In surgery (and this is reeeally simplifying it by the way), the surgeons grab the stomach and pull it up so it sits against the skin, sew it to the skin wall, then poke a hole and insert a valve that looks just like the valve cap thing on a beach ball (where you blow air into it). So when you open the cap, you have a hole directly into the stomach. A feeding tube pump can then connect to it and pump in baby/toddler formula, medicine, or honestly almost whatever you want if you prepare it properly (I hear there are recipes online if people want to make their own food).
The idea of having a direct entry to an internal organ was really unnatural and scary to me at first (it still kind of is), but remembering that this is a stomach - not lungs or a heart or something that requires an absolutely clean sterile environment - helps a lot. After all, some kids eat straight-up dirt. Even adults put awful things into their stomachs.
Brock would like me to assure you we are keeping the G Tube as clean as possible and are certainly NOT putting dirt into Damian's stomach. We only pump in the formula and medicines that have been approved by the doctors. I just think it is reassuring to know that I can't accidentally mess up Damian's body :)
Can Damian eat by mouth anymore?
He can! While we were at the hospital, I had a bunch of discussions with the Dysphagia (swallowing difficulty) department dietician who observed Damian eating different foods by mouth and we agreed to start feeding Damian for only 20 minutes at a time and only when he had a good amount of energy. One bonus about setting a timer is that I can then note how much he can eat in 20 minutes (safely) and track over time whether he is getting faster or slower / building muscle or losing muscle.
One thing the dietician told me was that pneumonia (literally a lung infection) can be caused by food going down the wrong tube, but it's more often caused by bacteria that multiplies in the mouth for hours before trickling down the throat during rest. She didn't cite any sources for that but it sounded logical to me. (I've since looked it up online and found a bunch of sources stating exactly that - correlating pneumonia with poor oral hygiene). Because of this, the dietician said that before we feed Damian anything by mouth, we should brush his teeth as an added precaution. That comes out to brushing Damian's teeth at least 5 times a day. WORTH IT, especially considering pneumonia is very common and often what ultimately takes the lives of babies with ASMD.
How am I (Brittany) feeling about all this? The surgery, the feeding tube...
For several months I had been, kind of, pushing off the surgery because I really wanted to see if ERT (the new liver treatment) was going to fix all our issues. Last year, our problem was just that Damian's stomach was squished and couldn't fit all the food Damian needed to be eating. When the issue became that it wasn't safe to eat all the food he needed, my mind changed immediately. I hated the idea of Damian's cute belly being cut into, but I knew it would be a momentary set-back. The pros of a feeding tube far outweigh the cons now, and so many of you have reached out and shared your very positive experiences with a feeding tube (thank you!!). I know this is what is best for Damian and I just pray that he will not always need it.
As far as thinking about life with a feeding tube now - I told a friend this week that "I am not over-whelmed, I am just a little-whelmed." After coming home though, I've grown to being very-whelmed. Still not overwhelmed, because I am here, I am doing this, and it's all going to work out. This feeding pump schedule is no joke though, and I've been spending what seems like nearly every hour of the day (and waking up three times in the middle of each night this weekend) doing something physically to Damian's body or the feeding tube - cleaning it, connecting it to a pump, adjusting and starting the pump, moving the pump and Damian to a different room, disconnecting the pump, turning off pump alarms, filling syringes and measuring medicines. Not to mention the twice a day nebulizer treatments, 5x a day or more teeth brushing, diaper changes, clothing changes, clothing super-cleaning (yes, we are still on vomit-watch)... and come this week we will be adding back in our therapies and at-home stretches and playtimes.
I hope that doesn't read as complaining - it's just listing it all out. And I'm just still trying to logically figure out if I can literally do it all and still, ya know, eat or sleep myself. Stay tuned, because this week I'm about to find out. The social workers at the hospital reminded me that at this point, Damian qualifies to have at-home nurse visits which could be a helpful extra set of hands, but they also warned that in the state of California, there is typically a hefty waiting period due to a staff shortage (we're talking at least several months). I'm not exactly sure what kind of help I need most at this point, so we'll see how things go.
And the most frequently asked question of the past week:
What did Damian's latest ultrasound show?? Has the liver shrunk?
Oh, this ultrasound was so highly anticipated!! The last time we had an ultrasound done was within days of Damian's first Enzyme Replacement Therapy infusion for ASMD, which is great because we'll forever be able to compare future ultrasounds to it and see if ERT is doing what it promises to do: shrink Damian's enlarged liver and spleen.
Damian's whole abdomen, by the way, has felt SO different in the past month. Before, I could physically feel around the edge of Damian's tight and enflamed liver (Damian's GI doctor showed me where it was and how to feel for it) and it was really pronounced. Now, I really can't tell where it is because the whole abdomen is really so much softer and squishier! It really got my attention when I started noticing that Damian's belly became skinnier after his lowest ribs rather than protruding out. I don't remember his belly EVER being like that!
December 2022, just before his first ERT infusion
March 12, 2023
Damian had an ultrasound the day before his G-Tube surgery last week and again, I was very much looking forward to seeing some evidential validation in what I was seeing and feeling. However, an ultrasound can only say so much. The technician can measure the liver and spleen tip to tip, but can't really say the volume of it.
That being said, in this ultrasound, the technician measured the liver at 15.1 cm long and the spleen at 11.8 cm long.
In December, the liver was measured at 15.7 cm long and the spleen at 13.4 cm long.
It might seem like this is proof that it has shrunk, but if you saw in real life how the technicians measure these organs, you'd know that the measurements are more like estimates (they're not able to make sure they are measuring from the same angles or anything like that). Because of that, according to the technician who did the latest ultrasound, there wasn't much of a change at all in the organ sizes since December.
It's not a surprise at all though—we've only been doing ERT infusions for 3 months and haven't even done a single full-sized dose yet, but there is still no doubt in mind that there is more space in Damian's abdomen (Damian's GI doctor agreed when she came and saw him in the hospital last week), and the enzyme levels we check in his biweekly blood draws have come down a TON since we started ERT, which tells us that Damian's liver is much less irritated and enflamed. Could it be that the liver is kind of "deflating" in size rather than shrinking in diameter at the moment? I can't say for sure obviously, but I have every reason to be hopeful!
Damian is going in for another ERT infusion today, so we'll just keep on moving forward and (I'm sure) continue to see benefits!
Thank you all so much for your prayers on our family's behalf as we went through this 9-day stay at the hospital for Damian's surgery! I'll be honest, in comparison to previous hospital stays, where Damian was actively battling a virus or worse every day and I was worried about his immediate safety at every moment - this most recent stay felt fairly comfortable (until the actual surgery of course). Now that we're home and I can't push a little red button for a nurse to help me or for room service bring me meals (haha), life is a little more complicated. I'm sure it'll just take some getting used to, with trial and error to figure out a new schedule that is doable, but I appreciate your prayers all the same.
As always, I'll keep you updated :)
Love,
Brittany
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