I usually end my posts with a disclaimer, but in this case I am going to start my post with one:
As a reminder, I am not a doctor. I do not have any formal education in medicine. I am simply a very resourceful mom who is extremely perceptive of her son and determined to help him. So if anyone reading this is looking for health advice for their own situation, please do your own due diligence in terms of research and consult with a doctor.
Let's go back to the end of March of this year, when Damian was an "emergency admission" to Children's Hospital.
Damian was classified as malnourished and his gastroenterologist (liver/digestive system doctor) had been wanting him to get a feeding tube for nearly a year. I'd wanted to start Enzyme Replacement Therapy first because I knew the reason Damian wasn't eating enough was because there was no room in his stomach. His enlarged liver was squishing it too much. If ERT worked fast enough to shrink his liver, maybe we could go without needing the feeding tube altogether.
While ERT did start working right away, it didn't work fast enough. But even more worrisome was that Damian's ability to swallow was weakening over time.
By March, Damian was hardly eating any solid foods at all - most of his nutrition was from a bottle (PediaSure), and even then I was beginning to spend more and more time holding the bottle for him.
When we had a feeding evaluation and swallow study done March 14th, the therapists told me it was no longer safe for Damian to eat by mouth at all. At that point, I agreed that it was time to get a feeding tube. I talked to Damian's GI doctor a few days later, and we were admitted as soon as a bedroom was available.
(click the link below to watch our G-Tube surgery experience)
As you saw in the video above, we spent most of our time in the hospital just building up Damian's diet, increasing the amount of food flowing through the tube little by little. Within days, Damian's skin was fuller and he was gaining weight just like we all hoped he would! And as an added bonus, Damian's dietician said we could still give Damian food by mouth - but only if Damian was up for it.
Our Post-Surgery Experience
There was a handful of reasons I was hesitant to get a feeding tube, and one of them was the extra time and care it took to maintain the feeding schedule. I'll be honest, I think I was justified in that worry. Damian's schedule dictated he be hooked up to his pump every 3 hours, for an hour-long feed each time. He needed to be fed overnight too, and the feeding bags couldn't hold a full night's amount of formula, so we would have to wake up in the middle of the night to refill it.
It reminded me a little of my 3-month breastfeeding stint with newborn Damian (without the intense pain and raging hormones, thank goodness!!), but it was certainly a relief to know that Damian was finally getting proper nutrition for the first time in a very long time, maybe ever!
More than anything, I was very much looking forward to Damian having so much more energy. More food = more energy, right?? This was going to be a great jump forward for us!
Except it wasn't.
The neurological red flags
The weeks went by and Damian was gaining more and more weight. His little body was starting to look so nice and plump, and yet... he was even more tired than usual. The first week or so I thought it was because he was just recovering from surgery. A few weeks in and I thought his increased fatigue and vomiting was simply a bad reaction to his ERT infusions. Or maybe he was having growth spurts.
A month after his surgery though, Damian began really showing concerning neurological behavior. It might be hard to describe with words, but I'll try:
Sometimes when I would lay down Damian flat on his back (to change his diaper or just lay him down), he would get a shocked expression on his face, his legs would fold up tightly towards his chest, and his arms would shoot outwards with his palms up. His limbs would get really really tight and his eyelids would start to flutter quickly.
Of course my first thought was that Damian was having a seizure, but he was responsive and I learned by trial and error how to ease him out of the tension. Several months ago (around December), this happened only every so often, but by late April it was happening every single time I laid Damian down.
Even more worrisome to me was that I noticed when he was really tired his eyes would start to roll up to the top of his head. He wouldn't close his eyes, his eyes would just roll up. It started happening only every so often (a few times a week), and then happened more and more over time.
On top of everything else, Damian also just seemed to be out of it, like his mind was foggy. He wasn't as responsive, his reaction time was slower than normal. Just like the other new behaviors - it started out infrequent, then happened more and more often.
What is happening??
In all honesty, I became very scared. What was going on?? Sure, Damian's strength had been regressing in some areas, but why was it suddenly happening so fast - seemingly getting worse and worse every day? At the beginning of May, I was at my wit's end. Damian seemed to be so out of it, so tired, and his eyes were rolling up towards the ceiling so often (a few times every hour or so). I remember crying every day for nearly a week as I watched him because I felt so out of control, so scared as things felt to be spiraling so quickly, and so unsure of what to expect.
Oftentimes, reasoning is a coping method for me. And that week I was wracking my brain to find some logic behind what was going on and ruling out what it wasn't: It wasn't the surgery. It wasn't a growth spurt. And once more than a few days had past since his last infusion, I was sure it wasn't a bad reaction.
What possible change could have made this much of a difference? Why was I seeing such a downward spiral in just a few weeks? And then a possibility hit me.
Was it the food?
Damian had never consumed this much food before. The sharp decline started about the time when Damian's food intake went up. Maybe it was the food.
I knew from the time Damian was diagnosed that ASMD is a metabolic disorder. The only thing wrong with Damian's body is that he can't break down (at least not very well) a fatty substance called sphingomyelin, which means the cells fill up with the substance, get enlarged, enflamed, and angry, and eventually die.
I also had already learned that most, if not, all foods contain sphingomyelin. So I thought it was logical to guess that food is where at least some of Damian's sphingomyelin build-up comes from. So was there any possible way I could limit how much he was getting? Or were there some foods that contained more sphingomyelin than others?
I spent a day looking up research papers and studies online about sphingomyelin content in food. And I found a bunch. Ironically they were all studies done to try and figure out how to increase the amount of sphingomyelin a person consumed, especially infants. In a normal-working body, sphingomyelin isn't just okay, it's actually necessary for good development.
All the studies I read outlined the same thing - sphingomyelin content is highest in animal products (I'll cite some sources at the end in case you are interested).
Sphingomyelin is high in animal products
I started analyzing everything. The PediaSure he was starting to drink so much of before his surgery was dairy. And his feeding tube formula was dairy-based - the formula we were literally pumping into him. I stopped the formula feeds that day.
I told Brock all this when he got home from work and he asked, "so is Damian going to be vegan now?"
Hearing him say "vegan," I suddenly felt so relieved - vegan diets are a normal thing. Plenty of people are vegan! Plenty of children are vegan! They've got to make formula for vegan babies.
I could go into all the details of how quickly we were able to get a hold of Damian's doctor and dietician, my conversation with people from the Wylder Nation Foundation and others about a diet change, etc. but I'll boil it down to this: when I stopped the formula feeds, I noticed an improvement the next day. And the next day after that. And the day after that.
How going vegan is going
Damian went vegan the second week of May (not just refraining from animal products, but also soy and some other foods that contain higher amounts of sphingomyelin), and within just a week or two his brain fog subsided. His eye rolling stopped. He still seized up his body when I lay him down, but even that seemed to lessen in severity.
The most impressive difference has been that while we were waiting for a vegan formula to arrive (it took a long long time), we had to give him fruit and veggie baby food. He was so weak at the start that he wasn't eating much, and I was really nervous about him eating enough and eating safely. But his swallowing has improved so much week to week that he is eating all day now without any of the problems he showed during the swallow study before his surgery.
Additionally, Damian has had absolutely no negative side effects from his biweekly infusions since we changed his diet. We were giving him strong nausea medication to combat the violent and frequent vomiting episodes he was having but we didn't need to give him any medication for the very next infusion he did after his diet change. He wasn't nauseous at all.
Again, I'm not a doctor, but there's no doubt that going vegan has made a huge difference.
I am just glad the timing worked out the way it did for Damian's surgery (that we did it much later than was initially planned). I'm not sure if he'd gotten it done early on that I would have recognized that the formula itself was a problem. How much worse off would we be right now if we'd been pumping him with dairy-based formula all this time?
That being said, it has now been a few months and I am starting to see that same regression happen again in Damian, though much slower. Now, when Damian is extra tired, his eyes sometimes start to roll back (though it doesn't happen too often, maybe once a week). He has been getting more tired, generally. Seeing some of these same issues occurring, though much slower, means to me that going vegan is certainly not a cure - it is just buying us time.
But time is so precious - and we need as much of it as we can get.
Scientific Studies on Sphingomyelin in Food
NOTE: There are many studies on sphingomyelin consumed in a diet - these are just a few. Scientific studies will always have a lot of references at the bottom, so if you want to go down a rabbit hole like I did, you can use that as a way to find more information (or just Google it!).
So glad I found this page. Lost track of all of you and was concerned for Damian and your family that things went terribly wrong. Thank God your little boy is still with us. All of you are in my prayers. Now I will be able to find updates on this sweet little child that blessed your life for as long as theCreator wills it.