9 Months on the "Brain 1.0" Treatment!
Here is our official report on Damian's skills, abilities, and any other observable differences now that he's been on the experimental drug for NINE whole months!!
Yesterday I uploaded a YouTube video that shows Damian doing a series of activities, with extra footage spliced in from when he did those same exact activities 1 month on the treatment and 3 months on the treatment:
Why was there not a report at the 6 month mark?
I actually tried multiple times. But Damian was just not in good enough health to do it. Check out this timeline:
The 3 month report was end of July
In September, Damian had a bad cold that landed us in the ER. He was sick for about a week.
In October, Damian got sick again for about a week.
In November he caught RSV, which landed us in the ER. He was sick for a few weeks.
In December he caught the flu and we were admitted to Children's Hospital on Christmas Eve - we stayed for a few days and then continued recovering at home for about another week.
Needless to say, all the sickness in the past 6 months has taken a toll on Damian. When he's sick he doesn't eat as much, throws up more frequently, and can't go to therapy (not to mention he doesn't have much energy). It's no wonder why the certain progress noted in our Month 3 Report hasn't multiplied three times over. My little sweetie just hasn't been able to catch a break during this extra-heavy cold/flu season.
And yet, we still have seen change! Some is great, some is subtle, and some changes have unfortunately been in the wrong direction (and I have an inkling why).
As always, my report consists of the following activities and assessments:
Most of this I've been able to capture in the YouTube video, but the circled items, as well as a couple extra observations, are going to be recorded below.
Overall, any setbacks right now are caused largely by Damian's lack of proper nutrition. He is getting taller, but his caloric intake is not increasing. You can see in the video that Damian has gotten skinnier - partially because of how sick he's been, and partially due to his liver and spleen enlarging ever still. We have just started Enzyme Replacement Therapy (FINALLY!!), which should eventually shrink his liver and spleen down to normal size, but we're probably still a good several months away from seeing a big difference.
Gross Motor Skills (Big Moves)
Damian's biggest improvement is his standing! Damian is getting so good at pushing weight through his legs and when he is not fighting too hard (he doesn't enjoy it) he can stand with help for a "pretty long time" - sometimes a couple minutes!
His posture is improving when he is in a standing or sitting position, but this comes with a big caveat: Damian's head. His SUPER cute but disproportionately large head (typical with this disease) is now the heaviest part of Damian's body and it's become much harder for his little neck to support it. As a result his head is now hanging down most of the time. I usually help hold his head up when he's in a sitting or standing position, and if I'm doing that, you really can see his improved posture.
Damian has stopped even attempting to crawl and is rolling over less and less during the day. He still rolls over often at night, but he's not so worried about lifting his head, so it seems to me that, again, it's the disproportionate weight that is the issue - I'm really hoping that as we continue ERT and are able to provide Damian with better nutrition, we'll see a big shift in Damian's weight and he will be able to build muscle much more effectively.
Fine Motor Skills (Focused Moves)
Damian has really improved with small movements - pulling, grasping, etc. It's not one of the official report activities, but Damian used to be so repulsed by holding onto things like markers and even pegs, but now Damian does so well holding on to them!
Bigger movements like reaching has become harder for Damian as he prefers to keep his arms bent and his hands on his chest, so we're continually working on stretching them out - again, with better nutrition and weight gain, I think we'll see a lot of improvement here
Speech & Communication
As far as specific sounds that Damian makes, not much has changed - he babbles the same kinds of consonants (though it's mostly vowel sounds like "yah-yah-yah"), however, he is babbling and making noises more often - those of you who watch our weekly Story Time with Damian on Instagram can attest to this!
I am really listening hard and trying to get Damian to copy sounds, but he's just not copying yet. Before Damian's diagnosis, when he was learning to talk, his progression of speech went from vowel sound babbling, to consonant sound babbling, to copying noises, to saying words and identifying them to their meaning. If and when Damian does start to copy noises... that is going to be a HUGE breakthrough!
"Note what Damian Understands"
Not much has changed here and I'll be honest that there has been some times where I wonder if this is getting worse, just in terms of what verbal communication he understands. I am not 100% sure either way, but I'm leaning towards it being about the same. In the end, my top concern is his neurological progression/regression and from my observations, I can take Damian somewhere he's been before and he will recognize it, like the therapy clinic. If I play or a sing a song he recognizes, he will often get really excited and start to kick or bounce his arms up and down. In contrast, if I play or sing a song he's not as familiar with, he will sometimes be interested and listen but he will not get nearly as excited. So... I think it's safe to assume he hasn't regressed?
"Note how Damian Communicates"
Damian still communicates mostly through crying, laughing, smiling, and being excited - but he's been using his hands a little more often and a handful of times he has even signed for "more" and "all done!!" It's not consistent, but it has happened often enough to give me a lot of hope! He also LOVES when people clap, and though his version is not as clean, he does try to clap when others do!
Another inconsistent occurrence: Damian loves when people wave to him... and every so often he'll bounce just one arm up and down in return! Since we first noticed him doing that, Brock and I have been waving to him a lot at home and helping move his arm back to us. I really feel like he wants to wave and boy, when he finally can - there will be waterworks (from me) for sure!!
Feeding & Digestion
"Note nutrition & observations"
I alluded to this earlier - feeding and nutrition has been our biggest setback. Damian's inability to get the nutrition he needs has made him skinnier and has no doubt stunted his bone growth, his muscle growth, and his overall energy.
There have still been positive changes though. Damian's GI doctor recommended Damian start taking MiraLax daily, which we started doing about a month and a half ago, and the far more consistent diapers have allowed Damian to eat more often (in my observations of Damian when he is hungry, satiated, full, and too full that he throws up - I estimate that Damian's squished stomach has a capacity of about 5 fl oz).
I've also noticed Damian has more strength in his mouth muscles, including his tongue. He crunches through things like cereal puffs or veggie straws more quickly, and he is really good about pushing food around in his mouth with his tongue. Damian's teeth are still really small though (stunted growth in general is typical of this disease) and most of his molars are still basically flush with his gums, so we really can't give him foods that require a lot of power to chew through.
Damian's Typical Diet per Day:
18-21 ounces of PediaSure (spread throughout the day)
1/2 a bowl of oatmeal
1/2 a baby food pouch (vegetable-based)
2/3 of a peanut butter & jelly sandwich
1 yogurt cup
a few cereal puffs or veggie straws
misc. bites of whatever Brock or I are eating (as long as its soft)
all his medicines & supplements
Gearing up for infusion #4 and a feeding tube surgery
This Friday we are going in for Damian's 4th infusion of Enzyme Replacement Therapy!!
If we stay on-schedule for the infusions (in other words, if we don't run into issues and are able to progress the dose amount as quickly as possible), we should work our way up to the "full" recommended dose amount by his 10th infusion, which will be at the end of April (and he will take that amount at every infusion from then on). Is it possible we start seeing results even with these mini infusions? I hope so! But it's also possible we don't start noticing change until summer. I'm hoping for the best, but we also have to plan for the worst, and that means moving forward with plans for the feeding tube surgery. I'll share more details as they come, but at the moment we're in the process of scheduling a preliminary feeding evaluation and another swallow study.
Another year of "excitement" ahead, that's for sure! The most exciting news of the week though: we have hit a BIG number on our GoFundMe!!! Go see for yourself!!!
Not only that, but we've started to gain some momentum on our YouTube channel, and if it keeps up, we'll be able to monetize our videos soon!! We're doing this y'all!! We are doing this!!!
Please remember I am not a doctor, nor do I have professional experience in the medical field. My observations and estimations are my own, based off of my experience of being Damian's mom.