Hey there! Somehow it’s been over a month since we’ve last spoke. I thought I’d give a quick update on Hayden, our (virtual) Here for Hayden walk and fundraiser and how the Nantzes have been surviving this global pandemic with two under two! Just last week we celebrated TWO months of Hayden being dosed with her $2M life saving gene therapy treatment. We frequently get asked if this is something that is an ongoing treatment. Luckily for us and our insurance, it was a one time, hour long infusion. But now that we have that monumental day behind us, the real work begins! Hayden has been given the SMN1 gene she was missing and it’s producing the protein her body needs, but we still have to work on strengthening all her muscles. During her first 25 days of life, prior to treatment she inevitably lost motor neurons that will never return. Her treatment isn’t a light switch, it’s a gradual progression. A very slow progression, actually. So we have lots of work to do to help her build big and strong muscles so that hopefully one day she can roll over, sit unassisted, stand, cruise, and our biggest prayer, walk.
A couple of our proudest moments to date: Hugh had her on his legs propped up at a 45 degree angle. With her hands is his, he slowly pulled her up off his legs. She has very wobbly head control, so her head inevitably lagged behind. We're still not really sure why he kept going but all of a sudden we both watched her pull her head up and inline with her spine. I cried. It was incredible. These things may seem so small, but for a baby with SMA, they're huge. A healthy baby at her age is most likely doing tummy time with their head lifted, no problem. Lifting her head against gravity will be one of the biggest milestones we hope to eventually achieve. In the meantime we inch along applauding her hard work in moments like these where she is showing us just how strong she really is.
Water therapy is huge for kids like Hayden. It allows them to move in ways that they may not be able to out of water. We decided to put her in our big tub and let her float (supported of course) and just see what kind of movement she had. I wish I could share the video on this blog because it might just make you cry too. Her little legs were froggin' all over the place! Out of water she wiggles her feet at the ankles and maybe scoots a leg a little here and there. She definitely doesn't lift her legs off the ground. Think of the yoga "happy baby" pose... hands to feet, rolling side to side. A healthy baby might be doing this or close to it at her age. While this is probably a little ways off for Hayden, it is so encouraging to see her move her legs in the water. THERE ARE STILL NEURONS FIRING! Praise Jesus. While we see lots of other little things each day, these stand out as the most impressive as of late.
Our biggest struggle continues to be this darn feeding tube. It's been nine weeks now. Nine awful weeks feeding our baby through a tube. If you're friends or family and not a fellow SMA family feel free to skip these (probably) boring details (just head on past the next cute picture below). But for my other SMA families:
If at any point on your journey you feel unsupported by your medical team, speak up. Don't wait. It's unfortunate that not even 2 weeks after our infusion the entire world shut down, which made it incredibly difficult to receive the help, support and services we needed to have weeks ago. At the end of each day it's impossible to not question "what should I have done today, what call should I have made to further her progress that I didn't make". The guilt is real. But what makes it even more challenging is when you have to blaze your own trail for success and aren't receiving proper direction and guidance from the healthcare professionals who are overseeing the health of your child, who are influencing her future wellbeing and quality of life. Due to the lack of support combined with some (in our opinion) improper care we decided it was in Hayden's best interest to move to a different team of doctors. While this was not a decision we took lightly, after a variety of red flags and concerns, we felt it was best.
The feeding tube is such a hard road to navigate. While at the time I agree it was most definitely essential for her survival, we literally got sent on our merry way with little to no follow up. The difference between Hayden (or any SMA baby that has been treated) and other babies who might need a feeding tube is that post infusion, her body starts to improve, albeit gradual, probably quicker than most who might get a tube for other reasons. So we knew that even though her swallow was dangerous a week post dosing, it would improve. Around 4 months of age most babies lose their swallow reflex. This doesn't mean they can't still suck, swallow, breathe. But it means they now have the choice, and it's not automatic. So if you're little one isn't using that skill, it makes it even more challenging to establish. All this to say that while a feeding tube solves one problem, it creates another: dependence. Which I'm worried is where we have found ourselves nine weeks in. We should have been instructed to begin seeking therapy almost immediately following the feeding tube placement, but we weren't. Four weeks went by and I *insisted* on another swallow study. The second study showed great improvement so they allowed us to begin bottle attempts. But after 3 weeks of offering a bottle with absolutely NO success (she wouldn't even latch) or further support for that matter, we suspected that even though we were following the dietician and developmental pediatrician's direction, we were over feeding her. Come to find out, they had given us a healthy babies feeding schedule. Well guess what. Hayden doesn't move like a healthy baby so she doesn't metabolize like a healthy baby, therefor doesn't needs as much food. So we were stuffing this poor little dumpling and she was gaining weight twice as fast as she should have been. So once we figured that out, we had also probably pretty much traumatized her with the bottle. Fast forward another 4 weeks and we end up with a pretty significant frenectomy (tongue tie release) that I've been questioning since before her diagnosis. Why has my child been seen by SO many physicians and specialists but I'M the one waltzing her into the ENT begging for help. And now here we are... 9 weeks, 100% tube fed with a very long road ahead of us. But just earlier this week we had our first session with a Speech Therapist who we love. We are so hopeful that under her coaching we'll be able to get Hayden working on a bottle in no time.
TRUST YOUR GUT.
A couple months ago my cousin offered to pull together a walk in support of Hayden and the SMA community. We added a little fundraiser to the mix and with the support of all you incredible people we mailed a check to CureSMA for over three thousand dollars!!! CureSMA is an incredible resource, especially to newly diagnosed families like ours. If you get the chance, check them out at CureSMA.org! While our "live" walk has been postponed tomorrow, our virtual walk is still on! So put on your Here for Hayden t-shirt and get out and walk. Walk for the kiddos who can't (yet!). Be sure to send us your picture in your shirt so we can help spread awareness (and have a little fun!) on our Facebook event page. Thank you again for this incredible outpouring of support. We are so so grateful.
This momma just completed her first week back to work (from home) with two under two raging upstairs while I hide out in my little closet office in the basement. Bless our sweet "Nanny Kat".
Avery turned TWO on Mother's Day and we celebrated with home made decorations, but the cake was purchased ;)
What a time to be alive, friends. Stay safe, stay healthy. Most importantly stay respectful of others. Just because you're healthy and think all of this is silly and over the top, some of us have little ones that we are working very hard to protect and keep healthy because their lives depend on it.
Happy weekend to you!
Love,
Hugh, Logan, Avery + Hayden
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