Hey everyone! Welcome back to another Then + Now spotlight, where we reconnect with a family who was featured in the past and see how they’ve grown and changed over the years, as well as how their condition or disability has changed as well as their views. I have LOVED bringing back some of our favorite spotlights and am so happy to have Michelle and her daughter Lydia with us once again. We first met Lydia in 2014 when she was still a toddler and you can read her spotlight here. She’s a big kindergartner now and has a much better grasp of her medical condition MCADD. I hope you take a minute to read because it will remind you once again, that you never really know what someone is going through just by looking at them. Enjoy!
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Miggy: Welcome back Michelle! I’m so excited to catch up with you and your daughter Lydia, Lets get started! First, let’s start with a brief overview of your daughter–what is her diagnosis and how does this diagnosis or condition specifically manifest in her?
Michelle: Lydia was born with an inborn error of metabolism called Medium Chain Acyl-CoA Dehydrogenase Disorder (MCADD for short). MCADD is a rare metabolic disorder where her body is missing the enzyme to break down medium chain fatty acids into energy. Essentially, our bodies break down food into various small particles (including fatty acids) and it turns these particles into energy. There are different lengths of fatty acid chains and Lydia is missing the enzyme to break down the medium length ones. This really becomes a problem when she gets sick and can’t maintain her own food intake. During a time of restricted food intake, a typical person’s body will eventually metabolize its own body fat for energy. Lydia’s body can’t do that because body fat is mostly medium chains, so if she can’t eat enough food to maintain her own energy, she will literally run out of energy in every single cell. If she goes without treatment, she will eventually have seizures, go into a coma, and die (typically within 24-36 hours of not eating). Luckily treatment is very easy and prevention is key! We just make sure she eats regularly and if she’s sick and can’t eat, she has to receive an IV with a dextrose (sugar-water) solution in the hospital until she can eat enough again. There’s more to it than that, but it gets pretty detailed and I still don’t understand all the biochemistry.
There are other aspects of her disorder as well, such as not being able to metabolize foods high in medium chain fatty acids (like coconut oil or fatty foods). While she can have those things occasionally or in small doses, the fats will build up in her cells and liver and cause problems later on. We eat a low-fat, high carb diet with lots of fruits and veggies. She also takes an enzyme supplement that helps transport the unused fatty acid chains out of her body, and she’ll probably be on that supplement the rest of her life.
Miggy: We did a spotlight on Lydia back in 2014, in the years since what has changed the most about her during this period of time? Any unexpected highs or lows you’d like to share?
Michelle: When we first did her spotlight in July 2014, Lydia was 15 months old and we had just gotten out of that awful, exhausting first year. During her first year she ate small amounts every 90 minutes to 2 hours around the clock. She was able to extend her feedings a little as she got older but her body was much happier with eating more frequently and we were under strict guidelines for how long she could go without food, even while sleeping. In July of 2014 Lydia had never been hospitalized with an illness. In fact, she hadn’t been sick that much at all, which was a huge blessing! She also didn’t need to take the enzyme supplement yet since her blood work for that enzyme was still in a normal range.
Since then Lydia has been hospitalized 7 or 8 times. She’s gotten sick a lot and we’ve had a few close calls where she almost had to go in, but we were able to manage at home. We’ve learned a LOT about food and metabolism and changed our own diet fairly significantly. She has been taking the enzyme supplement since she was 3. The enzyme supplement comes with its own side effects and it took us awhile to figure out the right dose for her body.
Michelle: Lydia was born with an inborn error of metabolism called Medium Chain Acyl-CoA Dehydrogenase Disorder (MCADD for short). MCADD is a rare metabolic disorder where her body is missing the enzyme to break down medium chain fatty acids into energy. Essentially, our bodies break down food into various small particles (including fatty acids) and it turns these particles into energy. There are different lengths of fatty acid chains and Lydia is missing the enzyme to break down the medium length ones. This really becomes a problem when she gets sick and can’t maintain her own food intake. During a time of restricted food intake, a typical person’s body will eventually metabolize its own body fat for energy. Lydia’s body can’t do that because body fat is mostly medium chains, so if she can’t eat enough food to maintain her own energy, she will literally run out of energy in every single cell. If she goes without treatment, she will eventually have seizures, go into a coma, and die (typically within 24-36 hours of not eating). Luckily treatment is very easy and prevention is key! We just make sure she eats regularly and if she’s sick and can’t eat, she has to receive an IV with a dextrose (sugar-water) solution in the hospital until she can eat enough again. There’s more to it than that, but it gets pretty detailed and I still don’t understand all the biochemistry.
There are other aspects of her disorder as well, such as not being able to metabolize foods high in medium chain fatty acids (like coconut oil or fatty foods). While she can have those things occasionally or in small doses, the fats will build up in her cells and liver and cause problems later on. We eat a low-fat, high carb diet with lots of fruits and veggies. She also takes an enzyme supplement that helps transport the unused fatty acid chains out of her body, and she’ll probably be on that supplement the rest of her life.
Miggy: We did a spotlight on Lydia back in 2014, in the years since what has changed the most about her during this period of time? Any unexpected highs or lows you’d like to share?
Michelle: When we first did her spotlight in July 2014, Lydia was 15 months old and we had just gotten out of that awful, exhausting first year. During her first year she ate small amounts every 90 minutes to 2 hours around the clock. She was able to extend her feedings a little as she got older but her body was much happier with eating more frequently and we were under strict guidelines for how long she could go without food, even while sleeping. In July of 2014 Lydia had never been hospitalized with an illness. In fact, she hadn’t been sick that much at all, which was a huge blessing! She also didn’t need to take the enzyme supplement yet since her blood work for that enzyme was still in a normal range.
[readmore title= “Click through to read more about Lydia and MCADD”]
Right now I’m waddling through the last five weeks of my 4th pregnancy and about to begin another year of breastfeeding and I feel pretty sad about my fashion future! Right now I live in sweatpants and even my maternity shirts barely cover me. I have a hard time feeling good when I can’t wear a nice underwire (too painful) and my necklines are constantly stretched out from pulling them down, and there is spit-up all over me…
I have struggled with wearing what I like vs. the feeling of being superficial if I care too much in the past definitely. My style is WILDLY different from yours but it’s fun to see what makes other people feel good.
I think this year would be a great year to do a secondhand shopping year because SO MANY people are kondoing their closets right now. The thrift stores are seriously overflowing with good stuff right now.
Angela–Oy, those last few weeks of pregnancy are a killer! I often didn’t feel like myself in maternity clothes and while it’s obviously worth it, it can take a mental toll. And now I’m curious about your style since it is WILDLY different from mine! Tell me, tell me!
My style tends towards all neutral/black with a pop of a jewel tone like a sweater or sleeveless shirt, sometimes with a cowboy boots or a leather jacket. I guess glam/cowboy/biker? I like to feel tough/cool/boss. It feels like forever since I’ve been able to get really into fashion though, maybe I’ll come out of this next baby fog with something a little different. I’m a light-skinned Latina–short, dark haired, tend towards a more rounded body type.
While it looks great on you, I’ve never worn a romper/jumpsuit or the color orange in my life! You look tall and confident and vintage, I think I’d look like a small chubby clown.
You are making more and more interested in “jumping in” on the jumpsuit/romper trend. Particularly the orange and the denim overall-style one. I saw an Old Navy commercial where there were a few other cute styles, too.
The only thing holding me back, if I am ridiculously honest is the idea that when I’m going to use the restroom in it, I’ll basically be unclothed, lol. But that doesn’t seem like enough of a reason to avoid at least trying one out. I have a feeling once I give it a spin, it’s going to be my Look For Summer. Or FOREVER. lol.
Connie,
Yes–there is the bathroom thing, but totally not a big deal. I did see this funny cartoon about that very thing: https://www.reddit.com/r/comics/comments/8co43q/check_out_my_hot_new_romper/
Ha! It doesn’t really bother me at all. Try it. You’ll like it. (Do I sound like a drug dealer yet?)
…Okay. That comic is hysterical.
And I’m going for it. I’m romping to the dark side.
😉
You look adorable! I love your style. My style is very different as well. Mainly because I’m less than petite. I love clothing and makeup and hair. I mostly shop at a store called “Free people”. The reason for that is their clothing is known for being free flowing and a bit hippyish looking. They have adorable stuff and it’s made for “normal” sized girls but their clothing runs really big. Not to get off on a tangent; but why can’t bigger girls find clothes that don’t look like flower Sacks?! I would gladly shop at thrift stores if they had things I like that fit.
I love jumpsuits. They don’t love me. Ha! In my much thinner days I wore them. It is hard to pull off when those Xtra pounds take up residence on my hips.
I have always been picky about my looks. I don’t feel shallow at all. Since I now raise my grandson I look frazzled a lot of the time! So when I do get dolled up it makes me feel like my old self again. It feels familiar and makes me smile and feel feminine and pretty.
I think as a society we could do so much better in recycling what we have. Using what we do buy until it wears out or passing it on to someone who will use it. Waste is terrible and I’m a bad offender! I have been cleaning out and trying to be a much more consientious buyer. I’m not there yet but working on it.