'I'm sorry . . . with what??'
'Eaosivaljvlkajsviwjv Bkcvalkjvalwkj' our visiting pediatrician repeated himself.
'Sure, okay, I guess . . . but you're going to have to write that down for me!'
Epidermolysis Bullosa, Dowling-Meara Type.
It's a rare genetic abnormality. VERY rare, in fact. And baby's skin is literally blistering and sloughing off wherever he is touched.
According to a website committed to research of the disease,
Epidermolysis Bullosa (EB) is a genetic condition where the skin breaks at the slightest touch, causing painful, open blisters and wounds. EB can mean a life of extreme pain, disability and, at its worst it is fatal in infancy.Basically, the epidermis (top layer of the skin) separates from the dermis (the deeper layer) due to a protein (keratin) malfunction. And if we're not careful, baby's going to end up looking like a little raw tomato . . . but even worse than how he'll look is how he will feel. Raw nerve endings will leave this helpless one in agonizing pain.
Specialists of EB call babies like my little guy butterfly children because their skin is 'as fragile as butterfly wings.' Shame.
On day two of his life, our very own Butterfly-Baby came with some blistering on both his legs. Day three some pin-head-size spots on his cheeks started forming. Today, his fourth-day anniversary, mom showed us the small blisters forming on his gums and tongue. When I took his bandages down from his legs, he was red and raw from the nail beds to mid-thigh on both legs. And despite pain meds that made him sleep, he still wiggled and writhed from the pain.
Dr. Orlando asked me to see this kiddo because of my experience with burn-care and dressing changes. But being an OT, there is so much more I'm interested in.
For starters, this is a really fascinating case (on a scientific level) . . . and I've learned so much reading up on it.
But more than that . . . as an OCCUPATIONAL Therapist, our Butterfly-Baby has really peaked my interest. I mean, what are the OCCUPATIONS of a four-DAY-old kiddo? Feeding, sleeping, crying, pooping, bonding with mommy, and thriving.
Goal number one: KEEP BABY ALIVE! The biggest (and most obvious) challenge to this is infection control. But so is thriving. Not only does Baby need enough calories to keep growing, he needs extra for his wounds to heal.
Now that our little Butterfly has wounds in his mouth, Dr. Orlando and I have begun discussing what our options are should he stop accepting milk from mama's breast . . . or if he's not getting a high enough caloric intake. I suggested trying to make our own Haberman Feeder and a goat-milk-based breastmilk-substitue. . . but I don't think Dr. O's been in Galmi long enough to jump on my locally-available bandwagon . . . eh, give him a few more days, he'll come around.
Please pray for our sweet Butterfly . . . he's starting life with unmeasurable disadvantages:
- He was born in one of the poorest countries on the face of the earth
- His skin is literally blistering when he is touched
- He's fighting infection in an already filthy place
- His 'interdisciplinary team' consists of a highly skilled Pediatrician and a rodeo clown . . . I mean, Occupational Therapist.
- Because of his age, we can't give him most of the 'formula' options that might (on the best of days) be available
- He's in a lot of pain . . . always . . . which increases even when his mama tries to comfort and console him