Our guest post today is by De De, mom to Evan and author of the Our Young Warrior Evan blog. Evan is 2.5 years old.
The toddler years I had envisioned for Evan are not what we are experiencing right now. Even with all the challenges he faced with his skin and as a 30 week preemie, by the age of two, I did not expect him to have the delays that he has. I never expected him to be walking by two but I thought he would, at least, be sitting. It has been quite a challenge. Not only caring for a child who requires specific, meticulous skin care and therapies but caring for a child who still requires those infant needs (two years and counting) is draining.
By no means am I disappointed with Evan’s progress. I am extremely proud and impressed with his accomplished milestones (which are endless) and his pleasant, happy demeanor. Even though Evan still has many infant needs, it is tolerable. Not only because I would do anything for him but because he rarely fusses, gets cranky or cries. He tolerates the long drives to Yale and the never ending doctor visits. He tolerates the skin care, baths and Aquaphor applications. He is a little social butterfly when we go out anywhere by smiling, waving and giggling at others and his surroundings. He loves to play with his toys, watch his favorite cartoons and snack on cheese puffs, veggie sticks or yogurt melts. He has made a significant leap in progress with his cognitive and receptive skills in the past 6-8 months. And he has made some progress in his fine and gross motor skills over the past 6 months as well. But I would refer to them as baby steps rather than leaps.
As of now, at 2 1/2 years old, Evan needs support when sitting, neglects to prop when tipping/falling, needs prompts and encouragement to rollover, tires easily when propping on his tummy, stands with support for up to 30 seconds, uses a prone-stander, pony walker and custom highchair to strengthen his core and legs, and will rarely bear weight on his hands when placed in four point. He receives multiple physical therapy sessions a week and weekly to bi-weekly occupational therapy, speech, aqua-therapy and developmental teaching sessions. His gross motor abilities have always been the most delayed and most difficult for him. With his excessive calorie needs and not being a good eater, he tires easily and looses calories quickly.
Diet has always been a struggle for Evan. He has never been a good eater and never had a big appetite. He is still on the bottle which is about 80% of his diet. He eats PediaSure Peptide 1.5. (which is 42.5 calories per ounce!!!). At meal times, he will eat a little bit of yogurt, pureed baby food or my dinner mushed up. Most of the time he refuses the spoon from entering his mouth. He doesn’t struggle to eat, chew or swallow, he is just choosing not to. He snacks on Gerber cheese puffs, veggie sticks (potato based), yogurt melts, baby cookies, crackers or cereal. And of course the snacks are his favorite thing to eat and opens his mouth wide when offered them! Evan will drink at least 5 ounces of water from a sippy cup everyday.
Carrying Evan around is exhausting. The baby carrier alone probably weighs close to 20 lbs and Evan is getting closer to 19 lbs. Since he cannot sit on his own, using the carrier is the only way to cart him around for errands and appointments. I put the carrier into the stroller when I can but for errands he gets popped right into the shopping cart. His tiny little feet stick out over the edge since he is definitely outgrowing this seat in length. About 95% of the strangers we bump into ask me “How many months is he?” Considering, many parents only use the carrier while their child is still unable to sit independently.
As far as practical tips or things I have to do (other than what I have mentioned) when caring for Evan, have to do with being clean. Since infection is such a risk I have to make sure things aren’t germy or dirty. And with Aquaphor, EVERYTHING gets gooey and gross. All toys with exception to electric toys get washed constantly. Whether it’s the plastic shapes, rings, animals, or cars being thrown into the dishwasher or the cloth books, rattles or stuffed animals being thrown in the washer, it’s a never ending battle. Plastic electric toys are wiped down with a clorox wipe, soapy rag then paper towel. Cloth electric toys are wiped down with a rag and once it gets to the point of no return, these toys get the boot. Books are constantly being wiped down as well as all of the surfaces Evan plays or eats on.
Since Evan isn’t walking, I don’t have to follow him around with a rag to clean up his Aquaphor fingerprints. Anything he touches needs to get wiped down. Not because I think Aqauhpor is yucky but because it can catch and collect all kinds of dirt and germs (and lots of Bruli hair). I use a lot of receiving blankets because since I know they are clean, I feel more comfortable with Evan’s head resting against it. Making sure he has a fresh & clean blanket to rest his head on when being held, in the car seat, rocker, couch, bed or when being changed bed helps to prevent Aquaphor build up which prevents bacteria build up. Yet another never ending battle especially since Aquaphor does cause some wear and tare around the house, especially to the washing machine.
Evan is a very smart little boy. He is soaking things up like a sponge and he is picking up on some sign language to communicate. He babbles a lot and attempts to repeat the sounds he hears in a word when prompted. He understands basic commands or requests and he knows what he wants and how to get it. He has a lively bubbly personality and always has a big grin on his face. It is interesting that he is smart enough to understand what we ask of him in therapy to master the gross motor skills that usually comes naturally to a child. (We will say things like rollover, push up, stand, hold on, pull, etc) Evan is making his way, slowly but surely.
There is no definite answer or reason why Evan is so far behind developmentally right now. Not every child with HI will or has experienced the same developmental progression as Evan. Everyone is different. Everyone develops differently and in his or her own time. Evan’s preemie status, intense birth, severe neonatal period, excessive calorie needs and lack of appetite combined could all be the reason for his developmental path. We don’t really know for sure. But looking at it through a different spectrum, Evan has avoided the need for a feeding tube (as of now), has never gotten a skin infection (knocking on wood), did not have any reflux issues, and is a very well behaved child for all he has to put up with. It has been a bumpy road but we are coming to a straight-away. I am excited for Evan’s future and am incredibly proud of how far he has come. He is the light of my life and puts a smile on my face day and night.
We are not doctors or medical professionals. A doctor or nurse looking to confirm or consult on a diagnosis of harlequin ichthyosis should immediately contact FIRST, the Foundation for Ichthyosis and Related Skin Types because time is of the essence and specialized expertise is critical in caring for a newborn with harlequin ichthyosis.
Part 1: Life with harlequin ichthyosis: The basic science behind harlequin ichthyosis
Part 2: Life with harlequin ichthyosis: Newborn Surprise
Part 3: Life with harlequin ichthoysis: In the NICU
Part 4: Life with harlequin ichthoysis: Going Home
Part 5: Life with harlequin ichthoysis: Ups and Downs in the First Year
Part 6: Life with harlequin ichthoysis: Toddler years <–You Are Here
Part 7: Life with harlequin ichthoysis: School Daze
Part 8: Life with harlequin ichthoysis: The Downs of Middle School
Part 9: Life with harlequin ichthoysis: The Ups of High School
Part 10: Life with harlequin ichthyosis: Moving Past School
Part 11: Life with harlequin ichthyosis: A Harlequin Pregnancy