I looked out the windows by the street and saw a shaggy large man sitting right on the curb we were parallel to. He was just rising to his feet and had a joint in one hand and a bundle of marijuana like asparagus in the other as he sat in front of the medical marijuana dispensary.
"Your cig-rett is sure stinky!" exclaimed 'Lou . The burly man called back as we pulled away, "This is good medicine, skinny little man - you should tell your mama to get you some, you could use some munchies!" All this time and money spent at National Jewish Health when Big Burly Man at the Colfax Street marijuana dispensary had the answer all along! ;-)
(He obviously has never seen 'Lou eat, though - not even double mummy wraps can stop him!!)
Dr. Leung came to see us at 9 am.
Ever since his last appointment with us when he said 'Lou needs to be eating 2100 calories per day, I've been very frustrated that my requests to log and calculate his caloric intake have been disregarded or forgotten. I haven't done a calorie count for him in over 2 1/2 years and have no idea what 2100 calories really looks like in relation to a kid his age and size.
What better time to gauge how much he's eating than when he's in a controlled environment like the hospital, and getting almost all of his meals from one location?
By yesterday, there was still no plan in place to track his calories despite me asking several nurses. I finally just got a sheet of paper and started listing every single morsel or drop to pass his lips. Sure enough, Dr. Leung opened his consultation today by saying that now that we have 'Lou's skin under control, we need to address his history of growth issues.
He is less than 3rd percentile for both weight and height at this time, despite recently having one of the biggest growth spurts of his life. I submitted my food log to his assistant and hope to finally have a calorie count tomorrow. I am very curious to see how his normal intake measures up - sometimes this little 29 lb boy eats more than his 180-something- lb, 6' 2" father !
Dr. Leung recommends seriously considering the human growth hormone injections we've already had recommended for 'Lou and encouraged us to seek a second opinion on the matter when we return to Michigan.
Dr. Leung also explained that although 'Lou does not have the genetic variation of filaggrin deficiency, he has every indication and manifestation of having the acute/acquired version of filaggrin deficiency.
Evidently, a minority of people with 'Lou's issues can also lose their filaggrin (a protective protein barrier in the skin) they suspect from having prolonged severe eczema and excessive scratching and infections which breaks the skin and protein barrier down and results in inflammation that also helps to erode the filaggrin, though they aren't exactly sure yet why it happens to some severe allergy and eczema sufferers and not others. Scientists claim peanut allergy'gene flaw' link - 03/11
As the Dr. spoke, I imagined a thin spiderweb-like network trying to form just under 'Lou's skin. Then I imagined his fierce and relentless scratching and digging and I saw the filament-like threads disintegrating and falling apart and his skin swelling with fluid and breaking out in bumps as the beneficial protein disintegrated and inflammatory chemicals came rushing to the spot he was damaging with his scratching, resulting in bumps and hives like he always seems to make worse and cause to spread with his violent scratching.
"...but this is really really good news, that he doesn't have the actual genetic defect for filaggrin null aberration, because this means that he can outgrow this! In maybe one year's time, with the things we are teaching you here, he can replenish his filaggrin protein and have normal skin."
Even as he was talking, my mind was racing and dropping puzzle pieces into place. This would explain his atypical presentation of eczema in unusual places and at such an extreme sensitivity, as well as his continued reactivity to contact with any irritating/allergenic surfaces despite his now perfect and completely intact skin. And why the hives and itching stop as soon as you get him away from the irritant/allergen.
They aren't true IgE-mediated reactions that might lead to anaphylaxis, they are isolated to his damaged skin that can't keep the bad things out. The rest of us have laminated skin, he has rice paper skin. It's really NOT all true food allergies!!!
I flashed back to talking to his future kindergarten teacher and Principal about him starting school this Fall. She is the teacher for his anaphylactic-peanut sister.
I remember trying to explain his 'transient reactions', as I call them: "His skin doesn't protect him, but the hives and rashes don't turn into anaphylaxis, respiratory symptoms or GI symptoms like his sister's do. If you get him away from whatever is in contact with him and causing the hives and rashes, the reaction will clear right up." It was so opposite of what we told her about his sister's allergy, that I felt bad for having such polar opposite and confusing allergy children - yet still relieved that I don't have two with contact anaphylaxis! As far as we know, he'd have to actually eat a quantity of egg, dairy, peanuts or tree nuts to have a serious reaction.
I thought of the Post-It with titles for blog articles I jot down when inspiration strikes. The top of the list was for a story I never quite got to about his first hospitalization here in Michigan, when his siblings all caught Strep in their throats and he got it in his skin.
"The illimitable strength of the boy with hyperpermeable skin."
I thought of all the times he needs stitches (4 boys here, did i mention that yet?) and how the ER docs are always completely amazed that the numbing gel sinks in and turns his skin bone white and totally numb in less than 5 minutes when it should take 20-30.
I thought of how I can't use anything but pure zinc on him for a sun block as everything else burns him like fire and gives him a nasty rash.
I thought about how he got his first wart recently- (something that people with the genetic mutation for filaggrin null aberration will be prone to for their entire lives, along with cold sores, fever blisters, etc...) and how the dermatologist said it was super tiny and only dropped one little drop of the freezing liquid on it and yet now he looks like he has a 3rd degree burn the size of a nickel.
I thought of my daughter, who has anaphylactic peanut allergy. By age 5, she'd had 4 anaphylactic episodes, the last of which happened AFTER she passed a medically supervised food challenge and had eaten her 3rd dose of peanut butter to keep her immune to it!
When we see hives on Jovie, it means anaphylaxis is on the way. I've been comparing 'Lou's hives to hers all this time and thinking that if it causes hives on him, it can be as dangerous as her allergy is and I need to get that food item out of his life completely.
And then there's our 3rd son, Jonah. He had severe reflux and tested positive to soy and peanuts (as well as a lot of seasonal allergens).
We completely removed soy from his diet, treated the reflux with apple cider vinegar and as long as he avoids soy, he remains 100% symptom free. If he gets an isolated accidental exposure, he gets hives. Just to soy. We habve a peanut free home and school, so I don't know what peanuts would do, but my point is, nothing causes hives for him except soy ingestion. It's consistent, it's predictable, it's only the thing we were told he's allergic to and nothing else. His skin is perfect 99% of the time, unless he eats soy.
It makes sense that 'Lou would eventually develop some true food allergies to the bigger and badder proteins in the worst of the top 8, like dairy, egg, peanuts and tree nuts. I know these allergies could lead to true anaphylactic reactions and would never allow him to ingest them, but now at least I feel like I understand how they happened.
I thought of our other child who'd had severe eczema. We battled it for over 2 years of his toddlerhood until he finally seemed to outgrow it. I've compared 'Lou's eczema to his so many times and not understood why I can't get 'Lou's under control when I did with my other son's. Removing dairy, frequent baths and slatherings, heavily coated footie jammies with aquaphor - why didn't any of this work for 'Lou? Now I know! Our other son had filaggrin and 'Lou didn't!
One piece of the puzzle that still doesn't fit is why 'Lou appeared to be born with skin problems if he doesn't have the genetic version of this issue, but the acquired one. By 2 days old he had strange, flattened hives all over his body, red spots and red, swollen eyes.
I asked Dr. Leung why he was born with skin issues but he couldn't say. He says not enough is really known about these skin disorders yet and filaggrin deficiency is still in the early stages of being researched - and they still don't even know exactly what causes "normal" eczema yet, either.
While there is a new cream on the market that actually contains filaggrin and which may help to replenish it, it can be costly and long-term use of emollient rich and barrier-type moisturizers can help to completely heal his skin in about a year, provided he doesn't have any major flares or setbacks and we work constantly at home and school to distract him from scratching.
I am over the moon to have an explanation that he can actually grow out of, get over or get cured of!
Another thing I love is that Dr. Leung remembers and agrees with my stance against excessive topical steroid use. He's only ordered the bare minimum use of the weakest prescriptions during our stay here and is adamantly opposed to any oral steroid use for skin flares, ever.
We did a challenge for almonds today, which 'Lou passed though he did not love eating them. He gagged for the first time that I've ever seen but finished all 3 servings with no reactions.
There was another parent group today, but I missed it due to hovering for the almond challenge. One of the kids in our program had to be transported to Denver Children's hospital today after a food trial due to a severe reaction, so a lot of us were skittish and helicopter-y today!
I went shopping tonight for his next food trial foods and stocked up for this weekend as my sister is coming from California to visit for 2 days.
I hope to take advantage of some of the free fun options for NJH patients like the zoo. Tomorrow's schedule holds nothing but food challenges for 'Lou, so we are hoping to begin as soon as we arrive. If we start early and he has no reactions, we could possibly fit 2 or 3 foods in. I've decided I do not want to extend our stay, so I'm willing to put in extra hours when I need to.