Today (Thursday) is the second full day of the government shutdown, and I’m already starting to feel a little stir-crazy. As a furloughed federal worker, that means today is the second day that I’m not working and not getting paid. That’s bad.
And my take on the latest media reports is that things aren’t going to resolve anytime soon. Which means I’m faced with figuring out what to do with myself for a bit, now.
I spent yesterday writing an email to my Congressman and helping Jennifer around the house. (The amount of chaos she organizes each morning is truly amazing.) I also took a little time to organize our cream closet. We took advantage of a sale on Jergens at around the same time that our regular shipment of Aquaphor from Beiersdorf arrived, so that means our cream closet is now fully stocked.
The picture here of the reorganized cream closet actually is a touch mtisleading, since we are really stocked up on Jergens now, and are actually running a bit low on Cetaphil, the cream that the boys use the most. (The jars of Cetaphil and Aquaphor on the floor are stacked all the way to the back, though.)
I have several thoughts to share here about the shutdown and how budget cuts to the NIH have harmed ichthyosis research. Stay tuned for that rant.
In the meantime, we haven’t received many responses to our post from earlier this week asking about what people with epidermolytic ichthyosis do to care for their hands and feet. We’d like to get as many data points as possible, so if you have a form of epidermolytic ichthyosis and have a few minutes to spare to talk about our skin, please contact us (by leaving a comment here, via Facebook, via email, or any of the other myriad ways available). We’re happy to keep responses anonymous, and you don’t have to share any pictures (although pictures will certainly help).
And just to be clear — we’d like to talk to you even if you don’t know your specific genetic diagnosis.
We’re starting a new project here, and if you have a form of epidermolytic ichthyosis, we’d like you to share with us and our readers how you take care of your hands and feet. We will collect, curate and then publish the responses on our blog here, with as much advice specific to each individual type of ichthyosis as we can.
Here’s how this project started. Last week, a friend with a toddler affected by epidermolytic ichthyosis (formerly known as epidermolytic hyperkeratosis, EHK, bCIE and other names) posted on a medical forum that she was having trouble dealing with her child’s feet. She told us she was unsatisfied with the responses from the panel of experts, because they only were able to tell her things like, “Well, generally, these creams…” and “ask your doctor.”
Rachel and I believe that as a community of affected people, we should aspire to have better information available. Now, I know we all have different mutations and differences in what our skin looks like and how it responds to various treatments — so as always, we have to be careful to pick out the relevant advice and disregard the rest, because what may be good advice for one person might be catastropically damaging to another. And we use different creams depending on our location and what our medical insurance will cover. I think we all understand that.
But just because there’s a wide range of different types of ichthyosis, and a wide range of treatments, it doesn’t mean that we need to keep the discussion at such a general level that it’s difficult for someone looking at the responses to figure out what might be applicable to them. And we also think the reality is, the affected families are the ones with experience, so this level of detail is only going to be available from affected families.
Each of us had to figure out, individually, what worked for us. Why can’t we share that information? So please, step up here to help out the new moms who might be just starting to invent their own wheels.
Below the jump, I have pictures of different types of keratinizing ichthyosis. Please tell us which one looks like you, and how you cope with it. And remember, we are looking for specifics tied to your specific type of ichthyosis.
Continue reading Epidermolytic Ichthyosis: Hands and Feet?
Now that the kids are all school-aged, slow-moving baby bikes have gone by the wayside. Just because they have ichthyosis doesn’t mean they don’t have typical kid desires. It has been a challenge to strike the balance between keeping them safe and letting them do things with their peers. One point of conflict has been bikes. Rachel grew up without a bike once she outgrew her tricycle, and grandma has told me several times that I am risking falls if I let them ride a bike, so don’t even consider it.
I wrote yesterday about some of the toys we used when they were younger, but kids grow and we’ve had to upgrade over the years. Read on for the great solution we found this summer.
Continue reading Our Bicycle Solution
One of the big challenges we’ve had over the years is letting our kids do normal things with the neighborhood kids. All summer, the boys are challenged by the heat in the afternoons and mosquitoes in the morning or dusk hours. We need to watch them carefully when they’re out in the summer heat, since overheating is a very real danger that the kids are sometimes all-too-willing to ignore. (Or, in the case of Cookie, all-too-eager to use as a reason to stay indoors.)
There are many other families with elementary-school-aged kids in our neighborhood, so we regularly see young kids on bicycles cruising near our house. Kitty’s friends on their bikes often stop by to visit. A kid with severe ichthyosis riding a bicycle (or learning how to ride a bicycle) presents some unique issues, which we’ve managed to avoid so far.
Read on for the solutions we found for our preschoolers.
Continue reading Scooters and Big Wheels
We’re seeing a picture of a baby with harlequin ichthyosis “going viral” on Facebook. Just like about 10 months ago. This won’t be the last time it happens, either.
The caption to the most recent viral idiocy, posted on September 20, says, ” 1 Like = 1 Pray / 1 Share = 2 Prays / This will b ignored only by those.. Who doesn’t have a heart..” As of this morning, three days later, the picture has been “liked” over a million times, and “shared” over 81,000 times.
If you care at all about ichthyosis and the families affected by ichthyosis, DO NOT “LIKE” OR “SHARE” OR OTHERWISE LINK TO THIS PICTURE. (When we saw this happen last year, we blogged about why you shouldn’t like or share those posts.)
Sadly, we know who this baby is; he passed away and praying for his recovery will not help. This is just a stolen picture that does nothing to promote ichthyosis awareness or advocacy. It’s just a blatant scam to profit from Facebook “likes”. Don’t participate in it.
If you are at all interested in this issue, please take a look at my previous blog entry, “A conversation with my 10-year old about harlequin ichthyosis“.
For more on this issue, also check out last year’s responses from Courtney (Brenna’s mom), DeDe (Evan’s mom) and Carly Findlay.
ISG in the UK co-sponsored an “online clinic on skin conditions” that’s taking place this week. It’s still going on, and there are some worthwhile and interesting responses now, but Jennifer and I think that the forum was marred by the inclusion of a hypnotherapist on the panel of medical experts.
In one of the first questions on the forum, a parent with a 16-month old daugher with a form of epidermolytic ichthyosis (formerly known as EHK or epidermolytic hyperkeratosis) posted that she was concerned with “thick yellow skin on palms and soles and really thin skin on hands and feet” with “a lot of flakes, build up and blistering” and overheating. The first reply, by the hypnotherapist, naturally suggested hypnotherapy. For a 16-month-old.
Our first reaction, which you might have seen on Facebook, was unprintable given our editorial standards for our blog.
Continue reading Hypnotherapy and Ichthyosis
As a young child, I was diagnosed with congenital lamellar ichthyosis (now classified as a type of autosomal recessive congenital ichthyosis (ARCI)). I dutifully parroted that diagnosis to any adult who asked (and even helpfully spelled it out). When confronted with skepticism that I didn’t exactly match up with others who had lamellar ichthyosis, I dismissed that skepticism with, “Well, I’m really fortunate that I have a very mild case of it.”
Today, in 2013, I not only have hindsight of that (mis)diagnosis, but also the knowledge that the connectedness of the Internet and the work of great organizations such as FIRST has provided. The diagnostic tools available in 2013 are also vastly superior to what was available in the 1970s and 1980s, when I was growing up. And programs like Dr. Choate’s ongoing genetics study and FIRST’s Tele-Ichthyosis program are increasing the reach and depth of our specialists.
So I’m blogging about this issue not to blame my childhood physicians or my parents (or even myself), but rather to examine what the mistakes were, and what frame of mind led to those mistakes and how others in a similar situation might be able to avoid similar mistakes in the future.
Continue reading Rachel’s Misdiagnosis Saga: Lamellar Ichthyosis
School started yesterday. School, for us, starts at 7:40 in the morning. This creates a bit of a problem for our family, since we need to get all three boys bathed and out the door before daylight.
The summer sunshine makes things worse. Up until last week, it was still somewhat light after 9pm. All summer, our kids have been on a daylight schedule, up sometimes until 11 at night. “Bed? What? It’s still daytime out!!” protested Monkey. And Kitty’s sleepovers would end up with girls up until 3am.
So last week I started pushing earlier bedtimes. Rather than trying to force them to go to bed three hours earlier, I just put them to bed a half hour earlier each night until we were down to 8:30 over the weekend. Fortunately, the sun cooperated and went to bed at a reasonable time, too. Breakfast at noon just won’t work once school starts. And the boys HAVE to have a bath in the morning. Three kids. Forty minutes of bathtime each. Even with two showers going and two kids crammed into each shower, they still need time to cream, dress, brush teeth, eat, etc, and all must be done by the time the bus rolls by at 7:10.
So I threatened to buy an air horn if the kids couldn’t start getting up in time. They laughed.
Continue reading Ichthyosis and School: Air Horn Reveille
Retinoids. You’ve heard of them, and maybe been prescribed them. You may have heard that some people say they are a wonder drug that made their ichthyosis go away, but others say scary, scary things about them.
We’ve previously written about sorting out the names of different retinoids and discussed how much one retinoid costs our family. And yesterday, Rachel wrote a post trying to explain issues with retinoids to our 1o-year-old.
So let’s move on and talk about what retinoids actually are.
First, the short version. Retinoids are present in our food. Everyone — not just people with ichthyosis — takes in retinoids by eating red and orange vegetables and fortified cereals. Retinoids come from Vitamin A. We can’t make them directly in our bodies, so we depend on our diet to get enough. Retinoids get stored in fat cells in an inactive form and convert into the active form wherever they are needed. Normally, retinoids are needed for (among other things) sight, sperm production, skin development, a healthy immune system, bone repairs and during early pregnancy they help form the spinal cord, brain, head and arms and legs of a fetus.
Because of their effect on the way the body makes skin, prescription retinoids have been a very effective way to manage severe ichthyosis. However, because of the general effects on the rest of the body, they also come with risks that have to be managed closely with your doctor. Some people seem to do fine, while others have trouble with various side effects. And pregnancy should be completely off-the-table while oral retinoids are being used. Prescription retinoids come in several varieties, both oral and topical, all of which we will discuss separately.
Now, the longer (but hopefully still not painful!) version for the chemically inclined.
Continue reading Retinoids: What are they?