A lot of people with cystinosis struggle with thermoregulation; the ability to regulate body temperature. The cause of this is not entirely known. As with most symptoms related to cystinosis, some people experience them and others don’t. A lot of parents have noted that their child doesn’t seem able to sweat, while others state that their child definitely does!
When I was looking into one of Elsie’s CTNS gene mutations, the 57 kb deletion, I found this research report, The 57 kb deletion in cystinosis patients extends into TRPV1, which stated that the 57 kb deletion not only knocked out the CTNS gene but also extended into the neighbouring TRPV1 gene. It states that the “TRPV1 plays a regulatory role in a number of physiological processes including thermoregulation,” but as far as I can tell no one has investigated this further.
Whatever the reason, we have definitely noticed thermoregulation becoming an issue for Elsie. Every year it seems to be more difficult for her, whether that’s because she’s getting bigger or just more vocal I’m not sure, but I do know that she is not pleasant to be around when she overheats. Not that a I blame her, being too hot is awful. Living in the Pacific Northwest means we don’t have to deal with it for very long, which is nice but can also be difficult because we don’t have air conditioning in our place and just got the AC fixed in our car last week.
Sometimes we need to get creative and today I wanted to share some of my tips that have made life in the heat a little bit easier for us. I also turned to the cystinosis community for their “insider” tips and found some pretty cool products I hadn’t known about.
- Hydrate – Dehydration is a big issue when the heat is on, so making sure our kids are properly hydrated is more important than ever. Elsie is super picky when it comes to her water and can be heard demanding “ice cold” water frequently. We’ve been using the Cool Gear water bottles that have a core you can take out and keep in the freezer when not in use. I found ours at Walmart. Other parents said they used a CamelBak Pack filled with ice and water, a Mist ‘N Sip from O2COOL, and the good ‘ol water bottle that’s been half-filled and placed in the freezer (the key is to leave it on it’s side).
- Appropriate Clothing and Accessories – This point we are not so good at. Mostly because Elsie is at that age where she knows what she wants to wear and won’t accept any substitutes. But when I can I try to steer her towards light weight clothing in light colours. A light weight, breathable hat with a good sized rim to keep the sun off their faces will also help but again this is a struggle for us. On the flip side at least Elsie knows what she wants! So I settle for sunglasses and make sure to either put her hair up or in braids so that it’s off her neck.
- Sunscreen – This is a bit of a no brainier but important to include none the less. Like Elsie, a lot of people with cystinosis tend to have fair skin and it’s super important to use a high SPF sunscreen and apply regularly throughout the day. Luckily Elsie’s never had a sunburn but I suspect she’s just like her fair skinned dad who burns really fast and really bad.
- Stay in the Shade – Whenever possible try to hang out in the shade. We have a 3 sided beach tent that we set up on hot days, this way we can create our own if we can’t find any. The one we have is actually a huge pain to set up so I don’t have a specific type to recommend, however I can recommend that you check out some online reviews before purchasing. You could also use a large golf umbrella which would at least provide enough shade for your child. Or if you have chairs and a beach towel you could hang the towel between two chairs, but this would probably only provide shade for a baby. We used to do that for my family dog, a tiny Bichon Poodle!
- Cooling Towels – This I never considered until asking the cystinosis community and it was been one of the best tips ever. Sports and golf stores carry cooling towels that you soak with water, wring them out and then snap to activate. You then place it around your kid’s neck or wherever else they want it. We got ours, the Mission Enduracool, from SportChek just in the quick grab section near the check out tills. A few parents have recommended the Frogg Togg Chilly Pad and the Ergodyne Chill-Its. My only tip is that we were given a knock off version that had the similar foamy pocket surface like the Frogg Toggs and Ergodyne but when it dried it was stiff as a board and we couldn’t roll it back up to put in the plastic carrying case. So one day we rolled it up and put it in it’s case while still damp and the next time we went to use it, it was completely moldy! It was disgusting, so I’d recommend sticking to towel brands that have good reviews.
- Other Cooling Accessories – Other little items you can bring with you that are super helpful are spray bottles and fans, or both combined! I saw a spray bottle with a battery operated fan attached to the nozzle at Michaels the other week and am now kicking myself that I didn’t buy it. But you can also get spray bottles and usually little hand held fans from any Dollar Store. The key is to look for a spray bottle you can set to mist. We actually got Elsie a little Frozen hand held fan from Toys R Us and they even had ones attached to tubes of candy.
- Water Play – Look for ways that your kids can play with water. Earlier this summer we had one of our best afternoons just playing with a hose, a $2 mini pool and some Tupperware containers in the courtyard. You could also take your kids to a water park, splash pad or outdoor pool. Or play with water guns, slip n slides, water play tables, water balloons, sprinklers, fill a big bucket full of water, or anything else you can think of. Water play doesn’t have to be an expensive outing and most kids love playing with water! Even just a cool bath on a hot day can work wonders.
- Keep Your Car Cool – If you’re like we used to be and don’t have air conditioning in your car there are still little things you can do to try keep the internal temperature down. We always try to park in the shade and usually leave a couple windows slightly cracked open. We have roller shades in both back passenger windows to block out the sun for the kids and when we park we put a reflective car shade in the windshield and another one over Elsie’s car seat so that the buckles and straps aren’t blistering hot when she gets in. I saw a post on Facebook where a woman put an ice pack in a Ziploc bag and buckled it into the car seat to keep the buckles cool. It’d be a useful way to use the ice packs we get from all the medication shipments! Another parent said they used a car fan that you could plug into the cigarette lighter, which she put between the front seats so that more air made it to the back, and after checking out car fans on Amazon.ca I can see there’s some pretty cool products out there.
- Keep Their Bedroom Cool – Elsie gets really hot when she sleeps and usually she has all the covers pushed aside when we come to give her cystagon at 1:30am. Last summer we installed a ceiling fan in her room and we also use a portable fan pointed towards the bed to help keep her cool. One mom in the cystinosis community has been struggling with keeping her daughter cool during the night and found two pretty cool products, a Gel Cool Pillow and a Bed Fan. We haven’t tried either yet but they are top of the list if Elsie gets too hot. I’ve also seen these Thermal Aid stuffed animals advertised, which can be used as heating and cooling pads for kids to snuggle with. And one of the best tips is to use natural fiber sheets, like cotton. They will breathe the best and wick moisture away.
Of course my favourite way to stay cool in the heat is to head to 7-Eleven and get a slurpee! But for children, water is always best (okay who am I kidding, that’s true for adults too) and my kids are too young to be drinking slurpees anyway.
I hope that you find some of these tips useful and I would love to hear any tips that others may have, so if you know of a cool product or even a super obvious tip that I missed please leave it below in the comments.
Stay cool everybody!