Bye-Bye Winter Hello Summer!

By Sybille Kraft Bellamy

We are done with snowstorms, freezing rain and below zero temperatures. Now it is time to enjoy the warmer weather.

Unfortunately our children with AS are not well prepared for the hot temperatures. Max, 12 years old, deletion + is extremely sensitive to hot weather and humidity. To avoid any serious health issues we have to ANTICIPATE.

Children and adults with Angelman syndrome have body temperature regulation problems. They cannot adapt to rapid temperature change or extreme temperatures. In Max’s case he turns pale, his heart rate gets elevated and he becomes very lethargic. A major priority is to control his hydration. He needs to drink a lot.

Children and adults following a ketogenic diet cannot have sweet beverages. Everybody should have plenty of water with electrolytes. Electrolytes are essential for the normal functions of our cells and organs.

When the body temperature fluctuates the level of electrolytes becomes imbalanced, which can trigger seizures.

Trader Joes and Whole Foods both have bottled water with electrolytes. It is good to keep a reserve of bottles in your freezer and fridge. We buy electrolytes that come in individual bags. They are easy to transport, we keep some in the car, in our bags and at school.

For children who don’t want to drink and/or if they don’t feel thirsty you have to find what will trigger the desire to make them drink. It can be temperature related. Some of our children prefer when it is cold or warm. Some like to drink in a cup, with a straw, or a sports bottle. You can also use organic unsweetened Jell-O to thicken the fluids to avoid choking problems and for children with sensory issues. Herbal teas are great for a base, like mint or orange blossom and you can add the electrolytes to the tea.

You can drink, freeze or use the tea with Jello as well. In case of emergency we use a large syringe and squeeze it in his mouth with the mixed electrolytes. It is messy but funny! The syringe is a great way to get children to drink. Bath time is also a great time to introduce it as they think it’s a game and there is no mess.

On the side of hydration we are always concerned about the bus ride, especially coming back from school when the temperature is the highest. We bought a cooling jacket which is comfortable and great for car travel, park and beach. If you can, avoid the warmest time of the day for outdoors activities.

For children in school you must specify on their IEP it is a priority that the bus has air conditioning. Some States may not require all bus companies to have all their buses provide air conditioning. However, when it is in your IEP they must comply so make it a priority and be in touch well in advance before the hot weather hits. If your child has an extended school program during the summer you may want to contact your case manager to be sure it is the same bus company and if it isn’t remind them of the IEP specification concerning the air conditioning. We had a bad experience with a new bus company and we had to keep Max at home until they provided us a bus with air conditioning.

If your child is showing signs of heat exhaustion you must react quickly to avoid heat stroke, which can be a very serious medical emergency situation. Undress your child and sponge bath him with room temperature water, avoid big temperature changes, too cold can be a trigger for seizures. Use a fan or stay in a room with air conditioning. Start rehydration with small amount at a time with a spoon, cup or syringe. Be sure to use electrolytes or home made options. Check the body temperature, when your child is feeling better you can give him a bath. If you do not see improvement in the next hour you should seek medical attention.

For more information about medical emergency check on the Angelman Syndrome Foundation web site for the webinar with Dr. Justin Grill on “Emergency issues with individuals with Angelman syndrome”.