Running For Research - PWSIt is widely recognized that choking is an issue in PWS. 18% of registry participants have had a choking incident. Of these choking incidents, 50% were severe enough to use the Heimlich maneuver. In addition, concerns around choking continue well past the toddler years. When specifically looking at data for participants who have had a choking incident, 50% of those events happened at ages 6 and older.
According to an article that was published in Pediatrics Nationwide in 2020, “Individuals with Prader-Willi Syndrome (PWS) suffer from poor oral muscle control, generalized low muscle tone that can make it hard to swallow properly, a poor gag reflex and an insatiable desire to eat. Together with a low production of saliva and a tendency to rush food consumption (not chewing properly, consuming large quantities or sneaking food quickly), patients can be uniquely predisposed to suffer from a choking incident — and, indeed, to die from one. Choking is common in this population, affecting at least 1 in 3 patients during their lifetimes, and leading to death in about 8% of the PWS population, according to research in the American Journal of Medical Genetics published in 2007. Furthermore, the risk is not restricted to very young children, with the average age of death due to choking at about age 24. It appears to predominantly occur in males.”
As a result of the prevalence of choking in our population, it’s very important that families, caregivers and those in the school system who regularly attend to our loved ones receive training in how to handle choking incidents. This training could include learning the Heimlich maneuver, CPR and basic first aid.
This is not something we have encountered with Clementine ( yet). Although she is usually eager to eat her meals and snacks, once she is seated she does not rush her meal. In fact, if she is eating something she really likes, she is slow and deliberate in her chewing and swallowing. You can see the pleasure she takes in the taste of whatever she’s eating. An almost dreamlike look will settle on her face as she is savoring the flavor of the food. Often she will close her eyes as if she’s concentrating ( which I think she actually is ). Although I’m very glad that Clem takes her time when eating, I’m also disturbed by the amount of pleasure she gets from eating her favorite foods. I’m not someone who really enjoys food so this is a difficult concept for me. If she didn’t have PWS, I would probably find it a bit amusing. But because she does, it’s not humorous in the least!
Although we haven’t yet experienced choking issues, we have had our share of difficulties with a similar issue, aspiration. When Clementine was an infant, we discovered that she was silently aspirating as she drank breast milk from a bottle. Aspiration means to draw in or out using a sucking motion. It has two meanings: Breathing in a foreign object (sucking food into the airway) or a medical procedure that removes something from an area of the body. Silent aspiration usually has no symptoms, and people aren’t aware that fluids or stomach contents have entered their lungs. Overt aspiration will usually cause sudden, noticeable symptoms such as coughing, wheezing, or a hoarse voice. Silent aspiration tends to occur in people with impaired senses.
When food, drink, or stomach contents make their way into your lungs, they can damage the tissues there. The damage can sometimes be severe. Aspiration also increases your risk of pneumonia. This is an infection of the lungs that causes fluid to build up in the lungs.Silent aspiration can be quite serious, especially in infants, because it can be hard to detect. When we discovered the aspiration, we had to have several rounds of swallow tests performed to determine the severity and frequency of the aspiration. Once that was established, we began to add a thickening agent to her milk. The thickening of the milk made it harder for her to suck, which she was already struggling with, so she initially lost weight. It was a very frustrating time! Luckily, as she grew stronger, she was able to adjust to the thickened liquid. Eventually she stopped aspirating.
