We are busting common myths about Prader-Willi syndrome
Prader-Willi syndrome (PWS) is a rare genetic disorder which affects around 1 in 10,000 - 20,000 people (Better Health Channel). This disability is quite complex and it’s caused by an abnormality in the genes of chromosome 15.
The most well-known, but often misunderstood symptom of PWS, is a hunger and obsession with food that simply won’t subside.
We are taking a look at a few myths that surround PWS and knocking them out of the park!
Myth #1: People with Prader-Willi syndrome can control what they eat
One of the most common symptoms of PWS is a constant and insatiable hunger which typically begins at two years of age. Some people think that people with PWS choose to overeat and it’s simply a lack of self-control. Not true.
People with PWS have an urge to eat because their brain (specifically their hypothalamus) won't tell them that they are full, so they are forever feeling hungry.
Kate, a House with No Steps customer, sums this up in her blog about living with PWS:
“People with PWS don’t have the signal that comes from the brain, the light bulb going on saying, ‘Hey you’re full’!”
The chronic feeling of hunger means that some people with PWS will go to extreme lengths to get food such as eating out of rubbish bins, stealing, hoarding, eating strange foods such as coffee grinds, as well as consuming uncooked, mouldy, or frozen food.
Myth #2: The only sign of Prader-Willi syndrome is overeating and obesity
Because the constant food craving comes to mind when people think of PWS, people often don’t understand what living with this disability can really mean, or understand the challenges people may experience.
The symptoms of PWS can be quite varied, but poor muscle tone is common, and when babies are held, they can feel 'floppy', much like a rag doll (Better Health Channel). Other early symptoms can include a poor sucking reflex when feeding, a weak cry, and difficulty waking up.
Someone with PWS may also be born with distinct facial features including almond-shaped eyes, a narrowing of the head, a thin upper-lip, light skin and hair (50% of people have fair skin, blonde hair and blue eyes, regardless of what their family members look like), and a turned-down mouth (Better Health Channel).
A level of intellectual disability is also common, and children can find language, problem solving, and maths difficult. However children with PWS can have other strengths, and it has been found that they can be particularly talented at doing jigsaw puzzles. In one study, children with PWS far outperformed those without, placing more than twice as many pieces (NCBI, Dykens EM).
For teenagers and adults, short stature is also common and sex organs can often be underdeveloped which causes infertility.
Myth #3: All people with Prader-Willi syndrome will be obese and die young
Because PWS can lead to obesity, this can come with its own set of health risks including diabetes, cardiovascular disease, sleep apnoea, and musculo-skeletal problems.
It is difficult to maintain a healthy weight with PWS, however, not all people are obese, or even overweight. Although it can be challenging, by taking steps to eat a healthy or calorie restricted diet, as well as exercising frequently, people with PWS can keep their weight down.
Also, early diagnosis and treatments such as growth hormone therapy can also help prevent obesity. With the range of treatments which are now available, the obesity rate among children with PWS is similar to the general population (Butler, Merlin Gene, 2006).
And as long as someone’s weight is controlled, the life expectancy for a person with PWS is actually the same as anyone else.
Myth #4: Prader-Willi syndrome cannot be treated
Although there is no cure for PWS, there are many different treatments available for children and adults.
For example, Early Intervention which includes physical therapy, speech therapy, and occupational therapy has been shown to help kids improve movement, and learn communication, social, and other everyday skills.
PWS is the most common genetic cause of morbid obesity in children, and therefore diet and exercise is very important to help lose weight and build muscle tone. Because of the nature of PWS, at times one of the strategies may also be to keep food out of reach to prevent overeating.
Treatments such as human growth hormone treatment can also promote growth in children, increase muscle tone, and decrease overall body fat.
Read Kate’s blog about living with Prader Willi syndrome.
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