Caring for Children with Prader-Willi Syndrome

Newborn holding mothers thumb.

Prader-Willi Syndrome affects thousands of children in the United States, yet is relatively unknown to the general population. This genetic condition, resulting from an abnormality on the 15th chromosome, affects both genders and all races equally, with estimates of 1 in 15,000 children currently living with this condition in the United States.

Newborns present with low muscle tone and difficulty feeding. Other physical characteristics include almond-shaped eyes, a narrow head shape near the temples, a thin upper lip, and a turned-down mouth. In addition, cognitive delays and behavioral problems, including tantrums and compulsive-type behaviors, are common.

Between the ages of one to six years, a child with Prader-Willi Syndrome will suddenly develop a voracious appetite, consuming large quantities of food and never appearing full. Rapid weight gain is common and often results in morbid obesity- a life-threatening condition, especially for children. The effects of obesity can not be underestimated, as many children with Prader-Willi Syndrome who consume large quantities of food are hospitalized each year from the side effects of binge eating.

This disorder is especially trying for families, educators, and caregivers who must monitor the child at all times to prevent over eating, as well as support their emotional and cognitive needs. Often times, refrigerators and food pantries are locked to minimize the child’s access to food, as children with this disorder find ways to manipulate limits that are set and gain access to desired items. In some states, limiting access to nourishment could cross the line and be considered child abuse, so parents are often mindful of how they monitor their child’s food intake, even if it is a life-saving technique.

Some children with Prader-Willi Syndrome are educated in special education classrooms to support their behavioral and cognitive challenges. Positive behavioral supports are commonly used to reduce impulsive and tantrum behaviors and, depending on a physician’s evaluation, psychotropic medication may be administered to help reduce such behaviors.

Though not a widely-discussed disorder, Prader-Willi Syndrome has a tremendous impact on thousands of families each year. The disorder calls for constant attention from caregivers and can be draining on a family’s emotional health. Many parents and educators utilize the Prader-Willi Syndrome Assocation (USA) for support and information on this life-long condition. The organization provides families and professionals the resources necesary to help children with Prader-Willi Syndrome live a better life.