Spreading the word-Keeping Our Health Care Providers in the “Know”

By: Melanie Ignatowski

“Let’s take your temperature,” the nurse said. “Show me where it hurts,” asked the Doctor. These are common diagnostic questions asked when a person enters a medical facility for treatment. But what if your body temperature in general is so irregular that despite a serious illness or infection, a fever may not be present? What if a temperature considered average for others, may in fact, for you, be a dangerously high fever signaling a life threatening medical condition? What if an unusually high pain threshold prevented you from accurately reporting pain to your doctor? These are just a few of the challenges an individual with Prader-Willi Syndrome faces when being medically treated—challenges which render the diagnostic tools doctors commonly rely upon virtually useless.
Sadly, the truth is, many medical professionals have never heard of Prader-Willi Syndrome and are unaware of crucial information necessary for providing appropriate routine or emergency treatment. Lack of knowledge can be detrimental to the health and safety of those affected by the syndrome. Therefore, parents and caregivers have a significant role and responsibility to help educate those who provide medical treatment to individuals who have PWS.
Recently, Prader-Willi Homes of Oconomowoc teamed up with other members of Prader-Willi Syndrome Association of Wisconsin (PWSA-WI) to present medical issues and concerns associated with PWS and further educate and give information to medical providers at Oconomowoc Memorial Hospital. PWHO was encouraged by the number of health care professionals interested in becoming more knowledgeable about PWS.
While presenting at a hospital is not practical for everyone, there are things that you as a parent or provider can do to educate your health care professional:
  • Educate yourself: If you are aware of the possible complications and health concerns associated with PWS, you are more likely to discover a potentially serious situation early.
  • Ask a lot of questions: challenge the medical provider’s decisions and educate them through asking questions and giving specific information about the syndrome.
  • Provide an informational guide: Handouts can be very informative and helpful, especially if you are not afforded a lot of time with the health care professional. Prader-Willi Syndrome Medical Alert books can be acquired from: Prader-Willi Syndrome Association (USA) www.pwsausa.org
Below is a list of suggested medical issues and concerns that should be reviewed with health care professionals who treat individuals with PWS:
  •   High pain threshold
  •   Lack of vomiting
  •   Body temperature abnormalities
  •   Unusual/adverse reactions to anesthesia and some medications
  •   Respiratory concerns
  •   Insatiable appetite
  •   Diet Restrictions/Environmental Supports needed
  •   Gastrointestinal problems-constipation and gastro paresis
  •   Abdominal distention or bloating
  •   Skin Lesions and bruises (often mistaken for abuse)
  •   Skin and rectal picking
  •   Water intoxication
  •   Osteoporosis
  •   Scoliosis and kyphosis
  •   Dental Issues (associated with thick saliva)
    Through continuing to educate and work with our health care professionals we can improve community awareness, and significantly reduce the risk of serious illness or even death for those with Prader-Willi Syndrome.
    We appreciated the opportunity to work with PWSA-WI and the staff at Oconomowoc Memorial Hospital in bringing more awareness about the syndrome, and participating in mutual efforts to enhance the overall health care of the people we support.