Wednesday, August 15, 2012

Causes of Death in People with PWS

Recently, the PWS community has learned about yet more deaths of people with PWS who were just too young. Ten years old. Twenty-three years old. Too young.

Whenever this news circulates, it is obviously so sad. Sad for the family who you know feared this would happen and their fears came true. Sad because you know what a joy EACH and every child is, and all the more the specialness of the bond with your child with PWS who you've fought for at every turn. Sad because you wonder if your own child with PWS will suffer the same fate. :(

For those who are concerned with the general causes of death, here is what Janalee Heinemann, Director of Research & Medical Affairs for the PWSA wrote to me (she gave me permission to share this email):

Hi Ali,

Barb sent your email on to me. I just had a long talk yesterday from another mom who had the same concerns. I appreciate the fact that not knowing is worse than knowing the cause of deaths in PWS. Sometimes we can give out more information than others, but no matter what we as an organization say or do, word spreads quickly on the internet through Facebook and email support groups. As I told the mom yesterday, the reality is that most PWS deaths are still obesity related. Due to the weak muscle tone, our children/adults who have PWS may even be more at risk from obesity related complications at a lesser weight than the general population. Right-sided heart failure, respiratory problems and diabetes all can happen due to the obesity which makes other illnesses or surgeries more of a risk. Of course, out of respect for the families, we are not going to advertise it was an obesity-related death.

As for the non-obesity related deaths, in the little ones, they are typically due to respiratory complications. For an older child or adult, there is a variety of reasons – from choking and GI perforation to accidents. It is no secret that the young man who died recently was hit by a car. At PWSA (USA) we do have a committee of doctors that study the cause of the deaths and every so often we put out another alert if we see a reasonable risk for which we can warn parents and caregivers.

Of course, as a parent you need to keep yourself armed with the Medical Alert booklet at all times and articles from our medical section of the PWSA (USA) web site as needed. Every day we are emailing or faxing articles to hospitals, physicians, and psychiatrists at the request of parents.

I hope this helps a little bit in answering your questions.

Warm regards,

Janalee Heinemann, MSW

Director of Research & Medical Affairs


Vice President, IPWSO


Cinra said...

Thanks for sharing this information.

Janis said...

If a parent wants to share how their child died that is okay but then you set yourself up to be judged. If your child dies from obesity-related causes, you will feel bad enough without others blaming you.

Ali Foley Shenk said...

Janis, I agree. And that's why when I contacted Barbara that I wasn't asking for a specific cause of any one death. I do think that it's helpful for the PWS community to know the general trends, and it is helpful to get that kind of information.