Monthly Archives: September 2012

DISABILITY AND GRIEF

THOUGHTS FROM A PARENT OF AN ADULT CHILD WITH PRADER-WILLI SYNDROME

I am currently experiencing a resurgence of grief at the impact that my daughter’s condition (Prader-Will Syndrome) is having on her life today.

Prader-Willi Syndrome (PWS) is a fairly rare genetic disorder resulting from an abnormality on the 15th chromosome.  The result is a malfunctioning of the hypothalamus region of the brain, leaving affected people with an appetite control centre that doesn’t function.

When Katherine was born she was like a tiny rag doll, with no discernible muscle tone at all.  She had no sucking reflex so required tube feeding for the first five weeks of her life.  There are several syndromes that present with similar symptoms to PWS in infants, so we weren’t given a definitive diagnosis until she was a year old.

Some of the characteristics that Katie shares with other people with PWS are:

  • Delayed milestones, e.g. didn’t walk until she was 35 months.
  • If given the opportunity she will find and eat food anywhere she can.
  • She is short (147 cm) and has extremely small hands and feet.
  • She has a high percentage of body fat, with a corresponding lower percentage of lean body mass and therefore she gains weight really easily.  She weighs around 125 kg.
  • Due to her reduced muscle mass she has a very low metabolic rate and therefore only requires 60% of the energy intake of her peers.  She desires about ten times this amount.
  • She has never gone through puberty (she is now 35).  One consequence of this is quite child-like behaviour at times.
  • Classified as borderline intelligent.  She is VERY smart in some regards, but her childish reactions to certain situations means she can present as being about as mature as a 9 year old.
  • She is a ‘skin picker’.
  • She can be stubborn, argumentative, manipulative, and repetitive and chatter incessantly.

As soon as she was big enough to be able to access where we kept food we put a lock on the fridge and pantry and everything she ate was closely monitored.  Her insatiable appetite set in when she was about six.

I felt I had to eat the same way that she did so that she didn’t think she was the only one in the family who couldn’t eat certain foods.  This created such a problem for me that I developed an eating disorder (compulsive over-eating).  It was almost as though I too had some of the symptoms of PWS because of my obsessive thoughts about food and inability to stop eating once I started with certain foods.  As soon as Kate went to sleep I’d have my head in the pantry or fridge practically inhaling food that I wouldn’t eat in front of her. I still battle with this condition.

When Kate was growing up I made the mistake of thinking that I was the only person who could take care of her.  Just before her 11th birthday my marriage ended and I placed even more importance on “being there” for her, especially as her two older siblings no longer lived at home.  This put me in the position of not really having any life of my own.

Five years later I was diagnosed with breast cancer.  I came to realize that if I died Katie would be totally lost because I had made her completely dependent on me.  The sobering truth is that we had an unhealthily enmeshed relationship and were totally co-dependent.

To ensure my recovery I sent her to live with her father and stepmother.  This was the hardest thing I have ever done in my life and I felt incredibly guilty, as though I had abandoned her, but I knew that if I were to survive I had to give both of us a life independent of each other.

A year later I brought Kate to Sydney where she was accepted as a client of an organization that assists people with disabilities to live independently.  With their support she has lived independently from her family for the past 17 years.  The most important thing we have learned is that she can survive without me.

However, given her current situation, without the level of support that she now requires, it is increasingly unlikely that I will die first because her health is becoming seriously compromised.

In the past 20 months she has been hospitalized five times because she has developed cellulitis.  This occurs as a result of her picking at leg ulcers or because no one has been available to check to see that she puts on clean compression stockings every day and washes the soiled ones.  The number of weekly carer hours (drop-in only) assigned to her with her current service provider has been reduced from 22 to six hours per week.  This is despite Kate recently being assessed as requiring 46 hours of support per week plus a sleep over at night.

We are in the process of applying for funding for her to move from independent living to a group home, where she will receive 24 hour care.  This has now become urgent if her health isn’t to deteriorate even further.   I am feeling increasingly anxious about her future and will feel much calmer if I know that she is receiving appropriate care that will be ongoing when I am no longer around to advocate on her behalf.

When Kate was born we were not offered any form of psychological support.  We were just expected to ‘get on with it’.  I believe that this contributed to my diagnosis of breast cancer; having nowhere to process the loss of hopes and dreams we had for our little girl left an open wound for many years.  At least I now have access to counselling around the grief I’m experiencing as my daughter’s current situation challenges us to find a solution that will allow her the best quality of life possible in her circumstances.

There are many parents who are in worse circumstances as they are still caring full-time for their disabled children, despite advancing years.  I urge anyone who is in this situation to seek counselling and advice on how to find suitable care for their children in the future.

Jane Gillespie © 2012

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