Prader Willi syndrome

Hi,

Im a 56 yo female and have just been diagnosed with Prader Willi Syndrome.  I have del 15 q 11.1-11.3

My BMI is 30.  I was referred to a surgeon for consideration of a gastric sleeve operation.  The doctor refused the surgery for two reasons. 1) a co morbidity of chronic bone marrow failure 2) the Prader Willi Syndrome is a contraindication for surgery.

I was extremely disappointed.

I am continuously hungry and never know when I've had enough to eat. If I'm eating with other people I often feel envious when they say things like" I'm so full I couldn't eat another thing".  I've never had that sensation and I always feel like I want more.

I'm starting to get weight related problems such as a cartilage tear and arthritis in my hip (ortho won't operate because of co morbidity) and I'm on spondylolisthesis no3. First was L5-S1 then L4-L5 now L3-L4. Ive also had a crush fracture T3-4 and now get thoracic back pain due to heavy breasts.

All in all, I feel desperate.  In the past I've managed to lose weight lots of times by not eating. It's easier for me not to eat at all rather than eat small amounts but I realize that this is unhealthy and I can't keep that weight off when I start eating again.

Do you know of any strategies to help with long term weight loss bearing in mind I have a continuous grhelin production.

I am wheelchair bound and can no longer exercise due to a demyelinating myelopathy.

Thanks for any help you can provide.

Regards

Chirley

[Vera Ingrid Tarman, MD]

You have a rare genetic disorder. As you may know, Prader-Willi Syndrome, can effect the body on many levels - structurally, cognitively and behaviourally.

The hallmark feature of Prader-Willi - which you have described yourself,  is the chronic feeling of hunger.  This can be very distressing: No matter how much you eat, you never feel full .... So you always want to eat. The reason for this insatiable hunger is still  unclear. Scientists believe there is a hormonal irregularity of our hunger and satiety hormones (such as insulin, gherlin, leptin) that control our appetite. These hormonal imbalances are due to an abnormality in the hypothalamus of the brain. The end result is morbid obesity.

This is not the same condition as the more common disease of hypothyroidism, which can also lead to weight gain and obesity. Hypothyroidism does not create this characteristic feature of never ending hunger. Nor is this food addiction. Food addiction does create an insatiable drive to eat — but this not due to a hormonal imbalance but a neurochemical imbalance (such as dopamine and endorphins — our 'happy chemicals'). The person wants to eat specific foods such as sugars, starches, and fats because they give comfort and a feeling of wellbeing, not because they still feel hungry. Food addiction is not about being hungry but about how to feel good, or better or just normal again.

If you find it easier 'not to eat at all rather than small amounts of food' as you have indicated, have you considered that you might be also / instead a food addict? One way of know this is if you experience cravings after the first 'bite' of a trigger food. In other words, when you start eating particular foods (esp something sweet), do you find that your desire for the food increases even more than before you started eating? This indicates that a craving has been kindled from the food itself. This is most likely to occur if the food is sugar, starch, bread, pasta, candy (rather than vegetables and proteins).

If this is the case, then I would recommend that you do not eat sugar or refined starches. They are not necessary for a healthy diet and for a food addict. These foods  can be a deadly trigger that will cause relapse after relapse for a food addict, into depression, despair, weight gain, diabetes and high blood pressure. My basic strategy for a food addict would be to stick to the healthy foods such as vegetables and proteins — and avoid sugar and its many manifestations as if it were a drug of abuse.  

See my webpage: to see if you qualify as a food addict.
http://addictionsunplugged.com/2011/03/30/are-you-a-food-addict/

The website also gives lots of information about food addiction and what to do about it.

Hope this helps!

[teenyturner]

Thank you very much for your response.

Just like most people I do like chocolate, ice cream and cake but these don't dominate my diet.

I find that im happy to eat meat and three vegetables but I could eat lots and lots of them.  In fact if I crave any one thing it would more likely to be salty or savory food but that's just because it's my taste in food.

For instance, for lunch today, I've had a corn meat and salad sandwich for lunch on wholegrain bread but I could easily eat four of them and not stop feeling hungry.

Tonight I'm planning on having baked skinless chicken thighs with a vegetable bake and home made potato chips but I will want lots and lots and even then I could eat a lot more.

It's really horrible and i have to exercise extreme willpower not to give in. I'm only just in the obese range and vary between overweight and obese on a continuing basis which is considered very good for someone with Prader Willi but it takes so much effort to not let my weight blow out that sometimes I don't think I have the inner strength.

I was wondering if hypnosis will help.

Regards

Chirley

[livewithcare]

Please call Prader-Willi Syndrome Association (USA) at 941.312.0400 or toll-free in the United States at 800.926.4797. This is a rare syndrome that needs support from those very knowledgable about it, which you will find at PWSA (USA). There are many considerations about your diagnosis which will be important for you to know and there are strategies and people who can help you. Those giving advice who are unfamiliar with the complexities of the syndrome may be well-intentioned, but misinformed. You can also visit www.pwsausa.org. Please note there is also the International Prader-Willi Syndrome Organization (IPWSO) and there are Prader-Willi Syndrome Association organizations in many countries throughout the world (if you are not from the states). Many blessings to you and all who live with PWS.

[Vera Ingrid Tarman, MD]

I am not an expert in Prader Willi Syndrome, so I cant answer from that perspective. I think that the post from 'live with care' has given you some good resources.

If you were a food addict, I would sugget that you cut out the carbs ie the bread and the chips and stick only to proteins and the green vegetables. Food addicts also have to weight and measure their foods so that they are ensured that they will eat enough food, not more and not less either.

I dont know how to advise you within the Prader Willi context. While there may be some similiarities, it would be best to consult a practioner with that expertise.