A Letter of Thanks

Dear ...

I am sorry to have forgotten your name.  I expect you have also forgotten mine.  Although perhaps a fragment of your recollection of me remains somewhere in your memory, filed under "unreasonable people".

You visited my house when my son, who has PWS, was 2.  You came at my invitation to conduct a speech and language therapy assessment on my son.  You were friendly and polite. I was delighted to see you as up to that point I had struggled to source speech and language therapy. 

Everything went smoothly until you casually mentioned that you would like to observe my son eating and you produced a rice cake from your bag.  I immediately intervened and said that, unfortunately, my son could not participate in this part of the assessment right now as it was not his meal time and he did not eat outside mealtimes.  

You were taken aback.  You pointed out that rice cakes are healthy.  You said that he would only need to take a few bites.  You argued that he would soon forget one break in his routine.  You said you were sorry, but that you could not wait until his next mealtime as you were due to see other clients.

I said that I was sorry for any inconvenience caused.  But I also refused to give in.  I told you that I felt it was very important to have a fixed meal regime in a house where there was a person who had PWS.  You became exasperated.  Your frustration was obvious.  Eventually you left.  I don't recall if that part of the assessment was conducted at another point or if it was simply omitted.

That happened over 8 years ago.  Everything you said on that day was correct.  I can understand why you were frustrated and why you might have found me unreasonable.  I can laugh now at how I must have appeared to you. I imagine you viewed the appointment as a failure.

Yet I consider it to have been a huge success.  Why?  Because it was the first occasion when I came under pressure to manage my son's diet differently to what I believed to be in his best interests.  It was the first of many occasions when someone else wanted him to have food that I did not want him to have.  This appointment provided me with an opportunity to test my commitment to standing firm in the face of pressure from someone in a position of authority over me.  And I did stand firm.  Having withstood the pressure from you, I became more confident and gained the courage to continue to resist pressure and to take full responsibility for my son's welfare myself.  

Eight years later my 10-year-old son is thin and healthy and we still have a fixed meal routine. He has grown up to understand that we do not eat outside of meal times and we do not eat spontaneously regardless of who suggests we should.  He also understands that if his Mom or Dad makes a rule it will be enforced.

So, genuinely, I am grateful to you.  Even though it was an unintended consequence of your visit, that appointment helped us more than you will ever know.

 Yours... 

PS: My son's speech is excellent now!    

Stomach Pain in PWS can be Life-threatening

The article written by  special teacher  Lene  Steensen, caregiver  and  personal  assistant   for a   young man with PWS at  “ Living  with Support” a special living facility in Holbæk, Denmark, is translated by Susanne Blichfeldt, MD, Denmark (who has known the young man since he was 12 years old, and has held training courses for his staff). 

The story demonstrates how a caregivers knowledge, together with written information about PWS (a medical alert booklet) can be lifesaving, and also that you have to insist, if you a not listened to.

Not all medical persons know about PWS.

Stomach pain in PWS can be caused by life threatening conditions

by Lene Steensen

 

“Live with support” is a house in Holbæk, Denmark, where a young man 38 years old  (here called J) with PWS is living. I am his personal assistant. He has his own flat in a so-called “group community”.  He has help and support daily especially concerning food and finances.

Last autumn it happened that J, during a Sunday night, experienced pain in his stomach.  J called a doctor himself (there are no staff on during the night). A doctor came, and concluded that the pain was due to a non-serious infection, gastro enteritis. When my colleague arrived on Monday morning, J was still complaining about pain, she called a doctor who referred him to the local Hospital in Holbæk. Here they said that the pain was caused by a non-serious infection, J  had blood tests that showed signs of a possible infection and he was given penicillin, and sent home. J  later said that perhaps the medication helped a bit.. When they looked at his abdomen, they did not find anything alarming, when they touched him/ area of the stomach J only complained very little.   As it was my day off, I came to work on Tuesday and was told the story.

We have participated in many courses about PWS, and here through fantastic  speakers we have been told how important it is that we react and insist, that the persons with PWS that we are responsible for have a high pain threshold, and need  very careful investigation when they have pain.

I called his GP doctor, as I did not understand why they had not made a scan of his abdomen as he has PWS. The GP had not received the papers from the hospital yet. The GP called the hospital.

Now it was lunch time and  J had not eaten his lunch.  By now, all alarm bells were ringing, so I called his GP again, who at that time had closed for lunch, then I called the hospital They (staff) would not admit J without a call from his GP 

So--- if I insisted for a referral to hospital the only possibility was to call 122 (like 911 in the USA). I finally did that, and J was able to walk himself to the ambulance!   I tried to explain that I had to call an ambulance as the man, J, had PWS, he had stomach pain and did not eat his lunch!

In the hospital they investigated/touched his abdomen and he complained a bit. They repeated the diagnoses of gastroenteritis, but I refused to take J home before a scan was performed. I had brochures and booklets about PWS with me, and showed the text every time the doctors said something. I think they finally decided for an Ultra Sound, just to “close my mouth”. 

When they had performed the scan they were in doubt about the size of the gall bladder and they decided for a CT scan. This showed air in the peritoneum, a sign of stomach or intestine perforation. They decided now for more investigations. J  had a tube through the nose to the stomach and all from there was “sucked up”, and he was not allowed to eat and drink. They said it was not an emergency situation, and he had to wait until the next day’s afternoon for a gastroscopy (where you look into the stomach with a so called binocular investigation).

Image

Here (with gastroscopy)  they saw he had a hole in his stomach wall close to where the intestine starts.  He ended up having an operation,  resulting in a very long scar with 25 clamps. They sewed up the hole in his abdominal wall. He did very well, was very brave, and finally had something to eat for the first time the following Saturday (the last time was Tuesday morning).  He was sent home on Monday, after 6 days in hospital.

It was Dr Hanne Hove, at the PWS Clinic at Centre for rare diseases at Rigshospitalet in Copenhagen,

where J goes for regular visits, who asked me to write this article, as it shows how important it is that the staff is educated and continuously updated about the special symptoms that can be seen with our clients with PWS.

It is also important that we dare to insist to the medical staff and doctors, who know more about medical conditions (than me) but perhaps sometimes they do not know all about PWS.

This story had a happy ending, but if I had accepted the diagnoses of gastroenteritis, and had not insisted, for a scan, it could have ended in another way.  A hole in the stomach can be life threatening.

Lene Steensen

Caregiver,  for J, PWS, Holbæk, Denmark

Dr Susanne Blichfeldt  comments:

This is a story about “hole in the stomach”. But not caused by overeating (binge eating). Probably there was an ulcer with perforation. The story tells how important it is that staff is well educated about PWS and possible diseases and symptoms

 This article was published recently in the Danish PWS –newsletter: PWS-NYT.