By Linda
Thornton
(please note I am not a physician; I am a parent whose daughter with PWS has had some severe gastrointestinal problems which some of you may also have experienced or been unsure about)
In 1999
The Gathered View included an article about the discovery by PWS specialist Rob
Wharton, M.D., of what he termed Acute Idiopathic Gastric Dilation. What Dr Wharton saw in his patient was that for some unknown (idiopathic) reason the
stomach (gastric) was quickly (acute) pushed out (distended), causing the
stomach tissues to die. If not immediately treated with surgery, this condition
may lead to death.
Recently
a great deal has been written about the issues around GI problems.
This is something that as a parent, I read
and thought, “well, I’ve not seen any of this, so we’ll be fine”.
How wrong was I?
It’s not an easy topic to discuss among
friends… (“well, now, tell me how many times do you poo a day and what do these
look like?” is the best starter, but probably not a conversation-opener for
many.)
I have always checked to see that my daughter is at least
regular in her bowel movements and it’s always seemed so. Looking back, and hindsight is a wonderful
thing, I realise that she has had many bouts of diarrhoea which I put down to food
that she had obviously stolen, or secreted away somewhere and eaten too much of,
or she hadn’t been washing her hands thoroughly and has caught a bug.
After all, we’ve always been told that people with PWS don’t
vomit and that if there is a bug going around, it’s more likely to present as
diarrhoea. My
daughter is now 31, and I started to notice the trend for diarrhoea about 10
years ago now. And as I say, I always
just put this down to her theft of foodstuffs.
I think I was wrong.
The PWS Diet
Diet in PWS has always been a corner-stone of how we manage
the syndrome.
We’ve been told for years
that the best way to help a person with PWS is to bulk up the meals, to add
high-fibre foods that take longer to digest and stay in the tummy longer, thus
keeping the notion of being ‘full’ more pronounced.
So, over the years I’ve happily watched my
daughter eat the cores of apples (only the stalk was thrown away), the skins of
Kiwi fruit, the gristle from chicken bones, nuts, seeds, berries, and I’ve
loaded her plate with coleslaw, salads, tomatoes and similar.
I’ve been delighted at her ability to
cheerfully eat broccoli stalks, the thick part of a cabbage, carrots, and other
raw vegetables and fruits.
I’ve counted calories, I’ve placed high-fibre foods in front
of her all the time thinking I was doing the right thing. Willingly she has eaten the skins, stalks, seeds, pips, of just about anything I have put in front of her, raw, or cooked.
I think I was wrong.
Suddenly
in the past year, she has developed very scary acute abdominal pain. So bad that she had more than once taken herself off to the
Emergency Department of the local hospital – along with a staff person carrying
all relevant information about PWS. She
has spent hours in the waiting room, patiently and painfully wanting to see a
doctor. She has been admitted overnight
on several occasions and tests done for appendix, constipation, and various
other extraneous things. All of which
have had no conclusion and all of which resulted in her being discharged from
hospital, mostly with painkillers and laxatives. On one occasion she was crying out for
morphine to help with the pain, and this was put down to ‘attention-seeking’. It didn’t matter how much information on PWS
was taken for doctors to see, it simply was skimmed over, or disregarded. Both her staff person and, on the occasions
when I travelled down be with her, were ignored. It is the greatest put-down you can
experience… that knowing look from the medical expert when you try to tell them
about PWS. They’re not interested. They are merely the ambulance at the bottom
of the cliff.
However, after each time at the hospital things looked a
little better and the laxatives did their job.
So back onto the high-fibre diet we went.
I now know I was wrong.
The first time she told me she had vomited, I thought to myself, “she’s
exaggerating, she’s never vomited before, why should she start now?” It was a cup of coffee, last thing at
night. Went all over her sofa as it was
so unexpected she really didn’t know what was happening. The second time it happened, she scared
herself badly, that she and her staff person went back to ED. The wait was too long for her to cope with
so, feeling better, she simply left and went home at 3am.
The third time, she was at home with us. She’d been complaining of a stomach ache and
later that evening, she asked me to feel her stomach. It was distended and as hard as a rock. I was very worried, particularly so as we
live way out in the country away from medical help. I put her to bed.
Within an hour she had vomited. Projectile, compulsive, chaotic
vomiting. Fortunately this time she had
made it to the bathroom, but no further than the door. I could see everything she had eaten that
day, nothing seemed digested at all.
This time, I was scared.
The very next day I turned to our experts for help. Asking Janalee Heinemann first, and then
being guided to Dr Jim Loker, I can only say that without this immediate
support and help, we could have been in a far worse position than I ever had
imagined.
The first thing I was advised to do was to get her to a
gastroenterologist for tests for gastroparesis.
Then at the same time I was to reduce her meals to 6 small (palm size) per
day and to make these low-fibre. She was
not to eat meat, skins of fruit or vegetables, grain bread, salads, tomatoes, onions, anything from the cabbage family, or
anything with pips which could stick in the lining of the stomach.
Within
hours, I had found a paper by Kate Beaver RN, and Barb Dorn, RN CNN, with a
menu suitable for gastroparesis in PWS.
I am so grateful that this had already been done! So together with Jim Loker’s dietary advice,
I mapped out a basic plan for the new
diet. The staff were fantastic and immediately
put this into action, discarding from the cupboards and fridge all the
foodstuffs she could no longer have.
What causes
Gastroparesis
How on earth had this happened? Surely I’d followed the rules? Surely I’d understood about PWS and the
diet’s special needs – after all, I’d run workshops on this very topic many
times in the past, encouraging high-fibre foods, and foods slow to digest in
the stomach.
How could I be so wrong?
It seems there are many contributing factors which add to
the misery of PWS. Kate Beaver, MSW, CSW, and Barb
Dorn, RN, BSN cite in their paper:
Risk factors for Gastroparesis
|
Risk Factors Seen in Persons with PWS
|
Diabetes – most common cause
|
Diabetes – many persons with PWS have diabetes.
|
Adrenal and thyroid gland problems
|
Many persons with PWS have been found to have low functioning of their
thyroid gland.
|
Certain drugs weaken the stomach – many antidepressants and heart
medications
|
Many persons with PWS take antidepressant medications as part of
behavior management and some may be taking heart medications.
|
Neurologic or brain disorders such as Parkinson’s, stroke and brain
injury
|
We continue to learn the effects of PWS on brain functioning.
|
There was an immediate result with the small meals. Well, two, if you like. The first was that her ability to digest the
smaller amounts was much, much easier.
The second was that she thoroughly enjoyed having these snack-meals, “I
feel like I’m eating all day!” and it kept her bright and happy.
Convincing the
doctors
I still needed to convince the doctors. I’d already convinced myself, and her staff
were grateful that something positive was forthcoming and this could be the end
of the midnight sieges of the Emergency Department.
So I made an appointment with a dietician at the hospital,
and another with her GP. The
dietician was dubious.
“I think you
should stick to the same diet as before.
We don’t know whether your guess at gastroparesis is the right one.” She was right about one thing; we didn't know whether gastroparesis was the right diagnosis, but entirely wrong about sticking to the former diet.
Next appointment, the same day, was with her GP. I asked the staff person to keep my daughter
occupied while I did this appointment alone.
I had taken with me the GI papers from Ann Scheiman and the latest one
from Barb Dorn and Kate Beaver. I sat
down and quietly told the doctor that I didn’t plan to leave his office until
he had listened to me. I said I didn’t
need a diagnosis of “over-anxious mother” as I already had that, but I really
and truly just wanted someone to listen.
Younger than me (they all are, these days), he assessed the
situation and probably decided it would be quicker if he listened! He wrote notes, he read the papers. I sat there and waited. He looked up and said, “I believe you are on
to something. We need to organise a gastroenterologist
appointment. We need to rule out this for a start."
I could have kissed him.
I asked whether I could wait until he’d confirmed the gastro
appointment as I had to travel some distance home and wanted to have things
sorted before I left. He said I should
hear within the next 4-5 days and to keep up with the new diet. It seemed so much better than just a few days
before.
I waited the 4-5 days – just to be polite – before I rang
the hospital gastroenterologist department and asked when I would be hearing
about my daughter’s appointment. The
nurse searched the waiting list and said it would be about 3 months. I cried.
She listened. She said she would
talk to the doctors and see whether my daughter could be squeezed in.
I waited the obligatory 2 days and phoned again. Braver this time. The nurse said she was about to call me and
that she had been able to get an appointment within 3 weeks.
I felt as though I was walking on eggshells for those 3 weeks. Among all of us – the staff, my daughter and
myself, we had worked out a menu that was satisfactory (and full of wonderful
stuff, she thought, starting with white bread and ending with custard).
During
this stressful time, she had to move house.
I decided to bring her home so that she wasn’t
living in a house full of boxes and packing strewn all over the place.
This was a good move as I could also see how
the new menu was working out.
It did
mean that I was constantly in the kitchen preparing delicious little mini-meals
for most of the day, but it was worth it.
There were new rules in place:
she was to sit up to the table to eat and not sit tucked up on the sofa.
This was to help with the digestion.
She could stand up to eat, if she wanted.
This is helpful especially after soup as it
doesn’t sit in the stomach so long.
She
was to walk after each little meal, at least 10 minutes which could add up to
an hour’s exercise each day.
A good
start.
Together, and with a staff person, we went to the
gastroenterologist appointment. Three
days before our appointment I wrote to the doctor and sent him the two
attachments which had become like my Ten Commandments. I had carefully couched the email so that it
didn’t sound like the mad mother from hell, but unemotionally and factually
centered just asking him if he could read these as I was not sure how well PWS
was understood in his world.
He was grateful! “I spent my Saturday reading and researching!” Respect!
He listened, asked questions, did a physical examination and then said
he would do a biopsy of the stomach followed by a “radio active scrambled egg
breakfast” to follow the movement through the digestive system.
We started with the biopsy of the stomach – she was
sedated, but not anaesthetised (which was good since she has an appalling
record of coming out of an anaesthetic, fighting), and said she “felt the
camera going down her throat” but thankfully she managed to stay with it.
A couple of weeks later she underwent the gastric emptying study which meant she had no breakfast, arrived at the clinic and was given a small amount of scrambled egg whites, told to wait an hour when she would have another small meal of scrambled egg whites (during this time there were plenty of phone calls to me begging me to allow her to "have something to eat"!) She lasted the distance with great support from her staff.
And now for the interesting part
- The results of the biopsy show no
necrosis.
- There was no evidence of delayed gastric emptying - in fact if anything, the gastric emptying was on the border of slightly rapid emptying.
So just what had been causing all the endless gastric problems that she was suffering for months and months?
A clinical visit to the Gastroenterologist to review the results of her tests brought up the possible prognosis of Irritable Bowel Syndrome (IBS). The Mao Clinic states:
"Irritable bowel syndrome (IBS) is a common disorder that affects the
large intestine (colon). Irritable bowel syndrome commonly causes
cramping, abdominal pain, bloating, gas, diarrhea and constipation. IBS
is a chronic condition that you will need to manage long term."
Wrapping it up
We have looked carefully at the diet, eliminating all high-fibre and wheat. We've eliminated onions, and all the cabbage family as these for years had been causing really revolting wind problems; eliminated all fizzy drinks; kept coffee to a minimum and introduced decaffeinated drinks, we've kept to the 5 small meals a day and very slowly we've re-introduced fruits (peeled), soft vegetables and kept away from high fibre food.
There have been no further visits to the Emergency Department, no vomiting, no constipation and no gut pain. We have decided to follow a diet which will manage the IBS condition.
I know
this is no dinner-party topic, but I thought I would blog it just in case you
notice a change in your son or daughter’s gut habits and to look at the amount
of high-fibre we use in their diets. It’s
not gastroparesis, but it is definitely a part of what is a critical piece of
the intestinal workings for all of us.
Read "Rethinking the PWS Diet" by Barb Dorn, RN and Kate Beaver, MSW, CSW. On the same link, read more about Constipation in PWS, and Gastroparesis.
Read more about gastrointestinal problems in PWS including constipation
