June 2008 - Prader-Willi Syndrome Association (South Africa)

Volume 11 Issue 2 June 2008
Prader-Willi Syndrome Association of South Africa
PO Box 2399 Brooklyn Square 0075
Nonprofit Organisation No. 035-837-NPO
PBO Exemption No. 930 016 853
Newsletter for parents by parents Website: www.praderwilli.org.za
Chairperson
Rika du Plooy
012 344 0241
rikadup@mweb.co.za
Secretary
Wilna Basson
bassons@iafrica.com
Medical Advisor
Dr Engela Honey
012 319 2269
ehoney@medic.up.ac.za
From the Chairperson / Van die
Voorsitter p1
Meet the Senne family p5
Skin picking p7
Locking p10
Beyond the diet p11
Growth hormone p14
What are febrile convulsions p15
Recipes p18
Fun pages p21
FROM THE CHAIRPERSON
Dear Readers
Rising petrol prices and interest rates are but
two realities. Another important reality is the
Prader-Willi syndrome which faces our
parents each and every day. I trust that this
newsletter contains something worthwhile
and useful for every family member. Enjoy
reading it! Continue next page
What is Prader-Willi Syndrome?
Genetic disorder
Low muscle tone
Short stature
Obesity
Increased appetite
People With Strength Volume 11 Issue 2 Page 1

I would like to introduce you to the Senne family. Their home language is Tswana but their
contribution was written in English by Welhemina. She went to much trouble in doing it. She
shares her worries and love for Metsiatsile with us. This family joined our association in 2006
and they also attended the past two annual general meetings. This makes one wonder. There
must be more black families affected by the Prader-Willi syndrome – but where are they?
Skin picking is common among people with PWS. Parents have a hard time controlling it.
Parents should read the article Skin Picking and Prader-Willi Syndrome in this issue. Many
thanks to PWSA (USA) who gave us permission to reprint this and other articles from their
newsletter, The Gathered View.
Our newsletter carried several articles on food security and its importance in controlling the
weight of a person with PWS. Locking fridges, freezers and food cupboards may sound cruel
to outside people, but it is but one of the strategies to prevent individuals with PWS from
getting into temptation. It remains a difficult task to control the food environment of people with
PWS, however it is possible at home by taking some “drastic” measures. Please read the
article by Allen Heinemann. He is Matt‟s dad. Matt was indeed relieved when his access to the
fridge was barred! In the same vein is the article by Janalee Heinemann. It illustrates the
importance of this kind of control. Our readers are invited to share their experiences with us. It
is hard to get definite solutions or answers, but learning from each other may be just the thing!
Please take note of the advice on precautions to be taken when starting with growth hormone
therapy. This information was provided by professional people who serve on the scientific and
clinical advisory board of the PWSA (USA).
Our previous two newsletters contained information about books and DVD‟s which can be very
helpful. We didn‟t get much response from our readers. I know that the time of our parents is
very limited. They even might not have the strength or energy left to enquire about information
which appeared in our newsletter. The DVD, Behaviour, Food and Beyond, was used by a
school in Bloemfontein and they found it most useful. Read the feedback received from a
teacher. The PWSA (SA) acquired resources to assist our members and I encourage you to
make use of this opportunity. It might just help to manage the problem better!
Enquiries to our association increased. Somebody enquired about febrile convulsions. Dr.
Engela Honey responded to this question and we appreciate her ongoing involvement. Dr
Honey is always available for enquiries or consultations.
I was quite surprised to find the first notice of the 7th International Prader-Willi Syndrome
Conference in the post box. It will be held in 2010 in Taiwan. The PWS association in Taiwan
was founded in 2005 and they are indeed on the move! The notice is a friendly invitation to all
to attend the conference in Taipei.
There is some improvement in Pam Eisen‟s condition. She is the president of IPWSO. Pam
still receives chemotherapy, and she was fortunate to travel in order to visit her children.
The winter holidays are around the corner. The Bassons gives the children something to do
with their FUN pages. Also included are some recipes from Weigh-Less – how to make a
delicious tuna sandwich, tomato and roasted red pepper soup and French toast for breakfast.
Try it!
Other important bits will be found all over in this newsletter. Please take note of the annual
general meeting on 24 August 2008.
Thank you to all the co-workers!
Winter greetings
Rika du Plooy
People With Strength Volume 11 Issue 2 Page 2

Beste Lesers
Te midde van stygende petrolpryse en rentekoerse bly die Prader-Willi-sindroom „n
werklikheid in ons lewens waarmee ouers daagliks mee moet rekening hou. Ek vertrou dat
daar in hierdie nuusbrief vir elke gesin iets waardevols en bruikbaar sal wees. Lekker lees!
Die Senne gesin van Rustenburg word aan ons voorgestel. Hulle huistaal is Tswana en
ons is dankbaar teenoor Welhemina wat dit in Engels geskryf het en hiermee moeite
gedoen het. Sy deel ook met ons haar bekommernisse en haar liefde vir Metsiatsile.
Hierdie gesin het in 2006 by die Vereniging aangesluit en was teenwoordig by die afgelope
twee Algemene Jaarvergaderings. Dit laat „n mens noodwendig wonder – daar moet tog
ook ander swart gesinne wees wat deur die sindroom geaffekteer is?
Velkrapgedrag by Prader-Willi-sindroom kom algemeen in verskillende grade voor en is vir
ouers „n kopseer, veral in die lig daarvan dat dit baie moeilik is om hierdie gewoonte te
beheer. „n Baie onlangse en insiggewende artikel Skin Picking and Prader-Willi Syndrome
verskyn in hierdie uitgawe en ons dank aan The Gathered View, nuusbrief van die
PWSA(USA) vir toestemming om hierdie artikel en ander in hierdie uitgawe te plaas.
Daar het al in People With Strength artikels verskyn oor food security en die belang
daarvan in die bestuur van gewigsbeheer by die person met die sindroom. Om koskaste
en yskaste te sluit, klink vir ouers en die wêreld daar buite baie wreed, maar dit is een van
die strategieë om vir die individu met PWS die versekering te gee dat daar nie versoekings
is nie! Dit bly byna „n onbegonne taak om die kos-omgewing van die individu ten volle te
beheer, maar dit is wel in die huis moontlik!
Lees gerus die artikel deur Allen Heinemann – hy is die pa van Matt, „n volwasse jongman
wat verlig was toe hy nie meer toegang tot die yskas gehad het nie! Aansluitend hierby is
daar ook „n artikel deur Janalee Heinemann waarin dit baie duidelik uitkom hoe
noodsaaklik hierdie beheer is. Is daar dalk van ons lesers wat hulle ervarings met ons wil
deel? Klinkklare oplossings is daar nie, maar ons kan bymekaar leer!
Neem ook kennis van die advies oor voorsorgmaatreëls wat in aggeneem moet word
voordat met groeihormoonterapie begin word. Hierdie inligting word verskaf deur
professionele persone wat almal op die adviesraad van die PWSA (USA) dien.
In die vorige twee uitgawes van People With Strength is daar inligting geplaas van
hulpmiddele wat vir ouers baie waardevol kan wees. Van die lesers was daar weinig
reaksie – ek verstaan dat die lewe soos dit is baie van ouers eis en dat ouers gewoon dalk
net nie die energie en tyd het om nog navrae te doen oor inligting wat in People With
Strength verskyn nie. Die DVD Behaviour, Food and Beyond is deur „n skool in
Bloemfontein gebruik, wat dit baie waardevol gevind het. Lees wat die terugvoer van „n
onderwyseres daaroor is. Ouers word aangemoegdig om hieraan aandag to gee – dit kan
dalk net help om die probleem beter te verstaan!
People With Strength Volume 11 Issue 2 Page 3

Navrae wat aan die Vereniging gerig word neem toe – so was daar „n navraag oor
koorskonvulsies. Lees gerus dr. Engela Honey se antwoord hierop. Lede moet onthou dat
dr. Honey altyd vir navrae of „n konsultasie beskikbaar is.
Groot was die verrassing toe die eerste kennisgewing van die 7de Internasionale Prader-
Willi-Sindroom Konferensie wat in 2010 in Taiwan gehou gaan word, in die pos is. Hierdie
Vereniging wat in 2005 gestig is laat nie op hulle wag nie! Dit is „n vriendelike
kennisgewing wat almal uitnooi om hierdie konferensie in Taipei by te woon.
Ek is dankbaar om verslag te doen dat dit beter gaan as wat daar verwag is, met Pam
Eisen, die President van IPWSO. Sy kry nog chemoterapie en was so tussen alles deur
instaat om te reis en by haar kinders te gaan kuier.
„n Skoolvakansie is om die draai – die Bassons se PRET-bladsye is weer eens daar!
Verder is daar idees vanaf Weigh-Less vir „n heerlike tunabroodjie, sop en fransebraaibrood
vir ontbyt – dit maak mens beslis lus om dit uit te probeer!
Verskeie brokkies inligting word geplaas. Neem ook kennis van die Algemene
Jaarvergadering wat vir 24 Augustus 2008 beplan word.
Dankie aan al die mede-werkers!
Wintergroete
Rika du Plooy.
Permission is granted to reprint the article on Skin Picking and
the Prader-Willi Syndrome and Precautions Adviced When
Starting Growth Hormone With PWS.
The two articles are from The Gathered View March-April 2008
edition, newsletter of
Prader-Willi Syndrome Association (USA)
8588 Potter Park Drive, Suite 500
Sarasota, FLORIDA 34238 - USA
www.pwsausa.org
People With Strength Volume 11 Issue 2 Page 4

MEET THE SENNE FAMILY
Written by Welhemina Senne,
mother of Metsiatsile, Boitshoko and Amogetlang
We are a family of five and we are Tswana
speaking. Metsiatsile is our first child and is born
on 12 September 1998 at the Jubilee Hospital in
Hammanskraal, north of Pretoria. It was a
normal birth and Metsiatsile weighed 3.5 kg. I
realised there was a major problem – my baby
was not crying, looked floppy and was only
sleeping. I was worried and the doctors decided
to run some tests. My baby could not suck and
therefore I was not able to breast feed her. It
was so painful to see my baby suffer. The
doctors at Jubilee Hospital kept us at the
hospital and the baby was put on tube feeding.
The doctors couldn‟t understand what was
wrong and after a week at Jubilee Hospital we
were transferred to Kalafong Hospital in
Attridgeville, Pretoria. We were admitted for one
month while doctors were trying to find out what
was wrong.
After that we went for monthly check ups, and
during that time some tests were done by Dr Engela Honey and the results
showed that Metsiatsile had the disease called Glutaric aciduria type ll which
falls under genetic disorders. The painful thing about that disease was that
they don‟t know what caused it, and they predicted that my child would not be
able to talk or walk. Metsiatsile was put on a medication called L-carnatine
and a multivitamin syrup, which she used for four years. At the same time
there were two other children in the Pretoria Academic Hospital with the same
problem of which Dr Honey was aware off. I often asked about the progress of
those children and then Dr
Metsiatsile and her brother,
Boitshoko at the AGM 2006
Honey told me that there was
no progress compared to
Metsiatsile – by that time
Metsiatsile was already walking
(at one year and eight months).
Metsiatsile received
occupational therapy and we
are still visiting the speech
therapist.
So I told Dr Honey to run some
tests to check again – maybe
the first diagnoses was wrong.
Urine tests were done which
showed nothing. Eventually
People With Strength Volume 11 Issue 2 Page 5
Metsiatsile Senne will
be 10 years
September 2008

after blood tests the diagnosis of Prader-Willi syndrome was made in August
2005. Strangely, another thing that made me suspects that something is
wrong was the fact that Metsiatsile was eating too much and started to gain
weight.
Dr Honey suggested that we visit a dietician who gave us an eating plan,
which I liked and worked for Metsiatsile. Part of Metsiatsile‟s daily routine is
running 100 meters and doing other exercises to keep her body fit. Dr Honey
helped me so much, I love her and I can cope with the Prader-Willi syndrome
problem. I also joined the Prader-Willi Syndrome support group in 2006 and
the advice and counselling helped me so much.
Metsiatsile is at the Iteko Special School
(Tlhabane Rustenburg) and is coping though. The
issue concerning food is also hard for Metsiatsile.
My family loves her and they enjoy her company.
She has two grandmothers and one grandfather.
There are two siblings, Boitshoko who is 5 years
old and the baby brother Amogetlang, who is 6
months old. They love their sister and Boitshoko
takes care of Metsiatsile when they are playing
with other children. Metsiatsile loves people and I
take her everywhere I go – I love her so much and
I do not want her to be hurt. I will take care of her
as long as I live. It is God‟s gift and we know we
are not perfect. Let‟s accept everything the way it
comes because Jehovah God loves us. Let us
enjoy every moment with our children with Prader-
Willi syndrome.§
Wilhemina with Amogelang,
5 months, their 3 rd child
Die AJV word DV gehou op Sondag 24
Augustus 2008
in Pretoria
Our AGM will be held on
24 th August 2008
in Pretoria
People With Strength Volume 11 Issue 2 Page 6

Medical View
Skin Picking and Prader-Willi Syndrome
The “skin picking” behavior of PWS has a wide range of severity across the
syndrome and sometimes in the same person over time. As Wigren reported in 1999,
stability over time is more typical. Some have occasional minor skin picking while
others maintain large open wounds. In the “PWS Personality” presented in 2006, we
separated skin picking as a habit behavior (common) from self mutilation associated
with extreme emotional distress (less common). Here we will only address the former
and leave the latter and rectal picking for another day.
Why do they pick? Some speculation.
Much of PWS behavior makes more sense when viewed as a failure to inhibit. The
eating behavior is largely due to defective “brakes” called satiety. The drive to skin
pick in PWS may be a normal drive (who has not picked a scab?), but the limiting
signals are weakened. We speculate that these signals are pain and disgust; both
are neurologically based but apparently reduced in PWS.
Skin picking as a habit behavior
Here, skin picking is defined as an activity that has no apparent function. It goes on
when the person is calm and it does not appear to be causing any emotional distress.
It has been related to boredom and anxiety, but objective evidence has been difficult
to establish. It is characterized by opportunistic topography,i.e., convenient location.
Features include:
1. Arms, face, scalp
2. Nose, nasal septum
3. Nail cuticles
4. Pulling out toenails, teeth
5. Peeling skin from soles of feet
No specific intervention has been uniformly effective. The behavior often
extinguishes if wound healing is achieved. Success has been limited using protective
dressings (Band-Aids) and an intense program of alternative activity until wound
healing occurs. Behavioral interventions have been effective in some cases (see box
on next page ).
Points on management
Because skin-picking behavior occurs intermittently and secretively, behavioral
interventions targeting the activity itself are difficult to implement. A basic principle is
that no attention (positive or negative) should be paid to the behavior itself other than
to require the person to observe social conventions and good hygiene.
Obsessional, but not OCD
The behavior appears to be “compulsive,” however, it is not an obsessive-compulsive
behavior, and medications targeting OCD (obsessive-compulsive disorder) or anxiety
have not been helpful. If the behavior is clearly related to other signs of anxiety, then
the anxiety should be addressed with environmental changes, including a reevaluation
of the person‟s food security. Note: Anti-anxiety medications, while helpful
for anxiety, carry the risk of increasing irritability or triggering mood activation
(hypomania, irritability, increased impulsivity, restlessness, and increased goaldirected
behavior including food seeking). Mood activation can begin weeks or
months after medication has been started even when effective for reducing anxiety.
Use of Topiramate
Topiramate (Topamax) in low doses has been effective for some people and should
be consideredin those with severe picking. In 2002 Shapira1 and others gradually
increased to 150-200 mg daily and reported that some patients responded and some
did not. Side effects include irritability, cognitive blunting, and RTA (renal tubular
acidosis), all dose dependent and reversible. RTA is diagnosed when serum
People With Strength Volume 11 Issue 2 Page 7

electrolytes show an elevated chloride and decreased bicarbonate in the blood.
These issues should not deter a trial of the medication, but provide a guide for what
the physician should monitor. If lesions heal, a trial off the medication makes sense,
since healed lesions are often left alone. Allow 2-3 months on the medication to
evaluate efficacy. Anecdotally, sensory stimulation has been quite effective for some
severe picking behaviors. Sensory modalities have included vibration or massage
administered on a schedule multiple times per day. The sensory stimulation should
not be linked verbally or temporally with the picking behavior, as this could result in
rewarding the behavior.
More information on using sensory integration techniques has been assembled by
Janice Agarwal, P.T. and is available on www.pwsausa.org. 1 Shapira, N.A., Lessig,
M.A., Lewis, M.H., Goodman, W.K. and Driscoll, D.J.: Effects of Topiramate in Adults
with Prader-Willi Syndrome. Am. J. Mental Retardation, 109:301-9, 2004. §
A Program To Address Typical Skin Picking
Pittsburgh Partnership
Linda M. Gourash, M.D. Janice L. Forster, M.D.
1. Tell the child that you want to help her sore to heal. Do not talk about the “picking.”
2. Tell him that when the sore heals you will celebrate with a special reward.
� This reward should be motivating but not too motivating; rewards that are too
motivating can create anxiety. Also there is the risk that an older, more clever
individual will deliberately create wounds to heal to earn the reward.
� Be prepared to offer the same reward on multiple occasions. * Try to anticipate
situations when picking tends to occur. Before he starts to pick, remind him he
is working to gain this reward.
� Once the reward is earned, offer it weekly (for very young or low functioning
children or for severe pickers) or monthly for “no new sores.”
� If a reward must be withheld because of a continued open sore, express your
disappointment that the reward was not earned and your optimism that he can
achieve it very soon. Most superficial sores if truly left alone will show marked
improvement in a matter of 2-3 days. 3. Tell her that you have medicine to help
the sore to heal.
� Plastic surgeons use Polysporin ointment on healing wounds to minimize
scarring. It also keeps the area soft and slippery, apparently less tempting and
less easy to pick.
� Use Polysporin ointment on the sore as frequently as possible (every ½ - 1
hour while awake) and apply thoroughly at bedtime. Use a dressing or some
other barrier where anatomically possible. Your purpose is to make the picking
less convenient.
� The ideal dressing is NOT airtight but is difficult to remove. On arms and legs
this can be gauze wrap covered with cling wrap.
� If there is no evidence of picking during sleep, leave the area open to the air.
Socks or mittens taped at the wrist have been used for nighttime picking. §
People With Strength Volume 11 Issue 2 Page 8

Wound Healing With PWS
How Many Calories?
I am often asked about situations where a person with PWS gains a lot of weight in the
hospital because the dietician states that patient needs to be on a high protein diet to
heal the wounds. A recent example is a 47-yr-old woman (height 4 ft., 9 in.) with two leg
ulcers put on 1,500 calories in the hospital despite the fact that her mom had her on
1,200 calories prior to admission. She was 195 lbs. when she entered the hospital and
gained 40 lbs. during that hospital admission. I asked two members of our PWSA (USA)
Clinical Advisory Board to comment. Their responses follow.
Janalee Heinemann, Director of Research & Medical Affairs
Response 1
People with PWS have healed deep bedsores while on a weight loss diet as low as 800
kcal per day and an exercise program. At no time should a person with PWS be
permitted to gain weight while in the hospital for wound healing or any other reason.
Linda M. Gourash, M.D., Developmental and Behavioral Pediatrics, Pittsburgh Partnership, Pa.
Response 2
For weight loss, I would limit calories to 800-900 per day. This includes any calories
provided from a protein supplement. Novartis (800-333-3785) makes Resource
Beneprotein Instant ProteinPowder. A scoop provides 6 grams protein, 25 calories.The
dietitian can recommend how much, based on baseline albumin and prealbumin level.
Prealbumin should be followed at least once a week while inpatient to help adjust the
Beneprotein additive. Note that other medical circumstances, such as kidney issues, may
negate addition of Beneprotein to the diet. Your medical team will decide if a protein
additive is appropriate. Supplementation with vitamins A, C, and zinc may be helpful with
wound healing of ulcers. The following regimen may be considered: vitamin C: 250 mg
BID; vitamin A: 2500 units/day; zinc: 220 mg/ day; complete multivitamin daily. This
would be given by mouth for 10 days. The dietitian should be sure a calcium supplement
is added to the regimen (especially if the calories are restricted) to ensure bone mineral
deposition. DRI/age for adults is 1300 mg calcium/day.
Norma Terrazas R.D., L.D.,
Pediatric Dietitian, Texas Children's Hospital §
* Wenk: My dogter met PWS het onlangs waterpokkies gehad. Soos almal weet smeer mens
Calamine lotion aan die letsels. My dogter sukkel baie met velkrappery en ek smeer toe
sommer aan hierdie krapplekke ook van die Calamine lotion (met „n oorstokkie). Dit was „n
groot sukses want sy sê dan is die irritasie weg. So nou gebruik ek Calamine lotion vir die
krappery met groot sukses.
Tip: My daughter with PWS recently had chicken pox. As everyone knows Calamine lotion is
applied to the spots. My daughter struggles with skin picking and I decided to apply
Calamine lotion with an ear–bud to these lesions as well. This was a huge success as she says
this takes away the itching. So now I use Calamine lotion for the skin picking with great
success.
People With Strength Volume 11 Issue 2 Page 9

Locking the Cabinets and Refrigerator for
Prader-Willi Syndrome
By Allen Heinemann
Courtesy PWSA(USA)
One of the questions that is often asked as the child with
Prader-Willi syndrome gets older is, “ How can we keep our
child from sneaking into the refrigerator or cupboards and
getting at food?” It‟s hard enough to have to keep food out of
sight and put away, but then to have to constantly keep one eye
toward the kitchen to catch someone before they get into food
can be very stressful.
So it becomes necessary for reducing everyone‟s stress level to
have the food locked up. Through the years, I‟ve seen many
approaches. I‟ve seen plastic chains draped around the entire
body of the refrigerator, bars dropped across the front like you
would see on a Pepsi machine, and even a cement block put in
front of the door making it hard to open. Here are some of the
more common solutions.
1. If your house lends itself structurally to it, a kitchen that
has lockable doors is a real possibility. However, more
and more homes are designed in an open format, (with the kitchen the center
of activity!) and it is not practical to enclose
2. When we needed to lock up the kitchen cabinets, rather than put locks on
every door, we took the area that was normally used for a small breakfast
table, purchased a full sized armoire (clothes cabinet) and used it to store all
our food. It had a double door that one simple hasp and padlock kept secure.
3. Kitchen cabinets are kept locked easily with a hasp and padlock arrangement
that can be purchased at a hardware store. However, flush mounted key
locks look better, but you may need a carpenter to install them.
4. I could find no manufacturer of home refrigerator/freezers that comes
equipped with a lock from the factory or that could be ordered that way. The
only thing I could find were the small hotel refrigerators, the chest style
freezers, or big commercial units that were either freezer or refrigerator only,
that were lockable.
5. A side-by-side refrigerator is the easiest to put a chain and padlock on. Plastic
coated bicycle chain with a combination lock works the best. (see image
below)
6. Standard refrigerators can also be locked with two hasps and padlocks. This
is basically a latch that swings over another looped piece on the door that the
padlock then slips over to secure. The problem you run into is that on some
refrigerators the Freon lines can run close to the surface. Putting screws into
the side can pierce the line and ruin the refrigerator! Another safer approach
is to simply epoxy the hasps to the side of the refrigerator. Both of these
methods can work but are not the prettiest to look at and do deface the
refrigerator.
7. There is another method that doesn‟t do any damage to the refrigerator and
can be done with standard hardware parts. It involves using the extra screws
that come standard with most newer units. If you look close to the handles
between the upper and lower compartments, you will see on the body of the
refrigerator several screw holes, which are normally used if you want to
reverse the doors. By purchasing a small flat metal strip calling a mending
plate (it is a 1/8” thick by ½” wide by 2” long strip with holes on each end), you
can mount it to the refrigerator with a tab sticking out with a hole to put a lock
on. Then take a short length of light swing-set chain and loop it around the
People With Strength Volume 11 Issue 2 Page 10

handles, locking the chain through the hole. If you followed me this far in the
explanation, you won‟t have any trouble installing this system. It‟s main
advantage it that it doesn‟t deface the refrigerator and it is relatively discreet.
I‟m sure there are other methods out there, and if you‟ve got one that works that you
think others might want to try, send it to the national office, and they‟ll get it published
in upcoming newsletters. My son, Matt, who has Prader-Willi, was actually relieved
when we first installed locks. As Matt said, “I try and try, but my hand go in the
refrigerator and I can‟t stop it.” §
BEYOND THE DIET – For The Dietician
By Janalee Heinemann - Prader-Willi Syndrome Association (USA)
From the number of crisis cases that come into our 800-telephone number, I feel the
need to address the most common errors I find in case management of weight
control with PWS and PWS-like children. In most of the cases we get involved with
that are due to the lifethreatening weight of a child, I find first that the physician first
sends the parents to a dietician, then views the parents as failures because the child
continues to gain weight in spite of the dietician’s clinical advice. One child who paid
the ultimate price was Christine Corrigan, the 13-year-old from California who died
weighing 680 pounds. I was involved as an expert witness when her mother was
charged with felony neglect, and the case brought international media attention. One
of the charges was that her mother had not taken her to a physician in a year. But
facts showed that before her final year of life, Christine’s mother had taken her to
both a doctor and nutritionist 90 times. During this time Christine had been put on a
typical diet, and Marlene, Christine’s mother, was admonished because Christine
kept gaining weight. What was not taken into consideration is that it takes far more
than a diet to get weight under control when you are dealing with a child who has
Prader-Willi syndrome or a PWS-like disorder such as hypothalamic obesity.
THE FOLLOWING ARE IMPORTANT REALITIES FOR A DIETICIAN TO
CONSIDER:
How much of a food forager is the child?
Some of our children with PWS are much more creative at getting food than most
people can imagine. One example from hundreds I could give is the boy who would
crawl into his parents‟ bedroom on his hands and knees in the middle of the night. He
would then slip his hand under the mattress directly beneath his mother to get the
kitchen keys she had hidden between the mattress and box spring. After getting food,
he would slip the keys back under the mattress without waking her.
People With Strength Volume 11 Issue 2 Page 11

It took his mother months to figure out the mystery of how he was getting food! Some
children with PWS do not have nearly as strong a food-foraging drive. On the other
hand, if a child keeps gaining weight and the parent states that the child never
sneaks food, do not necessarily believe it. Remember that the hand of a child with
PWS is often quicker than the eye of the parent or teacher.
How many calories does it take for this child gain weight?
Many dieticians put the child on a regular diet of 1,500 calories and then think that
the parents are not being honest when their child continues to gain weight. It is not
unusual to have to put a child or an adult with PWS on as low as 800 calories a day.
For our family, daily weigh-ins were important because Matt could gain five pounds in
a day. It also helped us in our role of playing detective regarding tracing down where
he was getting the forbidden food.
How do you lock up the food?
If the situation requires locks – who is going to show the family how to install and
maintain a locked environment in the home? Many a PWS family has ruined their
refrigerator or freezer by drilling into the Freon coils.
Do you suggest keys and doing careful key checks, or do you recommend using
combination locks or alarms? There is no right answer – but alternatives have to be
weighed, and often a family will need outside assistance to work out the system that
is best for their family. Once, our son Matt was so concerned about his weight gain
that without request, he handed over the hidden key he found that we forgot we had.
Another time, we discovered he could unlock the cabinet and refrigerator locks with a
bobby pin in 60 seconds or less! In another instance, his older brother Tad came
home to find Matt hot and sweaty – and working on unscrewing the last hinge on a
locked door. Tad took Matt with him to buy “Mattproof” locks (hinges with the screws
on the inside). Combination locks (you can buy them in pairs with the same
combination if you‟re locking more than one thing) solve the problem of hiding or
losing keys -- and eliminate the constant searching by the person with PWS for them.
Just make sure it is the type that you can change the combination if your child
mysteriously figures out the combination.
Working with young families
Locking up food is not as awful as it sounds as long as the family keeps a sense of
humor and a sense of compassion for their child, and teaches the same to their other
children. Also, if the family is considering buying a new refrigerator, they may want to
consider a side-by-side model where they can just wrap a lock around both handles.
A family may find it better to consider locks sooner rather than later. Our son was
actually relieved that we put locks on, saying “I try and try, but my hand still reaches
into the refrigerator.” It was nice to be able to go to the bathroom without worrying
about what Matt was doing.
I once received a call from a mother who decided to start locking when her daughter
was 18 or 19. Unfortunately, by then her daughter had a mind of her own and had
just chased two different locksmiths out of the house that week.
If the child is older – Who is in control in the family – the child or the parents?
If it is the child, (especially if the child is older), it will be essential to also involve a
professional who can assist the family with good behavior management skills. As
would be expected, food reduction will temporarily create more acting out, and
parents will need to learn “tough love” skills.
People With Strength Volume 11 Issue 2 Page 12

Who else in the extended family and community needs to be educated?
The parents may not be the problem at all. It may be grandma or the neighbor or the
day care provider who is giving extra food to the child. This list could go on and on,
since food is everywhere in society. Loved ones often think, “Just one cookie won‟t
hurt.” Since feeding our children is typically a sign of love in all societies, and food is
a basic essence of life to exist, it goes against all loving instinct to deprive our
children of food.
I remember one of the many times when Matt was gaining mysteriously. We had
previously educated the pre-school staff and told them we would feed Matt breakfast
at home to spare them the concern of what he could have. But we discovered that
unknown to us the cook had quit, and Matt had convinced the new cook that he was
never fed at home.
What and who make up the parents’ support system?
This is not something a single parent can handle alone without a lot of outside
support – and a critical or too lenient spouse may be worse than no spouse at all
when it comes to reducing or maintaining the child‟s weight.
If the family has several children or few resources – keep the diet simple.
It is unfair to think that you can set up a complicated diet requiring a lot of food
measurements and calculations and expect a parent overwhelmed with other
responsibilities to follow it long term.
If it is a single parent who is poor and has other children, remember to think about
resources. Does the family have a microwave? If so, think of foods in pre-prepared
packages that will work. Seven different Weight Watchers or Lean Cuisine dinners
that the child can choose from (if the calories fit) with a diet Jell-O pre-packaged
dessert may be much more realistic than expecting that a parent will weigh or
measure and cook each meal. It also may prevent conflict. Our children do well with
sameness. Special funding for these pre-packaged meals may need to be
considered, but remember, it is a lot cheaper to supplement the food cost than to pay
for all of the obesity related medical complications the child could potentially acquire.
Remember that diet is a lifetime issue. Make it a diet both parents and child can live
with.
Above all, as a dietician, try to remain patient with the family.
As I stated above, there are significant variances with each child with PWS. Some
will ask for more food and make mild attempts to acquire more food if it is available
but that is all. On the other hand, other children will go to such extent to forage for
food that they will sneak out of the house in the middle of the night, rummage
garbage cans, and con everyone including the bus driver at school into giving them
food. I have had parents out of desperation (more than one) lock their children in the
bedroom at night – then have charges pressed against them for doing so. Before
slapping a parent‟s hand, make sure you are willing to help them with all of the
details, funding, and personnel support needed to follow up with weight control – and
help them again – and again.
Although we feel that with this type of disability a child/ adult‟s right to live outweighs
their right to food and freedom, throughout the constant challenge of weight control,
we need to never lose our compassion.
A young man who had Prader-Willi syndrome, John Simon, wrote “We have a heart,
soul, mind and spirit and need you to understand us. Understanding and caring are
People With Strength Volume 11 Issue 2 Page 13

two of the most important things you can do for us…I could feel sharp teeth tearing at
my stomach like piranhas and still do. I know I need someone to keep the cupboards
locked and I need someone to keep me active and to control my weight. I want to
have some fun in my life…. If the government has money to fund crisis situations why
can they not prevent situations from becoming a crisis? I need full support NOW
before I get into a crisis situation. I want to live and I am sure you want the same for
your child.”
John died shortly after this was written.
We did not find a cure in his lifetime, but I
have promised my own son Matt, who is
27 years old, that in his lifetime we will
find a drug to abate the deadly hunger.
Meanwhile, we need all of the help,
support, and compassion you can muster
as a dietician to make the long road to
weight control a little easier.§
1 calorie = 4,186kJ
* Wenk: Die sg “fridge lock” werk vir ons. Die slot
sluit self d.m.v. „n draaibeweging en sluit oop m.b.v.
„n sleutel.
Tip: The so-called fridge lock works for us. The
lock closes by turning it and has to be opened with
a key.
Precautions Advised When Starting Growth Hormone With PWS
From The Gathered View, March-April 2008
We advocate a sleep study before the start of growth hormone (GH) on infants,
children and adults with PWS and a follow-up study 6-8 weeks later.
If obstructive sleep apnea (OSA) worsens on GH, temporarily stopping GH is
recommended until the cause is understood. Frequently OSA can be corrected by
removing the adenoids and tonsils or lowering the GH dose (in the face of an
abnormally high IGF-1). We also recommend taking precautions during bouts of
upper respiratory infections.
Dr. Merlin Butler also recommends obtaining a thyroid function test and cortisol levels
(in a.m.) before starting growth hormone treatment. He has done a recent study on
cortisol levels in 63 subjects with PWS and found one of four infants with PWS had a
low cortisol level. There has been some discussion about adrenal hypofunction in a
subset of PWS.
Studies have shown that in most individuals with sleep-disordered breathing due to
PWS, GH can actually improve (or at least doesn‟t worsen) the apnea (Haqq et al,
2004; Miller et al, 2006; Festen et al, 2006).
Withholding GH from those with sleep apnea may be detrimental on several levels,
thus the recommended approach is monitoring children with PWS closely when
starting GH to make sure that they do not worsen.§
Jennifer Miller, M.D., M.S., Endocrinologist,PWSA (USA) Clinical Advisory Board
Merlin G. Butler, M.D., Ph.D., Chair, PWSA (USA) Scientific Advisory Board
Daniel J. Driscoll, M.D., Ph.D., Chair, PWSA (USA) Clinical Advisory Board
People With Strength Volume 11 Issue 2 Page 14

PROBLEM CORNER
Q:
What are febrile convulsions? Does it happen more often in children with PWS?
A:
Febrile convulsions/seizures/fits are very common and happen between the ages of
6 and 60 months in about 2 to 5% of children. If the body temperature raises rapidly
these might present as a sudden loss of consciousness with jerking of the arms and
legs, frothing at the mouth and rolling of the eyes. There is not a specific temperature
when it happens but the temperature is usually above 38.5 degrees Celsius. This
obviously creates anxiety for the parents but in the absence of any underlying
pathology and if it lasts shorter than 15 minutes it is regarded as “simple” and the risk
for permanent damage is very low. The cause is unknown but it seems as if there is a
genetic component as it happens more often in certain families. The question is
always if it can develop into epilepsy. This can happen if it happens recurrently or if
there‟s a family history of epilepsy. The treatment will be to bring the temperature
down, protect the airway during the convulsion by putting the child on his/her side
and to give medication if it lasts longer than 15 minutes and the child is in the
hospital. There is a lot of controversy about the long-term treatment in cases where
there are recurrence and if these patients should be started with anti-convulsant
medication.
Febrile convulsions are not unique to children with PWS but it seems as if any child
with some neuro-developmental handicap is more prone to getting febrile
convulsions. In children with Angelman syndrome however seizures are part of the
syndrome and it usually starts with febrile convulsions. Temperature regulation,
which can present with either a too high or too low temperature and symptoms like
deficient shivering during a febrile episode are present in children with PWS but also
in other children with developmental problems.§
Sources:
1. American Academy of Pediatrics: Febrile
2. Seizures: Clinical Practice Guideline for the long-term Management of the
Child With Simple Febrile Seizures. Pediatrics 2008; 121: 1281-6.
3. Williams MS, Rooney BL, Williams J, Josephson K, Pauli R. Investigation of
thermoregulatory characteristics in patients with Prader-Willi syndrome. Am J
Med Genet 1994 Feb 1; 49(3): 302-7.
We thank Dr Engela Honey for answering the question on febrile
convulsions.
Dr Honey is a paediatrician at the Department of Human Genetics,
University of Pretoria§
People With Strength Volume 11 Issue 2 Page 15

PWSA (Taiwan) welcomes you to Taiwan in 2010!
About PWSA (Taiwan)
The PWS Association Taiwan was established in June 2005
for the purpose of serving and providing support to the PWS
individuals and their families and also assisting the PWS
individuals in exploiting their own future. The Association
was founded and based on the earlier PWS group, which
has been formed in 2001. There are now in total 142 PWS
family members in the Association. Besides routine care and
support services and promotional works (issuing a quarterly publication) and events,
the Association also constantly holds educational and communicational activities
such as seminars and conferences for families and professionals, support groups,
fitness classes, and summer camps for PWS children, and also the biggest event yet
to come, the 7 th International PWS Conference 2010, which will be held in Taipei,
Taiwan.
YOU ARE INVITED TO THE 7 TH INTERNATIONAL PRADER-WILLI SYNDROME
CONFERENCE IN TAIWAN 2010
More information on the 7 th International PWS Conference in Taiwan 2010
after July 2008 on http://www.pwsa.org.tw/
§
PWSA(SA) is a registered
nonprofit organisation.
Nr 035-837-NPO
Opinions expressed in People With Strength are those of
the authors or editors and do not necessarily reflect the
views of the management committee of the PWSA (SA).
People with Strength welcomes articles, letters, personal
stories and photographs, and news of interest to those
concerned with
Prader-Willi syndrome.
Please send your contribution to
PWSA (SA), PO Box 2399, Brooklyn, 0075.
People With Strength Volume 11 Issue 2 Page 16

New Publications
Red Yellow Green
System for Weight Management
(Karin Balko, RD & North York General Hospital, PWS Clinic)
This Weight Control Program has been used with great success, and it will make the difficult
task of losing weight easier for everyone in the family – it is about healthy eating and keeping
active and it is a way of life for the entire family. Foods are divided into different groups based
on their nutrient content and calorie levels and it provides a generous amount of food that is
pleasing to the eye. The RYG System also provides the necessary structure that persons with
PWS need. TIPS, recipes and examples of meal plan servings are provided.
DVD Food, Behavior and Beyond
This comprehensive DVD which is a joint project of PWSA(USA) and IPWSO is an
essential teaching and learning tool for parents and others involved with PWS.
Having years of hands on intensive experience, Dr. Linda Gourash and Dr. Janice
Forster shares very important TIPS for the every day management of Prader-Willi
Syndrome. Appetite and emotional aspects are addressed. The difference between
food control and food security are explained. To medicate or not? is also addressed.
Lettie Fouché Skool
Bloemfontein
10 Junie 2008
Beste Rika,
Baie dankie vir die DVD Food, Behavior and Beyond. Ons het die inligting onder mekaar uitgeruil,
sodat die personeel wat met die leerders met PWS werk, beter toegerus is. ‘n Mens het soveel meer
deernis en begrip vir die ouers en jy as onderwyser word bemagtig om met selfvertroue en goeie insig
op te tree tot voordeel van die kind met PWS.
Baie dankie dat julle hierdie waardevolle inligting met ons deel.
Groetnis
Ronel van der Ryst
Onderwyseres
(We received a letter from a staff member of the Lettie Fouché School in Bloemfontein, expressing
their thanks and appreciation for the information on Prader-Willi syndrome and the DVD Food,
Behavior and Beyond. They are now better equipped and found it a great help in understanding the
parent’s situation better. They felt that they are now empowered to handle the child with Prader-Willi
syndrome with more confidence and insight.)
People With Strength Volume 11 Issue 2 Page 17

Snack: Tuna health sandwich
Ingredients
240g Weigh-Less Canned Tuna Chunks in Spring Water, drained
125g fat-free cottage cheese
60g rye bread (two slices)
50g lettuce, shredded
120g tomato, sliced
40g lentil sprouts
4 radishes, thinly sliced
2 tsp chives, sliced
Instructions
1.Mix the tuna and cream cheese, set aside
2. Layer the rest of the ingredients starting with the lettuce and place the tuna mixture
on top.
3. Sprinkle with lentils and chives.
Soup: Tomato and roasted red pepper
Ingredients
200g red peppers
200g onion, chopped
2 sticks celery, chopped
800 Weigh-Less Canned Chopped Tomatoes
1 tsp minced garlic
650ml vegetable stock
salt and pepper
Method
1. Cut and deseed the peppers and place under a hot grill until the skin blackens.
2. Peel the peppers and place flesh in a large pan with the onion, garlic and celery.
3. Add the stock and simmer, covered, for 20 minutes.
4. Place in a blender with the tomatoes and blend till smooth.
Main Course: French toast
Ingredients
41g Albany Slim Slice White Bread (2 Slices)
1 egg (45g)
5ml Weigh-Less Extra Virgin Olive Oil
10ml warm water
Salt and pepper to taste
Instructions
1. Mix the egg, water and seasoning well and set aside.
2. Place olive oil in a flat bottomed pan on a medium heat.
3. Place bread in egg mixture for 30 seconds and quickly place in pan.
4. Allow to slowly brown on both sides. Serve immediately.
*Tip: allowing the French toast to cook on a low heat allows the egg to cook properly,
don't squash the bread with a spatula when cooking.
People With Strength Volume 11 Issue 2 Page 18

Thanks to
Paulette and
Johan for the
birthday
cards!
TO THE CO-WORKERS
AND ALL THOSE WHO
CONTRIBUTE TO
PEOPLE WITH STRENGTH
OUR SINCERE THANKS
AND APPRECIATION
We would like to thank
afrihost.com
for their generosity towards our
association
We are members of:
The International Prader-Willi Syndrome
Organisation (IPWSO)
www.ipwso.org
The South African Inherited Disorders
Association (SAIDA)
www.saida.org.za
WOULD YOU LIKE TO MAKE A DONATION?
PWSA (SA) is registered as a non-profit organisation (No. 035-837 NPO)
as well as a public benefit organisation (PBO Exemption no.930 016 853).
The PBO registration benefits donors and all donations made to PWSA
(SA) are exempt from income tax. We will issue an official certificate for
donations of R100.00 or more.
You are welcome to make a direct deposit.
Please ensure that your cell number is included as reference.
BANK DETAILS of SAVINGS ACCOUNT
PRADER-WILLI SYNDROME ASSOCIATION (SA)
ABSA BROOKLYN, PRETORIA
Branch number 632005 Acc. no. 113641800
DONASIES IS WELKOM!
Die PWSV (SA) is geregistreer as „n nie-winsgewende organisasie (Nr. 035-837 NPO) ook as „n
openbare weldaadsorganisaie (PBO Exemption no.930 016 853).
Hierdie registrasie hou voordele in vir die donateur en donasies wat aan PWSV (SA) gemaak
word is aftrekbaar van die donateur se belasbare inkomste. „n Amptelike sertifikaat sal vir bedrae
groter as R100.00 uitgereik word.
Maak gerus „n direkte inbetaling.
Sluit asseblief „n selnommer in as verwysing.
BANKBESONDERHEDE van SPAARREKENING
PRADER-WILLI SINDROOMVERENIGING (SA)
ABSA BROOKLYN, PRETORIA Taknommer 632005 Rek. nr. 113641800
People With Strength Volume 11 Issue 2 Page 19

Wat is die Prader-Willi Sindroom Vereniging van Suid-Afrika?
In Maart 1990 het ‟n groepie toegewyde ouers hierdie vereniging gestig.
Die hoofdoelwitte daarvan is die volgende:
� Om aan ouers en versorgers van persone met PWS ondersteuning te
bied
� Om kennis oor te dra en ‟n bewustheid van die sindroom by die publiek
sowel as lede van mediese en paramediese beroepe te kweek
� Om die vlak van versorging wat aan persone met PWS gebied word, te
verbeter
MEMBERSHIP FEES ARE DUE 1 APRIL 2008
Entry fee is R50.00. Annual membership fee is R200.00.
R220.00 for members outside RSA.
We appreciate direct deposits or payment through
Internet Banking.
Please ensure that your SURNAME is included as reference.
BANK DETAILS of SAVINGS ACCOUNT
PRADER-WILLI SYNDROME ASSOCIATION (SA)
ABSA BROOKLYN, PRETORIA
Branch number 632005
Acc. no. 113641800
� Publiseer en versprei “People With Strenth” (nuusblad van PWSA(SA)
vir ouers deur ouers)
� Hou jaarliks „n sosiale byeenkoms om ouers en belangstellendes met
kennis toe te rus en te ondersteun
Vir verdere inligting, tree in verbinding met:
Die Voorsitter: PWSA(SA), Posbus 2399, Brooklyn Square, 0075
Tel (012) 344 0241 / 348 8355
Besoek ons webruimte: www.praderwilli.org.za
§
How will a person with PWS be affected?
People with PWS are faced with a triple weight problem:
� they are always hungry (in 90% of people with PWS)
� they put on weight with fewer kilojoules
� they have difficulty in exercising due to poor muscle strength and coordination
People With Strength Volume 11 Issue 2 Page 20

People With Strength Volume 11 Issue 2 Page 21

Y R B U G
Compiled by the Bassons
Play ball
Unscramble the letters on top of the balls to find three
mystery words.
Sport Fun
Complete the names of the different ball games below. To make things a
little easier, we have put one letter for each word in the blocks provided.
W
C
N N I T E S
R T S
People With Strength Volume 11 Issue 2 Page 22
P
C C S O R E

Which sports are shown?
Use the pictures to solve the crossword
Answers:
Play ball:
Rugby, Tennis, Soccer
Sport Fun:
Pool, Bowls, Cricket
Crossword:
1.WHISTLE 2.FIELD 3.BAT 4.BALL 5.COACH 6.GOAL
People With Strength Volume 11 Issue 2 Page 23