M S U D Newsletter
Articles selected from Vol. 14, No.1,
Spring-Summer 1996
Continued from Vol. 13, No.2
The
first installment of this article appeared in the last Newsletter (Winter
1995-96).Ê In that issue I gave a brief
glimpse of our personal family history and our early contacts with other
families.Ê That article also reported on
our first family/professional Symposium (conference) which was held in 1982 at
our local parochial school with almost 100 attending.Ê The Symposium came about because of our desire to learn more and
to share with other families.Ê The
following continues the story of the development of our MSUD Family Support
Group.
At our first symposium, we
discussed the impracticability of continuing our circle letter which had served
as a means of sharing until this time.Ê
It was taking too long to circle with the growing number of families scattered
throughout the U.S. and Canada.Ê We
considered starting a newsletter and elected to have a symposium every other
year.
The first MSUD Newsletter was
published in February â83.Ê I gathered
information and sent it to Patty Swenson (mother of Sanse with MSUD) in
Englewood, Colorado.Ê She retyped all
the information and articles, formatting them on legal sized paper, and then
xeroxed them at her husband Larryâs office.Ê
She also did some of the writing and mailed the newsletters.Ê Larry and Patty did all this voluntarily,
donating their time and materials.
That first Newsletter
consisted of seven, well-filled, legal-sized pages plus a page listing 28
families with their addresses, number of children with MSUD (32) and their
birth dates.Ê It included a questionnaire
to be filled out by all persons interested in receiving the Newsletter.Ê Pattyâs history of their daughter, Sanse, in
that issue was the first in our continuing series of family histories.
In our second Newsletter
(Nov. â83), we reported some statistics from a survey by Virginia Schuett
entitled ãTreatment Programs for PKU and Selected Other Metabolic Diseases in
the U.S.: A Survey.äÊ The survey
reported that as of June 1982,Ê there
were 115 MSUD cases currently being followed in the U.S.Ê Of these 73 were classical, 13 thiamine
responsive, 23 other diagnosed forms and 6 for which a more specific diagnosis
was unknown.Ê By then we had contact
with 35 families involving 49 children.Ê
Our goal was to give the remaining families the opportunity to contact
us if they wished.
Peter Shaffer and his wife,
Sharon, from Kentucky had spent considerable time and effort sending over 200
form letters to major medical centers in the U.S. and Canada in April â83.Ê This notified them of our organization and encouraged
medical professionals to give Peterâs address and phone number to parents of
children with MSUD.Ê The mailing was
made possible by the Genetic Testing, Counseling and Education Services Program
at the University of Kentucky Medical Center which provided names, addresses,
materials and photocopying services.
Peter had also consented to
be our treasurer.Ê In the second, Nov
â83, issue of the Newsletter we asked for a donation of $5 to be sent to Peter
with checks made out to Families with MSUD÷our organizational name at that
time.
In this same issue, Sue Ann
Frederick from Wisconsin shared a paper she had written for one of her high
school classes.Ê It described MSUD and
told of her own diagnosis and childhood experiences with the disease.Ê One statement she made,Ê ãMy body chemistry may not be able to handle
all the changes of pregnancy, so I may not be able to have children,ä is of
special interest considering the cover story later in Vol. 8, No.3 (Dec.
â90).Ê A picture of Sue Ann (Frederick)
McKnightâs baby accompanied an article Sue Ann wrote about her successful
pregnancy experience and the birth of her perfectly normal little girl.Ê She proved it possible, although it may not
be safe for all women with MSUD to have children.Ê The maternal concern had been covered earlier in the March â90
issue.
Patty and I managed only one
Newsletter in â84 and one in â85. The â85 issue was 17 legal-sized pages with
another questionnaire attached÷probably our largest Newsletter.Ê Patty and I were both very busy as I said in
the next Newsletter÷we squeezed Newsletters out through nooks and crannies of
our very busy schedules.Ê Patty and I
also attended the ãGenetics Support Groups: Volunteers and Professionals as
Partnersä conference in Washington, D.C. in the summer of â85.Ê Patty reported on the meeting in the Feb.
â86 issue.
One of the ãextrasä that
interfered with publishing a 2nd issue in â85 was Shaylaâs accident.Ê In July of that year, I had a doctorâs
appointment and told Shayla she could ride her bicycle down the road to her
Aunt after she finished her work.Ê She
turned to cross the road suddenly because she forgot something.Ê A car hit her in the passing lane.Ê She was thrown onto the car windshield,
shattering it, and then into the yard, narrowly missing a tree and
mailbox.Ê Her bike was demolished.Ê In pain and bleeding badly from the head,
she tried to get up and run to the house÷it took a great deal to keep Shayla
down in those days.
The paramedics expected
serious head and neck injuries.Ê I met
her in the emergency room and knew she was fine because she was talking
constantly÷typically Shayla (Wayne says typically Mom, too).Ê She was home again that night with a very
sore body, some deep cuts and abrasions, and a broken collarbone.Ê She healed rapidly and was doing some
housework by the second week.Ê However,
she soon started having headaches which have plagued her since.
I wrote, ãIt is rather ironic
that this accident brought many visitors, cards, gifts and expressions of
concern, whereas a bad case of the flu is so much more critical.Ê Therein lies the difference between the MSUD
child and those without MSUD.ä
Personal accounts of the
children like this account of Shaylaâs accident were frequent in the early
Newsletters.Ê There were reports of
children having tonsils or teeth removed and their reactions.Ê Peter and Sharon Shafferâs Jessica falling
off a 10¸ foot wall holding her formula in a glass with a lid and not spilling
a drop.Ê Peter said, ãIn the kitchen it
always goes all over.äÊ New births and
deaths were reported with some details.Ê
Various accomplishments of the children were described and observations
made such as÷MSUD children have a keen sense of smell.Ê They gag easily at bad smells including
their own bathroom smells which can create embarrassing problems.
We had good reader
participation in the early Newsletters.Ê
Our sections on ãMSUD Family News,ä ãLow Protein Recipes,ä ãMSUD
Featured Familiesä and ãMSUD Parent Question and Answer Exchangeä were well
supplied.Ê In the third issue, April
â84, we printed the first professional article reviews.Ê That issue included reviews of presentations
given at the â83 conference of the Neuro-Metabolic Disorders held in Ann
Arbor.Ê These conferences are still a
source of information for our Newsletters.
Patty typed the two â86
issues of the Newsletter, but had them printed in the letter-sized format we
still use.Ê Patty had an artist design
our present logo for the February â86 issue.Ê
It first appeared on the masthead of the November issue.Ê That Newsletter had a little over 18 pages,
but the type was larger than our current print.
For the first â87 issue Patty
sent the information to a printer.Ê It
was typed on their computer, but Patty mailed them.Ê It was decided that I could do the same thing here.Ê Patty had fulfilled a very important role in
getting our Newsletter off to a good start.Ê
She and Larry did not spare of their time, money and facilities and
deserve much credit for the early success of the organization.
I was thankful for a printing
firm just two miles up the road from us.Ê
This firm was owned and operated by the Stanley Brubaker family (no
relation).Ê However, they were friends
and went far beyond the call of duty helping with the Newsletters.Ê My typewritten pages with corrections in the
margins were taken in stride.Ê They
tolerated my last minute rush requests with calm reassurance.Ê I am sure they could have sympathized with
Patty.
In Dec. â87 we included
pictures for the first time.Ê Pictured
were Elan Geffen from Virginia, Michael Toth from Ontario, Canada and Laura
Henry from Great Britain.Ê We had gone
international.
I published only one 7 page
Newsletter in â88.Ê This is the year we
designed and built our current house which involved a lot of my time.Ê An 18 year old friend of Shaylaâs, who had
helped me with the typing for the â87 issues, was killed in a tragic accident
soon after the Dec. issue was sent out.Ê
Nineteen eighty-eight was an exhausting year.
I tried to make up for it by
getting 3 issues out in both â89 and â90.Ê
Since then I have been back to publishing only 2 issues a year.Ê Other changes made during these years included:
t
Family Contact Person,
Bonnie Koons from Pennsylvania, and Professional Contact Person, Alice Mazur,
R.N., P.N.P. from CHOP in Philadelphia were added to the masthead in June
â89.Ê The format changed again in May â93
with the addition of Mary Kathryn Martin as Food News Editor and Kelly Green,
R.D. as Recipe Editor.
t
We added a Kidâs Klub
childrenâs section as an insert beginning with the Nov. â89 issue.Ê Bonnie Koons did a great job on this
section, but unfortunately after several issues, it was discontinued for lack
of response.Ê Lack of input caused the
demise of other Newsletter sections over the years.
t
I used a friendâs
computer to type the â91 issues while she did the final formatting for me.Ê In â92 we bought our own computer thus
opening up the whole new world of formatting for me.
Other organizational changes, improvements(?) and
accomplishments during these first 13 years:
t
At the second Symposium
we discussed printing a Free Protein Food List.Ê I compiled a list of ãfreeä foods using Virginia Schuettâs Low
Protein Food List and the Lo-Pro Guide from the Metabolism Clinic, Riley
Hospital, Indianapolis, IN.Ê Later the
list was printed on a card to be handed to persons involved with the care of a
child with MSUD.Ê It fell by the wayside
for lack of interest.
t
Information packets were
proposed in the April â87 issue of the Newsletter.Ê Bonnie Koons helped gather the materials and began making them
available to new families in â89.Ê She
continued until she handed over the reins of contact person to Dawn Hahn in
â93.Ê The packets are being upgraded at
this time.Ê Dawn is in charge of
distributing them.
t
After several years of
trying to find a professional who would write a brochure describing MSUD,
several families gathered to brainstorm.Ê
With this help I wrote an information sheet.Ê Later we were able to finance a brochure which was recently
updated.
t
In Dec. â90 the
subscription rate increased to $10.00 and a prayer hot line was started for
those who wished to be involved.
t
At a business meeting in
April â89, we discussed becoming a nonprofit organization.Ê We were small enough that it was not
essential, but we were growing.Ê Irv
Geffen, who lived in Virginia at that time, assumed the responsibility to
tackle the job.Ê We were legally
incorporated in Virginia in 1990.Ê Irv
graciously paid the yearly legal fees for the next couple of years.Ê It wasnât until â93 that all the red tape
was unwound and we had our nonprofit status.Ê
We have always operated on a nonprofit basis using volunteers.
t
Board members were
elected at the Symposium in Montreal in June â90.Ê Even though being so scattered creates a problem for face-to-face
board meetings, they all have served faithfully:
President- Wayne Brubacher, Goshen, IN
Vice President- Sharon Shaffer, Flemingsburg, KY
Bonnie Lou Koons- Secretary, Harrisburg, PA
Peter Shaffer- Treasurer, Flemingsburg, KY
Irving Geffen- Cherry Hill, NJ
Joseph Balinsky- Hampstead, Quebec
Barbara Rudd- North Adams, MA
Many persons have been involved in keeping the MSUD
Family Support Group a growing and evolving organization.Ê One very hard working group I havenât
mentioned yet÷those who organized and sponsored the Symposiums.Ê Here is a list of the Symposiums to date.
|
DATE |
PLACE |
FAMILY SPONSOR |
|
5/82 |
Goshen, IN |
Wayne Brubachers |
|
6/84 |
Flemingsburg, KY |
Peter Shaffers |
|
6/86 |
Denver, CO |
Larry Swensons |
|
6/88 |
Hinkletown, PA |
Pennsylvania Families |
|
6/90 |
Montreal, Quebec |
Joseph Balinskys |
|
6/92 |
Toronto, Ontario |
Toths & Sullivans |
|
6/94 |
Columbia, MO |
Missouri Families |
Medical centers in each of theses areas were very
helpful and supportive and sometimes did a major part of the planning.Ê Professionals and families working together
can accomplish so much.Ê Sandy and Dave
Bulcher in conjunction with the University Hospital in Ann Arbor, MI are
working hard on the next Symposium in Columbus, Ohio.
Two other persons I want to acknowledge are our
daughter, Shayla, and my ãteacherä and indispensable helper, Martha Stern.Ê Without Shayla to carry on the housework
when Mom is glued to the computer, I could not spend the time necessary to do
the Newsletter and the many other necessary tasks.
Martha came into my life soon after I started working
on a computer.Ê She is a very keen
editor and great teacher.Ê She has the
eye of an artist and the patience of Job.Ê
She is a behind-the-scenes major asset to the organization and lives
less than 10 miles from me.Ê The rest of
you seem so far away.Ê
Tish Fuller faithfully puts all the information I send
her on disk.Ê This is a great benefit
and I truly appreciate it.Ê And last but
not least is my long-suffering husband who puts up with a tense bundle of
nerves at least twice a year.
Above all the Lord has blessed this organization and
without His blessing we would fail.Ê
Thanks be to our gracious loving Heavenly Father in whom I trust.
÷Joyce Brubacher
Recipes
Low Protein Cheesecakes
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Crust: |
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6 Graham Cracker Cookies, crushed* |
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1 T sugar |
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1¸ T butter, melted |
Mix and spoon evenly on the bottom of a serving dish
or into 6 cupcake papers. Set aside.
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Filling: |
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¹ c water |
Cool Whip |
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¹ c coffee creamer (liquid) |
1 t cream cheese flavoring |
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4 oz marshmallows |
¸ c. cherry pie filling |
Heat water, creamer and marshmallows in microwave till
marshmallows are melted÷about 2¸ min.Ê
Cool and add Cool Whip and flavoring.Ê
Spoon over graham crust and top with pie filling.Ê Makes 6 servings.
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Per serving: |
Protein |
Leucine |
Calories |
|
Crust |
0.1 gm |
0.0 mg |
77 |
|
Filling |
0.5 gm |
0.0 mg |
74 |
|
Both |
0.6 gm |
0.0 mg |
150 |
|
Per Recipe: |
3.5 gm |
0.2 mg |
902 |
Cathedral Window Cookies
|
110 gm (approx. 2 in sq) white almond bark |
|
110 gm (approx. 2 in sq) chocolate almond bark |
|
2 1/2 to 2 3/4 c (125 gm) colored miniature
marshmallows |
|
6 Graham Cracker Cookies, crushed* |
Melt almond bark in microwave 2¸ min, stirring every
30 seconds till melted.Ê Mix in the
colored marshmallows.Ê Shape into a roll
and roll in the graham cookie crumbs.Ê
Refrigerate till chocolate hardens.Ê
Slice into 3/4 inch slices. Makes 20 slices.
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* Low protein Graham Cracker Cookies are similar to
regular graham crackers and can be used in the same way.
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2 3/4c (300 gm.) wheat starch |
1 1/2 t yeast |
|
3 T psyllium fiber** |
2 T sugar |
|
1 T methylcellulose*** |
3/4 t salt |
|
2 T coffee creamer (powder) |
1 1/3 - 1 1/2 c warm water |
Measure all ingredients in mixing bowl in the order
given, adding water last.Ê Beat until
thoroughly blended.Ê Let set 15-30
minutes.Ê Beat well again.Ê Put into bread pan; let set till double in
size.Ê Place in cold oven.Ê Turn oven on to 350¡.Ê (I place 6 ice cubes in the
bottom of the oven when I put pan in.Ê
Bread will rise another 2 inches as oven heats.Ê As the ice cubes evaporate, it keeps the
bread from getting too dry.)Ê Bake 30
minutes.Ê This dough is very sticky but
makes wonderful bread.Ê I also use this
for pita pockets and pizza crusts.Ê
Slice bread into 14 slices.
|
|
Protein |
Leucine |
Calories |
|
Per serving: |
0.2 gm |
0.0 mg |
89 |
|
Per recipe: |
2.8 gm |
0.2 mg |
1250 |
|
1 c wheat starch |
5-6 T water |
|
2 T coffee creamer (powder) |
¸ t egg yolk |
|
1 T methylcellulose*** |
|
Mix dry ingredients together.Ê Add water and egg yolk.Ê Mix well.Ê
Put through noodle machine or roll out and cut potpie noodles.Ê Makes 2 servings.
Note: To add
color and flavor, boil water with a pinch of saffron.Ê Cool water and add to dry ingredients.Ê
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**Psyllium fiber is available at health food stores,
and is used in place of Metamucil.Ê It
improves the texture and flavor of the bread, making it more like regular
bread.Ê Be sure to use the amount listed
in the ingredients.Ê The whole psyllium
fiber works better than the ground fiber.
***Methylcelulose is added as a stabilizer and makes
the wheat starch work better.Ê It is
available from Ener-G Foods, Inc.
Substitutes For Milk &
Ice Cream
Recently I discovered Rice Dream Non-Dairy Beverage
and Rice Dream Non-Dairy Frozen Desserts in our new local health food
store.Ê They seemed to be quite low in
protein, so I bought some for Shayla to try.Ê
She was delighted to find products that taste so nearly like real milk
and ice cream.Ê This is the first time
she can actually pour a ãmilkä directly on her cereal without diluting rich
non-dairy creamers.Ê This rice-based beverage
looks more like skim milk.Ê I found it
tasty with a mild ãriceyä flavor. Ê
I called the company to find out more exact
nutritional values and was thankful for the very low protein count.Ê However, asÊ
frequently happens, the products have not been analyzed for
leucine.Ê They claim it is nearly
impossible to test for amino acids in such minute quantities.
The Non-Dairy Beverage containers list the protein as
1 gm per 8 fl oz.Ê The company gave me
the analyzed amount as .303 gm per 8 oz.Ê
That is low enough to be listed as free protein on packages since 1 gm
can mean anywhere from .5 to 1.5 gm of protein.Ê The non-dairy dessert has the ¸ cup serving size listed as 1
gm.Ê The analyzed amount is .363 gm per
¸ cup.Ê So Shayla can make a tasty
ãmilkä shake using 1 cup of ãmilkä and ¸ cup of ãice creamä for .66 gm÷a little
over a ¸ gm of protein.Ê Both products
have 130 calories per serving.
The beverage is available as regular, enriched and
vanilla enriched.Ê The frozen dessert
flavors include Swiss almond, vanilla, lemon and mint carob chip.Ê It looks and tastes like ice cream.Ê The prices are not unreasonable.Ê It is marketed by Imagine Foods, Inc. in
Palo Alto, California.Ê
They also market Imagine Pudding Snacks and Rice Dream
Non-Dairy Frozen Bars which have only 1 gm of protein per cup or bar.Ê That is a rounded figure which could be
considerably less if listed in the same way as the others.Ê The company is doing more analyzing, since
they have received requests.Ê We will
try to keep you informed
Adapted from two articles published in the National
PKU News
Helping your child on a special diet eat right
Persuading a child to eat different and nutritious
foods, whether or not the child has special health care needs, can be an
exhausting and frustrating experience.Ê
However, you can help your child develop positive attitudes about
nutritious foods.
Children learn best by example.Ê Remember, your child wants to be like
you.Ê If the child sees you eating more
salads, fruits and vegetables, he or she may want to do the same.
Listening to our kids
My bright, six‑year‑old son, Jeffrey, has
phenylketonuria (PKU), a metabolic disease that requires him to follow a low‑protein
diet regimen.Ê This means plenty of
substitutions when his friends may be eating ãfunnerä food.Ê When a special diet is a lifelong requirement,
it is important that children develop a sense of responsibility for what they
eat.Ê At the same time, it is important
to allow them to express their feelings about the diet.Ê We can learn a lot by listening carefully.
For example, Jeffrey recently said to me, ãMom, I
really liked the Îpeanut butterâ (actually a low‑protein substitute) and
jelly sandwich you put in my lunch box today.äÊ
I realized that this sandwich was more than just a tasty lunch.Ê I knew that it also boosted Jeffreyâs self‑esteem
to be able to eat food that looked similar to that of his peers.
Another time Jeffrey came home from school, sad and
dragging, and said emphatically, ãI donât like this diet!äÊ Together, we figured out he was unhappy
because he couldnât eat a hamburger.Ê
Regular hamburgers had too much protein, but together we came up
with an acceptable substitute÷low protein mushroom burgers.Ê As parents, we have to follow medical
advice, but we can still learn to listen to our children and creatively manage
their diets.Ê Children appreciate being
able to express their feelings openly and having their needs treated
respectfully.
A voice and a choice
Parents can reinforce a sense of responsibility in
children by giving them a voice and a choice in matters that affect them.Ê Severely restricting your child without
explanation can lead to immaturity and rebellion.Ê Children will accept ãrulesä more readily when explanations are
given.Ê Although health conditions may
limit some options, we can help children accept inevitable choices gracefully.
Parents can involve young children in food choices by
talking about them.Ê For example, a two‑year‑old
can decide whether he wants a half glass of ãmilkä or a full glass.Ê A four‑year‑old can be asked if
he wants an apple or an orange, or whether he wants his bread toasted or plain.
Children can be deliberately presented with many
situations in which they have to make choices and are given chances to
succeed.Ê We can creatively select the
situations and let children make the choices.Ê
This sends a clear message that children are not just recipients of
ãorders,ä but participants in decisions that affect them.
For example, when Jeffrey was very young, I always
kept frozen low‑protein bread and pancakes in Ziploc bags in the freezer,
on a low shelf at Jeffreyâs eye level.Ê
I would let him choose between bread or a pancake by responding
verbally, by pointing or by actually getting it out himself.Ê This made him feel that he was ãin charge,ä
and was a very small step toward dietary self‑management in the future.
I also kept a variety of fresh fruit and vegetables in
the lower part of the refrigerator so Jeffrey could see the choices between
tangerines, grapes, plums and other fruits, or the choices between lettuce,
carrots, celery, tomatoes or cucumbers in the vegetable crisper.Ê The pantry was set up the same way with
small cans of fruits and vegetables on the lower shelf so I could ask Jeffrey,
ãDo you want carrots or green beans?äÊ
or ãDo you want Mottâs peaches or a Strawberry Fruit Pak?äÊ He would go to the drawer where the
measuring cups are kept and hold a cup up to the item he wanted.
Introducing new foods may take some effort.Ê However, you will be rewarded quickly when
you see your young child making the right choices from a smorgasbord of
food.Ê You can help your child develop
tastes and preferences that last a lifetime.
This article was adapted from two pieces by Marsha
Magol that first appeared in National PKU News.Ê ÊThe
article is reprinted from the Aug. â95 issue of exceptional Parent, a
monthly magazine offering practical information to parents and professionals
involved in the day-to-day lives of children and young adults with
disabilities.Ê For subscription
information phone 201-634-6550.
Marsha Magol is a regular
contributor to the National PKU News.Ê
She, her husband and one son live in Tampa, Florida, where Marsha works
for GTE.Ê She works nearly full-time and
in the spring/summer â95 issue of the National PKU News she gave some
planning and organizing tips for busy mothers who need to manage diets.Ê Following are several ideas she mentions in
that article which may be helpful to MSUD moms as well as others dealing with
special diets.
áÊÊÊ It
works best to set aside a few hours one day a week to focus on low protein
cooking like baking low protein bread and making, pizza and soup.
áÊÊÊ Involve your child
by asking, ãHow would you like to help me press out this pizza dough into big
circles?äÊ Encourage your childâs
participation in diet preparations at an early age.
áÊÊÊ Prepare in abundance
while you have your recipe book out and refrigerate or freeze as much as you
can for those harried nights.Ê Have your
own ready-to-eat convenience foods.