M S U D Newsletter
Articles selected from Vol. 12, No.1, May 1994
In
the last Newsletter (Dec. 1993) we printed a newspaper account of Dr. Morton
receiving the Albert Schweitzer Award.Ê
His acceptance speech has since been printed in the Clinic for Special
Children Newsletter.Ê Many were
impressed with his speech,and especially his philosophy, which is the
foundation of his work in this unique clinic.Ê
Since he is one of our professional contact persons, and probably serves
the largest number of children with MSUD of any medical center in the North
America, we think both families and professionals in our organization will find
his acceptance speech interesting and touching.Ê With permission, it is reprinted here from the Clinicâs
newsletter.
Dr. Morton Wins Albert
Schweitzer Prize for Humanitarianism for 1993
Dr. Holmes Morton, founder of
the Clinic for Special Children, is the recipient of the 1993 Albert Schweitzer
Prize for Humanitarianism.Ê Given in
Baltimore, Maryland on October 27 by The Johns Hopkins University on behalf of the
Alexander von Humboldt Foundation, the prize recognizes Dr. Morton for his
dedicated and effective work at the Clinic for Special Children.Ê Previous recipients include former President
Jimmy Carter, Marian Wright Edelman, Norman Cousins, and former Surgeon General
Dr. C. Everett Koop.Ê The Prize includes
an award of $10,000 to Dr. Morton which he will donate to the Clinic.
The work recognized by the
Schweitzer Prize is made possible through the support of many who helped raise
the Clinic from frustration and hope since the need became public through the
Wall Street Journal in the fall of 1989.Ê
In a sense the prize also belongs to many of you who have made the work
at the Clinic possible and to many of the families who come here.Ê In his acceptance speech, Dr. Morton spoke
of why children within the Amish and Mennonite cultures, born with genetic
disorders, who have complex medical problems, are thought of as Special
Children; how they are accepted as a gift rather than as a burden, and why
these children inspire his work.Ê Many
who heard his speech in Baltimore asked for a copy.Ê We decided to print his remarks in this newsletter as one way to
share his thoughts about his work and say thank you to all who have made it
possible.
ãI am honored and happy to
have the work at the Clinic for Special Children recognized by the Schweitzer
Prize.Ê I thank Randy Testa who
nominated me and the members of the committee who awarded the Prize.
There are several people who
in an immediate way share this Prize with me.Ê
My wife Caroline and I together decided to establish the Clinic.Ê Without her ideas, her hard work, and her
understanding, the Clinic would not be.Ê
Richard Kelley was my mentor.Ê He
taught me much of what I know about genetic diseases and much credit for the
scientific work done at the Clinic goes to Rick.Ê Without his help and friendship the Clinic would not be.Ê Enos & Anna Mae Hoover, Amos & Susie
Miller, Rebecca Huyard, and others in the Huyard family understood the need for
a clinic for special children long before the idea was widely accepted within
the Amish and Mennonite communities.Ê
Without their prayers and their work the Clinic would not be.Ê I also must thank again a writer named Frank
Allen, whose words helped the dreams of all of us become real.
The Prize is also a tribute
to my teachers.Ê A few of them are here
today.Ê I was never an easy person to
teach.Ê I doubted, questioned, and
argued my way through an unusual education.Ê
My interests in people and art, medicine and science, which are the
sustenance of my work each day, were fostered by a few teachers of literature,
writers, scientists, and doctors.Ê I
remember them as gifted teachers and thoughtful people.
After the letter came from
Dr. Richardson about this award, I began to read about Albert Schweitzer.Ê The Prize has caused me to think about the
work and words of a great person.Ê That
alone was a valued gift.Ê Dr.
Schweitzerâs example always now will be in my thoughts about our work at the
Clinic.
By age 30 Albert Schweitzer
had advanced degrees in music and theology.Ê
He was recognized in Europe as an authority on the music of Bach, his
theological books were widely read, and he taught at the University in
Strasbourg.Ê Yet he then turned away
from a life as an academic.Ê At age 30
he decided to learn medicine and surgery and go to Africa as a missionary
doctor.Ê He studied for eight years to
obtain his Medical degree.Ê When he was
38, he and his wife went to a remote region of western Africa to start a
clinic.Ê His first operating room was
fashioned from a chicken coop and his patients stayed in thatched huts with
dirt floors.Ê He repaired hernias and
broken limbs, treated diseases of malnutrition, and, in a time when medicine
had little to offer, he cared for those who would die of malaria, sleeping
sickness, tuberculosis, leprosy, and malignancy.Ê His difficult work at Lamberene continued for more than 50 years
until his death at age 90.Ê His writings
make me think that his work was sustained by his ideas and his ideas were ever
renewed and enriched by his work.Ê I
would say that is why his work endured.
Will our work at the Clinic
last as his did?Ê I too was 38 when I
went to Lancaster County to work with the special children.Ê If I am blessed with as many years as Albert
Schweitzer then I have 47 more years to work at the Clinic.Ê The Clinic for Special Children is in a
timber frame building with a roof of barn‑slate.Ê Such buildings have lasted hundreds of
years.Ê We are found at the end of a
long lane in the middle of an Amish farm and there are hitching posts in the
parking lot.Ê Dr. Schweitzer would have
understood why the Clinic is there÷it is where it is needed.
The natural histories of
diseases we treat make preventative care and ready access to special care
essential.Ê He also would have
understood that it is important that the Clinic was built and is supported by
people whose children need the care that the Clinic provides.Ê Our work and lectures have started to change
medical practice in Lancaster County.Ê
Midwives, nurses, and doctors, who staff the local hospitals and other
clinics in the region, are better informed about genetic disorders.Ê They know that some disorders, which are
elsewhere rare, are common in Lancaster County and should be recognized by a
general practitioner. More important, they have learned that some of these
conditions can be effectively treated, and they know we are available to help.
These are encouraging signs that the Clinic will last.Ê Nonetheless, I believe ultimately our work will
be sustained by the children we help.Ê I
want to tell you more about the special children.
Albert Schweitzerâs writings
about his reverence for all life have led me to think about an aspect of our
work that is often over-shadowed by scientific efforts, here and elsewhere, to
describe and prevent genetic disorders.Ê
As I care for children with complex, sometimes lethal, inherited
disorders, I am impressed by the hopes and worth of these children.Ê The Plain People call them Godâs Special
Children.
Amish friends, the Amos
Millers, spent Saturday afternoon at our home a few weeks ago.Ê Amos asked me about a small telescope on our
back porch.Ê I explained that my
children and I used it to look at mountains and craters of the moon and the
rings of Saturn.Ê Amos didnât know that
men had walked on the moon 13 times but seemed neither surprised nor impressed
by the fact. ÊHe asked,Ê ÎHave you thought much about why the stars
are there?Ê Do you think God made the
moon and stars just to look at?Ê What is
the moon for?â
Amos Millers had five special
children.Ê Amos & Susie asked many
times, ÎWhy does God give us these children?âÊ
What are special children for?Ê
The answer offered by modern genetics is not a sufficient answer for
them.Ê Scientific medicine does not even
allow such questions.Ê But these
questions are asked, and can be answered by the Plain families who have special
children.
For us to understand the
significance of such questions we must acknowledge that the world view of the
Plain People is different from that of most of us, and that these communities
of the Amish and Mennonite people are not simple and antiquated cultures.Ê To quote John Hostetler:Ê ÎThe Amish people are neither relics of a
bygone era nor a people misplaced in time.Ê
They have reached conclusions different from most moderns about how to
live in todayâs world.Ê Their past is
alive in their present.Ê They are a
different form of modernity.â (Amish Society 1983)
Within cultures that endure
for hundreds of years, as these have, beliefs, faith, events, stories, work,
histories, the stars, and the elderly and children do have purposes.Ê I believe that if we are to provide adequate
care for special children of the Plain People then we must appreciate the place
of these children in their families and communities.Ê You will better understand what I mean if I take you on a house
call.Ê To do that I will read part of a
letter I wrote last year to Jim Hopkins, who is here tonight, and who 25 years
ago taught me to read fine books.
I recently thought of you on
a November night as I walked out of an Amish farmhouse into cold rain and
darkness and paused to think about the dead boy and the gathering of people in
the room behind me.
The father sent word that the
boy died, and I went to the home to sign the death certificate.Ê Carriages and wagons of friends and family
were parked along the lane.Ê From where
I first stopped, I watched black figures move ahead of horses to the barn and
then to the house.Ê Through dark windows
I could see light from an open door at the center of the house.Ê As I stepped into the kitchen, a figure in
the lighted room motioned and said,Ê
ÎMorton, we are here.â
From the doorway I saw that
the harsh white light from a lantern above the bed made the hands and face of
the dead boy cold blue‑white.Ê
Bright silver light flashed from new coins placed over his eyes.Ê But then I saw that the lantern light was
softened in colors of the quilt gathered around him and the light was golden on
his hair and on the hair of the children who played quietly on the end of his
bed.Ê The now soft light washed over the
faces of those seated shoulder to shoulder around the room who one by one shook
my hand.Ê Several said, ÎI have heard
Dr. Mortonâs name often and now I am glad to meet you.â
ÎWhen did he die, John?âÊ ÎOh, not so long ago.Ê Maybe he is still warm.âÊ Then the father took the boyâs hand and
turned it in his with the gentleness used to hold a baby bird.Ê The fatherâs hand was large and thick from
heavy work.Ê The skin over the palm and
fingers was stained and cracked and looked like the bark of an oak.Ê The boyâs hand was so small. ÊÎNo,â he said, Îhe is cold now.âÊ Then he placed the lifeless hand in mine.
I sat on the chair by the bed
for more than an hour.Ê The boyâs mother
said just two days ago his grandfather carried him out to the barn to watch the
milking, and he pulled the tail of a cat and laughed.Ê And yesterday as she read to him, he pointed to pictures and
softly made the sounds of animals as pages were turned.Ê But today he was awake only a little
while.Ê At first his breathing was
harder, then weaker, and, toward evening, just faded.Ê He didnât seem to suffer.Ê
He had found peace.
I talked about how difficult
it is to care for children who have illnesses that are not understood and
cannot yet be treated.Ê I said that as a
doctor and scientist, when each new therapy fails, I must somehow renew my
efforts to learn more.Ê Then the boyâs
grandfather spoke.Ê As he spoke he
smiled and looked first at me then the children on the bed.Ê He said, ÎWe will be glad if you can learn
to help these children, but such children will always be with us.Ê They are Godâs gift.Ê They are important to all of us.Ê Special children teach a family to love.Ê They teach a family how to help others and
how to accept the help of others.â
We talked about the boyâs
sister who had lived a little longer, and about other special children who had
come and gone before.Ê And of those, ill
like this boy, who were living still, but may not live through winter.Ê We were thankful for the health of their new
baby.Ê Then we talked about the harvest
just finished, the needed rain falling outside, the weddings of November, and
signs that winter would be long.Ê John
said,Ê ÎWe are glad you came.Ê Thanks for your help.â
As I looked back into the
house, I remembered the children at play on the death bed and what the grandfather
said.Ê His simple words would change the
way those children, and I, would remember the life and death of the boy.Ê I understood that gathering in the room was
not only a ceremony about death and life after death, but was the means by
which the family would both endure and be strengthened by the loss of a
child.Ê That was the childâs gift to his
family and to all of us who knew him.
Special children are people
who hope to suffer less and lead fulfilled lives through the help of
others.Ê Within their families and
communities they are not merely the object of compassion and love, but often
are the very source.Ê Special children
shape the Amish and Mennonite cultures, and inspire work, such as that at the
Clinic, in important and forceful ways.Ê
We should not underestimate the value of their lives, however brief, or
however difficult.Ê We should not assume
that the Plain cultures, or our own cultures, would be better without them.
These special children are
not just interesting medical problems, subjects of grants and research.Ê Nor should they be called burdens to their
families and communities.Ê They are
children who need our help and, if we allow them to, they will teach us to love.Ê If we come to know these children as we
should, they will make us better scientists, better physicians, and thoughtful
people.Ê And because of them, the Clinic
for Special Children will likely endure.Ê
Our work, like Dr. Schweitzerâs, will be sustained by our ideas, and our
ideas will be ever renewed and enriched by our work.ä
Joyce Brubacher÷Editor
I had already started this
Newsletter when I talked on the phone with Cliff Webster, the Grandfather of a
child with MSUD.Ê I mentioned that
Jessica, Peter Shafferâs daughter, had been hospitalized for vomiting which did
not respond to IVs.Ê I mentioned the
good response to a specially modified Total Parenteral Nutrition (TPN)
treatment for MSUD that she and other children have experienced.Ê He encouraged me to print more about this
treatment in the Newsletter in laymenâs language.
In the April â91 Newsletter
under Resources we listed the paper titled ãBranched -Chain Amino Acid-Free
Parenteral Nutrition in the Treatment of Acute Metabolic Decompensation in
Patients with Maple Syrup Urine Diseaseä by Gerard T. Berry, M.D., et.al. from
the Childrenâs Hospital in Philadelphia (CHOP) and reprinted from the New
England Journal of Medicine 324:175-179 (January 17, 1991).
That column in our â91 issue
was submitted by Alice Mazur, R.N., P.N.P. from CHOP.Ê The description of the paper is given thus÷ãThis report explains
the treatment of acutely ill children using a new intravenous nutritional
therapy.Ê It involves nine episodes of
illness in five patients with MSUD at CHOP.Ê
A mixture of complete nutrients, except the branched chain amino acids
is administered intravenously.Ê This
proved effective in reducing the plasma branched chain amino acid (BCAA) levels
when patients were not eating or were vomiting.ä
Dr. Morton mentioned the use
of this type of hyperalimentation (more than normal nourishing) in his article,
ãMSUD News From the Clinic for Special Children,ä in the Dec â92 issue of the
Newsletter.Ê In the Dec. â93 issue,
Glenda Groff told how the TPN was instrumental in helping her son, Jordan,
recover from a severe illness.Ê In the
same issue, Dr. Richard Allen (from the University Hospitals in Ann Arbor,
Michigan) called TPN a great advance in treatment, avoiding the need for
dialysis that had previously been used during acute bouts of illness.Ê Dr. Allen, Dr. Morton and CHOP have used
this treatment very successfully on newborns with elevated levels of BCAA.Ê I do not know how many other medical centers
are using this treatment at this time.
Rather than reprint a
technical article on TPN for MSUD, I decided some personal testimonies from
parents may help to verify the importance of this apparently lifesaving
treatment.Ê It is very important that
you and your doctor are familiar with TPN and have a plan for its use before
it is needed.
I called three families who
had children treated with TPN.Ê I asked
them to write a short account of their experience and fax it to me as soon as
possible.Ê All three faxes arrived
within twelve hours.Ê I call that real
support!Ê Hopefully these reports will
help this treatment receive the recognition it deserves.
TESTIMONIALS
ABOUT THE USE OF TPN
Sandy
Bulcher÷Ohio
Last month was a trying, yet
educational time for our family.Ê Jordan
our 4¸ year old son with MSUD was sick from a viral infection.Ê I was in contact with Jordanâs dietician,
Julie Jacobson and Dr. Allen from the University of Michigan Hospital and was
hopeful that his levels wouldnât elevate significantly. Unfortunately, he awoke
the following Sunday morning irritable, ataxic, and with slurred speech.Ê He was admitted to our local Childrenâs
Hospital and it soon became obvious that his condition was deteriorating.Ê The medical staff communicated frequently
with Dr. Allen and it was decided that Jordan needed TPN.
TPN is the abbreviation for
total parenteral nutrition and is an effective means of lowering the leucine
level.Ê It is an IV solution that
provides adequate nutrition and calories without the branched-chain amino acids.Ê After some research, the staff located a 3
day supply at Childrenâs Hospital of Philadelphia.Ê The solution was sent to us via plane and Jordan was started on
the TPN.
To our amazement, Jordan
became more responsive, less ataxic, and spoke more clearly within hours.Ê Since this incident, the pharmacy staff at
our local hospital has gathered data about the TPN, so they can obtain it as
quickly as possible if needed in the future.
This was our first experience
with TPN and clearly Jordanâs recovery time was shortened.Ê I feel a sense of comfort knowing that it is
available and very effective if Jordan should need it again.Ê I would encourage everyone to discuss TPN
with their doctor and educate themselves also.
Leon & Dianne
Kennedy÷Michigan
Joyce asked for a paragraph
on our experience with MSUD TPN.Ê
Actually we have had to use it twice for Lewis, and I would highly
recommend it when an MSUD child gets sick with vomiting and is not responding
to the usual treatment.
Both times we have used it
for Lewis, he was very ill.Ê The first
illness was apparently triggered by an infection, during the second one he just
started falling asleep at his desk at school.Ê
By the time I picked him up and got him home, he was vomiting.Ê We took him to our local hospital (after
contacting Dr. Allen).Ê We got the usual
run-around there as they always want to wait and see if the child will quit
vomiting on his own.Ê I think maybe they
feel it is just the flu or something.
Anyway by seven oâclock in
the evening, Lewis could not walk, sit, or hardly hold his head up.Ê The hospital had started an IV which wasnât
doing a thing.Ê Dr. Allen told them how
to get the TPN (or ordered it for them).Ê
It took about 24 hours to get it.Ê
Within eight or ten hours on the TPN, Lewis was sitting up watching
cartoons.Ê The doctor in ICU called it
ãmiracle juice.ä
Carl & Sandy
Kiel÷Michigan
We had two experiences where
we used the MSUD TPN for Jenna. The first was as a newborn.Ê Although Jenna was caught by newborn
screening, she was suffering severe signs, such as loss of sucking reflex, very
fussy and irritable.Ê By 10 days of age
she had a slight seizure and was near comatose.Ê She began on the TPN at 10 days of age at the University of
Michigan Hospital.Ê They had the TPN
available there because the Kennedyâs Lewis had used it two weeks prior.Ê After two to three days on TPN, Jenna was
responding much better and we were able to come home after only one week!
The second time was when
Jenna was almost three years.Ê She
contacted the flu.Ê She had vomited
Monday, and I fought hard to keep her drinking well so she would not become
dehydrated.Ê She seemed better some
days, but by Friday she just lay around very quiet, not playing or talking,
just sleeping.Ê Dr. Allen arranged to
have the TPN shipped to our local hospital.Ê
It took 24 hours after we checked into the hospital for it to arrive and
Jenna to start receiving it.Ê Within 24
hours on TPN she was once again talking, sitting and by 48 hours was back to
normal.
Nicky Guthrie
With permission from Nicky
Guthrie, I am reprinting most of a letter she sent to me.Ê Nicky and her family live in
Scotland÷thousands of miles in distance from many of us, but close in heart and
experience. I think most families can identify with her candid account in some
aspect of our own relationship with others.Ê
She also gives sound advice which applies to any of our relationships.
I find the MSUD Newsletter a lifeline!Ê I have never met anybody with children with
MSUD, or indeed any other metabolic disease, and the information and
experiences shared in the Newsletter, I find absolutely invaluable!
I have been trying to think of some way I could
contribute something to the Newsletter, but I am not really sure that I can be
of any help.Ê It seems that our
situation is just so different from most of the other people that read the
Newsletter, that Iâm not sure our experiences would be relevant to them.Ê We have two children with MSUD÷Laura, aged
five, and Catherine, aged three.Ê Laura,
interestingly enough, has an almost identical early history to the boy, Nick
Lovrin, featured in the last Newsletter.Ê
She was diagnosed late, at 14 months, and really should not have survived,
as blood and skin tests showed her to be very severely affected indeed.Ê She was badly delayed mentally and
physically at the time of diagnosis, and we were told that she would be
permanently brain damaged; but, miraculously, she had closed the gap between
her and her peers by the age of three years, and is now above average
intellectually.Ê Catherine was diagnosed
antenatally, and, despite many hospital admissions to correct metabolic
imbalances (nine in seven months last year), is so far developing normally.
As you know, there are many different aspects to
caring for a child with MSUD, and the area I have been pondering over lately
has been relationships with other people÷friends and the extended family.Ê This is where we perhaps do not have much in
common with most of your readers.Ê
Geographically we are very different, and perhaps the people involved
are rather different.Ê Perhaps I have a
false view of Americans, and I know it must be wrong to lump all
Americans together, but it does seem that you guys are generally more ãopenä
people than we Scots, who are probably too private by half!
Anyway, I have found that we have felt a great deal of
support from our friends, but that things have not always been so easy with the
wider family.Ê I thought it might be
interesting to write about this.Ê I have
never heard anybody say this, and yet I am sure that other people must
have experienced it too.Ê It can make
you feel so isolated and hurt, despite all the support you feel flooding
through from your friends.
The first, and by far the most obvious area I have
felt this, is from my sister‑in‑law, my brotherâs wife.Ê (I write this knowing that they will never
read it, and with the feeling that to share this experience may be helpful to
other people who may find themselves in a similar position.)
I feel difficulties have arisen between our family and
theirs for several reasons.Ê The most
important one is that their eldest child is nine months younger than our eldest
child and their second child is four months younger than our second child.Ê Because our children are just slightly
older, and have a potentially very serious disorder, I think my sister‑in‑law
must have felt, subconsciously, that all the attention (of relatives) was going
to be focused on our children, and that theirs would be neglected.Ê Of course, ãnormalä babies and very small
children can be very stressful to look after, and parents of ãnormalä children
have many worries too.Ê I think that my
sister‑in‑law felt that nobody would pay any attention to her
worries and difficulties.Ê We live only
ten miles from them, but I had to spend so much time coaxing the children to
take their special diets when they were tiny, I could not spend as much time
with my brotherâs family as I would have liked.
As all parents with MSUD have done, we have been
through some very difficult times.Ê When
our first child, Laura, was eighteen months old (not an easy age), and I had
just been told that the child I was then carrying was also affected with MSUD
(devastating news), I started getting messages from my sister‑in‑law
to the effect that I cared about nobody but myself and my problems;Ê I didnât care at all about her and her
child.Ê At the time this felt like the
straw that would break the donkeyâs back.Ê
I was desperately hurt and upset by it, as I do care for them so
much, and I spent many sleepless nights because of it.
The point I need to make here is that my sister‑in‑law
is by no means a nasty person.Ê She is a
very kind, loving, generous person, who makes friends easily and is generally
an open, welcoming sort of person.Ê But
she is also impulsive, and very subjective.Ê
She is not an objective person.Ê
What I now think must have happened is that she had started to resent
the fact that we had a ãspecialä child.Ê
She felt that her child should be considered ãspecialä too, but was only
ordinary.Ê This was, and is, absolutely
untrue, but as all our other relatives live a long way away from us, it was
possible for this illusion to grow.Ê
When we were told our second child would be a ãspecialä child, this may
have, subconsciously, seemed even worse for her.Ê Of course my brother, to whom I had previously been pretty close,
spent a lot of time with his own family and very little with us.Ê His wifeâs feelings towards us began to
spread to him, though by no means to the same extent.
Again, I found this wildly hurtful, and felt powerless
to dispel these illusions.Ê The
isolation and loneliness I felt when dealing with my two children with MSUD was
greatly heightened because of it.
All four children are older now, and life is that much
easier for all of us.Ê Our relationship
with their family is better than it was, but not ideal, and we lead pretty
separate lives.Ê I do not know if they
know exactly what MSUD is.
If anyone else finds themselves in a similar position
to this, I think there is only one course to take.Ê Do not let any resentment build up within yourself against
the other people.Ê It will destroy
you.Ê You may find yourself hurt, but do
not let yourself resent them.Ê Even if
the other peopleâs feelings are subjective and untrue, you can
understand why they have arisen, and if you can continue to show them care and
love, then, hopefully, in time, their resentment will be dispelled.Ê There is nothing to be gained by animosity,
except more stress.Ê You are too
vulnerable.
I was four months pregnant when we were told that our
second child had MSUD too.Ê I phoned to
tell my mother‑in‑law, who was on holiday in the States at the
time, and to tell her we were keeping the child.Ê She thought we should terminate, and her immediate reaction was,
ãWell, itâs your child, and your decision!ä
True enough, but it was the way she said it!Ê She had told me previously that she thought
we should not have another child with MSUD.Ê
She said she would like her son to have the experience of bringing up a
ãnormalä child.Ê That was her immediate
reaction, but she phoned us a couple of days later to say that, of course, she
would support us to the full, whatever our decision.Ê I must say that she has.
Having a ãspecialä child within the family network is
not an easy business for so many different reasons.Ê Perhaps it is more difficult for the ãin‑lawsä because they
feel slightly on the outskirts of that particular family. ÊThe same expectations are perhaps not the
same with friends, which is possibly why difficulties do not arise so often
with them.
DIET AND
NUTRITION
Recipes
|
2/3 c. Wel-Plan Baking Mix |
1 tsp. low protein egg replacer |
|
1¸ tsp. baking powder |
3 T vegetable oil |
|
¹ tsp. cream of tartar |
3¸ T. Coffee Rich |
|
¹ tsp. salt |
|
|
Topping: |
|
|
4 oz. Cool Whip |
|
|
¸Ê (3oz.)
pkg. Jell-O or 3 to 4 T. Pronto Gelled Dessert |
|
|
Fresh, frozen or canned fruit |
|
Mix dry ingredients, add oil and Coffee Rich.Ê Mix well and press into a pan, 8x8 in., 7x11
in. or 9 in. round pan.Ê Bake at 400¡ for 10 to 12 min.Ê Cool.Ê Fold Jell-O into Cool Whip and spread on
crust.Ê Top withÊ drained fruit, arranging in an attractive
design.Ê Can use pineapple, peaches,
pears, kiwi, fresh blueberries, bananas, orange slices, etc.Ê Thicken fruit juice with Clear Jell or
cornstarch and spread thinly on top to keep fruit from darkening.
These nutritional amounts are for the total recipe but
do not include the fruit or fruit juice glaze.Ê
Add your fruit and juice values to these amounts and divide by the
number of servings.
|
|
Leucine |
Protein |
Calories |
|
With Jell-O: |
200 mg |
5.1 gm |
931 |
|
Gelled dessert: |
62 mg |
0.8 gm |
956 |
Easy Apple Salad
Ê For the whole family
|
5 apples, peeled & diced |
2 c. mini marshmallows |
|
1 large canÊ
(27 oz.) pineapple, ÊÊÊ crushed
or chunks |
2 c. Cool Whip |
If apples are tart, sprinkle with sugar.Ê Drain pineapple and mix with other
ingredients.Ê Chill.Ê (Add ¸ c. nuts for family portion.)Ê Serves 10.
|
Per serving: |
57 mg. leucine |
0.9 gm protein |
178 calories |
Orange Julius
|
1/3 c. (2 oz.) frozen orange juice ÊÊÊÊÊÊÊÊ
(undiluted) |
¸ t. vanilla |
|
¸ c. non-dairy creamer |
6 ice cubes |
|
¹ c. sugar (white or brown) |
¸ c. water |
Blend in a blender until thick and ice cubes are
chopped fine.
|
Entire recipe: |
69 mg. leucine |
0.6 gm protein |
518 calories |
Cool Summer Dessert or
Salad
|
1 lettuce leaf |
¹ c. Cool Whip |
|
1 pineapple ring |
Maraschino cherry |
Place pineapple ring on lettuce leaf.Ê FillÊ
with Cool Whip and top with cherry.Ê
Makes one serving.
|
45 mg leucine |
0.6 gm protein |
68 calories |
Helpful Hints
ð Virginia Schuettâs new Low Protein Bread Machine
Baking For PKU is a comprehensive cookbook for bread bakers.Ê Available from Dietary Specialities.
Dietary Specialties
MenuDirect Corp.
865 Centennial Ave.
Piscataway, NJ 08854
Phone: 888-636-8123
ð The Wel-Plan Baking Mix is used in making yeast
breads, and other mixes do not work as well.Ê
However, in the fall â93 issue of the National PKU News, Virginia
Schuett mentions the experiences of several ãveteranä PKU bakers.Ê These bakers say the dp Baking Mix from
Dietary Specialities makes muffins, quick breads and cakes more moist and
richer in flavor.Ê Some use plain wheat
starch for cookies.Ê The Wel-Plan Baking
Mix is more expensive, so you may want to experiment.
ð Verna (Mrs. John Burkholder) has a daughter with
MSUD who loves to eat.Ê To keep up with
her appetite, and supply her with plenty of low protein, high calorie food, she
makes a double recipe of low protein cake, cuts it into squares, and wraps them
for freezing.Ê Each day she takes out
the squares she needs for that day, cuts each square in half horizontally and
puts back together withÊ frosting.Ê Her daughter also likes rice cakes cut in
half, loaded with margarine, and put together as a sandwich.
ð Since low protein baking is expensive, parents are
reluctant to try recipes.Ê The children
may not like it, and it is wasted.Ê Try
cutting baked goods such as breads and cakes into serving sizes, wrap real well
in plastic wrap and then in aluminum foil and freeze.Ê Divide casseroles into small servings and freeze.Ê TheyÊ
keep well and you can give your child the opportunity to try the product
over a period of time.Ê Serve small
helpings to your child when the family has a similar item.
FAMILY
HISTORIES
As I finished reading ãNickâs Storyä in the Dec. â93
issue of the Newsletter, I felt compelled to write for the first time since
Brock was born.
Brock Douglas Wiles was born April 21, 1979 in
Jamestown, NY.Ê Iâd had my new son home
for less than 24 hours when the doctorâs office telephoned me.Ê There is no need to explain the shock,
disbelief and pain I faced that day, as all of you parents have felt the same.
Childrenâs Hospital in Buffalo, New York was our home
for the next three months.Ê Feeling at
home (in the hospital) was difficult.Ê I
had a 13 month old at home with Grandma from whom I hated to be separated.Ê Three months was like three years!
Brock has classic MSUD, strictly limited to 750 mg of
leucine per day.Ê What a challenge!Ê (I needed the insight to buy into a
McDonalds 14 years ago; Iâd be wealthy today!)Ê
His diet is potatoes, potatoes, and more potatoes, fruit, cereals and
Egg‑free Raisin Bread from Ener‑G‑Foods.Ê Brock dislikes vegetables, except corn.Ê His formula is Maxamum MSUD mixed with fruit
punch and water to yield 12 ounces per day.Ê
He drinks 4 ounces three times a day, followed with a glass of water, as
this mixture is very concentrated.
Brockâs most recent hospitalization was this past
Christmas.Ê He caught the flu complete
with vomiting and diarrhea, leading to rapid dehydration.Ê Brock begins to suffer from hallucinations
and difficulty with his sight when he becomes ill.Ê IV fluids are generally the ticket to prompt recovery.Ê Fortunately, hospital stays are far less
frequent than when Brock was younger.
Like Nick, Brock suffers from tendon tightness and
cramping.Ê He remains small at 95 pounds
and 5 feet even.Ê He suffers from
frequent cold sores and sores that crack at each corner of his mouth.Ê He has many hangnails and his finger
cuticles are generally red and cracked.Ê
He has an excess of ear wax which needs to be cleaned out frequently as
it drains.Ê Ear flushes are completed
two or three times per year.
Brock has ADHD along with a few other learning
disabilities.Ê He (again like Nick) is
not highly motivated, could care less about homework, assignments due, or
tests. To study at home is a major task.Ê
MTV, Nintendo and music are his favorites.Ê He can whip anyone at any video game, knows the words to most rap
songs, and can describe any music video in detail.Ê No trouble with motivation there!Ê He, too, loves sports, but energy levels are depleted too rapidly
to participate much.Ê Basketball seems
the sport best tolerated by him.Ê I wish
school grades were as high as his memory of football plays!Ê He remains in the 7th grade (mainstreamed)
with 45 minutes of Resource Room per day.Ê
Organizational skills are poor, which is very frustrating for the person
who is organized÷patience is not one of my virtues.
We are not yet worried about the future; we
concentrate on weight gain, school grades, and controlling the sassy mouth that
is common of most 14 year olds.Ê (This
story was written before his 15th birthday.)Ê His poor impulse control and vivid story telling make it
difficult for Brock to make lasting friendships.
He is looking for new friends, especially those that
can relate to this illness.Ê He loves to
write letters and would love to write to anyone who takes the time to write
him.Ê He sent out a few letters in the
past month attempting to reach out.Ê (HeÊ sent one to Nick just a week prior to
receiving the Newsletter with Nickâs story).
Brock is a truly special young man.Ê He requires firm limits and constant
guidance, enjoys testing those limits and resists guidance, but at the end of
the day heâs the first one to kiss you good night, give you a hug and express
his love in front of anyone present!Ê A
true gift from God!
÷Celeste C. Wiles-Battle
We were in Pennsylvania for the
holidays when we received word that Tony Casey had passed away.Ê It was such a shock.Ê We like to think that persons with MSUD have
passed through the critical period of their lives by the time they are in their
late teens or surely by young adulthood.Ê
Tony was born on Nov. 1, 1973 and turned 20 just two months before he
died on the morning of the 31st of Dec., 1993.Ê
The Casey family had shared their
history in our Feb. â85 issue of the Newsletter.Ê Of their nine children, one son and three daughters died of
undiagnosed MSUD.Ê When her youngest son
Tony was born, Donna became uneasy by the 5th day.Ê Tony was notÊ feeding
well, just as the other four siblings had acted.Ê Because of her concern, the doctors admitted him to the
hospital.Ê He was diagnosed at nine days
of age.
In Donnaâs earlier account, she
relates what she said when Dr. Ampola told her about MSUD.Ê ãI have learned something that I figured I
would not know until I stood before our Lord and could ask him, ÎWhat was wrong
with all our babies?âä
Tony had the benefit of current
health care and did well.Ê He was a
highly motivated young man and well liked by those who knew him.Ê Following is an account of his sudden
decline and death written by his mother on Feb. 7.Ê We extend our deepest sympathy to the Casey family who has
suffered so much.Ê May their faith in
the Lord continue to strengthen them.
I have had a very difficult time expressing my feelings
on paper.Ê We are still trying to grasp
the reality of Tonyâs death.Ê We miss
him so much.Ê He was our life.Ê Everything seemed to surround him.Ê He kept us on the go and thinking young.
Tony graduated from high school last June and was
enrolled as a carpentry student at a vocational school in Augusta.Ê He loved school and never missed unless he
was sick, which wasnât often.Ê He also
loved country music and knew all the songs and the stars.Ê He was very outgoing and friendly and
everyone commented about his smile.
He belonged to the youth group at our church and was
to have gone to Long Beach, California on Dec. 27.Ê The youth group went to a gigantic youth rally there.Ê They had worked hard all year to raise money
to go.Ê We knew the week before that he
could not go as he wasnât feeling well.
On Sunday, Dec. 19, Tony was in an accident with his
brother‑in‑law.Ê He was
shook up and complained of a stiff neck.Ê
Otherwise, he was fine.Ê On
Monday, he ran a fever.Ê I took him to
the doctor that evening, who checked himÊ
and thought he had the flu.Ê On
Tuesday, he was no better, so they ordered an antibiotic.Ê By Wednesday, he was over the fever, but still
didnât feel good.Ê He refused to eat or
drink much and vomited after eating.
On Thursday evening he wasnât much better, so we
returned to the doctor.Ê We just got in
the office when he started to hallucinate.Ê
This was the first time he realized that he was hallucinating and he
started to cry÷something he never did before when he hallucinated.Ê I told him to hug me and it would soon go
away.Ê He did, and it did for
awhile.Ê They immediately put him in the
hospital and started an IV.Ê He got
upset with me once and pulled it out.Ê
So they sedated him and put it back in.Ê
By the next morning he was much better, and that evening, being
Christmas Eve, they let him come home.Ê
He vomited the French fries he had eaten.
By Tuesday of the next week, he was still not feeling
much better, so I called Dr. Ampola, our doctor in Boston.Ê She advised me to take him back to be
checked and have blood work done again.Ê
After checking him the doctors were sure it was sinusitis and gave him a
new antibiotic.Ê He was complaining of a
headache.Ê (He had problems with
frequent headaches and sinusitis.)Ê All
tests were normal.
Wednesday evening, Tony asked if I were going to take
him to the hospital.Ê I guess he thought
he should go back.Ê Thursday, my husband
was admitted to the hospital with flu and fever.Ê While I was at the hospital, the pediatricianâs office called and
asked how Tony was doing.Ê I called them
back and expressed my concern.Ê They
decided to check him again.
He took a bath and I helped him get dressed.Ê As I patted his hair dry he complained that
the top of his head hurt.Ê His right
eyeÊ looked droopy to me.Ê He was dehydrated and they put him in the
hospital again.Ê He walked into the
hospital and signed his own papers.Ê
They put in an IV, and he went to sleep.Ê I left him and came home.Ê
(My heart aches to write this because, I wish I could have been with him
when the Lord took him.Ê But I guess the
Lord knows how much we can handle.)
They told me later Tony continued to complain about
his head, and they put ice on it.Ê His
belly also hurt.Ê At 2:00 a.m. they
called the doctor and told him that Tony was complaining about his head and he
ordered Tylenol with codeine.Ê Then he
went to sleep.Ê The nurse checked on him
at about 5:30 and found he was in trouble, (they didnât go into detail with me
about it), and they called a code.Ê It
was too late.Ê He died as they worked on
him.Ê One nurse who was with him and
knew him personally took it so very hard.
My husband came home from the hospital Saturday.Ê He really wasnât ready, but the doctor knew he needed to come home.Ê They performed an autopsy on Tony and we donated his eyes,Ê heart valves, and some of his bones.Ê It was a very hard thing to do, but we know he no longer needed them.Ê He has a perfect, whole body now, and of course, we always say÷now he can have his hamburgers.Ê The au