M S U D Newsletter
Articles selected from Vol. 16 No.1,
Spring/Summer 1998
The
Norman Burkholder family lives about a 2 hour drive northeast of Lancaster,
Pennsylvania.Ê The Burkholders are an
Old Order Mennonite family with eight children.Ê Their daughters, Kathryn and Ellamae were born with MSUD.Ê Kathryn was born in 1988 and Ellamae in
1992.Ê Mabel, their mother, heard that
some of the children with MSUD had bad teeth.Ê
Their local preventive care doctor suggested giving supplements of
vitamin A to maintain healthy teeth and improve Kathrynâs immunity.
Over
the next several years, Mabel gave Kathryn vitamin A in doses she assumed were
safe.Ê However, vitamin A in excess is
toxic.Ê When Kathryn was around 5 or 6,
she had toxoplasmosis twice which seemed to have caused her spleen to
enlarge.Ê Her liver was also enlarged at
times.Ê Her metabolic doctor, Dr. Holmes
Morton, was concerned and ran various tests.
Tests
showed a low level of vitamin A in her blood, so vitamin A toxicity was not
identified until early in 1996 when Kathryn was hospitalized for five
days.Ê She was in the end stage of liver
failure.Ê The family now faced the issue
of a liver transplant.Ê Before the
family made a decision, the doctor learned that the low blood levels of vitamin
A were masking a vitamin A toxicity.Ê
Knowing the cause, they hoped that the liver could recover from the
toxicity.
I had
hoped to have a medical account of Kathrynâs liver failure and transplant to
print with this article, but it was not available.Ê However, the transplant will be covered at the upcoming Symposium
â98.Ê Phone conversations with Mabel
provided some of the preceding information.
On January 2, 1997 we were on
our way to the Clinic for Special Children for a check up with Dr. Morton when
Kathryn vomited blood.Ê We didnât
recognize the brown ãcoffee groundsä as old blood, but our driver did.Ê Dr. Morton checked her leucine levels and
then sent us to the Lancaster General Hospital.Ê Our daughter Kathryn was in acute liver failure.Ê Dr. Morton fought for her life for several
days.
She was bleeding from a
ruptured vein in her esophagus.Ê They
gave medication to quickly rid her intestines of blood because the blood was
being digested.Ê According to Dr.
Morton, the leucine content of the blood was about equal to the amount of
leucine ingested when eating 3/4 of a hamburger, which caused her leucine
levels to reach 24 mg/dl.Ê She was
dizzy, groggy-like, with her eyes only half open the first day or two.Ê To stop the bleeding that first night, they
put ice water in Kathrynâs stomach through an NG tube and drew it out again to
help clot the blood.Ê It didnât
work.Ê So they took her to surgery and
cauterized the ruptured vein in her esophagus.Ê
The anesthesia made her groggy longer than normal.Ê The anesthesia along with her high leucine
level made it hard for her failing liver to work off all the toxins.
She wasnât allowed to have
any ãfreeä water by mouth that first night or the following day for fear it
would encourage brain swelling.Ê She was
given lots of saline in her IV which made her beg for water.Ê Dr. Morton thought he might lose her that
night.
The second day, she still did
not seem like herself.Ê I thought her
actions indicated brain damage, and I decided then weâll let her go and not do
a transplant.Ê That evening when Dr.
Morton came in, he told me I couldnât judge by what I was observing.Ê Her poor responses were due to the toxins
still in her system.Ê It was taking
longer to work off the toxins because of her failing liver.Ê Her leucine was no longer elevated and was
not affecting her.Ê So the whole turmoil
of indecision about a liver transplant started up again!
The half year before this
episode÷after discovering her liver failure÷Dr. Morton was trying to balance
the amino acids to keep her MSUD under control.Ê He did not give a bit more than necessary, so her liver wouldnât
have to work with an excess of amino acids.Ê
Two companies supplied individual amino acids which Dr. Morton used to
make a formula.Ê He used Farm Rich, a non-dairy
creamer, and the mix of amino acids he thought best.Ê He tried to eliminate all Vitamin A from her diet in order
to reverse the liver damage.
After this big bleed, Dr.
Morton was afraid Kathryn might not make it much longer.Ê He wanted us to decide whether weâre going
to do a liver transplant.Ê When we went
home that night, I explained the situation to the family.Ê There is no chance of life as she is now,
and she would have a 65% chance with a liver transplant.Ê We asked our children what they think we
should do.Ê Melinda said, ãOh, give her
a chance.äÊ And Edwin, the one who often
fought with Kathryn, said, ãYes, do the surgery!ä
Well, we felt lost, and you
always think doctors may be making it sound worse than it is.Ê We didnât decide right away, and then she
ãleveled outä a bit÷stabilized.Ê The
third day, she actually understood her schoolwork again.
After we were convinced the
doctors were right about her chances, we said okay to the transplant.Ê But by the time weâd made a decision, she
was so much better and no longer an intensive care patient.Ê She wasnât eligible for the number one
category anymore.Ê If we had made our
decision the first night, she might have had a new liver in 24 hours, Dr.
Morton told us.
With the decision to do
surgery, I prayed that God would take her before the surgery if the new liver
was not going to be a good match, or if we should not do the transplant at
all.Ê The way Dr. Morton talked it
seemed her life was very much ãon the edge.ä
We realized later if we would
have decided to let her die, her stay in the hospital might have been even
longer.Ê She couldnât eat because of the
pancreatitis and Dr. Morton didnât think she would have been able to eat again.
We are also glad we didnât
have to do the transplant then.Ê We have
heard since of other persons who were given a mismatched liver when it was a
life-and-death matter and later needed another transplant.
During the month and a half
that Kathryn was in the hospital before the transplant, she developed
pancreatitis, so she had to stay on an IV the whole time.Ê The back pressure caused by the blocked
liver created pressure on her pancreas and lungs.
Kathryn had low oxygen
saturation for a year or so before this episode.Ê Pressure built up from the blood not being able to go through her
liver freely and created shuntings in the lungs.Ê The blood actually bypassed some of the oxygen-making places in
the lungs, going through the little shuntings created by the extra
pressure.Ê That same pressure caused varicosities
(varicose veins) the thickness of an index finger all the way down her
esophagus into her stomach.Ê Dr. Morton
had been afraid of having those veins rupture during the half year that we
tried to reverse the toxicity of the liver but Iâd forgotten he said that.
She was coughing so much the
last week before her big bleed, coughing hard every time she drank her
formula.Ê She wanted it quite warm and
asked for ãquick waterä (water quickly) after her formula to keep her cough
from making her throw up.Ê We realized
later anything cold probably aggravated those big veins in her throat
dreadfully.Ê But since Iâd forgotten
about the possibility of varicose veins, I didnât tell the doctor about this
reaction right away.
Being on IVâs for a month
made her liver worse, so her pancreas didnât get better either.Ê Her eyes got very yellow in the
corners.Ê Some days she was better and
we almost had hopes of bringing her home.
Iâm glad now that I didnât
know that Dr. Morton believed she would never be able to eat food again without
having a new liver.Ê Knowing this, and
that being on the number two list for a liver transplant meant it could take
half a year before a liver was available, would have been quite
depressing.Ê I believed she would get
better and could go home.
The third week in January she
was taken to Philadelphia by ambulance (without sirens) to have an
evaluation.Ê After one week at
Philadelphia, she was back at the Lancaster Hospital which felt like home by
now.
During this time Kathryn was
on IVs which advanced the liver failure, and Dr. Morton kept fearing another
bleed.Ê This would cause very serious
problems with her MSUD.Ê He tried to get
the officials from the transplant center to hasten the transplant.Ê The officials finally said they would
discuss metabolic patients getting priority status at their next meeting.
Before two weeks were up,
Kathryn had another bleed.Ê It was not
as scary or as big a bleed as the first time, but, by needing intensive care,
it put her back on the number one list for eligibility.Ê So we rushed Kathryn to Philly, sirens going
this time, and admitted her to ICU in the Childrenâs Hospital of Philadelphia
(CHOP).Ê To me it was ãjust a
comfortable little bleed,ä even though she did pass out from toxins, etc.
As we entered CHOP, I was
scared to be without Dr. Morton for fear Philly doctors would miss something
with her complicated case.Ê After being
there almost a week, they found a good liver.Ê
The first liver theyâd prepared for her was turned down by Dr. Shaked,
which increased my faith in him.Ê He
knew that many doctors, nurses, etc. had been working towards a transplant, and
yet he was able to refuse the liver because it had abnormal routings, and
Kathryn was stable enough at the time.Ê
He couldâve used it if sheâd been too sick.Ê However, the extra rerouting of tubes to connect that liverâs
abnormal routings to Kathrynâs could have caused more chance of rejection.Ê Weâve been relieved often thinking about
it.Ê She barely had any rejection
with the liver they found for her three days later.
The week before her
transplant, while Kathryn was in the ICU, she got so much better that Dr.
Morton was afraid sheâd be taken out of ICU and put on the regular floor.Ê That would put her on number two status
again.Ê Then he wouldâve had to struggle
to get her through the next big bleed.Ê
We believe God saw to it that there were little problems that week in
ICU÷enough to keep her in the ICU and on the number one list for a liver.Ê Kathrynâs insulin and glucose levels
fluctuated, and another bleed one night caused her leucine levels to elevate to
14 mg/dl again.Ê When I got uneasy about
something, Iâd call Dr. Morton.
Kathrynâs liver came from a
small 22 year-old woman on Feb. 12.Ê
They had prepared to give an adult in an adjoining hospital the right
lobe of the liver and Kathryn the left (smaller lobe).Ê However, the liver was small enough to fit
the complete liver into the cavity from which Kathrynâs failed liver was taken.
Before the hallelujah of
coming home, we had many ups and downs in the ICU and later on the seventh
floor.Ê For five whole weeks we
experienced both anxiety and hope after surgery.Ê Reading back over the diary I kept, it now looks like this was
the biggest cliff we plunged off yet.
When we had questioned Dr.
Morton about the shuntings and the low saturation problem, he said it would
reverse itself immediately after the liver transplant.Ê But Kathrynâs oxygen saturation remained low
after the transplant.Ê Dr. Mortonâs
explanation made sense to me÷when they used hyperalimentation (TPN by IV)
after the surgery, the new liver was filled, making it larger which caused
slight pressure on her lungs, enough to keep the shuntings in her lungs open.
His theory proved
correct.Ê When IV intake was decreased
in preparation for going home, her oxygen saturation jumped from 60 to
80%.Ê In a week and a half after being
released from the hospital, they tested 99%.Ê
Relief!Ê We thought that
problem would vanish immediately after surgery, but it took a month!Ê Now no more purple lips and fingernails and
a normal flesh-colored face for Kathryn, just like other children!Ê No more getting short of breath from walking
or playing.
Kathryn was discharged from
the hospital on March 21.Ê After Kathryn
was at home, she started eating better.Ê
Sheâd been unable to eat for one and one-half months before the
surgery÷maybe thatâs why it took awhile for her appetite to return.Ê Or maybe because of so many
medicines.Ê Anyway, sheâd started eating
ham roll and other meats quite heartily about one month before her two months
of tummy aches started.
After she started having
tummy aches, Dr. Morton thought it might be an ulcer, so we tried to get
scheduled for an endoscopy.Ê I guess
other parents wouldâve said, ãHere we come, she needs to be hospitalized.äÊ But we struggled to feed her for four weeks
until she finally got scheduled for an appointment.
By that time, I was afraid
she was dehydrating from throwing up several times a day.Ê She would lie on the sofa with tummy ache
for hours at a time, sometimes throwing up tiny clots of blood.Ê I was afraid her stomach would rupture and
hemorrhage if we let it go one more weekend!Ê
Finally they checked her at CHOP and kept her.
The doctors diagnosed an
ulcer with lymphoma around the ulcer÷a drug-induced lymphoma they said.Ê So she was given acyclovir by IV.Ê In Philadelphia, they said if they canât
heal the lymphoma by withholding the Prograf, they would use chemotherapy.Ê (Prograf is an immunosuppressant drug to
prevent the body from rejecting the liver.)
In the meantime, I was in
contact with friends who had been treated in a hospital in Pittsburgh.Ê The doctors in Pittsburgh were much more
experienced with lymphoma.Ê Philadelphia
had 18 cases, but Pittsburgh had probably a hundred or more.Ê Pittsburgh treated all their cases without
chemotherapy.
We were at CHOP for 2 ¸ weeks
(Aug. 12 to 28, 1997) and at home with an oral acyclovir for the lymphoma for
1¸ weeks.Ê To heal the lymphoma, they
had to withhold the Prograf for as long as the liver didnât show signs of rejection.Ê When Kathryn was released from CHOP, she
tested positive for the Epstein-Barr virus, so they changed the oral acyclovir
dosage to a therapeutic level every 8 hours plus one dose at nighttime.Ê Kathryn went to the first week of school
with some tummy aches.
Dr. Reyes from Pittsburgh
said their research shows that oral acyclovir is worth almost nothing in
treating the Epstein-Barr virus.Ê It
seemed to prove him right when by the end of the week at home, Kathrynâs tummy aches
made her cry once or twice a day again.Ê
By then we had scheduled a trip to Pittsburgh to give her a thorough
check up and get a second opinion.
Well, the second opinion
turned into another 2 ¸ weeks stay at the Pittsburgh hospital (Sept. 8 to 25,
1997).Ê But she was the healthiest child
on the floor and to her it was a ãjoy ride.äÊ
We felt like the luckiest parents on the floor.Ê We also came to realize that no matter what
hospital you are in, Epstein-Barr virus and lymphoma in a transplant patient
are very hard to treat correctly.
One boy at Pittsburgh had the
same lymphoma as Kathryn.Ê He had been
treated in Boston first with chemotherapy which actually made the lumps
grow.Ê We felt heaps of relief that we
went to Pittsburgh before Kathryn was given chemotherapy.Ê I sincerely thank the three hospital staffs
for doing all they could for Kathryn and us.
Two days after Kathryn was
admitted to the Pittsburgh Childrenâs Hospital, they replaced the oral ayclovir
with IV gancyclovir.Ê On her fourth day
there, they put a scope down into her stomach and discovered CMV, a virus that
isnât touched by acyclovir.Ê Can you
imagine the relief we felt that it was discovered!Ê We were very glad we had sought a second opinion.Ê Now we were confident that putting her in
the hospital, even though she seemed much better, was the right move.
We had been afraid the
doctors would miss just one little thing that would make all the
difference.Ê Pittsburgh had a great deal
of experience with liver transplant follow-up and we liked the way they treated
aggressively.
We also heard of a child that
was given only one lobe of liver and later got leakage of either blood or bile
causing infection in the intestinal cavity.Ê
That makes us glad that God directed it all and Kathryn got a whole
liver (which is not the usual).
Right now Kathrynâs at home
and very healthy.Ê Just Friday morning,
it amazed me again that she can actually jump on her bike and easily make it up
the little hill past our barn.Ê For the
last few years, others had to use a wagon or cart to take her out the lane to
the school bus stop.Ê Sheâs actually
chubby now (without any Ensure or IV) and back to liking some meats.Ê While she had tummy aches, she preferred all
low protein foods÷because protein is harder on a sick tummy?
She still likes MSUD formula
on her cornflakes and likes Cremora ãcheeseä sandwiches made with rusks, or
rusks buttered and browned on both sides.Ê
She prefers low-pro bread to ours (even though the last loaf I made is
coarse and hard) and sometimes helps Ellamae eat her low protein noodles or
soup.Ê Tomato soup is still better her
way (Cremora instead of milk).Ê But I
have to think, which one of us could change from eating our cornflakes with
milk to eating them with MSUD formula!Ê
It is the same for her÷it doesnât taste right with our milk.
We want to thank the many
people who supported us with cards, letters, phone visits, prayers and
financial support.Ê All of these helped
us get through this ordeal.Ê Looking back,
it looks almost impossible to have stood it all without completely
collapsing.Ê Our thanks to everybody,
especially God.
÷Mabel Burkholder
UPDATE:
Kathryn
missed a lot of school and was so miserable before her transplant.Ê Now she loves school and her learning is
much improved.Ê She is nine and
repeating the first grade.Ê Because of
all the attention she received, she does have a problem with temper tantrums
which the family is dealing with.
Kathryn
enjoys her baby brother, born on October 29, 1997.Ê He has MSUD, so the family still has two children on the MSUD
diet, baby Norman Jr. and Ellamae, 5.Ê
Kathryn is gradually giving up her interest in her old diet and
adjusting to being able to eat any foods.Ê
However, she still likes to eat some low protein foods made for her brother
and sister and drink a little formula when she gets a chance.
Baby
Norman has been a healthy baby and weighs 17 lbs. at 4¸ months.Ê He was tested for MSUD within 24 hours of
birth and started on the diet on the second day of life.Ê He has had only one hospitalization÷an
overnight stay at the Lancaster General Hospital.Ê At that time he had an asthma-like tightness that was treated
with a nebulizer.Ê It seemed he got
tight from the slightest breeze or a cold room, but only when his leucine was
too low. ÊHe gained weight so fast the
first three months that his leucine level was often way too low.Ê His mother started sending blood on filter
paper to Dr. Morton by overnight Fed-Ex twice weekly at times.Ê This helped.
Although
Kathryn is doing really well, a liver transplant is not the cure-all for
MSUD.Ê It is a risky procedure and very
expensive.Ê The cost for Kathrynâs
family, who has no medical insurance, was over $500,000.Ê Costly anti-rejection medicine and follow-up
treatment will continue throughout Kathrynâs life.Ê After Kathrynâs transplant, the local community pitched in with
fund-raisers.Ê One auction raised
$27,635 for Kathrynâs medical fund.
This
family had many exhausting challenges in the last couple of years but through
their experiences, many persons have become aware of MSUD.Ê Medical research has been advanced.Ê Kathryn is enjoying life.Ê The Lord has brought blessing out of
adversity.
IMPROVING
PKU [MSUD] DIET COMPLIANCE
by Teresia Goldberg,
MS, RD and David Pelcovitz, PhD.
The
following article is reprinted with permission from the National PKU News,
Vol. 7, No. 3., Winter 1996.Ê Although
written specifically for the PKU population, the article has much good advice
for those dealing with MSUD diets.Ê Just
think MSUD instead of PKU.
Ask any parent what the
greatest challenge of the PKU diet is and the likely answer will be ãthe
medical food.äÊ Some children may begin
to refuse the ãmilkä from an early age.Ê
The problem may last only a few days, or can go on for a long time.Ê The struggle that begins can negatively
affect relationships between parents and other family members as well as the
childâs metabolic control.Ê To help
families with this and other compliance problems, we invited Dr. David Pelcovitz,
Chief of Child and Adolescent Psychology at Cornell University Medical College
to speak to our Metabolic Center parent support group at North Shore University
Hospital in Manhasset, New York.Ê We
later put the ideas into a paper from which this article is drawn.
(Pelcovitz, D., and Goldberg,
T., Enhancing nutrition compliance in children: Inborn errors of metabolism as
a paradigm.Ê Topics in Clinical
Nutrition, 10 (2): 73-81, 1995.)
To prevent power struggles
over feeding, parents should have an attitude of calm control.Ê But this is not easy when managing children
with PKU.Ê All parents know that not
following the diet can lead to serious consequences.Ê This makes it difficult for parents and other care givers to take
a ãlow-keyä approach when the medical food or other foods are refused.Ê Also, most problems related to poor
compliance are not seen immediately, adding to the difficulty.Ê For children, who by nature think in
concrete terms, the lack of immediate feedback is especially difficult.Ê In this article, we will make specific
recommendations to help diet compliance.
When the child begins to eat
table foods, the diets of children with PKU noticeably differ from those found
in normal meal patterns.Ê Not only must
they drink adequate amounts of the medical food, but they must avoid eating
many of the foods eaten by family and friends.Ê
Although there are a variety of modified foods, their appearance and
taste can differ somewhat from those of their regular counterparts.
The potential for noncompliance
begins when children reach an age where they start to have a say in what they
eat.Ê It is very normal for toddlers to
get into power struggles.Ê At this age,
almost all parents report significant problems in properly managing their
childâs diet.Ê In a European study of
eleven children with PKU, parents ranked the diet as their most difficult
problem.Ê At our metabolic center, eight
out of ten families of toddlers report becoming involved in intense struggles with
their children over drinking the medical food.Ê
Feeding problems in these children have been reported as early as
eighteen months of age.
Children of that age
typically are dealing with their beginning independence.Ê It is not surprising that the child with PKU
may now begin to refuse to drink the medical food.Ê Families may use various techniques to help this problem,
including concentrating the medical food, flavoring it, using reward systems,
etc.Ê Battles may continue for days or
weeks at a time, occasionally ending in forced feeding or noncompliance.Ê At this age, there is also increased
exposure to table foods, providing more opportunities for power struggles over
what and how much is eaten.
Donât be overindulgent
Parents may have strong
emotions due to having a child with a metabolic disorder.Ê These emotions may include feelings of guilt
or pity because of diet restrictions.Ê
The feelings may result in parents having difficulty setting limits.Ê Remember that the youngster you are dealing
with is a child first, and a ãpatientä second!Ê
The scientific literature on children with chronic conditions describes
the danger of responding to them by becoming more indulgent, or by placing
fewer demands on them.Ê A cycle may be
set in motion.Ê The child wants to gain
reassurance that he or she is like everybody else and tries to get care givers
to ãnormalizeä disciplinary practices.Ê
This would prove the child is no different from siblings or friends.
For the child with PKU,
eating is often the arena where such battles are fought.Ê As a parent, you may need help getting in
touch with underlying feelings of guilt and self-blame and in finding positive
way of dealing with them.Ê Do not
apologize for the restrictions to your child or to others in the presence of
your child.Ê Recognize that consistent regulation
of your childâs diet is an act of love, even in the face of tears and
angry rejections.
A related problem is the
tendency for some parents and care givers to encourage unusually high levels of
dependency in the child.Ê Concerns
related to possible consequences of having PKU often result in
overprotectiveness.Ê Children with PKU
have been shown to be more dependent than non-PKU adolescents.Ê Although the high levels of concern are
understandable, these feelings may set the stage for heightened rebellion.Ê Conflicts over diet limits are a likely
battleground in the struggle of the overprotected child to reach
independence.Ê The important thing is
not to ãbabyä your child.Ê Toddlers
should be weaned to a cup, be expected to self-feed and be treated at the table
in a way that is appropriate for the childâs age.
Avoid power struggles
Recent research, including
children with diabetes, shows that when parents display a high level of
expressed emotion there is more likely to be poor diet compliance.Ê Examples of ãexpressedÊ emotionä are criticism, overprotectiveness
and intrusiveness.Ê But when care givers
dealing with the children are helped to become more calm, consistent and
supportive, theÊ situation
improves.Ê Unfortunately, having a child
who can have serious problems because of diet noncompliance is a situation that
can easily lead to high levels of expressed emotion.Ê For healthy children, guidelines for eating are straightforward.Ê Power struggles are avoided by care givers
taking responsibility for when and what the child eats, but the child taking
responsibility for how much and even whether he or she eats.Ê Care givers of young children with PKU
cannot afford the luxury of their children deciding the quantity or even the
timing of the food they consume.
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ÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊ Recommendations for
Improving Diet Compliance IssueÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊÊ Associated ProblemsÊÊÊÊÊÊÊ Recommendations
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Child psychologists have
found that when parents are in situations where they have little control, they
are likely to deal with their children in a way that is characterized by high
levels of emotion and criticism, and low levels of praise.Ê But studies show children eat less,
not more, when care takers become too active.Ê
Children quickly learn to manipulate theirÊ care giversâ desperation to get them to eat.Ê (Statements like ãDo mommy a favor and drink
thisä are counterproductive.)Ê Once the
child realizes his parents are no longer emotionally invested in all his or her
actions, cooperation will improve.
One very important thing that
parents can do to reduce power struggles is to give the child the feeling that
he or she has some control over a situation that is restrictive by nature.Ê Whenever possible, parents should offer the
child an element of choice.Ê For example
ãYou can either take your drink from the red mug or the blue cup.äÊ Also, children can be allowed some control
overÊ preparation of their food and the
medical food.Ê For example, for the
medical food, the child can be given a choice of flavorings (chocolate,
strawberry, etc.), of consistency (how much water is added) and of temperature
( room temperature, cold or even frozen).
Coordinate teamwork
It is not uncommon in
families of children with chronic conditions for one parent to become
over-involved with the child while the other takes a very minor role.Ê Most often, mothers are intensely involved
in the day-to-day care of their child while fathers flee into the world of
work.Ê More equal distribution of
responsibility of care givers may be a crucial factor in improving
compliance.Ê Sometimes, a motherâs
over-involvement adds to the emotional intensity of the situation.Ê Her expressed emotion becomes greater and
power struggles increase.Ê If one parent
has the burden of exclusive responsibility for the diet, the level of stress is
great for that parent.Ê A study of
families of children with PKU has shown that parental cooperation and family
cohesiveness are important for dietary adherence.Ê Often the parent who is less emotionally caught up in the childâs
diet may be the preferred parent to supervise the childâs diet precisely
because of the greater detachment.
Consider a reward system
Formal reward systems at
times have their place in PKU management.Ê
Use of stickers and other reward systems can jump-start a stalled
situation marked by a power struggle.Ê
However, care givers need to be careful that the child does not view
such rewards as a subtle form of pressure.Ê
In many studies the children who were rewarded for trying new foods
showed less enthusiasm for the food than children who were not rewarded.Ê If after a short trial you find the use of
rewards causes increased resistance or conflict, simply stop the rewards.Ê If you or the clinic decides that a reward
system is worth trying, it is important to make sure that the reward is
age-appropriate, the chosen item or privilege is one that the child can get
excited about, and it is realistic for the parent to give the reward
quickly.Ê Some examples of non-food
rewards which have proved effective for preschool-age children include extra
bedtime stories, stickers, puzzles and small toys.Ê If a longer term reward is appropriate, a trip to the park or zoo
could be used.Ê Children ages six to
twelve can be offered baseball cards, later bedtimes on a weekend, extra time
alone with a parent, or a chance to attend a concert or sporting event.Ê Consult with your PKU clinic if you have
difficulty carrying out a reward system.Ê
The clinic should make recommendations based on family dynamics, or work
with you and a mental health professional to carry out a plan to deal with the
problem.
DIET WISE
Low Pro Pretzels
Submitted by Esther Stauffer
|
3 c. (454 gm) wheat starch |
2 T. (14 gm) yeast |
|
1 t. salt |
2 T.+1 t. (14 gm) methylcellulose* |
|
1 T. sugar |
1 3/4 to 2 c. (105-115¡) warm
water |
Mix dry ingredients adding enough water to make a soft dough.Ê Let rise 20 minutes.Ê Sprinkle work area with wheat starch and
knead dough 5 to 10 minutes.Ê Add more
wheat starch if sticky.Ê Roll out dough
to ¸ inch thickness.Ê Cut into strips
approximately 2¸ to 3 inches long and ¸ inch wide with a pizza cutter making
about 100 pretzel sticks.Ê Mix 2 cups
water with 4 teaspoons baking soda and bring to a boil.Ê When all the pretzels are cut, begin putting
the first-cut pretzel sticks into the boiling water for 5 to10 seconds.Ê (If you wait too long to wet them, they may
rise too much.)Ê Place pretzels on a
greased baking sheet and sprinkle with coarse salt.Ê Bake at 475¡ till brown.Ê
After all the pretzels are baked, lower the oven temperature to 225¡.Ê Lay baked pretzels across the
oven racks in the oven for 2 hours until crisp.Ê These pretzels are very good and are well worth the time and
work that goes into them. Yield: 100 sticks
|
|
Protein |
Leucine |
Calories |
|
Per recipe: |
7.0 gm |
522 mg |
1727 |
|
Per pretzel: |
0.1 mg |
5.2 mg |
17 |
Chocolate Mint Brownies
|
¸ c. sugar |
1 t. methylcellulose* |
|
¹ c. Miracle Whip |
1 t. baking powder |
|
2 T. cocoa |