M S U D Newsletter
Articles selected from Vol. 17, No. 1,
Spring/Summer 1999
The
New England Connection for PKU and Allied Disorders held their 1999Ê PKU Parent/ Professional Conference in
Tauton, Massachusetts on March 20.Ê
Sandy Bulcher represented our MSUD Family Support Group at the
conference. On the evening of the 19th, a group of persons interested in
newborn screening met to discuss issues relating to state screening programs.
Here Sandy reports on this meeting.Ê
(More information on the meeting on the 20th farther on in this issue.)
I've been interested in the
issue of newborn screening since our son Jordan was diagnosed with MSUD nine
years ago.Ê At that time, it was painful
to learn that had we lived in one of the 21 states that screen for MSUD, Jordan
would not have had to suffer 17 days before a right diagnosis was made.Ê Over the last few years, I have been trying
to have MSUD added to Ohio's newborn screening program, but had never expanded
my thinking beyond that.Ê Fortunately,
Trish Mullaley, a PKU parent and former president of the New England Connection
for PKU and Allied Disorders, had a vision to improve newborn screening on a
national level.
Last fall, we started making
plans for the first gathering of parents and professionals from across the
country interested in newborn screening.Ê
We were able to see this vision become a reality on Friday, March 19,
1999 in Taunton, Mass.Ê Approximately 25
parents and professionals met to discuss flaws in our current newborn screening
system, as well as the need for expanded screening nationally.Ê Parent representatives from the following
organizations attended.
PKU of Florida
PKU of Illinois
Children's PKU Network (California)
Mid Atlantic Connection for PKU
PKU Organization of Wisconsin
Ohio Coalition for PKU and Allied Disorders
New England Connection for PKU and Allied Disorders
MSUD Family Support Group
Parents of Galactosemia
Homocystinuria (family representative)
Organic Acidemia Association
In addition to these parent representatives,
professionals from several formula companies, a low protein food company, and
two newborn screening companies were present.Ê
We were honored that Dr. Richard Koch from California took interest in
our group and attended.
I've learned a lot since researching the issue of
newborn screening.Ê First of all, even
though all 50 states and the District of Columbia screen for PKU and Congenital
Hypothyroidism, this does not necessarily detect all these infants, and
numerous infants are missed annually.Ê
Frequently, the problem occurs because of the lack of education, poor
communication, or inadequate follow-up.Ê
We discussed numerous ways to address these problems including printing
educational materials, developing a Web site, and seeking the support of
organizations such as the National Institutes of Health and Center for Disease
Control.
Except for PKU and Congenital Hypothyroidism, states
vary in the diseases for which they screen.Ê
(Only
twenty-one states screen for MSUD at this time.)Ê In the last
several years, new technology has been developed, tandem mass spectrometry
(MS-MS), which has the capability to test for approximately 30 diseases
including MSUD.Ê Several states are
using this new technology for newborn screening in addition to their current
program.Ê All present at the coalition
meeting felt that promoting expanded screening through MS-MS would be
beneficial, in addition to addressing problems in our current system.
Also, everyone agreed to forming a national non-profit
organization called the National Coalition for PKU and Allied Disorders.Ê Initially, we will be looking at newborn
screening issues, but will expand into issues related to insurance coverage for
formula and low protein foods.Ê The
coalition will be set up so that a representative from each of the metabolic
organizations can obtain information and disseminate it to their local
organizations.Ê I'm really excited about
being involved with the National Coalition and feel confident that we can make
a difference.Ê I will keep you informed
of our progress.Ê Thanks to SHS, Wallac,
and Neo Gen for funding this first important meeting.
Sandy is involved in legislative
efforts to improve newborn screening in Ohio and on the national level.Ê ContactÊ
her for information: Sandy Bulcher, 82 Ravine Rd., Powell, OH
43065.Ê Phone: 740-548-4475 E-mail:
dbulcher@juno.com
Neo Gen Screening, Inc. is a laboratory specializing
in neonatal screening and the diagnosis of inborn errors of metabolism. The lab
has analyzed over a half million samples for inherited metabolic disorders
using tandem mass spectrometry and other biochemical genetic methods.Ê Other specialty and diagnostic testing
services are offered including urine organic acid testing and quantitative
amino acid analysis.Ê More than 30
clinically significant and manageable diseases that affect newborns are tested
for in their Supplemental and Comprehensive Newborn Screening Program.Ê
Automation and high volume testing in their routine
newborn screening laboratory allow them to offer programs and specialty testing
services at considerably lower prices than currently available in other
laboratories.Ê They will provide
additional information on request detailing their clinical and newborn
screening programs and the procedures for specimen collection, shipping and
handling, pricing and billing information.
Free screening packages are available for clinical
metabolic screening services.Ê The
package includes a Neo Gen Screening filter paper for collection of specimen, a
glassine envelope, a pre-paid pre-addressed envelope, a list of disorders,
instructions for specimen collection and order forms for additional ãfreeä
testing packages.Ê Screening is
available for 30 diseases at a cost of $25.
Contact: Neo Gen Screening, 110 Roessler Road, Pittsburgh,
PA 15220-1014.Ê Phone: 866-4636436Ê
Fax: 412-220-0784Ê E-mail: info@neogenscreening.comÊ Web site: www.neogenscreening.com
NEWBORN
SCREENING IN NEW ENGLAND
Harvey Levy, MD
Reprinted from the New England
Connection Update, March 1999, a newsletter of the New England Connection for
PKU and Allied Disorders.Ê This article
provides a brief history of newborn screening and explains the importance of
tandem mass spectrometry.
New England has traditionally been the center of
action in newborn screening.Ê In fact,
newborn screening really began in New England.Ê
Dr. Robert Guthrie (Buffalo, New York) had developed a newborn screening
test for PKU, and he knew that it could prevent mental retardation in thousands
of children with PKU.Ê However, the
medical and public health establishment in New York at the time opposed making
the test routine for all newborn infants.
Fortunately at a meeting in 1962, Dr. Guthrie met Dr.
Robert MacCready, Director of the Diagnostic Division of the Massachusetts
Public Health Laboratories.Ê Dr.
MacCready learned about the screening test from Dr. Guthrie, was convinced that
it would indeed prevent mental retardation in PKU, brought the test to
Massachusetts, and asked doctors throughout the state to obtain heel stick
blood specimens from newborn babies.Ê
This became the first newborn screening program in the world.Ê In 1963 Massachusetts became the first state
to pass a law requiring a PKU test in all newborn infants.Ê Other New England states soon followed
Massachusetts, and this was followed by still other states throughout the
country.
When Dr. Guthrie developed other tests for disorders
such as HCU, MSUD and galactosemia, he looked to Massachusetts and other New
England states to add these to newborn screening.Ê When I became involved in the Massachusetts newborn screening
program, New England had already become world famous as the center of newborn
screening activity.Ê This fame continued
until a few years ago when other programs began to use new methods that
expanded the number of disorders that could be identified and made the
screening much more efficient.Ê
By far the most important of these methods is tandem
mass spectrometry, often abbreviated MS-MS.Ê
This method was pioneered by Dr. Don Chace when he was at Duke
University and applied to routine newborn screening by Dr. Edwin Naylor in
Pittsburgh. Dr. Naylor has now formed a private newborn screening laboratory
known as Neo Gen Screening, Inc., where he and Dr. Chace are now screening
almost 200,000 babies a year by this method.Ê
They have shown they can identify many infants with important disorders
in addition to PKU and prevent disability or even death in these infants by
beginning early treatment made possible because of newborn screening.
Getting MS-MS adopted in New England, however, proved
to be a formidable task.Ê By this time
the individual New England states had decided to have a regional newborn
screening program with the screening performed in only one laboratory, the one
in Massachusetts.Ê All New England
states except Connecticut are part of this program; Connecticut preferring to
retain its own testing laboratory.Ê
Thus, advocacy for adding MS-MS to newborn screening had to center on
Massachusetts.Ê The metabolic doctors in
Massachusetts, including Dr. Mary Ampola, Dr. Mark Korson, Dr. Vivian Shih and
myself, vigorously fought to have MS-MS adopted here.
The matter was hotly debated within the Newborn
Screening Advisory Committee of the Massachusetts Department of Public
Health.Ê Fortunately with allies such as
Dr. Philip Reilly, Director of the Eunice Kennedy Schriver Center, and with the
very strong support of the Connection led by Trish Mullaley, we were able to
get the Public Health Department to pass new regulations that established MS-MS
screening on a 2-year pilot basis.Ê This
screening program began in Massachusetts on February 1, 1999.Ê We are confident that this expanded
screening will continue once its many advantages are realized.Ê We are also confident that the other New
England states will soon add MS-MS.Ê If
so, New England will once again become a world leader in newborn screening.
I cannot emphasize enough how important parents and
parent organizations are in this process.Ê As I have mentioned, the
Connection has been critical in helping the Massachusetts laboratory turn in
the right direction.Ê Now needed
is an effort to make newborn screening consistent throughout the U.S.,
so no longer will identifying a disorder in a baby depend on which state the
baby is born in.Ê The federal government
is finally beginning to take a role in this matter, but constant vigilance both
within individual states and at the federal level will be required to assure
appropriate final results.
Review by Joyce
Brubacher
Wayne and I attended the 1999 PKU Parent/Professional
Conference in Massachusetts in March.Ê
We met Dr. Richard Koch from Childrenâs Hospital in Los Angeles and his
wife, Jean.Ê Dr. Koch spoke on ãPositive
Results of Late Treated PKUä at the conference.Ê He has been very involved in treating PKU for many years. He and
Jean were very good friends of Dr. Robert Guthrie.Ê In the early 60s, Dr. Guthrie developed the bacterial inhibition
assay used for screening for PKU and MSUD.Ê
If it had not been for Dr. Guthrie, persons with MSUD would have a much
greater risk of damage and death today.
Jean Koch wrote a biography of Dr. Guthrie, Robert
Guthrie: The PKU Story.Ê She tells
the story of Bob Guthrie as a family man as well as a scientist.Ê The father of a retarded son, Dr. Guthrie
dedicated his life to the prevention of mental retardation.Ê His crusading spirit led him to present
programs throughout the world on the need for the early detection of conditions
leading to mental retardation.Ê In 1968
he took his wife and children on an adventure around the world, camping out
with their two VW buses in various countries, sightseeing and visiting medical
centers to encourage newborn screening.
Jean was kind enough to autograph my copy of her
book.Ê I have found the biography quite
interesting.Ê Our own interest in
newborn screening also started in the 60s when our son Monte was born in
Oregon.Ê He was the first infant in the
world with MSUD to be picked up through a state screening program.Ê Dr. Guthrie flew to the state of Oregon when
Monteâs second Guthrie test was confirmed positive.Ê Monte was making medical history, and Dr. Guthrie was the reason
it was possible.
It was many years later that we met Dr. Guthrie
personally.Ê Then in 1988, he and his
wife Margaret attended our Symposium at Hinkeltown, PA.Ê They stayed for the full symposium.Ê He took an interest in our support group and
was very encouraging.
Dr. Guthrieâs dedication to preventing mental
retardation was instrumental in saving the life and health of our son in 1965
and many others with MSUD since.Ê This
book reveals the real Dr. Guthrie and the many obstacles he overcame to
leave this contribution to mankind. I highly recommend it to those interested
in the history of newborn screening, medical history or general interest in a
dedicated father and scientist.
The book is available from your local bookstore or can
be ordered from:
Hope Publishing House
P.O. Box 60008
Pasadena, CA 91116
E-mail: hopepub@loop.com
Web site: www.hope-pub.com
GLEANINGS
FROM THE
NEW ENGLAND
CONNECTION CONFERENCE
Editorâs Observations
The New England Connection for PKU and Allied
Disorders held their 7th annual PKU & Metabolic Conference in Massachusetts
for parents and professionals on March 20, 1999.Ê Wayne and I attended and appreciated the presentations.
There are many similarities in PKU (phenylketonuria)
and MSUD.Ê One of the biggest
differences in the past has been in dietary control.Ê In the 60s and 70s, most clinics allowed those with PKU to go off
diet when they were around six years old.Ê
Only a few clinics thought it necessary to maintain the diet for life.
Today it is evident that those with PKU, who stop the
diet, gradually suffer the toxic effects of elevated levels of the amino
acid,Ê phenylalanine.Ê These problems have only recently been
recognized to be related to elevated levels in those who were early
treated.Ê Reports indicate lowered IQs,
mood alterations, impulse control disorders, migraine headaches, lack of
ability to concentrate, loss of short term memory, poor academic functioning,
inability to hold a job, muscle weakness, tremor and poor social and personal
relationships.
These problems vary in individuals with PKU.Ê However, those who go back on diet or
maintain the diet from infancy, find these conditions disappearing or greatly
improved.Ê There are adult persons with
PKU with high IQs and in professional positions.Ê Scott Merrill, an attorney at law, has PKU and was a master of
ceremonies at this PKU meeting.Ê He went
back on diet as an adult and found his academic abilities improved.
Can we apply this observation to MSUD?Ê I am hearing from adult persons with MSUD
who are having some of these same problems.Ê
Although these adults were on diet since infancy, in the 70s and 80s it
was not common practice to maintain leucine levels as low as recent evidence
indicates best.Ê Today improved
treatment, including the addition of isoleucine and valine to daily diets,
appears to help maintain lower and more controlled levels even in adults with
MSUD.
The make up of individuals certainly plays an important
part in the way high levels affect them.Ê
It is difficult to know for certain the cause and effect relationship of
physical and mental problems to MSUD.Ê
Which effects are related to the time treatment began or to lack of
dietary control or other causes?Ê There
are reports of headaches, sensitivities, body aches, tremors, social problems,
inability to reason adequately, excessive talking, poor academic functioning,
fatigue and weakness in muscles and joints.Ê
Some adults with MSUD may not experience any of these problems.Ê As with PKU, some individuals may not
recognize that their problem is similar to others with the same disorder.
At the PKU meeting there was a strong emphasis on
maintaining diet for life.Ê I would like
to also encourage very strict dietary control for all those with MSUD
regardless of age.Ê Donât compromise
future mental and physical health.
I am very interested in more documentation on the
effects of MSUD on adults.Ê I encourage
those with MSUD and their parents to contact me.Ê All information will be kept confidential.
ÊÊÊÊÊÊÊÊÊÊÊÊ ÷Joyce Brubacher
Imagine adults with PKU going back on diet and facing
the challenge of drinking the less-than-desirable tasting (to put it mildly J) medical formula for the major part of their diet.Ê As with MSUD, those on the PKU diet keep
wishing for more normal foods.Ê The many
new low protein products help but they cannot substitute for the medical
formula which contains needed protein.
One person responding to this plea for a more normal diet
is Dr. Bryan Hainline from the Department of Pediatric Metabolism and Genetics,
Indiana Medical Center, Indianapolis, Indiana.Ê
He spoke on ãDevelopment of a New High Protein Phe-Free Diet Supplementä
at the PKU Conference.Ê (Phe is the
abbreviation for phenylalanine, the amino acid involved in PKU and one of the
essential amino acids found in all protein.)Ê
Following is a summary taken from this speech and an article on the same
topic by Dr. Hainline in the PKU News (Vol. 10, No. 3, Winter â99
issue).
Dr. Hainline and his associates became interested in
developing a phe-free protein which can be added to low protein foods.Ê This would add a high quality protein to the
diet in the form of foods that can be baked and heated and have the protein
content of ãnormalä foods.Ê Commercial
companies could make high protein-low phe chips, breads, pastas, cookies,
etc.Ê By eating foods containing this
new protein, persons with PKU could reduce the amount of formula they need
because they would be getting some of their phe-free protein from these foods.
Using medical genetic techniques, they proposed to
remove the phenylalanine from a corn protein called gamma zein found in the
kernel of the corn.Ê This protein
contains 203 amino acids, two of which are phenylalanine.Ê They have already modified the gene that
codes for the production of gamma zein, allowing them to remove the two
phenylalanine residues.Ê Now they need
to improve the nutritional content of the protein and produce the phe-free
protein in a plant.
This naturally occurring gamma zein protein purified
from corn does not have a distinctive flavor so is expected to take on the
flavor of any food it is mixed with.Ê
Gamma zein is related to gluten, the protein in wheat that gives food
baked with flour much of its structure.Ê
Therefore, it has an excellent chance of improving the texture of low
protein foods.Ê To be effective, large
quantities of this protein would need to be eaten, so it is expected to be more
useful for older persons with PKU.Ê
Other nutrients found in the formula would have to be supplied with
vitamin/mineral supplements and additions of dietary fat and
carbohydrates.Ê The new foods could only
be used under medical supervision.
If this research is successful in producing a phe-free
protein, the underlying technology will be useful for designing similar proteins
for other inborn errors of metabolism including MSUD.Ê The projected time for this new protein to become available is
five years according to Dr. Hainline.Ê
However, he recently told me that a lack of funding is hindering the
research.Ê He is currently looking for
personnel to work in the laboratory.Ê We
can only hope the research in gene repair therapy discussed in the following
article is successful so we may not need genetically changed protein for MSUD.
NOTE: The
Scott Foster Metabolic Research Fund has supported Dr. Hainlineâs
research.Ê It is the type of research
for which the fund was established÷ research which can benefit a number of
metabolic diseases including PKU and MSUD.Ê
However, much larger sums of money are needed to make an impact. To
donate to The Scott Foster Metabolic Research Fund, send your donation to:
The Scott Foster Metabolic Research Fund
_ New England Connection for PKU and Allied Disorders
16 Angelina Lane
Mansfield, MA 02048
Phone: 508-261-1291
Dr. Blaese spoke on ãAre We Getting Closer to Gene
Therapy?ä at the PKU Conference.Ê
(Michael Blaese, MD is Chief of Clinical Gene Therapy Branch, National
Institute of Health and President of Kimeragen Molecular Pharmaceuticals.)Ê In the Fall/Winter â98 MSUD Newsletter,
we reported on the technology of gene repair therapy as explained by Dr. Blaese
at Symposium â98.
Dr. BlaeseÊ
explained the difference between this new gene repair therapy and the
traditional gene therapy.Ê Gene repair
actually corrects the gene code by using a chimeric molecule (the chimeraplast)
to harness the cellâs normal DNA repair system to correct the gene.
Certain diseases cannot be corrected, only those with
single cell mutations.Ê Dr. Blaese gave
a list of ten diseases that are targeted for this gene repair.Ê MSUD and PKU are both on this list.Ê However, the first disease scheduled for
human trials is Crigler-Najjar÷a very rare mutation in an enxyme required to
properly metabolize and eliminate bilirubin.Ê
The bilirubin levels cause organ damage and the child often dies in the
second year of life.
I asked Dr. Blaese for an update
on the first human trials and his response follows.
ãProgress is moving well toward our goal of beginning
treatment for the Crigler-Najjar children before the end of this year.Ê The clinical protocol is now being written
and the documents needed for FDA review are being assembled.Ê Last week we made our final determination of
the exact structure and formulation for the chimeraplast that we will use for
the Crigler trial, and we have now started to manufacture the larger amount of
pharmaceutical grade material needed for the trial.Ê We expect to submit our formal application to the FDA after all
the required safety testing is complete in September and hope to begin treating
three patients by November.Ê However, we
still have a lot of work to do before I am comfortable enough to actually begin
treating the children. We do not want to begin prematurely or potentially cause
harm, so it is possible that this preliminary schedule could be bumped back if
issues come up that take more time to resolve.
Kimeragen was able to find some investors who provided
funding for several months, and we are hopeful that additional money will
become available soÊ we can complete the
initial trial÷and demonstrate to the world that this unique therapy really does
have the potential to change people's lives for the better.Ê We are also looking for ways to obtain
grants and contracts to support development of treatments for other orphan
diseases, such as MSUD, and we are absolutely committed to finding a way to
bring this treatment to those other conditions.ä
NEWS
& NOTES
Books Reviewed
Denise Pinskey
I received a request through Joyce Brubacher
concerning books that may help MSUD families cope with MSUD issues.Ê The request was made by Lori Smith Thornton,
Aunt of Kayleigh Hahn.Ê I called
Magination Press so I could review any books I felt would help our
situations.Ê Two books sounded helpful.
What about Me?Ê
When Brothers and Sisters Get Sick by Allan Peterkin, M.D.
In this book you are introduced to a family with a
daughter and a younger son.Ê It is written
from the sisterâs perspective.Ê It tells
how the sister really likes having a little brother.Ê But when her brother does something to upset her, she wishes she
never had a little brother. Unfortunately her brother becomes tired and cranky,
and has to go to the hospital.Ê The
story does end happily with the parents talking with the doctor and the sister.
And the brother does get better.
I found this book to be very helpful with my
four-year-old daughter who sometimes feels very left out when Zachary (our son
with MSUD) gets sick.Ê We had read this
book once while Zachary was in therapy.Ê
A few days after reading this book, Zachary became ill and our
four-year-old referred back to the book in explaining why Mommy and Daddy had
to take Zachary to the hospital, and she had to stay at Grandmaâs house.Ê So for us, this book was helpful in starting
conversations with her.
Little Tree: A Story for Children with Serious Medical
Problems by Joyce C. Mills,
Ph.D.
In this book you are introduced to a happy tree,
Little Tree and a squirrel, Amanda.Ê
During a storm, Little Treeâs branches are hurt and Amanda calls upon
the Tree Wizards of the forest to help.Ê
Unfortunately, they canât fix Little Treeâs branches.Ê They have to remove them in order to save
her life.Ê With Amandaâs help, Little
Tree learns to accept her new, changed self and to find other strengths inside
herself.Ê The story also introduces the
practice of the ãMagic Happy Breath.ä This is where Little Tree had to take a
deep slow breath and imagine the beautiful sky swirling through its whole trunk
and branches.Ê I think this practice
would be helpful to relax your children when they get upset over medical
procedures.
If you are interested in either of these books or want
a list of all the books available from Magination Press, you can visit their
Web site: www.apa.org/books, or call 800-374-2721 or write to Magination
Press, American Psychological Association, P.O. Box 92984, Washington, DC
20090-2984.
Melissa Berman, 17, daughter of Bob Berman, was named
Student of the Month for the Physical Education/Health Department at Peabody
Veterans Memorial High School, Peabody, Massachusetts.Ê She received a certificate of recognition, a
Peabody Tanner Pride Pin, a bumper sticker for her dad, gift certificates for
combo meals at a new food concession, and a free buffet luncheon with her
nominating teacher.Ê Additionally, her
picture and a narrative explaining why she received this special honor appeared
in the Student of the Month display case at the school.Ê She was applauded for her hard work and
commitment to her studies.Ê This was
quite an honor in a high school with 2000 students.
CONGRATULATIONS FOR A JOB WELL DONE, MELISSA!
Twenty MSUD families in the Michigan, Ohio, Indiana,
and surrounding area were interested in getting together.Ê It was decided to have a fun weekend of
sharing at a central location.Ê Denise
Pinskey reserved rooms for families at a Howard Johnson motel in Perrysburg,
Ohio.Ê The price was good and the pool
was just what the children needed on a this hot day, April 17, 1999.Ê The seven families who were able to attend
really enjoyed that weekend.
We would encourage families in other areas to get
together and share.Ê It takes some
effort to plan, but is well worth it. Maybe your area has only a few scattered
families. The number isnât important, just get together.Ê It may be two or more families visiting the
zoo or just an afternoon at the park.Ê
It means so much to the children to share with others who have the same
diet which could include families with PKU children.
CARING IS SHARING.
Since we have a Web site, we hear about persons with
MSUD from all over the world and have been able to help them in various
ways.Ê Several recent countries we have
heard from are Brazil, South Korea, New Zealand and Israel.Ê It is so sad that children with MSUD die
undiagnosed or are critically ill before being diagnosed in many countries.
What rationale is there for this still happening in
the United States?Ê Every state has a
newborn screening program for at least PKU.Ê
At very little additional cost, all newborns could be screened for MSUD
as well as a number of other diseases.Ê
In May of this year, a child was in the hospital in Florida for two
weeks in critical condition until diagnosed at four weeks of age with classic
MSUD.ÊÊ This should not happen.Ê This issue needs to be addressed.Ê If you are interested in helping, contact
Sandy Bulcher (see more information and how to contact Sandy under NEWBORN
SCREENING COALITION at the beginning of this issue).
÷Editor
Lo Pro Pancakes
(Submitted by Esther Stauffer)
|
2 ¸ c. wheat starch |
1 tsp. salt |
|
2 T. methylcellulose |
2 ¸ tsp. baking powder |
|
2 T. egg replacer |
2 T. powdered coffee creamer |
|
1 T. sugar |
Mix all together and store in a tight container.Ê To make pancakes: Use 3 tablespoons
pancake mix and add 1 tablespoon applesauce and 1 ¸ tablespoons water.Ê Fry on a hot griddle.Ê Makes 1 serving.Ê Total mix makes 15 servings.
|
|
Leucine |
Protein |
Calories |
|
Per serving: |
11 mg |
0.1 gm |
77 |
|
Total mix: |
164 mg |
1.9 gm |
1148 |
Following recipes are Glendaâs suggestions for using
the pancake mix:
Mozzarella Cheese Sticks
|
3 oz. low protein mozzarella cheese 3 T. pancake mix Dried low pro bread crumbs |
Cut cheese into 6 sticks (¸ x ¸ x 2 in.).Ê Combine pancake mix with 1 tablespoon applesauce
and 1 ¸ tablespoons water.Ê Dip cheese
sticks into pancake batter and then into bread crumbs.Ê Deep fry in hot oil until golden brown.Ê Serve with ¸ cup pizza sauce.Ê 2 servings (3 sticks each).
|
|
Leucine |
Protein |
Calories |
|
Per serving: |
96 mg |
1.6 gm |
158 |
|
Per recipe: |
191 mg |
3.1 gm |
315 |
Breaded Mushrooms
Mix pancake batter for 1 serving.Ê Cut four mushrooms in half.Ê Dip each half into pancake batter and drop
into hot oil. Fry until golden brown.
56 mg leucineÊÊÊÊÊÊ
0.8 gm protein ÊÊÊÊÊÊÊ Calories 187
Breaded Cauliflower
Mix pancake batter for 1 serving.Ê Dip 3 cauliflower florets into batter and drop
into hot oil.Ê Fry until golden brown.
69 mg leucineÊÊÊÊÊÊ 0.9
gm protein ÊÊÊÊÊÊÊÊ Calories 190
Fruit Slush
|
1 qt. diced peaches |
6 bananas, sliced |
|
1-20 oz. can crushed pineapple |
¸ lb. red seedless grapes, halved |
|
1 qt. diced pears |
6 oz. frozen orange juice concentrate |
Drain fruit, saving the juice.Ê Add water to reserved juice to make 3 cups;
stir in ¸ cup sugar and orange concentrate until dissolved.Ê Pour juice mixture over combined fruits and
mix.Ê Freeze in a shallow pan.Ê Remove from freezer ¸ hour before serving.Ê
16 servings.
|
|
Leucine |
Protein |
Calories |
|
Per serving: |
73 mg |
1.5 gm |
256 |
|
Per recipe: |
1168 mg |
23.5 gm |
4100 |
Ginger Glazed Carrots
|
1 ¸ c. carrot juice |
1/3 c. brown sugar |