PGC Newsletter
Volume 11 Issue 1 - Summer 1999

Table of Contents

Ohio Support Group for Families Living with Galactosemia
Galactosemic Families of Minnesota
Parent's Networking

National Metabolic Disorder Meeting Held
Tremors in Galactosemic Children from a Parents Perspective
poem - 'To All The Mothers Like Mine'
Inadvertant Life Lessons
Pen Pals
We Are Growing
May Be Of Interest

Ohio Support Group for Families Living with Galactosemia

Thanks to Karen Allen, an Ohio Support Group for Families Living with Galactosemia has been formed.  The first get together was in February in Columbus, OH.

The day was incredible!  This was a fun and informal get together with 19 families from all over Ohio being represented.  The families had children with galactosemia ranging in
ages from newborns to their 30’s.  For many parents this was the first time they had ever met anyone else raising a child with galactosemia.  Information, tips, ideas, laughter
and stories were exchanged by all.

The food was outstanding and all lactose free.  Jackie Hutchison had mouth watering, homemade cookies and treats as well as numerous wonderful lactose free items for all to
try.

The enthusiasm for the National Conference was great.  Topics were suggested.  It seemed nobody wanted the day to end.

Everyone is looking forward to the next meeting.



Galactosemic Families of Minnesota -Summary of meeting

Twenty-three adults were in attendance and there were thirteen families represented at the meeting on Saturday, April 17, 1999.

The tax exempt process is completed and we have a tax exempt number.  We also have our brochure completed.   The brochures were distributed to the families to use when
asking for donations from businesses and/or individuals.  The University of MN Hospital staff will also hand out these brochures to families who come to the clinic for their
appointments.

Someone in our group created a directory of all the families in MN (We also have families from IA, WI, and ND in our group.).  The directory has a photo (if supplied) as well as
the information on our mailing list (name, address, phone number, e:mail, names of children, etc.) .

Funding is a topic that will be discussed at our next meeting.  Several families recommended that we need to have a cause or purpose for fund raising before we can go out
asking for donations.  At the next meeting, our group will be discussing some fund raising ideas.

One family explained that there is a need to inform television networks, magazines, clinics and hospitals about galactosemia and the importance of the newborn screening
tests.  Check out the Tyler for Life website at www.tylerforlife.com for more information and the story about Tyler's life.

The group recommended having a food technology specialist present at our April 2000 meeting. Some families brought recipes and information to share.

The next meeting will be on Saturday, September 25 in the Twin Cities.  It will be an informal meeting and lots of time for social activities and interaction.  We will be having the
election of officers at the next meeting also.



Parent's Networking

What is the difference beween Duarte and Classic Galactosemia

Each of us has two genes at the Galactose-1-Phosphate Uridyl Transferase Loci.  If someone has classical (GG) each gene product has no enzyme activity.  Those that are
normal (NN) have 100% normal activity.  Those that are duarte at both loci (DD) have approximately 50% activity.  If they are GD (one classical galactosemia gene and one
duarte gene) they have gene products with about 25% or less of normal activity.  These children may also be picked up at birth on newborn screening.
 

Andre

Greetings!  My son Andre was 13 last December and has galactosemia.  We have read your newsletter for years and this message is for the newsletter.

As galactosemic children go, Andre has been severely affected with the secondary problems we associate with our kids.  Namely, speech and language delays, poor cognitive
development and behavior problems.

Recently Andre has also been diagnosed as being Autistic.  Andre’s school progress has been so poor that I initiated a re-evaluation at the University of Michigan.  From the
little that I have learned about autism it seems to fit Andre’s situation.  This has lead to a new school and a program specifically set up to serve the autistic child.  We are very
hopeful that Andre will finally get the help he needs to be his best.  The doctor is also trying behavior modification medications to see if that will also help.  For years we have
contacted others like the University of Wisconsin, Andre participated in the five year Uridine Study, for help with Andre’s lack of progress.

Perhaps other parents with the same needs will ask their doctor to look closely at autism.  The very frustrating thing is with all the professionals from three major University
Hospitals no one mentioned or looked at autism as a possibility.  Again this gives us hope that Andre will finally be in a program that serves his special needs.  We love him
dearly and he is truly a special child (as all of our children are).  I hope that in some small way this information may help other families.

Sincerely,
Richard Krist
Parma, MI
 

Vitreous Hemorrhage

I recently received the newsletter by PGC.  I was compelled to write because of the recent children that have vitreous hemorrhages.  My son is now 19 and has Galactosemia.
When he was born he also had one eye with a cataract and a vitreous cloudiness in the eye.  There was never any reference to me by his doctors of the term although he had
corrective surgery at the time that was only known to be done on diabetics with vitreous hemorrhage.  This surgery was done at 3 months of age by Dr. Duain Fuller of Texas
Retina Assoc. in Dallas, Texas.  I need to share the outcome with you and other parents to let them know whether this is something they wish to explore for their child.

When my son had this problem no one knew at the time that it was a complication of Galactosemia.  The surgery was quite new and had only been done for the 5 years prior.
We were defiantly in the unknown territory.  The surgery involves taking the cloudy vitreous fluid out and replacing it with clear fluid.  The eye then makes its own clear fluid.
When the surgery was done the doctor decided to leave the cataract covered lens and not remove it.  The cataract looks like the shape of a snowflake.

After the surgery we went through patching the good eye to build nerve pathways to the weaker eye.  This was all done with contact lenses.  You can only imagine how
difficult this was for a child this young.  We started with contacts at 7 months.  One contact was black to patch the good eye for a period of time during the day.  This patching
went on in various forms; we tried the bandaid patch, glasses for a short time with a contact on the weaker eye.  I can only say it was a trial.  We discontinued patching earlier
than we could have because of numerous other learning disabilities.

My son’s vision in that eye stabilized at 20/100 with a contact lens on.  With this large difference in vision between both eyes he no longer wears a contact on that eye and
does not use this eye for vision.  This surgery and patching was only intended to obtain vision in his weaker eye in case he should lose vision in his good eye.  In other
words, it is a 'spare tire eye'.  His eye turns in slightly but is barely noticeable with glasses on.  You would never know he does not use this eye, and at least if his good eye
were to lose some vision he could see something.

My son also suffers from diabetes that he developed at 16 months.  His diabetes had nothing to do with this eye problem which occurred at birth.  Actually because of
complications of diabetes, namely vitreous hemorrhage, I feel we made the right decision at the time. If he were to lose the vision in his good eye, he could at least use his
weaker eye for some things and not be totally blind.

I hope this helps other parents.
Sincerely,
Beth Cook
Columbia Falls, MT


National Metabolic Disorder Meeting Held

On March 19, 1999 there was a national meeting of concerned individuals to discuss issues involving metabolic disorders and newborn screening.  The meeting was moderated by Trish Mullaley, a PKU Parent and founder of the New England Connection for PKU, and by Sandy Bulcher, a MSUD parent from Ohio.  In attendance were parents representing PKU, MSUD, homocystinuria, HCU, organic acidemia, and galactosemia.  Also present were professionals directly involved in the metabolic field.  Representing galactosemia was Tera Mize, whose infant son died last year from direct complications of undiagnosed galactosemia.

Many problems with the current newborn screening systems were discussed including batching of tests, transporting the tests from the collection site to the lab, hours of lab
operation, repeat testing procedures, qualified physician follow-up, early discharge issues, and screening procedures of military bases.  Roger Eaton of the Massachusetts
Newborn Screening Program was present to answer some of the questions regarding the system in Massachusetts.  Also present at the meeting was Dr. Richard Koch, one of
the leading doctors in the metabolic field, who talked in great detail about the potential improvements that could be made to better the system.

Ideas on how to help in improving the present system and education of the public about metabolic disorders included forming a national organization dedicated to these
issues. The group voted to form this new organization and it is being called the National Coalition for PKU & Allied Disorders.  This new group is currently being set up and is
expected to be fully operational this year.  Some of the goals for the organization are to promote national efforts to improve awareness of metabolic disorders, advocate for
expanded newborn screening, establish a National Newborn Screening Day to be observed each year, establish treatment guidelines, and to provide research for treatments
and cures.  The group will consist of individuals, families and professionals interested in issues regarding inborn errors of the metabolism which require a low protein diet.
Although, galactosemia is not one of the advocated disorders for the group, their efforts for betterment of the overall system will benefit families affected by galactosemia as
well.

Another hot topic at the meeting was expanded screening using tandem mass spectrometry.  Tandem mass spectrometry is a relatively new method of detecting inherited
metabolic disorders, which can detect many more disorders than conventional methods do.  This testing method is said to detect over 30 metabolic disorders, many of which
are not currently detectable through any other method.  Some states are already offering expanded screening through pilot studies.  The results of these pilot studies will affect
the decisions of many other states that are keeping a close eye on the effectiveness of expanded screening.  There are also some hospitals voluntarily offering expanded
screening through private laboratories.  This 'supplementary' screening is in addition to the mandatory disorders screened for in their state.  NeoGen and Walac, Inc provided a
professional overview of tandem mass spectrometry.  NeoGen is a private laboratory that presently uses the tandem mass screening method and Walac, Inc. is a company that
manufactures the machines that perform tandem mass screening.

A conference was held the following day by the New England Connection for PKU & Allied Disorders.  Sandy Bulcher spoke at the conference regarding the issues discussed
from the meeting the night before.  She also discussed the forming of the national organization and its mission.  Other presentations were on gene therapy research,
development of diet supplements and the need for expanded newborn screening.

In closing, much was learned at this meeting.  It can not be emphasized enough how important a role parents and parent organizations play in the process of improving
screening and treatment procedures.  It is a slow, exhausting battle that must be fought unceasingly as better screening techniques and treatments are developed.  Regardless
of what disorder or group that a person chooses to support and be active in, together we can make a difference in the lives of families living with metabolic disorders, and in
the lives of families still yet to be affected . . . as we all know can happen too quickly.

Tera Mize
Tyler For Life Foundation
www.tylerforlife.com


Tremors in Galactosemic Children from a Parents Perspective

WHO I AM
I am the founder of Parents of Galactosemic Children. Fifteen years ago when my son, Adam, was born with galactosemia, I discovered that no support network existed for
galactosemic parents. Our metabolic clinic in New York (at the time) agreed to send letters to other families for me, through that I met Jane Wicks and Laura Norych. We found
that there were only a handful of us and decided that we would have to go national. We sent letters to clinics across the United States and asked them to photocopy the letters and send them to galactosemic parents. Gayle Dennis, Christine Kovach and Barbara Sprow were among those parents who responded to this letter campaign. From there, we formed Parents of Galactosemic Children.

I was intensely involved with PGC for ten years, then life's circumstances led me down a different path. Fortunately, Gayle, Christine, Rhonda and others have continued to
make Parents of Galactosemic Children the respected, informative and national support network it is today.

ADAM
Adam (15) is one of our three children. He has an older sister, Melissa, who is seventeen (you can see her article this newsletter) and he has a twin sister, Allison. Both girls are
carriers.

I believe Adam to be one of the most severely affected galactosemic children. He has an IQ of 73 (70 is considered mentally handicapped). He also has an expressive language
delay, low muscle tone in his shoulders and arms, and tremors, which he developed when he was about four years old. Adam was diagnosed at 8 days after being extremely
affected and being transferred to a Neonatal Intensive Care Unit.

Adam is a sweet, happy child with an engaging smile. I often refer to him as my "love child," because he is full of love and doesn't have a mean bone in his body. At 8 months,
Adam was tested and enrolled in an early intervention program for speech, physical and occupational therapies and special ed. He has been in special ed classes through
school  and through these classes, we have met some of the most compassionate, wonderful people one would ever want to meet.

THE TREMORS
DEFINITION: Tremor is a rhythmic, involuntary muscular contraction characterized by oscillations (to-and-fro movements) of a part of the body. The most common of all
involuntary movements, tremor can affect various body parts such as the hands, head, facial structures, vocal cords, trunk, and legs; most tremors, however, occur in the
hands. Tremor often accompanies neurological disorders associated with aging. Although the disorder is not life-threatening, it can be responsible for functional disability and social embarrassment.

There are different types of tremors: I have been told that Adam has Kinetic or Intention tremor  which occurs during purposeful goal-oriented tasks, for example finger-to-nose testing.

TREATMENT
Through occupational therapy Adam has learned how to do tasks despite his tremors. About three years ago, I came upon an article that said tremors could be treated with
medication. We went to a neurologist and after a number of tests including a CAT Scan, decided to try medication. NOTE: Through the years Adam has undergone a number
of neurological tests and examinations, which have shown abnormalities in the brain, often referred to as "white masses."

The medication recommended was Enderol. This medication unfortunately contains lactose, so we found a pharmacist who first "made" it in a lactose-free liquid form and more recently in a lactose-free capsule. The medication has helped somewhat, but we continue to be dissatisfied as his tremors are still apparent and sometimes difficult for him to deal with. On the advise of his current neurologist we have made an appointment with a neurologist who specializes in Parkinson's disease at Miami Children's Hospital, in the hopes that there might be a medication that might work better. Unfortunately, for purposes of this newsletter, the appointment is next month.

HOW WE DISCUSS TREMORS
We have always been open with Adam and all our children not only about his tremors but his other disabilities and of course, diet. We have told Adam that because of the
galactosemia his hands shake. He doesn't like it, which is why we continue to look for ways to make it easier for him.

We also point out the numerous things Adam can do, including typing on the computer  for hours, he emails and "instant messages" friends and family. (He loves to receive
email: Abenm@aol.com). He can search the internet better than anyone in his family. He is a whiz at Nintendo 64 and Sega, which confounds not only us, but the experts.

DOES YOUR CHILD HAVE TREMORS?
I think we should pool our information. If your child has tremors, please contact me (see below). I feel that by doing this, we might come up with a better understanding and/or
treatment for tremors in galactosemic children. If there is a physician, who has experience in this area or who is interested in this topic, I would ask that you also contact me.

Linda Manis
1189 Hillsboro Mile #11
Hillsboro Beach, FL  33062
Ph# (954) 429-0377
e-mail:  LMScript@aol.com
Fax#: (954) 429-2781

Linda Manis has also written a story/book about galactosemia but she needs an illustrator. If you are talented in this area and interested please contact her.


To All The Mothers Like Mine

A long time ago, as the story goes.
A child was born with ten fingers and ten toes
Two arms, two legs, eyes, ears, mouth and nose
And the mother loved her...
The professionals said wait, something is not right.
Your child is different and quite a sight
One leg longer than the other and club feet
Facial paralysis.  Yes, your child’s incomplete.
But the mother loved her...
She is nothing more than a blob they did say
Can’t walk, can’t talk, she is in the mentally impaired way
Yet the mother still loved her...
Years have gone by and you should see
the result of my mother’s belief in me
I have two legs and two feet that can walk
A mouth that can talk talk talk
Two eyes that can intellectually see, and have allowed me to earn two degrees
Because my mother loved me!!
 

To all the mothers like mine, thank you for following your heart and believing in the possibilities.


Inadvertant Life Lessons By Melissa Manis

Being the sibling of a galactosemic child has been both inspiring and heartbreaking, seeing my younger brother overcome so many things, and yet watching him lose out on so many others has been an intense contradiction in my life. My name is Melissa Manis, I am seventeen years old, and my younger brother Adam has galactosemia. As far back as I can remember I have been overtly conscientious of special dietary needs and activities that could never be a part of Adam's life. By looking at what could not be done I never noticed what he could do. Adam got to eat special food, and go to special doctors, he got to go to a special school program, and the realization came to me that Adam was a special kid. Adam views the world from a perspective I will never come to know.

 I learned to look at the world from a different angle because of Adam. Although, he doesn't know it, by being Adam, he has taught me about himself, and children like him. My younger brother inadvertently teaching me life lessons, I know itdoesn't sound plausible, but here is an example. If you were to turn to Adam and ask him what he wanted to do on a Saturday afternoon most likely the answer you would receive would  be "I don't know," accompanied by a big knowing smile. Adam is always smiling, it's like he knows a secret, he taught me to smile, to smile in doubt, in fear, and in happiness. Adam smiles through them all and every now and then I will catch myself doing the same.

 Adam has accomplished so many things in his short life, and won the hearts of countless people. I don't know quite how to explain Adam's personality but somehow people
gravitate around him. I've never looked at Adam as being very outgoing or a loud character, but when I look at the infinite number of lives Adam has touched it makes me look
at myself and question if I too share these qualities and wonder how I can measure up to the impact embedded within so many people by my younger brother.

 I think that the answer is love, unconditional love, a topic which I have learned so much about from being the sibling of a galactosemic and disabled child. I think that I am a
better, more understanding, and patient person because of Adam. Adam has forced me to constantly evalutate myself in terms of my treament and judgement of other people.
Adam is an exceptional child and I have learned so much about the kind of person I am, and want to be from him.



Pen Pals

Dan Lambert is doing a great job with the Pen-Pals!

The Pen-Pals are for children, teenagers, or adults with galactosemia as well as the parents of anyone with galactosemia.

If you would like more information or would like to be a pen-pal contact:

Dan Lambert
PGC Pen-Pal Coordinator
1936 Cottonwood Dr.
Aiken, SC  29803

e-mail:  dlambert@scescape.net



We Are Growing

As you will recall in our last newsletter we were seeking help in some specific areas.

Thank you to those of you who responded!!

Student Requests:  Barb Bense agreed and is already doing a great job responding to the challenges of answering the Student Requests.

ACT Data base:  Eric Graeber agreed to and has done an outstanding job at taking on the huge task of  getting and keeping the ACT Data base in order.  However, all address
changes and new addresses should still be sent to 2148 Bryton Drive, Powell, OH  43065.

E-mail Roster:  Therese Cozzo took on the e-mail roster request.  Therese put together a great e-mail roster.  A copy for everyone is at the end of this newsletter.  If you are not
included on it but would like to be, e-mail your information to Therese:
tfcozzo@home.com

Duarte Variant Chairperson:    We are still in need of a Duarte parent to fill this position.  Again this would involve being the main contact person for parents of Duarte
children  Possibly writing an article for the newsletter, but mainly being there to talk with new parents of Duarte variants.



May Be Of Interest

Vances Darifree Non-Dairy Beverage

Vance’s is fat free.  It contains as much calcium as milk, and is also a good source of vitamins.  It is cholesterol and lactose free.  It is free of soy, rice, corn oils and MSG and it
has a tasty vanilla flavor.  It can be used for drinking, cooking, and baking and is great for use on cereal in place of coffee rich or coffeemate liquids.  It comes in a 7 oz package
which makes 2 quarts and cost is $2.92.  It is also available in a 5 lb jar that costs $25.00;  I personally recommend the 5 lb jar as the shelf life is a minimum of 1 year and if used
frequently, it is more economical to get it this way.

This information actually came from  PKU mom, Nancy Rogers.
 

Lactose Free Chocolates
Illinois Nut Company & Fantasia Chocolates have great lactose free chocolates for any special occasion or just everyday use.  You can contact them for a brochure and price
list at:
Illinois Nut Company & Fantasia Chocolate, Inc
3745 West Dempster St.
Skokie, IL 60076
Phone: (847) 677-5777
1-800-590-NUTS.
Fax: (847) 677-5778
E-mail: illnut@aol.com
 

Tyler for Life Foundation
Dallas and Tera Mize have put together a great foundation to raise awareness of inborn errors of the newborn, which of course includes galactosemia.  You can visit this great
web site at:

 www.tylerforlife.com
 

Great Networking Site for Parents
Ken Herndon has put together an outstanding web site for parents to exchange ideas, information, and stories of raising children with galactosemia. To find it and particiapte,
or just read, go to

www.galactosemia.com
 

Web Site for Recently Diagonosed
this is a nice web site for people that have been recently diagnosed.

www.ahsc.aarizona.edu/~msrgsn/pract/prac21.htm
 

PGC Web Site

If anyone has suggestionas or information for the PGC website please email that information to Nate Bauer at bauer@cy-net.net or Bill Cozzo at cozzo@home.com. We are especially interested in website links for metabolic clinics, any researchers, and state newborn screening sites.



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