RECIPES

Bread Stuffing

Potato Stuffing

Saute celery, onions, and green pepper in butter until soft. Add seasonings, Coffee Rich and water. Combine with mashed potatoes. Carefully fold in bread cubes. Bake in a greased casserole dish for 30 minutes at 350°. Makes 2 servings.

Combine ingredients in a small bowl. Mix until crumbly. Spoon over batter in muffin pans. Bake at 350° for 25 to 30 minutes. Makes 12 muffins.

Apple Goodie

Submitted by Elsie Newswanger

Mix sugar, wheat starch, salt and cinnamon together and toss with apples. Spread in a greased 9" x 13" or 2 quart baking dish. Mix topping ingredients until crumbly and pour over apple mixture, patting it firmly over the apples. Bake at 350° until browned and bubbly, 30 to 40 minutes. Makes 12- ² cup servings.

Peppermint Crunch Cookies

Melt almond bark in microwave. Stir in a drop or two of food coloring. Mix in other ingredients. Spoon onto waxed paper making 60 cookies. Let cool.

Easy Punch

Submitted by Elsie Newswanger

Combine all ingredients and serve. Makes 28- 8oz. servings.

Pie Crust

Submitted by Martha Newswanger

Cut shortening into wheat starch until crumbly. Add remaining ingredients using more wheat starch as necessary. (Use applesauce for apple pie and pumpkin for pumpkin pie.) Roll out quickly. Makes a 9 inch pie crust.

Apple Pie

Submitted by Martha Newswanger

Combine all ingredients and pour into an unbaked, low protein pie shell. Dot with 2 T. butter

Combine dry ingredients and stir in melted butter. Sprinkle on top of apples. Bake at 375° for 30 to 40 minutes. Makes 8 servings.

Pumpkin Pie

Submitted by Martha Newswanger

Combine all ingredients in a blender in order listed. Pour into an unbaked, low protein pie shell. Sprinkle with pumpkin pie seasoning or cinnamon. Bake at 350° for 45 minutes. Makes 8 servings.

Fruit Cupcakes

Submitted by Dawn Hahn

Combine sugar, oil, molasses and water in a mixing bowl. Combine dry ingredients and add to sugar mixture. Add applesauce and mix well. Stir in pears and pour into cupcake liners. Bake at 350° for 12 to 20 minutes. Makes 22 cupcakes.

We try our best to see that the nutritional values listed with the recipes are accurate. They are figured with the help of nutritionists from Emory University. We are not responsible for errors. Contact your clinic before making diet changes.

FOOD NEWS

Cambrooke Foods

Shayla Brubacher, holding an energy bar, and Sharlene Woorman with Lynn Paolella, of Cambrooke Foods.

Lynn Paolella, owner of Cambrooke Foods, supplied free samples of her energy bars and bagels at Symposium 2000. They were a big hit with parents and children. Both items contain less than 1 gm of protein. The 4 oz. energy bars have 39 mg of leucine and the 5 oz. bagels contain 24 mg of leucine. The bagels and energy bars can be ordered on-line or call 508-276-1800. They will soon have a toll free number and plan to add other products. Keep updated on this exciting new company by checking their web site: www.cambrookefoods.com

NEWS FROM SHS NORTH AMERICA

New product: SHS North America is pleased to announce a new product, Loprofin Drink Mix, a milk substitute! One 14 oz. can of the powder makes more than 5 quarts of Drink Mix. It can be added to Loprofin Cereal Loops or used in everyday cooking and baking. This new product will be available in early 2001. Call 1-888-LOPROGO for free recipes with your order.

New packaging: Beginning in 2001, Loprofin will have a new face! Throughout the first months of the new year, you will see the Loprofin brand in new, attractive packaging with easy-to-read nutrition information. No changes have been made to the products themselves.

Discontinued: SHS North America is going to discontinue Loprofin Canned Bread in the spring of 2001 (items #TWL 501 and TWL 503). Please place your orders by early spring.

For more information, visit their web site at www.shsna.com or call 1-888-LOPROGO (567-7646).

National Coalition for PKU & Allied Disorders

This organization, not to be confused with the New England Connection for PKU and Allied Disorders Inc. in Massachusetts, is becoming very active and influential. Included with this issue is a registration form for their first big Metabolic Conference to be held in Dublin, Ohio. The agenda is on the back page of this issue.

The National Coalition for PKU and Allied Disorders Conference is planned for families and professionals from seven different groups: MSUD, PKU, the organic acidemias, glutaric aciduria, tryosinemia, homocystinuria, and the fatty oxidation disorders. During the day on Friday, each group will have its own speakers and agenda. On Friday evening, there will be a Newborn Screening meeting open to anyone interested in that topic. There is no cost to those who attend only Friday evening.

On Saturday, the topics will be of general interest to all attendees. On Thursday and Saturday evenings there will be receptions at the hotel with cheese and crackers, fruit, and a cash bar for those who want to attend. Be sure to make reservations soon at the Dublin Embassy Suites Hotel. The toll free number is 1-800-362-2779 or call the hotel directly at 614-790-9000.

Be sure to state that you are attending the National Coalition for PKU and Allied Disorders Conference in order to get your $99 room rate. You will have a choice of 2 double beds or 1 king size bed. Each room has a pull-out couch, microwave, refrigerator, coffee maker, blow dryer, etc. This price includes a continental breakfast and an early evening snack and soft drink. There is a $50 registration fee which includes lunch both days.

There is an indoor pool, whirlpool, and small exercise room. They ask that children with MSUD under 12 not attend the conference as there is no program or daycare planned for them. For more information on this event or organization, check out their web site: pku-allieddisorders.org

Addition to Clinic

A full time pediatrician, Dr. Kevin Strauss, will join Dr. Morton and the staff at the Clinic For Special Children in Strasburg, Pennsylvania this summer. The Clinic is expanding to make room for the increase in staff. On December 29, with the help of many volunteers, a timber frame was raised for the addition.

The following paragraph from the Winter 2000 issue of the Clinic For Special Children Newsletter describes the work of the Clinic.

Unlike most centers which specialize in genetics, the Clinic was designed not only to provide specialized diagnosis, laboratory work and consultation, but also to provide primary medical care to children for all their medical needs however minor or critical. This comprehensive approach has given the Clinic an opportunity and advantage of learning more about disease processes related to metabolic disorders through observation in many different circumstances and timely intervention for more effective treatment. When we moved into the present building, we thought we would see and treat children with a dozen to two dozen different genetic conditions. To date we have seen and treated children with over 80 different genetic conditions. Our work has led us toward a greater role in teaching others about what we have learned. We hope with the expansion in staff and facilities we will be able to do more in sharing progress through lectures, publications and conferences and in educating students of medicine.

The expansion of the Clinic is a key part of a five year plan which includes a second (and potentially a third) pediatrician who specializes in rare genetic disorders and the growth of an endowment fund to give the Clinic financial security. The Clinic has a non-profit status and relies on donations as well as proceeds from three annual auctions. This keeps fees for patient services at a minimum rate since most of the families who use the Clinic do not have insurance and do not participate in federal or state aid programs.

Another paragraph from their Winter 2000 Newsletter states the reason for the changes and the Clinic's goals for the future.

During the next year we are having a carefully planned and needed growth spurt. We are building . . . building staff, building space, and building the Clinic's future through the endowment fund. We do not plan to grow like this every year or to grow so that it changes the fundamental and personal way in which we operate and serve children. But, we need to grow in a way that we can continue to provide state of the art medical care, laboratory services, and [to] support research and education that is based on the needs and experiences of our patients.

Article on Gene Therapy

Those who are interested in the gene repair therapy that Dr. Blaese spoke to us about at Symposium '98 will find an article worth reading in the May 1999 issue of Fortune Magazine. "Can Gene Therapy Cure This Child?" reviews the Kimeragen company's struggle to break into the field of gene therapy. The article chronicles the problems involved in bringing the therapy to human trials. The Floyd Martin children with Crigler-Najjar disease, who are scheduled to be the first to receive the new gene therapy, are featured in the article. It is a lengthy and interesting article although MSUD is not mentioned.

Follow-up on Fenugreek Tea and the Smell of Maple Syrup

In the Spring/Summer 2000 issue of the Newsletter, a short article stated that fenugreek tea may cause an odor in the urine similar to maple syrup. We receive many calls from persons who smell maple syrup in their own urine or on their child and wonder if they could have some form of MSUD even though they are not sick. Recently a woman, 39, who had raised a family, was diagnosed with intermittent MSUD. However, she had been very sick several times prior to the diagnosis. Are there many more undiagnosed cases?

After reading the article on the fenugreek tea odor, Dr. Neil Buist responded with a letter to me providing some additional information. I certainly appreciate his response, which follows.

The most common "MSUD" question that I usually get is about "healthy" children who smell of maple syrup (MS) as mentioned in the Spring/Summer issue. Almost all of these children are developmentally normal. Clearly, fenugreek is not the cause in most of these children, and indeed, it is not common to smell MS in children or adults who eat lots of fenugreek in curries.

I think that the smell (which derives from some unknown compounds) comes from what older clinicians used to call the "second liver"—the bowel. Gut bacteria are responsible for making certain essential nutrients and there are many types of bugs, either aerobic or anaerobic, that live in the gut. Many are not even identified! Not everyone has the same collection of bacteria nor is the population constant all the time. It has been shown that the adult bowel contains about a half pound (220 g) of living bacteria; what a metabolic powerhouse!

Anyway, I think that the MS smell comes from some of the anaerobic bacteria in the gut, which explains, of course, why there are no changes in the plasma amino acids or the urine organic acids in these cases.

—Neil M. Buist, M.D.

Join the Exclusive MSUD eGroup

Emily Talley is a 43 year old adult with MSUD. She has set up a group e-mail account for anyone who would like to use it as a forum for group discussion concerning MSUD support. The group e-mail account is absolutely FREE. It's hosted by egroups.com, and only members can access it. The fact that it is a member only group e-mail account makes it a safe place for people to receive and give support around the issues of MSUD.

For those of you who aren't familiar with group e-mail accounts, here's how they work. When you post a message to the group's e-mail address (msud@egroups.com), your message will automatically be sent to every member in the group. Likewise, when someone responds to a member's e-mail, their reply will automatically be sent to every member in the group.

Emily Talley is the owner/moderator of the MSUD group e-mail account. She can be reached at emily@inherhands.com. She will trouble-shoot and fix any technical difficulties that come up (e.g., bounced e-mails, etc.).

To become a member of the MSUD group e-mail account, just send an e-mail to: msud-subscribe@egroups.com

Newborn Screening Campaign & Unity Quilt

Tyler for Life Foundation, Inc., a newborn screening awareness organization, recently launched a national campaign, "Handout America," during which volunteers supply pamphlets entitled "A Parent's Guide to Newborn Screening." These pamphlets are to be displayed in hospitals, doctors' offices, clinics and any place that serves children and families.

The organization claims that each year there are 2700 children in the U.S. who die or are brain damaged but could have been saved with a newborn screening test. If you would like to volunteer to distribute the pamphlets, use the order form on the Tyler for Life web site or e-mail Kileen Hall at kileen@tylerforlife.com giving your address and the number of pamphlets you want. Ten pamphlets per doctor's office is the suggested maximum.

The Tyler For Life Foundation will be collecting quilt panels for a NBS Unity Quilt from families with children who have disorders detectable through newborn screening. This new project will involve all the families in a unified effort. The quilt will allow the families of children with these disorders to celebrate and share the lives of their children.

The mission of the quilt is to create a visual testimony of the most compelling symbol of newborn screening—the children touched by it. The quilt will be unveiled on Sept. 1, 2001 in celebration of NBS Awareness Month. To participate or learn more about the project, visit the Tyler for Life web site. You can also learn more about newborn screening, related disorders, and the Tyler for Life Foundation at tylerforlife.com or call 1-888-454-3383.

The Tyler for Life Foundation also hosts a discussion listserv for persons interested in corresponding with others involved in newborn screening. To join, send an e-mail to: NBSAdvocacy@listbot.com

Tax Deductions for Conferences

The IRS will allow deductions for some travel expenses when parents attend medical conferences for their children. On May 8, 2000, responding to a request from Congressman George Miller, the IRS issued a ruling that will allow parents to deduct some of the costs associated with attending medical meetings related to their children's health condition. Parents will be able to deduct "amounts paid by an individual for expenses of admission and transportation to a medical conference relating to the chronic disease of the individual's dependent." See IRS Bulletin 2000-19 for more information: http://ftp.fedworld.gov/pub/irs-irbs/irb00-19.pdf

McDonald's Salads

Greg and Ann Fredericks checked into the possibility of getting garden salads at McDonald's restaurants without the egg and cheese included on the new shaker salads. According to the McDonald's web site, they are willing to make adjustments to "make you smile!"

The Fredericks received a letter saying it should be possible to get a made-to-order garden salad at any McDonald's. They suggested calling 30 minutes ahead of time so they have time to make it. However, their local McDonald's said they don't need to call, and they will only need 5 to 10 minutes to prepare it.

The Fredericks have requested the salads when traveling and usually have had no problem. Sometimes the employees refuse and need to be reminded that it is possible to make a special order. It is best to ask only when the McDonald's is not busy as it does require time. Better than the older type of garden salad, the shaker salads have greener lettuce, small tomatoes, and scallions.

So check with your favorite McDonald's and make arrangements to have your request honored. Have your salad, keep your diet, and SMILE!