M S U D Newsletter

Articles selected from Vol. 13, No.1 , Spring 1995

TEACHING DIET TO YOUR CHILD:

A DEVELOPMENTAL APPROACH

This is a summary of a talk given by Cristine M. Trahms, M.S., R.D. at the MSUD Symposium in June of Î94. Ê Cristine is with the Department of Pediatrics, Division of Pediatric Genetics, University of Washington, Seattle, Washington.Ê Several parents requested copies of the information she presented, and she graciously submitted this article.Ê She informed me in February that a similar summary would appear in the proceedings of the 8th Annual Meeting of the European Society for PKU (ESPKU) under the title: Self-Management Skills: the Key to Successful Treatment.

Parents whose children have MSUD have been presented a challenge unique to parenting.Ê Their tasks are to:1) provide metabolic balance for the normalization of growth and development and 2) support appropriate developmental stages of eating and relationship to food.Ê The second task is more achievable if a framework for supporting developmental milestones is constructed.

Compliance with dietary management guidelines is the cornerstone of effective treatment for MSUD.Ê The goals for successful self-management of MSUD, as we have defined them in our clinic, are that the child: 1) understands the basics of the disorder; 2) understands the food pattern and can identify acceptable and unacceptable food choices; 3) develops skills for making appropriate food choices; and 4) increases independence by learning how to prepare food, plan menus, and keep food records.

Parents are the initial managers of treatment.Ê However, children with MSUD must themselves learn to adequately follow the therapy to the best of their abilities.Ê To support children intellectually and emotionally as they begin self-management requires a basic framework built upon principles of learning.Ê The method that has worked most effectively for our group is based on the principles of Piaget which have been widely described and have been used in many educational settings.Ê The learning theories of Piaget described simply are: 1) that a child progresses through a series of stages of mental development and maturation; 2) a framework of readiness for intellectual development is necessary to promote learning; and 3) activity and exploration on the part of the learner is essential.Ê We know that the progress of development proceeds in an organized fashion; that the child has an inner drive to make progress, but at an individual pace; and that our job as parents and professionals is to understand these developmental stages and support the childâs readiness to move forward.

Piagetâs model describes the developmental learning stages in a manner that outlines the capabilities of the learner:

1. The Sensory-Motor Stage÷infants to age 2, who manipulate objects, are action oriented, are aware of present time, have representational thought and respond to their environment based on their own experience.

2. The Pre-operational Stage also called the Intuitive Stage÷children ages 2ö6, who have language and begin to respond to their environment based on semi-logical thinking, who are perceptively impulsive and blend fantasy and reality.

3. The Concrete-operational Stage also called the Practical Stage÷children ages 7ö11, who are eager to develop and use their skills at thinking, mathematics and reasoning.

4. The Formal-operational Stage also called the Reflective Stage÷ages 12ö15, which is adult style thinking with the ability to deduce and reason and learn to make decisions independently.

The practical application of Piagetâs constructs is that:

5. Learning is based on cognitive readiness.

6. Parents and children must work together through the process one-step-at-a-time.

7. Parents work together to decide priorities for their child, that is, negotiable vs. nonnegotiable behaviors and what level of compliance is expected.

8. The more integrated the childâs life pattern and the restricted food pattern become, the more likely long-term compliance will be achieved, that is, the food pattern is woven into family life rather than each food-related event individually negotiated.

These stages of learning can be translated into developmental self-management tasks for individuals with MSUD based on age and competence.Ê The operational aspects of learning become more sophisticated as the child becomes cognitively more mature, but the goals themselves do not change.Ê To be effective in promoting learning, education must be directed at the level of the learner.Ê Additional information is added only when the learner is ready.

With the added stresses of management of a metabolic disorder, we must still remember that the development of a healthy relationship with food starts in early infancy with the response of the caregiver to the infantâs cues of hunger and satisfaction. Some of these guidelines are:

n The food patterns of young children are shaped by many factors that parents can facilitate or ignore.

n Food acceptances of infants and young children are shaped by an innate preference for sweet tastes.

n The attitudes and acceptance of foods by adults and peers has a profound influence on food acceptance by the older infant and young child.

n Young children respond by accepting or rejecting foods based on the social context of their being offered÷that is, a positive interaction from family or adults offering the foods enhances acceptance and a negative or neutral interaction tends to decrease the probability of acceptance of a food.Ê

n Acceptance of foods is also based onÊ learning÷that is, familiarity and presentation of the food in a form that is easy for the child to manage.

n Children learn from family and other adults how to regulate their own food intake÷that is, they learn cues of satiation based on the social and cultural environment during meals.

These concepts can be translated into action by: introducing new flavors and textures gradually; enlarging the childâs experience with as many forms of individual ãyesä foods as possible; offering individual foods rather than mixtures so that foods may be appreciated for their flavors and textures; being patient with first efforts and allowing the infant to learn to feed himself/herself.

Table I (below) indicates tasks for young children.Ê Younger school-aged children learn much from involvement in the process of food preparation. Cooking can be used to: 1) enhance a sense of accomplishment for the child; 2) have fun while increasing the childâs self-esteem and self confidence; 3) support cognitive and social learning; 4) learn about the role of food as nourishment; 5) learn proper use of kitchen tools/utensils; 6) learn to weigh and measure foods; 7) learn to follow directions; 8) learn how to plan, organize and complete a project; 9) learn to work with other children/adults.Ê These skills, when learned in early childhood, support effective self-management throughout life.

The tasks of primary grade children are shown in Table II (below). Children of these ages learn from problem-solving discussions and role-playing to practice the decisions that they need to make for themselves.

Adolescent children are responsible for the development of: 1) judgment; 2) appropriate responses to social pressures; 3) positive coping behaviors; 4) positive self-concept; and 5) assertiveness skills.Ê The learning tasks of older children are shown in Table III (below).

In summary, we can expect children with MSUD to grow and develop at the maximum level allowed by their disorder by maintaining their therapy and having access to a learning environment that supports cognitive development and directs the development of appropriate self-management skills.Ê A model for supporting the development of self-management skills is presented here.

References:

1. Bybee, R.W. and Sund, R.B. Piaget for Educators, 2nd edition. Charles Merrill Pub. Co. 1982.

2. Trahms, Cristine M. Self-Management Skills: The Key to Successful PKU Treatment. Part I. First steps: Teaching Your Young Child the Basics. National PKU News 3 (3), Winter, 1992.

3. Trahms, Cristine M. Self-Management Skills: The Key to Successful PKU Treatment. Part II. Moving Ahead and Walking Strong: Promoting Self-Management for the School-aged Child, National PKU News 4(1), Spring/Summer, 1992.

Ê

4. Trahms, Cristine M. Self-Management Skills: The Key to Successful PKU Treatment. Part III. Standing on Your Own two Feet: The Adolescent years and Beyond, National PKU News 4 (2), Fall, 1992.

Ê

5. Trahms, Cristine M. Long-term nutrition intervention model: the treatment of phenylketonuria. Topics in Clinical Nutrition 1(1): 62-72, 1986.

6. Rees, J. M. and Trahms, C.M. The adolescent and phenylketonuria: promoting self-management. Topics in Clinical Nutrition 2 (3) 35-39, 1987.

Table I

Self-management Tasks for Young Children
Age (year)	School level	Learning tasks
2ö3	Preschool	Learns to distinguish yes/no foods
3ö4	Preschool	Learns to count foods: how many Learns concept of formula first
4ö5	Preschool	Begins use of scale for measuring: how much
5ö6	Kindergarten	Begins to prepare own formula with supervision Begins weighing foods regularly on a scale with supervision
6ö7	Grades 1ö2	Begins to list foods on food record

Table II

Self-management Tasks for Children
Age (year)	School level	Learning tasks
7ö8	Grade 2ö3	Prepares formula with supervision
		Lists food on food record
		Understands portion sizes
8ö9	Grade 3ö4	Prepares formula daily with little supervision
		Packs school lunch
		Chooses after school snack
		Prepares simple breakfasts
		Independently lists quantities of foods on food record
10ö11	Grade 5ö6	Prepares formula independently each day
		Prepares week-day breakfasts
		Prepares simple entrees independently
		Consumes full amount of formula independently each day

Table III

Management Tasks for Adolescents and Young Adults
Age (year)	School level	Learning tasks
12ö14	Grade 7ö9	Begins to independently manage total intake for the day
		Responsible for menu planning
		Responsible for food records
15ö17	High school	Responsible for all aspects of self-management with continued parent support
18+	Post-high school	Transitions to adult based clinic care and independent living

NOTE: An Interesting Article by Dr. Morton

The Dec. â94, Vol. 94, No. 6 issue ofÊ Pediatrics printed a speech by Dr. Holmes Morton as a Special Article.Ê In a note prefacing the article, the editor praises Dr. Mortonâs talk.Ê He describes it as moving and thought-provoking÷not your usual journal article.

Dr. Morton gave this speech at the 125th Year Celebration of Childrenâs Hospital of Boston.Ê It reveals the motivating force and factors behind the atypical doctor who established the unique clinic at Strasburg, Pennsylvania.Ê It is a practical, earthy view of his work among common people and his dedication to it.

The article is too lengthy to reprint in our Newsletter.Ê Dr. Morton has kindly given us permission to make copies which are available from our contact person, Dawn Marie Hahn.Ê It is stimulating reading for anyone interested in metabolic diseases and not too technical for parents.Ê Donât miss it.

÷Joyce Brubacher

FORMULA COMPANIES RESPOND

The editor of the National PKU News, Virginia Schuett, asked the three companies marketing medical food in the US to respond to questions PKU parents frequently ask about formulas.Ê She summarized the responses in an article entitled ãAnswers to Your Questions about PKU Medical Foods (Formulas)ä printed in the fall, 1994 issue.Ê Mead Johnson, Ross Laboratories, and SHS North America provide formulas for MSUD and PKU so most of the information in the article is relevant to both disorders.Ê I added MSUD references (italicized in parenthesis) where applicable.

Why canât the formula be made to smell and taste better, when it is the cornerstone of PKU (MSUD) treatment?

All of the companies acknowledge that the smell and taste of the medical foods (formulas) are problems for some people.Ê All three have made major efforts in the past several years to improve the taste and smell, but this is not an easy task.Ê The problem lies in the basic raw materials that need to be used.Ê Formulas such as Lofenalac that are made to be low in phenylalanine through a chemical hydrolyzing process have a very distinct odor and flavor.Ê Phe‑free formulas (all of the other formulas distributed in the U.S.) are made of individual amino acids.Ê Some of the essential amino acids contain sulfur and have a bad taste and smell.Ê Certain non-essential amino acids required for extra nitrogen contribute to the undesirable properties.Ê The vitamins and minerals that need to be included also add a strong taste.ÊÊ Due to the nutritional importance of most of the ãoffendingä substances, it is not feasible to simply remove them.

Masking the flavor and smell is not easy either.Ê No completely adequate coating to encapsulate the bad tasting amino acids has been found.Ê Some flavoring agents actually strengthen the taste of the amino acids; some flavorings can trigger allergic reactions in some people.Ê And of course not everyone likes the same flavor.Ê Use of some ingredients that could improve taste would triple or quadruple the cost and they are not approved by the FDA.

One approach taken recently has been to decrease amounts of certain of the nonessential ãbad‑tastingä amino acids.Ê The new formula distributed by SHS North America, Periflex, uses this approach.Ê Phenex, (Ketonex) by Ross Laboratories, also is an attempt to improve the flavor and odor.Ê Phenex (Ketonex) formula comes with an optional flavoring agent in two flavors.Ê SHS is working on some innovative new presentations of the medical food that they hope will be available in the near future.

Why canât the formula be put into a pill form?Ê Couldnât you just put in the necessary amino acids and leave out all the fat, carbohydrate, vitamins, and minerals to be obtained from another source?

This is a wonderful idea, but there are major obstacles to a pill becoming a successful alternative to other forms of the medical food.Ê Even if only the required amount of amino acids were put into pill form, the pills either would need to be large, or the quantities required to replace all formula would be more than the average child or person could reasonably manage (40 to 60 or more pills per day!)Ê Also, traditional pills require the use of binders and other ingredients to hold the tablets together.Ê These ingredients cause tablets to be less soluble in the gastrointestinal tract.Ê This would decrease the amount of amino acids available.Ê Capsules could be used, but the shells contain protein in the form of gelatin.

There are other potential problems:

n If the medical food did not contain minerals and vitamins, there would be the risk of the child or young person forgetting to take the supplement and becoming severely deficient.

n For infant products, the Food and Drug Administration requires fat, carbohydrate, vitamins and minerals unless the company provides a major medial justification for an exemption.

n Unless a phe‑free (BCAA-free) supplement of fat and carbohydrate is used to supply calories, persons on the diet cannot eat enough regular table foods to meet energy needs without exceeding their phe (leucine) tolerance.

Despite the problems, the Milupa Company of Germany experimentally produced a pill many years ago that was tried mainly in Europe.Ê The pills were not well accepted in the quantities required and the idea was abandoned.Ê Recently, one young woman during her pregnancy hand‑stuffed one of the formulas into empty capsules in order to maintain the diet.Ê But she had to consume 90 to 100 capsules per day and the capsules added 100 mg phe.

Still, the idea of a pill continues to have appeal.Ê Despite the technical and other difficulties mentioned above, SHS North America is exploring a pill form of the medical food as one partial alternative.Ê Clinical trials have taken place in Europe.Ê Results of their marketing research and the regulatory environment here in the U.S. will determine suitability of this product for the North American market.

Why are the formulas so expensive?

As you are aware, the PKU (MSUD) medical foods are made of many compounds.Ê These are obtained from raw material manufacturers located in various parts of the world.Ê The basic nature of the formulas is very complex.Ê The raw materials are rare compounds isolated from batches of starter material.Ê They are extracted and purified to high food or pharmacological grade compounds.Ê Amino acids, and protein hydrolysates treated to remove most of the phenylalanine (leucine), are simply much more expensive per gram of protein than is whole protein in normal infant formulas

Strict quality controls and laboratory confirmation of the manufacturing process also add to costs.Ê Frequent analysis of the formulas is needed to insure that the ingredients meet label specifications.

These and all other costs associated with production are increased on a per unit basis due to the small production of the formulas.Ê Regular infant formula is made in huge batches (60,000 to 120,000 pounds or more); so the cost of analytical tests required by the FDA for it prior to product release and during shelf life is proportionately much smaller per unit.

Also, because of the number of people needing the medical foods, batch sizes made at any one time must be small enough so that it is not outdated by the time it reaches the consumer.Ê This increases costs per unit.Ê In this regard, many state health agencies do not appropriately manage their inventory and often return products after their maximum shelf‑life has passed.Ê These products must be destroyed.Ê At least two of the companies have liberal refund policies for outdated products, adding to costs.

Finally, mark-up on the price of the products when they are distributed through pharmacies can be very substantial and is not controlled by the companies.

The suppliers claim there is so much expense involved in producing the formulas for so few people that they donât make a profit on them.Ê So why do we have so many different companies coming up with new formulas?

In the U.S., only three companies provide medical foods.Ê They are dedicated to providing quality nutritional products for people with inborn errors of metabolism, not to making big profits.Ê The companies also help support many PKU‑related activities such as parent and professional meetings.Ê Mead Johnson, the first company to supply such products, has done so for over 35 years.Ê A reflection of their interest in families is their program called ãHelping Hands for Special Kids,ä which makes sure that children of financially needy families get the Mead Johnson formulas required (interested families should contact their physicians).Ê Ross Laboratories entered the inborn errors of metabolism market a few years ago.

Major formula companies want to provide a complete line of products even if some products are made available at a loss to the company, for humanitarian or public relations reasons.Ê This is also advantageous for selling products to some hospitals, and state health agencies that will not contract with a company for the regular baby formulas unless they have a complete line of metabolic formulas.Ê SHS North America, a company based in England, is the only one of the companies that does not manufacture or market regular infant formulas.Ê Their sole function is to provide disease‑specific nutritional products that will help patients with PKU and other metabolic diseases.

ãThree companies are better than one,ä I say.Ê I think that we should all be glad to have the three trying to better the lives of those who have inborn errors of metabolism like PKU (MSUD).

Why couldnât there be a ready‑to‑use formula?Ê It is very inconvenient to have to mix it up every day, especially when traveling or away from home.

There are many reasons why medical foods have been produced in powder form.Ê These reasons include the following:

n Powders have a longer shelf‑life than liquids (about twice as long).Ê Because of shorter shelf‑life, a liquid form of the formula would cost considerably more per gram of protein than powder (up to twice as much or more).

n Ready‑to‑use medical foods are difficult to make.