This is a summary of a talk given by Cristine M. Trahms, M.S., R.D. at the MSUD Symposium in June of '94. Cristine is with the Department of Pediatrics, Division of Pediatric Genetics, University of Washington, Seattle, Washington. Several parents requested copies of the information she presented, and she graciously submitted this article. 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. 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. 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. 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. However, children with MSUD must themselves learn to adequately follow the therapy to the best of their abilities. To support children intellectually and emotionally as they begin self-management requires a basic framework built upon principles of learning. 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. 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. 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 child's readiness to move forward.

Piaget's 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 2-6, 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 7-11, 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 12-15, which is adult style thinking with the ability to deduce and reason and learn to make decisions independently.

The practical application of Piaget's constructs is that:

  1. Learning is based on cognitive readiness.
  2. Parents and children must work together through the process one-step-at-a-time.
  3. Parents work together to decide priorities for their child, that is, negotiable vs. nonnegotiable behaviors and what level of compliance is expected.
  4. The more integrated the child's 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. The operational aspects of learning become more sophisticated as the child becomes cognitively more mature, but the goals themselves do not change. To be effective in promoting learning, education must be directed at the level of the learner. 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 infant's cues of hunger and satisfaction. Some of these guidelines are:

  • The food patterns of young children are shaped by many factors that parents can facilitate or ignore.
  • Food acceptances of infants and young children are shaped by an innate preference for sweet tastes.
  • The attitudes and acceptance of foods by adults and peers has a profound influence on food acceptance by the older infant and young child.
  • 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.
  • Acceptance of foods is also based on learning-that is, familiarity and presentation of the food in a form that is easy for the child to manage.
  • 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 child's experience with as many forms of individual "yes" 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. 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 child's 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. 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. 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. A model for supporting the development of self-management skills is presented here.

  1. Bybee, R.W. and Sund, R.B. Piaget for Educators, 2nd edition. Charles Merrill Pub. Co. 1982.
  2. 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.
  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.
  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
2-3 Preschool Learns to distinguish yes/no foods
3-4 Preschool Learns to count foods: how many
Learns concept of formula first
4-5 Preschool Begins use of scale for measuring: how much
5-6 Kindergarten Begins to prepare own formula with supervision
Begins weighing foods regularly on a scale with supervision
6-7 Grades 1-2 Begins to list foods on food record
Table II
Self-management Tasks for Children
Age (year) School level Learning tasks
7-8 Grade 2-3 Prepares formula with supervision
Lists food on food record
Understands portion sizes
8-9 Grade 3-4 Prepares formula daily with little supervision
Packs school lunch
Chooses after school snack
Prepares simple breakfasts
Independently lists quantities of foods on food record
10-11 Grade 5-6 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
12-14 Grade 7-9 Begins to independently manage total intake for the day
Responsible for menu planning
Responsible for food records
15-17 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. '94, Vol. 94, No. 6 issue of Pediatrics printed a speech by Dr. Holmes Morton as a Special Article. In a note prefacing the article, the editor praises Dr. Morton's talk. He describes it as moving and thought-provoking-not your usual journal article.

Dr. Morton gave this speech at the 125th Year Celebration of Children's Hospital of Boston. It reveals the motivating force and factors behind the atypical doctor who established the unique clinic at Strasburg, Pennsylvania. 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. Dr. Morton has kindly given us permission to make copies which are available from our contact person, Dawn Marie Hahn. It is stimulating reading for anyone interested in metabolic diseases and not too technical for parents. Don't miss it.


The MSUD Family Support Group is currently funding several research projects and we are proactively looking for researchers interested in developing new treatments or finding a cure for MSUD. Significant funding is necessary if we are to accomplish this goal.
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