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Presented by Cristine M. Trahms, MS, RD, FADA

Cristine Trahms, from the Department of Pediatrics, University of Washington Seattle, presented these models and guidelines for managing a chronic condition - such as MSUD. This material also appeared in Pediatric Nursing 2000.

Children with chronic conditions encounter all the typical challenges of growing up in addition to special ones that stem from their condition and its management. However, overall health outcomes remain similar despite the presence of the chronic condition. These outcomes are well detailed in the Bright Futures model (See graphic). The model begins with the development of a therapeutic alliance when the child is an infant and progresses through a continuum of social, developmental, and health outcome achievements that lead to an independent, healthy adult. Children with chronic conditions should achieve these same health outcomes.

Children and youth ideally progress through appropriate developmental stages to become knowledgeable and capable participants in their own care. Typically this developmental process parallels development in other areas of the children's lives. When successful, the process enables children to share in the management of their health condition and to achieve the outcomes articulated in Bright Futures.



Bright Futures Health Supervision Outcomes

Central to the concept of health supervision is the belief that specific preventive and health-promoting interventions lead to desired outcomes. The social, developmental, and health outcomes summarized below contribute to the overall health and well being of infants, children, adolescents, and families. These outcomes occur along a continuum, varying in their timing from child to child and family to family.


Taken from Bright Futures: Guidelines for Health Supervision of Infants, Children, and Adolescents, M. Green, Ed. Arlington, VA: National Center for Education in Maternal and Child Health, 1994. http://www.brightfutures.org

Developmental models underline the concept that learning is based on cognitive readiness. The developmental approach also provides a forward momentum for the child's self-management skills and responsibilities. Parents monitor the child's progress and emotional status and support the child's efforts and negotiate changes in responsibility as appropriate. By working with the child, rather than doing for the child, the parent comes to know when the child has mastered skills at the current level and is ready to move on.

The second model, the Leadership model, shows the direction of shifting responsibility for care of the chronic disorder from parent to child. It depicts by bold arrows the need for the parent to guide this movement and the important idea that both parents and child have a role to play. (See graphic)

The Leadership model indicates the dynamic nature of the parent-child relationship during effective management of a chronic disorder. Initially, the parent provides all of the necessary care of the child, regardless of the child's age. As the child matures in cognitive and physical skills and gains experience in managing the condition, the parent transfers some of the responsibility for self-care to the child. The parent becomes the manager and the child the provider for these carefully articulated skill-appropriate responsibilities. The parent is available to support the child's provider skills and stands ready to re-assume some of these tasks for a short time if it is necessary because the child is ill or other life complications require additional parent support.

(LEADERSHIP MODEL GOES HERE) put heading The Leadership Model

As the child becomes more confident and competent in self-management, the parent and child negotiate the next step. The parent becomes the supervisor and the child becomes the manager of specific tasks. The parent is, again, poised to resume the managerial role or specific tasks for a short time, if necessary. However, the parent and child must both understand and agree that the child should not be allowed to regress to a previous stage. The parent provides additional support during times of stress, and the child continues to develop self-management skills and responsibility.

Eventually, the parent assumes a consultant role in the child's management of his/her disorder and the child becomes the supervisor, manager, and provider of care. The parent supplies information, support, decision-making guidance, and resources, but the child assumes ultimate responsibility for his/her health care as the CEO.

The question remains: How do families keep focused on the big picture of their child's general growth, implement the actions necessary over time to move their child forward in shared management, while remaining ever mindful of the condition specific needs of their child? Successful families operate by changing the focus to immediate medical needs or to normative family life depending on the current needs of the child and family.

Each child with a chronic medical disorder has a component of specific immediate medical needs that must be interwoven into normative individual, family, and community life. During times of wellness, these specific medical needs may appear to be in the background and remain relatively out of focus. They are, however, present and always able to be brought back into clear focus by the family if the need should arise.

The models require bringing together many components into an effective dynamic system that is day-to-day family functioning. Some of the components can be systematized so that they function as integral parts of normative family or community life and thus effectively guide management of the chronic condition so that it remains in normative rather than crisis focus. Some systematic approaches that have been reported as effective are:

  1. A system of monitoring of the condition. Parents who are successful develop a system for monitoring the disorder that is integral to family daily life and activities. Some successful system components are (1) the weekly structured family meeting; (2) disease management tasks integrated as a part of daily family tasks, i.e., the family chore schedule indicates take out the garbage, brush your teeth, drink your "milk," pack your lunch for school; (3) a notebook is kept on the kitchen counter for recording food eaten or other activities related to medication (notes are added as events occur).
  2. A system for involving the child at skill level. The tasks that are the child's responsibilities are based on the child's physical and cognitive capabilities, e.g., a toddler may count the number of crackers for lunch while a third grade student may prepare formula independently on Saturday morning.
  3. A system for evaluating the child's success/errors and remediating these errors. Parents and family members support success by pointing out to the child evidence of successful management as it occurs, such as consumption of formula without a reminder, as well as the natural consequences of poor management, such as developing strategies to insure that after school formula is consumed.
  4. A system for celebrating small successes. If something is a two-stage process, celebrate each step along the way rather than just the final outcome. For example, celebrate the child's remembering to select the correct snack foods at a surprise party for a friend, as well as maintenance of a low weekly branched- chain amino acid level.
  5. A system to prevent parental burnout. Parents need ongoing support for themselves since management of the disorder is a lifelong parental and child activity. Support of the child, emotional or physical, doesn't stop at age 10, or 13 or 18 years. Successful children have parents who remain involved in a qualitatively different way but do remain involved in the child's life. Parents who are able to remain consistent and supportive of their child but also negotiate the direct management role based on the child's skills and needs do not suffer from "parenting burnout" as readily as parents who assume the total and on-going responsibility for the child's health and health management.

Effective parental support of a child with a chronic condition requires a thoughtful parenting strategy. Parents do not have the luxury of casual or inattentive parenting. They are required to be focused and anticipatory in their parenting style if their child is to successfully accomplish both the developmental tasks of childhood and disorder management.



Actions that Support Leadership Skills

Stage/AgeChild Capabilities/Actions That Form the Basis for Leadership SkillsParent's Leadership/Actions to Support the Child's Growing Capabilities
Infant

(0-12 months)

Though dependent on parents for care, it is helpful if the child gives clear cues of distress so parents can grow in the recognition of emergent needs and make appropriate responses

For example, clear cues of hunger and satiety help the parent understand when to offer formula and when to withdraw it

Clear cues of optimal health (adequate growth, development, and social interaction) also enable parents to identify the positive impact of their actions to promote good management

Learn ramifications of the condition and how/what resources can help

Learn how to ask questions that can assist managing the condition in the context of an overall healthy living pattern

Participate in support activities to increase knowledge of disorder and its management

Develop routine regarding daily treatment that fits with family life patterns

Recognize signs of immediate distress and seek emergency care

Recognize signs of early distress and seek evaluation

Learn to acknowledge those challenges that are developmentally typical for most children versus challenges specific to the child's condition

Learn how to share information with extended family and daycare providers

See/acknowledge evidence that the child is thriving under attentive management

Assume the role of "repository for condition specific information" regarding the child's reaction to the treatment

Toddler

(1-3 years)

Cooperate with routine treatments

Help hold equipment and work with parent to make equipment function as needed, for example, use of blender to prepare formula

Develop a sense that parents are a source of help/comfort

Accept constraints of condition and treatment with limited behavioral acting out, for example, "yes foods/no foods"

Understand firm limits of parents, for example, "no"

Develop rituals regarding treatment so child knows what to expect and can begin to learn through repetition

Begin to recognize that the child needs to have roles in the management of the condition

Identify possible roles the parents are willing to begin to share with the child

Change the established management routine based on the child's growing capabilities and areas of cooperation

Continue to build clinical and community support network

Preschool

(4-5 years)

Identify body parts important to early identification of a problem or treatment

Test limits of cooperation

Magical thinking may lead to fears

Imitate adult's behaviors

Learn labels for condition specific "problems" in order to communicate treatment needs

Learn labels for feelings associated with condition and its treatment so can communicate feelings

Acknowledge regressions, allow very brief period of reorganization and then resume and praise prior skill performance

Set fair and appropriate limits

Model acceptance of the management routines and limits

Encourage some flexibility in rituals of treatment so child begins to experience multiple ways to accomplish same goal

Develop relationships with school personnel regarding specific needs

Early School Age (6-9 years)Recognize and act on 1 or 2 of major internal body cues of a problem

Actively participate in concrete monitoring of condition

Increase understanding of condition, i.e., cause and effect, a concrete level of what's going on inside the body to necessitate management

Continue to label cues and give positive reward for child's recognition

Start negotiating with child for what each party will do regarding management and set criteria for forward movement that fits with family life

Be prepared to re-negotiate for cause!

Establish logical consequences for actions

Negotiate the "rules" for working together to get all necessary treatments completed

Be positive and reinforcing about what needs to get done

Support normative activities and integrate treatment needs

Model telling others about the disorder for the child

Discuss the approach to telling teachers, friends, coaches, etc. about the disorder and the amount of detail necessary to share

Late school age (10-12 years)Increased level of understanding of condition - begin to understand long-term needs

Use labels that are medically correct in order to effectively discuss with providers

Learn how and when to respond to peer pressure yet still take care of self

Enact most psychomotor skills associated with treatment with parental support

Learn more sophisticated system for reporting symptoms, management steps, outcomes

Develop specific set of self-management tasks that are completed independently

Remain present for the child that is involved in care and monitoring decision-making

Accept the manager versus CEO role in much of treatment

Insure child has told important others: friends' parents, coaches, etc. of the condition and what assistance they could provide if needed

"Be there" in case of emergencies and new aspects of disorder

Provide the tools so the child can self-manage (get the formula, get the prescriptions)

Support the child in actively communicating with their provider

Encourage discussion of the child's monitoring system so as to help them grow in understanding

Early adolescentMain manager of daily, routine care

Develop strategies to complete all of the necessary routine management tasks

Know how to effectively ask for assistance in complex situations

Know where can be flexible vs. not flexible and be able to enact the flexibility when appropriate

Shadowing of parent activities

Negotiating and re-negotiating of who does what. Becoming the consultant versus remaining the manager

Discuss new issues (sex/drug/alcohol) for their normative and any special condition effects

Late adolescentMake a commitment to lifetime treatment

Increase understanding of the disorder and its long term as well as short term consequences on other aspects of life- vocations, intellectual achievement, well being etc.

Sense of self as capable manager of disorder

Integrate the realities of the condition with the invincible nature of their years

Appreciate benefits that the constraints of management allow

Continue to develop more independent clinic and community support network as transition to adult-based care services

Develop a flexible way of communicating with the youth in order to stay informed while not seen as interfering

Remain present for support and problem solving with the youth

Provide support and guidance as the youth transitions from pediatric to adult care services

C.M. Trahms and G. Keickhefer, "Chronic Illness in Children: Supporting the Development of Children as They Move From Compliance Toward Shared Management." Pediatric Nursing, 2000.

MSUD Management Timeline


Age of Child

Tasks for Children and Parents

0-6 monthsParents learn about and adjust to MSUD
6 monthsStart low-protein solid foods
6-7 monthsIntroduce cup
8-9 monthsIntroduce finger foods
10-15 monthsConsider weaning from bottle (discuss transition with clinic staff)
2-3 yearsLearn the concept of "formula first"
Learn to distinguish "yes" and "no" foods
4-5 yearsBegin to learn to count foods - "how many"
Begin to use scale - "how much"
5-6 yearsAssist in formula preparation
Teach children how to deal with other children's curiosity about MSUD
7-10 yearsPrepare formula with decreasing supervision
Choose after school snack
Learn to pack school lunch
Begin to list foods on food record
Begin weighing food regularly on scale
10-12 yearsBegin to prepare and consume formula independently each day (with parental monitoring)
Prepare simple entrees independently
Know what blood levels are ideal
13-14 yearsIncreasing self-monitoring (with continued parent support) in formula preparation and consumption Independently manage total leucine intakefor the day
Learn menu planning
Responsible for food records
15-17 yearsResponsible for all aspects of self-management
Able to do "finger poke" for blood test
Able to explain basics of MSUD - "What is it?" Responsible for remembering recent blood levels Continued parent support
18 yearsTransition to adult-based clinic care
Ready to live independently, including: - formula preparation and consumption - food preparation and records
- blood tests for serum BCAA levels as requested

University of Washington, 2000

CHANGE THE LIVES OF MSUD ADULTS AND CHILDREN

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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