Interdisciplinary Management of Cystic Fibrosis Patients

By Patricia J. Settle, MS, RD

Pediatric Pulmonary Center Department of Pediatrics University of Arizona College of Medicine

Improving Nutrition is a Team Effort

It is important for the entire CF team to: Be educated on the importance of nutrition
Emphasize the importance of nutrition

But also to understand that the nutritional aspects of CF care often result in concurrent psychosocial family issues

Food is an Integral Part of Our Lives

In addition to meeting caloric needs, we use food for:
Comfort Expressions of emotion Reward Punishment Socialization

It becomes very complex when we try to use food as part of a CF treatment plan, because we use food to meet so many other needs besides nutrition.

Case Study - Failure to Thrive

Maggie K is a 23 month old child. Maggie was diagnosed

with cystic fibrosis at age 6 months with symptoms of chronic cough and failure to thrive. She is the only child of an accountant and a stay- at- home Mom. Both sets of grandparents live close by and are involved in Maggie's care. Maggie was started on standard CF nutrition therapies including pancreatic enzymes, vitamins, and high calorie infant formula.

Case Study - FFT

Maggies mother relates that she will only eat a very few

foods such as hot dogs, pizza, and ice cream. She prefers to drink liquids such as juice and tea to milk. Mother states that meal times have become "very stressful. Maggie refuses to sit at the table and "screams" when she is given any foods she is not familiar with. Mrs. K states "I am so worried about Maggie's weight and her eating." She admits that she wakes up at night and thinks about Maggie and her diet. She also states that she does not allow Maggie to play with other children or attend preschool because "she might get sick.

Case of Failure to Thrive CF

What emotional factors might be involved?

Staff Anxiety

Mothers Anxiety

Childs Anxiety

Maternal Stress Factors

Maternal depression

Maternal problems in coping

Another Family Stress: High Energy CF Diets

Various studies show adherence to a high energy diet in children is between 16% and 50%
Study Tomezski, 1992 Stark, 1995 Stark, 1997 Anthony, 1998 Age 5-10 2-5 6-12 7-12 23% 20% 50% 16% % Adherent

Why Are Adherence Rates so Low?

Adherence to a high energy diet is hard because CF nutritional guidelines:
Are just one part of complex treatment regimes

Are time consuming to plan

Require extensive lifestyle change Require long-term change

May be conflict with eating patterns of other family

members Are often not associated with immediate feedback

Why Are Adherence Rates so Low?

Tend to blow the nutrition issue out of proportion
Force parents into treating the CF child with special

care instead of equally with siblings Complicates normalization of family life and meal times with respect to food choices Presents challenge for parents to maintain their own weight goals Interferes with the development of a childs autonomy in eating

What We Know
CF Children, ages 6 months to 12 years, consume
100% of the Daily Recommended Intake (DRI) But not the 110%-200% required by the CFF

Recommendations
Are psychosocial issues a factor in our inability to meet the

CFF Recommendations?

Overview of Behavioral Issues

Behaviors Associated With Children
Behaviors Associated With Parents Strategies for Behavioral Modification

Sam, a typical CF child

Take Sam:

Constantly asked, in a whiny voice, how much more he had to eat Argued and negotiated each bite with parents Tried to distract parents by telling long stories to delay eating Complained of being full from beginning of meal

Sams Parents
Used coaxing to encourage Sam to eat Turned eating into a game: counting bites,

chanting Go, Go, Go! Gave Sam their full attention when he was NOT eating When Sam was eating, the parents used the opportunity to talk to each other or Sams brother

Typical CF Child Behaviors

Dawdling
Excessive talking and/or chewing Complaining and whining

Arguing or negotiating about food

Prolonging mealtime, especially by talking in an attempt to distract parents from focusing on the

childs eating Starting altercations with siblings

Typical CF Parent Behaviors

Coaxing or coercing child into eating

Typically focusing all the attention on the CF child in an attempt to get the child to eat more. The more the child resists, the more attention the child gets.
Often turning their attention to other children or each other only when the CF child is engaged in

eating because it is the first time they feel able to divert their attention away from the CF child

Typical CF Parent Behaviors

The main strategy parents have is to keep the child at the table longer. This leads to higher rates of negative child behaviors and increased parent behaviors Parents often make a second meal to accommodate the CF child

Comparison of Child Behaviors

Children with CF and healthy peers engage in the same pattern of behavior during a meal. In the second half of the meal, children of both groups:
Eat less

Refuse food more

Leave the table more Are more noncompliant

However, children with CF engage in these behaviors at

twice the rate of children without CF

Comparison of Parent Behaviors

Parents of children with and without CF try similar strategies to encourage eating and show the same pattern of increasing their efforts in the second half of the meal:
More commands More coaxing More feeding More physical prompts

However, parents of children with CF are engaging in these behaviors twice as much as parents of children without CF

CF Parent Feelings
Parents with CF children feel:
Tremendous pressure to push their child to

eat large amounts of food when the child doesnt feel like eating
Fearful that that the child's not eating will

hurt the childs health

CF Parent Feelings
Concerned that physicians will think parents

are not following through on nutrition

Frustrated and exhausted for trying so hard Defeated when the child cannot or will not

comply
Worried about the effects of the mealtime

stresses on other siblings

Related Observations
Many parents of CF children did not have specific

caloric goals the more food the better there is never a stopping point Many parents increased caloric intake by increasing food volume, not adding calorically dense foods

Common Misconceptions
Myths or family bias that may have crept into the feeding situation:
Families thinking a low fat diet is good Parents feeling guilty about the number of

pills (antibiotics, vitamins, acid blockers, appetite stimulants, enzymes) a child has to take, thus they give snacks that do not require enzymes

Common Misconceptions
Parents believing that increasing enzyme

dose means their child is more ill not that the child is growing or eating more food or that the food is higher in fat content

Making Nutrition Education Behavioral

Set goals and gradually increase calories one meal at a time so parents and kids know when to stop and can feel good at the end of a meal
Provide individual suggestions of food choices and boosters based on childs usual intake and preferences Provide feedback on progress

Making Nutrition Education Behavioral

Start With Snacks Most parents are not routinely giving 2-3 snacks a day Even if giving snacks, most are not giving the most calorically dense foods It is easy to increase calories through snacks because snacks can be given throughout the day Snacks are not as stressful as meals because they usually do not require preparation (quick and easy)

Making Nutrition Education Behavioral

If more calories are needed after increasing snacks,

then chose to augment the meal that has the lowest number of calories and/or the meal the family identifies as the easiest to target

Sometimes a Referral is Needed

Typical child behaviors and parenting strategies may :
Be insufficient and create barriers Inadvertently reinforce not eating giving attention to

children when they are engaged in behaviors incompatible with eating such as dawdling, pouting, complaining, excessive talking, leaving the table

Behavioral Intervention Needed

When parents consistently express concern about child behaviors during meals, it is helpful to equip parents with extra skills to enable them to work more effectively with their children

Behavioral Intervention
Provide behavioral skills in addition to presenting nutritional recommendations
Examples: Teach parents to set limits on meal length Provide reward for appropriate eating such as compliments, attention, and activities Ignore behaviors incompatible with eating (this is hard)

Behavioral Modification
Reinforcement Setting Rules Praise positive eating Contingent Privileges Shaping Behavior Reward system Behavioral Contracting Ignore negative eating

behavior
behavior

Reinforcement
Reinforcement - An event that makes the behavior that precedes more likely to occur in the future
It can be:
Positive: A compliment, hug, pat on the back Negative: A scolding, nagging Verbal

Physical

Setting Mealtime Rules

A rule clearly states IN ADVANCE a relationship between a

specific behavior and a specific consequence

At meals, rules are used for things like getting up and

leaving the table before eating the required amount of food

A reason for the rule should be given like it is important

that you eat your dinner to grow and to stay strong and healthy
Parents should be consistent with rules

Mealtime Rules
Rules should be not be presented at meal time. This

prevents the parent from being drawn into a negotiation about the rule at the time a child has misbehaved while eating
The parent should sit down with the child at a time

other than a mealtime, state the rule simply, and provide the reason for it. The child should be asked to repeat the rule back to the parent to make sure the rule and consequences do not come as a surprise to the child

Praising
A parents attention is a valuable reward to a child.
Through intervention, parents are taught to notice and compliment behaviors that are compatible with eating
Listening and following parents instructions Taking bites Taking one bite after another Chewing and swallowing more quickly Loading their fork while talking Eating a bite of food before talking

Praising
Praising
Increases childs desirable behaviors
Teaches child what a parent likes Motivates child to please the parent

How to provide praise:

Describe specifically what child is doing that the parent

likes Actively compliment the child often Be timely provide praise immediately when child does things the parent likes

Praising Statements
I really like the way you take a bite, talk, and then take another bite. I like the way you are sitting up in your chair and eating. I enjoy meals when you are eating so well and we can discuss your day while we eat.
The parent should be encouraged to praise the child in a way that is comfortable and natural to both the parent and child. It may seem uncomfortable or awkward at first and may take some time to find the best style for parent and child.

Ignoring
Behaviors parents are taught to ignore:
Excessive talking or story telling that interrupts

eating for more than 10 seconds Complaints about food or amount Whining Child sitting without taking bites Child chewing for prolonged time Goofing around

Ignoring
When using ignoring, parents should:
Continue conversations with spouse and other children Be ready to give attention to the CF child immediately if he

engages in a desirable behavior

Timing Is Very Important!

Contingent Privileges
The awarding of privileges to the child for meeting his

calorie/meal goal
Giving child something he desires for doing something

good for himself (eating sufficient calories) One-to-one time with a parent doing an activity of the childs choice Access to video games TV viewing

Contingent Privileges
The privilege system will not work if:
The awarding of the privilege is more important to the parent than to the child The child does not have a vested interest in the reward and would rather forego award than eat

The parent cannot be consistent in awarding privileges

Behavioral Contracting
Formalizes the use of contingency management

Defines the behavior that is to occur

Defines the consequences that will be delivered, by

who and when

Energy Contract
This week, I agree to: Date: __________________ 1. Get more energy at snack, breakfast, and lunch by eating the food my Mom or Dad gives me. 2. Eat the same amount of food at dinner. My Mom and Dad will tell me how much I need to eat. 3. Eat my meals within the time limit. My parents will tell me how much time I have to eat my meals. When the time is up, they will take away my plate. If I work really hard and meet my energy goals, then my parents agree to let me choose one of the activities written here: ______________________________ ______________________________ My signature: _________________________ My parents signature: ____________________________

Shaping
The gradual attainment of a target behavior through the rewarding of successive steps that gradually build upon one another
Food acceptance is increased Calorie goals are broken down by meal so only one meal is

targeted each week Calorie goals are gradually increased each week until the end goal is achieved

Start with small amount of food on plate Child needs to taste (put to tongue) Child needs to take one bite

Provide Emotional Support

Explore sources of parental anxiety/depression Relaxation techniques or psychotherapy Provide calm, confident environment (hard if child

is at risk -- paradox)
Help families feel supported to offset helpless or

fatalistic feelings

Adolescent and Adult Issues

Adolescents and adults should self-monitor to judge

whether energy needs are obtainable orally

Contracting can be with healthcare providers instead of a

parent
Patients should set their own goals that are small,

reasonable and gradually build over time

Patients should be encouraged to self-reward

Families experience reduced stress once adolescents can

drive to go out for meals and siblings mature and understand the demands of CF on their sibling

Issues Specific to Adolescents

Allan Age 17 I had a hang-up for the longest time about taking my enzymes in front of other people. I never wanted to stand out or be different from the other kids at school. So sometimes I skipped taking them altogether. It got to the point that the greasy, high-calorie food in the cafeteria was really causing me problems with malabsorption.
Dealing with gas and bloating was even more embarrassing. So I decided it was worth trying to answer questions about why I take enzymes and to take them before I ate. It turned out that it wasnt a big deal. No one was turned off that I took them and I felt a lot better.

Issues Specific to Young Adults

James Age 19 I think one of the things that was hard for me to deal with was that lots of people thought I was younger than I really was in high school. I was shorter and smaller than most everyone else. I was really worried, too, that I wouldnt grow or reach puberty when everyone else did. I hated being different.
What helped me adjust was I really made an effort to eat a lot and keep my weight up. I wanted to do everything I could to help myself grow. Finally, I grew some and was just about as tall as some people in my class. And even though I was still little, I joined the swim team and did pretty well. Just getting into something I liked doing helped me feel a lot better about myself.

Title

References

Stark, L.J., Bowen, A.M., Tyc, V.L., Evans, S.J., & Passero, M.A. (1990). A behavioral approach to increasing calorie consumption in children with cystic fibrosis. Journal of Pediatric Psychology, 15, 309-326. Stark, L.J., Knapp, L.G., Bowen, A. M., Powers, S.W., Jelalian, E., Evans, S., Passero, M.A., Mulvhill, M.M., & Hovell, M. (1993) Increasing calorie consumption of children with cystic fibrosis: Replication with two-year follow-up. Journal of Applied Behavior Analysis, 26, 435-450. Stark, L.J., Mulvhill, M.M., Jelalian, E., Bowen, A. M., Powers, Tao, S., Creveling, S., Passero, M.A., Harwood, I., Lapey, A., Light, M., & Hovell, M. (1997) Descriptive Analysis of Eating Behavior in School-age Children With Cystic Fibrosis and Healthy Control Children. Pediatrics, 99, (5) 665-671.

Stark, L. J., Opripari, L.C., Spieth, L.E., Jelalian, E., Quittner, A. Q., Higgins, L., Mackner, L., Byars, K., Lapey, A., Stallings, V.A., Duggan, C. (2003) Contribution of behavior therapy to nutrition adherence in cystic fibrosis: A two-year randomized controlled study. Behavior Therapy, 34, 237-258.

References

Crist W., McDonnell P., Beck M., Gillespie CT, Barrett P., Mathews J. Behavior at mealtimes and nutritional intake in the young child with cystic fibrosis. Developmental and Behavioral Pediatrics 1994; 15: 157-161. The Behavioral Treatment Be In Charge! www.oup.com/us/pediatricpsychology University of New Mexico CF Center, Incorporating Behavioral Management Into Dietary Counseling, Cystic Fibrosis Foundation, Adapted by Angie M., King, MS, PPC Nutrition Fellow, August 2007. Cystic Fibrosis Nutrition Guidelines: Optimizing Strategies to Improve Nutrition. Cystic Fibrosis Foundation Webinar, May 27, 2008.

Case Studies Parent Perspective

Felicia The Poster Child
Makes parent feel

Andrew The Problem Child

Makes parent feel like a

successful Has hunger, eats well, is compliant Experiences typical adolescent behavior and responds positively to input by healthcare providers

failure Is never hungry, doesnt eat, non-compliant Apathetic to continued input and education from healthcare providers

Observation of Child Behaviors

Meals in families with a CF child tend to be excessively long