1. Interdisciplinary Management of Cystic Fibrosis Patients By Patricia J. Settle, MS, RD Pediatric Pulmonary Center Department of Pediatrics University of Arizona College of Medicine

2. 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

3. 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. Food is an Integral Part of Our Lives

4. 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.

5. Case Study - FFT Maggie’s 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.”

6. Case of Failure to Thrive CF

7. What emotional factors might be involved?

8. Household stress and tension Household stress and tension Maternal depression Maternal depression Mother’s own parenting Mother’s own parenting Maternal problems in coping Maternal problems in coping Maternal anger, denial, guilt Maternal anger, denial, guilt Maternal Stress Factors

9. Various studies show adherence to a high energy diet in children is between 16% and 50% Another Family Stress: High Energy CF Diets StudyAge% Adherent Tomezski, 19925-1023% Stark, 19952-520% Stark, 19976-1250% Anthony, 19987-1216%

10. 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

11. 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 child’s autonomy in eating

12. 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 ?

13. Overview of Behavioral Issues Behaviors Associated With Children Behaviors Associated With Parents Strategies for Behavioral Modification

14. 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

15. Sam’s 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 Sam’s brother

16. 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 child’s eating Starting altercations with siblings

17. 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

18. 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

19. 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

20. 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

21. CF Parent Feelings Parents with CF children feel: Tremendous pressure to push their child to eat large amounts of food when the child doesn’t feel like eating Fearful that that the child's not eating will “hurt” the child’s health

22. 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

23. 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

24. 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

25. 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

26. 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 child’s usual intake and preferences Provide feedback on progress Making Nutrition Education Behavioral

27. 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”)

28. 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

29. 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

30. 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

31. 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)

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

33. 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

34. 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

35. 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

36. Praising A parent’s 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 parent’s 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

37. Praising Increases child’s 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

38. 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.

39. 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”

40. 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!

41. 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 child’s choice  Access to video games  TV viewing

42. 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

43. 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

44. 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 parent’s signature: _____________________________________________________

45. 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

46. 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 Provide Emotional Support

47. 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

48. 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 wasn’t a big deal. No one was turned off that I took them and I felt a lot better.”

49. 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 wouldn’t 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.”

50. Title

52. 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

53. 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. References

54. Case Studies – Parent Perspective Felicia “The Poster Child” Makes parent feel successful Has hunger, eats well, is compliant Experiences typical adolescent behavior and responds positively to input by healthcare providers Andrew “The Problem Child” Makes parent feel like a failure Is never hungry, doesn’t eat, non-compliant Apathetic to continued input and education from healthcare providers

55. Observation of Child Behaviors Meals in families with a CF child tend to be excessively long