1. Nutrition and Digestive Health in Cystic Fibrosis
Hello and thank you for joining us for today’s presentation, Nutrition and Digestive Health in Cystic Fibrosis.
I’d like this presentation to be more of a group discussion, so as we go through the slides, I will pose some questions, which we can then discuss. 1
© 2013 Aptalis Pharma 5/13 ZP J

2. Deborah Kaley, RN, MSN, AE-C, CCRC Bay Area Pediatric Pulmonary
Faculty Disclosures
Deborah Kaley, RN, MSN, AE-C, CCRC
Nurse Case Manager
Bay Area Pediatric Pulmonary
Oakland, California
This interactive discussion is given on behalf of Aptalis Pharma™, which has provided compensation for this presentation.
Other Speaker Disclosures: None
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3. Learning Objectives
Review clinical insights for digestive health management of patients with Cystic Fibrosis (CF) and Exocrine Pancreatic Insufficiency (EPI)
Understand the challenges associated with transitions in care
Review key components of nutrition for patients with CF and EPI
Evaluate social and economic challenges encountered by patients with CF
Review the unique offerings and rewards of the Live2Thrive™ Patient Support Program
The learning objectives for today’s presentation are to:
Review some clinical insights around digestive health management for patients with Cystic Fibrosis (CF) and Exocrine Pancreatic Insufficiency (EPI)
Understand the challenges associated with transitions in care
Review some of the key components of nutrition for patients with CF and EPI
Evaluate some social and economic challenges faced by those with CF
Review the unique offerings and rewards of the Live2Thrive™ Patient Support Program

4. Digestive Health Management in CF
Rogers/pg10/col1/P1/ln10-14
The gastrointestinal tract is often affected in CF:
Thick mucus secretion and inflammation
Progressive destruction of the pancreas
Insufficient amount of digestive enzymes
Diminished absorptive function in the small intestine
Key Takeaway:
The gastrointestinal tract is often affected in CF, leading to deficits in growth and poor nutritional status
The gastrointestinal tract is often affected in CF
The thick mucus secretion and inflammation associated with CF can lead to progressive destruction of the pancreas
Correspondingly, an insufficient amount of digestive enzymes is delivered to the small intestine, causing diminished absorption of nutrients
This is the reason for poor digestion and absorption of food in CF, which can result in growth retardation and poor nutritional status
Reference:
Rogers CL. Nutritional management of the adult with cystic fibrosis—part I. Nutrition Issues in Gastroenterology, Series #113. Practical Gastroenterol. 2013;37:10-24.
Poor digestion and absorption of food
Growth retardation and poor nutritional status
Rogers CL. Practical Gastroenterol. 2013(January):10-24.

5. Adequate Nutrition in CF Is Fundamental
Higher body weight has been shown to correlate with good lung function in CF patients1
Many people with CF cannot digest/absorb nutrients and fat from food due to pancreatic insufficiency2
CF patients require 110% to 200% more calories than people who do not have CF3
A balanced, high-calorie diet with salt, fat, and protein is crucial for people with CF2
Good nutrition promotes a strong immune system to fight infections; fewer infections can minimize lung damage and extend life2
The CF care team dietitian plays a critical role3
Rogers/pg13/col1/P2/ln13-16
Stallings/p837/figure 8/ row 1
CFF/pg1/P1
Key Takeaway:
Optimization of nutritional status for CF patients is critical
Adequate nutrition in people with CF is a fundamental part of disease management
For all ages, higher body weight has been shown to correlate with good lung function1
Because people with CF cannot fully digest or absorb nutrients and fat from food due to pancreatic insufficiency, they require 110% to 200% more calories than people who do not have CF2
Therefore, it is crucial for people with CF throughout their lives to eat a balanced, high-calorie diet with salt, fat, and protein, so that they can achieve3:
Normal gains in weight and height for children3
Puberty and maintenance of health in teens4
Maintenance of weight and health in adults3
Good nutrition also helps to build and maintain a strong immune system to fight infections. Fewer infections can minimize lung damage and help extend life3
A dietitian specializing in the needs of people with CF can work with clients as part of the multidisciplinary CF care team to assure weight maintenance, sufficient levels of vitamin and minerals, and minimal complications1
References:
1. Rogers CL. Nutritional management of the adult with cystic fibrosis—part I. Nutrition Issues in Gastroenterology, Series #113. Practical Gastroenterol. 2013;37: Stallings VA, Stark LJ, Robinson KA, et al. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108: Cystic Fibrosis Foundation. Nutrition. Cystic fibrosis: Changes through life. NutritionThroughLife/Nutrition-and-CF-Changes-Through-Life.pdf. Accessed March 30, Cystic Fibrosis Foundation. Nutrition for teens with cystic fibrosis. Accessed March 30, 2013.
Rogers/pg13/col1/P2/ln13-16
Stallings/p837/figure 8/ row 1
1. Rogers CL. Practical Gastroenterol. 2013;37: Cystic Fibrosis Foundation. Accessed March 30, Stallings VA et al. J Am Diet Assoc. 2008;108:
CFF/Life/pg1/P1
CFF/Teens/pg1/P1
CFF/Life/pg1/P1
Rogers/pg22/col2/P2/ln3-10

6. Clinical Guidelines for Nutrition Management in Patients With CF
Recommendations include1:
High-calorie diet, including supplements when needed
Behavioral intervention to encourage good eating habits in children
Keeping track of nutritional indicators, such as body mass index (BMI)
Appropriate doses of pancreatic enzymes when needed
Stallings/pg833/col2/P2/ln2-5, P5/ln1-5, col2/P4/ln3-5, pg836/col1/P3/ln1-5, pg834/col2/P2/ln3-9
Borowitz/pg S76/Table I, #15 & pg S75/Table I, #3,5
Routine monitoring and care recommendations for the infant diagnosed with CF2
Borowitz/ PS78/ Table III
Key Takeaway:
The Cystic Fibrosis Foundation provides clinical guidelines for nutrition management
Because nutrition is one of the most essential components of effective treatment of individuals with CF, clinical guidelines for nutrition-related management for children and adults with CF are available
These guidelines come from the Cystic Fibrosis Foundation
The guidelines include the following recommendations1:
A high-calorie diet, including supplements when needed1
Behavioral intervention to encourage good eating habits in children1
Keeping track of nutritional indicators, such as body mass index (BMI)
Appropriate doses of pancreatic enzymes when needed1
In addition to the general nutrition guideline recommendations for children, there are also recommendations for infants under 2 years of age.2 The table shown provides an example of routine monitoring and care recommendations around care issues, including nutrition2
Discussion Questions: Are the routine monitoring and care recommendations shown here the same as what your CF care team recommends to patients? What, if anything, is different?
References:
1. Stallings VA, Stark LJ, Robinson KA, et al. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108: Borowitz D, Robinson KA, Rosenfeld M, et al. Cystic Fibrosis Foundation evidence-based guidelines for the management of infants with cystic fibrosis. J Pediatr. 2009;155:S73-S93.
Stallings/pg833/col2/P2/ln2-5, P5/ln1-5, col2/P4/ln3-5, pg836/col1/P3/ln1-5, pg834/col2/P2/ln3-9
* In some circumstances, care may be shared with PCP; infants growing poorly may need to be seen more often; some stable infants can be seen every 6 weeks.
C= consider doing at this visit; PERT = pancreatic enzyme replacement therapy; OFC = occipital frontal circumference; P&PD = percussion and postural drainage.
1. Stallings VA et al. J Am Diet Assoc. 2008;108: Adapted from Borowitz D et al. J Pediatr. 2009;155:S73-S93.
Borowitz/ PS78/Table III

7. Pancreatic Enzyme Supplementation Is Critical in CF
>90% of patients with CF have EPI, resulting in chronic nutrient malabsorption1
Symptoms of malabsorption include2:
Poor weight gain despite good appetite
Frequent, loose and/or large bowel movements
Mucus or oil in the bowel movements
Excessive gas and/or stomach pain
Distention or bloating
CF patients with EPI should take pancreatic enzyme replacement therapy (PERT) with all meals and snacks in order to properly absorb carbohydrate, protein, and fat1
Dosing guidelines for PERT are based on the North American CF Foundation consensus statement and the Consensus Conference on Enzyme Therapy and Fibrosing Colonopathy1
Rogers/pg13/col1P3/ln1-3
CFF/pg1/col3/P3
Rogers/pg16/col1/P2/ln1-4
Rogers/pg16/col1/P3/ln1-4
Rogers/pg16/col1/P3/ln8, col2/P1/ln1
Rogers/pg13/Table1
Borowitz/ PS75/ Table 1
Pancreatic Enzyme Dosage
General recommendations1
Units lipase/kg/meal
Start with 500 U lipase/kg/meal
Increase to max of 2500 U lipase/kg/meal
Provide ½ meal dose with snacks
Caution with doses >2500 units lipase/kg/meal
Units lipase/grams of fat
Start with 500 units lipase/gram fat
Increase to max of 4000 units lipase/gram fat
Caution with doses >4000 units lipase/gram fat
Recommendations for infants with CF under 2 years of age3
Start in all infants with:
2 CFTR mutations associated with PI
Fecal elastase <200 μg/g or CFA <85% (in infants <6 months of age), or other objective evidence of pancreatic insufficiency
Unequivocal signs or symptoms of malabsorption, while awaiting confirmatory test results
Initiate at: units lipase/kg at each feeding
Adjust to: ≤2500 units lipase/kg per feeding to maximum 10,000 units lipase/kg/day
Key Takeaway:
90% of CF patients require pancreatic enzyme replacement therapy in order to properly absorb and digest food
More than 90% of patients with CF have EPI, resulting in chronic nutrient malabsorption1
Symptoms of malabsorption include2:
Poor weight gain despite good appetite
Frequent, loose and/or large bowel movements
Mucus or oil in the bowel movements
Excessive gas and/or stomach pain
Distention or bloating
CF patients with EPI should take pancreatic enzyme replacement therapy (PERT) with all meals and snacks in order to properly absorb carbohydrate, protein, and fat1
Dosing guidelines for PERT are based on the North American CF Foundation consensus statement and the Consensus Conference on Enzyme Therapy and Fibrosing Colonopathy1
Most recommendations are based on units of lipase per kilogram of body weight.1 Please note that there are specific recommendations for infants with CF under the age of 2 years3
Discussion Question: What is the method your CF care team uses to ensure patients who require enzymes are prescribed the appropriate amounts? What kinds of challenges does your team face with regard to patients and enzyme replacement therapy?
References:
1. Cystic Fibrosis Foundation. Nutrition: pancreatic enzyme replacement in people with cystic fibrosis. Accessed March 18, Rogers CL. Nutritional management of the adult with cystic fibrosis—part I. Nutrition Issues in Gastroenterology, Series #113. Practical Gastroenterol. 2013; 37: Borowitz D, Robinson KA, Rosenfeld M, et al. Cystic Fibrosis Foundation evidence-based guidelines for the management of infants with cystic fibrosis. J Pediatr. 2009;155:S73-S93.
Rogers/pg13/col1P3/ln1-3
CFF/pg1/col3/P3
1. Rogers CL. Pract Gastroenterol. 2013;37: Cystic Fibrosis Foundation. Accessed March 18, Borowitz D et al. J Pediatr. 2009;155:S73-S93.
Rogers/pg16/col1/P2/ln1-4
Borowitz/ PS75/ Table 1
Rogers/pg16/col1/P3/ln1-4
Rogers/pg16/col1/P3/ln8, col2/P1/ln1

8. Vitamins and Minerals Are Essential in CF
Vitamins and minerals are necessary for normal growth, function, and health, but patients with CF need more for good health1
Improvements in CF nutrition management are one factor that is believed to lead to an increase in life expectancy2
Vitamin A
Numerous roles including normal vision, bone and tooth formation, cell function, immunity, to help maintain healthy intestines
Vitamin D
Involved in building and maintaining strong bones and teeth
Vitamin E
Antioxidant, helps keep red blood cells healthy, helps fight infection, and helps maintain healthy intestines
Vitamin K
Role in blood clotting and keeping bones healthy
Calcium
Essential element involved in building strong bones and teeth; maintains nerves and muscles
Iron
Essential element with a key role in carrying oxygen in blood cells
Sodium chloride
Important in maintaining fluid balance
Zinc
Many important functions in the body ranging from growth and healing to taste and appetite
CFF/pg1
Morton/pg431/col1/P2/ln7-10
Key Takeaway:
Improvements in nutritional management, including ensuring patients get the appropriate amounts of vitamins and minerals, are one factor that is believed to lead to an increase in life expectancy for people with CF2
Vitamins and minerals are necessary for normal growth, function, and health, but patients with CF need more for good health1
Here is a list of some of the vitamins and minerals particularly important for patients with CF and their role in the body’s functioning:
Vitamin A is necessary for normal vision, bone and tooth formation, cell function, immunity, and healthy intestines
Vitamin D is involved in building and maintaining strong bones and teeth
Vitamin E is an antioxidant, helping to keep red blood cells healthy, fight infection, and maintain healthy intestines
Vitamin K plays a role in blood clotting and in keeping bones healthy
Calcium is essential for building strong bones and teeth, and for maintaining nerves and muscles
Iron is key in carrying oxygen in blood cells
Sodium chloride is important in maintaining the body’s fluid balance
Zinc has many functions ranging from growth and healing to taste and appetite
References:
1. Cystic Fibrosis Foundation. The importance of vitamins and minerals in CF. Accessed March 18, Morton AM. Conference on ‘Malnutrition matters.’ Symposium 6: Young people, artificial nutrition and transitional care: The nutritional challenges of the young adult with cystic fibrosis: Transition. Proc Nutr Soc. 2009;68:
Morton/pg431/col1/P2/ln7-10
CFF/pg1
1. Cystic Fibrosis Foundation. Accessed March 18, Morton AM et al. Proc Nutr Soc. 2009;68:

9. Improvements in CF Care Have Increased Survival
Patients with CF are living longer than ever (figure left)
In 2011, the median predicted survival age was 36.8 years compared with <28 years in 1987
In addition, the number of adults is increasing (figure right)
In 2011, >48% of patients were adults compared with >32% in 1991
CFF/pg6/P2/ln1-3, pg6/Graph
CFF/pg8/P4/ln1-3, pg8/P5/ln1-3
Median Predicted Survival Age, in 5-Year Periods
Number of Children and Adults with CF, 40 15 12 36 9
Age (Years) 32
Number of People with CF (Thousands) 6 28
Key Takeaway:
Medical advancements and optimization of nutritional status have helped to increase the lifespan of patients with CF
With advances in CF care, CF patients are living longer than ever
Based on the Cystic Fibrosis Foundation’s Patient Registry Annual Data Report 2011, the median survival age has risen steadily over time, as shown in the graph on the left. In 2011, the age was 36.8 years, compared with less than 28 years of age in 1987
In addition, the number of adults with CF is increasing. The graph on the right shows how fast the number of adults with CF has grown in relation to the number of children with CF. The data show that today more people with CF are living well into adulthood. Twenty years ago, more than 32 percent of people in the Patient Registry were 18 or older. In 2011, more than 48% of people with CF were adults, and that number continues to grow
Reference:
Cystic Fibrosis Foundation. Patient Registry Annual Data Report Accessed March 18, 2013.
Children
Adults 3
CFF/pg6/P2/ln1-3, pg6/Graph 24 86 87 88 89 90 91 92 93 94 95 96 97 98 99 00 01 02 03 04 05 06 07 08 09 10 11
Cystic Fibrosis Foundation. Accessed March 18, 2013
CFF/pg8/P4/ln1-3, pg8/P5/ln1-3

10. Living Longer Poses New CF Care Challenges
With advances in treatment, CF patients are living longer and thus creating new challenges in care
New nutritional challenges associated with CF-related complications, such as diabetes and bone disease have emerged
Bone disease is becoming more common in patients with CF
The CF care team can play an important role in addressing CF-related life changes
CFF/pg22/Graph
Common Complications by Age, 2011 50 30 20 10
Percent of People with CF 40
<6
6-10
11-17
18-24
25-34
35-44
45+
Diabetes (CFRD)
Bone Disease
Depression
Arthritis Arthropathy
Age (Years)
Key Takeaway:
Living longer means the emergence of CF-related complications, such as diabetes or bone disease
The increasing average lifespan of the CF population poses new challenges in care
New nutritional issues associated with CF-related complications, such as diabetes and bone disease have emerged
This line graph shows how CF complications can increase with age. The green line at the top represents cystic fibrosis-related diabetes (CFRD), one of the most common complications. In 2011, 19% of all people in the CF Patient Registry had CFRD, and of those who were 18 and older, 33% had CFRD
Bone disease is another complication that’s becoming more common in patients with CF. The blue line represents the increase in bone disease as CF patients in the Registry got older. Data from the Patient Registry show that 11% of people with CF reported some type of bone disease, such as fracture or osteoporosis
Preventing bone disease begins in childhood when bones are growing. A diet with enough calories, vitamin D, calcium, and other vitamins and minerals, plus maintaining a healthy weight and exercise, can help
Discussion Question: In your experience, what do you think works well when helping families understand and learn how to live with CF?
Reference:
Cystic Fibrosis Foundation. Patient Registry Annual Data Report Accessed March 18, 2013.
CFF/pg22/Graph, pg22/P2/ln1-3
Cystic Fibrosis Foundation. Accessed March 18, 2013
CFF/pg22/P5/ln2-3
CFF/pg22/P6/ln1-3

11. Living Longer Also Affects Transition Management
CFF/Slide7-10
Because patients with CF are living longer and well into adulthood, there are unique issues that arise around the transition from pediatric to adult CF care
This period of time may be challenging for the care team, caregiver(s), and the patient
Ideal Transition to Adult CF Care
Parents/Family
Child
Responsibility
Key Takeaway:
Living longer also means unique issues for the CF patient, their families, and the CF care center with regard to transition to adult-centered care
Because people with CF are living longer and well into adulthood, there are unique issues that arise with the transition from pediatric to adult CF care—it can be a challenging time for care teams, parents, and the patient
This graph represents the ideal transition from pediatric to adult CF care; such that, as the child’s age increases, his or her responsibility increases, while simultaneously the family’s responsibility decreases.
[Note to Facilitator: The following notes are not shown on the slide]
Adolescence is a time of rapid psychological and physiological changes that pose unique challenges to the management of CF
Goals for year olds:
Know what causes CF and how the body is affected
Know the names of medications and when to take them and patient actually does take medications
Understand the purpose of each medication
Perform airway clearance without help (most of the time)
Understand choices about smoking and drinking and effect on health in CF
Goals for year olds
Ability to recognize symptoms and describe them
Know dates of clinic appointments and other tests and can schedule them
Aware of medication supply and participates in refilling prescriptions
Speaks directly to CF team to discuss changes in health
Learns to maintain equipment
Goals for 21 year olds
Contact CF healthcare providers directly to discuss changes in health
Schedule all appointments and tests
Refill prescriptions independently
Awareness of details about insurance and strategies for future coverage
Discussion Questions:
What do you think is important to communicate to patients and their families about transitioning and when do you do so? What are some important resources for families?
Reference:
Cystic Fibrosis Foundation. Partnering for care: transition to adult CF care. Accessed March 22, 2013.
Age 21
Cystic Fibrosis Foundation. Accessed March 18, 2013.

12. Transition Can Be a Complex Process for CF Care Centers
For CF care teams responsible for the transition from pediatric to adult CF care, the transition may be an ongoing process over a period of time
There are multiple components of care that must be addressed
Each care team member has a unique role to play
Towns/pg70/Table 1
Sample CF Self-Management Scale
Component of care
Level 1:
Always dependent
Level 2: Frequently dependent
Level 3: Sometimes dependent
Level 4: Fully independent
Physiotherapy and clearance
Caregiver manages care and routine
Individual initiates care
Minimal supervision or assistance
Independent
Nutrition/enzyme management
Some knowledge
Responsible for some aspects of nutrition
Independently manages
Medication usage
Maximal caregiver management
Moderate caregiver supervision
Independent or minimal supervision
Disease management
Knows general principles
Asks questions and knows genetic aspects
Knows when to seek help
Knows how to access help
General life skills
Managed by caregiver
Becoming more independent
Increasing sense of control
Independent and actively involved
Key Takeaway:
Transition is a long-term process that requires the coordinated efforts of the entire CF care team
The transition from pediatric to adult CF care is a multistep process. For CF care teams responsible for this transition, it may be an ongoing process that happens over a period of time
There are multiple components of care to be addressed, with each member of the care team having a specific role
The table shown here is a sample CF self-management scale, which categorizes levels of dependence from 1-4, and each level’s relationship to 5 different components of care1.
Level 1 is “always dependent”; Level 2 is “frequently dependent”; Level 3 is “sometimes dependent”; and Level 4 is “fully independent.” In general, as age increases, the level of independence increases, such that a 10-year-old might be a Level 1, and a 17-year-old a Level 41
Components of care perhaps requiring decreasing levels of assistance from caregivers as the child gets older can include: physiotherapy and exercise clearance technique, nutrition and enzyme management, medication management, disease management, and general life skills1
So for example, the progression from more dependent to more independent with regard to physiotherapy and exercise clearance can look like the following1:
At Level 1 (dependent), a caregiver may completely manage physiotherapy and clearance for the child with CF, progressing to the child initiating this component of care at Level 2, to minimal supervision at Level 3, and finally to independently managing this aspect of care at Level 41
Likewise for nutrition management, at Level 1 the child may have some knowledge about the importance of nutrition in CF; at Level 2 the child may begin taking responsibility for some nutritional aspects such as taking enzymes while at school and with minimal supervision; to Levels 3 and 4 where the child independently manages nutrition and enzymatic support on a daily basis1
And so on for taking medications, understanding and managing the disease as a whole, and general life skills1
Transition programs in CF centers should include evaluations by team members, including nurses, dietitians, social workers, respiratory therapists, physical therapists, and psychologists2
Discussion Questions: How does your CF care center assess patients for transition from pediatric to adult CF care? Do you have standardized transition programs in place? What do they consist of?
References:
1. Towns SJ, Bell SC. Transition of adolescents with cystic fibrosis from pediatric to adult care. Clin Respir J. 2011;5: Flume PA, Taylor LA, Anderson DL, Gray S, Turner D. Transition programs in cystic fibrosis centers: perceptions of team members. Ped Pulmonol. 2004;37:4-7.
Towns/pg70/Table 1
Towns SJ et al. Clin Resp J. 2011;5:64-75.
Flume/pg5/col2/P3/ln1-8

13. CF Care Team Perceptions May Not Align With Patients Regarding Transition
Challenges for patients within adult services1:
Independence in decision making, goal setting, and issues of confidentiality and privacy
Implications of CF as an adult
Establishing good communication and developing a good relationship
Introduction of new staff
Appropriately supporting caregivers
Adult centers with appropriate resources
Team member’s perceptions of patient concerns2:
Patient is not familiar with the adult staff
Concerned about adult staff being able to meet medical needs
Patient has strong relations with pediatric staff
Patient is reluctant to leave the pediatric care setting
Patient has difficulty with taking on responsibilities for self-care
Anderson
Flume/pg6/Table1
Key Takeaway:
CF care team members are in a vital position to clarify and help address the young adult patient’s emotional concerns about transition
Transition is also the time to revisit the changing physical needs of the young adult CF patient—particularly regarding nutrition management
Here we’ll discuss some of the concerns patients with CF have around the transition process. First we’ll talk about some of the challenges for patients, followed by the results of a survey given to care team members that revealed their perceptions of patient concerns
Challenges for patients transitioning to the adult services setting may include: independence in decision making, goal setting, and issues of confidentiality and privacy; what the implications of CF are as an adult; establishing good communication and developing a good relationship; introduction of new staff; appropriately supporting caregivers; and adult centers with appropriate resources1
According to a survey of 291 care team members, their perceptions of patients’ concerns regarding the transition to adult services included2:
Patient is not familiar with the adult staff
Patient is concerned about the adult staff being able to meet medical needs
Patient has strong relations with pediatric staff
Patient is reluctant to leave the pediatric care setting
Patient has difficulty with taking on responsibilities for self-care
Discussion Question: In your CF care center, what concerns do you think patients have regarding the transition process? Do you ask patients what they are?
References:
1. Anderson DL, Flume PA, Hardy KK, et al. Transition programs in cystic fibrosis centers: perceptions of patients. Pediatr Pulmonol. 2002;33:327– Flume PA, Taylor LA, Anderson DL, Gray S, Turner D. Transition programs in cystic fibrosis centers: perceptions of team members. Ped Pulmonol. 2004;37:4-7.
Anderson
1. Anderson DL et al. Pediatr Pulmonol. 2002;33:327– Flume PA et al. Ped Pulmonol. 2004;37:4-7.
Flume/pg6/Table1

14. Nutrition Management May Be Particularly Challenging During Transition Period
Nutrition and digestive health are essential components of care of the patient with CF1
Achieving nutrition and digestive health may be challenging around the transition period, which is typically during the teen years1
Dietitians at CF care centers can provide specially tailored dietary programs for each stage of life1
Morton/pg431/col2/P1/ln5-6
Morton/pg436/col1/P2/ln21-24, P5/ln3-5
Morton/pg433/col1/P4/ln6-9, pg434/col2/P1/ln4-6
Leonard/pg10/Figure1
100%
Optimal
Acceptable
Concerning
At Risk
Failure
90%
80%
70%
60%
50%
40%
Key Takeaway:
Nutritional status can vary with age; the teen years may be particularly challenging
Nutrition and digestive health are essential components of care for the patient with CF1
Achieving nutrition and digestive health may be challenging around the transition period, which is typically during the teen years1
Dietitians at CF care centers can provide specially tailored dietary programs for each stage of life1
This graph shows the distribution of nutrition classifications by age for CF patients at an initial clinic visit, according to a 2010 study. The nutrition classifications were as follows: optimal, acceptable, concerning, at risk, and failure. The Optimal distributions are shown by the light blue sections of each bar, versus the Failure distributions indicated by the orange areas of the bars2
In this study, a greater percentage of the youngest group of CF patients (2-5 years) had Optimal nutrition, versus a greater percentage of the oldest group of CF patients—the teenagers—fell into the Failure category of nutrition classification2
These results demonstrate that nutritional status varies with age, and that the teenage years are likely to represent a challenge with regard to optimal nutrition for the patient with CF
Involvement of the entire CF care team to develop a standardized approach to classification and treatment of patients with suboptimal nutrition can substantially improve nutrition in a CF center in a short period of time2
Discussion Question: What would you say to teenage patients who do not want to follow the recommendations for their nutrition requirements?
References:
1. Morton AM. Conference on ‘Malnutrition matters.’ Symposium 6: Young people, artificial nutrition and transitional care: The nutritional challenges of the young adult with cystic fibrosis: Transition. Proc Nutr Soc. 2009;68: Leonard A, Davis E, Rosenstein BJ, et al. Description of a standardized nutrition classification plan and its relation to nutritional outcomes in children with cystic fibrosis. J Ped Psychol. 2010;35:6-13.
30%
20%
10%
Morton/pg436/col1/P2/ln21-24, P5/ln3-5 0%
Morton/pg431/col2/P1/ln5-6
2-5 (n=45)
6-10 (n=61)
11-15 (n=75)
≥16 (n=65)
Age (years) 2
Morton/pg433/col1/P4/ln6-9, pg434/col2/P1/ln4-6
1. Morton AM et al. Proc Nutr Soc. 2009;68: Leonard A et al. J Ped Psychol. 2010;35:6-13.
Leonard/pg10/Figure1
Leonard/pg10/Figure1
Leonard/pg12/col1/P2/ln4-7, col2/P2/ln8-11

15. Social and Economic Considerations
Let’s discuss some of the social and economic issues in CF

16. The Cost of CF Care Is High
CF imposes a burden on everyday life, particularly around cost of care
Lifetime cost of treatment for a person with CF: $2.3 million1
Total annual medical care expenditures averaged $48,098 for people with CF, more than 22 times the average of $2,172 for people without CF.2
1 in 4 have delayed care or skipped treatments because of the cost3
Costs associated with nonadherence may add to economic burden4
Money wasted on unused medications or other therapies that are not followed
An increase in clinic appointments, emergency room visits, and hospitalizations because of nonadherence
Tur Kaspa/pg190/col2/P1/ln5-7, pg191/col1/P1/ln11
Ouyang/pg991/col2/P3/ln1-3
Ouyang/pg992/Table2
CFF/pg1/P3/ln1-4
Rapoff/pg35/P1/ln6-7
Rapoff/pg34/P5/ln1-4
Key Takeaway:
The cost of treating CF is high
Beyond the burden of having CF and the burden of treatment, CF imposes a burden on everyday life, particularly around cost of care
Medical care utilization and expenditures are substantially higher for patients with CF compared with those without CF1
Lifetime cost of treatment for a person with CF can exceed $2.3 million2
In 2006, total annual medical care expenditures averaged $48,098 for people with CF, more than 22 times the average of $2,172 for people without CF.1
This bar graph compares annual medical costs for people with CF (the blue bars), and for people without CF (the red bars), broken out by age range. As you would expect, health care costs are much higher for people who have CF than for those who do not1
A recent study showed that 1 in 4 CF patients have delayed care or skipped treatments because of cost3
Costs associated with nonadherence may add to economic burden4
For example, money wasted on unused medications or other therapies that are not followed
An increase in clinic appointments, emergency room visits, and hospitalizations due because of nonadherence
References:
1. Ouyang L, Grosse SD, Amendah DD, Schecter MS. Healthcare expenditures for privately insured people with cystic fibrosis. Ped Pulmonol. 2009;44: Tur-Kaspa I, Aljadeff G, Rechitsky S, et al. PGD for all cystic fibrosis carrier couples: novel strategy for preventive medicine and cost analysis. Reprod Biomed Online. 2010;21: Cystic Fibrosis Foundation. New study finds more with CF struggle to afford care. Accessed March 30, Rapoff MA. Adherence to Pediatric Medical Regimens. 2nd Edition. Springer Science+Business Media: New York, NY; 2010.
Ouyang/pg994/col1/P2/ln1-3
1. Tur-Kaspa et al. Reprod Biomed Online. 2010;21: Ouyang L et al. Ped Pulmonol. 2009;44: Cystic Fibrosis Foundation. Accessed March 30, Rapoff MA. Springer Science+Business Media: New York, NY; 2010.
Ouyang/pg991/col2/P3/ln1-3
Tur Kaspa/pg190/col2/P1/ln5-7, pg191/col1/P1/ln11
Ouyang/pg992/Table2
CFF/pg1/P3/ln1-4
Rapoff/pg35/P1/ln6-7
Rapoff/pg34/P5/ln1-4

17. People With CF Face Unique Employment Challenges
Improvements in care and life expectancy has led to a larger number of patients with CF seeking higher education, vocational training, and employment1
According to the 2011 CF Patient Registry, 45% of adult patients with CF were employed full time or part time2
Economic and health insurance considerations can impact employment decisions—particularly because US employers often offer health insurance1
But employment for some people with CF may be challenging1
Time required for daily treatment regimen, activity limitations due to disease, possibility of missed work days due to hospitalizations, concerns about disclosure to employers, risks of infection or pulmonary irritants in the work place
Additionally, there may be a lack of formal job counseling from the CF clinician1
Demars/pg922/col1/P1/ln9-11
CFF/pg9/Pie Chart
Demars/pg922/col2/P2/ln7-11
Demars/pg922/col1/P1/ln16-19, col2/P1/ln1-3
Demars/pg923/col1/P1/ln1-3
Key Takeaway:
Balancing CF care with employment may be challenging for some patients
Improvements in care and life expectancy has led to a larger number of patients with CF seeking higher education, vocational training, and employment1
According to the 2011 CF Patient Registry, 45% of adult patients with CF were employed full time or part time2
Economic and health insurance considerations can impact employment decisions for CF patients—particularly because US employers often offer health insurance1
But employment for some people with CF may be challenging1
Time required for daily treatment regimen (often 2 hours a day), activity limitations due to disease, possibility of missed work days due to hospitalizations, concerns about disclosure to employers, risks of infection or pulmonary irritants in the work place
Additionally, there may be a lack of formal job counseling provided by the CF clinician1
Discussion Question: How does your CF care center address the issue of job counseling for the young adult patient with CF?
References:
1. Demars N, Uluer A, Sawicki GS. Employment experiences among adolescents and young adults with cystic fibrosis. Disabil Rehab. 2011;33: Cystic Fibrosis Foundation. Patient Registry Annual Data Report Accessed March 18, 2013.
Demars/pg922/col1/P1/ln9-11
CFF/pg9/Pie Chart
1. Demars N et al. Disabil Rehab. 2011;33: Cystic Fibrosis Foundation. Accessed March 18, 2013.
Demars/pg922/col2/P2/ln7-11
Demars/pg922/col1/P1/ln16-19, col2/P1/ln1-3
Demars/pg923/col1/P1/ln1-3

18. Living Longer With CF Affects Health Insurance Coverage
Some patients with CF are unable to maintain private insurance because of their disease
The Cystic Fibrosis Patient Assistance Foundation (CFPAF) helps people with CF living in the United States get their prescribed FDA-approved drugs and devices, regardless of health insurance coverage or financial resources1
However, additional resources may be needed—health care professionals can play an important role in helping CF patients obtain the insurance coverage and other financial assistance they need2
CFF Report/pg24/P3/ln1-2
CFF Work/pg1/P2
CFF Report/pg25/table
Percentage of People with CF by Type of Health Insurance Coverage, 2011*
Type of Insurance
17 Years and Younger
18 Years and Older
Health Insurance Policy (e.g. Private Insurance)
55.8
65.0
Medicare/Indian Health Service
0.6
16.6
Medicaid/State Programs
52.6
33.6
TriCare or Other Military Health Plan
2.7
2.1
Other
2.2
No Health Insurance**
0.4
1.8
Key Takeaway: Two-thirds of CF patients are privately insured, leaving the other third to rely on government aid for health insurance
Some CF patients are unable to maintain private insurance because of their disease
The Cystic Fibrosis Patient Assistance Foundation (CFPAF) helps people with CF living in the United States get their prescribed FDA-approved drugs and devices, regardless of health insurance coverage or financial resources1
However, additional resources may be needed—health care professionals can play an important role in helping CF patients obtain the insurance coverage and other financial assistance they need2
This table shows the percentage of people with CF by type of health insurance coverage and by age group in 2011
According to the CF Patient Registry, the majority of health care insurance coverage was either private, or via Medicaid and other state programs. This was true for the age groups 17 and younger and 18 and older, though once patients turned 18, the percentage of people covered by Medicaid dropped significantly—leaving the majority (65%) to seek and obtain their own private health insurance policies1
Nearly half of adults with CF 18 through 25 years of age in the Patient Registry were covered by their parents’ health insurance—a significant percentage likely due to the health care reform law passed in 2010 which allows children to stay on their parents’ health insurance until the age of 261
Discussion Questions: How involved are the members of your CF care team in helping young adult patients or their parents with insurance issues (claims, appeals, prior authorizations, etc.)? What about with finding and navigating financial assistance programs to help pay for their care? What resources would you recommend?
References:
1. Cystic Fibrosis Foundation. Patient Registry Annual Data Report Accessed March 18, Cystic Fibrosis Foundation. Insurance & finances: work with your CF team. Accessed March 24, 2013.
* Data are not mutually exclusive, except the “no health insurance” category, as people with CF may have more than one type of insurance.
** The “no health insurance” category shows the percentage of people with CF who said they did not have any health insurance in 2011.
CFF Report/pg24/P3/ln1-2
1. Cystic Fibrosis Foundation. Accessed March 18, Cystic Fibrosis Foundation. Accessed March 24, 2013.
CFF Work/pg1/P2
CFF Report/pg25/table
CFF Report/pg24/P5/ln1-3

19. CF Care Team Is Critical in Ensuring Transition Success
The CF care team plays a pivotal role in transition solutions management
Transition solutions can include:
1. Implementing standard programs in the CF care center for transfer of young adults from pediatric to adult care settings, including preparing the transitioning patient to1,2:
Interact with doctors on their own (without parent)
Advocate for themselves
Discuss “adult” issues, including the management of medical bills (working/affording to pay), understanding insurance, and handling difficult information/”bad news”
2. Providing job support/counseling as part of self-management and health care transition programs3
3. Teaching patients how to save recipes, create a meal plan, build grocery lists, and cook to meet their nutritional needs
4. Helping patients navigate insurance obstacles via letters of medical necessity and prior authorization, insurance appeals, exceptions, and reconsiderations
Happy Hearts/pg1/P6-7, pg2/P1
Flume/pg5/col1/P1/ln9-11, pg7/col2/P2/ln13-14
Demars/pg926/col1/P2/ln4-8
Key Takeaway:
The entire CF care team can coordinate to offer standardized solutions that ensure successful patient transition
The CF care team is critical to ensuring successful transition—they play a pivotal role in providing transition solutions for their care center
Transition solutions can include:
1. Implementing standard programs in the CF care center for transfer of young adults from pediatric to adult care settings, including preparing the transitioning patient to1,2:
Interact with doctors on their own (without parent)
Advocate for themselves
Discuss adult issues, including the management of medical bills (working/affording to pay), understanding insurance, and handling difficult information/bad news
2. Providing job support/counseling as part of self-management and health care transition programs3
3. Teaching patients how to save recipes, create a meal plan, build grocery lists, and cook to meet their nutritional needs
4. Helping patients navigate insurance obstacles via letters of medical necessity and prior authorization, insurance appeals, exceptions, and reconsiderations
Discussion Questions: How well does your CF care team communicate to the rest of the multidisciplinary team? What would you do to improve multidisciplinary communication so that all of the needs of the transitioning young adult with CF (medical, psychological, financial) are met?
References:
1. Flume PA, Taylor LA, Anderson DL et al. Transition programs in cystic fibrosis centers: perceptions of team members. Ped Pulmonol. 2004;37:4–7. 2. Greene LC, Cline FW. Successful transitions: moving from childhood to adulthood. Accessed April 3, Demars N, Uluer A, Sawicki GS. Employment experiences among adolescents and young adults with cystic fibrosis. Disabil Rehab. 2011;33:
Happy Hearts/pg1/P6-7, pg2/P1
1. Flume PA et al. Ped Pulmonol. 2004;37:4–7. 2. Greene LC et al. Accessed April 3, Demars N et al. Disabil Rehab. 2011;33:
Flume/pg5/col1/P1/ln9-11, pg7/col2/P2/ln13-14
Demars/pg926/col1/P2/ln4-8

20. Resources
General:
The CFF Patient Assistance Resource Library provides CF care guidelines, evidence-based medical journal articles, white papers for medical professionals:
Tools and tips for CF parents:
Transition:
Transition checklists for 3 different age groups:
Self-care checklist:
Additional resources about transition:
Nutrition:
High calorie recipes:
Insurance:
The Cystic Fibrosis Patient Assistance Foundation (CFPAF) provides information on a number of programs available to help people with CF afford the care and medications they need: or call
The CFF Patient Assistance Resource Library also provides sample letters of medical necessity and prior authorization; how-to guides to navigating common insurance obstacles; templates for insurance appeals, exceptions and reconsiderations:
CF Legal Hotline:
Phone: (800)
Website:
Here are some useful online resources for your care team, patients, and their parents
General resources:
The CFF Patient Assistance Resource Library provides CF care guidelines, evidence-based medical journal articles, and white papers for medical professionals:
Tools and tips for CF parents:
Resources about transition:
Transition checklists for 3 different age groups: CheckLists/default.aspx
Self-care checklist: hecklist/default.aspx
Additional resources about transition:
Nutrition-related:
High calorie recipes:
Insurance-related:
The CFF Patient Assistance Resource Library also provides sample letters of medical necessity and prior authorization; how-to guides to navigating common insurance obstacles; templates for insurance appeals, exceptions and reconsiderations:

21. Case Studies
Now let’s discuss 3 case studies from the perspective of the CF care center

22. Case Study 1: Transition in Care
Patient background:
17 year-old Caucasian female
Height: 5’5”
Weight: 102 lb
BMI: 17
Current medical history
Cystic fibrosis diagnosed at birth
Baseline FEV1: 85% of the predicted value
Past medical history
Hospitalized for respiratory infection 2 years ago
Labs: WNL
Notes:
CF is usually well controlled
Is able to participate in a number of extracurricular activities, including dance and cross country
Over the past 6 months she has been taking on more responsibility for management of her treatments and has mentioned that she would like to be more independent. She will be going to college in a few months
First we’ll discuss a case where the patient is transitioning from pediatric to adult CF care
Patient background:
Female, 17 years old
5’5”, 102 lb
BMI = 17 kg/m2
Current medical history
Cystic fibrosis diagnosed at birth
Baseline FEV1=85% of the predicted value
Past medical history
Hospitalized for respiratory infection 2 years ago
Labs: within normal limits
Notes:
CF is usually well controlled
Is able to participate in a number of extracurricular activities, including dance and cross country
Over the past 6 months she has been taking on more responsibility for management of her treatments and has mentioned that she would like to be more independent. She will be going to college in a few months
Discussion Questions:
How would your CF care team communicate with the adult CF care center?
What are some areas of concern?
How would you best mobilize care team members, such as nurses, social workers, and dietitians, to help the adult CF care team during the patient transition process?
Questions
How would your CF care team communicate with the adult CF care center?
What are some areas of concern?
How would you best mobilize care team members, such as nurses, social workers, and dietitians, to help the adult CF care team during the patient transition process?

23. Case Study 2: A Hospitalized Patient
Patient background:
23 year-old Caucasian male
Height: 5’6”
Weight: 125 lb
BMI: 19
Current medical history
Diagnosed with cystic fibrosis at 18 months old
He presents to the hospital with a lung infection (Pseudomonas aeruginosa)
FEV1: 65% of the predicted value
Labs: HbA1c: 6, WBC: 15, all else WNL
Past medical history
Hospitalized 3 times in the last year
Social history: full-time employment with irregular hours
Notes:
Recently graduated from college and living independently
Has been nonadherent to treatment plan
Next we review the case of a hospitalized patient
Patient background:
Male, 23 years old
5’6”, 125 lbs
BMI = 19 kg/m2
Current medical history
Diagnosed with cystic fibrosis at 18 months old
He presents to the hospital with a lung infection (Pseudomonas aeruginosa)
FEV1: 65% of the predicted value
Labs: HbA1c: 6, WBC: 15, all else WNL
Past medical history
Hospitalized 3 times in the last year
Social history: full-time employment with irregular hours
Notes:
Recently graduated from college and living independently
Has been nonadherent to treatment plan
Case Study # 2 Discussion Questions
How would you communicate with the emergency department, given this patient was hospitalized 3 times in the last year?
How would you follow up with the patient?
What recommendations do you have for preventing a future hospitalization?
Questions
How would you communicate with the Emergency Department given he was hospitalized 3 times in the last year?
How would you follow up with the patient?
What recommendations do you have for preventing a future hospitalization?

24. Case Study 3: New Diagnosis of CF
Patient background:
24 day-old Caucasian female
Current medical history
NBS +
IRT +
Genetic test: CFTR mutation positive
Notes:
Parents married 6 years; previously unaware that they are carriers for CF
Both employed full time
No relatives living nearby
4 year-old sibling without CF
Labs
Sweat Chloride (mmol/L) 62
Fecal elastase (µg/g)
175
WBC
Wnl
HGB
HCT
The third case study is about a new diagnosis of CF.
Patient background:
24 day-old Caucasian female
Current medical history
NBS +
IRT +
Genetic test: CFTR mutation positive
Notes:
Parents married 6 years; previously unaware that they are carriers for CF
Both employed full time
No relatives living nearby
4 year-old sibling without CF
Case Study # 3 Discussion Questions
In your center, what role does each member of the care team play (nurse, social worker, or dietitian) for parents with a newborn who is newly diagnosed with CF?
How would you train the parents?
What resources would you direct them to?
Questions
In your center, what role does each member of the care team play (nurse, social worker, or dietitian) for parents with a newborn who is newly diagnosed with CF?
How would you train the parents?
What resources would you direct them to?

25. Now I would like to review all the features and benefits of the Live2Thrive™ program.
© 2013 Aptalis Pharma 5/13 ZP J 25

26. What’s It All About?
Live2Thrive™ is a comprehensive support program for patients with CF, their loved ones, and caregivers
One online community designed to help patients of all ages manage their condition
While some of you may already know about the Live2Thrive™ Program, you may not be aware of everything that this comprehensive program has to offer your patients with cystic fibrosis and their caregivers
The Live2Thrive™ Web site is one online community designed to help patients of all ages manage their condition

27. Engaging, Inspiring, and Informative
Goals of the program
Help educate patients and caregivers about living with CF—and the importance of good nutrition
Motivate and encourage members to keep up with their treatment
The goals of Live2Thrive™ are essentially to engage, inspire, and inform patients with CF and their caregivers
The program does this by helping to educate them about life with CF—and the important benefits of good nutrition
It also strives to motivate and encourage members to keep up with their treatment

28. Joining the Program Is Easy
Convenient enrollment online or by phone 
Hassle-free system—no additional paperwork or forms to fill out or mail in 
Toll-free number for program questions
Live2Thrive™ makes it as easy as possible for people to join the community
Patients can enroll online or by phone. It’s a hassle-free system, and there’s no additional paperwork or forms to fill out or mail in
If members have questions or need to contact us, they are invited to simply use the toll-free number

29. One Community, Many Benefits
Aptalis pancreatic enzyme product for eligible patients up to 24 months old
Savings Card to offset copays up to $50 or allows patients to pay no more than a $40 copay
Age-specific educational content for managing CF and supporting nutritional health
vitamins and supplements shipped automatically each month
Points-based reward redemption program
The benefits of Live2Thrive™ are substantial
First, it provides free eligible pancreatic enzymes for patients up to 24 months old. Members also receive a savings card to offset copays up to $50 or allows patient to pay no more than a $40 copay, as well as free vitamins and supplements, which are shipped automatically each month
And one of the most popular features of the program is its points-based reward redemption program. Members can earn points and then trade them in for health- and fitness-related items
PLEASE NOTE: This program is not available to patients 2 years of age or older who are on Medicare, Medicaid, or participating in any other state or federally subsidized pharmacy benefit program, or where otherwise prohibited by law
Program is not available to patients 2 years of age or older who are on Medicare, Medicaid, or participating in any other state or federally subsidized pharmacy benefit program or where otherwise prohibited by law.

30. Helping Patients Save Money
As of March 2013, Aptalis has saved patients an estimated $1.1 million*!
As you just heard, one of the many benefits of Live2Thrive™ is the Loyalty Card. Patients can use this card to offset eligible prescription copays of up to $50
To date, Aptalis has saved patients an estimated 1.1 million dollars!
$550,000 accounted for by patient savings on cost of Aptalis supplements and vitamins;
Source: Live2Thrive Fulfillment Patient Savings Report_Dec 2011 thru Mar xlsx
*In the form of copay assistance and savings on the cost of supplements and vitamins.

31. Providing Patients With Free Vitamins and Supplements
Choosing or changing vitamins and supplements is easy—online or through a toll-free number
AquADEKS™* A highly absorbable, antioxidant-rich nutritional supplement containing vitamins A, D, E, and K**
SCANDISHAKE® A calorie-rich shake mix which can be mixed into many foods and beverages, including whole milk, juices, sauces, and desserts
SCANDICAL® Calorie-rich powder that comes in a convenient and portable 8-ounce shaker-top can
BOOST® VHC® A calorically dense, nutritionally complete beverage kcal/mL3
Automatic monthly shipments
While good nutrition is important for everyone, it’s especially important for patients with CF. In addition to helping educate patients about the importance of good nutrition, Live2Thrive™ provides FREE vitamins and supplements to eligible patients
Choosing which vitamins and supplements are most appropriate for a particular patient is easy. You can do it online or through a toll-free number. Vitamins and supplements are automatically shipped to patients each month
According to Keller and Layer,1 EPI may be associated with malabsorption of the lipid soluble vitamins A, D, E, and K
Both of the vitamin options available through the CF Patient Support Program are designed to meet the needs of patients who have trouble absorbing fat soluble vitamins and nutrients.
AquADEKS is a highly absorbable, antioxidant-rich nutritional supplement containing vitamins A, D, E and K. Please note that this statement has not been evaluated by the FDA. This product is not intended to diagnose, treat, cure, or prevent any disease.
The supplement choices available through the program include:
SCANDISHAKE, a calorie-rich shake mix that can be mixed into many foods and beverages, including whole milk, juices, sauces, and desserts. Each SCANDISHAKE contains approximately 600 calories
SCANDICAL, a calorie-rich powder that comes in a convenient and portable 8-ounce shaker-top can. SCANDICAL mixes easily with almost any food or beverage, hot or cold, right at the table. Each tablespoon of SCANDICAL contains 35 calories
BOOST VHC, a calorically dense, nutritionally complete beverage—2.25 kcal/mL. Boost VHC contains 560 calories per serving to help meet daily nutritional needs
*Available in chewable tablets, pediatric drops, and softgels.
**This statement has not been evaluated by the FDA. This product is not intended to diagnose, treat, cure, or prevent any disease.
Reference:
Keller J, Layer P. Human pancreatic exocrine response to nutrients in health and disease. Gut. 2005;54(Suppl VI):Vi1-Vi28.

32. Offering Diverse Educational and Interactive Content
General content about living with CF
Designed to help patients and caregivers better understand the disease
Provides tips for managing the condition
Age-specific interactive content such as quizzes and videos on CF and nutrition for
Tykes and Tweens (newborn - 12 years)
Teens ( years)
Adults (18 and older)
The Live2Thrive™ Web site is a dynamic environment that provides diverse educational and interactive content
There’s a large section that offers general content about living with CF. It is designed to help patients and caregivers better understand the disease and provides tips for managing the condition
A particularly innovative feature of the site is that it provides age-specific interactive content on CF and nutrition. Formats include straight text, videos, and quizzes. In this way, important content is delivered in the most appropriate and appealing way for each age group. The age segments are
Tykes and Tweens (newborn - 12 years)
Teens ( years)
Adults (18 and older)

33. One of the pages most viewed by patients and caregivers!
Knowledge Is Power
“Understanding CF”: General educational overview
What Is CF?
What to Expect With CF and EPI
Tips for Managing CF
Lung care
Nutrition
Enzymes
The more patients and caregivers know about CF, the better they are able to cope with the condition. So let’s take a look at some of the topics covered in the Understanding CF educational overview
What Is CF? is a topline look at the condition, including the prevalence and causes of CF
What to Expect With CF and EPI looks at how CF affects the body and the kind of symptoms patients may experience
Tips for Managing CF covers key areas that patients and caregivers should be aware of as they manage the condition. Topics include
Lung care
Nutrition
Enzymes
And it’s interesting to note—this content is some of the most frequently viewed by patients and their caregivers
One of the pages most viewed by patients and caregivers!

34. Content Designed With the User in Mind
Age Specific Interactive Content
Tykes and Tweens (newborns to 12 years)
Teens (13 to 17 years)
Adults
(18 and older)
Life with CF video 
Nutrition and CF video
Nutrition quiz
Chef cooking video
Support groups and the CF healthcare team video
Transitioning to an adult CF healthcare team video
CF healthcare team quiz
As you can see in this table, the interactive content on the Web site is specifically tailored to each age group
We’ve found that the content for tykes and tweens and for adults has been of particular interest to viewers
The content tailored to tykes and tweens and to adults has been of particular interest to viewers.

35. Nutrition Information: What Teens Need to Know
Facts About Nutrition
Nutrition
Nutrition information presented in teen-friendly language
Videos capturing patient and parent discussions on nutrition and CF
Quiz to reinforce learning and earn rewards points
Food for Thought
Smart food choices for ages 13 to 17
Pancreatic Enzymes
Video of parents discussing their children’s CF treatments
Video to see how teens manage pancreatic enzymes and treatment
Tips for taking enzymes
Tasty Treats
Downloadable Scandishake’s “Recipes for Maintaining Weight” recipe book
Video of celebrity chef Fabio Viviani preparing breakfast pizza
Downloadable recipe for breakfast pizza
Let’s take a look at a specific segment—teens—and how information about nutrition has been designed to be relevant to them
The Facts About Nutrition section is broken up into 3 manageable parts:
Nutrition, which has easy-to-understand information teens can read and watch. The accompanying quiz also helps reinforce the learning. Plus it’s fun, and teens earn points by completing it
Food for Thought—offers smart food choices for teens aged 13 to 17 and discusses the importance of a balanced diet
Enzymes- offers tips for taking enzymes, as well as videos of parents discussing their children’s CF treatments, and videos for teens to show how other teens manage pancreatic enzymes and treatment
Tasty Treats—empowers teens to take nutrition into their own hands with simple high-calorie recipes that they can download and try

36. Making Learning Particularly Rewarding
Members earn points through:
Taking educational quizzes
Watching supportive and encouraging videos
Filling eligible pancreatic enzyme prescriptions
Members trade in points for health- and fitness-related items:
Omron HJ-112 Digital Pocket Pedometer
VORTEXTM Holding Chamber
Bubbles the FishTM II Pediatric Aerosol Mask
PARI Nebulizers
OMRON HR-100C Heart Rate Monitor
As mentioned briefly, Live2Thrive™ takes learning a step further by rewarding members for their efforts. They can earn points by taking educational quizzes, watching supportive and encouraging videos, and by filling eligible pancreatic enzyme prescription
When members acquire enough points, they can trade them in for health- and fitness-related items, such as:
Omron HJ-112 Digital Pocket Pedometer
VORTEXTM Holding Chamber
Bubbles the FishTM II Pediatric Aerosol Mask
PARI Nebulizers
OMRON HR-100C Heart Rate Monitor

37. A Brief Summary… Prescription copay savings
Live2Thrive™ - a comprehensive program for patients with CF, their loved ones, and caregivers
One online community designed to help patients of all ages manage their condition
Benefits include
Prescription copay savings
Aptalis pancreatic enzyme product for eligible patients up to 24 months old
vitamins and supplements
Age-specific, interactive educational content for managing CF and supporting nutritional health
In summary, Live2Thrive™ is a comprehensive program for patients with CF, their loved ones, and caregivers
A couple of the program benefits are—Loyalty Card that offsets prescription copays of up to $50 (which has already saved patients an estimated $600,000!), free Aptalis pancreatic enzyme product for eligible patients up to 24 months old, free vitamins and supplements, and educational materials for specific age groups

38. Summary
The gastrointestinal tract is often affected in CF, leading to deficits in growth and poor nutritional status
90% of people with CF have EPI, causing malabsorption and maldigestion
Optimizing nutritional status in CF patients is critical to extending life expectancy
Clinical guidelines for nutrition management in CF recommend a high-calorie diet along with appropriate doses of enzymes with every meal
Each member of the multidisciplinary CF care team plays an important role in optimizing patients’ nutritional status
CF care advancements have led to an aging CF population, which poses new challenges in care—particularly around CF-related complications and transition of care – from teen to adult
Transition is a multistep, multidisciplinary, and ongoing process
The CF care team can provide effective transition solutions that address the patient’s physical, emotional, and even financial concerns
The gastrointestinal tract is often affected in CF, leading to deficits in growth and poor nutritional status
90% of people with CF have EPI, causing malabsorption and maldigestion
Optimizing nutritional status in CF patients is critical to extending life expectancy
Clinical guidelines for nutrition management in CF recommend a high-calorie diet along with appropriate doses of enzymes with every meal
Each member of the multidisciplinary CF care team plays an important role in optimizing patients’ nutritional status
CF care advancements have led to an aging CF population, which poses new challenges in care—particularly around CF-related complications and transition of care from teen to adult
Transition is a multistep, multidisciplinary, and ongoing process
The CF care team can provide effective transition solutions that address the patient’s physical, emotional, and even financial concerns