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© 2013 Aptalis Pharma7/13ZP182-0113J-1 Nutrition and Digestive Health in Cystic Fibrosis 11 © 2013 Aptalis Pharma5/13ZP182-0113J
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 2
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Digestive Health Management in CF The gastrointestinal tract is often affected in CF: 4 Rogers CL. Practical Gastroenterol. 2013(January):10-24. Thick mucus secretion and inflammation Progressive destruction of the pancreas Insufficient amount of digestive enzymes Diminished absorptive function in the small intestine Poor digestion and absorption of food Growth retardation and poor nutritional status
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Adequate Nutrition in CF Is Fundamental Higher body weight has been shown to correlate with good lung function in CF patients 1 Many people with CF cannot digest/absorb nutrients and fat from food due to pancreatic insufficiency 2 CF patients require 110% to 200% more calories than people who do not have CF 3 A balanced, high-calorie diet with salt, fat, and protein is crucial for people with CF 2 Good nutrition promotes a strong immune system to fight infections; fewer infections can minimize lung damage and extend life 2 The CF care team dietitian plays a critical role 3 5 1. Rogers CL. Practical Gastroenterol. 2013;37:10-24. 2. Cystic Fibrosis Foundation. http://www.cff.org/UploadedFiles/treatments/Therapies/Nutrition/ForTeen/Nutrition-For- Teens%2Epdf. Accessed March 30, 2013. 3. Stallings VA et al. J Am Diet Assoc. 2008;108:832-839.
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Clinical Guidelines for Nutrition Management in Patients With CF Recommendations include 1 : –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 6 *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;108:832-839. 2. Adapted from Borowitz D et al. J Pediatr. 2009;155:S73-S93. Routine monitoring and care recommendations for the infant diagnosed with CF 2
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Pancreatic Enzyme Supplementation Is Critical in CF >90% of patients with CF have EPI, resulting in chronic nutrient malabsorption 1 Symptoms of malabsorption include 2: –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 fat 1 Dosing guidelines for PERT are based on the North American CF Foundation consensus statement and the Consensus Conference on Enzyme Therapy and Fibrosing Colonopathy 1 7 Pancreatic Enzyme Dosage 1. Rogers CL. Pract Gastroenterol. 2013;37:10-24. 2. Cystic Fibrosis Foundation. http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Diet/EnzymeReplacement/Nutrition- Pancreatic-Enzyme-Replacement.pdf. Accessed March 18, 2013. 3. Borowitz D et al. J Pediatr. 2009;155:S73-S93. General recommendations 1 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 age 3 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: 2000-5000 units lipase/kg at each feeding Adjust to: 2500 units lipase/kg per feeding to maximum 10,000 units lipase/kg/day
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 health 1 Improvements in CF nutrition management are one factor that is believed to lead to an increase in life expectancy 2 8 Vitamin ANumerous roles including normal vision, bone and tooth formation, cell function, immunity, to help maintain healthy intestines Vitamin DInvolved in building and maintaining strong bones and teeth Vitamin EAntioxidant, helps keep red blood cells healthy, helps fight infection, and helps maintain healthy intestines Vitamin KRole in blood clotting and keeping bones healthy CalciumEssential element involved in building strong bones and teeth; maintains nerves and muscles IronEssential element with a key role in carrying oxygen in blood cells Sodium chlorideImportant in maintaining fluid balance ZincMany important functions in the body ranging from growth and healing to taste and appetite 1. Cystic Fibrosis Foundation. http://www.cff.org/LivingWithCF/StayingHealthy/Diet/Vitamins/#. Accessed March 18, 2013. 2. Morton AM et al. Proc Nutr Soc. 2009;68:430-440.
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 9 Cystic Fibrosis Foundation. http://www.cff.org/UploadedFiles/research/ClinicalResearch/2011-Patient-Registry.pdf. Accessed March 18, 2013 Median Predicted Survival Age, 1987-2011 in 5-Year Periods 40 36 32 28 24 1987-19911992-19961997-20012002-20062007-2011 Age (Years) 868788899091 15 12 9293949596979899000102030405060708091011 9 6 3 0 Number of Children and Adults with CF, 1986-2011 Number of People with CF (Thousands) Children Adults
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 10 Cystic Fibrosis Foundation. http://www.cff.org/UploadedFiles/research/ClinicalResearch/2011-Patient-Registry.pdf. Accessed March 18, 2013 Common Complications by Age, 2011 50 30 20 10 0 Percent of People with CF 40 <66-1011-1718-2425-3435-4445+ Diabetes (CFRD) Bone DiseaseDepressionArthritis Arthropathy Age (Years)
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Living Longer Also Affects Transition Management 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 11 Cystic Fibrosis Foundation. http://www.cff.org/UploadedFiles/LivingWithCF/Webcasts/ArchivedWebcasts/Partnering-for-Care-Part-3-Slides-Adult-Transition.pdf. Accessed March 18, 2013. Child Age Parents/Family Ideal Transition to Adult CF Care Responsibility 21
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 12 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 careMinimal supervision or assistance Independent Nutrition/enzyme management Some knowledgeResponsible for some aspects of nutrition Independently manages Medication usageMaximal caregiver management Moderate caregiver supervision Independent or minimal supervision Independent Disease management Knows general principles Asks questions and knows genetic aspects Knows when to seek help Knows how to access help General life skillsManaged by caregiver Becoming more independent Increasing sense of control Independent and actively involved Sample CF Self-Management Scale Towns SJ et al. Clin Resp J. 2011;5:64-75.
© 2013 Aptalis Pharma7/13ZP182-0113J-1 CF Care Team Perceptions May Not Align With Patients Regarding Transition Challenges for patients within adult services 1 : –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 members perceptions of patient concerns 2 : –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 13 1. Anderson DL et al. Pediatr Pulmonol. 2002;33:327–331. 2. Flume PA et al. Ped Pulmonol. 2004;37:4-7.
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Nutrition Management May Be Particularly Challenging During Transition Period Nutrition and digestive health are essential components of care of the patient with CF 1 Achieving nutrition and digestive health may be challenging around the transition period, which is typically during the teen years 1 Dietitians at CF care centers can provide specially tailored dietary programs for each stage of life 1 14 1. Morton AM et al. Proc Nutr Soc. 2009;68:430-440. 2. Leonard A et al. J Ped Psychol. 2010;35:6-13. 100% 90% 80% 70% 60% 50% 40% 30% 20% 10% 0% 2-5 (n=45)6-10 (n=61)11-15 (n=75)16 (n=65) Age (years) 2 Optimal Acceptable Concerning At Risk Failure
© 2013 Aptalis Pharma7/13ZP182-0113J-1 15 Social and Economic Considerations
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 million 1 –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 cost 3 Costs associated with nonadherence may add to economic burden 4 –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 16 1. Tur-Kaspa et al. Reprod Biomed Online. 2010;21:186-95. 2. Ouyang L et al. Ped Pulmonol. 2009;44:989-996. 3. Cystic Fibrosis Foundation. http://www.cff.org/Mobile/aboutCFFoundation/NewsEvents/9-21-Study-Finds-More-with-CF-Struggle-Afford-Care.cfm. Accessed March 30, 2012. 4. Rapoff MA. Springer Science+Business Media: New York, NY; 2010.
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 employment 1 –According to the 2011 CF Patient Registry, 45% of adult patients with CF were employed full time or part time 2 Economic and health insurance considerations can impact employment decisionsparticularly because US employers often offer health insurance 1 But employment for some people with CF may be challenging 1 –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 clinician 1 17 1. Demars N et al. Disabil Rehab. 2011;33:922-926. 2. Cystic Fibrosis Foundation. http://www.cff.org/UploadedFiles/research/ClinicalResearch/2011-Patient-Registry.pdf. Accessed March 18, 2013.
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 resources 1 However, additional resources may be neededhealth care professionals can play an important role in helping CF patients obtain the insurance coverage and other financial assistance they need 2 18 1. Cystic Fibrosis Foundation. http://www.cff.org/UploadedFiles/research/ClinicalResearch/2011-Patient-Registry.pdf. Accessed March 18, 2013. 2. Cystic Fibrosis Foundation. http://www.cff.org/Adults/Insurance/Basics/Work/. Accessed March 24, 2013. Percentage of People with CF by Type of Health Insurance Coverage, 2011* Type of Insurance17 Years and Younger18 Years and Older Health Insurance Policy (e.g. Private Insurance)55.865.0 Medicare/Indian Health Service0.616.6 Medicaid/State Programs52.633.6 TriCare or Other Military Health Plan2.72.1 Other2.22.7 No Health Insurance**0.41.8 *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.
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 to 1,2 : –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 programs 3 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 19 1. Flume PA et al. Ped Pulmonol. 2004;37:4–7. 2. Greene LC et al. http://www.happyheartfamilies.com/articles/article/7883882/154132.htm. Accessed April 3, 2013 3. Demars N et al. Disabil Rehab. 2011;33:922-926.
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Resources General: The CFF Patient Assistance Resource Library provides CF care guidelines, evidence-based medical journal articles, white papers for medical professionals: http://www.cff.org/LivingWithCF/AssistanceResources/Library/ Tools and tips for CF parents: http://www.happyheartfamilies.com/TipsForCFParents.html Transition: Transition checklists for 3 different age groups: http://www.harthosp.org/CysticFibrosis/PatientEducation/Transition/TransitionCheckLists/default.aspx http://www.harthosp.org/CysticFibrosis/PatientEducation/Transition/TransitionCheckLists/default.aspx Self-care checklist: http://www.harthosp.org/CysticFibrosis/PatientEducation/Transition/SelfCareChecklist/default.aspxhttp://www.harthosp.org/CysticFibrosis/PatientEducation/Transition/SelfCareChecklist/default.aspx Additional resources about transition: http://www.happyheartfamilies.com/articles/article/7883882/150604.htmhttp://www.happyheartfamilies.com/articles/article/7883882/150604.htm Nutrition: High calorie recipes: http://www.cysticfibrosisidaho.org/d/content/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: http://www.cfpaf.org or call 1-888-315-4154www.cfpaf.org 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: http://www.cff.org/LivingWithCF/AssistanceResources/Library/ CF Legal Hotline: Phone: (800) 622-0385 Website: http://www.cff.org/livingwithcf/assistanceresources/cflegalhotline/http://www.cff.org/livingwithcf/assistanceresources/cflegalhotline/ Email: Cflegal@cff.org 20
© 2013 Aptalis Pharma7/13ZP182-0113J-1 21 Case Studies
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Case Study 1: Transition in Care 22 Patient background: 17 year-old Caucasian female Height: 55 Weight: 102 lb BMI: 17 Current medical history –Cystic fibrosis diagnosed at birth –Baseline FEV 1 : 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 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?
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Case Study 2: A Hospitalized Patient 23 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? Patient background: 23 year-old Caucasian male Height: 56 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) –FEV 1 : 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
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Case Study 3: New Diagnosis of CF 24 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? 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 WBCWnl HGBWnl HCTWnl
© 2013 Aptalis Pharma7/13ZP182-0113J-1 25 © 2013 Aptalis Pharma5/13ZP182-0113J
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Whats 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
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Engaging, Inspiring, and Informative Goals of the program Help educate patients and caregivers about living with CFand the importance of good nutrition Motivate and encourage members to keep up with their treatment
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Joining the Program Is Easy 28 Convenient enrollment online or by phone Hassle-free systemno additional paperwork or forms to fill out or mail in Toll-free number for program questions
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 29 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.
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Helping Patients Save Money As of March 2013, Aptalis has saved patients an estimated $1.1 million*! 30 *In the form of copay assistance and savings on the cost of supplements and vitamins.
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Providing Patients With Free Vitamins and Supplements Choosing or changing vitamins and supplements is easyonline 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- 2.25 kcal/mL 3 Automatic monthly shipments 31 *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.
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 (13 - 17 years) –Adults (18 and older) 32
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 33 One of the pages most viewed by patients and caregivers!
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Content Designed With the User in Mind Age Specific Interactive Content 34 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 The content tailored to tykes and tweens and to adults has been of particular interest to viewers.
© 2013 Aptalis Pharma7/13ZP182-0113J-1 Tasty Treats –Downloadable Scandishakes Recipes for Maintaining Weight recipe book –Video of celebrity chef Fabio Viviani preparing breakfast pizza –Downloadable recipe for breakfast pizza 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 childrens CF treatments –Video to see how teens manage pancreatic enzymes and treatment –Tips for taking enzymes 35
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 –VORTEX TM Holding Chamber –Bubbles the Fish TM II Pediatric Aerosol Mask –PARI Nebulizers –OMRON HR-100C Heart Rate Monitor 36
© 2013 Aptalis Pharma7/13ZP182-0113J-1 A Brief Summary… 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 38
© 2013 Aptalis Pharma7/13ZP182-0113J-1 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 careparticularly 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 patients physical, emotional, and even financial concerns
© 2013 Aptalis Pharma7/13ZP182-0113J-1 39
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