Simmer Gently for 40 Years: Evolving Impact of RD Research, Education, and Practice on CF Care

1. �Simmer Gently for 40 Years: Evolving Impact of RD Research, Education, and Practice on CF Care� Nancy H. Wooldridge, MS, RD Co-Director and Nutrition Faculty, Pediatric Pulmonary Center Pediatric Nutrition Coordinator, CF Center University of Alabama at Birmingham nwooldridge@peds.uab.edu In mid-Dec., I received an e-mail from Dawna Mughal from Erie. Dawna and I had served on ADA�s Nominating Committee together a few years ago. After some pleasantries, Dawna wrote this in her e-mail: �My purpose in contacting you is to first check if you are available to speak (on pediatric nutrition) at our state association�s annual conference in Pittsburgh�..If you are available, I will provide details and we will go from there�.� My thought processes: I have worked in pediatric nutrition for over 25 years. I have collaborated with Dr. Bonnie Spear on her annual Intensive Course in Pediatric Nutrition since the early 1990s. Yes, I can give a talk on pediatric nutrition. I responded affirmatively a few days later. Dawna�s reply: �We are delighted to know that you are available to present (what I did not tell you in my first e-mail) a special lecture. The lecture is in honor of Anna dPlanter Bowes, an outstanding professional who left a lasting legacy in the field of children�s nutrition and nutrition education.� My anxiety level went up at this point! I responded that I would be most comfortable talking about nutrition management in the care of patients with cystic fibrosis, since most of my clinical expertise is in this area. I communicated this with Dawna. Then I started thinking about all of the RDs in Pennsylvania who are national leaders in this field and my anxiety level increased even more. There were any number of RDs who could have given a talk on CF nutrition. Then I thought about how you can�t be a prophet in your own land or my colleague, Bonnie Spear�s definition of an expert: someone who is from out of town and who brings slides! I thought that this must be more than a talk on the nutrition management of CF. In mid-Dec., I received an e-mail from Dawna Mughal from Erie. Dawna and I had served on ADA�s Nominating Committee together a few years ago. After some pleasantries, Dawna wrote this in her e-mail: �My purpose in contacting you is to first check if you are available to speak (on pediatric nutrition) at our state association�s annual conference in Pittsburgh�..If you are available, I will provide details and we will go from there�.� My thought processes: I have worked in pediatric nutrition for over 25 years. I have collaborated with Dr. Bonnie Spear on her annual Intensive Course in Pediatric Nutrition since the early 1990s. Yes, I can give a talk on pediatric nutrition. I responded affirmatively a few days later. Dawna�s reply: �We are delighted to know that you are available to present (what I did not tell you in my first e-mail) a special lecture. The lecture is in honor of Anna dPlanter Bowes, an outstanding professional who left a lasting legacy in the field of children�s nutrition and nutrition education.� My anxiety level went up at this point! I responded that I would be most comfortable talking about nutrition management in the care of patients with cystic fibrosis, since most of my clinical expertise is in this area. I communicated this with Dawna. Then I started thinking about all of the RDs in Pennsylvania who are national leaders in this field and my anxiety level increased even more. There were any number of RDs who could have given a talk on CF nutrition. Then I thought about how you can�t be a prophet in your own land or my colleague, Bonnie Spear�s definition of an expert: someone who is from out of town and who brings slides! I thought that this must be more than a talk on the nutrition management of CF.

2. During this lecture, I plan to give an overview of Cystic Fibrosis, the correlation between nutritional status and patient outcomes, disease management including nutrition management. But during the last part of the lecture, the presentation will focus on the work and accomplishments of registered dietitians in the field of CF nutrition over many years. I will spotlight several nationally-recognized RDs from Pennsylvania. They exemplify the level of profesionalism that Anna dePlanter Bowes espoused. During this lecture, I plan to give an overview of Cystic Fibrosis, the correlation between nutritional status and patient outcomes, disease management including nutrition management. But during the last part of the lecture, the presentation will focus on the work and accomplishments of registered dietitians in the field of CF nutrition over many years. I will spotlight several nationally-recognized RDs from Pennsylvania. They exemplify the level of profesionalism that Anna dePlanter Bowes espoused.

3. Learning Objectives Upon completion of this educational activity, participants will be able to: list 3 components of the nutrition management of cystic fibrosis. explain the significance of optimal nutritional status in CF care. give 3 examples of successful strategies employed in by CF dietitians in research, education, and clinical practice. Education of professionals as well as patient/family education.Education of professionals as well as patient/family education.

4. Cystic Fibrosis is a genetic disease in which there is widespread dysfunction of the exocrine glands or the outward secreting glands of the body. Many organ systems are affected. The disease has been recognized for a long time. In fact, there is an old German saying that translates �The child who�s brow tastes salty when kissed will soon die�. The disease was first described as a distinct clinical entity by Dr. Andersen in 1941. Early investigators called the disease cystic fibrosis of the pancreas because they recognized that the pancreas was affected. In these days, most patients with cystic fibrosis died in infancy of malnutrition. Cystic Fibrosis is a genetic disease in which there is widespread dysfunction of the exocrine glands or the outward secreting glands of the body. Many organ systems are affected. The disease has been recognized for a long time. In fact, there is an old German saying that translates �The child who�s brow tastes salty when kissed will soon die�. The disease was first described as a distinct clinical entity by Dr. Andersen in 1941. Early investigators called the disease cystic fibrosis of the pancreas because they recognized that the pancreas was affected. In these days, most patients with cystic fibrosis died in infancy of malnutrition.

5. Discovery of CF Gene September 8, 1989 The CF gene was discovered in 1989 by a team of scientists, including Dr. Francis Collins. The CF gene is on the long arm of chromosome # seven. The little boy pictured on the front of Science magazine in Sept. 1989 is Danny Bessette, who has cystic fibrosis. September 8, 1989 The CF gene was discovered in 1989 by a team of scientists, including Dr. Francis Collins. The CF gene is on the long arm of chromosome # seven. The little boy pictured on the front of Science magazine in Sept. 1989 is Danny Bessette, who has cystic fibrosis.

6. The CF gene codes for CFTR, which is a cell membrane protein that regulates sodium and chloride transport.The CF gene codes for CFTR, which is a cell membrane protein that regulates sodium and chloride transport.

7. CF Gene Mutation Malfunction of the chloride channels in cells Increased sodium reabsorption Decreased chloride secretion Dehydrates mucus Increased viscosity of secretions Losses of sodium and chloride in the sweat This is the basic biochemical defect. This is the basic biochemical defect.

8. ?F508 Mutations Since the discovery of the CF gene in 1989, more than 1500 different CF gene mutations have been identified. Because of this, there is significant variability in the disease among patients. The most common mutation is ?F508. Since the discovery of the CF gene in 1989, more than 1500 different CF gene mutations have been identified. Because of this, there is significant variability in the disease among patients. The most common mutation is ?F508. About 70% of patients with CF have at least one copy of this mutation. Since the discovery of the CF gene in 1989, more than 1500 different CF gene mutations have been identified. Because of this, there is significant variability in the disease among patients. The most common mutation is ?F508. Since the discovery of the CF gene in 1989, more than 1500 different CF gene mutations have been identified. Because of this, there is significant variability in the disease among patients. The most common mutation is ?F508. About 70% of patients with CF have at least one copy of this mutation.

9. GENETICS Autosomal recessive trait Both parents are carriers Each pregnancy has a: 1 in 4 chance that the child will have CF 1 in 2 chance that the child will be a carrier of the mutation 1 in 4 chance that the child will not inherit the mutation Affects about 30,000 people in the U.S. and about 70,000 worldwide Incidence in the U.S. is about 1:3,500 births in the U.S. Carrier frequency: 1:25Affects about 30,000 people in the U.S. and about 70,000 worldwide Incidence in the U.S. is about 1:3,500 births in the U.S. Carrier frequency: 1:25

10. Excessive/abnormal mucus production ? bacterial infection � inflammation � airway destruction The lung disease is progressive and is responsible for most of the morbidity and mortality of the disease. Excessive/abnormal mucus production ? bacterial infection � inflammation � airway destruction The lung disease is progressive and is responsible for most of the morbidity and mortality of the disease.

11. Pulmonary Manifestations Chronic cough Bronchial obstruction Secondary bacterial infection Nasal polyps Chronic pneumonia

12. Pulmonary Manifestations Increased respiratory rate Cyanosis Digital clubbing Bronchospasm Chronic sinusitis

13. Pancreatic Insufficiency: plugging of pancreatic ducts � about 85 � 90% of patients with CF are PI. Hepatic Disease � plugging of bile ductules; about 5% have overt cirrhosis CFRD � the endocrine function of the pancreas is also affected. Pancreatic Insufficiency: plugging of pancreatic ducts � about 85 � 90% of patients with CF are PI. Hepatic Disease � plugging of bile ductules; about 5% have overt cirrhosis CFRD � the endocrine function of the pancreas is also affected.

14. Gastrointestinal Manifestations Meconium Ileus Failure to thrive Steatorrhea Bulky stools Intussusception of the small and large bowel Meconium Ileus � bowel is obstructed by very thick meconium (first stool of newborns) Inutssusception � telescope effectMeconium Ileus � bowel is obstructed by very thick meconium (first stool of newborns) Inutssusception � telescope effect

15. Gastrointestinal Manifestations Rectal prolapse Hypoproteinemia Anemia Vitamin deficiencies Gallbladder disease There are other manifestations: Most males with CF are sterile. Cervical mucus is affected in females Hyponatremic dehydration Idiopathic pulmonary osteoarthropathyThere are other manifestations: Most males with CF are sterile. Cervical mucus is affected in females Hyponatremic dehydration Idiopathic pulmonary osteoarthropathy

17. DIAGNOSIS Elevated levels of chloride on sweat test (2 or more times) AND one or more characteristic of CF OR family history of CF OR positive newborn screening test result Nasal Potential Differences can also be performed; NPD measures the voltage across the nasal mucosa (respiratory epithelium) � used only in research centers as there is a lack of commercial equipment and other problems with its large scale use. Genetic testing is also available, but has limitations such as not being able to test for all CFTR mutations; 18% of patients with CF only identify 1 abnormal gene.Nasal Potential Differences can also be performed; NPD measures the voltage across the nasal mucosa (respiratory epithelium) � used only in research centers as there is a lack of commercial equipment and other problems with its large scale use. Genetic testing is also available, but has limitations such as not being able to test for all CFTR mutations; 18% of patients with CF only identify 1 abnormal gene.

18. Newborn Screening All state now have NBS. In AL, it was initiated in April 2008. The advantages of NBS is early diagnosis and initiation of treatment. There is improved growth because treatment is started before patient is symptomatic. This baby is almost 6 months old and was in our NBS Clinic last Friday and is doing very well nutritionally and from a pulmonary standpoint. All state now have NBS. In AL, it was initiated in April 2008. The advantages of NBS is early diagnosis and initiation of treatment. There is improved growth because treatment is started before patient is symptomatic. This baby is almost 6 months old and was in our NBS Clinic last Friday and is doing very well nutritionally and from a pulmonary standpoint.

19. The Median Predicted Survival Age of patients with CF has improved tremendously from 1986 � 2008. Currently, it is 37.4 years, up from about 26 years in 1986. This improvement has resulted from earlier diagnosis and initiation of treatment, new drugs, timely treatment of pulmonary exacerbations, and improved nutrition and has occurred over the course of my career.The Median Predicted Survival Age of patients with CF has improved tremendously from 1986 � 2008. Currently, it is 37.4 years, up from about 26 years in 1986. This improvement has resulted from earlier diagnosis and initiation of treatment, new drugs, timely treatment of pulmonary exacerbations, and improved nutrition and has occurred over the course of my career.

20. This is Danny Bessette. In 2003, he was 19 years old, a college sophomore, and a terrific athlete and a remarkable young man. Since 1989, he has benefited from the availability of three new drugs available to people with CF, and witnessed a 25 percent increase in his life expectancy. He is planning for his future and his career. He is beginning to live out his dreams and that of his parents, and everyone who deals with CF. Danny spoke at last fall�s North American CF Conference on the 20th anniversary of the discovery of the CF gene, now 25 years of age. This is Danny Bessette. In 2003, he was 19 years old, a college sophomore, and a terrific athlete and a remarkable young man. Since 1989, he has benefited from the availability of three new drugs available to people with CF, and witnessed a 25 percent increase in his life expectancy. He is planning for his future and his career. He is beginning to live out his dreams and that of his parents, and everyone who deals with CF. Danny spoke at last fall�s North American CF Conference on the 20th anniversary of the discovery of the CF gene, now 25 years of age.

21. Treatment of Pulmonary Disease Chest clearance Antibiotics Anti-inflammatory medications Infection control Chest clearance: Chest PT Chest vest PEP � Postive Expiratory Pressure Mucolytics (Pulmozyme) B agonists, anticholinergics Antibiotics: Be lcatam Quinilones Aminoglycosides Anti-inflammatory medications: Steroids, NSAIDs Infection control � to avoid bacteria colonization from one patient to another PsA positive and negative clinicsChest clearance: Chest PT Chest vest PEP � Postive Expiratory Pressure Mucolytics (Pulmozyme) B agonists, anticholinergics Antibiotics: Be lcatam Quinilones Aminoglycosides Anti-inflammatory medications: Steroids, NSAIDs Infection control � to avoid bacteria colonization from one patient to another PsA positive and negative clinics

22. Treatment of Gastrointestinal Disease Adequate nutrition Pancreatic enzyme replacement therapy Vitamin supplementation Enzymes with every meal and snack I�ll talk more about each of these. Enzymes with every meal and snack I�ll talk more about each of these.

23. Increased sweat electrolytes Supplemental salt High salt foods Patient is prone to dehydration; need to emphasize adequate hydration. Patient is prone to dehydration; need to emphasize adequate hydration.

24. Pulmonary Complications Atelectasis Bronchiectasis Pneumothorax Hemoptysis Cor pulmonale Respiratory failure Atelectasis � collapsing of air sacs Bronchiectasis � airways are dilated and infected Pneumothorax � collapsing of lung Hemoptysis � coughing up of blood Cor pulmonale � right sided heart failure which occurs as the pulmonary damage progresses Respiratory failureAtelectasis � collapsing of air sacs Bronchiectasis � airways are dilated and infected Pneumothorax � collapsing of lung Hemoptysis � coughing up of blood Cor pulmonale � right sided heart failure which occurs as the pulmonary damage progresses Respiratory failure

25. Gastrointestinal Complications Meconium ileus Distal intestinal obstructive syndrome (DIOS) Fibrosing colonopathy Rectal prolapse Biliary disease Pancreatitis There is reduced hydration in the GI tract because of the thick secretions, which contributes to the intestinal blockages, meconium ileus and DIOS. Fibrosing colonopathy � colonic strictures associated with high doses of pancreatic enzymes.There is reduced hydration in the GI tract because of the thick secretions, which contributes to the intestinal blockages, meconium ileus and DIOS. Fibrosing colonopathy � colonic strictures associated with high doses of pancreatic enzymes.

26. Other Medical Complications CF Related Diabetes Bone Disease CF � related Diabetes has characteristics of Type I and Type II Diabetes. The endocrine function of the pancreas is affected as the pancreas changes morphologically by the disease. CFRD is more common in the adolescent and young adult age group. Risk factors for bone disease include: inadequate calcium and viatmin D and others. CF � related Diabetes has characteristics of Type I and Type II Diabetes. The endocrine function of the pancreas is affected as the pancreas changes morphologically by the disease. CFRD is more common in the adolescent and young adult age group. Risk factors for bone disease include: inadequate calcium and viatmin D and others.

27. As the Median Survival Age has improved, so have growth parameters. This graph shows the differences in the CDC BMI Percentiles of patients with CF from 1990 to 2007. During this time, there has been improvement by 5 or more percentile points at all ages, particularly in the early adolescent age group. During this time, there have been many advances in CF care, but there is also an association between improved survival and growth parameters. As the Median Survival Age has improved, so have growth parameters. This graph shows the differences in the CDC BMI Percentiles of patients with CF from 1990 to 2007. During this time, there has been improvement by 5 or more percentile points at all ages, particularly in the early adolescent age group. During this time, there have been many advances in CF care, but there is also an association between improved survival and growth parameters.

28. Growth and Pulmonary Function Better growth associated with longer survival (Corey, et al., 1988). Continuous weight gain associated with better FEV1 (Peterson, et al., 2003). Increased stature of CF patients associated with longer survival (Beker, et al., 2001). Many studies have been conducted to examine the relationship between improved growth and improved pulmonary function. It is the pulmonary disease of CF that causes the most morbidity and mortality from the disease. Corey et al. compared the patients at 2 different CF Centers. They found that the patients at 1 center who had better growth parameters also had an increased mean age of survival. This center advocated for the use of an unrestricted fat diet for patients with CF and this study became the basis for this recommendation in CF care. Peterson et al. found that children with CF who experience continuous weight gain at a given rate will have better FEV1 (a pulmonary function test) than children who have weight gain patterns that deviate from expected rates of weight gain for age and gender. Lelia Beker, PhD, RD and associates found that stature is a significant prognostic indicator of survival in CF. It is advantageous for the patient with CF to reach their genetic potential for height. Many studies have been conducted to examine the relationship between improved growth and improved pulmonary function. It is the pulmonary disease of CF that causes the most morbidity and mortality from the disease. Corey et al. compared the patients at 2 different CF Centers. They found that the patients at 1 center who had better growth parameters also had an increased mean age of survival. This center advocated for the use of an unrestricted fat diet for patients with CF and this study became the basis for this recommendation in CF care. Peterson et al. found that children with CF who experience continuous weight gain at a given rate will have better FEV1 (a pulmonary function test) than children who have weight gain patterns that deviate from expected rates of weight gain for age and gender. Lelia Beker, PhD, RD and associates found that stature is a significant prognostic indicator of survival in CF. It is advantageous for the patient with CF to reach their genetic potential for height.

29. Malnutrition and Lung Function Malnutrition at any age has negative impact on lung function. Malnutrition associated with a more rapid yearly decline in FEV1. The Steinkamp study was a cross sectional and longitudinal analysis of a patient cohort of the German CF Quality Assurance Project. Malnourished patients of all ages groups had a mean FEV1 that was 12-24% lower than that for normal weight patients. Over a 2 year period, mean FEV1 of malnourished patients continued to decline where that of normal weight patients held steady. The Steinkamp study was a cross sectional and longitudinal analysis of a patient cohort of the German CF Quality Assurance Project. Malnourished patients of all ages groups had a mean FEV1 that was 12-24% lower than that for normal weight patients. Over a 2 year period, mean FEV1 of malnourished patients continued to decline where that of normal weight patients held steady.

30. Growth and Lung Health 1990s data from Epidemiologic Study of Cystic Fibrosis (ESCF) Better growth at age 3 ? better pulmonary functions at age 6 Improved growth from age 3 to age 6 ? better pulmonary functions at age 6 More recently and with the help of the CF community, Dr. Konstan and colleagues analyzed 1990s data from the Epidemiologic Study of Cystic Fibrosis (ESCF) These investigators looked at wt/age %, ht/age %, and % IBW and PFTs. Better growth at age 3 ? better pulmonary functions at age 6 (The reason that pulmonary functions were noted at age 6 is because that is the age at which a child can cooperate and manage to do PFTs). Improved growth from age 3 to age 6 ? better pulmonary functions at age 6 These investigators suggest that the relationship between nutritional status and lung disease be studied even earlier in life. Now, performing pulmonary functions on infants is possible, so we should see new research in this area. More recently and with the help of the CF community, Dr. Konstan and colleagues analyzed 1990s data from the Epidemiologic Study of Cystic Fibrosis (ESCF) These investigators looked at wt/age %, ht/age %, and % IBW and PFTs. Better growth at age 3 ? better pulmonary functions at age 6 (The reason that pulmonary functions were noted at age 6 is because that is the age at which a child can cooperate and manage to do PFTs). Improved growth from age 3 to age 6 ? better pulmonary functions at age 6 These investigators suggest that the relationship between nutritional status and lung disease be studied even earlier in life. Now, performing pulmonary functions on infants is possible, so we should see new research in this area.

31. We see this association between better growth parameters and FEV1 in the 2008 CFF Patient Data Registry. Based on this association, the goal BMI for age % of patients with CF = 50th%; another goal is achieving genetic potential for height growth. Because of this important correlation, more and more emphasis is being place on maintaining good growth parameters in patients with CF and presents an important opportunity for RDs to contribute to the care of patients with CF. We see this association between better growth parameters and FEV1 in the 2008 CFF Patient Data Registry. Based on this association, the goal BMI for age % of patients with CF = 50th%; another goal is achieving genetic potential for height growth. Because of this important correlation, more and more emphasis is being place on maintaining good growth parameters in patients with CF and presents an important opportunity for RDs to contribute to the care of patients with CF.

32. Pulmonary Factors Affecting Nutritional Status Increased work of breathing Cough/emesis cycle Chronic infection Chronic antibiotic therapy There are many aspects of the disease that impacts nutritional status/needs. There are many aspects of the disease that impacts nutritional status/needs.

33. GI Factors Affecting Nutritional Status Fat and protein losses in stools Fat soluble vitamin losses Essential fatty acid losses Bile salts and bile acid losses Even with pancreatic enzyme therapy, there are still nutrient losses in the stool. Even with pancreatic enzyme therapy, there are still nutrient losses in the stool.

34. GI Factors Affecting Nutritional Status Gastroesophageal reflux/esophagitis Distal intestinal obstruction syndrome (DIOS) Fibrosing colonopathy Fibrosing colonopathy is the presence of stricture in the colon and has been associated with very high doses of pancreatic enzymes. Fibrosing colonopathy is the presence of stricture in the colon and has been associated with very high doses of pancreatic enzymes.

35. Nutrition Assessment Anthropometric Measurements Biochemical Data, Medical Tests Physical Examination Findings Client History (including medications) Food/Nutrition History What is the role of the RD on the CF Care Team and in the care of the patient with CF? Performing the components of the Nutrition Care Process: Nutrition Assessment Nutrition Diagnosis Nutrition Intervention Nutrition Monitoring and Evaluation Because of limited time, I am going to focus on the components of nutrition assessment and nutrition intervention. Nutrition Assessment: Anthropometric Measurements Biochemcial Data, Medical Tests Physical Examination Findings Client History Food/Nutrition HistoryWhat is the role of the RD on the CF Care Team and in the care of the patient with CF? Performing the components of the Nutrition Care Process: Nutrition Assessment Nutrition Diagnosis Nutrition Intervention Nutrition Monitoring and Evaluation Because of limited time, I am going to focus on the components of nutrition assessment and nutrition intervention. Nutrition Assessment: Anthropometric Measurements Biochemcial Data, Medical Tests Physical Examination Findings Client History Food/Nutrition History

36. CFF Recommendation(Pediatrics) For children and adolescents with CF, ages 2.0 to 19.9 years, utilize the BMI percentile method for weight-for-stature assessment. Maintain BMI percentile =50th %. Dr. Virginia Stallings, Division of GI, Heptatology, and Nutrition, The Children�s Hospital of Philadelphia, was the lead author on this report published in JADA in 2008. This paper reported the results of a systematic review of evidence-based practice recommendations for nutrition-related management of children and adults with CF and Pancreatic Insufficiency. These are 2 of the recommendations from that paper: BMI % - preferred method for weight-for-statue assessment Goal � BMI 5 = 50th%Dr. Virginia Stallings, Division of GI, Heptatology, and Nutrition, The Children�s Hospital of Philadelphia, was the lead author on this report published in JADA in 2008. This paper reported the results of a systematic review of evidence-based practice recommendations for nutrition-related management of children and adults with CF and Pancreatic Insufficiency. These are 2 of the recommendations from that paper: BMI % - preferred method for weight-for-statue assessment Goal � BMI 5 = 50th%

37. Example of a growth curve of a child with CF. Weight for age % was drifting down; with nutrition intervention, his weight for age % increased. Example of a growth curve of a child with CF. Weight for age % was drifting down; with nutrition intervention, his weight for age % increased.

38. Weight gain is reflected in BMI for age % and this patient did achieve BMI for age % >50th%. Weight gain is reflected in BMI for age % and this patient did achieve BMI for age % >50th%.

39. Biochemical Monitoring at Diagnosis Hemoglobin, hematocrit Serum albumin Plasma or serum retinol Alpha-tocopherol 25-hydroxy Vitamin D Prothrombin time or PIVKA-II Serum sodium, if exposed to heat stress Fecal elastase, a measurement of pancreatic enzyme function, is also now available and usually obtained at diagnosis. Fecal elastase, a measurement of pancreatic enzyme function, is also now available and usually obtained at diagnosis.

40. Biochemical MonitoringAnnually Hemoglobin, hematocrit Plasma or serum retinol Alpha-tocopherol 25-hydroxy Vitamin D Serum albumin Random blood glucose There are also guidelines for monitoring for CF-Related Diabetes, which are currently being revised. There are also guidelines for monitoring for CF-Related Diabetes, which are currently being revised.

41. Including medication historyIncluding medication history

42. Food/Nutrition History Analysis of 24-hour dietary recall or a 3-day food record Nutritional adequacy Meeting estimated needs Caloric distribution At our center, we try to obtain 3-day food records, kept prospectively, at least once a year. We check the intake for nutritional adequacy, not only of energy and protein, but also for key nutrients including calcium, vitamin D, iron, and zinc. We are also interested in caloric distribution. At our center, we try to obtain 3-day food records, kept prospectively, at least once a year. We check the intake for nutritional adequacy, not only of energy and protein, but also for key nutrients including calcium, vitamin D, iron, and zinc. We are also interested in caloric distribution.

43. Nutrition Intervention in CF Promote normal growth and development through: High Calorie, high protein, unrestricted fat diet Enzyme replacement therapy Vitamin and mineral supplementation Addressing behavioral issues Referral to food and nutrition resources I�ll talk more about each of these strategies. I�ll talk more about each of these strategies.

44. General Recommendations Aim for 35% to 40% of kilocalories from fat. Encourage snacks. Include high calorie foods. Discourage �grazing�. Discourage/limit sweetened beverages. Diet is higher in fat than is normally recommended because of the high energy needs. The basis of the diet is well balanced with a variety of foods. Healthier fats can be emphasized. Must take into consideration the needs of the entire family and help come up with strategies for increasing the caloric content of the foods of the child with CF. Grazing � constant access to food/beverages; hard to dose enzymes when a child is eating constantly and blunts appetite for meals and snacks. At our center, we discourage sweetened beverages because we do not feel they are part of a healthy diet. Plus with young children, we find that they drink a lot of these beverages, which blunts their appetite at meal and snack times. Diet is higher in fat than is normally recommended because of the high energy needs. The basis of the diet is well balanced with a variety of foods. Healthier fats can be emphasized. Must take into consideration the needs of the entire family and help come up with strategies for increasing the caloric content of the foods of the child with CF. Grazing � constant access to food/beverages; hard to dose enzymes when a child is eating constantly and blunts appetite for meals and snacks. At our center, we discourage sweetened beverages because we do not feel they are part of a healthy diet. Plus with young children, we find that they drink a lot of these beverages, which blunts their appetite at meal and snack times.

45. Estimating Calorie Needs 12 year old girl with cystic fibrosis Daily recommended intake = 2000 Calories Multiply by 1.3 � 1.5 for cystic fibrosis = 2600 � 3000 Calories per day There are many formulas that could be used in estimating the needs of a patient with CF. This is one of the methods used at our center. In the Stallings 2008 paper, the evidence analysis showed that energy needs for weight gain range from 110-200% over those of healthy peers. There are many formulas that could be used in estimating the needs of a patient with CF. This is one of the methods used at our center. In the Stallings 2008 paper, the evidence analysis showed that energy needs for weight gain range from 110-200% over those of healthy peers.

46. Sample Menu Breakfast 2 (4� square) waffles 2 tsp. margarine 1 oz. syrup 2 slices bacon 8 oz. whole milk 1 banana AM Snack 4 oz. fruit cup 4 oz. juice drink Lunch 1 slice pizza 1 medium serving fries 1 cup salad 2 Tbsp. salad dressing 8 oz. whole milk PM Snack 1 whole sandwich (2 slices bread, 1 slice lunch meat, 1 slice cheese, 1 Tbsp. mayo) 4 oz. juice drink This is menu planned to meet the estimated needs of this 12 year old girl with CF. This is menu planned to meet the estimated needs of this 12 year old girl with CF.

47. Sample Menu (continued) Supper 3 oz grilled chicken 2 oz. gravy 1 cup cooked rice � cup greens 1 roll 1 tsp. margarine 8 oz. sweet tea Bedtime Snack � cup dry cereal 8 oz whole milk TOTAL CALORIES: 3000 47% carbohydrate 13% protein 40 % fat This menu does meet her estimated needs in the caloric distribution that is recommended. However, she would need to average this intake over time in order to consistently meet her needs. It may be difficult for some children to eat this amount of Calories consistently. This menu does meet her estimated needs in the caloric distribution that is recommended. However, she would need to average this intake over time in order to consistently meet her needs. It may be difficult for some children to eat this amount of Calories consistently.

48. Children with CF often have poor appetites and for good reasons: Acute pulmonary exacerbation Sinusitis Abdominal pain GER depression/anxietyChildren with CF often have poor appetites and for good reasons: Acute pulmonary exacerbation Sinusitis Abdominal pain GER depression/anxiety

49. Behavioral Interventions Gradually increase calories by working on one meal at a time. Take full advantage of offering snacks. Parents can learn alternate ways of responding to child during meals. Identify appropriate rewards for eating the expected amount of food. Consider behavioral therapy. Identify behavioral issues related to eating early and address. Children learn at a very young age that whether or not they eat is very important to their parents/caretakers. Chronic illness, especially one like CF where nutritional status is being emphasized, can further complicate this situation. I think that we ask parents of children with CF to walk a very fine line � trying to help their children meet their nutrition needs while keeping meal time as pleasant and �normal� as possible. These are some behavioral intervention strategies recommended by the CF Foundation. Identify behavioral issues related to eating early and address. Children learn at a very young age that whether or not they eat is very important to their parents/caretakers. Chronic illness, especially one like CF where nutritional status is being emphasized, can further complicate this situation. I think that we ask parents of children with CF to walk a very fine line � trying to help their children meet their nutrition needs while keeping meal time as pleasant and �normal� as possible. These are some behavioral intervention strategies recommended by the CF Foundation.

50. Nutrition Intervention of CF Nutritional Counseling Oral Supplements Enteral Feedings Nutrition Counseling/Education: routine part of the patients� outpatient clinic visits and hospitalizations. Oral Supplements: can be home made or commercial products Enteral Feedings: sometimes there is a widening gap between what a child with CF is able to consume and the estimated energy needs; enteral feedings can be an adjunct therapy to help meet these needs; usually the feedings are dripped in at night while the child sleeps and the child can eat regular meals throughout the day.Nutrition Counseling/Education: routine part of the patients� outpatient clinic visits and hospitalizations. Oral Supplements: can be home made or commercial products Enteral Feedings: sometimes there is a widening gap between what a child with CF is able to consume and the estimated energy needs; enteral feedings can be an adjunct therapy to help meet these needs; usually the feedings are dripped in at night while the child sleeps and the child can eat regular meals throughout the day.

51. Nutrition Intervention of CF Enzyme replacement therapy Taken before each meal and snack Not to exceed 2500 units lipase/kg body weight/meal May need to add medicines to decrease gastric acidity to improve effectiveness Stallings article does not recommend using generic enzymes; enzymes should be stored in a cool dry place; have recently required FDA approval. Dosing limits are because of the possibility of fibrosing colonopathy with high enzyme doses. Stallings article does not recommend using generic enzymes; enzymes should be stored in a cool dry place; have recently required FDA approval. Dosing limits are because of the possibility of fibrosing colonopathy with high enzyme doses.

52. Nutrition Intervention of CF Vitamin and mineral supplementation ADEK�, AquADEK�, SourceCF�, or Vitamax� daily Use of salt on foods and inclusion of high salt foods in diet Calcium supplements if intake not meeting needs Vitamin D supplements if levels low CF Vitamin and Mineral Supplements provide fat-soluble vitamins in a water-miscible form. CF Vitamin and Mineral Supplements provide fat-soluble vitamins in a water-miscible form.

53. Nutrition Intervention in CF Referral to Food/Nutrition Resources Supplemental Food Program for Women, Infants, and Children (WIC) State Children with Special Health Care Needs Program Child Nutrition Programs State Medicaid Patient Assistance Programs Asking about Alternative/Complementary Medicine

54. Nutrition Goals Provide energy intakes greater than standard for general population. Maintain normal growth parameters and weight for length or BMI-for-age =50th%. Provide intensive intervention, including nutrition counseling, behavioral intervention, and nutrition supplements to help meet goals. The RD who cares for children with CF plays a huge role in the patient�s ongoing care and is an integral member of the CF Care Team. Applying the components of the Nutrition Care Process (Nutrition Assessment, Nutrition Diagnosis, Nutrition Intervention, and Nutrition Monitoring and Evaluation) to the care of a child with CF provides the RD many nutrition intervention opportunities. The RD who cares for children with CF plays a huge role in the patient�s ongoing care and is an integral member of the CF Care Team. Applying the components of the Nutrition Care Process (Nutrition Assessment, Nutrition Diagnosis, Nutrition Intervention, and Nutrition Monitoring and Evaluation) to the care of a child with CF provides the RD many nutrition intervention opportunities.

55. Although not the sole reason for the improvement in the median predicted survival age of patients with CF, improved nutrition has played a role and RDs have played important roles in the promotion and achievement of improved nutritional status of patients with CF. Although not the sole reason for the improvement in the median predicted survival age of patients with CF, improved nutrition has played a role and RDs have played important roles in the promotion and achievement of improved nutritional status of patients with CF.

56. Individual RDs promoted better nutritional status for their patients by being an active and contributing member of CF Care Teams, by going to work every day, speaking up and being assertive when necessary, and bringing nutritional issues that needed to be addressed to the other team members� attention. Collectively, RDs were also making an impact through their work with agencies, such as the CF Foundation, the Pediatric Pulmonary Centers, and the Pediatric Nutrition Practice Group of the ADA. I am going to now talk about the work that has been accomplished by RDs through these organizations and spotlight the work of Pennsylvania RDs. In the examples that I will give, often times the areas of education, research, and clinical practice overlap and often individual RDs have been involved at various levels. Collective efforts have made an impact. Individual RDs promoted better nutritional status for their patients by being an active and contributing member of CF Care Teams, by going to work every day, speaking up and being assertive when necessary, and bringing nutritional issues that needed to be addressed to the other team members� attention. Collectively, RDs were also making an impact through their work with agencies, such as the CF Foundation, the Pediatric Pulmonary Centers, and the Pediatric Nutrition Practice Group of the ADA. I am going to now talk about the work that has been accomplished by RDs through these organizations and spotlight the work of Pennsylvania RDs. In the examples that I will give, often times the areas of education, research, and clinical practice overlap and often individual RDs have been involved at various levels. Collective efforts have made an impact.

57. 25th Annual Meeting of the CF Club San Jose, California, April 1984 Planning Committee: Physicians Nurses Social Workers PhDs NO RDs!!! Nutrition topics covered by MDs This was the first CF Meeting that I attended � the first time that I had traveled to CA! My colleagues, Donna Mueller, Suzanne Michel, both from Philadelphia, and Sarah Walker, a PPC nutritionist were there. RDs were not included on the planning committee or in the nutrition presentations. After this meeting, Suzanne Michel, who I will tell you more about in a minute, decided to �stir the pot� at this point and decided to call Bob Beall, CEO of the CFF about the absence of an RD on the meeting planning committee. I interviewed Suzanne by phone about this phone call. She remembers that her heart rate was about 300, her palms were sweaty, and knees shaking when she called him. She pointed out that she was very concerned that there was no RD on the planning committee. He was very receptive and asked for her recommendation of someone to serve. Also, about this time, the name of the meeting was changed to the North American CF Conference (NACFC) and included the Canadians as well as other international participants. This was the first CF Meeting that I attended � the first time that I had traveled to CA! My colleagues, Donna Mueller, Suzanne Michel, both from Philadelphia, and Sarah Walker, a PPC nutritionist were there. RDs were not included on the planning committee or in the nutrition presentations. After this meeting, Suzanne Michel, who I will tell you more about in a minute, decided to �stir the pot� at this point and decided to call Bob Beall, CEO of the CFF about the absence of an RD on the meeting planning committee. I interviewed Suzanne by phone about this phone call. She remembers that her heart rate was about 300, her palms were sweaty, and knees shaking when she called him. She pointed out that she was very concerned that there was no RD on the planning committee. He was very receptive and asked for her recommendation of someone to serve. Also, about this time, the name of the meeting was changed to the North American CF Conference (NACFC) and included the Canadians as well as other international participants.

58. Dr. Donna Mueller was the first RD invited to serve on the Program Committee for the 2nd Annual North American Cystic Fibrosis Conference). Donna recently told me that she has had these italicized words on her CV since 1987 and never deleted them, �even after all of these years.� When Donna accepted her faculty position at Drexel University, she thought someone else still in clinical practice should be the RD on the Program Planning Committee. Dr. Donna Mueller was the first RD invited to serve on the Program Committee for the 2nd Annual North American Cystic Fibrosis Conference). Donna recently told me that she has had these italicized words on her CV since 1987 and never deleted them, �even after all of these years.� When Donna accepted her faculty position at Drexel University, she thought someone else still in clinical practice should be the RD on the Program Planning Committee.

59. Suzanne H. Michel, MPH, RD, LDN Current Position: Clinical Dietitian, Pediatric CF Center, The Children�s Hospital of Philadelphia Previous CF Positions: Nutritionist, Adult CF Program, Drexel School of Medicine Department of Pediatrics, Hahnemann University Hospital

60. Suzanne H. Michel, MPH, RD, LDN Highlights of CF Foundation Activities Member, Planning Committee, NACFC, 1990, 1991 Member, Consensus Committee, Nutrition Assessment & Management in CF Member, Clinical Practice Guidelines Member, Working Group on Care of the Infant Diagnosed with CF through NBS

61. Suzanne H. Michel, MPH, RD, LDN CF Nutrition Source: Online nutrition and cystic fibrosis professional education series. Initiated 2002 and currently ongoing. Educational Grant from CF Services Pharmacy for Patient Booklets, 2005 �Color Your Calories� �Grab �N Go, Meals and Snacks for People on the Run� Throughout her career, Suzanne has partnered with industry and various agencies in the development of CF education materals. These are some of her most recent examples. She also has CF Education Cards available at: PORTCF. Org and Source CF.com Suzanne indicated to me that what makes her the proudest, in terms of professional accomplishments, would have to be all of the educational materials that have been developed over the years that help RDs help patients. Suzanne�s dedication throughout her career to the development of nutrition education materials exemplifies the same commitment to nutrition education that I understand was important to Anna de Planters Bowes. Throughout her career, Suzanne has partnered with industry and various agencies in the development of CF education materals. These are some of her most recent examples. She also has CF Education Cards available at: PORTCF. Org and Source CF.com Suzanne indicated to me that what makes her the proudest, in terms of professional accomplishments, would have to be all of the educational materials that have been developed over the years that help RDs help patients. Suzanne�s dedication throughout her career to the development of nutrition education materials exemplifies the same commitment to nutrition education that I understand was important to Anna de Planters Bowes.

62. Cystic Fibrosis Services Pharmacy Justine Spisak, BS, Nutrition Student Suzanne Michel, MPH, RD, LDN, Editor Donna H. Mueller, PhD, RD, FADA, LDN, Editor Elizabeth McFarlane, Format Approved by the CFF Education Committee An example of one of Suzanne�s educational pamphlets; Donna Mueller is an editor. An example of one of Suzanne�s educational pamphlets; Donna Mueller is an editor.

63. CFF�s Golden Apple Award Given in recognition of personal commitment to excellence in the advancement of professional education and patient care. To date, Suzanne is the only RD to have received this award!!! Something else that Suzanne is quite proud of is receiving the CFF�s Golden Apple Award in 1994. To date, she is the only RD to have received this award.Something else that Suzanne is quite proud of is receiving the CFF�s Golden Apple Award in 1994. To date, she is the only RD to have received this award.

64. Midwest CF Nutrition and Social Work Consortium Grew out of an Ohio multidisciplinary consortium Became an Ohio CF RDs consortium Expanded to the Midwest in 2007 Social workers joined in 2009 Have met in Chicago (2007), Milwaukee (2008), Cincinnati (2009), Indianapolis (2010) I am going to deviate slightly here and you�ll understand why in the next slide! The consortium secures funding and plans an excellent continuing education program. Participants only have to pay their travel. This is an excellent CE as well as networking opporunity. I am going to deviate slightly here and you�ll understand why in the next slide! The consortium secures funding and plans an excellent continuing education program. Participants only have to pay their travel. This is an excellent CE as well as networking opporunity.

65. �There is a very special addition to my CV � the Midwest RDs picked me to receive the Midwest RD Achievement Award. I was VERY surprised and very honored. The award is for RDs from the Midwest so there was no way I would ever have thought that the person they were describing during the introduction was me. Was I ever shocked when they called my name.� And I finally got a picture! Suzanne was a shining example of what an RD could bring to a CF Team in terms of clinical expertise and patient/family education. �There is a very special addition to my CV � the Midwest RDs picked me to receive the Midwest RD Achievement Award. I was VERY surprised and very honored. The award is for RDs from the Midwest so there was no way I would ever have thought that the person they were describing during the introduction was me. Was I ever shocked when they called my name.� And I finally got a picture! Suzanne was a shining example of what an RD could bring to a CF Team in terms of clinical expertise and patient/family education.

66. Jean Tomezsko, MPH, PhD, RD, LDN CF-related Clinical Experience: Clinical Nutritionist, CF Center, Hahnemann University, 1991-1995 Clinical Nutritionist, CF Center, St. Christopher�s Hospital for Children, 1995-1999 Clinical Nutritionist, CF Center at Children�s Hospital of Philadelphia (CHOP), 1999 � 2007 Retired from CHOP, October 2007

67. Jean Tomezsko, MPH, PhD, RD, LDN CF Foundation related activities: Member, Program Planning Committee, NACFC, 2002 through 2008 Chaired numerous Discussion Sessions and Workshops at NACFCs Publications: PhD Dissertation � Energy expenditure, body composition, and dietary intake of children with CF Numerous publications with Dr. Virginia Stallings in peer-reviewed journals Jean was on the planning committee for 7 meetings. Jean was involved in clinical practice and research as well as being involved in the continuing education of her colleagues. Jean was on the planning committee for 7 meetings. Jean was involved in clinical practice and research as well as being involved in the continuing education of her colleagues.

68. Judy Fulton, MPH, RD, LDN Current Position: Clinical Dietitian/Research Coordinator, Children�s Hospital of Pittsburgh of University of Pittsburgh Medical Center Judy works with adult patients with CF at the Antonio J. and Janet Palumbo CF Center, Children�s Hospital of Pittsburgh She also facilitates the research process for Phase I-IV studies under the Good Clinical Practice guidelines. At our center, one of our CF RDs also became one of our Research Coordinators, a testiment to the multiple talents of the RD. Judy works with adult patients with CF at the Antonio J. and Janet Palumbo CF Center, Children�s Hospital of Pittsburgh She also facilitates the research process for Phase I-IV studies under the Good Clinical Practice guidelines. At our center, one of our CF RDs also became one of our Research Coordinators, a testiment to the multiple talents of the RD.

69. Judy Fulton, MPH, RD, LDN CF Foundation related activities: Selected Member, Pediatric Nutrition Consensus Conference, 2001-2002 Selected Member, CFF Education Committee, 2006-present Pediatric Nutrition Practice Group, ADA CF/Pulmonary Nutrition Networking Coordinator Member Support Co-Chair, Chair Author of CF Chapter for Pediatric Diet Manual Judy has promoted the role of the RD on the CF Care Team in multiple ways. She has been very active in the CFF as well as the PNPG of the ADA. Judy has promoted the role of the RD on the CF Care Team in multiple ways. She has been very active in the CFF as well as the PNPG of the ADA.

70. Since 2007, Amanda Leonard of Johns Hopkins and Terri Schindler, Rainbow Babies & Children�s Hospital in Cleveland, OH. Have coordinated a CF Nutrition Mentoring Program through the CFF. Amanda came up with the idea and pitched it to the CFF. It is a program where new nutrition pracitioners to the field of CF care are matched with experienced mentors. Amanda made a presentation during FNCE in Denver on Monday, Oct. 19� her handout still available on ADA web site. Currently, there are about 35 mentors available to work with mentees. 51 apprentices/mentees have been mentored and 10 more are scheduled to spend time with mentors this sprign and summer. Because the program was so successful with the nutritionists, the CFF has expanded the program to other disciplines, including social work, RT, and nursing, but nutrition was the first this time!!! I have worked with 2 mentees, Elizabeth Revilla, MS, RD, Emory CF Center in Atlanta, GA and Kanan Patel, MS, RD, CSP, Children�s Health Care of Atlanta Scottish Rite Campus. I am looking forward to working with Katie Fowlkes from Memphis next week. Since 2007, Amanda Leonard of Johns Hopkins and Terri Schindler, Rainbow Babies & Children�s Hospital in Cleveland, OH. Have coordinated a CF Nutrition Mentoring Program through the CFF. Amanda came up with the idea and pitched it to the CFF. It is a program where new nutrition pracitioners to the field of CF care are matched with experienced mentors. Amanda made a presentation during FNCE in Denver on Monday, Oct. 19� her handout still available on ADA web site. Currently, there are about 35 mentors available to work with mentees. 51 apprentices/mentees have been mentored and 10 more are scheduled to spend time with mentors this sprign and summer. Because the program was so successful with the nutritionists, the CFF has expanded the program to other disciplines, including social work, RT, and nursing, but nutrition was the first this time!!! I have worked with 2 mentees, Elizabeth Revilla, MS, RD, Emory CF Center in Atlanta, GA and Kanan Patel, MS, RD, CSP, Children�s Health Care of Atlanta Scottish Rite Campus. I am looking forward to working with Katie Fowlkes from Memphis next week.

71. Other CF Foundation Activities RD presenters included in international Teleconferences RD members of CF Education Committee RD members of CFRD consensus guidelines RDs have been presenters at live teleconferences, which were subsequently archived on the CFF web site. The CFF has been very supportive of RDs as essential members of the CF Care Team. RDs have been presenters at live teleconferences, which were subsequently archived on the CFF web site. The CFF has been very supportive of RDs as essential members of the CF Care Team.

72. Maternal and Child Health Bureau Pediatric Pulmonary Centers The Pediatric Pulmonary Centers are MCHB-funded interdisciplinary graduate and post-graduate level leadership training programs. Disciplines include: medicine, nursing, nutrition, respiratory care, and social work. The PPCs were first funded in the mid-1970s. There is a competitive renewal every 5 years and we have just completed grant applications last month. We are anxiously awaiting the results of the grant reviews. The configuration of PPCs has changed over the years and the numbers of dwindled. With this most recent competitive renewal, it is our understanding that the number will be further decreased to 6. The PPCs have been important training grounds for many professionals. We had advocates at the Maternal and Child Health Bureau for the inclusion of the discipline of nutrition in these programs. Professionals such as Lib Brannon and Ann Prendergast were our advocates at the national level. Many PPC nutritionists and our former trainees have been active with the CFF and the PNPG. The Pediatric Pulmonary Centers are MCHB-funded interdisciplinary graduate and post-graduate level leadership training programs. Disciplines include: medicine, nursing, nutrition, respiratory care, and social work. The PPCs were first funded in the mid-1970s. There is a competitive renewal every 5 years and we have just completed grant applications last month. We are anxiously awaiting the results of the grant reviews. The configuration of PPCs has changed over the years and the numbers of dwindled. With this most recent competitive renewal, it is our understanding that the number will be further decreased to 6. The PPCs have been important training grounds for many professionals. We had advocates at the Maternal and Child Health Bureau for the inclusion of the discipline of nutrition in these programs. Professionals such as Lib Brannon and Ann Prendergast were our advocates at the national level. Many PPC nutritionists and our former trainees have been active with the CFF and the PNPG.

73. Pediatric Nutrition Practice Group of ADA Practice group started in 1978 Cystic Fibrosis included in Quality Assurance Manual published in early 1980s CF/Pulmonary Nutrition Networking Group 1990s

74. The Dietitians in Pediatric Practice�s Quality Assurance Manuel included outcome and process criteria for selected pediatric conditions, including cystic fibrosis. Through the encouragement of Lib Brannon at MCHB, I became involved in this project and it was really my portal to becoming involved with ADA at the national level. These criteria were field-tested and this publication was one of ADA�s first on the topic of quality assurance or qualtiy improvement. The Dietitians in Pediatric Practice�s Quality Assurance Manuel included outcome and process criteria for selected pediatric conditions, including cystic fibrosis. Through the encouragement of Lib Brannon at MCHB, I became involved in this project and it was really my portal to becoming involved with ADA at the national level. These criteria were field-tested and this publication was one of ADA�s first on the topic of quality assurance or qualtiy improvement.

75. The Network between the PNPG and the CFF was established in 2007 and is designed to increase opportunities for interaction and information sharing between the CFF and the PNPG. Mary Marcus is currently serving her 2nd term as the PNPG Network Representative to the CFF. Her contributions: Developed instructions for the CFF nutrition mentors and apprentices on obtaining CPE credits for their involvement in the CFF nutrition mentoring program. Coordinates communication between the PNPG and the CFF Keeps PNPG members updated on CF nutrition related issues Increase participation in CFF activities by RDs who are also PNPG members. The Network between the PNPG and the CFF was established in 2007 and is designed to increase opportunities for interaction and information sharing between the CFF and the PNPG. Mary Marcus is currently serving her 2nd term as the PNPG Network Representative to the CFF. Her contributions: Developed instructions for the CFF nutrition mentors and apprentices on obtaining CPE credits for their involvement in the CFF nutrition mentoring program. Coordinates communication between the PNPG and the CFF Keeps PNPG members updated on CF nutrition related issues Increase participation in CFF activities by RDs who are also PNPG members.

76. Through all of the efforts and dedication of so many RDs in the areas of education, research, and clinical practice, and with physician and other team members support: The RD has been established as an integral member of the CF Care Team. Acceptance by the CF community that nutrition plays a key role in patient outcome, well-being and life expectancy. Donna Mueller: �The genuine support of MDs and other clinicians and researchers along the way definitely is a sub-topic. Without the support (financial and all else) of the federal government, our universities, and colleagues (RDs and others), I don�t think RDs would have grown to their pre-eminent statue you and I experienced. What do you think?� My response: � I agree that we have reaped the benefits of support from MCHB and forward thinking physician colleagues. We also worked hard!� Donna: �And yes, I completely agree with you � it is the RD, individually, who established herself/himself, as a professional and person of statue recognized by colleagues in other professions. Nobody can make an already excellent person �more� excellent. Outstanding individuals then join together. I guess somewhat like the word synergy, except in this case, excellent was made �more� excellent.� There is an integral member of the team who I have said little about and that is the patient and family/caretakers. I am going to close with a brief case study about a young man with CF from Meadville, PA. Through all of the efforts and dedication of so many RDs in the areas of education, research, and clinical practice, and with physician and other team members support: The RD has been established as an integral member of the CF Care Team. Acceptance by the CF community that nutrition plays a key role in patient outcome, well-being and life expectancy. Donna Mueller: �The genuine support of MDs and other clinicians and researchers along the way definitely is a sub-topic. Without the support (financial and all else) of the federal government, our universities, and colleagues (RDs and others), I don�t think RDs would have grown to their pre-eminent statue you and I experienced. What do you think?� My response: � I agree that we have reaped the benefits of support from MCHB and forward thinking physician colleagues. We also worked hard!� Donna: �And yes, I completely agree with you � it is the RD, individually, who established herself/himself, as a professional and person of statue recognized by colleagues in other professions. Nobody can make an already excellent person �more� excellent. Outstanding individuals then join together. I guess somewhat like the word synergy, except in this case, excellent was made �more� excellent.� There is an integral member of the team who I have said little about and that is the patient and family/caretakers. I am going to close with a brief case study about a young man with CF from Meadville, PA.

77. This is Ethan DeVantier, who lives in Meadville, PA and whose mother, Allyson, is a registered dietitian and works in long-term care. Ethan was born at a hospital in Pittsburgh almost 11 years ago where Newborn Screening for CF was performed. He was diagnosed with CF as an infant. He started pancreatic enzymes at 8 weeks of age. Currently, he uses the chest vest for chest PT and is on TOBI and Pulmozyme. He has not had pulmonary problems and has never been hospitalized. Current growth parameters: Weight = 29.9 kg (10th-25th %s) Height = 140 cm (25th-50th%s) BMI for age = 15.25 kg/m2 (10th-25th%) He does have GERD and delayed gastric emptying, for which he is on a low dose of erythromycin. His mother reports that it is almost like he doesn�t get hungry. He is willing to try new foods, but eats only a small amount and is a very slow eater. Physical Activity: Swim team Runs cross country His mother describes him as �fidgety� (made Ethan left when he saw this part!)This is Ethan DeVantier, who lives in Meadville, PA and whose mother, Allyson, is a registered dietitian and works in long-term care. Ethan was born at a hospital in Pittsburgh almost 11 years ago where Newborn Screening for CF was performed. He was diagnosed with CF as an infant. He started pancreatic enzymes at 8 weeks of age. Currently, he uses the chest vest for chest PT and is on TOBI and Pulmozyme. He has not had pulmonary problems and has never been hospitalized. Current growth parameters: Weight = 29.9 kg (10th-25th %s) Height = 140 cm (25th-50th%s) BMI for age = 15.25 kg/m2 (10th-25th%) He does have GERD and delayed gastric emptying, for which he is on a low dose of erythromycin. His mother reports that it is almost like he doesn�t get hungry. He is willing to try new foods, but eats only a small amount and is a very slow eater. Physical Activity: Swim team Runs cross country His mother describes him as �fidgety� (made Ethan left when he saw this part!)

78. Ethan�s mother says that he definitely has some challenges with his nutrition and sometimes �it�s more difficult to have my background because I know what he should be doing, and he doesn�t always want to do it.� Ethan typically has 3 meals plus an after school snack each day. For breakfast, he drinks a Scandishake (powder mixed with whole milk = 600 kilocalories); Scandical and melted ice cream are also added and his mother estimates that these shakes may have about 750 kilocalories per serving. He also takes a Resource in his lunch during the school day and another with his after school snack. He has another Scandishake at night. Ethan has 2 younger siblings and the family tries to eat a healthy diet. His younger siblings sometimes want to know why they can�t have extra butter on their pancakes like Ethan does. When asked how she would like to receive nutrition management information, she said that she thinks the health care provider needs to take into consideration the background of the family member/caretaker. She thinks RDs need to learn to speak to families on different levels and to customize their approach with different families. Ethan has gone through periods where he wasn�t gaining weight and because of her professional background, Allyson knew in the back of her mind, that a feeding tube was a possibility. She admits that she did not want him to have a feeding tube, not because there was a stigma attached to the feeding tube, but because she saw it as a sign that he may not be as healthy as he has been. Ethan�s mother says that he definitely has some challenges with his nutrition and sometimes �it�s more difficult to have my background because I know what he should be doing, and he doesn�t always want to do it.� Ethan typically has 3 meals plus an after school snack each day. For breakfast, he drinks a Scandishake (powder mixed with whole milk = 600 kilocalories); Scandical and melted ice cream are also added and his mother estimates that these shakes may have about 750 kilocalories per serving. He also takes a Resource in his lunch during the school day and another with his after school snack. He has another Scandishake at night. Ethan has 2 younger siblings and the family tries to eat a healthy diet. His younger siblings sometimes want to know why they can�t have extra butter on their pancakes like Ethan does. When asked how she would like to receive nutrition management information, she said that she thinks the health care provider needs to take into consideration the background of the family member/caretaker. She thinks RDs need to learn to speak to families on different levels and to customize their approach with different families. Ethan has gone through periods where he wasn�t gaining weight and because of her professional background, Allyson knew in the back of her mind, that a feeding tube was a possibility. She admits that she did not want him to have a feeding tube, not because there was a stigma attached to the feeding tube, but because she saw it as a sign that he may not be as healthy as he has been.

79. This picture was taken on Easter Sunday. This is Ethan with his brother, Cole, who is 5, and his sister, Keira, who is 3. This picture was taken on Easter Sunday. This is Ethan with his brother, Cole, who is 5, and his sister, Keira, who is 3.

80. Anna dePlanter Bowes Lecture April 27, 2010 Pittsburgh, PA I have presented an overview of cystic fibrosis, with emphasis on nutritional management and have highlighted how the persistent professional performance of registered dietitians have contributed to the improved outcomes of patients with CF. I want to thank Dawna for the invitation to speak, the RDs and Ethan and his mother, Allyson, who provided photos, and allowed me to interview them and share their stories for this presentation. It was a wonderful opportunity to re-connect with some long-time colleagues. And now I challenge you to adopt similar strategies and to look for networking opportunities with your RD and other health professional colleagues to advocate for the specific patient populations with whom you work. Keep stirring the pot!I have presented an overview of cystic fibrosis, with emphasis on nutritional management and have highlighted how the persistent professional performance of registered dietitians have contributed to the improved outcomes of patients with CF. I want to thank Dawna for the invitation to speak, the RDs and Ethan and his mother, Allyson, who provided photos, and allowed me to interview them and share their stories for this presentation. It was a wonderful opportunity to re-connect with some long-time colleagues. And now I challenge you to adopt similar strategies and to look for networking opportunities with your RD and other health professional colleagues to advocate for the specific patient populations with whom you work. Keep stirring the pot!