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Nutrition Assessment and Management in CF Pennsylvania

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									“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
      IMPACT OF RDs

                  EDUCATION




RESEARCH

           CLINICAL PRACTICE
     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.
 CYSTIC
FIBROSIS
  (CF)
Discovery of CF Gene
 Cystic Fibrosis
Transmembrane
  Conductance
   Regulator
    (CFTR)
      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
        ∆F508 Mutations
Homozygous ∆F508                50.2 %


Heterozygous ∆F508              37.7 %


No ∆F508 or both                12.1 %
unidentified


Cystic Fibrosis Foundation Patient Registry, 2002
         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
 Lungs – viscid secretion

 Bronchial & Secondary
 Obstruction Infection

      Bronchiectasis

   Recurrent Atelectasis

Pneumonia or Lung Abscess

   Progressive Fibrosis
      & Emphysema
         Pulmonary
        Manifestations
• Chronic cough
• Bronchial obstruction
• Secondary bacterial infection
• Nasal polyps
• Chronic pneumonia
         Pulmonary
        Manifestations
• Increased respiratory rate
• Cyanosis
• Digital clubbing
• Bronchospasm
• Chronic sinusitis
       Gastrointestinal
           Disease




Pancreatic Insufficiency
Liver Disease
CF related diabetes mellitus
        Gastrointestinal
        Manifestations
• Meconium Ileus
• Failure to thrive
• Steatorrhea
• Bulky stools
• Intussusception of the small and
  large bowel
       Gastrointestinal
       Manifestations
• Rectal prolapse
• Hypoproteinemia
• Anemia
• Vitamin deficiencies
• Gallbladder disease
DIAGNOSIS
          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
Newborn Screening
                                          Median Predicted Survival Age, 1986-2008

Median Survival Age (years)   40




                              36




                              32




                              28




                              24
                                   '86   '88   '90    '92    '94   '96          '98   '00   '02   '04   '06   '08

                                                                         Year




                              CF Foundation, Patient Data Registry, 2008
Danny Bessette – 2003!
Treatment of Pulmonary
       Disease
• Chest clearance
• Antibiotics
• Anti-inflammatory medications
• Infection control
     Treatment of
Gastrointestinal Disease
• Adequate nutrition
• Pancreatic enzyme replacement
  therapy
• Vitamin supplementation
      Increased sweat
        electrolytes
• Supplemental salt
• High salt foods
Pulmonary Complications
• Atelectasis
• Bronchiectasis
• Pneumothorax
• Hemoptysis
• Cor pulmonale
• Respiratory failure
       Gastrointestinal
        Complications
• Meconium ileus
• Distal intestinal obstructive
  syndrome (DIOS)
• Fibrosing colonopathy
• Rectal prolapse
• Biliary disease
• Pancreatitis
         Other Medical
         Complications
• CF Related Diabetes
• Bone Disease
Cystic Fibrosis Foundation, Patient Registry, 2007
   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).
     Malnutrition and Lung
           Function
• Malnutrition at any age has negative
  impact on lung function.
• Malnutrition associated with a more
  rapid yearly decline in FEV1.




 Steinkamp et al., Thorax, 2002
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


  Konstan, et al. J Pediatr 2003
                                             FEV1 Percent Predicted vs. BMI Percentile in
                                                       Children 6 to 20 Years
                                  100




                                  90
         FEV1 Percent Predicted




                                                                                                            Males
                                                                                                            Females
                                  80


                                                     Goal
                                                     50th Percentile

                                  70




                                  60
                                        <5      10     20              30   40            50      60   70       80    90
                                                                                 BMI Percentile


FEV1 percent is positively correlated with BMI percentile for patients 6 to 20
(correlation > 0.95, p < 0.0001).

CFF Patient Data Registry, 2008
    Pulmonary Factors
Affecting Nutritional Status
• Increased work of breathing
• Cough/emesis cycle
• Chronic infection
• Chronic antibiotic therapy
    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
    GI Factors Affecting
     Nutritional Status
• Gastroesophageal reflux/esophagitis
• Distal intestinal obstruction
  syndrome (DIOS)
• Fibrosing colonopathy
   Nutrition Assessment
• Anthropometric Measurements
• Biochemical Data, Medical Tests
• Physical Examination Findings
• Client History (including medications)
• Food/Nutrition History



 American Dietetic Association, 2009
      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 %.




Stallings VA, et al., JADA, 2008
 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

 Borowitz et al, J of Pediatr Gastro and Nutr, 2002
 Biochemical Monitoring
       Annually
• Hemoglobin, hematocrit
• Plasma or serum retinol
• Alpha-tocopherol
• 25-hydroxy Vitamin D
• Serum albumin
• Random blood glucose


 Borowitz et al, J of Pediatr Gastro and Nutr, 2002
Client History/Physical Examination Findings
     Pulmonary Status
     GI Status
     Other medical conditions
  Food/Nutrition History
• Analysis of 24-hour dietary recall or
  a 3-day food record
• Nutritional adequacy
• Meeting estimated needs
• Caloric distribution
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
         General
     Recommendations
• Aim for 35% to 40% of kilocalories
  from fat.
• Encourage snacks.
• Include high calorie foods.
• Discourage “grazing”.
• Discourage/limit sweetened
  beverages.
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
                 Sample Menu
• Breakfast                     • Lunch
  –   2   (4” square) waffles     –   1   slice pizza
  –   2   tsp. margarine          –   1   medium serving fries
  –   1   oz. syrup               –   1   cup salad
  –   2   slices bacon            –   2   Tbsp. salad dressing
  –   8   oz. whole milk          –   8   oz. whole milk
  –   1   banana                • PM Snack
• AM Snack                        – 1 whole sandwich
  – 4 oz. fruit cup                 (2 slices bread, 1 slice
  – 4 oz. juice drink               lunch meat, 1 slice
                                    cheese, 1 Tbsp. mayo)
                                  – 4 oz. juice drink
             Sample Menu
              (continued)
• Supper                     • Bedtime Snack
  –   3 oz grilled chicken     – ¾ cup dry cereal
  –   2 oz. gravy              – 8 oz whole milk
  –   1 cup cooked rice
  –   ½ cup greens             TOTAL CALORIES:
  –   1 roll                   3000
  –   1 tsp. margarine         47% carbohydrate
  –   8 oz. sweet tea          13% protein
                               40 % fat
Poor Appetite
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.
Cystic Fibrosis Foundation, 2001
Nutrition Intervention of CF

• Nutritional Counseling
• Oral Supplements
• Enteral Feedings
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




  Borowitz 1995; FitzSimmons 1997
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
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
        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.
                                          Median Predicted Survival Age, 1986-2008

Median Survival Age (years)   40




                              36




                              32




                              28




                              24
                                   '86   '88   '90    '92    '94   '96          '98   '00   '02   '04   '06   '08

                                                                         Year




                              CF Foundation, Patient Data Registry, 2008
         IMPACT OF RDs




                 CF Foundation

         Pediatric Pulmonary Centers


Pediatric Nutrition Practice Group, ADA
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
                      CF Foundation Activities

                      •Member, Program Planning
                      Committee, 2nd Annual North American
                      Cystic Fibrosis Conference (NACFC),
                      1988 [First Registered Dietitian invited
                      to serve on Program Committee]
                      •Member, Program Planning
                      Committee, 3rd Annual NACFC, 1989
                      •Member, Nutrition Grants Review
                      Committee, 1989
                      •Member, 1st Consensus Panel on
Donna H. Mueller,     Diabetes Mellitus and Cystic Fibrosis,
PhD, RD, FADA, LDN    1990
Associate Professor   •Member, 1st Consensus Panel on
Drexel University     Nutrition and Cystic Fibrosis, 1990
 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
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
 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”
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
 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!!!
   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)
      Suzanne H. Michel, MPH, RD, LDN
2010 Recipient, Midwest RD Achievement Award
       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
       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
   Judy Fulton, MPH, RD, LDN

• Current Position:
  • Clinical Dietitian/Research Coordinator,
    Children’s Hospital of Pittsburgh of University
    of Pittsburgh Medical Center
   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
Amanda Leonard, MPH, RD, CDE,     Terri Schindler, MS, RD
Johns Hopkins Children’s Center   Rainbow Babies &
Baltimore, MD                     Children’s Hospital,
                                  Cleveland, OH
  Other CF Foundation Activities

• RD presenters included in international
  Teleconferences
• RD members of CF Education Committee
• RD members of CFRD consensus
  guidelines
Maternal and Child Health Bureau
  Pediatric Pulmonary Centers
  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
Mary Marcus, MS, RD, CSP



Senior Clinical Nutritionist and
Co-Director, PPC,
University of Wisconsin – Madison

1st Pediatric Nutrition Practice Group
Network Representative with the CFF
      IMPACT OF RDs

                  EDUCATION




RESEARCH

           CLINICAL PRACTICE
Anna dePlanter Bowes
      Lecture
     April 27, 2010
     Pittsburgh, PA

								
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