Transcript Lily Johnson

Cystic
Fibrosis
Diyana Zainal
Lorena Ferchaud
What is Cystic Fibrosis?
 Inherited mutation in the protein:


Cystic Fibrosis Transmembrane Conductance
Regulator (CFTR)
> 1500 mutations possible, not all resulting in
cystic fibrosis
 Affects lungs and digestive system

Body produces thick and sticky mucus due to
electrolyte imbalance
 Occurs in the first decade of life, average lifespan is
31 years
(O’Riordan, 2009) (O’Sulivan, 2009) (Stallings, 2008)(Farrell, 2008)
Symptoms of Cystic Fibrosis
http://www.nhlbi.nih.gov/health/dci/images/cysticfibrosis01.jpg
Who is at risk for
Cystic Fibrosis?
 1 in every 2,500 live births worldwide; 1 in 31
Americans are carriers
 Conception between 2 carriers will result in
 25% chance their child will have Cystic Fibrosis
 50% chance the child will be a carrier
 25% the child will be a non-carrier
 Most will be diagnosed by age 3, 10% of new cases
are diagnosed at 18 years or older
(Nelms, 2007)
Prevalence of Cystic Fibrosis
(O’Sullivan 2009)
http://2.bp.blogspot.com/_AH0As01Tvu8/SamqR-uzuyI/AAAAAAAAAAU/sT2bBu5Nb5s/S1600-R/inheritance.gif
Diagnostic Process
(Farrell, 2008)
Sweat Chloride Test

Tested only at accredited
CF care centers
1. Stimulate sweat glands
2. Collect onto filter paper
3. Analyze chloride
concentration
 Chloride concentrations of
>60mmol/L is diagnostic of
the disease, but not the
severity.
(O’Sullivan, 2009) (Ferrell, 2008)
Are All Newborns Tested?
 Not all states require testing at birth.
 40 states today have testing, all states by 2010
 Patients can be pancreatic sufficient or insufficient
 Pancreatic sufficient patients have a milder case and
are not diagnosed until later in life.
 Age of onset of symptoms are variable
2 CF mutations vs. 1 CF mutation
 Some infants die in utero, some require surgery at
birth for meconium ileus
 In medieval times midwives would instruct mothers to
lick their newborns

(Hudson 2002) (Farrell 2008)
Medical Nutrition Therapy
 110% to 200% of energy needs with high calorie,
high fat diet



To support weight maintenance
Strong correlation between nutritional status and pulmonary
function
Recommended to maintain BMI at or above 50th percentile
 Nutritional supplements
 Pancreatic enzyme
 Fat soluble vitamins
 Babies who are breastfed confer lifelong benefits
 500 to 2,500 units lipase per kilogram body weight
per meal
(O’Sullivan, 2009) ( Stallings, 2008)
Cystic Fibrosis Treatment
Cystic Fibrosis Physiotherapy
University Hospital
Cystic Fibrosis
Case Study
Patient: Lily Johnson
14 years old
Female
Diagnosed with Cystic Fibrosis at 6 months
Lily Johnson
 Age: 14 years old
 Sex: Female
 Participates in ballet, jazz and cross country running.
Typically runs 3-5 miles, 5-6 times a week.
Chief Complaint
Patient reports trouble breathing and her inhaler
is not helping. She believes she may have caught a
cold from working at a camp for the past two weeks.
Her doctor assumes it is pneumonia due to the fact
she is hospitalized approximately once a year for
pneumonia.
Medical History
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Diagnosed at 6 months
Hospitalized for respiratory infections
approximately 1x per year, but otherwise,
uneventful disease course
Treatment: High frequency chest compression
vest, 1 hour twice daily
High frequency chest compression video
Nutrition History

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Appetite fine until the last few
days
Enjoys fruits, vegetables and
most foods but doesn’t drink
milk
Mom and grandmother cook
healthy, but not always home to
eat meals
Fried foods like French fries
and pizza causes diarrhea
Realizes the need for a healthy
diet, and willing to make
changes
24 hr recall/usual food intake
24 hour recall
 Breakfast - nothing
 Lunch – 2 oz hotdog and bun, 1½ c macaroni and cheese
(boxed mix, 2% milk)
 Dinner – 5 oz Salisbury steak with ¼ c gravy, few bites green
beans, 1 roll + 2 T margarine, 2 c grape juice
Usual intake
 Breakfast – usually nothing
 Lunch - 3 T peanut butter or 2 oz ham and 2 oz Swiss cheese
sandwich, 2-3 oz chips, orange or other fruit, water
 Dinner – 5-6 oz lean meat, grilled or baked, 1-2 c green salad,
¼ c ranch dressing, 1 c starch side dish, water
Medical Diagnosis
 Acute pneumonia confirmed by chest x-ray and
sputum cultures
Physical exam

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Vitals
 99.1 F
 BP 114/60mm Hg
Anthropometric
 Ht 5’5”
th+ percentile)
 Wt 102 lbs (25
 UBW: 110-115lbs (3 months ago)
Chest/lungs:
 percussion hyperresonant
 rhonchi
 rales decreased breath sounds
Stature for age
• 75th percentile
Weight for age
• 25th-50th percentile
Lab Values
 WBC - 13 (normal 4.8 –11.8) indicating infection
 HGB - 11.5 (12-15)
 HCT - 33 (37-47)
 Ferritin - 19 (20-120)
iron deficient anemia
(due to medication)
 Transferrin – 219 (250-380)
 Magnesium – 1.6 (1.8-3)
 HbA1C – 6.3 (3.9-5.2) family history of T2DM,
possibly cystic fibrosis-related diabetes
All other labs within normal range
Cystic Fibrosis
Related Diabetes (CFRD)
 Common and serious problem, in pancreatic-
insufficient individuals
 Close correlation with deterioration in lung function
and loss of weight
 Oral Glucose Tolerance Testing (OGTT) is the most
sensitive method to detect CFRD
Food Preferences and Allergies
 The patient is an active 14
year old and will eat almost
anything, but doesn’t like milk.
 She tries to avoid fried foods
due to resulting diarrhea.
 She has no known allergies.
Nutrition Care Process
 Cystic Fibrosis patients need 110 - 200% more kcals
than normal to maintain homeostasis.
 Harris Benedict Equation
BEE = 655 + (9.6 x 46kg) + (1.8 x 165.1cm) - (4.7 x 14y/o) =1328 kcals
BEE x IF x AF =1328 kcals x 1.5 x 1.5 = 2988 kcals/day
Macronutrients:
Protein – 15% - 113g = 452 kcals
Fat
– 35% - 117g = 1053 kcals
CHO – 50% - 374 g = 1496 kcals
3001 kcals
(Stallings 2008)
Actual vs Recommended Intake
1775 kcals
80g fat
65g protein
198g CHO
3001 kcals
117g fat
113g protein
374g CHO
(actual)
(recommended)
Nutrient Intake
400
4000
3000
2000
1000
0
grams
kcals
Actual vs. Recommended intake
300
Actual Intake
200
100
Recommended
Intake
0
Actual Intake
Recommended
Intake
p
Intake
n
ei
t
ro
(g
)
g)
t(
fa
C
HO
)
(g
Macronutrient
Potential Diet-Drug Interactions
 Pancrease (1-3 caps after meals)
Pancreatic enzyme replacement
Prevacid (20mg daily)
 May decrease abs of Fe and B12
Humabid (1/2 tablet every 12 hours)
 Expectorant (such as Mucinex or Robitussion)
Multivitamin
Proventil PRN
 Bronchodilator
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(Pagna 2007, Progna 2008)
Various intake formats
 Main format - oral intake
 A relatively healthy teenager hospitalized with
pneumonia would not be a good candidate for enteral
nutrition supplementation
 She will need to consume meals with high nutrient
density several times a day until her health (and
weight) is restored.
PES Statement
1) Food and nutrition related knowledge deficit (NB-1.1)
related to time lapse since last education and
change in energy expenditure as evidenced by
inconsistent and unregulated use of prescribed
medications.
2) Involuntary weight loss (NC – 3.2) related to
increase in exercise and failure to increase caloric
intake as evidenced by a 10% weight loss over a 3
month period.
Scope
of
Practice
Sample Menu:
44% CHO, 19% protein and 38% fat
 Breakfast
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Honey nut cheerios and whole milk
Sourdough bread
Banana
 Lunch
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Ham and cheese sandwich
Romaine salad and ranch dressing
Orange

Mid morning snack
Cheese crackers

Afternoon Snack
Peanut butter and jelly
sandwich
Yogurt

Supper
 Dinner
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Steak
Mashed potatoes
Baby carrots
Dinner roll
Caesar salad with dressing
Grapes
Frozen yogurt
Outcome goals
 Short term: no further weight loss
 Long term: maintain BMI at or above the 50th
percentile
Action oriented goals
 Patient can demonstrate knowledge of diet plan
appropriate for a highly active cystic fibrosis patient
Monitor and evaluate
 Track weight
 Monitor if patient is following an increased energy
intake diet
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WBC
HGB
HCT
Ferritin
Transferrin
Magnesium
HbA1C
Follow up
 RD visits every 3 months
 Referral to diabetes educator, if needed
References
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Britto C., Oliveira C., Gomes M., Cunha A. Bras Pneumol. 2009;35(5):409-414
Farrell P, et al. The Journal of Pediatrics. 2008; 153:S4-S14
Hudsen, V. Leaven. 2002;38:5:99-102.
Moran A. Pediatric Diabetes 2009:10(Suppl.12):43–50.
Nelms, M. Nutrition Therapy and Pathophysiology. 2007:731-737
O'Sullivan B., Freedman S. The Lancet. 373: 9678, 30 May 2009-5 June 2009, Pages
1891-1904
O’Riordan S, Robinson P, Donaghue K, Moran A. Pediatric Diabetes 2009: 10(Suppl. 12):
43–50
Pagana, Kathleen Deska, and Timothy J. Pagana. Mosby’s Diagnostic and Laboratory
Test Reference. Saint Louis: Mosby Elsevier, 2007.
Pronsky, Zaneta M. comp. Food and Medication Interactions. Birchrunville: FoodMedication Interactions. 2008.
Stallings V, Stark L, Robinson K, et al. J Am Diet Assoc. 2008;108:832-839.