Cystic Fibrosis

Question 1

What is Cystic Fibrosis?

Answer 1

Is a genetic disease from the mutation of the (CFTR) gene.

If both parents are carriers there is a 25% chance that each parent will pass on their defective gene, meaning their baby will have CF.

Question 2

How to Test for CF?

Answer 2

Newborn screening

Heel-prick test

Checks for CF and other genetic conditions at birth

If positive, further testing is required (blood, sweat test)

Question 3

How CF affects nutrition?

Answer 3

1. Increases energy expenditure: Chronic lung inflammation and infections/ Increased work of breathing and coughing.
2. Maldigestion, Malabsorption & other losses: Exocrine Pancreatic Insufficiency, Reduced Bile Salts, Increased Intestinal Mucus
3. Inadequate Intake: Poor appetite worsened by infection-related anorexia Dyspnoea, early satiety, dry mouth & taste changes due to oxygen or drug therapy, Behavioural Factors

Question 4

How does CF affect the Pancreas?

Answer 4

Causes;

Low bicarbonate secretion

Reduced or nil enzyme secretion

Hyperacidity- HCl

Reduced bile acid secretion

Question 5

What are the symptoms of malabsorption?

Answer 5

Loose stools
large stools
greasy/ floating stools
foul-smelling
frequent trips to the bathroom
excess gas
bloated stomach
stomach cramps/ pains
weight loss

Question 6

What are the dose guidelines for PERT?

Answer 6

500-4000 U lipase per gram dietary fat
Max dose of 10 000 lipase per KG body weight/day
Aim for the lowest effective dose
Distribute the enzymes throughout the day in relation to
fat content of fats & fluids

Question 7

What Vitamins are CF patients usual deficient in?

Answer 7

A, D, E & K have been reported in CF patients even those
receiving exocrine pancreatic enzymes and multiple
vitamin preparations

Question 8

What if the overall summary of nutrition intervention for CF patients?

Answer 8

A balanced quality diet supplemented with fat-soluble
vitamins, (A,D,E,K) and additional sodium to meet individual energy demands accompanied by adequate provision of pancreatic enzyme replacement therapy (PERT) to match the dietary fat intake are required to achieve adequate nutritional status.

In addition:
It is also important to Review the GI /or liver for problems and check for CF Bone Disease.

Question 9

What is an example PES of a patient with CF?

Answer 9

Malnutrition related to poor appetite and malabsorption evidenced by Clinical undernutrition present on admission BMI 18.1 and wt loss 6kg 10.7% TBW in 1/12 PERT history, malabsorption symptoms (loose bowels), low fat-soluble vitamin values(vit A 0.5umol/L, vit D 14 nmol/L , vitE 7 umol/L).

Question 10

What are intervention strategies for CF?

Answer 10

Review current enzyme and food intake

Educate re: appropriate distribution of PERT per fat content. Provide fat counters, amounts of fat in favourite foods. Investigate usual intake at home.

Question 11

What are the Calculation steps for PERT?

Answer 11

Calculate total fat intake/day eg 200g

Calculate total no. of enzymes/day eg 20

Calculate average enzyme to fat ratio eg 200/20 = 10

Calculate IU lipase /g fat eg 25000/10 = 2500 IU lipase/g fat

Divide the amount of fat in each meal by the ratio eg LUNCH: 77/10 = 7.7

PERT ONLY WORKS WITHIN 30 MINUTES OF EATING.

Question 12

How would you monitor and evaluate a CF patient?

Answer 12

clinical: check weight, Fat-soluble vitamins, Bowels / GI symptoms, Blood glucose levels.

Intake: Diet history - intake

Behavioural: PERT/fat knowledge, Adherence to recommendations.

Telephone contact within 1-week post
discharge