CF nutritional issues in children

Cystic fibrosis:
Nutritional Issues in
children
Dr Ola Alkhars
Pediatric Consultant
30/11/2022
1

Outlines
• PATHOGENESIS
• RIASK FACTORS FOR NUTRITIONAL DEFICIENCIES
• PREVELANCE
• ASSESSING AND MONITORING NUTRITION
• NUTRITION SUPPORT
• NUTRIENT DEFICITS AND GOALS
2

Increased energy
requirements
Reduce appetite
Malabsorption
Growth failure in CF Patients

Risk Factors for Nutritional Deficiencies
and growth failure in CF Patients
5
pancreatic
insufficiency( 90 % of
patients with CF)
Malabsorption of, protein, fat and several
micronutrients ( the vitamins A, D, E, and K.)
Chronic Pulmonary
infection
Increase work of breathing
Increased energy requirements
cause cytokine-induced catabolism
Reduce appetite

•Other GI Disorders : Altered GI motility, Intestinal dysbiosis,Small
bowel bacterial overgrowth, GERD , DIOS, constipation
•CF-related liver disease (CFLD)
•CF-related diabetes (CFRD)
6
Comorbidities which may aggravate
Nutritional Deficiencies

The spectrum of CF disease varies according to
Genotype
Individual
Environmental
factors
The nutritional risks and
requirements for a
patient with CF also vary
along this disease
spectrum but do not
precisely coincide with
the severity of
pulmonary disease.

The nutritional status of individuals with CF tends to decline during childhood. However, data from the CF Foundation (CFF) show that,
on average, the BMI percentile for both children and adults has been increasing over the past 20 years .

The CF Foundation (CFF) patient registry has documented substantial improvement in life expectancy of patients with CF
To a large degree, the longer life achieved

ASSESSING AND MONITORING NUTRITION
The most effective way to maintain good
nutritional status in CF, is to prevent
suboptimal nutrition from occurring.

Growth
For children younger than 2 years of age, the same percentile
criteria are applied to weight-for-length rather than BMI.
For children with CF, the BMI target range
between the 50th and 85t percentile for age.
BMI
10th - 50th percentiles
At nutritional risk
< 10th percentile In need of nutritional
rehabilitation.

Another important indicator of nutritional sufficiency is the achievement of full genetic potential for height; an approach that only
emphasizes BMI may overlook children with stunting.

Monitoring
• A number of studies used open and closed
indirect calorimetry and doubly-labeled
water to determine energy expenditures.
• These studies consistently show that
energy expenditure is directly associated
with the severity of the CF genotype and
inversely associated with pancreatic
function
18

NUTRIENT DEFICITS AND GOALS
• In the past, the caloric requirement of a CF patient was estimated to be 130 %
of recommended dietary allowance for calories .
• Calories — A wide range of energy requirements are reported in CF patients
ranging from normal to 150 % of normal, depending on the CF genotype, the
patient's age, and current state of health, including pulmonary function and
the presence of CF-related liver disease (CFLD).
• 22

NUTRITION SUPPORT
Oral
• For infants with CF, human milk feeding is specifically encouraged.
• If infants are fed formula, a standard infant formula may be used

BehaviouralTechniques
●Providing attention and praise for positive eating behaviors
● Gentle persistence when offering new foods
●Ignoring negative eating behaviors such as food refusal
●Offer meals and substantive snacks on a regular schedule
●Keep mealtimes relaxed and time-limited; do not enforce specific goals for intake at
each meal

A large multicenter trial of children aged 4 to 12 years assessed the efficacy of a behavioral intervention designed to promote energy intake .
The group receiving the behavioral intervention achieved improved energy intake and weight gain at the end of a 9-week intervention, and a slower rate of declinein
(BMI) Z-score over the subsequent two years, as compared with control patients receiving nutritional education alone.

Copyrights apply
.
If dietary counseling is not successful, a liquid supplement that is high in calories and protein can be added to the diet
These supplements tend to displace ordinary food rather than being taken in addition to a usual diet , and then patients often
complain of taste fatigue with oral supplements.

As
.
a systematic review found that calorie-protein supplements do not confer benefits above dietary advice and monitoring in CF
patients who have moderate malnutrition
.

.
Practice of enteral feeding is supported by a number of observational studies that suggest improved nutritional status and
stabilization of lung function in CF patients receiving enteral nutrition.

Enteral feedings
Route
• usually provided via gastrostomy tube (G-tube) because nasogastric tubes are
usually poorly tolerated due to chronic cough, nasal polyps, and the sensation of
suffocating.
• Gastrojejunostomy and jejunostomy feeding may also be used, but these tubes
are more difficult to place and maintain in position.
• Jejunal feeds must be given continuously rather than in boluses, making the
route inconvenient for ambulatory patients.
• A skin-level device (or "button") can be used so that the patient can disconnect
from all tubing between feeds, to optimize mobility and cosmesis.

Enteral feedings
• For the school-aged child, a frequently schedule supplies approximately
40 % of daily requirements via a slow infusion overnight, and the remainder
of the requirement is supplied through food taken by mouth during the day.
• For the younger patient who is at home during the day, nutritional
requirements can be supplied through a combination of nighttime
continuous feeds, daytime meals, and daytime bolus feeds.
• If the child fails to ingest the prescribed amount at any daytime meal,
a bolus feed is added to compensate.

Copyrights apply
Some formulas supply protein that is either elemental or semi-elemental.

Formulas
• Formulas containing extensively hydrolyzed protein are not helpful unless the
infant has a milk protein intolerance in addition to CF.
• However, more extensive protein digestion does not have a clinical advantage as
long as pancreatic enzyme replacement is given, and it may have the
disadvantage of increasing the osmolarity of the formula.

• Concentrated formulas with 1.5 to 2 Kcal/mL have the advantage of delivering
more calories in a smaller volume.
• Since nighttime urination is a problem when using high-volume overnight feeds,
a smaller volume can make the feeding schedule more tolerable.
• When using the more concentrated formulas, care must be exercised to avoid
carbohydrate overload and possible inadequate supply of free water.
• Use of formulas with concentrated carbohydrates may uncover CFRD.

Enzyme replacement
• LCT is the main determinant of the lipase requirement because (MCT)
require relatively low concentrations of lipase to hydrolyze the fatty acids
from the glycerol backbone.
• Therefore, MCT accounts for little of the fat malabsorption in CF.

Enzyme replacement
Lipase Dose : 1600 to 2000 units per gram LCT .
•For cycled and continuous overnight feed: administer 3/4 of the enzymes at the
beginning of the feeding and 1/4 of the enzymes at the end of the feeding.
•For bolus feedings, administer the calculated amount of enzymes just prior to the
feeding.
•For continuous 24-hour feedings, divide the total calculated lipase into 6 doses,
and give each dose at 4 -hour intervals.

A cartridge delivery
system (Relizorb)
This system has been approved by the
US FDA for use in children 5 years of
age and older.

Efficacy data are still limited, but a small trial suggests that it can help reduce early morning satiety and bloating for some individuals .

Parenteral nutrition
• INDICATION :
• If GI function is inadequate to supply complete nutrition.
• Extreme metabolic stress
• Recovery from gastrointestinal surgery
• Transplantation procedure.
• PN should not be a part of palliative care.

One study observed that PN promoted weight gain in patients with CF but was
also associated with higher rates of sepsis .
Moreover, after PN was discontinued, weight decreased again, and no long-term
gain was achieved

Fat-soluble vitamins
• (CFF) recommends supplementation of these vitamins for all children with CF.
• Supplements should be started as soon as CF is diagnosed, including in
asymptomatic infants and in individuals without pancreatic insufficiency.

Vitamin A
• Except at the time of diagnosis, vitamin A deficiency is a rare occurrence in CF.
• Supplements exists as "preformed vitamin A" (retinol, retinal, retinoic acid, and
retinyl ester) and as "provitamin A“
• Most supplements contain preformed vitamin A.

Vitamin A
Toxicity from vitamin A supplements is probably a more important clinical
issue.
• This is particularly concerning because long-term vitamin A toxicity results
in bone mineral loss and liver abnormalities.
• In some supplements, part of the vitamin A is provided in the form of beta-
carotene.
• Beta-carotene is a provitamin A, and because its conversion to vitamin A is
physiologically regulated, it has a lower risk for toxicity than preformed
vitamin A

Vitamin D
• Desirable levels : 30 to 60 ng/mL (75 to 150 nmol/L).
• The CFF recommends vitamin D3 (cholecalciferol) because a
small study suggests that it is somewhat more likely to achieve
target 25-hydroxyvitamin D levels than vitamin D2
(ergocalciferol).

Vitamin D
• Even with high-dose ergocalciferol treatment (50,000 IU 3 times weekly for 8
weeks), vitamin D levels remained below the target range in 57 % of patients.
• Similarly, a report from three Canadian CF centres documented that 95 % of CF
patients had suboptimal vitamin D status despite having daily vitamin D intake
above 400 IU .

Vitamin D
• However, whether achieving 25-OH vitamin D levels in the recommended
target range will cause clinically important improvements in bone health
has not been established.

Vitamin E
• Physiology –Vitamin E is a fat-soluble vitamin with 8 different forms, each
of which has its own profile of activity.
• All forms act as antioxidants at cell membranes, preventing membrane damage.
• The form most commonly used as a supplement is alpha-tocopherol acetate.
• Gamma tocopherol may play an important and complementary role to alpha-
tocopherol in scavenging free radicals

Vitamin E
Recommended intake
• The doses are approximately 20-fold higher than the recommended intake
for healthy individuals
• The known role of vitamin E as an antioxidant has generated interest in the
possibility that higher doses of vitamin E, might reduce inflammation and
end-organ damage in CF.
• Clinical efficacy of this approach not been established, and supplements in
excess of the above dosing ranges are not generally recommended.

Vitamin E
Monitoring
–Serum vitamin E levels are strongly influenced by the concentration of serum
lipids, do not accurately reflect tissue vitamin levels, and patients with CF
typically have lower serum cholesterol levels than the reference population.
• Therefore, the alpha-tocopherol: cholesterol ratio or alpha-tocopherol:
total serum lipid ratio may be a better measure of sufficiency.

Vitamin K
Physiology.
o Vitamin K is a cofactor required for the activity of several key proteins in the
coagulation pathways, including prothrombin.
o Vitamin K-dependent carboxylation is also necessary for function of osteocalcin
and other bone-related proteins.
Patients with CF are at risk for vitamin K deficiency because of
• Fat malabsorption
• Disturbances in the bowel flora associated with
• Small intestine bacterial overgrowth (SIBO)
• Frequent use of antibiotics.

Vitamin K
Recommended intake
• These doses are over 100-fold higher than the recommended intake for
individuals without CF.

Vitamin K
• Monitoring – PT & PIVKA-II
• PIVKA-II is more sensitive than prothrombin time in detecting vitamin K
deficiency.
• Using this measure, 40 % of patients with CF were found to have
suboptimal vitamin K status, despite meeting the goals for vitamin K
intake recommended by the CFF

Essential fatty acids
Physiology – EFAs can be categorized as:
• Omega-3 fatty acids Including alpha-linolenic acid and docosahexaenoic acid (DHA).
• They exhibit profound antiinflammatory effects, raising the possibility that they could be
beneficial in chronic inflammatory states including CF.
• Omega-6 fatty acids – Including linoleic acid and its active metabolite, arachidonic
acid, which have proinflammatory effects but are also important markers of
nutritional status in children with CF

• Clinical manifestations – EFA deficiency: scaly dermatitis, alopecia,
thrombocytopenia, and growth failure.
• Overt symptoms of EFA deficiency are uncommon but there is
some evidence for subclinical deficiency in this population.
• EFA deficiency is more common among infants and patients with
pancreatic insufficiency.

• Monitoring – Routine EFA monitoring is not recommended for patients with CF .
• Periodic monitoring for patients at high risk for as infants with growth failure
• The preferred method is to measure the total fatty acid profile in red blood cells.
• EFA deficiency is likely when the levels of linoleic, alpha-linolenic, and
eicosapentaenoic acids and DHA are below reference range.
• This test has the advantage of providing information on omega-3 as well as
omega-6 fatty acids but is not widely available .
• If this test is not available, EFA status can be partially evaluated by measuring the
triene : tetraene ratio in blood; a ratio >0.2 suggests EFA deficiency.

Sodium
• Infant with CF are prone to hyponatremic dehydration under conditions of heat
stress, and also for children with exercise.
• A similar picture can develop in infants without heat stress .

Fluoride
• Infants and children with CF require fluoride for dental health at the same
levels as healthy children .
• Vitamins formulated forCF do not generally include fluoride.
• Supplements should be supplied separately beginning at six months of age,
if the fluoride concentration of the water supply is not adequate.

Zinc
• For infants with CF under two years of age who are not
growing well despite adequate energy intake and
pancreatic enzyme supplementation, the CFF suggests an
empiric trial of zinc supplementation (1 mg elemental
zinc/kg/day in divided doses for 6 months).
• Rarely, infants with undiagnosed CF may present with a
dermatitis caused by zinc deficiency, often in combination
with EFA deficiency and/or protein-energy malnutrition.
• The dermatitis resembles acrodermatitis enteropathica.

Pharmacologic options
• Growth hormone –A few clinical trials have
examined the use of recombinant growth
hormone in children with CF and growth failure,
with 12 months follow-up .
• These have shown modest improvement in
height, weight, lean tissue mass compared with
no treatment, and a very small improvement in
(FVC)
• No adverse effects on blood glucose were noted.
• Current data are insufficient to justify routine use
of GH in this population outside of a clinical study.

Appetite stimulants
• That have been tried include megestrol acetate, cyproheptadine, dronabinol , and mirtazapine (an
antidepressant).
• There have been concerns about the efficacy, the length of treatment, and side effects with all of
these drugs.
• Megestrol acetate can cause short-term weight gain, including LBM, and can result in improved lung
function.
• S/E steroid-like side effects, Adrenal suppression and testicular failure have been reported.
• Long-term use has not been fully evaluated.
• Cyproheptadine generally leads to short-term improvement in appetite, and is primarily useful to
overcome an acute and temporary anorexia
• S/E antihistamine-like side effects.
• The long term efficacy is less well-established

• Insulin for patients with CFRD (with or without fasting hyperglycemia), has
beneficial effects on nutrition, and probably also on pulmonary function.
• The benefits are less well-established for individuals with earlier stages of
CF-related dysglycemia.

• The most effective way to maintain good nutritional status
in CF, is to prevent suboptimal nutrition from occurring
• Optimizing nutrition forCF individual associated with
improved quality of life, slow rate of declining in pulmonary
function and prolong life

REFERENCES
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2. Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2002; 35:246.
3. Borowitz D, Durie PR, Clarke LL, et al. Gastrointestinal outcomes and confounders in cystic fibrosis. J Pediatr Gastroenterol Nutr 2005; 41:273.
4. Bass R, Brownell JN, Stallings VA. The Impact of Highly Effective CFTR Modulators on Growth and Nutrition Status. Nutrients 2021; 13.
5. Riordan JR, Rommens JM, Kerem B, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989; 245:1066.
6. Hayden HS, Eng A, Pope CE, et al. Fecal dysbiosis in infants with cystic fibrosis is associated with early linear growth failure. Nat Med 2020; 26:215.
7. Zemel BS, Jawad AF, FitzSimmons S, Stallings VA. Longitudinal relationship among growth, nutritional status, and pulmonary function in children with cystic fibrosis: analysis of the Cystic Fibrosis Foundation National CF Patient Registry. J Pediatr 2000; 137:374.
8. Reilly JJ, Edwards CA, Weaver LT. Malnutrition in children with cystic fibrosis: the energy-balance equation. J Pediatr Gastroenterol Nutr 1997; 25:127.
9. Stallings VA, Stark LJ, Robinson KA, et al. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc 2008; 108:832.
10. Lahiri T, Hempstead SE, Brady C, et al. Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis. Pediatrics 2016; 137.
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