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Nutritional Management In A 3-month-old Infant Suffering With Cystic Fibrosis And Cytomegalovirus Infection
By - Dr. Srinivasa Murthy
Consultant Neonatologist and Paediatrician MBBS, MD Pediatrics (PGI Chandigarh), MRCPCH (UK) Motherhood Hospital, HRBR layout, Bangalore
Introduction:

Cytomegalovirus is one of the most common causes of congenital infection. Most of infected infants will remain asymptomatic, but about 10% of infected newborns will have symptomatic disease and 10–15% will develop problems during the first 6 years of life. Symptoms of cytomegalovirus infection include growth restriction, microcephaly, seizures, hepatitis syndrome, thrombocytopenia, anemia, and pneumonitis.1

Cystic fibrosis is characterized by a progressive decline in pulmonary function secondary to chronic lung infections, exocrine pancreatic insufficiency leading to malnutrition, liver disease, and growth impairment.2 Malnutrition in cystic fibrosis results from increased energy expenditure, decreased energy intakes and malabsorption of ingested nutrients.3

Malnutrition and lung disease are inextricably woven together in cystic fibrosis. Studies have shown that lung function decline and malnutrition are related and are dependent factors. The occurrence of malnutrition is associated with reductions in lung function and survival. Chronic pulmonary infections and decreased lung function result in increased calorie needs and reduced appetite, which worsen the nutritional status of cystic fibrosis patients.4

Nutritional assessment, early identification of malnutrition, and prompt initiation of supportive treatments are essential parts of cystic fibrosis patients’ care as nutritional status can affect the patients’ response to treatment. Weight gain and normal muscle mass are linked to normal growth and good pulmonary functions.5

Here we present a case of 3-month-old infant suffering with cystic fibrosis and cytomegalovirus infection and not thriving well.

Patient Profile:

A 3-month-old male infant presented with chief complaint of cystic fibrosis and cytomegalovirus infection. He was not thriving well. At admission, his weight was 2.9 kg, height was 48 cm, head circumference was 35 cm and mid-upper arm circumference was 8.3 cm.

Relevant Medical History:

Infant was born through normal vaginal delivery. He was not gaining significant weight. By the end of one-month, his weight was similar to the birthweight (2.9 kg).

Relevant Feeding History:

Infant was on breastfeeding. Since, he was unable to gain weight therefore started on formula feeding in addition to breast milk.

Investigations:

Sweat chloride test, polymerase chain reaction (PCR) and complete blood count (CBC) were ordered.

Diagnosis:

Both sweat chloride test and PCR came out positive. Infant was diagnosed to be suffering with cystic fibrosis and cytomegalovirus infection. CBC report revealed severe anemia and thrombocytopenia.

Since patient’s weight was less than 3rd percentile on WHO growth chart, he was found to be suffering from severe faltering growth.

Management:

Cytomegalovirus infection was managed using Valganciclovir and cystic fibrosis was managed using nutritional management. Aim of the nutritional management was to achieve satisfactory weight gain. In addition to that normal growth and development of infant was desired.

Infant was hospitalized for period of 10 days. He was started on breastfeeding and energy and nutrient dense formula (ENDF) top ups. Around 50ml/kg/day of ENDF were given every 3 hourly. Total 7-8 feeds were given per day. Infant tolerated the formula well and started gaining weight by day 6 of hospital stay. Infant gained 500 g of weight over a 10 days period making his weight 3.4 kg at the time of discharge.

Follow-up:

ENDF was continued post discharge along with breastfeeding. Infant was started on weaning food from 5th month of age. Especial diet was advised for managing cystic fibrosis.

Infant gained 2.2 kg of weight making his weight 5.6 kg at 6 months. His growth and development improved significantly. Infant’s height was 55 cm, head circumference was 40 cm, and mid upper arm circumference was 10 cm. He was able to hold neck and turn over.

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Case Study 8 figure 2

Discussion:

Cytomegalovirus, a member of the human herpesvirus family, is the most common viral cause of congenital infection, affecting 0.2–2.2% of all live births. It is responsible for significant morbidity, especially in infants who are symptomatic in the neonatal period.6 Early cytomegalovirus infection, namely in utero or during early infancy, may contribute to reduced growth, altered or impaired immune functions, and sensory and cognitive deficits.7

Cystic fibrosis causes thickening of mucous that primarily affects the pulmonary and gastrointestinal systems. Thickening of the airway mucous blocks gas exchange and provides a growth medium for bacteria, which then leads to airway inflammation and infection, causing permanent lung damage, chronic sinus problems, or both. In the gastrointestinal system, the thickened mucous decreases pancreatic enzyme secretion into the intestine and absorption of nutrients, causing problems with malabsorption that can lead to malnutrition.8

Cystic fibrosis is strongly associated with poor nutritional status linked directly by factors related to the underlying genetic mutation, as well as indirectly by factors such as higher energy needs, energy losses, greater essential fatty acid turnover, and decreased nutrient intake and absorption.9

In infants with cystic fibrosis, poor nutritional status results in stunted growth, as detected by low weight- and height for- age percentiles. If untreated, such cystic fibrosis-related undernutrition in infancy can lead to the serious consequence of impaired cognitive function. In cases of severe undernutrition in infants, lung function worsens markedly, and survival is poor.9

Initiation of nutritional management should begin as early as possible after diagnosis. Energy intake should be increased for breastfed infants who have poor weight gain despite efforts to optimize pancreatic enzyme replacement therapy by providing more frequent feedings and fortifying expressed breast milk. For formula fed infants, energy and protein intake can be increased by using high-energy/protein infant formula.9 Energy and nutrient dense formula (ENDF) may help in weight gain in infants with faltering growth. In a study by Clarke SE et al., all 49 infants [median age of 5 weeks (range 2-31 weeks)] with faltering growth gained weight with ENDF. Intake of ENDF also resulted in significantly greater intakes of all nutrients, protein, sodium, potassium, calcium, zinc, iron, vitamin D, vitamin C and vitamin A.10

In the case presented here, infant was showing faltering growth due to cystic fibrosis and cytomegalovirus infection. He was unable to gain weight with breast feeding and regular formula feeding. Infant therefore started on ENDF along with breastfeeding. ENDF led to satisfactory weight gain. Use of ENDF can be considered as one of the important measures in infants who are not thriving well.

Key features of ENDF:
  • Highest energy in small volume - 1kcal/1ml
  • 15-14% more vitamin and minerals than standard infant formula
  • Optimal protein: 2.6g/100 ml
  • Fortified with nucleotides
  • Osmolality within recommended guidelines
Conclusion:

We can conclude that ENDF can help in managing infant with faltering growth. Nutritional management is of utmost importance in any failure to thrive case. In infants less than 6 months, where solids are not an option, giving ENDF would be of great benefit.

References:-

  1. Walter-Nicolet E, Leblanc M, et al. Congenital cytomegalovirus infection manifesting as neonatal persistent pulmonary hypertension: report of two cases. Pulm Med. 2011;2011:293285

  2. Kulkarni H, Kansra S, et al. Cystic fibrosis revisited. J Postgrad Med. 2019 Oct-Dec;65(4):193-196

  3. Hankard R, Munck A, Navarro J. Nutrition and growth in cystic fibrosis. Horm Res. 2002;58 Suppl 1:16-20.

  4. Barni GC, Forte GC, et al. Factors associated with malnutrition in adolescent and adult patients with cystic fibrosis. J Bras Pneumol. 2017 Sep-Oct;43(5):337-343

  5. Isa HM, Al-Ali LF, Mohamed AM. Growth assessment and risk factors of malnutrition in children with cystic fibrosis. Saudi Med J. 2016 Mar;37(3):293-8.

  6. Khalil A, Heath P, Jones C, Soe A, Ville YG on behalf of the Royal College of Obstetricians and Gynecologists. Congenital Cytomegalovirus Infection: Update on Treatment. BJOG. 2018 Jan;125(1):e1-e11.

  7. Filteau S, Rowland-Jones S. Cytomegalovirus Infection May Contribute to the Reduced Immune Function, Growth, Development, and Health of HIV-Exposed, Uninfected African Children. Front Immunol. 2016 Jun 30;7:257

  8. Pitts J, Flack J, Goodfellow J. Improving nutrition in the cystic fibrosis patient. J Pediatr Health Care. 2008 Mar-Apr;22(2):137-40

  9. Turck D, Braegger CP, et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 2016 Jun;35(3):557-77.

  10. Clarke SE, Evans S, et al. Randomized comparison of a nutrient-dense formula with an energy-supplemented formula for infants with faltering growth. J Hum Nutr Diet. 2007 Aug;20(4):329-39.

Important Notice:

The World Health Organization (WHO)* has recommended that pregnant women and new mothers be informed of the benefits and superiority of breast-feeding, in particular, the fact that it provides the best nutrition and protection from illness for babies. Mothers should be given guidance on the preparation for and maintenance of lactation, with special emphasis on the importance of the well-balanced diet both during pregnancy and after delivery. Unnecessary introduction of partial bottle feeding or other foods and drinks should be discouraged since it will have a negative effect on breast-feeding. Similarly mothers should be warned of the difficulty of reversing a decision not to breastfeed. Before advising a mother to use an infant formula, she should be advised of the social and financial implications of her decision. For example, if a baby is exclusively bottle-fed, more than one can (500g) per week will be needed, so the family circumstances and cost should be kept in mind. Mother should be reminded that breast milk is not only the best but also the most economical food for babies. If a decision to use infant formula is taken, it is important to give instruction on correct preparation methods, emphasizing that unboiled water, unsterilized bottles or incorrect dilution can lead to illness.

*See: International Code of Marketing of Breast Milk Substitutes, adopted by the World Health Assembly in Resolution WHA 34.22, May 1981.

Mothers should be explained the following advantages & nutritional superiority of breastfeeding:

  1. Immediately after delivery, breast milk is yellowish and sticky. This milk is called colostrum, which is secreted during the first week of delivery. Colostrum is more nutritious than mature milk because it contains more protein, more anti-infective properties which are of great importance for the infant's defence against dangerous neonatal infections. It also contains higher levels of, Vitamin 'A'.
  2. Breast miIk:
    1. Is, a complete and balanced food and provides all the nutrients needed by the infant (for the first six months of life).
    2. Has anti-infective properties that protect the infants from infection in the early months.
    3. Is always available.
    4. Needs no utensils or water (which might, carry germs) or fuel for its preparation.
  3. Breastfeeding is much cheaper than feeding infant milk substitutes as the cost of the extra food needed by the mother is negligible compared to the cost of feeding infant milk substitutes.
  4. Mothers who breast-feed usually have longer periods of infertility after child birth than non-lactators.

Details of management of breast feeding, as under:

  1. Breast-feeding.
    1. Immediately after delivery enables the contraction of the womb and helps the mother to regain her figure quickly.
    2. Is successful when the infant suckles frequently and the mother wanting to breast-feed is confident in her ability to do so.
  2. In order to promote and support breast-feeding the mother's natural desire to breast feed should always be encouraged by giving, where needed, practical advice and making sure that she has the support of her relatives.
  3. Adequate care for the breast and nipples should be taken during pregnancy.
  4. It is also necessary to put the infant to the breast as soon as possible after delivery.
  5. Let the mother and the infant stay together after the delivery, the mother and her infant should be allowed to stay together (in hospital, this is called "rooming-in").
  6. Give the infant colostrum as it is rich in many nutrients and anti-infective factors protecting the infants from infections during the few days of its birth.
  7. The practice of discarding colostrum and giving sugar water, honey water, butter or other concoctions instead of colostrum should be very strongly discouraged.
  8. Let the infants suckle on demand.
  9. Every effort should be made to breast-feed the infants whenever they cry.
  10. Mother should keep her body and clothes and that of the infant always neat and clean.
    Breast-feeding is the best form of nutrition for babies and provides many benefits to babies and mothers. It is important that, in preparation for and during breast-feeding, you eat a healthy, balanced diet. Combined breast and bottle feeding in the first weeks of life may reduce the supply of your own breast-milk, and reversing the decision not to breast-feed is difficult.
    Always consult your Health-care Professional for advice about feeding your baby. The social and financial implications of using infant formula should be considered. Improper use of an infant formula or inappropriate foods or feeding methods may present a health hazard. If you use infant formula, you should follow manufacturer's instructions for use carefully - failure to follow the instructions may make your baby ill.