Dietary Disasters in the Management of GI Disorders

1. Dietary Disasters in the Management of GI Disorders NASPGHAN Annual Meeting New Orleans, LA October 23, 2010 Frank R. Sinatra, M.D. Professor of Pediatrics Keck School of Medicine University of Southern California

2. Why Do Dietary Disasters Occur in the Management of Infants with Gastrointestinal Symptoms? • Infants are at increased risk to suffer nutritional consequences when inadequate and/or inappropriate diets are substituted for breast milk, standard infant formulas and appropriate solid foods • High nutritional requirements for normal growth and development • Low nutritional reserves • Infants are dependent on others to make nutritional decisions • Poor understanding of infant nutrition by some parents and/or medical providers • Common occurrence of GI symptoms in infancy (vomiting, diarrhea, constipation, colic) and the common beliefs that all GI symptoms are due to dietary intake and can, therefore, be treated by dietary manipulations.

3. Iatrogenic Kwashiorkor • A 7 mo-old girl presents with marked edema • Hx: • 2.9 kg product of a FT, uncomplicated pregnancy • Grew and developed normally on a cow milk-based formula until 4 mo of age when she began to have intermittent episodes of vomiting and diarrhea • A diagnosis of cow-milk allergy was made and she was tried on multiple formulas without changes in symptoms • At 5 ½ mo of age she was placed on a non-dairy creamer as a replacement for her formula. Her only other intake consisted of <60g/day of either cereal, fruits or vegetables. • At 7 mo of age she was noted to be markedly edematous and was referred to Childrens Hospital Los Angeles for “r/o nephrotic syndrome”

4. Iatrogenic Kwashiorkor • PE: • Pale, irritable infant with generalized edema • Weight: 8.1 kg Length: 68 cm -both 50th-75th %tile • Skin: generalized pallor but no rashes, hair-nl • Abdomen: moderately distended; liver palpable 6 cm below the right costal margin; spleen not palpable. • Extremities: grossly edematous • Initial Laboratory Studies: • Marked hypoproteinemia, elevated AST/ALT, total and direct bilirubin • Urinalysis-normal (negative for protein) • Serum electrolytes, sweat chloride and a-1-antitrypsin concentrations – normal • Negative serology for congenital infection and hepatitis B.

5. Iatrogenic Kwashiorkor • Hospital Course: • Patient was placed on a cow milk-based formula with added solids • She tolerated her diet well without emesis or diarrhea and after 7 days her serum albumin had improved and her edema was markedly decreased • She continued to have hepatomegaly, and elevation of her serum bilirubin and transaminases • A liver biopsy was performed on the 14th day of hospitalization

7. Iatrogenic Kwashiorkor • Follow-up: • During the remainder of the hospital course she had progressive resolution of her edema, hyperbilirubinemia and elevated transaminases • She was discharged after a 25 day hospitalization on cow milk-based formula with added solids consisting of cereal, fruits, meat and vegetables • On follow-up examination 3 weeks following discharge she was asymptomatic and without edema; the liver was palpable 3 cm below the right costal margin; laboratory studies revealed a serum bilirubin of 1.0 mg/dL and AST/ALT of 40 and 52. • She continued to grow and develop normally with no evidence of cow milk-protein sensitivity.

8. Iatrogenic Kwashiorkor – Summary of Clinical and Laboratory Findings Modified from: Sinatra, et al Am J Dis Child 1981;135:21-23

10. Nondairy Creamer Caloric Distribution and Source * Caloric Density: 133 kcal/dL Mocha Mix: Presto Food Products, Inc Los Angeles, CA Note: Product information as of January 1981 (during time patients were seen)

11. Low protein intake • Continued insulin secretion Diet: Adequate Calories: 59% fat, 30% carbohydrate (corn syrup) Low Protein: 1% of calories (soy protein) • Insulin stimulates uptake of • branched chain amino acids • (valine) by muscle • Decreased hepatic • protein synthesis due • to inadequate precursor • essential amino acids • Decreased b- lipoprotein • synthesis • Hypoalbuminemia • Edema • Hepatic steatosis • Elevated AST/ALT • Cholestasis

12. Malnutrition With the Use of an Inappropriate “Formula” • 5-month old male was admitted to the hospital following 3 weeks of intermittent fever, coughing, poor feeding, vomiting and diarrhea • PMH: Born at term (BW: 4.1 kg), breast fed for 1 week then switched to a formula of barley water, corn syrup and whole milk in order to “prevent stomach problems” • PE: Wasted appearing infant Weight: 5.1kg (<5th %tile) Height:68 cm (75th %tile) Bilateral otitis media. Decreased breath sounds left chest.

13. Malnutrition With the Use of an Inappropriate “Formula” • Laboratory Studies: • WBC 28,200 (43% polys, 28% band forms) • Hgb 10.2, Na 137, K 3.9, Cl 101, Bicarb 17 • Total protein 5.9, Albumin 3.3 • Chest X-ray – Left lower lobe pneumonia • Lumbar puncture, stool cultures and sweat chloride - normal

14. Malnutrition With the Use of an Inappropriate “Formula” • Hospital Course: • Otitis media and pneumonia responded well to antibiotic therapy • After intravenous fluid therapy he was started on a standard cow’s milk containing formula and solids • He tolerated the feedings well and gained 740 g during a 16-day hospitalization • Follow-up: • Outpatient evaluation at 7 months of age – weight was 6.92 kg (5th-10th %tile). He was tolerating his formula and age-appropriate solids well

16. Malnutrition With the Use of an Inappropriate “Formula” Modified from: Fabius, et al Am J Dis Child 1981;135: 615-617

17. Barley Water “Formula” • “This formula is most like human milk … 15 oz of barley water (made by boiling barley in water for several hours until there is a thick, pink syrup), 10 oz of homogenized milk, 3 oz Karo syrup…” (L.R. Hubbard “Processing a New Mother,” Ability, 1959, vol 89, pg 1). • Honey may be substituted for the corn syrup (L.R. Hubbard, Flag Information Letter No. 43, 1977) As quoted in: Fabius, et al Am J Dis Child 1981;135:615-617

18. Barley Water “Formula” • Problems: • Low Fe, Vitamin A and Vitamin C content • High osmolality • Corn syrup formulation: 405 mOsm/kg • Honey formulation: 1,056 mOsm/kg • Questions: • Chronic low Fe, Vit C and Vit A intake contributed to infections requiring hospitalization? • Poor GI tolerance during illness due to high osmolality? Contribution to poor weight gain? Fabius, et al Am J Dis Child 1981;135: 615-617

19. Malnutrition Following Restricted Diet • History: • A 7 month old male is admitted for marasmus • 3.3 kg product of a full-term uncomplicated pregnancy, labor and delivery to a 16 year old mother • He tolerated a standard infant formula for the first 2 months of life with appropriate growth, weight gain and development (per records from primary care provider at a local clinic). • At 2 mo. of age he had the acute onset of fever, emesis and diarrhea. He was treated with oral rehydration therapy with resolution of the fever and emesis after 36 hours. He continued to have 3-4 soft to loose stools per day. • Mother was instructed to continue on the oral rehydration solution “until his stools are normal”.

20. Malnutrition Following Restricted Diet • Over the next 5 months his mother attempted to re-introduce multiple infant formulas and made several attempts to start solids. His stools remained unchanged. The infant was seen in several clinics and emergency rooms and on each occasion she was told to stop the formula and solids until the “diarrhea” resolved • At 7mos of age she took the infant to a pediatrician who referred him for admission and treatment of marasmus

21. Malnutrition Following Restricted Diet • Physical Examination: • Length 60 cm (<3rd %tile) • Weight 4.9 kg (<<3rd %tile) • Head Circumference 41 cm (<3rd %tile) • Severely malnourished infant with poor head control and generalized increased tone. Unable to sit without support. Poor interaction with surroundings and mother.

23. Malnutrition Following Restricted Diet • Hospital Course and Follow-up: • An extensive evaluation for genetic, infectious, endocrine, metabolic and primary gastrointestinal disorders was unremarkable • He was slowly started on a standard infant formula and was able to tolerate full volume and caloric requirements by the 7th hospital day. Age-appropriate solids were added and during a 4- week hospitalization he had an average daily weight gain of 48g/day • By 2 years of age he was growing at the 10th %tile for height, weight and head circumference. He was able to walk without support but had only a 5-word vocabulary. • At age 5 he was able to start a regular kindergarten program but has subsequently required a special education program and has been treated for speech delay and ADHD.

24. Final Comments • These cases represent extreme examples of inappropriate diets in infancy resulting in acute and potentially long term nutritional complications • In each case inappropriate diets were used to treat or prevent real or perceived gastrointestinal disorders. • In each case dietary advice was provided by individuals with inadequate training/knowledge of infant nutrition • Gastroenterologists need to continue to educate both parents and primary care providers of the limitations and potential long term complications of dietary management of GI symptoms in infants and the importance of providing adequate amounts of breast milk or a standard infant formula. • Infants who are truly unable to tolerate breast milk and/or an appropriate infant formula due to GI symptoms should be evaluated by a pediatric gastroenterologist.