Fetal Distress and Hypoxia and Low Apgar Scores

Infants who have been compromised in utero or during delivery because of insufficient placental reserve, cord accidents, or other causes of intrauterine hypoxia have very low Apgar scores at birth and need special treatment. An asphyxiated infant cannot be fed for at least 48 hours, and, depending on associated findings, it may be 96 hours or more before it is safe to put food in the gastrointestinal (GI) tract, which has been poorly perfused during the hypoxia. The infant must be maintained on IV fluids. If the mother is to breastfeed or donor milk is available, human milk can be started sooner. Her colostrum will be valuable to the infant and will be better tolerated by the infant’s intestinal tract, which has usually suffered hypoxic damage in these circumstances. Small amounts of colostrum can be given in 24 hours. Hypoxia decreases the motility of the gut and decreases stimulating hormones. The colostrum should be pumped and become the first oral feedings drop by drop.

Mothers will need help initiating lactation and understanding the pathophysiology of the infants’ disease. These infants often have a poor suck that does not coordinate with the swallow, making nursing at the breast and bottle equally difficult. The mother may need to hold her breast in place and hold the infant’s chin as well. These infants are especially susceptible to “nipple confusion,” so means of sustaining nourishment other than a bottle should be sought. Cup feeding has been well tolerated using a soft plastic one-ounce medicine cup. Even infants who will not be breastfed but feed poorly from a bottle for neurologic reasons will do better with a cup. ^{, ,} Weaning slowly from the IV hyperalimentation fluids while introducing breastfeeding is helpful. Using a dropper and employing the nursing supplementer are options if milk supply from the breasts is low. These infants may continue to feed poorly for neurologic reasons. They do not do better with a bottle. If the mother is taught to cope with the problem, nursing should progress satisfactorily. She may always need to hold her breast in place, which would be the best evidence of residual damage from the hypoxia.

Infants can be held in positions that may help an individual baby adapt better. The “football hold” is a popular but poorly named position in which an infant is held close to the mother’s body with the feet to her side. The head and face are squarely in front of the breast and steadied by the mother’s arm and hand on that side. Cupping the breast and the jaw in one hand facilitates the infant’s seal around the breast with the mouth ( Figure 14-1 ). This position has been called the “dancer hold.” One of the most valuable suggestions is the use of a sling or pleat-seat to hold an infant’s body in a flexed position, thus giving the mother both hands free to hold the head and the breast in position for feeding ( Figure 14-2 ).

Dancer hold. A, Hand position of mother. B, Infant in position at breast with support.

(From McBride MC, Danner SC: Sucking disorders in neurologically impaired infants: assessment and facilitation of breastfeeding, Clin Perinatol 14:109, 1987.)

Pleat-seat or sling baby carrier holds the infant in a flexed position that facilitates infant suckling, leaving the mother’s hands free to support her breast and the infant.

(Redrawn from McBride MC, Danner SC: Sucking disorders in neurologically impaired infants: assessment and facilitation of breastfeeding, Clin Perinatol 14:109, 1987.)

Pacing the feedings and pumping after feedings will increase a mother’s milk supply when the infant is unable to suck vigorously enough. Giving the pumped milk by lactation supplementer, small cup, or dropper ensures proper weight gain in the early weeks. Holding an infant in a flexed position that mimics the fetal position relaxes an infant who is hypertonic or arching away from the breast.

In a study of energetics and mechanics of nutritive sucking in preterm and term neonates, Jain et al. compared 38-gestational-week infants with 35-gestational-week infants and noted that preterm infants use less energy to suck the same volume of milk. The preterm infant took only up to 0.5 mL per suck and generated lower pressures and a lower frequency.

Exploring the hypothesis that milk flow achieved during feeding contributes to ventilatory depression during rubber-nipple feeding, Mathew compared nipples with different flow rates. Decreases in minute ventilation and breathing frequency were significantly greater with high-flow nipples, thus confirming that milk flow influences breathing in premature infants who are unable to self-regulate the flow.

Tracings were made from the first oral feeding to time of discharge in term and premature infants. Serial oxygen pressure values showed small undulations across baseline (above and below) while breastfeeding. Substantial dips while bottle feeding were shown with recovery, but not above baseline. The quality and quantity of variation were different in the two modes of sucking (i.e., breast or bottle), with large drops in oxygen saturation occurring during actual sucking of the bottle but only during burping or repositioning while breastfeeding. Meier ^, concludes that the findings do not support the widely held view that breastfeeding is more stressful. The comparative data suggest that both pacifier and bottle feeding are more stressful than suckling at the breast. For further discussion of the stress of breastfeeding versus bottle feeding see Chapter 15 , feeding the 28 to 32 week premature infant. If an infant has significant motor tone disabilities or lacks the usual oral reflexes in response to stimulus of the rooting and sucking reflexes, a neonatal neurologist should assess the infant before any routine exercises are initiated.

It has been suggested that perioral stimulation enhances an immature or neurologically impaired infant’s ability to suck and to coordinate suck and swallow. Perioral stimulation, consisting of stimulating the skin overlying the masseter and buccinator muscles by manually applying a quick-touch pressure stimulus lasting 1 second, was studied. This is accomplished by simultaneously squeezing the buccal fat of both cheeks. Suck-monitoring equipment revealed that perioral stimulation increased the sucking rate, suggesting that this may facilitate sucking. Exercising the mouths of infants who already have excessive mouth stimulation may not be appropriate. Many infants in a neonatal intensive care unit (NICU) are being suctioned, tube fed, and orally stimulated for other reasons, which may lead to oral aversion.

Kangaroo care is recommended for full-term infants who are neurologically or metabolically impaired. It involves holding the infant skin to skin inside the parent’s shirt. It can stabilize temperature, respirations, and heart rate and be neurologically calming. For a mother who is to breastfeed, it facilitates milk production and helps a mother learn to handle her infant. Kangaroo care is further discussed in Chapter 15 .

Galactagogues: Medication-Induced Milk Production When Pumping

Stimulating milk production pharmacologically in mothers of LBW infants who are pumping to provide milk for their infants has been recommended by several authors, as reported by Ehrenkranz and Ackerman. They used 10-mg metoclopramide orally every 8 hours for 7 days, tapering during 2 days more. Milk production increased within 2 days, but after therapy decreased, milk production decreased. Prolactin levels also increased during the treatment. Extensive use (more than 2 weeks) may cause cardiovascular symptoms in the mother.

Improved lactation occurred in 67% of mothers with no breast milk at onset and in 100% of mothers with poor supply given metoclopramide (10 mg three times per day for 10 days) by Gupta and Gupta. They reported that the improvement persisted when the drug was discontinued. None of the 32 women had any symptoms or side effects. This drug is a substituted benzamide, which has selective dopamine-antagonist activity.

Although growth hormone has been observed to enhance milk supply, no recommended protocol exists for its clinical use. In one study, 20 healthy mothers with insufficient milk who delivered between 26 and 34 weeks were given growth hormone, 0.2 international units/kg/day subcutaneously for 7 days. A group of 10 mothers received a placebo. Milk volume increased in the treated mothers. No change was noted in plasma growth hormone levels, but an increase was seen in insulin-like growth factor. No other changes were noted during this short-term therapy.

Other drugs have been noted to enhance milk production. Domperidone (Motilium) is currently unavailable in the United States because the FDA banned its distribution. It is widely available in Canada, Europe, and Australia. It is fully discussed in Chapter 12 . A dosage of 10 mg three times per day is reported to increase milk supply in some women. The drug is not without side effects, however. Other galactagogues are discussed in Chapter 12 .

Buccal Smears in Breastfeeding Infants

Guidelines for buccal smear collection in breastfed infants should be followed when genetic review is indicated. A buccal smear is a noninvasive, fast, and relatively inexpensive diagnostic method for collecting genetic material. It is used for sex determination as well as aneusomy, microdeletion syndromes, and a variety of polymerase chain reaction-based molecular genetic tests. Maternal cells can contaminate smears taken from breastfed infants. The recommendation is to wait at least 1 hour after a feeding. Buccal mucosa should be cleansed thoroughly with a cotton swab applicator. These procedures apply to both neonates and older nursing children.

Gastrointestinal Disease

Bouts of diarrhea and intestinal tract disease are less common in breastfed infants than in bottle-fed infants, but when they occur, the infant should be maintained on the breast if possible. ^, Human milk is a physiologic solution that normally causes neither dehydration nor hypernatremia. Occasionally, an infant will have diarrhea or an intestinal upset because of something in the mother’s diet. It is usually self-limited, and the best treatment is to continue to nurse at the breast. If a mother has been taking a laxative that is absorbed or has been eating laxative foods, such as fruits, in excess, she should adjust her diet. Intractable diarrhea should be evaluated as it would be in any infant. Allergy to mother’s milk is extremely rare and would require substantial evidence to support the diagnosis. Allergy to a foreign protein passed into the milk, such as bovine β-globulin, as in cow milk, however, can cause severe allergic symptoms in an infant (see Chapter 17 ).

Colitis While Breastfeeding

Severe colitis in a totally breastfed infant, usually with onset in the neonatal period, suggests an intrinsic metabolic disorder in the infant or an exquisite intolerance to something in mother’s milk, such as cow milk protein. Six infants with protein-induced enterocolitis presenting in the first month of life with severe bloody diarrhea responded to weaning and use of hydrolyzed protein formula. Other cases have been reported, requiring long periods of hyperalimentation and utilization of special formulas such as Nutramigen.

Induced colitis in infants is usually caused by some dietary insult, such as exposure to cow milk. ^, It has been reported in breastfed infants, most of whom responded to removal of cow milk from the maternal diet. Several had been given formula at birth, which is believed to have sensitized them. The symptoms included bloody diarrhea, and sigmoidoscopy revealed focal ulcerations, edema, and increased friability of the intestinal mucosa. On relief of symptoms by dietary change, the intestinal tract biopsy returns to normal. Removal of all bovine protein, not just cow milk, from the mother’s diet may be required to ensure recovery while returning to breastfeeding. It may take 10 to 14 days to clear the bovine protein from the mother’s milk.

A prospective study examined 35 consecutive infants who had fresh blood mixed with stools at approximately 4 weeks of age. The infants were otherwise asymptomatic and had no infection, bleeding diathesis, or necrotizing enterocolitis (NEC); 31 had histopathologic evidence of colitis characterized by marked eosinophilic infiltrate (more than 20 eosinophils per high-power field) compared with control subjects and low mean serum albumin. Ten of these 31 were exclusively breastfed, nine were fed cow milk formula, nine soy formula, two mixed breast milk and formula, and one Nutramigen. The low serum albumin and high peripheral eosinophil count suggested the diagnosis of allergic colitis. All cases cleared with dietary change. The breastfed infants were weaned, unfortunately, and not managed by dietary adjustment in the mother in this series.

Protein-induced colitis can follow a benign course with proper treatment. Israel et al. studied 13 infants with blood from the rectum, negative stool cultures, and colonoscopic and histologic evidence of colitis. The infants were all less than 3½ months of age, and six were breastfed and five had been supplemented. All were gaining weight well. The mothers of the breastfed infants restricted cow milk in their diet, and the infants were able to return to exclusively breastfeeding. All recovered.

Dietary protein-induced proctocolitis in exclusively breastfed infants should be taken into consideration as a cause of rectal bleeding or blood-streaked stool in the neonatal period and early infancy (hematochezia). Benign eosinophilic proctocolitis diagnosed by colonoscopy the is best treated by the exclusion of the allergen from the mother’s diet. Resolution has taken place within 72 to 96 hours of elimination of the offending protein so temporarily stopping breastfeeding may not be necessary in some cases.

An 8-week-old infant boy presented with irritability and projectile vomiting for an ultrasound to rule out pyloric stenosis. The ultrasound revealed colitis, and further history revealed bloody stools. He responded to removing bovine protein from his mother’s diet and continuing to breastfeed.

Harmon et al. described a case of perforated pseudomembranous colitis in a breastfed infant. Other cases had been associated with giving antibiotics to an infant. The infant’s stool was Clostridium difficile toxin positive, and the child required bowel resection for abscess and perforation. The mother had taken ciprofloxacin without consulting a physician for days before the infant’s admission.

The Lactation Study Center has been notified of other cases of bloody diarrhea with a diagnosis of colitis that did appear to respond to maternal dietary restrictions. One infant showed brief improvement when all cow milk products were removed from the mother’s diet and then had a relapse. Removing all bovine (both meat and milk) products from the maternal diet resulted in recovery without relapse with exclusive breastfeeding. In retrospect the mother recalled switching from a vegetarian diet to high meat, especially beef, intake throughout pregnancy.

A case of fucose intolerance is reported in a breastfed infant who was not intolerant of lactose but of the by-product of the oligosaccharides in human milk, passing large amounts of fucose in the stool. The infant tolerated Pregestimil and then was weaned to regular formula.

It has been recommended by Haight that severe cases of allergic colitis and also severe GI colic can be alleviated by treating the mother with pancreatic enzymes, 25 mg three times per day. It is safe for the mother and often dramatic for the infant. This is especially effective when eliminating cow protein has not solved the problem.

A formal study of this therapy was reported by Repucci who described four term infants who were exclusively breastfeeding between 1 and 3 months of age who had positive family history for atopy. Elimination of bovine protein had not relieved the blood in the stools. Mothers were prescribed pancreatic enzymes (Pancrease MT4 USP units: 4000 lipase, 12,000 amylase, and 12,000 protease), two capsules with each meal and one capsule for snacks. Blood cleared within 2 days. One mother had to increase the dose to three capsules per meal and two with snacks. Mothers experienced no side effects due to this therapy. Anecdotal reports continue to confirm this therapy.

The management of protracted diarrhea in infants never breastfed is reported by many human milk banks on a case-by-case basis. Eleven of 24 children managed by MacFarlane and Miller in a hyperalimentation referral unit recovered when fed banked human milk orally without protracted IV therapy. All the infants had been tried on all the available special formulas first. A study of oral rehydration in 26 children younger than the age of 2 years showed that the children who continued to breastfeed while receiving rehydration fluid had fewer stools and recovered more rapidly than those receiving only rehydration fluid. The Pima Infant Feeding Study clearly showed that in less developed and more disadvantaged communities in the United States, exclusive breastfeeding protected against severe diarrhea and other GI disorders.

Lactose Intolerance

Suckling milk is the defining characteristic of mammals. Lactose, the major carbohydrate in milk, is hydrolyzed by lactase-phlorhizin hydrolase, an enzyme of the small intestine. Lactase plays a critical role in the nutrition of mammalian neonates. Congenital lactase deficiency, present from birth, is extremely rare and is inherited as an autosomal recessive gene. Most humans (except Northern Europeans) and other adult mammals do not drink milk beyond infancy; it causes indigestion and mild to severe GI symptoms because of an adult’s inability to digest lactose. Low lactase levels result from injury or genetic expression of lactase. The enzyme hydrolyzes lactose, phlorhizin, and glycosyl ceramides. A decline in lactase-specific activity occurs at the time of weaning in most mammalian species. In humans it may occur as early as 3 to 5 years of age; in other species the elevated juvenile levels of lactase-specific activity persist. The developmental patterns of lactase expression are regulated at the level of gene transcription.

Premature infants and those recovering from severe diarrhea have transient lactose intolerance. The only treatment is a temporary lactose-free diet. Reports of lactose-hydrolyzed human milk suggest that banked human milk can be treated with lactase (Keralac), which will hydrolyze the lactose (900 enzyme activity units to 200 mL breast milk degraded 82% of the lactose). In one case the reason for using human milk was that the infant became infection prone when he was weaned from the breast at the time the initial diagnosis was made. He showed marked improvement with treated human milk. In a breastfed infant, lactase deficiency may be manifest by chronic diarrhea and marked failure to thrive.

An additional clinical syndrome related to slow gaining or failure to thrive is excessive lactose, resulting when the fat level in the milk is low and an excessive amount of milk is consumed because of the low-calorie content. The first documented case was reported by Woolridge and Fisher. Lactose production drives the milk-making capacity. When a feeding at one breast does not last long enough for the fat to let down, the result is low-calorie high-lactose milk. The authors recommend in such cases that an entire feeding be taken at one breast. (For further discussion of this phenomenon, see Chapter 8 .)

Celiac Disease, Crohn’s Disease, and Inflammatory Bowel Disease

Some chronic diseases are better controlled by keeping an infant on breast milk, as symptoms usually become more severe with weaning. If an infant is weaned and does poorly on formula, relactation of the mother should be considered. With the availability of the nursing supplementer, this possibility is no longer remote (see Chapter 19 ).

Celiac disease or permanent gluten-sensitive enteropathy is an immunologic disease dependent on the exposure to wheat gluten or related proteins in rye and barley.

A case-control study was done on the effect of infant feeding on celiac disease to investigate the association between duration of breastfeeding and age at first gluten introduction into the infant diet and the incidence and age of onset of celiac disease. A significant protective effect on the incidence of celiac disease was related to the duration of breastfeeding after 2 months. It was not related to the age of first gluten in diet, although the age of first exposure did affect the age of onset of symptoms.

The risk for celiac disease was reduced in children younger than 2 years old in a study of 2000 Swedish children if they were still being breastfed when dietary gluten was introduced. The effect was more pronounced if breastfeeding continued after gluten was introduced. The authors conclude that gradual introduction of gluten-containing foods into the diet while breastfeeding reduces the risk for ever getting celiac disease. The declining incidence of celiac disease and transient gluten intolerance has been associated with changing feeding practices, which include later introduction of dietary gluten, the use of gluten-free foods for weaning (rice), and the increased initiation and duration of breastfeeding.

The risk for celiac disease autoimmunity and timing of gluten introduction into the diet of infants at increased risk for the disease was determined by Norris et al. who studied 1560 children prospectively. They had been determined to be at increased risk because they possessed either HLA-DR3 or DR4 alleles or had a first-degree relative with type 1 diabetes. Diagnosis of celiac disease was based on positive small bowel biopsy and positive for tissue transglutaminase autoantibody. Children exposed to gluten in the first 3 months of life or not until after 7 months of age developed the disease; 4 to 6 months of age appeared to be a safe period when gluten was tolerated. Breastfeeding may offer protection against the development of celiac disease. Breastfeeding during the introduction of gluten in the diet (wheat, barley, or rye) and increasing the duration of breastfeeding was associated with reduced risk for developing the disease, as reported by Akobeng et al. who did a systematic review and meta-analysis.

The discussion is not over and the guidelines are not confirmed. It is agreed that celiac disease is an immune-mediated disease that is not uncommon. An estimated 1% of the population is affected. It negatively influences the quality of life of affected individuals. Prevention strategies focusing on early infant feeding practices (i.e., breastfeeding) and timing of introduction of gluten into the infant’s diet have had conflicting results. Large multiple country prospective studies are underway. Although breastfeeding in multiple smaller studies has shown to be protective, there may prove to be limitations to that. Because risk can be anticipated by family history and testing for HLA-DQ2 or HLA-DQ8, dietary management appears to at least postpone the onset of symptoms if not prevent it.

A window of opportunity has been suggested to reduce the risk of celiac disease by introducing gluten no sooner than 4 months and no later than seven months of age and exclusive breastfeeding until 12 months of age but not beyond. In progress is a multicentered, randomized, double blind, placebo controlled dietary intervention study involving 944 HLA positive children; the Norwegian mother and child cohort study of 324 cases out of a cohort of 82,167, and a systematic review of available data from a large randomized controlled trial in 10 European countries.

The current consensus is that gluten should be added to the diet between 4 and 7 months and breastfeeding should continue until 12 months in at-risk infants. The AAP states that breastfeeding reduces the risk of celiac disease by 52%.

A family with two sons at ages 33 months and 8 months came to the attention of the Lactation Study Center. Both were breastfed. They developed an inability to sleep comfortably after having slept well previously. They cried and thrashed about, needing constant attention and motion around the clock. At age 27 months, the older son had x-rays, biopsies, and genetic testing. Endoscopy was inconclusive with moderate inflammation and smoothing. He was positive for one of the three genetic markers for celiac disease. He had been weaned at 18 months and was started on a gluten-free diet, which “cured” him. At the center, the mother was recommended a gluten-free diet while she continued to breastfeed the 8-month-old child. In 48 hours he was remarkably improved and is now symptom free, breastfeeding, and eating gluten-free solids. With the availability of gluten-free foods in supermarkets, the diet for mothers is more accessible. The public interest in gluten-free food has increased and many are trying it at random.

The development of Crohn’s disease later in life has increased in recent decades. Because it has been suggested that breast milk is essential for the development of the normal immunologic competence of the intestinal mucosa, investigators have studied the association between breastfeeding and later Crohn’s disease. Bergstrand and Hellers studied 826 patients who developed Crohn’s disease between 1955 and 1974 and their matched control subjects. Mean length of breastfeeding was 4.59 months among patients and 5.76 among control subjects ( p < 0.01). Patients with Crohn’s disease were overrepresented among those with no, or short, periods of breastfeeding. The role of infant feeding practices in the development of Crohn’s disease in childhood was reported by Koletzko et al. in a study of 145 families with similar results. Although Crohn’s disease may develop in genetically susceptible people as a result of an immunologic response to unidentified antigen in the mucosa, early feeding practices are significant.

Early determinants of inflammatory bowel disease have pointed toward infectious diseases in childhood, especially measles, and even in utero infections as possible causative factors. It has become a major disease of adults in Europe with 5.12 cases per 1000 individuals older than 43 years (National Survey of Health and Development of 1946) and 2.02 to 2.54 cases per 1000 adults by age 33 years (1958 National Child Development Study). In examining early determinants, these cohorts did not show a protective effect of breastfeeding. The authors comment, however, that the study recorded “ever breastfed” with no distinction for length of breastfeeding.

A systematic review with meta-analysis of breastfeeding and risk for inflammatory bowel disease was conducted by Klement et al. who concluded that breastfeeding is associated with lower risks of Crohn’s disease and ulcerative colitis. The reports that were included were published between 1961 and 2000. A report was published in 2005 of a pediatric case-control study of inflammatory bowel disease and 60 cases of ulcerative colitis in children younger than 17 years of age. The results did not support a protective effect of breastfeeding and suggested an association with the disease. When these data were included in the meta-analysis by Klement, however, the results still showed a protective effect of breastfeeding for these two bowel diseases.

Breastfeeding is associated with a 31% reduction for childhood inflammatory bowel disease according to the AAP. It is thought that the reduction results from the interaction of the immunomodulating effect of human milk and the underlying genetic susceptibility of the infant. The abnormal colonization of the gut in formula-fed infants may increase risk.

The severity of GI infection is attenuated if not prevented in breastfeeding infants according to the AAP. Infections due to enteric pathogens such as Rotavirus, Giardia, Shigella , Campylobacter and entero toxigenic Escherichia coli . The risk of these illnesses is reduced by 64% for infants who are breastfeeding.

Respiratory Illness and Otitis Media

Infants who develop respiratory illnesses should be maintained at the breast. The added advantages of antibodies and antiinfective properties are valuable to infants. Sick infants can nurse more easily than they can cope with a bottle. Furthermore, the comfort of having the mother nearby is important whenever the infant has a crisis; weaning during illness may be devastating to infants.

Wheezing and lower respiratory tract disease and other respiratory illnesses are lower in frequency and duration when the infant is breastfed. Recovery is accelerated if breastfeeding is maintained. The AHRQ reported a 72% reduction in the risk of hospitalization for respiratory infections in children under a year of age who were exclusively breastfed for at least 4 months.

Otitis media in infants occurs less frequently in breastfed infants because of the infection protection properties of human milk and the protective effect of suckling at the breast. Recurrent otitis media is associated with bottle feeding in a study of 237 children, in contrast to prolonged breastfeeding, which had a long-term protective effect up to 3 years of age.

A regional birth cohort of 5356 children was followed prospectively regarding the occurrence of infectious disease in the first year of life. One third developed otitis media. Median age of onset was 8 months, and 10% had had three episodes by 1 year of age. Breastfeeding for 9 months or longer had a significant impact on otitis, as did the number of siblings and daycare. Otitis media in 3- to 8-year-old children in Greenland was studied as a national concern for the incidence and associated deafness. Children who were breastfed were spared, especially if nursed a long time.

A protective effect of breastfeeding in otitis media was shown in a large prospective study. The AHRQ reported that exclusive breastfeeding for 3 to 6 months provided a 50% reduction in otitis media compared to formula feeding even when controlling for socioeconomic status, parental smoking, and the presence of siblings.

Young infants who have older siblings may well be exposed to some virulent viruses and bacteria. Developing croup, for instance, may make an infant seriously ill. Hydration can be maintained by frequent, short breastfeedings. Studies have shown that respirations are maintained more easily when feeding on human milk than on cow milk, even from a bottle. Nursing at the breast permits regular respirations, whereas bottle feeding is associated with a more gasping pattern. Thus breastfed infants should continue to nurse when they are ill. If an infant is hospitalized, every effort should be made to maintain breastfeeding or to provide expressed breast milk if the infant can be fed at all. Staff should provide rooming-in for the mother if a care-by-parent ward is not available.

Colostrum and milk contain large amounts of IgA antibody, some of which is respiratory syncytial virus (RSV) specific. Breastfed but not bottle-fed infants have IgA in their nasal secretions. Neutralizing inhibitors to RSV have been demonstrated in the whey of most samples of human milk tested. IgG anti-RSV antibodies are present in milk and in reactive T-lymphocytes. Breastfeeding-induced resistance to RSV was associated with the presence of interferon and virus-specific lymphocyte transformation activity, suggesting that breastfeeding has unique mechanisms for modulating the immune response of infants to RSV infection. Clinical studies indicating a relative protection from RSV in breastfed infants were clouded by other factors. The populations were unequal because of socioeconomic factors and smoking (i.e., bottle-feeding mothers were in lower socioeconomic groups and smoked more). In general, if breastfed infants become ill, they have less severe illness. ^, Although breastfeeding protects, parental smoking and daycare are important negative factors in the incidence of respiratory infection. Respiratory illness in either infant or mother should be treated symptomatically and breastfeeding continued. If the infant has nasal congestion, nasal aspiration and saline nose drops just before a feed are helpful.

Galactosemia

Galactosemia, caused by deficiency of galactose-1-phosphate uridyltransferase, is a rare circumstance in which an infant is unable to metabolize galactose and must be placed on a galactose-free diet. The disease can be rapidly fatal in the severe form. The infant may have severe and persistent jaundice, vomiting, diarrhea, electrolyte imbalances, cerebral signs, and weight loss. This is a medical emergency. This does necessitate weaning from the breast to a special formula because human milk, as with all mammalian milks, contains high levels of lactose, which is a disaccharide that splits into glucose and galactose. The condition is suspected when reducing substances are found in the urine in the newborn, and the diagnosis is confirmed by measuring the enzyme uridyltransferase in the red and white blood cells. The several forms can be distinguished by genetic testing, but except for the mild form, the infant must be weaned to a lactose-free diet. An infection with E. coli in the newborn period may be the trigger that precipitates serious symptoms associated with this or other metabolic disorders. Galactosemia is screened for in most states in the United States along with phenylketonuria (PKU) and other metabolic disorders.

When the diagnosis is made, genetic testing should be done. The Duarte variant of the disease is mild; some enzyme is available. Breastfeeding is permitted but the infant should be followed closely initially. Some infants can only be partially breastfed, with some lactose-free formula in addition for necessary calories. An endocrinologist should make the decision for the exact balance of milks. Classic galactose-1-phosphate uridyltransferase deficiency makes breastfeeding contraindicated.

Inborn Errors of Metabolism

Other metabolic deficiency syndromes are usually only apparent as mild failure-to-thrive syndrome until the infant is weaned from the breast and the symptoms become severe. This particularly applies to inborn errors of metabolism caused by an inability to handle one or more of the essential amino acids that are in higher concentration in cow milk than human milk. Infection is often a complication early in the lives of these infants, with inborn errors most commonly due to E. coli bacteria. While the acute infection is being treated, the infant may be weaned from the breast, and the metabolic disorder then becomes apparent precipitously.

Certain amino acids, including phenylalanine, methionine, leucine, isoleucine, and others associated with metabolic disorders, have significantly lower levels in human milk than in cow milk. Management of an amino acid metabolic disorder while breastfeeding depends on careful monitoring of blood and urine levels of the specific amino acids involved. Because these are essential amino acids, a certain amount is necessary in the diet of all infants, including those with disease. An appropriate combination of breastfeeding and milk free of the offending amino acid should be developed. The care of such infants should be in consultation with a pediatric endocrinologist. Transient neonatal tyrosinemia, which has been reported to occur in a high percentage (up to 80%) of neonates fed cow milk, is associated with blood tyrosine levels 10 times those of adults. Wong et al. have associated severe cases with learning disabilities in later years. Tyrosine appears in human milk at low levels. Tyrosinemia type I is an inherited autosomal recessive trait. Symptoms are caused by accumulation of tyrosine and its metabolites in the liver. It is treated by dietary control consisting of low protein with limited phenylalanine and tyrosine. Some breastfeeding is possible combined with protein-free supplements. 2-(2-Nitro-4-trifluoromethylbenzyl)-1-3-cyclohexanedione reduces the production of toxic metabolites. Liver failure is common. Dietary restrictions are lifelong.

Screening programs that test all newborns have identified many victims early. Almost all programs test for PKU, galactosemia, and hypothyroidism, and increasingly maple syrup urine disease, homocystinuria, biotinidase deficiency, tyrosinemia, and now cystic fibrosis are included. Most cases can be managed with continued breastfeeding and diet modification. Congenital adrenal hyperplasia requires corticosteroids but the feeding can be breast milk. If it is the salt wasting variety, an infant must have added salt.

Phenylketonuria

The most common of the amino acid metabolic disorders is PKU, in which the amino acid accumulates for lack of an enzyme. The treatment has been phenylalanine-free formula, available from Abbott Laboratories and Bristol-Myers, combined with added standard formula or breast milk to provide a little phenylalanine because every infant needs a small amount. If an infant is breastfed, the mother is usually willing to continue on an adjusted schedule. An infant may supplement the Lofenalac or Analog XP with breast milk. With careful monitoring of the blood levels and control of the amount of breastfeeding, a balance can be struck that permits optimal phenylalanine levels and breastfeeding. The infant will require some phenylalanine-free formula to provide enough calories and nutrients. A detailed outline of management called Guide to Breast Feeding the Infant with PKU , prepared by Ernest et al., is available from the Superintendent of Documents, U.S. Government Printing Office, Washington, DC 20402.

Literature values for phenylalanine range from 29 to 64 mg/dL in human milk. The amount for Lofenalac or Analog XP and human milk for a given baby is calculated by weight, age, blood levels, and needs for growth. As an example, a 3-week-old baby weighing 3.7 kg whose blood level was 52.5 mg/dL when he was ingesting an estimated 570 mL of breast milk would receive 240 mL Lofenalac and 360 mL breast milk (four breastfeedings per day with before and after weighing). The details of every step of management are available in the guide to assist a physician in planning treatment. Test weighing, which is now a simple home procedure with a digital scale, greatly facilitates the accuracy of this management.

As soon as the diagnosis is made, an infant should be placed on a low-phenylalanine formula to reduce the levels in the plasma promptly. The mother should pump her breasts to maintain her milk supply. Human milk has less phenylalanine than formula, but it exceeds the tolerance of most infants with PKU. The breastfed infant is offered a small volume of special formula (10 to 30 mL) first and then completes the feeding at the breast. As long as the blood phenylalanine levels can be maintained between 120 and 300 mmol/L, exact intake need not be measured. Initially, weight checks to ensure adequate growth are essential because poor intake leading to a catabolic state will interfere with control. Because human milk is low in phenylalanine, the offending amino acid, more than half the diet can be breast milk.

Another protocol for breastfeeding an infant with PKU was studied by van Rijn et al. The feeding schedule was based on alternating breastfeeding and phenylalanine-free formula by bottle. Each child had a separate schedule convenient for the mother-baby dyad depending on tolerance and age. At the beginning of treatment, the mother breastfed once daily allowing the infant to feed until satiated, and the mother pumped the rest of the day. Breastfeedings were increased while monitoring phenylalanine plasma levels. Ultimately breast and bottle feeding were alternated and equal. At all feedings, the infant drank until satisfied. The breastfed infants did well on this protocol and plasma levels were stable. An essential member of the management team is a board certified licensed lactation consultant to assist the mother in managing her milk supply.

The weaning of this special infant should be similar to that of other infants. Adding solid foods can be initiated at 6 months. The liquid part of the diet continues as before, that is, two feeding components of low-phenylalanine formula and breastfeeding plus solids with little or no phenylalanine (fruits, vegetables, low-protein foods). Rice and wheat contain too much phenylalanine. When the decision is made to wean from the breast, solid foods can be used to replace the phenylalanine in the breast milk as needed. Growth should be followed closely. When weaning is complete, the infant should be given other less bulky sources of protein free of phenylalanine. This stage will be carefully orchestrated by the endocrinologist and nutritionist. Because infants with PKU are more prone to thrush infection, the mother should be alerted to watch for symptoms in the infant and the onset of sore nipples that could be caused by Candida albicans . Treatment is nystatin for both the mother and baby initially. (See discussion in Chapter 16 .)

The other benefits of human milk make the effort to breastfeed valuable for the infant and for the mother, who usually wants to continue to contribute to her infant’s nurturing and nourishment. The prognosis for intellectual development is excellent if treatment is initiated early and the blood levels maintained at less than 10 mg/dL phenylalanine (120 to 300 mmol/L).

A retrospective study of 26 school-age children who had been breastfed or formula fed for 20 to 40 days before dietary intervention was conducted by Riva et al. The children who had been breastfed had a 14-point IQ advantage, which persisted at 12.9 points when corrected for maternal social and educational status. The age of treatment onset for PKU was not related to IQ scores. This study strongly supports the belief that breastfeeding in the prediagnostic stage has an impact on the long-range neurodevelopmental performance of patients with PKU ( Figure 14-4 ).

Intellectual quotient (IQ) in patients with phenylketonuria, evaluated by Wechsler Intelligence Scale for Children score, in relation to the type of feeding in the first weeks of life.

(From Giovannini M, Verduci E, Salvatici E, et al: Phenylketonuria: dietary and therapeutic challenges, J Inherit Metab Dis 30:145–152, 2007.)

Nutrition management of infants with organic acidemias involves limiting the intake of the offending amino acid(s) to the minimum necessary for normal growth and development and suppressing amino acid degradation during catabolic periods by providing alternative fuels such as glucose. In some disorders, including isovaleric acidemia, specific treatment is included to increase the excretion of toxic metabolites by enhancing the body’s capacity to make isovalerylglycine, an acylcarnitine translocase. As more specific amino acid-free formulas are made available, a recipe for combining breastfeeding with the special formula can be engineered to specific infants’ needs. The endocrinologist and the nutritionist can provide such a recipe. Dietary precautions for the mother of a breastfeeding child with PKU are to avoid the artificial sweetener aspartame (NutraSweet), which metabolizes to phenylalanine.

Cystic Fibrosis

Cystic fibrosis is an autosome recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The CFTR protein is in the epithelia of tissues including lung, sweat glands, pancreas, and GI tract.

Screening tests for cystic fibrosis (CF) have been initiated in many state-mandated metabolic screening programs for newborns, so a greater number will be identified early. Meconium plug, especially large plugs and full-blown meconium ileus, have a high correlation with pancreatic enzyme deficiency and CF. As clinicians are alerted to meconium plugs, early tests for CF can be carried out and management adjusted. Breastfeeding is optimal not only for the nutrition but for the presence of enzymes to facilitate digestion and absorption of nutrients. Because infection is a significant morbidity in these children, the infection protection properties of human milk make a critical impact. A study of infants exclusively breastfed or formula fed showed that breastfeeding does not compromise growth and is associated with fewer infections and respiratory problems in infants with CF.

The first symptom in infants with CF is often failure to thrive. If an infant is breastfed, the mother may be forced to wean, yet the infant feeds even less well and has no weight gain on formula. Infants do better if placed back on the breast. Pumping to increase the mother’s milk supply will help the child’s hunger. In a study of CF centers, 77% recommended breastfeeding either alone or with pancreatic enzyme supplements. The recommended breastfeeding duration was 3 to 6 months by 43% of the centers ( Tables 14-1–14-3A and 14-3B ). If supplementation is required, hydrolyzed formula is recommended. Generic and name-brand enzymes are not biologically equal, and some formulas were more frequently associated with greasy stools and abdominal cramping. Use of enzymes may be a way to improve tolerance and weight gain in these special breastfed infants rather than weaning to formula. Prescribing pancreatic enzymes for a mother while breastfeeding, as described earlier, is also a consideration.

Alpha ₁ -Antitrypsin Deficiency

Alpha ₁ -antitrypsin is a serum protease inhibitor that inactivates a number of proteases. More than 24 genetic variants of this disease are designated B through Z, with the M variant being most common. Children with α ₁ -antitrypsin deficiency are at increased risk for liver disease, which occurs most often during infancy and often progresses to cirrhosis and death. Udall et al. investigated the relationship between early feedings and the onset of liver disease. Severe liver disease was present in eight (40%) of the bottle-fed and one (8%) of the breastfed infants (breastfed for only 5 weeks). Of the 32 infants, 24 were still alive at the end of the study; 12 had been breastfed and 12 bottle fed during their first month of life. All eight of the deceased children had been bottle fed; small-for-gestational-age (SGA) and preterm infants had been excluded from the study so that all infants were equally stable at birth and capable of breastfeeding. A bottle-fed infant was seven times more likely to develop liver disease.

With the increasing early diagnosis of α ₁ -antitrypsin deficiency, encouraging a mother to breastfeed if her infant is affected would appear to have a significant impact on reducing the chance of long-range liver disease in her infant.

Acrodermatitis Enteropathica (Danbolt-Closs Syndrome)

Acrodermatitis enteropathica is a rare and unique disease in which feeding an infant with human milk may be lifesaving. It is an autosomal recessive disorder with an onset as early as 3 weeks old. It is inherited as an autosomal recessive trait and is characterized by a symmetric rash around the mouth, genitalia, and periphery of the extremities. The rash is an acute vesicobullous and eczematous eruption often secondarily infected with C. albicans. It may be seen by the third week of life or not until late in infancy and has been associated with weaning from the breast. Failure to thrive, hair loss, irritability, and chronic severe intractable diarrhea are often life threatening. The disease has been associated with extremely low plasma zinc levels. Oral zinc sulfate has produced remission of the syndrome. Zinc deficiency was seen frequently in premature infants on peripheral alimentation until zinc was added to the solution.

Human milk contains less zinc than does bovine milk, with zinc concentrations of both decreasing throughout lactation. Eckert et al. studied the zinc binding in human and cow milk and noted that the low-molecular-weight binding ligand isolated from human milk may enhance absorption of zinc in these patients. Gel chromatography indicated that most of the zinc in cow milk was associated with high-molecular-weight fractions, whereas zinc in human milk was associated with low-molecular-weight fractions. The copper/zinc ratio may also be of significance because the ratio is lower in cow milk.

The zinc-binding ligand from human milk was further identified as prostaglandin E by chromatography, ultrafiltration, and infrared spectroscopy by Evans and Johnson. Patients also have low arachidonic acid levels. Arachidonic acid is a precursor of prostaglandin. The efficacy of human milk in the treatment of acrodermatitis enteropathica results from the presence of the zinc-prostaglandin complex. The primary deficiency in an infant is an inability to absorb zinc except in this complex form.

The clinical significance of the relationship of human milk to onset of the disease and its treatment is in developing lactation in the mother of such an infant, rare as the disease may be. Delayed lactation or relactation is possible and should be offered as an option to the mother of such an infant (see Chapter 19 ).

Several reports of isolated cases of zinc deficiency during breastfeeding have appeared in the literature. ^, In some cases, zinc levels in the milk were low; in others, they were not measured. One child had a classic “zinc-deficient” rash that responded to oral zinc therapy. One should keep in mind that any deficiency is possible and consider intake deficiency when symptoms occur in a breastfed infant. The basic defect is presumed to be related to GI malabsorption of zinc.

The treatment of choice is oral administration of zinc in the sulfate or gluconate form. It is usually well tolerated, safe, inexpensive, effective, and expedient. When zinc deficiency occurs in a breastfed infant, the possibility of zinc deficiency in the milk, although a rare disorder, should be considered. Treating the mother would be the appropriate therapy in such a case.

Premature infants have a negative zinc balance associated with inadequate mineral stores and high requirement associated with rapid growth. Transient zinc deficiency in breastfed infants has been described as manifest by the classic zinc deficiency rash and was treated by oral zinc to the infant because milk levels are normal in the mother.

The regulation of iron, zinc, and copper in breast milk and the transport of these minerals across the mammary gland epithelium is poorly understood. Milk values at 9 months postpartum were not associated with maternal mineral status. This suggests an active transport mechanism according to the investigators. Milk zinc levels increase at weaning time while iron levels decrease.

Neurologically Impaired Infants

In addition to infants who have been neurologically impaired by perinatal hypoxia or asphyxia and low Apgars, a rare infant may have an inherited neurologic problem as in a trisomy or a congenital abnormality such as spina bifida. These infants can be breastfed in most cases but it requires patience and perseverance. Holding the infant in a flexed position is an essential element of breastfeeding. A sling works well.

Down syndrome is one of the more common syndromes, occurring in 1 of 800 to 1000 births. Hypotonia is a major feature that, along with small mouth and large tongue, make breastfeeding a challenge. At first, the infant may quickly drift off to sleep at the breast and weight gain is slow. The mother learns to hold her breast in place as the infant’s grasp is not strong enough to overcome gravity. The sling works also to hold the infant in place and free both the mother’s hands to hold both the breast and infant jaw.

When the suckling is weak initially, mother should pump between feedings to stimulate production of milk. If supplements are required, it is best to provide them with a Lact-Aid or a cup as the infant with Down syndrome is easily confused.

Down Syndrome

Infants with Down syndrome or other trisomies may be difficult to feed. When they are breastfed, mothers need patience to teach the infants to suck with sufficient vigor to initiate the let-down reflex and to stimulate adequate production of milk. Using manual expression to start flow and holding the breast firmly for the infant so that the nipple does not drop out of the mouth when the infant stops suckling will assist the process.

Initially however, an infant with Down syndrome may have surprisingly good tone and may even suck well at the breast, only to develop problems after mother and infant have been discharged home. Providing support for the head, the jaw, and the general body hypotonia will require considerable coordination by the mother. Propping the baby firmly with a pillow in the mother’s lap or supporting the infant in a sling frees a much-needed hand for steadying the jaw and breast (see Figure 14-2 ).

A nurse clinician in the hospital who is knowledgeable and experienced in dealing with neurologically impaired infants should be available to the parents. The initial goals for the mother-infant pair are developing confidence in handling the infant, adjusting to the infant’s problem, and dealing with the parental grief and sense of loss—loss of the normal infant that was expected. If the mother has breastfed other children, the emphasis on breastfeeding modifications are more successful, and milk supply usually responds to manual expression and pumping. Initiating sufficient stimulus to the breast to increase milk production is critical in the first few days to induce good prolactin response, especially in primiparas. Renting an electric breast pump is a good investment, justifiable for reimbursement from health insurance by physician prescription.

With ultrasound and amniocentesis, the diagnosis is often known before birth so that the family can be prepared. In developing a discharge plan for an infant with Down syndrome, a pediatrician will need to coordinate a team to avoid the fragmented care that develops with a multiproblem situation, which may require the consultation of a geneticist, genetic counselor, cardiologist, and other medical experts to deal with the problems. Ideally a pediatrician and an office nurse practitioner can provide the additional support and counsel necessary. Many families prefer to leave the hospital early to retreat to the comfort and privacy of their home and the health care provider they selected. Home visits by the pediatrician’s staff can provide the necessary monitoring of weight gain and nutrition and counseling by someone capable of handling all the problems that arise, including breastfeeding. No referrals should be made without the pediatrician’s knowledge and agreement. The pediatrician or family physician has the advantage of knowing both the family and the child.

In a study of 59 breastfed infants with Down syndrome, Aumonier and Cunningham reported that 31 had no sucking difficulty, 12 were successfully nursing within a week, and 16 required tube feeding initially, which was associated with other medical problems, including low birth weight (LBW), cardiac lesions, and jaundice. Hyperbilirubinemia is common in trisomy and was seen in 49% of the infants in this study. Eighteen babies had multiple medical conditions, and 11 of them sucked poorly. The authors point out that the initial sucking ability of the infants did not appear to be a major cause for nonmaintenance of breastfeeding; 10 of the 13 mothers who discontinued breastfeeding cited insufficient milk as a contributing cause, which might have been prevented by early pumping of the breasts between feedings. With amniocentesis, genetic testing, and screening in older mothers (older than 35 years), many are diagnosed prenatally. Parents are then partly prepared before birth.

The birth of an infant with a major genetic abnormality is a shock, even to the strongest parents. If the mother wants to breastfeed, she should be offered all the encouragement and support necessary. Usually she needs to talk with someone to express her anguish about the infant, not the feeding per se. A sympathetic nurse practitioner in the pediatric office can be invaluable in providing support and the expertise to help with the various management problems. If the mother chooses not to breastfeed, appropriate support can also be provided without disrupting treatment continuity.

It is especially important that these infants be breastfed if possible because they are particularly prone to infection, especially otitis media. Before the advent of antibiotics, they often died of overwhelming infection and rarely survived past 20 years of age. These infants and most other infants with developmental disorders do better with stimulation and affection, so the body contact and communication while at the breast are especially important. Those who have associated cardiac lesions not only can suckle, swallow, and breathe with less effort at the breast, but also can receive a fluid more physiologic for their needs. Breastfed or bottle fed, these infants gain poorly; thus switching to a bottle does not solve the problem. The recommendation that children with Down syndrome receive extra vitamins was tested in a controlled study in children 5 to 13 years of age, and no sustained improvement in the children’s appearance, growth, behavior, or development was seen with added vitamins.

Growth charts from birth to 18 years illustrate the deficient growth through the growing periods. In infancy they fall behind, so this observation should not be used to discontinue breastfeeding. Breastfed infants remain healthier. Children with Down syndrome are usually overweight throughout life, beginning in infancy.

Down syndrome is a lifelong condition. Having a support system is important for a family. Support groups of other families in the community serve as vital peer support.

Hypothyroidism

Bode et al. reported that an infant with congenital cretinism was spared the severe effects of the disease because he was breastfed. This was attributed to significant quantities of thyroid hormone in the milk. In a prospective study of 12 cases of hypothyroidism in breastfed infants, however, no protective effect against the disease was found, nor was the onset of the disease delayed. Anthropometric measurements, biochemical values, and psychologic testing at 1 year of age did not differ from those in the 33 bottle-fed hypothyroid infants. Abbassi and Steinour also reported successful diagnosis of congenital hypothyroidism in four breastfed neonates.

Sack et al. measured thyroxine (T ₄ ) concentrations in human milk and found it to be present in significant amounts. Varma et al. studied T ₄ , triiodothyronine (T ₃ ), and reverse T ₃ concentrations in human milk in 77 healthy euthyroid mothers from the day of delivery to 148 days postpartum. From their data, they calculated that if infants received 900 to 1200 mL of milk per day, they would receive 2.1 to 2.6 mg of T ₄ per day, based on 238.1 ng/dL of milk after the first week. This amount of T ₄ is much less than the recommended dose for the treatment of hypothyroidism (18.8 to 25 mg/day of levo-T ₃ ). T ₄ was essentially immeasurable in the milk sampled. In another study, however, comparing 22 breastfed and 25 formula-fed infants who were 2 to 3 weeks old, the levels of T ₃ and T ₄ were significantly higher in the breastfed infants. No definite relationship between the levels of T ₃ and reverse T ₃ could be found.

A 6-week-old girl was diagnosed to have congenital hypothyroidism by routine neonatal screening when T ₄ was reported at 3 mg/dL (normal greater than 7 mg/dL). The mother gave a history of multiple applications of povidone-iodine during pregnancy and continuing during lactation. Further testing revealed thyroid-stimulating hormone levels of 0.9 mU/mL (normal 0.8 to 5 μ/mL). Iodine treatment was stopped and breastfeeding continued while treatment of thyroid replacement was begun. At 1 year, growth and development were normal. It is, therefore, suggested that neonatal screening for thyroid disease may be even more urgent if the clinical symptoms are apt to be masked in a breastfed infant. No contraindication exists to breastfeeding when the infant is hypothyroid, and it would be beneficial. Appropriate therapy should also be instituted promptly. Mandatory screening for hypothyroidism is available to newborns in developed countries. Many infants that screen positive do not have at birth the characteristic signs and symptoms associated with cretinism, but therapy is just as urgent. Breastfeeding is ideal for these infants as well.

Adrenal Hyperplasia

In an analysis of 32 infants with salt-losing congenital adrenal hyperplasia who were in adrenal crisis, eight had been breastfed, five had been breastfed with formula supplements, and 19 had been formula fed. Infants who were breastfed were admitted to the hospital later than the formula-fed infants, although the breastfed infants had lower serum sodium levels on admission. The breastfed infants did not vomit and remained stable longer, although they had severe failure to thrive. Weaning initiated vomiting and precipitated crises in the breastfed infants. The authors suggest that congenital adrenal hyperplasia should be considered in a breastfed infant with failure to thrive. Electrolytes should be obtained before weaning to make the diagnosis and avoid precipitating a crisis by weaning. Then breastfeeding can continue as treatment is initiated.

Hypernatremic Dehydration Associated with Breastfeeding

The consequences of inadequate intake of breast milk range from hyperbilirubinemia, infant hunger, and low weight gain to life-threatening dehydration and starvation. The number of reported cases of hypernatremic dehydration has significantly increased because more infants have been breastfed and more infants are managed outside the hospital by lactation experts without pediatric oversight. Term breastfed infants with serum sodium levels of 150 mEq/L or higher were found to be 4.1% of the 4136 term infants hospitalized and reviewed by Unal et al. in the Children’s Research Hospital in Ankara, Turkey. These children had lost 15.9% birth weight (range 5.4% to 32.7%). The presenting symptom in 47.3% of cases was hyperbilirubinemia and poor suck in 29.6%. Other complications included acute renal failure in 82.8%, elevated liver enzymes in 20.7%, disseminated intracranial hemorrhage in 3.6%, and thromboses in 1.8%. Ten patients developed seizures and two died. In another study, 60 term infants were readmitted to the hospital with ketoacidosis with plasma serum sodium levels greater than 145 mmol/L. The hospital had recently upgraded its newborn discharge policy to include weights by trained midwives at 72 to 96 hours and at 7 to 10 days of age. Voiding, stooling, and breastfeeding were also checked, and infants who lost more than 10% of birth weight were sent to the hospital. The incidence of hypernatremia with plasma serum sodium levels greater than 145 mmol/L was 7.4 and 5.0 per 10,000 live births before and after the new policy, respectively, but the percentages of cases with plasma serum sodium levels greater than 150 mmol/L was 56.5% versus 18.9%. It was concluded that weighing and lactation support resulted in less dehydration and less severe hypernatremia and better breastfeeding rates. Hypernatremic dehydration in neonates due to inadequate breastfeeding is serious, a well-recognized cause of permanent neurologic abnormality, and life threatening. Sodium levels in breast milk vary, and highly elevated levels impair lactogenesis and cause failure to breastfeed. The etiology can be associated initially with poor milk production. As milk decreases in volume in the normal weaning process the sodium level increases. With lactation failure the sodium level is usually elevated. When levels in the infant reach 145 mEq/L urgent therapy is required. Serum osmolarity is also elevated, urine output is low, and the specific gravity elevated. Sodium levels in early milk are 300 to 400 mEq/L, and as volume increases the levels drop to 120 to 250 mEq/L. The problem is seen more commonly in primiparas. Loss of weight of over 10% deserves evaluation. It is recommended that these dyads be seen by the pediatrician within 2 days of hospital discharge. Treatment not only includes aggressive rehydration of the infant but skilled intense establishment of a full milk supply. Follow-up of infants with proper medical care by pediatricians is essential.

Neonatal Breasts and Nipple Discharge

A newborn may have swelling of the breasts for the first few days of life, whether male or female; this is unrelated to being breastfed. If the infant’s breast is squeezed, milk can be obtained. This has been called witch’s milk . The constituents of neonatal milk were studied in the milk of 18 normal newborns and infants with sepsis, adrenal hyperplasia, CF, and meconium ileus. Electrolyte values were similar to those in adult women in all infants except one with mastitis in whom the sodium level was elevated and the potassium decreased. Total protein and lactose were also similar to those in adult women. The fat was different, increasing with postnatal age and being higher in short-chain fatty acids. It was indeed true milk.

Two infants, one female and one male, were reported to have bilateral bloody discharge from the nipples at 6 weeks of age. Cultures and smears were unrevealing. No biopsy was done. The female infant’s swelling and discharge cleared after 5 months; the male infant’s was present at 10 weeks when he was lost to follow-up. Galactorrhea or persistent neonatal milk has been reported in association with neonatal hyperthyroidism. In another report, a 21-day-old female infant was seen because of a goiter and galactorrhea. The infant had 50% 24-hour I uptake and elevated prolactin levels, which slowly responded to Lugol solution treatment for hyperthyroidism.

Neonatal Mastitis

Neonatal mastitis occurs infrequently, although it was a common event in the 1940s and 1950s, when staphylococcal disease was rampant in nurseries. It occurs in full-term infants 1 to 5 weeks of age and in as many girls as boys, usually unilaterally. It is unrelated to maternal mastitis and usually occurs in bottle-fed infants. Before IV antibiotic therapy, surgical incision and drainage were common. Prognosis for cure is excellent. In recent years the rare cases that occur are seen in conjunction with manipulation of the neonatal breast to express the natural secretion when the newborn breast is engorged (witch’s milk). In some primitive cultures expressing milk from swollen newborn breasts is done and often leads to mastitis.

Hyperbilirubinemia and Jaundice

Jaundice in newborns has become a source of considerable misinformation, confusion, and anxiety. Incidence of jaundice is higher in full-term infants than a decade ago. From 1994 to 2002 11.9% of newborns were hospitalized for hyperbilirubinemia; rates rose to 20.0% in 2003 to 2005. The incidence of kernicterus dropped from 5.8 per 100,000 live births to 1.6 per 100,000 live births as a result of aggressive preventive measures in these years according to Burke et al. More physicians are paying attention to the development of hyperbilirubinemia in newborns. These two factors serve to increase the frequency of the question of the role of breastfeeding in the development of hyperbilirubinemia. Some of the confusion and inconsistencies associated with the management can be attributed to indecisive terminology. This discussion attempts to clarify the issues and outlines the causes and effects of hyperbilirubinemia.

Clinical Risk Factors in Hyperbilirubinemia

Clinical examination by visual assessment of jaundice in newborns is not reliable in a study comparing visual estimates with laboratory values by Moyer et al. They suggested bilirubin testing should be based on risk factors. Clinical risk factors significantly improve prediction of hyperbilirubinemia compared with the use of early total bilirubin levels, as reported by Newman et al. based on a study of almost 54,000 infants older than 36-weeks’ gestational age and at least 2000 g birth weight. From this group, 207 cases were found with elevated bilirubins drawn before 48-hour discharge. The authors found the risk index was the best predictor of elevated bilirubin ( Table 14-4 ). Clearly, prematurity carries the greatest risk. The TSB before 48 hours was an accurate predictor of reaching a bilirubin of 20 mg/dL ( Figure 14-5 ).

Table 14-4

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1. The Revolution in Infant Feeding
2. Physiology of Lactation
3. Protocol 3: Hospital Guidelines for the Use of Supplementary Feedings in the Healthy Term Breastfed Neonate
4. Protocol 16: Breastfeeding the Hypotonic Infant

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