Another rare metabolic disorder is PKU (phenylketonuria). PKU is a lack of a liver enzyme that causes the baby to be unable to breakdown the amino acid phenylalanine so that it builds up in the blood, causing irreversible damage to the brain and nervous system if untreated. PKU screening is done routinely in all 50 states and in more than 30 countries. Health care professionals used to think that babies with PKU could not breastfeed due to the fact that breastmilk contains phenylalanine. However, research has shown that since babies need some phenylalanine for normal growth, and since breastmilk contains lower levels than formula, the mother can continue breastfeeding while supplementing her baby's diet with a special low-phenylalanine formula called Lofenalac. Babies with PKU need to have their weight and phenylalanine levels carefully monitored, but in the long run, breastfeeding can make the management of PKU babies easier. Studies have found that babies with PKU who are breastfed score 12-14 points higher on IQ tests than babies fed a diet consisting solely of Lofenalac. The treatment plan for PKU babies is handled by a doctor and a dietician specializing in metabolic defects, and the plan should include breastfeeding.
Other medical conditions that may cause difficulties with breastfeeding include cleft lip and/or palate, Down syndrome, neural tube defects (such as spina bifida), hydrocephalus, hypoglycemia, jaundice, congenital heart defects, reflux, cystic fybrosis, hypothyroidism, celiac disease, and allergies. (A special note about allergies: infants are not "allergic" to their mother's milk, but on rare occasions, they may be allergic to a food the mother has ingested - most often, the offending food is cow's milk, and eliminating it from the mother's diet eliminates the problem). In all these situations, breastfeeding is not only possible, but is recommended. Careful monitoring of milk intake, adjustment of medications, corrective surgery, and supplemental feedings may be required, but breastfeeding these infants offers many important health benefits, as well as increased bonding and closeness. The mother who has a child with a medical problem already has to deal with the stress of losing the ideal of the "perfect infant" she imagined. Every effort should be made to encourage and support these mothers in their efforts to nurse their special babies, so that their infants receive the benefits of breastfeeding and the mother doesn't have to deal with the additional loss of not being able to nurse her baby.
The mother who is unable to breastfeed or who has to wean prematurely experiences the loss of something very important to her, and often goes through the same stages of grief as the person who is coping with the loss of a loved one: denial (Of course I can breastfeed!...), anger (Why me?), bargaining (If I could just nurse this baby, I'll never ask for anything again.) depression (It makes me so sad to see other mothers nursing their babies) and finally, acceptance (I know that this is not something I can control, and I did everything I could.lots of babies do fine on formula.). It is helpful to be aware of these normal stages, and try to work through each one. It is important that you work with your health care team to explore all the options that might make breastfeeding possible, such as the use of breast pumps, tube feeding devices, and delivery of supplemental feedings (whether expressed milk, donor milk from a milk bank, or infant formula). Make sure that all these options are fully explained to you.
Sometimes even mothers who are strongly committed and follow all suggestions or instructions to the letter are still unable to breastfeed. In these cases, you need to try to feel good about the fact that you try to provide the best for your child, while making every effort to r accept the reality of the situation and the fact that sometimes factors beyond anyone's control make breastfeeding impossible no matter how highly motivated you are or how hard you tried. Once you have reached the acceptance stage, it is easier to place your breastfeeding experience in context, and move on to focus on dealing with the other important aspects of your health and your baby's.