Published onHI Hope
Many children with hyperinsulinism (HI) have feeding aversion — as many as 60%. The use of tube feedings, and possibly continuous dextrose, to control their blood sugar, among other things (effect of medications, the appetite suppressing effects of insulin, etc.), may exacerbate their aversion to eating by mouth.
While it might seem obvious that it is because with a belly full of sugar water, a baby might not feel hungry. But that might not be the case, according to Liesje Carney, RD, CSP, LDN, a nutritionist who works with families in the Congenital Hyperinsulinism Center at Children’s Hospital of Philadelphia (CHOP).
“We can’t definitely say ‘this is why,’” Carney says. “Whether the child has had a partial pancreatectomy or is being medically managed, they still continue to make insulin, so the body doesn’t give the hunger signal, telling them it’s time to eat.”
In hyperinsulinism (HI), a rare genetic condition, the child’s pancreas secretes excessive insulin, lowering blood glucose to dangerous levels. After a near-total pancreatectomy, the remaining small part of the pancreas can still release too much insulin, sending a child into hypoglycemia. That’s why continuous dextrose may be needed to raise blood sugar levels. For children whose HI can be controlled without surgery, dextrose may be part of their management plan, along with medication and dietary changes.
Upon arrival at CHOP
In the case of an infant arriving at CHOP with suspected or confirmed HI, the HI Center team will encourage the mother to continue feeding her baby by mouth, preferably breastfeeding or pumping and feeding breast milk by bottle. Babies are weighed before and after each feeding so their breastfeeding intake can be estimated: a gram in weight equals 1 milliliter (mL) of milk. For perspective, it takes 5 mL to fill a teaspoon. “There have been a lot of studies that show that breast milk is best,” Carney says. “Families have access to a lactation consultant if they want one.”
Initially, a baby may receive dextrose intravenously, a tube inserted into a vein. If the baby isn’t eating enough by mouth, they may need to receive supplemental breast milk or formula by nasogastric (NG) tube, a thin, flexible tube that goes in nose, down the esophagus and into the stomach.
A gastrostomy tube, or G-tube, gives direct access to the child’s stomach and is usually placed during the pancreatectomy for children with diffuse disease, because these children are likely to continue to need support to prevent hypoglycemia. Then the baby will receive dextrose, for blood sugar control if needed, and feeds, for supplemental nutrition if needed, through separate ports in the G-tube.
A delicate balance
The goal is to balance what the child is receiving through the G-tube so their HI is controlled, they get enough nutrients and calories to grow, and are still able to feel hunger so they want to eat by mouth — either breastfeeding, a bottle or, as they age, table food.
CHOP is one of the only pediatric hospitals that recommends using a G-tube for continuous dextrose, vs. the conventional IV delivery.
The HI Center’s experience with treating hundreds of HI patients has allowed clinicians to create protocols that deliver proper amounts of dextrose in concentrations that don’t disrupt a child’s digestion but still boost their blood sugar levels to keep the child in the safe zone.
CHOP’s feeding approach
While babies with HI are heterogeneous and each will receive a care plan specific to their individual needs, Carney and HI outpatient dietitian Lauren Cherry MS, RD, LDN, aim to keep them on an eating trajectory that matches a typical child.
“We focus support on normal, age-appropriate eating and nutrition,” she says. “Nutrition needs to change for an HI baby just like it would for a typical infant. They need to start cereals at 6 months and then solids foods.”
Hospitals with less experience with HI patients may try baby formulas with added carbohydrates, which — in theory — sound as if they might help raise blood sugar. “But with HI babies, it doesn’t work,” Carney says. “Dextrose is a different type of carbohydrate, and that’s what these babies need to control their HI.”
Despite the best efforts of families and support from CHOP, some children with HI do have an aversion to eating by mouth. “It all depends on the child,” Carney says, “and there are varying degrees of aversion.”
CHOP has therapies to help children learn to chew and swallow, how to use a spoon, how to get used to soft foods and different textures. Carney and Cherry also work with parents to coach them in ways to encourage the kids on dextrose and/or tube feedings to maximize their oral intake with healthy foods.
Feeds through the G-tube will ensure the child receives proper nutrition and calories for healthy growth until they can consume enough food by mouth. They may need to use the feeding tube only at night, or they may need feeds more frequently. While the exact age varies, by 4 years old many children are eating the typical three meals and two snacks a day and no longer need to use the G-tube for nutrition, although some children may still receive dextrose via the G-tube.
Children can wear a small backpack that holds their dextrose, as well as formula sometimes, with a small pump about the size of a double deck of cards, that allows them to continue playing like usual.
“Kids are very adaptable,” Carney says. “They adjust quickly and accept that this is part of their life.”