Managing HI into Adulthood

The Congenital Hyperinsulinism Center at CHOP has been with your family every step of the way as you’ve treated and managed your child’s HI.

As they grow, we will continue to support you — through adolescence, the teen years and beyond. With guidance, knowledge and an expert team in their corner, children will continue to thrive.

Each child is different, of course, but here is what you can expect in the coming years.

Children with Surgically Managed HI

Except for those with focal disease whose hypoglycemia was cured with a partial pancreatectomy, most children with hyperinsulinism (HI) will need to manage the condition into adulthood.

Those who have had a near-total pancreatectomy tend to go through several phases. In the first phase, nearly half will need octreotide (or its longer-lasting sibling lanreotide) and/or dextrose in the first few years after surgery. In the second phase, sometimes called the honeymoon stage, their remaining piece of pancreas produces just the right amount of insulin. This is usually followed, eventually, by the onset of diabetes.

Each child is an individual and passes through these phases at different ages.

Unique class of diabetes

The diabetes children with HI experience is different than either type 1 or type 2. While they will be insulin-dependent, diet and exercise play a larger role in determining their blood sugar levels. They tend to be highly sensitive to simple sugars, making healthy food choices is extremely important. In fact, the honeymoon period, before starting insulin, may be able to be extended by following a well-rounded diet and getting plenty of exercise.

Each family can prepare its child for a lifetime of healthy, mindful eating by including children in grocery shopping and meal planning early on, so it is second nature to them by the time they will be making eating decisions on their own.

To help manage their diabetes, young adults can elect to use continuous glucose monitors (CGM), and/or insulin pumps.

No pancreas, no pancreatic enzymes

The natural pancreatic enzymes that allow people to break down foods so the nutrients can be absorbed are removed along with the pancreas in children with HI. Sometime during childhood or adolescence, post-pancreatectomy patients will start taking pancreatic enzyme pills. This will continue throughout their life.

CHOP’s HI Multidisciplinary Clinic features a team that includes Endocrinology, GI and Nutrition to determine the optimum pancreatic enzyme dose for each child with HI to take before they eat. Typically, patients take two pills before a meal and one pill before a snack so they reap the nutritional rewards of their (healthy!) diet.

Non-surgical medically managed HI

Children with diazoxide-responsive HI who manage their disease with medication might be able to reduce their medication or go off it completely and control their blood sugars with diet. A well-balanced diet at each meal that contains carbohydrates, protein and healthy fats will keep levels steady. It’s best to avoid eating sugar-loaded foods, especially alone, as they will send sugars soaring, followed by a steep drop.

Children with hyperinsulinism/hyperammonemia (HI/HA) syndrome are used to managing their disease with medication and, critically, with diet, being sure to carb-load at all meals. Into adulthood, protein will continue to be a trigger for low blood sugar.

Children with diazoxide-unresponsive HI who are managed medically, rather than surgically, are likely to require treatment for their hyperinsulinism throughout childhood and adolescence. Very intense treatment regimens with combination therapy (octreotide or lanreotide +/- dextrose through a gastrostomy) is usually require during infancy, toddler years and beyond. However, the severity of the hyperinsulinism tends to ameliorate over time and some therapies can be discontinued after adolescence.

No matter their type of HI, most children have learned to sense by adolescence (if not earlier) when they’re “going low.” Listening to their bodies — and having healthy sugar-boosting snacks or drinks on hand — will be important going forward. Of course, they will need to test their glucose levels to confirm a drop in blood sugar.

CHOP can remain your treatment home

Because HI is so rare, many adult endocrinologists have worked with few, if any, patients with HI and may not fully appreciate the complexities that come with caring for adults who don’t fit into more traditional models of diabetes care or those whose HI is medically managed.

When the Congenital Hyperinsulinism Center was named a CHOP Frontier Program, a recognition of its unique contribution to advancing the care of those with HI, it confirmed that older patients can continue to benefit from the center’s expertise.

To see if the HI Multidisciplinary Clinic would be a good fit, patients of any age can call 215-590-3174 to schedule an appointment.