What is Management for Congenital Hyperinsulinism and its Cost?

Effective Therapies and Surgical Options for CHI: From Glucagon to Pancreatectomy

Congenital Hyperinsulinism (CHI or HI) is a genetic disorder that is mostly prevalent in infants, neonates and children. This condition is the most common cause of hypoglycaemia (low blood sugar) during infancy and can lead to permanent brain damage. Hence, the management of CHI is critical.  

Popular Treatment Options for the Management of Congenital Hyperinsulinism

Medical care is ideally recommended for people with the condition of CHI. Patients with CHI  frequently require medications to maintain normoglycaemia. Some effective medicines used for the treatment of CHI are Dextrose, Sirolimus, Octreotide, Nifedipine and Diazoxide.

Certain therapies also help manage congenital hyperinsulinism, such as:

• Glucagon Administration : Glucagon administration is considered an initial treatment approach for patients with CHI. It is even more helpful in emergencies where intravenous access or oral feeding becomes difficult. Such an infusion enables a reduction in GIR (Glucose Infusion Rate) in the majority of infants.
• Pancreatectomy : Severe and persistent hypoglycaemia can lead to permanent neurological damage, especially in an infant. Surgical procedures like pancreatectomy become necessary for people who do not respond well to medical therapy.
  Pancreatectomy, particularly near-total pancreatectomy, is considered for patients with diffuse CHI who do not respond to medical therapy. The success of the procedure varies depending on the type of CHI, with focal forms often having better outcomes post-surgery.

Cost of Congenital Hyperinsulinism Treatment in India

A non-response to the first line of treatment for CHI is what mainly drives the total costs higher. Here is an overview of the estimated treatment costs of CHI treatment in India:

Causes of Congenital Hyperinsulinism

CHI is mainly driven by genetic factors. Mutations in at least 12 key genes involved in regulating beta-cell insulin secretion are known to contribute to its development. These genetic variations are typically categorised into the following subtypes:

• MCT1-hyperinsulinism
• KATP-hyperinsulinism (diffuse or focal)
• GDH-hyperinsulinism
• UCP2-hyperinsulinism
• SCHAD-hyperinsulinism
• GK-hyperinsulinism
• HNF4A and HNF1A-hyperinsulinism

Dietary Measures to Manage Congenital Hyperinsulinism

Here are a few dietary measures that can help manage CHI:

• Following a diet of 3 meals and 3 snacks daily can help maintain an adequate glucose level.
• Patients need to avoid any fasting to prevent a rise in hypoglycaemia.
• Carbohydrate-rich diets can provide a long-lasting source of glucose to counter the release of insulin.
• Patients need to increase carbohydrate intake before any strenuous exercise.

Furthermore, people should also have access to glucose tablets, hard candy, sugar cubes and fruit juice.

What is a Normal Blood Glucose Level in Congenital Hyperinsulinism?

In the condition of CHI, hypoglycaemia refers to the condition where blood glucose levels are less than 3.5 mmol/litre. In the absence of any ketone bodies, infants with CHI become constantly dependent on using blood glucose circulation as the basis for normal neurological functioning.

Hence, it is of utmost importance to maintain blood glucose levels above 3.5 mmol/litre.

Why is an Ongoing Management of Congenital Hyperinsulinism Important?

The main goal of the treatment of CHI is to keep the blood glucose levels in check. To monitor glucose, families can use point-of-care glucometer devices for regular testing. There are also devices like continuous glucose monitors, or CGMs, that are helpful. These devices also help measure blood glucose levels for patients with CHI.

New technologies and advancements are impacting almost every field of medicine. This is also applicable to the diagnosis and management of CHI. In recent years, scientists have identified new biomarkers that can be more specific and sensitive to better manage glucose levels in hypoglycaemic patients.