The Congenital Hyperinsulinism (CHI) Resource Guide

Last updated: March 10, 2026

Congenital Hyperinsulinism (CHI) is a rare genetic condition causing the pancreas to produce excessive insulin, leading to severe low blood sugar in children. Management requires specialized care, including continuous glucose monitoring, medications like diazoxide, and potentially surgery.

Key Takeaways

• Congenital Hyperinsulinism (CHI) is a genetic disorder causing severe, persistent low blood sugar due to excessive insulin production.
• Diagnosis involves specialized testing, including a fasting critical sample and 18F-DOPA PET/CT scans to identify the disease subtype.
• Treatment options range from first-line medications like diazoxide and feeding tubes to targeted surgical intervention.
• Long-term management requires continuous glucose monitoring, neurodevelopmental tracking, and clear emergency protocols for severe lows.
• Caregiver support and self-care are essential, as managing a child with CHI involves significant monitoring to prevent hypoglycemia.

Welcome to the Congenital Hyperinsulinism (CHI) Resource Guide. This guide is designed to empower parents and caregivers of children newly diagnosed with CHI.

Congenital Hyperinsulinism (CHI) is a rare genetic disorder where the beta-cells of the pancreas secrete too much insulin, causing severe and persistent low blood sugar (hypoglycemia) ^[1]. Unlike the temporary blood sugar dips many newborns experience, CHI requires specialized medical management, careful monitoring, and a deep understanding of your child’s unique genetic subtype ^[2].

Navigating a CHI diagnosis can feel incredibly isolating and terrifying. The constant alarms, the medical jargon, and the fear of brain injury from low blood sugar are overwhelming. This resource is built to give you the objective, evidence-based facts you need to understand the condition and advocate for the best possible care for your child.

Please use the following pages to explore the different facets of your child’s care:

Understanding a Congenital Hyperinsulinism Diagnosis: A Guide for Families: Start here for an overview of the condition, what separates it from transient hypoglycemia, and reassuring facts to help orient you.
The Biology of CHI: Genes, Channels, and Subtypes: Dive into the “why” behind CHI. Learn about the cellular “gatekeepers” and the crucial difference between focal and diffuse disease.
The Roadmap to Diagnosis: Blood Markers and Specialized Imaging: Learn how doctors confirm the diagnosis using a controlled fasting “critical sample” and the gold-standard 18F-DOPA PET/CT scan.
Standard of Care: Managing and Treating CHI: Understand the standard treatment decision tree, from first-line medications like diazoxide and feeding tubes to targeted surgical options.
The Long-Term Journey: Survivorship and Ongoing Care: Prepare for the future with information on neurodevelopmental monitoring, the long-term risk of diabetes, and strategies for managing caregiver burnout.

Frequently Asked Questions

What is Congenital Hyperinsulinism (CHI)?

Congenital Hyperinsulinism is a rare genetic disorder where the pancreas produces too much insulin. This leads to severe and persistent low blood sugar, requiring specialized medical care to prevent complications like brain injury.

How is CHI different from normal newborn low blood sugar?

While many newborns experience temporary dips in blood sugar, CHI causes severe, persistent hypoglycemia that does not resolve on its own. It requires careful monitoring, genetic understanding, and specific medical or surgical interventions.

How do doctors diagnose congenital hyperinsulinism?

Doctors typically confirm a CHI diagnosis using a controlled fasting test to capture a critical blood sample. They may also use a specialized 18F-DOPA PET/CT scan to determine the specific type and location of the overactive cells in the pancreas.

What are the main treatments for CHI?

Standard treatments for CHI include medications like diazoxide and the use of feeding tubes to maintain safe blood sugar levels. In some cases, particularly for focal CHI, targeted surgical removal of part of the pancreas is necessary.

What should I ask my doctor about managing my child's CHI at home?

You should ask your medical team for your child's specific target blood sugar ranges for both day and night, as well as a clear, written emergency protocol for treating severe lows at home. It is also important to establish a primary point of contact for blood sugar emergencies.

Questions for Your Doctor

• What is the target blood sugar range you want us to maintain for our child, both day and night?
• Who is our primary point of contact if we have a blood sugar emergency or if the continuous glucose monitor fails?
• Can you provide us with a clear, written emergency protocol for treating severe lows at home?
• How often will we have multidisciplinary team meetings to review our child's overall progress?

Questions for You

• Have I taken time to organize my child's medical records, genetic tests, and emergency protocols into a dedicated binder or folder?
• Who in my support network can I train to use the emergency glucagon kit or help with tube feedings so I can get a break?
• Am I neglecting my own physical and mental health while caring for my child?

Want personalized information?

Type your question below to get evidence-based answers tailored to your situation.

1
Early diagnosis of focal congenital hyperinsulinism: A fluorine-18-labeled l-dihydroxyphenylalanine positron emission tomography/computed tomography study.
Burroni L, Palucci A, Biscontini G, Cherubini V
World journal of nuclear medicine 2021; (20(4)):395-397 doi:10.4103/wjnm.wjnm_159_20.
PMID: 35018160
2
New approaches to screening and management of neonatal hypoglycemia based on improved understanding of the molecular mechanism of hypoglycemia.
Stanley CA, Thornton PS, De Leon DD
Frontiers in pediatrics 2023; (11()):1071206 doi:10.3389/fped.2023.1071206.
PMID: 36969273

This guide provides educational information about Congenital Hyperinsulinism (CHI) for caregivers. Always consult your pediatric endocrinologist or medical team for specific advice on managing your child's hypoglycemia and treatment plan.

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