Refer a Patient With Congenital Hyperinsulinism (HI)

Why refer to CHOP?

Congenital hyperinsulinism is rare; most children’s hospitals encounter only one or two cases a year. Since October 1998, the Congenital Hyperinsulinism Center at Children’s Hospital of Philadelphia (CHOP) has evaluated and treated more than 1000 patients — making it the largest program of its kind in the world.

Among the highlights:

  • We provide the full spectrum of care, from diagnosis through treatment, including education, psychosocial support and long-term follow-up. Our team is among the best equipped anywhere to diagnose and treat HI, resulting in shorter hospital stays and more cost-effective treatment.
  • Our Center draws on the expertise of specialists from throughout the Hospital, all uniquely suited to caring for the patient and family. We offer support from a wide variety of subspecialties, including a nationally top-ranked neonatal intensive care unit, allowing us to provide seamless care for children with complex conditions.
  • Conventional preoperative radiological studies — such as CT scan, MRI or ultrasound — can’t differentiate between focal and diffuse HI. Our Center performs 18F-DOPA PET scanning under an FDA approved research protocol (for appropriate cases). This innovative imaging technique helps surgeons pinpoint abnormal tissue and focal lesions, sparing healthy cells in the pancreas, significantly reducing the risk of diabetes in patients with focal HI, and potentially leading to a cure. Without 18F-DOPA imaging, many children at other centers have unnecessary near-total pancreatectomies.

When should I refer?

We invite you to contact us at any point in your patient evaluation process to discuss options or to seek advice on patient management. We serve as a resource for physicians and families, providing information to gauge when a child can be diagnosed and managed at their local institution, and when the condition requires a visit to CHOP. Once a patient fails a trial of diazoxide therapy, it is usually an appropriate time to consider a transfer to Children’s Hospital.

How do I refer?

Contact the Congenital Hyperinsulinism Center by phone at 215-590-7682 or email hyperinsulin@email.chop.edu.

  • If the patient may need surgery, it is useful to send a specimen to a specialty genetic reference lab for mutation analysis as early as possible, since results may take more than two weeks.
  • Initiate precertification from the insurance company (allow a minimum of 72 hours to complete). We can advise and assist you in this process. Listed below are items needed to start the referral process. If a letter of medical necessity is required, please contact us for assistance.

Here is a list of items that are needed to start the referral process. Please have your case manager begin the insurance authorizations for both admission and transport.

  • Insurance face sheet and front/back of insurance card
  • Medical summary/genetic results (HIPAA release attached)
  • Name and contact information (including email address) for the case manager and social worker involved with the patient
  • Transport availability, your team or ours
  • Patient bed number, unit and nurse's station phone number
  • Meter, strips, lancets along with glucagon emergency kit to send with parents to CHOP. These items are needed for when the family goes back home.  
  • Complete pre-admission checklist (PDF)

Paperwork can be faxed to 215-590-3053, attention HI center, or scanned and emailed to hyperinsulin@email.chop.edu.

Please call 215-590-7682 or email hyperinsulin@email.chop.edu if you have any further questions.

Please note: Out-of-state referrals may require prior authorization and/or a negotiation of rates with patient's insurance company. The time frame can range from a few days to weeks. All insurances have to be reviewed and processed by the CHOP transport financial counselors. If you have any questions, please contact CHOP transport financial counselors at 215-590-4313.

What happens after I refer?

Children's Hospital works with referring physicians before transport and maintains close communication and collaboration after the patients return home.

Congenital hyperinsulinism care profiles

Once a patient arrives at CHOP, our multidisciplinary team of physicians, fellows, residents, nurse practitioners, registered nurses, case management, social workers, nutritionists and pharmacists will meet with the family and decide on the best approach for managing each patient’s specific hyperinsulinism needs. Our team will explore different strategies for medical management and review genetic results to determine if a PET scan is indicated.

If failure of medical management, our experts will explore surgical options:

  • Partial/total pancreatectomy as determined by genetic results and/or PET scan
  • G-tube placement for ongoing medical management
  • Safety/cure fast at the end of the hospitalization to determine status for discharge

After a patient is referred to The Children’s Hospital of Philadelphia, she may fit one of four different types of care profiles.

Patient type A: HI patient with focal disease — cured

  • Fasting test
  • Drug withdrawal
  • Localization studies
  • Surgery
  • Recovery in Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU) 7–14 days
  • Fasting test
  • Discharge

Anticipated discharge planning needs:

In nearly all these cases, the patient is cured and requires only one visit with the local pediatric endocrinologist, with routine well-child follow-up care by the local pediatrician.

Patient type B: HI patient requiring medical therapy postoperatively

  • Fasting test
  • Drug withdrawal
  • Localization studies
  • Surgery
  • Recovery in N/IICU (7–14 days)
  • Medical therapy (10–14 days)
  • Fasting test
  • Discharge

Anticipated discharge planning needs:

Patient will require ongoing medical treatment by the local pediatric endocrinologist, including at least one fasting test and three outpatient visits per year.

Patient type C: Patient requiring diagnostic evaluation for HI

  • Overnight blood sugar monitoring
  • Oral glucose tolerance test (case-dependent)
  • Oral protein tolerance test (case-dependent)
  • Fasting test

If diagnosis is established:

  • Medical therapy
  • Fasting test
  • Discharge

Anticipated discharge planning needs:

Patient will require ongoing medical treatment by the local pediatric endocrinologist, including periodic re-evaluation.

Patient type D: HI patient requiring follow-up evaluation

  • Overnight blood sugar monitoring
  • Oral glucose tolerance test (case-dependent)
  • Oral protein tolerance test (case-dependent)
  • Fasting test
  • Blood sugar monitoring and medication adjustments until stable

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