Congenital Hyperinsulinism International

CHI Urges Adoption of PES Hypoglycemia Guidelines

Congenital Hyperinsulinism (HI), which causes severe and prolonged hypoglycemia, can lead to brain damage and death if not detected and treated in a timely manner. Currently, many patients born with this congenital disease are discharged from newborn nurseries without adequate study of their hypoglycemia, often with tragic consequences.

In “Recommendations from the Pediatric Endocrine Society team of experts for Evaluation and Management of Persistent Hypoglycemia in Neonates, Infants, and Children,” an article available online August 2015 in Journal of Pediatrics, the authors* provide guidelines for evaluating and managing newborn hypoglycemia. These guidelines, if widely implemented, will significantly reduce brain damage and death from hypoglycemia caused by HI. “Re-evaluating Transitional Neonatal Hypoglycemia,” also published in the June 2015 Journal of Pediatrics by the same group of authors, clearly shows the difference between transient newborn hypoglycemia and persistent or recurrent hypoglycemia. Together, these two articles demonstrate a straightforward pathway to diagnosing hyperinsulinism before damage and death occur.

Congenital Hyperinsulinism International (CHI), the organization dedicated to improving the lives of people born with hyperinsulinism, strongly recommends every hospital adopt these hypoglycemia guidelines as outlined in these two articles in order to ensure that all babies who leave the newborn nursery do so without the risk of preventable brain damage and death.

The guidelines should be read and adopted in their entirety. These points require highlighting:

  • Recurrent hypoglycemia in newborns, infants, and children is not normal. Healthy newborns who experience a blood sugar level drop, do not typically fall below a level of 50mg/dL (2.7mmol/L). Newborns and infants with blood sugars levels lower than 50 (2.7mmol/L) or those whose blood sugar levels drop past the usual transitional newborn nadir should be evaluated and managed according to the hypoglycemia guidelines recommended by the authors of the two articles.
  • At-risk infants suffering from low blood sugar levels must remain in an appropriate clinical setting with blood glucose levels maintained at normal levels until euglycemia is achieved and sustained. These babies must pass a fasting test or receive a diagnosis and management plan to treat the underlying causes of hypoglycemia before they are transitioned home.
  • Newborns who show signs and symptoms of hypoglycemia even if not known to have a risk factor must be evaluated and managed for hypoglycemia to prevent brain damage and death.
  • Newborn seizures present very differently from seizure in older babies, children and adults. Medical professionals caring for newborns should be familiar with the presentation of newborn seizures.

Healthcare professionals caring for newborns should be alert to the possibility of hypoglycemia when witnessing the following signs and symptoms:

  • Bluish-colored or pale skin
  • Breathing problems, such as pauses in breathing (apnea), rapid breathing, or a grunting sound
  • Irritability often followed by listlessness
  • Loose or floppy muscles
  • Poor feeding or vomiting
  • Problems keeping the body warm
  • Tremors, shakiness, sweating, or seizures

In the newborn and infant, these signs and symptoms may be hallmarks of a variety of conditions. Investigating these signs and symptoms for hypoglycemia saves lives and prevents brain damage. CHI knows of instances where nurses, midwives, pediatricians, family physicians, emergency department physicians and neonatologists have investigated for hypoglycemia and saved lives and prevented brain damage.

CHI believes every healthcare professional should be activated to the possibility of hypoglycemia, to end preventable brain damage and death from undetected prolonged hypoglycemia. The evaluative process outlined in the hypoglycemia guidelines are simple, relatively inexpensive and will not substantially lengthen stays for newborns and infants. CHI urges the adoption of these guidelines.

The authors of these guidelines are: Paul S. Thornton, MD, Charles A. Stanley, MD, Diva D. De Leon, MD, MSCE, Deborah Harris, PhD, Morey W. Haymond, MD, Khalid Hussain, MD, MPH, Lynne L. Levitsky, MD, Mohammad H. Murad, MD, MPH, Paul J. Rozance, MD, Rebecca A. Simmons, MD, Mark A. Sperling, MBBS, David A. Weinstein, MD, MMSc, Neil H. White, MD, Joseph I. Wolfsdorf, MB, BCh

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