Abu Dhabi: A multidisciplinary team of physicians at Sheikh Shakhbout Medical City (SSMC), a joint-venture between Mayo Clinic and Abu Dhabi Health Services Company (SEHA), has successfully treated an extremely rare case of complex congenital hyperinsulinemic hypoglycemia in a four-month-old baby girl from Socotra Islands in Yemen, who was transferred to SSMC from another health care facility.
Dr. Gamal Ahmed, division chair of the Pediatric Intensive Care Unit at SSMC, said: “The young patient came to us, and after a few days, developed convulsions that were associated with refractory hypoglycemia, which her laboratory workup confirmed. After we managed to control the convulsions with unremarkable brain sequelae or complications by MRI and EEG, the baby was diagnosed with congenital hyperinsulinemic hypoglycemia, a rare endocrine disease. A multidisciplinary team was gathered to discuss management and care of the patient to ensure the best approach for patient care and long-term outcomes.”
Congenital hyperinsulinism is the most severe cause of persistent hypoglycemia in newborn babies and children. If hypoglycemia is prolonged, it can be the most common cause of irreversible but preventable brain damage. Hyperinsulinemic babies usually need up to five times more glucose than children with typical glucose requirements do, and about 60% of babies with hyperinsulinism develop hypoglycemia within the first month of life. Of the remaining 40 per cent, nearly all will develop hypoglycemia before one year of life.
There are many genetic, transient, and metabolic causes of hyperinsulinism, but it’s very hard to diagnose. Signs and symptoms of hypoglycemia to look out for are lethargy or lack of energy, irregular body temperature, a weak or high-pitched cry, seizures, fast heart rate, amongst others.
Uncommon treatment
In most countries, hyperinsulinism has an incidence of one to every 25,000 to 50,000 births. For those with hyperinsulinism, the pancreas, which is responsible for insulin secretion, is blind to the blood glucose level and makes insulin even when blood sugar levels are not high, causing severe, and often prolonged, hypoglycemia.
“After the diagnosis of the patient’s condition was confirmed, we used quite an uncommon treatment, but one that is recommended in such a case of refractory hypoglycemia. The patient’s blood glucose continued to fluctuate for a few days with the escalation of treatment until a steady state of blood glucose was reached. By the end of the treatment course, we observed no hypoglycemic episode,” said Dr. Ahmed.
Physicians were, however, conscious that treating a four-month-old baby with refractory hypoglycemia with uncommon medication that may not be available in her home country did pose its own challenges They had to get compliance from the baby’s parents on management in their home country and to educate and train the parents on home blood glucose monitoring, providing them with a glucometer.
The primary approach the treating multidisciplinary team at SSMC had to decide upon was whether to pursue medical versus surgical treatment, both of which had its own benefits. They consulted with colleagues at the parent partner company, Mayo Clinic, for a second opinion, along with other international experts in order to determine that a medical approach was the better course.
“This case was a success and a prime example of how we exercise our model of care at SSMC. We had a collegiate and cooperative multidisciplinary team who took consultation from international expertise, including our colleagues at Mayo Clinic, and we had the patient’s parents who were involved in the patient’s care at every step of the way. Both the parents were highly appreciative of the compassionate care shown and the treatment provided to their baby. The referring hospital shared their appreciation for the successful management of the baby’s condition and for carrying out the best treatment suitable to the uncommon condition,” added Dr. Ahmed.
