Abu Dhabi, United Arab Emirates: A multidisciplinary team of physicians at Sheikh Shakhbout Medical City (SSMC), one of the UAE’s largest hospitals for serious and complex care and a joint-venture between Mayo Clinic and Abu Dhabi Health Services Company (SEHA), has successfully treated an extremely rare and uncommon 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%, 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.
In most countries, hyperinsulinism has an incidence of 1 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.” Continues 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,” concludes Dr. Ahmed.
About Sheikh Shakhbout Medical City (SSMC):
Sheikh Shakhbout Medical City (SSMC) is a joint venture between Abu Dhabi Health Services Company (SEHA), the UAE’s largest healthcare network, and Mayo Clinic, a non-profit global leader in medical care, education and research headquartered in the United States of America. Established in 2019, SSMC is the largest tertiary hospital in the UAE and serves to elevate the provision of healthcare services in the nation under the mandate of the Abu Dhabi Economic Vision 2030.
Supported by the latest diagnostic and treatment modalities available, SSMC offers care in 24 specialties. A team of locally and internationally trained physicians and allied health staff work seamlessly together, which promotes comprehensive interdisciplinary learning, allowing SSMC to become a leading hub for integrated patient-centric medical services in the region.
As the region’s leading tertiary facility, SSMC has 742 patient beds, 18 operating theatres and includes a hybrid operating room, 26 beds in the neonatal intensive care unit, and the UAE’s largest pathology lab.
The Abu Dhabi Health Services Company PJSC–SEHA – is an independent, public joint stock company created to develop the curative activities of the public healthcare system in Abu Dhabi. The company owns and operates all the public hospitals and clinics of the Emirate of Abu Dhabi.
SEHA is part of ADQ, one of the region’s largest holding companies with a broad portfolio of major enterprises spanning key sectors of Abu Dhabi’s diversified economy.
SEHA is committed to continuous improvement of customer care to recognized international standards and providing the community with world-class healthcare. SEHA operates 13 hospitals with 3,385 beds, 70 ambulatory care, family care and urgent care centers and 2 blood banks. Its facilities accommodate 117,162 inpatients annually and conduct 43,262 surgeries, as well as treating more than five million outpatients. SEHA is one of the largest integrated healthcare providers in the Middle East with more than 18,000 doctors, nurses, ancillary care, and administrative personnel.
About Mayo Clinic:
Mayo Clinic is a nonprofit organization committed to innovation in clinical practice, education and research, and providing compassion, expertise and answers to everyone who needs healing.