Aibha Conroy may have had ‘extremely rare’ hormone deficiency

A young girl who died from brain swelling having gone into hospital with low blood sugar may have had an “extremely rare” hormone deficiency, an inquest has heard.

However, the pathologist who carried out the autopsy on Aibha Conroy (6) from Gowla, Cashel in Connemara, Co Galway told Dublin Coroner's Court the underlying illness leading to her death may never be known.

Aibha died at Temple Street Hospital on December 14th, 2011. She was transferred there from Galway University Hospital (GUH) after being sent to the emergency department four days earlier with a history of vomiting, weakness and hypoglycaemia or low blood sugar. She went into respiratory arrest within 90 minutes of arrival at the hospital and later began to show signs of neurological abnormality.

The inquest previously heard that Aibha had presented at Galway hospital on two previous occasions suffering from hypoglaecemia. Her mother Kathleen Conroy gave evidence that she believes her daughter would still be alive today if she had been sent to Dublin for testing six months prior to her death.

On the fourth day of the inquest, neonatal pathologist Dr Deirdre Devaney said Aibha died from cerebral oedema or brain swelling following a documented episode of hypoglaecemia.

The inquest previously heard that doctors treating Aibha at Temple Street believed she had suffered a “massive cerebral insult” due to hypoxia or oxygen deprivation. However, Dr Devaney said the autopsy found evidence of cerebral oedema and not hypoxic brain injury. Both hypoglycaemia and a respiratory arrest can cause cerebral oedema, she told the court. If it had been solely due to respiratory arrest she would have expected to see changes due to depletion of oxygen, she said.

Despite extensive investigations including consultation with colleagues at Great Ormond Street Children’s Hospital in London, Dr Devaney said “no definite cause was identified” for the hypoglycaemia.

The autopsy also revealed that Aibha’s adrenal glands were two thirds the expected size raising the possibility of an “isolated ACTH (adrenocorticotropic hormone) deficiency” – a hormone condition in which the adrenal glands do not produce cortisol. The body releases cortisol as response to stress and low blood sugar levels. A cortisol level taken during Aibha’s final illness was “inappropriately low” suggesting a possible deficiency.

“It is more common in adults and it is extremely rare in young children. Extremely rare,” said Dr Devaney.

Only one other child of Aibha’s age has been recorded as having an isolated ACTH deficiency in global medical literature, the inquest heard. Dr Devaney said everybody concerned had tried their best to diagnose the underlying problem so the family could understand what had happened.

“It was a difficult problem and we may never get to the end of it,” she said.

The inquest was adjourned to April for further evidence from GUH staff. The family's solicitor Damien Tansey also made a submission that the coroner should call independent expert witnesses to give evidence including an endocrinologist to address the hormone issue.

“The Conroy family at a minimum deserve to hear from an endocrinologist”, he said, “There is a very significant suggestion that a short stature, overweight, and hypoglycaemia equals almost always endocrinology and if that is the case we are advised that the remedying of that problem was very simple”.