Inquest told missed test risked life

Robbie Powell was not given a crucial test months before he died

A child specialist has told an inquest into a boy's death from a rare illness that he would have given him different treatment months before he died.

Robbie Powell, 10, from Ystradgynlais, near Swansea, died from the rare but treatable Addison's Disease in 1990.

A jury heard on Thursday that a hospital consultant ordered a crucial test, which was never carried out, four months before Robbie died. Professor Ieuan Hughes told the jury he would have carried out the test.

The inquest jury at Swansea Guild Hall heard that Robbie was at risk of dying at any time after he was discharged from Morriston Hospital, Swansea, without having had the test.

If I suspected Addison's Disease...I would have done the test Professor Ieuan Hughes

He had been first admitted in December 1989 with the rare glandular condition Addison's Disease suspected.

Dr William Forbes, the hospital consultant at the time, ordered an ACTH test for the disease - but it was never subsequently carried out.

Robbie was later diagnosed with gastroenteritis and discharged when he appeared to recover.

Expert evidence

But on 17 April 1990, he was rushed back to hospital by his parents, where he suffered two heart attacks and died within hours.

A post-mortem examination later established that Addison's Disease, which affects the adrenal gland, was the cause of death.

In his expert evidence to the jury, Mr Hughes, a consultant paediatrician at the University of Cambridge, said he would have treated the case differently.

Under questioning by Will Powell, the schoolboy's father, Mr Hughes outlined how he would have acted during Robbie's December hospital admission.

Mr Powell had previously explained that a disfunctioning adrenal gland would have deprived his son of the hormone cortisone, which he needed to survive.

'Signs strong'

"The treatment for Addison's Disease is hydrocortisone," Mr Hughes said.

"If I suspected Addison's Disease, and I think the totality of the signs were quite strong for Addison's Disease, I would have done the test and started him on hydrocortisone pending the results of the test."

He added that even following Robbie's apparent recovery and discharge he could nevertheless have been called back in and undergone the test within a week.

Mr Hughes also agreed the schoolboy was at risk of suffering a second life-threatening "Addisonian crisis" at any time after his discharge.

'Extremely rare'

Mr Powell said: "When Robert was discharged from hospital he was at risk of dying, prior to having the ACTH test, of an Addisonian crisis, and I hope you accept that?"

Mr Hughes replied: "Yes."

The expert witness was later cross-examined by Jonathan Whitting, a barrister for Swansea NHS Trust.

Mr Hughes agreed that Addison's Disease was an "extremely rare" condition he had encountered only 10 times in children and twice in adults during his career.

He acknowledged it was so rare a condition that no statistical analysis existed anywhere in the developed world to measure its frequency.

The inquest was adjourned to be resumed at a later date.