Coroner calls for health warnings on protein shakes after teen died

Coroner calls for ‘life-saving’ health warnings to be added to supermarket bought protein shakes after schoolboy, 16, suffered ‘irreversible brain damage’ triggered by a rare genetic disease after drinking one

• Rohan Godhania fell ill after drinking a protein shake, he died 3 days later
• READ: Organ donor feared to have passed on illness after recipient became ill 

A coroner has called for ‘life-saving’ health warnings to be added to supermarket bought protein shakes after a 16-year-old schoolboy suffered ‘irreversible brain damage’ triggered by a rare genetic disease after drinking one.

Rohan Godhania, from Ealing, west London, fell ill after drinking a protein shake on August 15, 2020. He died three days later at West Middlesex Hospital. 

A post-mortem examination could not identify his cause of death – a rare disease, ornithine transcarbamylase (OTC) deficiency – because his organs were donated for transplant before the mystery of the previously healthy schoolboy’s sudden illness was solved, an inquest at Milton Keynes Coroner’s Court in Buckinghamshire previously heard.

But months later, when the recipient of his liver started suffering seizures, a biopsy was done on the organ and it was discovered Rohan had had OTC. 

The disease is a type of urea cycle disorder – a group of genetic disorders caused by a deficiency of one of the six enzymes in the urea cycle, which is responsible for the removal of ammonia from the blood stream.

Rohan Godhania, from Ealing, west London, fell ill after drinking a protein shake on August 15, 2020. He died three days later at West Middlesex Hospital

Rohan Godhania with his father and mother Pushpa and Hitendra Godhania and his sister Alisha on a family holiday in August 2019

The inability to breakdown ammonia can cause it to build up to lethal levels in the bloodstream, and can be triggered by a protein load.

The deficiency is extremely rare and is estimated to affect one in 50,000 to 80,000 people. 

Speaking on Tuesday, Coroner Tom Osborne said: ‘Concerning these protein drinks, my preliminary view about them is that I ought to write to one of the regulatory authorities that some sort of warning ought to be put on the packaging of these drinks because, although OTC is a rare condition, it can have harmful effects if someone drinks (one) and it causes a protein spike.’

Finbar O’Callaghan, professor of paediatric neurology at the Institute of Child Health, University College London, agreed intervention was needed, describing it as ‘potentially life-saving’.

What is ornithine transcarbamylase deficiency (OTC)? 

Ornithine transcarbamylase (OTC) deficiency is a rare genetic disorder where a sufferer has a complete or partial lack of the enzyme ornithine transcarbamylase (OTC).

It is extremely rare and estimated to affect one in 50,000 to 80,000 people.

Symptoms of OTC deficiency can include vomiting, refusal to eat, lethargy and coma. 

OTC is one of six enzymes that play a role in the break down and removal of ammonia from the body. This process is known as the urea cycle. 

The lack of this enzyme means ammonia can build up – which can then to the central nervous system through the blood.

OTC deficiency is often treated as an emergency when sufferers cannot tolerate a normal diet and show symptoms.

Treatment usually given is a high glucose energy drink. 

Source: NHS Scotland 

Last week Mr Osbourne told the inquest that Rohan’s organ was signed off for tranplant despite there being no cause of death determined. 

The organ recipient later developed a ‘serious illness’ and only then was the cause of death for Rohan given.

The link was reportedly identified by an independent expert after the organ recipient was hospitalised with similar symptoms of seizures, though they are believed to have survived. 

At the inquest, lawyers acting for Rohan’s family have questioned whether opportunities were missed to screen him for ammonia prior to his death, despite this being recommended.

They suggested ammonia testing may have identified he was suffering from a urea cycle disorder, such as OTC.

Professor O’Callaghan told the inquest on Tuesday that if an ammonia test had been carried out on the day Rohan was admitted to hospital, it was ‘probable’ he would have survived, agreeing with a suggestion by the family’s lawyers that it was a ‘missed opportunity’.

He was also critical of the decision by a specialist hospital – known as a tertiary centre – not to accept a referral for the 16-year-old to their paediatric neurological team because the West Middlesex Hospital had classified him as an adult patient.

Professor O’Callaghan said that Rohan – like other patients his age across the country – had ‘fallen between two stools’ and as a result did not receive ‘quality of care’, explaining: ‘I am critical of the fact that if he (Rohan) goes down that adult pathway he was refused admission to the tertiary hospital because he was 16 years of age.

Pictured is Rohan Godhania with his parents Pushpa and Hitendra Godhania in Barcelona in 2015

‘If a hospital doesn’t have an adequate pathway for treatment, say if it cannot guarantee that 16-year-olds get accepted at the tertiary hospital, then that’s a big problem.’

He added: ‘There shouldn’t be discussion about whether he should be admitted to a paediatric hospital or an adult unit.

‘If he was treated as a paediatric patient I think it is more likely that he would have received a correct diagnosis more rapidly and that he would have had a smoother pathway.

‘This is a problem within our health service. The discussion goes on far too frequently around the country and the danger is that when there is uncertainty or debate then the patient suffers as I think has happened in this case.’

The inquest continues.

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