Baby Joseph's death spotlights Leigh Syndrome, end-of-life care

In this April 21, 2011, file photo provided by Cardinal Glennon Children's Medical Center, Moe Maraachli, center, with his son Joseph, reviews discharge plans with Dr. Robert Wilmott in St. Louis before leaving the hospital to return home to Ontario. AP/Cardinal Glennon Children's Medical Center, Mary Aita

baby joseph
In this April 21, 2011, file photo provided by Cardinal Glennon Children's Medical Center, Moe Maraachli, center, with his son Joseph, reviews discharge plans with Dr. Robert Wilmott in St. Louis before leaving the hospital to return home to Ontario.
AP Photo

(CBS/AP) Little Joseph Maraachli has died four months shy of his second birthday, a family spokesman said Wednesday.

The Canadian boy had been fighting a fatal neurological disorder known as Leigh Syndrome, a.k.a. Leigh's disease, and his parents' controversial bid to keep him alive against desperate odds had triggered an international debate on end-of-life care.

Widely known as Baby Joseph, the boy died at home Tuesday afternoon, according to Brother Paul O'Donnell of St. Paul, Minn., the family's spokesman and spiritual adviser. He said it was likely that the boy died of complications related to Leigh's disease but that the cause of death hadn't been announced.

Earlier this year, doctors at London Health Sciences Centre in Joseph's native Ontario refused to perform a life-extending tracheotomy, saying it was futile. An Ontario court decided doctors could remove the child's breathing tube.

His family sought help from U.S. hospitals. Cardinal Glennon Children's Hospital in St. Louis agreed to treat Joseph, and he was brought to St. Louis in March. He spent one month there.

Joseph's story drew international attention after doctors in Canada determined that he was in a permanent vegetative state and his condition was deteriorating.

When those doctors decided to take him off of assisted breathing, Joseph's parents, who lost an 18-month-old to the same disease eight years ago, unsuccessfully challenged the hospital's finding in court. They also began a social media campaign on their son's behalf.

The "Save Baby Joseph" Facebook page, which has more than 14,000 "likes," had several messages of condolences on Wednesday.

Eventually, Cardinal Glennon agreed to take Joseph, and he seemed to make progress. When the child was released from the hospital in April, O'Donnell said the family was "overjoyed." Doctors performed a tracheotomy that provided Joseph with increased mobility and comfort.

At the time, they said the tracheotomy could extend his life by up to six months - as they say it did for their other child who died - and that it would allow him to die at home.

That proved to be the case. Joseph died six months after the procedure in St. Louis, and he died at the family's Ontario apartment.

Leigh Syndrome is a rare inherited disorder that affects the central nervous system. It typically begins between the ages of three months and two years, although teens and adults are sometimes affected. Symptoms of the disease - which is caused by genetic mutations or enzyme deficiency - include poor sucking, impaired movement, seizures, continuous crying, and generalized weakness.

Treatment options are limited, and the prognosis is poor. Death with the first few years of life is common, though some Leigh sufferers have survived into their mid-teenage years.

The National Institute of Neurological Disorders and Stroke has more on Leigh's disease.

  • David W Freeman

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