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ss triple helix - spring 2002,  Goldenhar Syndrome - A Tragic Breakdown in Communication

Goldenhar Syndrome - A Tragic Breakdown in Communication

A high profile disagreement between doctors and the parents of a baby with a rare facial disorder was finally resolved after a seven hour High Court hearing in Leeds, when the parents agreed to an urgent tracheotomy (BBC News, 7 March).

Twelve week old Maria Aziz Al-Rafi, the only survivor of triplets born in Saudi Arabia, has oculoauriculovertebral dysplasia (Goldenhar syndrome), a rare condition affecting only one in 500,000 babies, and has no right eye or ear, and only half a nose and jaw. She will require 18 years of surgery to correct abnormalities, and doctors at Royal Victoria Infirmary in Newcastle-upon-Tyne had wanted to perform a preliminary tracheotomy to secure her airway and assess the anatomy.

But the parents, who had already started a public appeal to raise £500,000 for private reconstructive surgery in the US, threatened to withdraw their child from the intensive care ward after the mother clashed with a consultant over proposed treatment. They were worried about losing their daughter during surgery, and also about 'medical staff experimenting and trying different procedures'.

Lord Fenwick, of the Newcastle Hospitals Trust, in taking the case to court said, 'The hospital was obligated to act in the best interest of the child'. Legally, the balance of power over a child is weighted in the parents' favour, on the assumption that parents will look after a child better than the State. But these rights do not extend to letting a child die or suffer by refusing necessary medical treatment.

But it is tragic that what began as a breakdown of communication between doctors and parents should escalate into a full-blown media circus and court proceeding. It is a reminder that good medicine involves far more than technical expertise. It is as much about understanding anxieties, addressing fears, patiently answering questions and giving information, and communicating empathy in a way that gains trust. The doctor/patient relationship, is a relationship, not a contract.

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