/ 20 December 1996

Mother beats curse of the gods

A unique project has ‘saved a little girl from a lifetime in hospital, reports Marion Edmunds

The evil has for once been taken out of Ondine’s Curse, thanks to a determined mother and the Red Cross Children’s Hospital in Cape Town. They have saved a ‘cursed’ toddler from life in a hospital ward. On Wednesday, Juliage Jackson was for the first time able to take her two-and-a-half-year-old daughter, Mumtaas, home to her family in the northern suburb of Blackheath.

That morning, Mumtaas was up and down and all over the tracheotomy ward which had been her entire life until then. She was a little unsure about what was happening, but sensed the growing excitement of the adults around her ‘ the ward sisters and her mother.

‘I think she knows something is going to happen, I think she knows she is going home,’ said Jackson, holding her proudly on her knee. ‘Say how old you are, tell the lady how old you are.’ Mumtaas managed a throaty gurgle which, with careful listening, translated as ‘twee’.

Her speech is impaired by the hole cut in her throat ‘ a tracheotomy ‘ to make it possible for her to be connected to a breathing machine, or ventilator. This hole is her lifeline because her medical condition, Ondine’s Curse, inhibits the functioning of her central nervous system while she sleeps. This means she cannot breathe when sleeping ‘ sufferers normally die shortly after birth.

The name Ondine means mermaid, and it was chosen to describe the rare condition after a fairytale in which a mortal was cursed by the god of the sea with loss of bodily functions for jilting a mermaid.

Mumtaas’s breathing stopped shortly after she was born at Somerset Hospital on February 2 1994. She was immediately put on to a respiratory machine.

‘A month after her birth,’ said Jackson, ‘a doctor called my husband and me in and said we had to take a decision as to when we should switch off the machine, the respirator, to let her die. And I begged for a second chance, I pleaded for a second opinion, and we moved her to the Red Cross Children’s Hospital, where they told us she had Ondine’s Curse.’

Sister Jane Booth said at that time one child with Ondine’s Curse was living in the tracheotomy ward at the hospital. She had suffered brain damage from lack of oxygen, and was mentally retarded. She had spent her life in ward E1, and died last year, at the age of 10.

‘I saw this girl when we brought Mumtaas in,’ said Jackson. ‘I said to my husband, ‘Even if she lives here for the rest of her life and we have to come and visit her every day, it’s better than switching off any machine.’ I never thought this day would come. It’s a miracle, it’s like taking a newborn baby home for the first time. I was so excited last night I could not sleep and Sister Dorothy Schulman said she woke up at 4 o’clock this morning and was making notes for me.’

What made Mumtaas’s homecoming possible was the purchase of a ventilator which can be operated by her mother. The head of the intensive care unit at the hospital, Professor Max Klein, managed to negotiate the purchase of four ventilators at ‘R28 000 each, instead of the normal price of R36 700.

It was up to Jackson, a general worker in a printing works, to raise the money to buy one of these machines. She collected R6 000 from friends and relatives, she sold tickets for local musicals and then money poured in from the public after she advertised the plight of her little girl on a community radio station.

She raised R20 000 in four months, and this was supplemented by the Friends of the Children’s Hospital. Then she had to be trained by the ward’s sisters to use the ventilator at home.

Mumtaas has not suffered any mental retardation from the disease, and is a lively and inquisitive toddler. Booth calls her a ‘busy bee’, and says her speech has developed rapidly since her mother moved into the ward, to sleep in and care for her, in anticipation of her homecoming.

Jackson has had to give up her job to be her child’s full-time nurse. Mumtaas must be watched all the time because she cannot be allowed to fall asleep, not even to doze, without being hooked up to the ventilator.

Booth said she would be visiting the family in Blackheath every two days or so in the beginning to see how they are getting on. An alarm has been connected to the ventilator so that if, by chance, the connection slips from Mumtaas’s throat while she sleeps, Jackson will be woken up to reconnect it.

Jackson is planning to raise money for a generator so that her child’s life is not endangered by freak power cuts in the middle of the night.

‘Nowhere in the world would somebody like Mumtaas be able to go home without full nursing care,’ said Booth. ‘Here we just don’t have the infrastructure or the resources to provide nurses, so unless you are very wealthy, the child is doomed to stay in the hospital her whole life long. But because we have been able to train the mother, it is all possible.’

Mumtaas and her mother are part of a unique initiative by the Children’s Hospital to pioneer a home-care programme for children with tracheotomies, to provide the children with a better quality of life and to ease the pressure on the hospital’s shrinking resources. Despite a shortage of funds, the hospital has managed to return 70 children with tracheotomies ‘ as a result of conditions such as weak throat muscles or tumours ‘ to their homes, saving the hospital almost R200 000 per child each year.

Klein said of the programme, the only one of its kind in the world: ‘It’s probably the most exciting thing we are doing at the hospital at the moment. We are finding human solutions to what are usually thought of as mechanical problems.

‘We use the most sophisticated technology available anywhere in the world ‘ the mother.’