A family's little star is at peace; Incurable condition brings deep heartache.

Byline: By Kevin Donald

A HEARTBROKEN family blighted by an incurable genetic disorder has been shattered by the death of their youngest member from the cruel muscle-wasting condition.

Tiny Sophie Elizabeth Hart survived for just eight weeks after being born with the rare congenital myotonic dystrophy.

Her mother, uncle and grandmother all live with the debilitating condition, which causes the muscles to stiffen, weaken and waste away.

Babies born with congenital myotonic dystrophy have difficulty breathing, and Sophie was on a ventilator from birth.

Aware that babies on ventilation for more than 30 days are unlikely to survive, her family made the agonising decision, based on medical advice, to turn off her life-support machine.

She was baptised in hospital with mum Donna Marie Hart, 26, of South Shields, and died four hours later in the arms of her aunt, Amanda Hobbs, 27.

Miss Hart, who was looking forward to becoming a mum for the first time, said: "I told her, 'You're at peace now. No more machines'."

Mum-of-three Mrs Hobbs, of Sycamore Avenue, Cleadon Park, South Shields, said: "I just sat and talked to her. It was lovely. It was as if she was letting us know she was at peace."

Miss Hart was diagnosed with the congenital myotonic dystrophy when she was six months pregnant and faced an anxious wait to see if her baby would be born with it.

Sophie arrived by Caesarean section on April 15, eight weeks early, weighing 3lb 12oz.

She was transferred to the neo-natal unit at Sunderland Royal Hospital, where she remained until she died on June 8.

Mrs Hobbs said: "It was an emotional rollercoaster from day one. We would go in and everything seemed to be all right, then we'd get a bit of bad news and everything went downhill.

"The amount of support we got from the nurses was unreal. It was nice to know they were there for us."

Miss Hart had to wait four weeks before she could hold her daughter. She said: "I just broke down. I wanted to be in her place."

Grandmother Lorna Hart, 53, said Sophie recognised their voices and they would sing Twinkle Twinkle Little Star to her every day.

The song was played at her funeral at South Shields Crematorium on Wednesday.

Mrs Hart, whose son Stewart, 33, was also diagnosed with the condition in adulthood, said: "She was a very special baby and she will never be forgotten."

On average, half the children of a women with myotonic dystrophy will be affected themselves.

If a child is diagnosed with the condition soon after birth, symptoms are likely to be severe and can be fatal, especially in the early weeks of life.

A child who lives beyond his or her first birthday is likely to live to become an adult, though.

Pre-natal testing is available at an early stage of pregnancy, but there is no cure for the condition.

CAPTION(S):

LOVING ARMS: Baby Sophie Elizabeth nears the end of her short life in the arms of her aunt Amanda.