As Natasha Clarke held her newborn baby for the first time she felt herself brimming with pride.
Ava, born a healthy 7lb 2oz, looked perfect and was the daughter the former Vogue model and singer had always wanted.
'She was just beautiful,' says Natasha.
Natasha's pregnancy had been normal, and the birth itself was straightforward. It was with huge happiness that she and Ava went home the day after the birth.
But already in those idyllic first hours a terrible story was unfolding.
Ava had been born with a genetic condition called methylmalonic acidaemia (MMA). This meant her body didn't produce an enzyme to break down protein.
In the womb, Natasha, 33, had been breaking the protein down for her.
But on her own, Ava's tiny body was unable to cope with any protein and even her own mother's milk was highly poisonous, leading to a build up of toxic substances, methylmalonic acid and ammonia. Untreated, it can lead to a coma, brain damage and death.
In America, newborns are routinely tested for this condition. There are no such tests in the UK and Natasha and her husband Grant, 37, were unaware of Ava's condition.
The first time they suspected something was wrong was when Ava was three days old and she stopped feeding properly. But reassured by the midwife that this was completely normal, they kept Ava at home.
But, by the next day, Natasha says maternal instinct - the couple already had a son, Harrison, then three - told her something was wrong and she arranged to see the midwife again.
'I told her Ava really hadn't fed very well. She would startle to loud sounds but she was not waking up. Her body felt a little bit rigid.
'I could see the midwife was slightly concerned and when she took Ava's temperature, it was only 35C, worryingly low. I realised then it was serious.'
Natasha immediately drove Ava to Frimley Park hospital, Surrey, where she was born. There, doctors carried out a series of tests.
'They put a needle into her and she didn't flinch,' recalls Natasha. 'Then they asked me if I was related to my husband - which of course I am not. I think they were trying to rule out a genetic abnormality. They then started treating her with intravenous antibiotics to try to get her temperature up.
'I sat there expressing milk for her because I thought she'd get better'. Little did she realise that her milk was toxic to her baby.
By then Ava's condition was deteriorating rapidly and it was clear she was gravely ill. 'When I asked the doctor: 'Is she going to die?' he told me: 'I hoped you wouldn't ask me that.' I couldn't speak.'
Ammonia levels in Ava's body were off the scale - a normal reading would be around 20, hers was more than 1,000 - a sign that her body was being poisoned by the by-products created by unbroken down protein.
Doctors desperately needed a drug to clean her blood and bring down the ammonia levels and, with the readings getting higher each time, every minute counted.
But a frantic attempt to find the medication needed in the local hospitals failed and Ava didn't receive the drugs until several hours later, when specialists from London's Evelina Children's Hospital arrived to transfer her to their unit.
'I am sure it would have saved her life if she'd had the drug earlier,' said Natasha.
When the drug didn't bring down her soaring ammonia levels doctors put a tube into Ava's stomach to try to flush the blood that way. But it was to no avail: a scan of Ava's brain showed it was no longer functioning.
And, just hours after she'd arrived in hospital, the doctors said they would have to turn off the life support machines.
'We asked them how long Ava would live without the machines, they said about an hour. But Ava stayed with us for another 12 hours until 6am the next day. She was only five days old.
'During those last hours she had her eyes closed most of the time.
Nurses brought us a camera so we could take photos, and we took a lock of her hair and did hand and footprints. Then, while I was holding her, she opened her eyes and had a really good look at me. Moments later she died.'
Ava suffered from the severest form of the condition. Had she survived, she would have been severely brain damaged because her ammonia levels reached such high levels.
'I would have taken that over a dead baby,' says Natasha quietly. 'It's your worst nightmare going through the birth, the joy of having a baby girl, going home with what you think is a healthy baby and then, days later, watching your baby die.
'I'd have traded every success I'd had in my life to have my daughter alive and healthy today.'
Ava's symptoms developed within days of birth. However, other children are often not diagnosed until they are two or even older. With careful management, including a strict low-protein diet people can live with MMA (kidney problems are common, and some need an organ transplant).
As Dr Mike Champion, a consultant in paediatric metabolic medicine at the Evelina Children's Hospital, explains: 'The earlier you make a diagnosis the more you can tailor the treatment to reverse the problem.'
After their baby's death the Clarkes faced another ordeal. MMA is caused by a defective gene passed on by both parents - though the chances of getting together with someone who has the same faulty genes to create a condition such as MMA is one in 50,000, Natasha and Grant's risk of another child with MMA are high, one in four.
One way of preventing this was to have IVF - doctors could have checked any embryos against Ava's DNA to see if they were affected before being put back into the womb.
But this option was not available - the biopsy taken from Ava after she died was contaminated.
So, when she became pregnant again, Natasha had to wait until she was 17 weeks pregnant before doctors were able to determine from blood tests whether the baby was affected.
'I kept telling myself if I could do it once with Harrison I could do it again. I knew that if the baby was affected this was treatable but, thankfully, it was good news - the baby was free of MMA.'
Austin is now 11 months old.
Following Ava's death on August 18, 2006, Natasha and Grant set up the Ava
Clarke Foundation in her memory to raise money for children affected by life-limiting inherited genetic disorders.
One in 800 babies is born with one of these conditions, which also cause 40 per cent of neonatal deaths.
The Clarkes are trying to raise money towards the cost of a new piece of equipment known as a tandem mass spectrometer (TMS) for the Children's Metabolic Unit at the Evelina Hospital.
These machines can identify rare metabolic conditions, but there are only a few in the UK.
The current TMS machine at the Evelina Children's Hospital has analysed nearly one million samples as well as helping with research. But the latest model, which the Clarkes are hoping to help fund, is more sensitive than previous models and will radically increase the range of genetic disorders that can be diagnosed from a single blood sample within 10 minutes.
'We want something positive to come from Ava's death,' says Natasha.
'And the Foundation will raise awareness of all types of genetic disorders as well as raising money for new equipment to help diagnose and treat children like Ava.
'I am so grateful to have two healthy boys but you never get over the death of a child. When you carry them for nine months, you are planning their future and thinking about their name and how they will fit into the family.
'On Ava's second birthday, I sang happy birthday to her picture. I will never watch my little girl go to school. Losing a child is like losing part of yourself. You learn to live with it but you never get over it.'
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