Finding little in the way of specialist knowledge or support available to help her through her ordeal, Liz set out to provide answers for herself and others in her situation. What began as an informal network of family and friends quickly grew into a national charity which today supports over 1300 families. Run by a small team of dedicated staff backed by a medical board drawn from the UK’s top centres for mitochondrial disease research, The Lily Foundation forms a vital link between patients, doctors and medical science bodies.
On August 24th 2006, Lily was delivered by caesarean section 5 weeks early, as she hadn’t grown in the womb. Although very small, weighing only 3lbs 8oz, Lily was otherwise a healthy baby. She was admitted to the special baby care unit, and for the first couple of weeks Lily’s weight gain was good, but then it slowed down. It took Lily 4 weeks to reach 4lbs which was the weight she had to be for the hospital to allow her to come home.
On September 20th Lily came home. However, we began to get concerned over the next few weeks as she wasn’t gaining weight at all – she was still 4lbs. We were referred to a consultant for tests. Before the tests began Lily had a seizure and stopped breathing. I was out shopping at the time, and when I took Lily out of the pushchair it was clear that she’d stopped breathing. We called an ambulance and she was rushed to the nearest hospital. Lily began to breathe again by herself and so the hospital discharged her after a couple of hours. When we got home, we were uncomfortable and felt that something was very wrong with Lily. We took her back to East Surrey to the hospital where she was born. She was admitted and the tests started.
Something was wrong
After a week of tests, a chest x-ray was done on Lily which revealed that her heart was enlarged on one side. She was rushed to the Royal Brompton Hospital by ambulance to see a specialist cardiologist.
He confirmed a heart condition/heart disease but stated no urgency nor that Lily was in immediate danger. We were travelling back to our local hospital by taxi when Lily suffered another seizure. This time she required mouth-to-mouth resuscitation by a nurse accompanying us. Again an ambulance was called, and Lily was taken to the Chelsea and Westminster Hospital. She recovered well and was kept there overnight for observation. The following day we returned to our local hospital.
Two days later a doctor spoke to us about Lily possibly having a disease called mitochondrial disease. She told me that it wasn’t good news and that it could affect Lily’s development. Just hours later, Lily had another seizure. This time she experienced a cardiac arrest. She was transferred by the retrieval team to the Evelina Children’s Hospital at Guy’s and St Thomas’ Intensive Care Unit.
Nothing could be done
The following day the consultant in charge of the intensive care unit introduced herself to Dave and I. My initial meeting with this particular doctor will stay in my memory forever. She congratulated us on our beautiful baby girl. She then went on to tell us how concerned she was about Lily. She told us again that they suspected Lily had mitochondrial disease, that there was nothing they could do for her, and that she was going to die.
When we asked how long, they said probably days. In this day and age, it cannot be often that doctors have to tell parents of an 8-week-old baby that there is nothing they can do for them. There was nothing they could even try. As parents we just had to wait for our baby to die.
Lily had to undergo more tests. Her most important test was a muscle biopsy. This was taken to test for mitochondrial disease. however, we were told that the results could take between 6 and 8 weeks. This seems like forever when you’ve been told that your baby has days to live. We were introduced to the metabolic team and Dr Mike Champion, the metabolic consultant. Their advice in terms of how long Lily would live for was less definite than the intensive care team. They insisted that mitochondrial disease affects individuals very differently. However, they were more definite that Lily was not going to have a medium- or long-term future.
During her time in intensive care Lily was ventilated and dosed up with morphine. Over a week they tried to take her off the ventilator, but each time they turned the machine down Lily was unable to breathe on her own.
Eventually we decided it was time to take her off the machine. We were advised to get friends and family to come and say their goodbyes to Lily in the hospital, and we were moved to a private room so that we had privacy when she passed away.
A little miracle
However, Lily shocked everyone. When the machine was turned off Lily was able to breathe on her own and sustained a regular breathing pattern. She continued to breast feed, something that doctors told me she would never do again, that if she did survive, she would need to be tube fed.
From this point we had to make a decision – to leave Lily in hospital to die, go to a hospice with Lily and our other two children, or take Lily home. We decided to go home. Our other two children were 1 and 3 at the time and we knew that we didn’t have Lily for long, but that we wanted to have ‘normal’ family time with what we had left.
No one could tell us how long Lily was going to survive. Every day was a bonus, but also a very difficult way to live. We were basically waiting for our baby to die. Every night she slept on my tummy, because I couldn’t bear the thought of her dying on her own. I wanted her to know that I was always there with her.
Lily came home on October 30th 2006. We lived like this until the end of January. In January doctors were pleased with Lily, she had done much better than they had ever imagined. She was still very tiny, she hadn’t been weighed since knowing that she was dying, but I guess that she was well under 4lbs.
A sad goodbye
Doctors decided to put Lily on high-calorie formula milk and Lily responded really well. She started to gain weight and develop. She started to play with her toys, and we saw her smile. They also thought it was worth trying Lily with a heart drug to try to improve her heart condition. Unfortunately, this was unsuccessful. By the middle of April 2007, Lily’s weight-gain slowed again and she stopped smiling as much. She started to show signs of heart failure. We had hoped that she was going to be that miracle child, be the one child to recover from the ‘unrecoverable’.
Lily died in hospital on April 30th 2007.
Our thank yous
We would like to thank the medical teams that looked after Lily.
Despite losing Lily, we know that we are among the lucky ones. If Lily had died when I was out shopping with her that day, her death may have been put down to a cot death.
It may have been a similar story on the day she needed mouth-to-mouth resuscitation in the back of the taxi. If the nurse hadn’t shown such a high level of professionalism and skill, Lily would have died.
Finally, on the night that Lily had the cardiac arrest, a magnificent team of doctors saved her life. Once she had been diagnosed with mito, Lily had regular home visits from the paediatric consultant from East Surrey. These visits gave Dave and I hope, reassurance and, most importantly, strength.
The metabolic team at the Evelina Children’s Hospital were a great strength also. The specialist team were, and still are, always at the end of the phone to answer questions and give advice.
These acts of skill and professionalism allowed us the time to get to know Lily, spend some time with her, and watch her develop. Most importantly it gave us the time to learn about what was wrong with Lily. To learn that there was nothing we could have done to save her, that she did not die because of something we had done. It’s given us the opportunity to set up The Lily Foundation in Lily‘s memory. A charity that is going to find out more about mitochondrial disease, find more efficient ways to diagnose mitochondrial disease, and raise awareness of mitochondrial disease.
