Ella East celebrated her first birthday with a special party. It was a birthday that her Mum and Dad, Natalie and Jack, feared she would not see when she was diagnosed with Spinal Muscular Atrophy Type 1 and was seriously ill in intensive care in February of 2017. A few months on, the family has accepted that Ella will never go to school, but she is now at home with her older brother Aydan and new baby Charlie and is at the heart of family life. Here Natalie and Jack tell us how the help and support of the Rennie Grove children’s nursing team makes it possible for them to care for Ella and about how much it means for them to be able to make precious family memories together in their own home…
“When Ella was born after a normal pregnancy and birth, everything was absolutely fine initially but slowly we started to realise that she wasn’t progressing and that she was in fact doing less.
“We discussed it with our Health Visitor who didn’t seem to think there was anything wrong and it wasn’t until we took Ella to the doctor for a bad cold that concerns were raised. The doctor arranged for us to see a paediatrician who then quickly referred us to the hospital.
“It wasn’t clear what the problem was and all initial tests came back normal so they decided to do a full genetic screening.
“In the meantime Ella started to struggle with breathing and spent a few nights in hospital with bronchiolitis. We received the results of the tests on the day we got home. The doctors told us that Ella has Spinal Muscular Atrophy Type 1 and I remember them saying that there were no adults with this condition. They explained that it would get worse. I remember talking about her going to school; we didn’t realise at this stage that she would never go to school.
“She was still struggling with her breathing and it was decided that she needed to be admitted to hospital. We then did some research and that’s when it really dawned on us that Ella would not live for long.
“On top of our concern for Ella, Natalie was pregnant again and we wanted to know what it might mean for the new baby. We were offered a fast-tracked amniocentesis that was able to look for the condition and found out that Charlie was unaffected but that he was a carrier of the condition.”
“Ella spent two weeks in hospital so that we could get used to looking after her and when she was discharged we were sent home armed with syringes for her naso-gastric tube. While she was in hospital she had been regularly suctioned and by the morning after her discharge it was clear that she needed suction again and we had no way to do it. We had no choice but to call an ambulance. After this we were then given a suction machine but it really didn’t work very well.
“It was at this stage that the Rennie Grove Children’s Nursing Team became involved and Terry, one of the hospice at home nurses, visited us. He said the suction machine was dangerous and stepped in to source a machine that worked properly.
“We were at home for three or four nights but then she had a very bad episode and she was admitted to hospital again and put on a vapotherm machine which delivered high flow oxygen straight into her lungs. She also received intensive physiotherapy but her condition was deteriorating and the decision was taken to intubate her even though there was a chance she might not survive the procedure.
“We were desperate for her to be christened so before she was intubated we managed to gather together the godparents and a vicar. The ceremony was performed in theatre in the end - it was certainly not the christening we had wanted for her.
“Once she was intubated she was transferred to a specialist unit and there we met with a number of consultants. During the next 18 days she had three courses of antibiotics to tackle pneumonia, her lungs collapsed and she had intensive chest physiotherapy to get her lungs in the best possible condition.
“Her lungs were still struggling though and all the specialists told us that they didn’t think she would survive extubation. They wanted to take her to the local children’s hospice and extubate her there. We did go to look around the hospice but we never doubted that she would survive and in the end we insisted that she was extubated in hospital.
“The extubation was delayed by a day so that Natalie could go for her 20 week scan and we were given a private room and were able to invite the family over and bring Aydan in to spend some precious time together. We also told Aydan and Ella about the new baby that night. We wanted her to know in case she didn’t survive.
“When the time came to extubate the doctors wanted to give Ella every chance and they gave her a dose of steroids before leaving us for two hours together. They then took the tube out while Ella was in our arms. They kept coming in every half an hour to turn the oxygen down and obviously expected to see Ella struggling, but, against all the odds, she was doing ok.
“We were then transferred to the local children’s hospice and both Ella and Aydan loved it there and Terry visited us too. We stayed for a few days but really wanted to set a date to go home.
“Terry was absolutely brilliant at helping us to get home. The last time we were discharged from hospital we were badly prepared and there was no follow-up, no equipment and no-one to contact. Terry actually made sure he was here when we got back from the hospice – he was waiting for us in his car. He helped us to get all the equipment in and set up and spent hours on the phone getting it all right.
“We’ve had a couple of hospital trips since being home for chest infections, but every time Terry would visit us and look after us all.
“Ella has deteriorated naturally. She is now oxygen dependent and needs a bipap machine at night but we have all the equipment here now – everything we would have in hospital -and we know how to use it.
“If it hadn’t been for the Rennie Grove children’s nursing team it wouldn’t have been possible for us to be at home. They made us feel comfortable and confident. If we have any concerns – day or night – they come straight out. They have been absolutely brilliant.
“It means everything to us to be at home. Although the hospice was lovely, we didn’t want our memories to be there. We wanted to make our memories together at home.
“It is so much better for Aydan for us to be at home and the regular visits of the nurses frees up time for us to spend with him, playing football, making cakes or collecting him from school. And now that Charlie has arrived too, it is even more important for us to be here.
“Ella loves being at home too surrounded by her toys, her favourite films and her brothers. She hates being in hospital.
“Ella is now getting extra support from carers and Terry has been a wonderful source of advice about what help we qualify for. He knows the system and is able to guide us through the maze. Housing is a problem for us now as our two bedroomed maisonette isn’t big enough for the five of us and all the equipment we need and Tina, another Rennie Grove nurse has helped us by phoning and putting our case to the Housing Association.
“We also value Terry as someone to talk to. He is like a friend of the family and he even came to Ella’s birthday party.
“There has to be two of us in the house at all times to deal with Ella’s care because her oxygen levels often dip with no warning. But we know what to do now. There are alarms on the machines when this happens but we don’t really sleep properly any more.
“Even though we get more help with the carers now, we couldn’t do without Rennie Grove. The carers go home at 6, the community nurses finish at 6, but the Rennie Grove children’s nursing team is there 24/7. They will always come if you need them and they don’t mind even if it turns out that there’s nothing wrong. It’s so reassuring to know that you’re not alone, particularly in the middle of the night.
“The upheaval of going into hospital is massive and Rennie Grove has helped us avoid this on numerous occasions. Sometimes you just need someone qualified to offer practical help and advice.
“This year has undoubtedly been the worst year of our lives but the Rennie Grove children’s nursing team has made us feel that we are not alone. We did feel alone before, especially that first time we were discharged from hospital, but Rennie Grove changed that. We now plan to stay at home and continue making memories together for as long as possible.”