Effie Hadman was diagnosed with Batten Disease in December 2013. She died in February 2017, aged just 6 years old.
Effie lived with her family - mum Carly, dad Paul, and brother George, who is now four years old, in Cambridgeshire. The family received care and support from EACH Milton during Effie’s illness and at the end of her life – here Carly and Paul share their story...
Effie started life as a perfectly healthy little girl. She loved walks around our local forest, singing and dance classes and playing with her friends. By the age of 2, Effie could count to 20 and knew all her shapes and colours. She had a wicked sense of humour, yet was the kindest and most gentle little girl imaginable.
When Effie was 2 and a half years old, she suddenly had a drop seizure in the bath. Over the following weeks, the seizures became more frequent and difficult to control. On the day before her 3rd birthday, Effie went to get out of bed and we discovered, to our horror, that during the course of one night she had lost the ability to walk.
Changes to her medication brought back the ability to walk, but this was only temporary. At this point, we noticed that Effie was struggling with her memory. We would go out for the day but when we arrived home, Effie would have no recollection of where she had been. She could no longer count or remember any colours or shapes.
In December 2013, Effie’s neurologist decided to test for Batten Disease, a rare neurological disease which would leave Effie blind and bedridden.
The life expectancy for children with Batten Disease is 6-12 years.
The results came back positive.
By the age of 4, Effie couldn’t stand and her speech was lost. She could no longer swallow and had a feeding tube fitted. Effie’s brain cells had started to break down, causing her to scream for hours on end.
We started to have more frequent stays at the hospice when Effie turned 5. She had lost the ability to sit up and hold her head up. Her sight deteriorated until she became completely blind. Effie stopped sleeping at night and her seizures were uncontrollable, meaning she needed 24/7 care. By this point Effie had lost all control of her body, which meant that she needed to be hoisted to be moved, as well as regular turning during the night. Staying at EACH meant that Effie was cared for overnight, safely in the hands of the EACH nurses, while we were able to get some much-needed sleep.
After Effie lost her sight we had to focus all of her entertainment around the senses she had remaining. She never lost her hearing or sense of smell, so we focused on activities using these skills. Effie’s favourite part of the week was a Tuesday morning, when the music therapist from EACH came to our home. Effie had started to lose her sight when we first started music therapy and we used these sessions to find out which sounds Effie preferred and learn new songs. When her sight had gone, we would play games of ‘hide and seek’. We would shake a cabasa or tap a triangle, and Effie would turn her head to the direction the sound was coming from. She was so proud of herself when she guessed correctly! As the disease progressed, Effie appeared to have little awareness of her surroundings - except during music therapy! We would tap out a beat on an ocean drum and Effie would tap the same beat back. Priceless memories, showing that our little girl was still with us. Despite her progressing illness, Effie’s love of music never changed. In a world where our princess was blind, bedridden and suffering severe brain damage, moments like these became priceless. Whether it was the games we’d play when Effie was having an alert day, or simply jamming along to Disney songs when she was too weak to do anything but listen, the music therapy gave us something to look forward to every week.
We had many overnight stays at Milton. George called it ‘Effie’s holiday home’, as he knew how much fun they both had there. It gave us precious time together as a family, playing in the beautiful garden and sensory room. We got to take part in some wonderful activities and outings, including ‘Dream Night’ at Colchester Zoo, and our whole family loved the Christmas parties!
We also received invaluable support from the Symptom Management Nursing Service, meaning that we could keep Effie out of hospital as much as possible. They helped us to create an end of life plan that we felt comfortable implementing when the time came, enabling us to have a choice of where Effie was to spend her final days, which was very important to us. The EACH Wellbeing team helped us to plan Effie’s funeral, explaining what the different options entailed and helping us to plan in advance, which meant we didn’t have to make many decisions at the most difficult of times.
At 6, Effie lost the ability to control her temperature, breathing and heart rate regulation. By this point she needed regular chest physio and suctioning, and up to 52 doses of medicine every day. Just after her 6th birthday, Effie went into a seizure that lasted 6 weeks. EACH helped us to bring her home and worked with us to prepare for the end of her life.
After 6 weeks, the seizure stopped and Effie was able to spend one last Christmas at home with her family. Effie passed away in February 2017, aged just 6 and a half years old.
People would often say ‘We don’t know how you do it’, when referring to caring for Effie. The truth is that, as parents, you just have to plough on and make the most of the situation. EACH enabled us to have some rest, to allow us to keep going. They would also take care of Effie so that we could take time to do ‘normal’ things with George.
The EACH Symptom Management Nursing Service were a great support to us on our journey. They would be completely honest with us about what was happening, or about to happen. Without their team, who were always on call when we needed advice, we would have spent so much more of the little time we had with Effie stuck in hospital.
To need to use a children’s hospice is every parent’s worst nightmare, however, when you get there, it is the most beautiful place imaginable. It’s a lifeline to parents going through the worst possible journey and enables families to find the support that they need. Most importantly for us, we were able to make some priceless memories that we will treasure forever.
To quote Winnie The Pooh, ‘We didn’t realise we were making memories, we just knew we were having fun’.