Although we always knew it could happen, seeing the rapid change in Diana from the beginning of 2016 was a huge shock and an experience we will never forget. It’s hard to witness the person you know and love changing before your eyes, almost on a daily basis.
At the beginning of 2016 Diana was working and driving although colleagues noticed that she looked very tired and thin.
She was presenting with the sort of symptoms you would associate with long hours and stress but we know now that wasn’t the reason.
Friends at work noticed that Diana was a little clumsier – she struggled to pick up a pen and fell off her chair. She was losing weight – perhaps forgetting to eat.
She began to struggle to follow the thread of conversations if they went off at a tangent. Diana lived alone so it was only those who saw her every day that noticed the subtle changes.
Diana began to feel nervous driving and stopped. She confided with close friends that she felt unwell – lacking in energy and focus and we found she was anaemic. She took sick leave from work and colleagues worried that maybe she was depressed.
Her speech began to slur, texting became very tricky and she felt that her brain was ‘foggy’. We went swimming one day and she just adored the feeling of free balance and movement again. Walking became difficult without support.
Additional symptoms included trouble with coordination and balance; slurred speech; difficulty remembering arrangements or simple plans; mood swings and high anxiety; fixated thinking; becoming repetitive.
It was at this stage that we talked to Professor Andrea Németh; Associate Professor at the University of Oxford and Consultant in Neurogenetics at Oxford Centre for Genomic Medicine. Andrea was familiar with the family's history and in particular of Judy, Diana's mother.
She was able to talk to us about what we might expect over the next few months. Although it was a painful and difficult conversation to have, we so appreciated her ability to talk to us plainly but with genuine kindness and care. It was Andrea who referred us to the MRC Prion Unit and Professor Simon Mead.
Diana was tested for Gerstmann-Straussler-Scheinker (GSS), and it came as no surprise that she had a positive result.
Diana now needed help with most tasks including cooking, shopping, household chores, washing, dressing and toileting. All this help was needed but Diana was unable to understand why. She became suspicious and sometimes angry. We hated to see her so agitated and distressed. It was a very difficult time. Her appetite changed completely and food became almost an obsession and a great comfort and pleasure.
Time keeping was lost and strange worries became consuming. Close friends set up a rota to check in on her at meal times and took her out as much as they could, but by the middle of the month, she couldn't be left alone.
The support and guidance of Professor Simon Mead from the the MRC Prion Unit and the National Prion Clinic was invaluable. With his backing, we arranged a multi-disciplinary team meeting with a full range of health and social care professionals.
Our aim was to make the best decision for Diana. We all agreed that Diana would have wanted to be cared for in her own home. The only way this could happen was with home adaptations and a more specialist care package.
Later stages (May onwards)
Diana soon became bed-bound but perhaps happier to receive help. The last month or so were plagued with swallowing problems, seizures and pain. We worked daily with the district nurses and hospice staff to make sure she had the right medication to control symptoms and we believe that bought us more time to enjoy Diana being peaceful and content.
Diana died on September 4th on a warm Sunday morning with her brother and best friend since primary school Marjie by her side. She was peaceful and pain free - we will always get much comfort from that.
We can’t thank enough all the health professionals that helped us in those six months. We are forever grateful for their expert help, emotional support and kindness throughout the most heart-breaking time of our lives.
We loved our time with Diana: Feeding her, watching films and listening to her favourite music. We had a constant stream of visitors and our focus was always to keep her home full of love, laughter and happiness. Caring for the kindest and gentlest person we knew was a privilege and we miss her every day.
Find out more about Gerstmann-Straussler-Scheinker (GSS) >>
If you would like any more information about the experience of caring for someone with GSS or if it might help you in your situation - please do contact us. We’d love to hear from you.