“Only connect the prose and the passion, and both will be exalted, and human love will be seen at its highest. Live in fragments no longer”
E.M. Forester, Howards End
I’ve been giving a lot of thought recently to why I volunteer. What am I trying to achieve? What does the charity want to do, and how?
Who gets what out of this- the hours and the energy that nobody pays us for? The work that nobody else sees or cares about. The work and the emotion that has Spurs Fan wondering at times why I don’t just give up.
Connection, story telling, getting stories heard. That works for me. I believe in what I do. It drives me batty sometimes, but my work has value.
Connection is vital to us all. Each of us important and our stories cannot be dismissed. We all have our place in the family of things.
Part of the volunteering I do is talking about Progressive Supranuclear Palsy, the rare neurological condition my late mum had. Below is the text of the first talk I gave, a few months after she died. Some days, and today is one of those, the full force of it comes back to hit me. Today I am licking my wounds; tomorrow I'll plough on with the current world, and walk beside the families dealing with PSP right now.
This is a story of my mum, Ann, and our PSP experience. While this is an individual story, you’ll find traces of many of the points raised today; delayed diagnosis, lack of local specialist support, rare disease not rare care needs, systemic problems and the ongoing education of health professionals by families.
Ann was widowed at 50, lived in Spain for a while, came back to Omagh, worked and got remarried. By 60, she had grandchildren, a happy home life and would drive to Belfast on a whim to get her hair done, to babysit, or to bring one of her elder siblings for hospital appointments. She was very active; playing golf and indoor bowls, attending set dancing and yoga.
Ann had a few unexplained falls, resulting in black eyes, cuts and bruises. She didn’t feel dizzy- she just fell. These falls were unpredictable and she stopped going to yoga and dancing after falling in public and becoming increasingly self conscious. She got prisms on her glasses to deal with double vision in Jan 05, and stopped driving. Ann saw her GP regularly, but talked about dry skin, or constipation, or anything at all to avoid mentioning the falling. I was talking to my mum a couple of times a week on the phone and began to notice that her speech was a bit funny- as if she’d had too much wine with her meal, or had got new false teeth. Except she didn’t wear false teeth, and hardly ever drank wine. Eventually, I realised that she’d never told the GP about her series of falls (this, even though one fall had broken her ankle requiring surgery and 2 weeks in hospital).
I went with her to the GP in July 06, and helped her outline our concerns about the falling, the speech and the double vision. Blood tests were carried out and a referral to the elderly care consultant was made. He ordered a CT scan, and then made referral to a neurologist. In parallel, the orthoptists were realising that the continuing deterioration of her eye movement was probably caused by something neurological, and would need to be checked out.
By the time Ann saw a neurologist in Jan 07, she was desperate for a label. Something to call whatever was wrong with her. Once she had a label, then she’d get the medicine and all would be well. She’d not be a hypochondriac if she had a label. People would take her seriously if only there was a name for this.
She had an MRI and saw the neurologist, but still had no label. We had never considered the possibility that her condition may be difficult to diagnose. We were new to the world of neurology and had no frame of reference, no experience to build on. We fully expected a process of get diagnosis, get treatment, get better. We were baffled by the absence of an immediate diagnosis and treatment plan. Ann went to see another neurologist privately in June 07. By this stage her symptoms included unsteadiness/staggering, memory and concentration difficulties, difficulty getting up from sitting and with handwriting. We were told that she had a progressive condition (we had to check what that meant) and given the label ‘Progressive Supranuclear Palsy’. I drove us back to my house, and put on the kettle and the computer at the same time. By the time the coffee was ready I had to lie to Ann and tell her I’d only found heavy medical jargon, and would look again later.
The GP knew no more than we did, telling us to look it up on the internet. I’d already done that and then was in the unwelcome situation of having to give my mum some of that information- as if I knew anything!
Anything I did know I’d printed out from the PSP Association website. I copied this information to my mum’s brothers and sisters and to her close friends. This meant that she didn’t have to keep explaining her diagnosis, and that those who care for her knew as much as we did. We joined the Association and were quickly contacted by them. The staff of PSPA have been an incredible support. They became our first port of call for queries and concerns; the availability of support at the end of a phone has been invaluable.
Ann was referred to social services, and in order to provide the best care, so was her husband, Liam, by now diagnosed with Alzheimer’s Disease. The social worker organised meals on wheels and carers were provided to help Ann get up and washed and to get to bed. After some months, this was increased to 5 visits per day. The support was needed and very welcome, but still didn’t meet all the needs.
Neither Ann nor Liam was fully able to recognise all the challenges facing them, and it felt like the home situation was very precariously balanced. We were just waiting on a crisis. There was an eldercare neck alarm, adapted bathroom, wheelchair, grips and other OT aids, riser chair, door buzzer- the full works. All of these things are great, but nowhere near enough on their own. When Ann took a notion to wander down to the bathroom herself, then she’d just go. Reckless behaviour, simply not recognising the reality of the situation or seeing the potential for danger, is part and parcel of PSP. We had multiple hospital visits for head wounds, stitches and x-rays. We ended up teasing Ann about having a rubber head that just bounced, and avoided serious injury.
Liam was involved in an accident and was in hospital for some weeks. I moved in until respite care was sorted, and I was exhausted! Despite the array of carers, Liam was still doing an amazing amount for Ann, and he’s an elderly man with significant health issues. I’m over 30 years younger and I was struggling after only a few days.
Ann went into a nursing home in her local area. We realised very quickly that the quality of care there was significantly better than anything we could organise for the home environment. Ann visibly relaxed, so we recognised just how really tense and anxious she had been. However, once Liam was fit to go home again, Ann wanted home too. We had a really difficult period where basically I became the villain of the piece, insisting that she stay in nursing care. Of course, she could have moved out if she’d been convinced, I couldn’t keep her there! Ann was always content with the care and with the nursing home, but she didn’t like the idea of being in a nursing home at 66. Who would? Soon, Ann was unable to do anything for herself. She needed help eating, moving, personal care. She couldn’t use a phone or a remote control, and couldn’t lift anything from a surface without knocking over everything else.
We arranged for Ann to use the Door2Door service to travel to the family home 4 afternoons a week, and paid for a carer to be there for 3 hours at a time. She had a few falls in the nursing home, like when she decided to get out of the wheelchair for something, so we got an alarm for the chair. At one point the nurse wheeled her round with him, as the best way to keep her supervised. Being very sociable, she enjoyed that.
Ann had a PEG tube for feeding inserted, requiring 2 nights in hospital. We were fortunate that it was a local hospital, so she didn’t have a long way to travel, and could still have visitors, but she found being in a ward, away from carers who knew her and her condition, to be distressing. Within a few weeks of having the PEG, Ann was much brighter, her speech was stronger, and she could articulate more complex sentences- when I was ending a phone call with ‘Be good’ she responded ‘I don’t often get the opportunity to be anything other than good’, when I was expecting a simple grunt! As her condition progressed, she lost that, but it shows how much of herself was still there.
Due to limited eye movement, Ann’s vision became very poor, her eyes were almost closed, and she had difficulty tolerating light. 2 carers would take her for walks along the corridor, but she was in the wheelchair the rest of the time. She was unable to open her jaw, which also created challenges for her oral health. Her speech became more and more difficult to understand, but that never stopped her communicating with us. When there was something to be said, we knew about it! I still phoned several times a week, but couldn’t understand anything she said- we needed to be together for that to work- but she could understand me alright, and wanted to be kept up to date on all the news and gossip. Her processing slowed- as if she were a few sentences behind you in conversation- so we had to pause and give her time to catch up. She was always keen to know what was going on, to spend time just being with people, to enjoy time with the grandchildren, to be part of family life. From time to time her thinking was confused and she had delusions, but generally she was sharp, knew what was going on and was able to convey her feelings about it.
PSP is a very individual disease- each person experiences symptoms in a different combination or in a different order- his/her own cocktail. Symptoms progress at different rates. The detail of an individual’s deterioration is unpredictable, and presents all sorts of challenges for care, support and management. But deteriorate they will, and they will need support.
Health professionals may never have met a person with PSP. This adds to the difficulties in getting appropriate and timely treatment. When Ann has fallen out of a ‘list’ and needed treatment, we’ve had to ask and ask for re-referral to e.g. occupational or speech therapy. Although I wasn’t a full time carer, and lived 75 miles away, Ann’s care took a remarkable amount of my time and energy. There was always something to be chased up, queries to be made, buses, carers, doctors or therapists to be phoned. Any change in arrangements required about 5 phone calls- not only to transport providers and carers, but to Liam and to his sisters, because he wouldn’t remember the call. Also, because of Ann’s inability to write, I’d had to be given Power of Attorney to deal with the finances and so paid all the bills, dealt with pension providers, benefits, banks etc. My diary was full of peripheral tasks associated with my mother- shopping, house maintenance, form filling, or even getting new lights for the Christmas tree. I found out where to stay in Enniskillen, where to get pizza in Omagh, where the cash machines are. These tasks are not hands on personal care, but they are the boring things that need done by somebody when there’s illness in the family. Nothing about these is rare. Because Ann’s disease was rare, I had a supply of PSP information packs which I left with medical staff in wards, in waiting areas and health centres. I left contact details for the specialist nurse and the website address. I talked about PSP to anybody who would listen.
In June 2011, Ann was still going to her own house when she was fit, but now needed two carers to be there. The OT began to have concerns about her travelling by bus or taxi in a wheelchair. She had a few low weeks where she was in bed a lot and barely able to communicate. She had emergency hospital admissions due to chest infections and subsequent difficulty breathing. Medical staff weren’t familiar with her condition, which caused them concern. They were happy for us to be in the hospital almost full time, even when her condition was improving. Antibiotics worked for a while and Ann was in good spirits about returning to the nursing home-she was delighted to be back amongst people who knew her and cared for her, the people who had saved her life- and, of course, she was looking forward to me getting her some new clothes.
I bought the clothes, but she never saw them. Ann died in hospital in July, of pneumonia. She was comfortable and pain free; that’s as much as we can hope for with PSP.
“Once you get to fifty you become young again.” said the friendly GP, “Young to die.”
I told him I might need to change practice. We laughed, but I knew what he meant.
I realised recently that I remembered clearly the 50th birthday of each of my parents. Each day was defined by the old man’s health. On his birthday, he went to hospital for a minor procedure, which became major surgery, Christmas on a protein drip, and a cancer diagnosis. Two years later he was in a different hospital, pleading to get out for a few hours to help herself celebrate her 50th. That didn’t happen. The old man was young to die, at 52. Herself was still young to die, at 68.
Many of mine are long lived, but nobody has reached a century. I’m past the middle.
So we gathered together, the Belfast based McSpecs and the Scotland based McSpecs. We swam and lounged and laughed. We walked and ate and looked around us at loveliness. Children played, bounced on all the beds and had adventures with a golf buggy. We went to bed at a reasonable hour and were dressed, sociable and devouring cooked breakfasts by 8.30 am. Eventually we had to come home to an eccentric dog and real life.
In a few weeks, there will be no sedate loveliness, or early bed. There will be a wee room, a bar and a dance floor. Spurs Fan is compiling a playlist. There will be a gathering up of friends. There may be silliness.
Family, friends, togetherness, celebration- what’s important in the world.
It’s PSP awareness week. The long term reader will have noticed me mention Progressive Supranuclear Palsy before: it’s what my mum had; it’s why I was at the Houses of Parliament recently; it’s why I’m interested in rare disease. PSP shaped my mother’s life; it’s shaping mine too.
Herself was in her early sixties when she took to falling. She had double vision too, but that didn’t seem to be why she was falling- she wasn’t tripping, just ending up in a heap on the ground. Her mood wasn’t great, and her confidence dipped. She stopped driving and dancing and playing bowls and playing golf and doing yoga. She went to the doctor every few weeks, but never quite gathered herself to tell him about the falling. We began to wonder about her speech…
I went with her to the doctor. A scan, MRI, consultant appointments followed. Eventually we ended up with a neurologist, and were innocent enough to still expect a diagnosis and a tablet. Later still, we had a diagnosis, but no clue.
If I read the internet right, my mum would end up in a wheelchair- maybe bed bound?- have difficulty with speech and swallow and communication and vision and continence and, and… No. That couldn’t be right. Ok, she wasn’t quite herself, but surely a dynamic, vibrant woman wouldn’t just fall apart and be bed bound and unable to communicate without something dreadful happening? But the dreadful thing was already happening, inside her brain. There was nothing anybody to could do to stop it, or slow it down. We just had to work out how to live with it, how to ease things, how to hold her hand and be with her through this.
Four years after the diagnosis, my mum died. She was indeed bed bound and hardly able to communicate, but in her last days we got gifts of a hug, a smile, a shaken head, a thumbs up. She was still there, still making her opinions felt, still wowing us with the force of her will. She’d spent 2 years in a nursing home, challenging systems, causing havoc by falling out of bed and refusing to press the buzzer, singing while she could, continuing to be indignant at living with the old people, continuing to believe that she should be at home looking after Handsome Husband, who’d been diagnosed with Alzheimer’s.
PSP always wins, because once the progression starts, it keeps on going. Sometimes there’ll be a big change, sometimes little bits, but the dreadful thing keeps on going.
My mum had to deal with the many, overwhelming aspects of living with, and dying from PSP. I have one challenge this awareness week.
I dug out the ancient, hardly worn, trainers and swapped a lace. I’m wearing #EdsLace all week. I will not be stylish, but that’s no change.
#EdsLace is one of those simple, clever ideas. The coloured lace can be bought from PSP Association. It can be worn as a shoe lace, a hair band, bracelet, anything at all. The key thing is that the end is cut off. The lace will fray. Sometimes there’ll be a big change, sometimes little bits, but the lace will keep on fraying. We can’t stop it or put it back together. The lace progressively falls apart, representing the impact of the dreadful thing on those who live with PSP.
So if you see an oddly laced person this week, chat to them. They will have a story to tell.