I don’t look forward to the day when I may be unable to swallow the Holy Communion which the priest will bring me, writes Kieron Wood
It’s estimated that five in every 100,000 people over 50 are affected by progressive nuclear palsy (PSP). I’m a male aged 70, so I’m in the most common target group for this rare disease – and I have PSP.
Following a piece I wrote about my illness, I received a card saying the sender’s mother had died of PSP. But more men than women die of PSP, and they tend to be older. As the population becomes older, it is sure to become more common.
It’s called PSP because it’s progressive, it damages parts of the brain above the pea-sized nuclei that control eye movements and it results in palsy (the disease Jesus cured). It’s caused by the death of nerve cells in the brain.
PSP does not affect everybody at the same rate. Early symptoms may include loss of balance and unexpected falls (normally backwards – I spent several days in the Queen Elizabeth teaching hospital in Birmingham following a backwards fall down my sister’s stairs, and the hospital produced a comprehensive copy of my notes on discharge).
The difficulties can include sensitivity to light and a syndrome where the eyes go ‘round the houses’, instead of going smoothly up and down).
Some people can experience depression or cramped handwriting. (My writing has really slowed down – but is bigger, rather than smaller – and the necessity of filling in complex forms means I had to give up advising in the Free Legal Advice Centres in Dublin.)
My voice occasionally goes quiet, and speech and swallow problems can develop as the condition progresses. Falls become more frequent.
It all started more than three years ago with problems typing. The left side of the brain (the speech area) controls the right side of the body, and vice-versa. A consultant psychiatrist spotted that I held my right arm at an unusual angle, and referred me to the Dublin Neurological Institute suffering from slowness of movement.
Prof. Tim Lynch, the State’s leading expert on so-called ‘tauopathies’ (the involvement of tau proteins) said: “If I had to say it’s anything, I’d say it’s PSP.”
PSP is a form of Parkinsonism, related to Parkinson’s Disease (which Pope St John Paul II died of), but without the tremor. The microtubule-associated protein tau (the MAPT gene) is found throughout the nervous system, including in nerve cells in the brain. If the MAPT gene mutates, changes can occur that result in abnormal clumping of tau proteins. But no definitive cause has yet been found – though Prof. Lynch says he sees a disproportionate number of farmers suffering from PSP. So maybe the cause is environmental, something to do with weed killers?
PSP is difficult to diagnose, but eye problems give a clue. Symptoms may mimic those of other neuro-degenerative diseases, such as Parkinson’s Disease or Alzheimer’s (though there’s a difference between tau and amyloid protein in the brain, present in advanced Alzheimer’s). Clinical assessment involves a battery of tests, including a lumbar puncture in the day clinic of the Dublin Neurological Institute (the fluid has to be sent to Britain for a result), several MRIs and a PET scan.
The report said my phosphorylated tau levels were increased. A paper published by the ‘Frontiers in Neurology’ programme said: “Aberrant tau phosphorylation is acknowledged to be a key disease process, influencing tau structure, distribution and function in neurons.”
I don’t look forward to the day when I can’t write any more, and may be suffering from dementia and unable to swallow the Host which the priest will bring”
There is no effective treatment for PSP, so managing symptoms is essential for controlling the progress of the disease.
Swallowing difficulties increases the risk of aspiration pneumonia. Regular exercise – physical and mental – is vital. (I do Sudoku and go for a walk most days. My two elder daughters assist me in this.) It is also important to see an experienced neurologist to get a definitive diagnosis, though Ireland has a marked shortage of neurologists.
The HSE community services can help. Among other things, I had a second set of banisters fitted in my home to prevent falls on the stairs, and the occupational therapist referred me to a physiotherapist, a public health nurse and a speech and language expert.
Everyday tasks such as tying your shoelaces, pressing the buttons on the TV remote control or even personal hygiene issues become increasingly difficult”
There’s also a group which supports people with PSP and their carers: the PSP Association Ireland, based in North Brunswick Street, Dublin 7, which organises events and monthly meetings.
I’m also suffering from Cortico-Basal Degeneration (CBD) and Primary Progressive Aphasia (PPA), which means I do not know what I was going to say – though I have an app which expresses my emotions for when I lose my voice entirely. The brain changes in PSP and CBD are similar, but not the same. CBD is a degenerative brain disease, which often starts with progressive numbness.
Everyday tasks such as tying your shoelaces, pressing the buttons on the TV remote control or even personal hygiene issues become increasingly difficult.
Eye issues are normally less debilitating than in PSP. Fortunately, my daughter (who lives in Madrid) is a speech and language therapist, so she helps out by Skype.
I don’t look forward to the day when I can’t write any more, and may be suffering from dementia and unable to swallow the Host which the priest will bring.
All I can do is what newspaper columnist Nigel Dempster did in his video on YouTube – pray. I go to Mass with some of the children every Sunday (at St Kevin’s, Harrington Street) and I consider that, if Jesus cured a man of palsy, he can do it for me. It’s the only hope I have left.
Kieron Wood is a former RTÉ Religious Affairs Correspondent and a family law barrister.