-Laura Finlay speaks to Sarah Speakman on PSP, the neurological condition that is hoping for a word in edgeways
By Laura Finlay
For many people around the UK and the rest of the world the term PSP may be misunderstood and thought to be a computer consol or something alike. This couldn’t be further from the reality. The term is actually an abbreviation for progressive supranuclear palsy, a terminal brain degenerative disease.
The very act of misunderstanding the term PSP is possibly one of the downfalls for the charity behind the condition, the PSP Association. The condition is rare, and the cause is unknown, neurologists believe that the number of those living with PSP in the UK is around 10,000. Because of this, there is a lack of awareness and a lack of education about the disease.
Sarah Speakman’s father was diagnosed with PSP at the age of 67. For two years his neurologist was unable to tell him whether he had Parkinson’s or PSP. “I was absolutely devastated by my father’s diagnosis… my wonderful dad would never get better and worse still, the progression of this cruel disease would little by little rob him of his personality, speech and dignity.”
The PSP Association, the hardworking charity behind the condition, work together with medical and health and social care professionals raising awareness and providing education of this rare condition. Raising awareness is one of the main aims of the charity as sadly there is no effective treatment or cure for the disease. Raising the charities profile and getting as much funding as possible is the best way that they can help this condition, by researching to try and find a cure for this devastating disease.
Progressive supranuclear palsy is associated with an over production of a protein called Tau in certain areas of the brain, this forms into neurofibrillary tangles which are believed to damage nerve cells. This leads to difficulty with balance, movement, speech and swallowing, and can cause rigidity, depression and memory difficulties.
PSP gets progressively worse over time, for the last 2 years of life; the patient is usually wheelchair or bed bound, and completely reliant on 24-hour care. The patient relies on being tube fed, and is unable to communicate with the rest of the world. Though the cause of death isn’t actually PSP on its own, the disease means that it increases the risk of pneumonia, choking from swallowing and recurrent falls.
Sarah’s father has become reliant on his wife, who is disabled and to whom he used to care for. As the roles reversed, this soon became too much for her mother and as Sarah’s father experienced recurrent falls, the subsequent injuries became more significant. Now they have a live-in carer as well as additional carers for support throughout the day.
There are currently no simple tests to diagnose PSP. Often in the early stages symptoms may be misdiagnosed as Parkinson’s disease, a stroke or a brain tumour, and sadly in the very elderly PSP often goes undiagnosed especially for those living in care homes. The average age of symptoms is 63, though there has been known cases in patients as young as 40. The average life expectancy is around 7 years from onset.
Though there are no effective treatments for PSP, and nothing that can be done to halt the disease, treatment is based around support and symptom relief. The PSP association provides a range of services to make sure that those living with PSP have as much access to information and care and support as possible.
A diagnosis for PSP is something that GPs would see once in three working lifetimes, according to GP Mike Baird. “Usually if a condition such as PSP were suspected there would be a generic referral to neurology-I doubt it if many GPs would diagnose it in their surgery.”
Doctor Baird highlights a prime concern surrounding PSP and other mental health issues and funding, “would you rather research money went to cancer or illnesses such as PSP or dementia?” Combining all types of cancer, 325,000 people were diagnosed in the UK in 2010 according to Cancer Research. Though this number is overwhelmingly larger than those diagnosed with PSP, it creates less funding from those who have chosen to support charities such as Cancer Research, and makes the gap for low profile charities harder to reach the public attention. This is something that Sarah feels little can be done about, “because there is no cure for PSP, there are no survivors to campaign vociferously as there are in cases of cancer. There is nothing we can do about this, of course, but it’s one of the reasons why we know so little about it.”
There isn’t much that can be done to combat this issue. Each charity, large or low profile is each as important as each other. The only solution to try and get more funding is to raise the awareness of PSP. High profile cases such as Lord Coe’s mother, Nigel Dempster and Dudley Moore has helped raise the charities profile.
Dudley Moore the English born Hollywood actor, comedian and musician was diagnosed with PSP in 1998, and died in 2002. Before he died, Moore went public with his disease and wanted to make a video in order to reach out to others who also had PSP. Moore started to experience symptoms in 1994, and became depressed at not being able to obtain a diagnosis; it took four years to discover what was wrong. Moore describes in the video how he misses playing the piano the most, “I love it, I miss it very much.” Moore wanted to tell his story because some people may be suffering without knowing what is wrong just as he did. His video has received over 86,000 hits.
Nigel Dempster, The British Daily Mail columnist tragically died in 2007 aged 65, after his diagnosis four years before. Dempster produced a YouTube video, ‘Life with Progressive Supranuclear Palsy’ where he describes what it is like living with PSP and the impact that it was having on his body, “It’s taken hold of me over the last year..I miss my freedom and my friends, I miss being able to laugh” The video has had over 10,000 hits.
The most recent high profile case of PSP is surrounding Lord Sebastian Coe’s mother, Angela Coe who died with PSP in 2005, aged 75. Since then Lord Coe, the former champion runner and Chairman of the London Organising Committee of the Olympic Games organised his first fundraising event in 2012 and raised over £200,000 for the PSP Association. In a Daily Main interview, Lord Coe described his experience of his mothers illness “It was a big burden on my family and on my father, who was a lot older than her and having to deal with his own frailties as well as become a full-time carer”. Lord Coe has supported the ‘Million to beat PSP’ campaign in 2012.
The PSP Association was registered as a charity in 1994 and was established by Mickael Koe and his wife Sara after she was diagnosed with PSP. The Koe family were shocked and upset by the lack of support, awareness and information available about PSP, and the lack of interest within the medical profession. After finding no organisation that specifically supported those who have PSP and the afflicted families, the PSPA was set up, and since has invested over £2 million in research.
Recent predictions show the population of elderly people growing and within this the number of the very old increasing. This creates a higher demand for care services, and health research is becoming more and more prominent. The Projecting Older People Population Information System (POPPI) has predicted that the population of England in 2030 aged 65 and over will make up to 21.46%. This could be yet another issue facing conditions such as PSP in the nearby future. As PSP is a condition most common within this age bracket this causes concerns for the number of patients with PSP to rise.
Though the PSPA are using social media affectively, Sarah Speakman thinks that the best way to raise awareness and raise the charities profile is to launch a campaign that is combined with other neurological conditions. “ If we combined PSP with Parkinson’s and other diseases that we are all familiar with, they would have greater media collateral, a more readily available media platform, better media coverage and therefore, hopefully increased awareness.”
As with many other hardworking charities, the PSP Association is determined to find a solution and to be able to eventually cure, and if not treat this devastating disease. Their research relies purely on donations, and for the public to merely understand the term PSP, is a step in the right direction.
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