Ellen Rossiter speaks to Andrew Symons,  Chief Executive of PSP Association, about the work of the association and his hopes for the future.

Imagine striving daily to raise awareness of, and to find a cure for, a condition which few have heard of and for which there is no funding from central government. Such is the lot of the dedicated and energetic team at the PSP Association (PSPA).

The who? PSPA is the only national charity offering advice, support and information to people living with Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration (CBD) while supporting research into treatments and ultimately, a cure.

“It’s all about awareness,” comments Andrew Symons, Chief Executive of PSP Association (PSPA), “raising awareness is our biggest challenge.” When explaining where he works, Andrew is often met with consternation and bemusement – so building awareness amongst the general public, medical professionals, and the administrators of medical and related records is a key focus of their work.

PSP and CBD are extremely rare, many medical professionals will go through their entire career without coming across a case, and therein lies their challenge both in raising awareness and in procuring funding. PSP is estimated to affect about six or seven people in every 100,000. Yet understanding and finding a cure for PSP and CBD would not only be a breakthrough for those with these conditions but will deepen our understanding of other related conditions too, including Alzheimer’s and Parkinson’s.

For those not in the know, PSP and CBD are cruel and devastating neurological diseases, caused by the progressive death of nerve cells in the brain, leaving people unable to balance, walk, talk, eat, swallow, drink and see. Typically, people live between five and seven years after the onset of symptoms.

PSP and CBD are associated with an accumulation of a protein called tau in certain parts of the brain; tau is also associated with Alzheimer’s and Parkinson’s. Whilst there are differences between PSP and CBD, many people with CBD do develop features of PSP and vice versa.

An estimated 6,000 people are living with PSP and CBD in the UK, but this number could be more than 10,000 as many are misdiagnosed with other conditions. There is currently no treatment and no cure, but there is hope in the shape of the work undertaken by the PSPA, their supporters and the researchers with whom they work. “Our goal is a world free of PSP and CBD and our three-year strategy is to help people with these conditions lead the best life they can,” continues Andrew.

Andrew came to the role from a commercial background, having worked in change management, corporate services and run his own company too. Having confirmed the sale of his company on a Tuesday, Andrew saw the advert for the Chief Executive’s role on Thursday and duly applied, joining the team in 2017. Andrew had a desire to give something back, but his professional goal also reflected a personal motivation, for Andrew’s father had PSP.

PSPA provides information and support for families and helps them access assistance from statutory and other public or voluntary bodies. PSPA is making a difference; greater than 3 in 4 of the users of their frontline services rate them as “very good or excellent”. PSPA is currently supporting 1450 people who have PSP, 289 people with CBD and a further 2,423 people who are supporting someone with these conditions.

Raising awareness amongst health and social care professionals of these devastating diseases, is another of their missions because, with early and accurate diagnosis, people can get the support they need, when they need it. An estimated 40% of people with PSP are misdiagnosed, many of whom are initially diagnosed with Parkinson’s. At the moment there are no simple tests or brain scans available to assist with diagnosis – which is why raising awareness amongst the medical professionals and medical students is so important. Conversely, Neurologists who specialise in PSP will recognise someone with PSP almost immediately from their gait.

Another of Andrew’s frustrations is the way in which deaths from PSP and CBD are recorded, often giving pneumonia as the cause of death, even though PSP or CBD are at the root. NHS records allow for only one cause of death and all too often PSP and CBD are not mentioned, making gathering accurate statistics even more difficult. So one of the campaigns they are involved in is to raise awareness of these conditions amongst those involved in administering medical records and records of deaths.

“We want to modernise the way in which death is recorded,” says Andrew, “we want those involved in administering medical records to provide more accurate records of these diseases, which will help us in building up a more accurate picture of them and help us when it comes to the better allocation of resources.”

When it comes to funding, “we are financed entirely by voluntary donations,” comments Andrew “and like the majority of UK charities, we receive no funding whatsoever from central government. We depend on donations from our volunteer fundraisers, many of whom go to remarkable lengths to raise funds for us in sponsored events like parachute jumps. As we speak, 72 volunteers are readying themselves to run the London Marathon – which is our biggest single fundraising event with many runners raising over £1,000 each, and some, much more. We’ve also been awarded grants from private medical research and other general Trust funds in the UK, and we’ve been the beneficiary of legacies from people who’ve been diagnosed with and subsequently died from PSP or CBD.”

The PSP Association was founded by Michael Koe 24 years ago after his wife was diagnosed with the condition, and he found it impossible to find information about the disease. From the start, the work of the association has been visionary. Early steps taken by PSPA included funding the research fellowships of two promising young medics, now those medics are amongst the leading neurologists in the country, based at universities in London and Cambridge; and their research continues.

A landmark for PSPA, has been the creation of the PSP Research Network, co-ordinated from the Institute of Neurology at UCL, headed up by Professor Huw Morris. The Research Network currently incorporates seven primary centres with a strong history of PSP and CBD research and care, plus 15 sub-centres. The network brings together leading experts from many areas of biomedical research, including neuroimaging, clinical analysis, pathology and genetics. It will allow the pooling of patient samples and data and will provide a basis for communication and collaboration through which proposals for further research can be built.

“A third of our funding goes into research and a long-term study is underway which will model the progress of the disease. We are desperate to get on with these studies, to find treatments and cures,” explains Andrew. The network has embarked on a major study known as PROSPECT. This involves the collection of samples, scanned images and clinical information from a group of patients over several years, enabling researchers to make new discoveries based on the way that PSP changes over time. A separate arm of the study will collect one-off blood samples from patients across the UK, generating a resource for investigating indicators of disease in blood as well as genetic data.

Progress is being made in other areas too, figures from the last financial year show we recruited a record number of local groups in the UK. 2017 has also been an important year for PSPA as they received an historic high in terms of trust fund grants. In the North East, PSPA was awarded £25,000 of funding from the Northumberland Freemasons to raise awareness in the region and to measure the impact of PSPA’s work. PSPA is hoping to use part of the Mason’s funding to appoint an engagement officer in a pilot scheme there to coordinate our fundraising, local group and supporter activity in the region, as well as helping with the education and general of awareness of PSP/CBD amongst health and social care workers. In 2017 PSPA also reached more people than ever before through educational events, and this work continues.

Research has also begun to look at the effectiveness of healthcare delivery in Scotland, tackling one of the major frustrations of people affected by the disease – care is all too often fragmented and uncoordinated in response, necessitating multiple visits to hospital for example.

2017 has also seen a reorganisation of their Information and Support Services, enabling them to help even more people with PSP and CBD. Part of this work included making their Helpline the single point of contact for people affected by both conditions, while PSPA is also looking for more volunteer support workers so that they can provide local support to more people with PSP and CBD.

Looking forward, 2018 looks to be a milestone year, the association will hold the first International Symposium on PSP, organised jointly with their American counterparts – CurePSP – to be held at the Royal College of Physicians in October.

“Please turn up to our international research symposium,” comments Andrew “We have 300 places for researchers interested in neurodegenerative conditions – come along and see the excellent research that is being done around world to find treatments and cures for these devastating conditions.”

This symposium is to be held every two years and is one element of their broader efforts to coordinate the international response to PSP and CBD. Ultimately, they’d like to organise and participate in international research projects, working with their counterparts in Sweden, the rest of Europe and the US.

PSPA is also at work developing a new strategy, the draft of which is available to view on their website now, with the opportunity for you to provide feedback. Many of the ideas incorporated into the strategy have come from the community, with over 160 ideas submitted by family friends and carers of those affected by the condition.

Fundraising efforts continue apace, with PSPA organising a virtual 5K race, an accessible event open to all, with the option to complete the race in stages – whenever you want, wherever you want over a five day period. Some willing volunteers in the PSPA office are running 1K a day in their lunch hour over five days. More fundraising events are in the pipeline, all planned with accessibility in mind.

When it comes to raising awareness, a new hard-hitting social media campaign is being created as we speak and PSPA is also working with medical students at Imperial College who are developing a range of projects to help raise awareness amongst GPs and consultants. Moving into 2019, PSPA will be celebrating 25 years since its foundation.

Research into tau proteins and tau therapies is the key to developing treatment for PSP and CBD, and such work is likely to provide fresh insights into Alzheimer’s and Parkinson’s too. So to funding decision makers everywhere one can’t help but ask – why would you not be investing in research that could herald such a medical breakthrough? Why would you not fund research into diseases that are amongst the most devastating from which a person can suffer?