Michael Alexander talks to wife Fife Elizabeth birrell who, after the death of her husband Ian, continues to raise funds and campaign for Progressive supranuclear palsy (PSP).
This is a little-known neurological condition caused by the premature loss of nerve cells in parts of the brain.
But when East Neuk’s progressive supranuclear palsy (PSP) fundraiser Elizabeth Birrell remembers the experiences of her husband who died of a terminal illness in 2014, it still upsets her that her diagnosis of PSP has been delayed.
Ian Gordon Birrell, who was diagnosed with degenerative brain disease in 2009, was only 71 when he died at home on October 7, 2014, after “locking himself” in his body.
A former president of Fife Licensed Trade Association, Ian and Elizabeth led the Craw’s Nest Hotel in Anstruther for 33 years and was a highly respected honorary member of the town’s Rotary club.
Following his diagnosis of PSP, he and Elizabeth raised around £ 30,000 to help others with the condition, which causes difficulty with balance, movement, vision and speech.
In the years that followed, she was determined to keep this legacy alive.
However, with 4,000 people living with PSP in the UK at any given time and at least six local people diagnosed since Ian’s death, she is still concerned that many doctors will not know and that a misdiagnosis will take place. .
“Ian spent two weeks in the hospital for various tests and was diagnosed with Parkinsonism,” recalls Elizabeth, now 73.
“But six weeks later, he went to the neurologist and was told it was PSP he had.
“Our local GPs hadn’t heard of PSP. When you are diagnosed, your GP should have a hospice plan in place, but Ian was not.
“One of the doctors told me to make a monthly appointment for Ian and it would help them see how it affected him.
“As time went on and Ian got worse, we walked into the medics room and he said ‘what can I do for you today?’ My jaw could have hit the ground! Ian didn’t want to go back to see him.
Sarah Day is the Challenge Events fundraiser with the Association for Progressive Supranuclear Palsy (PSPA) – the only charity supporting people with this disease in the UK.
She says sadly, even now, Birrells’ experience of misdiagnosing PSP remains far from unique.
“Unfortunately, Elizabeth’s experience of her husband’s late diagnosis is not uncommon,” she says.
“In fact, research has indicated that up to 50% of people with PSP and CBD are first diagnosed with other neurological conditions such as Parkinson’s, before being reassessed as the symptoms progress. ”
What is PSP and what are the symptoms?
According to PSPA, a protein called tau builds up in certain areas of the brain and forms into clumps (neurofibrillary tangles), which are believed to damage nerve cells.
Over time, this can lead to difficulty with balance, movement, vision, speech, and swallowing.
It is estimated that around 4,000 people are living with PSP in the UK at any one time.
There are no simple tests or brain scans for PSP.
In its early stages, symptoms may resemble those of other neurological conditions such as Parkinson’s disease, Alzheimer’s disease, stroke or multisystem atrophy, and misdiagnosis is common.
The condition gets its name because it is progressive – it gradually gets worse over time; it is supranuclear – it damages the nuclei that control eye movements; and it leads to paralysis – it causes weakness.
Each case of PSP is unique and symptoms can be experienced with different degrees of severity and at different stages of progression.
Symptoms may include difficulty with movement and balance, including falling backwards. The movements can be very slow and the muscles get stiff.
PSP can lead to changes in behavior or personality. For some people, these changes can be very subtle, while others may notice obvious changes.
As people become more and more still as PSP progresses, constipation can lead to poor bowel control and urinary problems.
Difficulties in eating, drinking and swallowing are common in PSP and can occur quite quickly after diagnosis.
Coughing while eating or drinking is an indication that the food or drink has “fallen the wrong way” into the airways. If this happens regularly, it can lead to lung infections or pneumonia.
People with PSP have a reduced swallowing reflex, which leads to a buildup of saliva in the mouth. Sometimes the saliva can also be thick and difficult to swallow, and some medications can dry out the saliva, resulting in a dry mouth.
People with PSP may experience visual problems that cannot be corrected with glasses.
The main symptoms include double vision, tunnel vision, blurry or hazy vision, slow and jerky eye movement, and difficulty looking down.
In PSP, the areas of the brain controlling the movement of the tongue, lips and throat can impact speech, making it cloudy and difficult to understand.
Many people with PSP experience fatigue. It is an overwhelming feeling of fatigue, lack of energy and exhaustion.
Pain can be a direct result of PSP or as part of any other condition that you are having.
Like many other chronic illnesses, there is no cure for PSP.
The average life expectancy is around seven years from onset.
However, there are many symptoms that can be managed to help people achieve the best possible quality of life.
PSP tends not to run in families and the disease is not thought to be inherited.
However, research indicates that some people may have a genetic disposition that makes them more vulnerable.
Elizabeth says there is no doubt that Ian’s death left a “huge hole” in her heart.
Recovery in East Neuk as a youngster
Born and raised in Danbury, Essex, Ian was brought to East Neuk by his father John as a child to recover from an illness and returned to visit during the school holidays.
As a young adult, he decided to move to the area and met Elizabeth, who quickly became his fiancee.
His future father-in-law, Eddie Clarke, had opened Craw’s Nest Hotel in 1965 and two years later he invited Mr. Birrell to join him in the business.
It was in the hotel he ran until his retirement in 2000 that he and Elizabeth celebrated their wedding in 1968.
The couple had two children, Liza and Ian, and he was also Scott and Julian’s grandfather when he passed away.
Over the months and years that followed, Elizabeth was comforted by her continued efforts to raise thousands of pounds in the name of PSP Research.
A determination to continue was evident when, before her husband’s death, she planned a charity jazz afternoon at the Craw’s Nest Hotel.
Instead of canceling the pre-arranged event for just two days after his funeral, the family decided to make it a celebration of his life.
Support Elizabeth’s 1000 mile charity cycle
These efforts now continue with Elizabeth in the middle of a 1,000 mile cycle to raise awareness about the PSP and raise funds for the PSPA.
She started her marathon effort at the end of April after developing a love for cycling during the Covid-19 lockdown.
Since her husband’s death, Elizabeth has participated regularly in her local Rotary’s Coastal Walks.
She has raised over £ 70,000 by participating in coastal walks, hosting morning cafes and street parties, and she says she never could have done it without the generous help of friends and the community local.
She aims to complete her 1,000 miles of cycling by the end of June, or about 30 miles per day, weather permitting.
Elizabeth adds, “The PSP is so little known. To help raise funds for PSPA and spread awareness of PSP, I participated in 10 sponsored Coastal Walks organized by my local Rotary.
“Sadly, the walk in 2020 was canceled due to the pandemic, so after looking into cycling during the lockdown, I thought a cycling challenge would be a great idea.
“I hope that by rising to the challenge and talking about it, I can help spread the word about PSP so more people know how to spot the signs and symptoms.
Sarah Day, Fundraiser for PSPA Challenge Events, adds, “Elizabeth’s passion for fundraising and outreach continues to inspire us not only at PSPA, but those in her region and the PSP community as well. and CBD in the broad sense.
“We are honored by his continued support which helps us expand our reach so that more people can detect the signs of PSP and CBD and facilitate early diagnosis.”
You can keep up to date with Elizabeth’s progress and sponsor her cycle at: www.justgiving.com/fundraising/ianbirrell2021
For more information on the PSPA, see www.pspassociation.org.uk