The rarity of PSP means the majority of healthcare professionals will probably never come across someone with the disease during their entire career.
Yet for those that do, like Liz Williams, the impact of working with some one with PSP can be long-lasting and life changing.
Liz, a Parkinson’s Specialist Practitioner from Essex, is a firm advocate for people with PSP and a co-founder of the Essex PSP Support Group. Here she tells the Association about herself and why she is so passionate about helping people with PSP….
“Liz Williams, right, with co-founder of the Essex Support Group, Jenni Relf”
Why did you decide to specialise in Neurology? What drew you to it?
Working as a Community Occupational Therapist I frequently worked with people with neurological conditions, and gradually developed a special interest in Parkinson’s. One of my patients was re-diagnosed with PSP and I was able to act as a case manger over the next seven years co-ordinating his care.
I enjoy the variety in neurology and the fact that we have time to spend with our patients and see them over a considerable period of time enabling us to build up good relationships with both the patient and carer. In Essex we have a holistic approach to treatment and enjoy joint working with other healthcare professionals and the voluntary sector.
What do you think are the main challenges for a healthcare professional when treating someone with PSP?
I think one of the main challenges is to raise awareness amongst other healthcare professionals and carer organisations. If staff are not fully aware of the condition, it can increase the frustration for the person with PSP if they have to keep explaining their symptoms each time they come into contact with a new professional or carer.
Another major challenge is diagnosis of PSP. This can take a long time in some cases and a person may also have had an incorrect diagnosis to begin with, which can increase their feelings of isolation and make it harder for them to come to terms with a diagnosis of PSP.
Informing a newly diagnosed person of the PSP Association and putting them in touch with their local support groups really helps people to understand the condition.
What changes would you make to health care services for people with PSP?
Every person should be given a key worker as soon as they receive their diagnosis of PSP. This could be in the form of a case manager, a Parkinson’s Nurse, a Community Matron or a therapist, but someone who has overall knowledge and understanding of the person with PSP and can take responsibility for their care. I know this is something the PSP Association is working towards through its Care Pathway.
Access to the appropriate services can often take time. I think people with PSP need an open referral to healthcare and therapy services. This would enable them to self refer as needed
I would ask consultants to copy the patient into all correspondence as it is difficult to absorb all that is being said, especially at the time of diagnosis.
Liz Williams works in South West Essex and is a joint founder of the Brentwood Support Group for people with PSP. For more information PSP Support Groups around the UK visit www.pspeur.org.
Liz Williams is speaking on the Role of the Parkinson’s Nurse / PD Specialist in supporting people with PSP or CBD at the PSP Association Study Day for Health and Social Care Professionals on Monday 24th September. The event is aimed at professionals involved in the care and support of people with PSP and CBD. The content of each lecture will be aimed at the multidisciplinary team and will highlight best practice within those areas.
For more information download the Study Day Event Programme or call us on 01327 322410
A challenging walk, stunning backdrops and fascinating history – come and conquer Hadrian’s Wall for PSPA!
Your trekking challenge…
Trek 25 miles along Hadrian’s Wall – an amazing Roman defense that was built in AD122 on the orders of the Roman Emperor Hadrian. The Wall was over 80 miles long and stretched from the east to west coasts of Britain, from Bowness on Solway in the west to Wallsend in the east.
Our trek takes us from Chollerford to Gilsland – a superb section of the wall encompassing some breathtaking views and fascinating ruins. Hadrian’s Wall is
the most remarkable Roman monument in the UK and quite unique throughout the former Roman Empire. Now a UNESCO World Heritage Site which cuts through the Northumberland National Park, Hadrian’s Wall attracts trekkers from far and wide, as it offers interesting and challenging walks that are surrounded by beautiful landscapes and steeped in ancient history.
One wall, one epic adventure in just one weekend!
We meet at our campsite near Once Brewed in the early evening for a group meal and event briefing. Pick ups from Haltwhistle train station can also be provided. After getting acquainted over a few glasses of wine, we get our heads down for the night and prepare for our challenge
Chollerford to Once Brewed
After breakfast, we take the short transfer to Chollerford, where our trek begins! It is not too long before we come across our first taste of Roman history – Black Tarts Turret.
We then push on and reach the classic ruin – The Temple of Mithras, where the Romans worshipped the God of the Sun. Walking westwards, the trail today is quite demanding as the
Wall snakes its way across hills, descending and rising sharply over small distances.
The breathtaking views across Northumberland National Park are well worth the effort. We follow the Wall along crags and past several stunning lakes until we finally come to the end of our first day’s trek along the Wall, trekking straight into our campsite for overnight.
Distance trekked – approx. 13 miles
Once Brewed to Gilsland
Following our hearty breakfast we trek straight from our tents, up the hill back to the Wall. It’s not long before we’re trekking on the Northumberland highlands, which overlook spectacular cliff tops and stunning countryside. The trail today doesn’t get any easier though – undulating all the way until we reach Thirlwall Castle – we’re not too far from the finish now!
We push on for the last section and trek straight into the small charming village of Gilsland, where the welcome sight of ‘finish’ banners will be held high to celebrate our accomplishment. There will be time to sample the local brew before transferring back to our campsite for farewells and goodbyes, following a very rewarding weekend challenge!
Distance trekked – approx. 12 miles
Do you have questions about this challenge? Please read ‘Your questions answered’ by clicking here.
Plan your invasion and conquer the wall!
Call us on 01327 356132
This is the third and final post in Cilla’s series, compiled and written by Cilla’s husband.
Over those years my speech became somewhat alien. It appeared slurred and I even found myself being repetitive with single words. Soon, this became whole sentences and I found some of the people I was talking to were trying to help me finish what I was trying to say. You see, I knew exactly what I wanted to say BUT found it had been lost after it had left my brain. Somewhere, between my brain and my mouth the words had ‘sort of’ gotten jumbled up and didn’t come out as I had intended.
This, somewhat inhibited my ability to be around people. Those that have known me from years ago have, in the past, found me sociable and dare I say an extrovert. I feel that this is an important point since it is how I was generally known. It made me… Me!
I have already used the word alien… in that sense I was talking about myself and my speech. What happened next made me feel even more like an alien! Many people I had been in constant touch with started to fade into the background. Whether they found it difficult to understand me or whether it was me shying away from them, I don’t know. Bit by bit they faded away and left ‘us’ to it. I must admit, in some respects this was a blessing since it left me with less of a need to explain myself, which of course was already difficult.
In some respects, with fewer people in my life it became easier to get on with what was happening around me.
Coupled with the regular falls came the inevitable injuries. These, mostly resulted in bruising, sometimes quite severe. What is strange about this is in the way I fall! Never forward… always backwards! I have been seen doing it, and by all accounts I just ‘go’ without the capacity of self-restraint. By that, I mean I am, somehow, unable to put my arms or hands out to brace for impact or prevent it from happening.
On one occasion I hit my head so badly I needed medical assistance and an ambulance was called. Five hours in A & E and several stitches later I was home. The strange thing here though was the reaction of the nursing staff at the hospital. Initially, my wound was not even looked at and the emphasis seemed to centre on how I came to ‘fall’. It was only in the last ten minutes of my stay at A & E that my injury was looked at. By then, my hair was so matted with blood I had to have some of my hair cut off so they could view the wound. I am convinced that the staff thought there was an element of spousal abuse at work! You see, there are many people in the health profession who do not know about, or understand PSP! Indeed, some have never even heard of it or, therefore, its symptoms! The reason I mention this is to show how difficult it is to convey what had happened. If I had the full power of speech then it would be easy to answer some of the questions. Because I was slow and because of their lack of understanding, it was left to my husband to explain what had happened. Even when he mentioned PSP he received blank looks and more than a certain amount of incredulity.
There have been many more falls since then and I only really feel safe when I am in bed. There have also been more hospital visits… and more stitches. In some respects I am lucky since I have taken to wearing a padded ‘rugby style’ helmet. This, although unflattering to look at, has recently saved my life. How? Simple! I fell so hard that even with the helmet on I ended up with a massive bruise to the back of my head. Without the helmet, I am convinced I would have died through severe head trauma!
I rarely try and get up by myself now… indeed, it is almost impossible, so my bruises and subsequent trauma has somewhat waned.
Despite this, I have other issues, which are now becoming more prominent. I went to the hospital to see if I could have help with my vision. This followed a routine visit to the opticians who tested my sight, then referred me back to my Doctors. With a little anxiety and some expectation, the appointment date finally came. The problem with my sight is not just based on light sensitivity; No, the problem is also rooted in blurred and double vision. With PSP, some sufferers find relief after being fitted with prism lenses. From what I understand, the prisms allow the wearer to look at the world from another perspective. You see (no pun intended); I find it difficult to look up or down, which in my case, also affects my balance. The prisms changes ones perspective and partly rights what my brain interprets.
Unfortunately, in my instance, after my eye test and the subsequent testing with various lenses, it was deemed a failure. I was told by the Specialist Doctor, “Sorry, there is nothing we can do for you! You could try taping up one of your eyes, which would allow the brain to concentrate its efforts more efficiently.” So, there you have it! Prisms won’t help me but some masking tape might! Still, we’ll give it a try…
Since then, I have had numerous checks and tests, including a DOPA CHALLENGE. For those of you who do not know, medication is administered to monitor the effects it has on ‘your’ symptoms. These tests are rigorously monitored, timed and even videoed. The tests, in principle, would be simple to somebody like my husband but I found my coordination, lack of speed and restricted sight worked against me. The first set of tests were carried out without medication, timed and recorded. After a high dose of the medication the second set was done in a rigorous pattern, which repeated the first… The result, identical markers, times and patterns! In other words, the medication might just as well have been water!
Where does that leave me now? Well, my life is solitary, with the exception of a few family members and friends I survive the best I can. I need day to day help and am unable to sustain a ‘normal’ life. Sometimes, my mind goes blank and even a simple task of trying to turn stations on the TV is challenging. I look at the controls and cannot, for the life of me, work out which ones to select! Here, I call my husband by pressing the pre-programmed number on a mobile phone to which he responds. He doesn’t answer the phone, just looks at the number and comes in to rectify the problem.
Well, for now, I have said enough… hopefully, I can update you on my life.
Cilla & Steve x
Photo above: The most recent to be taken showing what actions need to be taken to protect Cilla from both herself and natural elements such as gravity. She is unable to focus using conventional methods and glasses are no longer an option. By wearing a patch, which has been brightened up with ‘bling’, she is now able to concentrate her focus on specific points. Interestingly, Cilla will rarely focus on individual faces and will often talk to an inanimate object rather than address the person who is speaking to her. If asked to address this she would slowly bring her gaze towards the speaker but this would be short lived since she would revert back to looking away. This gives people the impression of disinterest, although I know this is not the case. The head protection is crucial since falls without support are the norm.
Read the first and second part of Cilla’s blog:
The PSPA would like to say a big thank you to Cilla and Steve for contributing to our blog and for Cilla’s moving words.
This is the second post in Cilla’s series, compiled and written by Cilla’s husband.
Cilla in 2002 on Christmas Day. At this stage, Cilla had no idea what was looming ahead.
So, how did this all come about? What were the first signs? How did the Medical profession deal with me?
Let’s start with the early signs!
Like everybody, we sometimes rely on lists. In this instance I remember going to a supermarket and wondering what the scrawl was I had written down as a shopping list. This particular list was as difficult for me to read as it was for others. Over the coming months, trying to decipher what I had written became more of a problem. Eventually, my husband Steve had to take over writing cards etc on my behalf. In truth, it was first deemed a bit of a joke, but as time went on it became worrying since other signs started to become to the fore.
Take my eyesight as an example; here, I became more prone to bright light than ever. By this I mean, even in the dullest of days I felt the need to wear sunglasses when I went out. Apart from the occasional odd look from strangers, I felt comfortable wearing the glasses and wore them every time I left the house.
Overriding all of the first few symptoms was my lethargy and tiredness. I felt tired for no apparent reason and felt comfortable in bed. Here I could sleep, however, even after a good sleep I felt just as tired. This affected my daily life and things became less important around me. I didn’t neglect housework or anything like that; no, I just found everything harder to do. Things took longer to do! Sometimes, just getting out of bed was an effort, so it seemed easier to stay!
After a while, I felt that the situation was becoming ridiculous, so I made an appointment with a Doctor. The Doctor examined me and came to the conclusion that I had ME. Commonly known as Yuppie Flu, it is in fact Chronic Fatigue Syndrome or Myalgic Encephalomyelitis. I suppose I cannot really blame the medical profession for that diagnosis since these are the symptoms of ME:
1. Debilitating low energy levels
2. Painful muscles and joints
3. Disordered sleep
4. Gastric disturbances
5. Poor memory and concentration
6. Neuropsychological complaints
7. Painful lymph nodes
8. Prolonged fatigue after exercise
Having, what I thought was ME, somewhat helped my state of mind since my condition had been going on for far too long. Giving the ailment a name was rather comforting, since it meant I was not making it all up. I am still convinced there were a few people who could not understand the predicament I found myself in. Anyhow, I had some form of respite due to this revelation.
As the years progressed two things happened, both of them at the behest of my Doctor. Firstly, I was assigned a psychologist since the events over the years had taken its toll and I felt depressed. Secondly, I had an appointment to be examined by a professional within the ME Association. The important of the two, in my mind, was the visits to the ME Associations Centre based close to where I live. What made these visits important was that I saw other ‘patients’ and somewhat came to a separate conclusion myself. Seeing them arrive, pick up a magazine, read for a while then freely move into their appointment made me wonder. I wondered if they were the success stories and were there for ‘follow up’ appointments. As it happens, I found out that they were, in some cases, like me, first time visitors. My final supposition was that there were several levels and I must have been at the top of the tree!
Then something happened to make me look at everything again!
I was suffering from hot flushes and wanted to speak to a Doctor about my medication for HRT. Hormone Replacement Therapy is something I saw as separate from my ‘condition’ and wanted to seek out better medication. Since my Doctor was not available I accepted an alternative appointment with a different Doctor.
Aided, I dutifully attended the practice and was able to tell the Doctor everything that had happened over the last four years. She listened, showed concern and compassion before referring me to see a specialist.
The specialist in question was an expert in Parkinson’s. It took three appointments for him to recognise the possibility that I might have PSP… My husband asked, “PSP, what the hell is PSP?”
The subsequent referral confirmed my predicament!
That was just over a year and a half ago and I have now been catapulted into another dimension…
Read the third part of Cilla’s blog, like our Facebook page to keep updated: http://www.facebook.com/PSPAssociation
By December 2008 there was already a marked difference, which manifested itself in many ways. Here Cilla is pictured with our son Joe and grandson Jorge who came to stay for a few days before Christmas. Cilla was unable, at that time, to travel too far although this special visit to view some local Christmas lights was an exception. It is noticeable that Cilla was unable to apply her makeup despite this previously being a main feature in her life. It would have once been unheard of for her to leave the house so unadorned
This blog is about me, Cilla, and my relationship with the progressive disease PSP. Although compiled and written by my husband I have given a nod of approval for its distribution. Ironically, a nod is almost all I am capable of these days!
PSP is the understandably shortened version for Progressive Supranuclear Palsy.
The abridged version of the medical term is outlined directly below.
Progressive supranuclear palsy (PSP) is a rare degenerative disease of the brain. The disease impairs movements and balance. Many people with PSP also experience changes in mood, behaviour, and personality. A decline in cognitive mental processes, such as thinking, memory, attention, and speech, is not uncommon. When these mental changes are severe enough to interfere with everyday activities, they are called dementia.
The name progressive supranuclear palsy seems like a mouthful, but it accurately describes the disease. PSP is progressive; meaning that it gets worse over time. The disease affects the part of the brain above the nuclei (“supranuclear”), which are pea-sized structures in the part of the nervous system that controls eye movements. Palsy means weakness, and it is this characteristic weakness in eye movements for which the disease is named. PSP is sometimes called Steele-Richardson-Olszewski syndrome, after the 3 scientists who first described it in 1963.
About 20,000 people in the United States have PSP. The disease usually develops in people aged 60 years or older. Symptoms typically become noticeable in the early 60s, although the disease sometimes affects people in their 40s or 50s. PSP is slightly more common in men than in women.
Because PSP mainly affects older people and has somewhat similar symptoms, it is often mistaken for Parkinson disease, a much more common movement disorder. The distinction is important, because treatments that help many people with Parkinson disease do not help those with PSP. Unfortunately, we do not yet have an effective treatment for PSP.
Firstly, I would like to explain how it now affects me:
1. I now lack writing skills
2. My speech is soft, stuttered and repetitive
3. I have lost the ability to balance unaided
4. Because of point 3, I often fall if unaided
5. Something I do not really wish to admit to, is urinary incontinence
6. Extreme tiredness
7. Loss of normal sight and vision
9. Bouts of Anger and Frustration
10. Occasional Forgetfulness
Well, I think that is enough to be getting on with for now!
By the way, this is me:
This photo was taken in a pub garden with our son Adrian. Although not obvious, I’m in my wheelchair and on the way to one of the many hospital visits in Winchester. We stopped off to meet up with Adrian who was in the area. Lunch is an experience in itself and eating with conventional cutlery is a problem. Being adverse to light means that the sunglasses are a must when out!
Read the next edition of Cilla’s blog, keep up to date on our Facebook page: http://www.facebook.com/PSPAssociation