Progressive Supranuclear Palsy: The Other Side of the Fence

Progressive supranuclear palsy (PSP) is an incurable degenerative neurological disease, as recently summarized by Rowe et al. ¹ I was recently given this unwelcome diagnosis by a consultant neurologist. As a former academic and health professional, I thus find myself stuck on “the other side of the fence.”

Prior to retirement, I was a Reader at a local university, working as a Social Scientist on the consequences of chronic pain, particularly in the elderly. In addition to research and teaching, I was a counselor in a local pain clinic.

As well as being a mother to three (now grown-up) children, I had always had a very active social life; enjoying travel, concerts, and opera; and entertaining at home. I did embroidery, made jewellery, painted in oils and did some research into family history. This active life carried on after retirement, and I was looking forward to more of the same, with my husband also retiring from the Civil Service. I did start to experience increasing fatigue and breathlessness, and then my balance and gait started to suffer. Despite these setbacks, I managed to continue largely as normal. The symptoms were difficult to explain, and following various respiratory and cardiac investigations (and in the absence of any specific neurological disorder) were given a diagnosis of Mylagic Encephalopathy, commonly known as Chronic Fatigue Syndrome.

Worsening of symptoms coincided with the start of the first UK Covid-19 lockdown in early 2020, which meant that a good deal of “normal life” stopped anyway, though at that point we hoped that it would restart for us at some point. Then in April 2021, reexamination by a consultant neurologist revealed not only deterioration in gait and balance but the restricted eye movements that are (so we learnt) a key feature of PSP. This, then, became the new diagnosis. Although a diagnosis of Mylagic Encephalopathy/Chronic Fatigue Syndrome was bad enough, there is at least some chance of recovery. Not so, it seems with PSP. As the name suggests, this is a relentless and progressive disease, for which there is no effective treatment at present. Although not all patients suffer all symptoms, these include a gradual loss of balance and mobility, of speech, and even of the ability to swallow. Seldom fatal in itself, PSP can often lead to death as a result of falls or choking. Rowe et al. stressed that it needs to be recognized and managed as a disease in its own right rather than as “atypical Parkinsonism.” PSP is a rare disorder that few nonspecialists will have encountered. Still, my elder daughter (a professor) commented that I wouldn’t want to have anything “common”—my tastes having always been eclectic and unusual!

Life is far from brilliant on this side of the fence, especially if one doesn’t fit the textbook picture completely—though one consultant did say to me “I have to read about this….” The PSP Association is a helpful source of information. Its online forum allows those with PSP and their carers to share and reflect on their experiences—this can be supportive, though the posts can make for very grim reading. Everybody is different, and the disease itself can develop along with many different paths. This is something that medical professionals seem to find difficult to comprehend (because they go by the textbooks). The politics of medicine need urgently to be revamped.

Given the current lack of treatment for the underlying disorder, the management of PSP focuses on mitigation of symptoms. Paucity of research, the rarity of the disease and the variability of symptoms all make for a lack of evidence on what works for individuals. For me, the most bothersome symptoms remain extreme fatigue, increasingly poor balance, insomnia, and sudden and sometimes very severe sweats. Insomnia means constantly-disturbed nights (for us both), while I feel I have no energy at all most days. I have had some episodes of choking and (so far) two falls since diagnosis. Getting around requires much care and increasing amounts of help. We have installed a stair lift and a wet room. Typing is still possible but increasingly slow and effortful. I was unaware of having the limited eye movements that prompted the clinical diagnosis of PSP. Shortness of breath is not typically seen as a symptom of PSP, but one explanation suggested by a respiratory consultant is that the sensation of needing air could be due to a disturbed autonomic nervous system incorrectly “detecting” an excess of carbon dioxide in the blood. Following his suggestion, I have found respiratory coaching to be helpful.

Management of symptoms is based on others’ experiences (for example, via the PSPA Forum), and common sense. The main risks to survival with PSP are inhalation pneumonia and falls. Neurophysiotherapy exercises were suggested by the neurologist and my GP referred me to the local neurological team for Speech and Language Therapy (SaLT). With SaLT, there has been a tension between monitoring decline and “preparing for the worst”—for example, through voice banking—and trying to maintain as much function as possible through vocal exercises. I have accessed some exercises online designed for the management of Parkinson's disease. There is no evidence that these will help with PSP, but nor is there evidence against, so the “use it or lose it” principle may apply.

This is a dismal diagnosis to receive, and some of the response has to be about preparing for the worst. The support from husband, carers, therapists, hospice nurse, and GP has all been very good. My husband, formerly a senior civil servant in the Department of Health, is now my chief carer, typist/PA, cook, bathroom assistant, and knicker-hoister. He is very good at helping me but very slow at doing things. I have no patience left at all and the frustration and weakness is really horrid and getting worse. I think of all the things we used to do: now I need help standing or sitting, dressing, and undressing: we talk about “the condition,” the weather and what is for lunch!

There is the danger of becoming defined by one's disease. Years ago, I found myself described as “the cholecystectomy in Bed 21,” ² and the danger seems even worse for a long-term condition. I feel that the real person in me is getting lost behind the label. And I wait, not knowing what is going to happen but contemplating a possible future with no ability to communicate my wishes. So in addition to my Will, I have completed Powers of Attorney, Advance Directive, and a “Respect” form from the local hospice on care and eventual death.

One visiting nurse recently said to me “we talk about empowerment, and then we take control.” How true that is! Waiting is so terribly undignified. But in the end, “life is what it is” and what you make of it—from both sides of the fence, it seems!

Prior to retirement, Dr Beatrice Sofaer-Bennett PhD (University of Edinburgh) was an academic social scientist, a trained counselor, and a former heath professional. She researched into social aspects of pain, taught medical students and nurses worldwide, and worked in pain clinics in the United Kingdom and the Middle East.