FAQ's
We strongly advise you contact your GP and one of our PSP Nurse Specialists before following the broad guidance below. It cannot be overstressed that PSP is a very individual disease and needs individual care. These Questions and Answers are intended to provide broad guidance for carers and others working with patients, very much subject to the directions given by your GP/Neurologist.
Inheritance of PSP
Progression of PSP
Treatment of PSP
Coping with Falls
Vision
Speaking and Swallowing Problems
Effects on Intellect
Accessing Care
Lifespan
Inheritance of PSP
Q . My husband has PSP. Are my children likely to get it?
A . No. Research studies to date across the UK have failed to identify familial clustering of the disease. However, there do appear to be a few families worldwide, in whom more than one relative has PSP. There is believed to be some genetic susceptibility in most neurodegenerative diseases. That is, given a certain grouping of genes (which are inherited), the persons concerned will be more susceptible to one or more of the as yet unknown environmental 'triggers' which, the theory goes, might set the disease off.
In the case of PSP, many people have such a genetic grouping, but very few actually develop PSP. For, as in Alzheimer’s and Parkinson’s Disease, the risk level is clearly extremely low. In Huntington’s Disease, there is a 50/50 chance of inheritance if either parent has the disease, whereas there is no proven cases of a child in the UK inheriting PSP from their parent. Genetic susceptibility is currently the subject of intense research in PSP and related neurodegenerative diseases.
Progression of PSP
Q. Tell me how the disease will progress.
A. As the neurons in the brain die, capabilities ‘controlled’ by these neurons are progressively affected, over time. PSP is a very individual disease and a patient may remain on a plateau not deteriorating for long periods. Some of the symptoms described may not appear at all or may appear in a different order than expected. The rate of progression of the disease and its effect is largely dictated by the rate of death within the brain of the ‘controlling’ neurons.
Some literature on PSP attempts to break the progression of the disease down into ‘stages'. This tends to assume the patient will follow a classical and evenly progressive route. In practice this is rarely so. This is one of the problems faced by clinicians, both in initially diagnosing PSP and in advising on its likely progression and on drugs to help with symptoms.
Treatment of PSP
Q. Is there any treatment?
A. There are a range of treatments to help with symptoms and related strategies and techniques for lessening their effects. However, unfortunately, today there are still no disease modifying drugs for PSP that are known to be effective. An ever increasing amount of research is taking place across the world into the cause, treatment and eventual cure of this and other neurodegenerative diseases and much of this is into disease modifying strategies for these diseases.
Treatments, strategies and techniques in helping with symptoms are outlined in The Guide to PSP and CBD.
Coping with Falls
Q . How will I cope with falls?
A . Falls are a common symptom of PSP and cannot entirely be prevented. Unfortunately, these are often unexpected and backward, so more likely to be damaging than the forward falls more characteristic of Parkinson's Disease.
There are many strategies for reducing risk of falls. In particular, take advice from your Occupational Therapist on the environment and safety of your house, for example, installing grab rails, stair lifts etc as necessary. Common sense dictates removal of loose rugs, low coffee tables and other hazards that are not easily seen by patients, because of problems with the downward movement of the eyes in PSP. Ensure good lighting, give assistance on stairs and maybe consider the use of a ‘walker’ weighted at the front. Surgical Appliances Departments at local hospitals will fit and provide lightweight helmets to protect the head if this is deemed advisable; and hip protectors when walking .
Often, the arm of a carer is the best support , when and where available, to prevent a fall. It also relieves a patients anxiety and helps with balance. Falls in PSP can mean fractures and hospital admission and this setback often seems to take the disease off a plateau on to a downward path, so risk assessment and such prevention strategies to avoid falls are very worthwhile.
Vision
Q. What about my Vision?
A . A key symptom of PSP involves eyes and vision. Early dislike of bright lights and tunnel vision can develop into the classic ‘dolls eye’ syndrome when, with the head held steady, horizontally, the patient is unable to look up at the ceiling or down at the floor, yet if you move their head up and down, the eye movement ‘roll’ is there. Saccadic or horizontal tracking movement of the eyes are often slowed and blink rate low. This, in turn, can cause ‘dry’ and painful eyes.
The problems caused by the inability to look down include an increased risk of falls, particularly on stairs and difficulty with eating or reading. Some patients find prism lenses help here. Blethoraspasm involves the eyelids closing and difficulty in opening them. There are various strategies to help keep the eyes open, including manual stimulation, Ptosis crutches and botox injections. The last involves an injection every four months or so of a weak solution of botulinus toxin by your neurologist or opthamologist. Remember, always seek advice from your GP or Neurologist.
Speaking and Swallowing Problems
Q. What about Speech?
A. Unfortunately, PSP, as it progresses, can and often does affect progressively speech and the ability to swallow. This is because the neurons controlling the swallowing mechanism are often sited in the affected areas of the brain. As the disease progresses, speech may become slurred and ‘growly’; and harder to understand.
To help with speech and language problems keep background noise to a minimum (e.g. TV or radio), face the patient and allow time for response. Later in the disease, with the often-increasing difficulty in writing (usually in a tiny cramped scrawl), communication itself can become difficult or even impossible, even though the patient’s hearing and intellect remain largely intact . Communication aids such as alphabet boards, light writers and amplifiers can all be helpful. A home devised list of often asked questions, each represented by a letter of the alphabet, can allow effective communication without speech. Speech and Language Therapists (SLTs) have a key role to play here, so consult your GP and/or SLT.
Q. And ability to swallow?
A. A video swallow can be used to assess the effectiveness of the swallowing mechanism. Food may need to be cut up or softened and liquid thickened (it then becomes easier to swallow). There are strategies to help ease this often progressive problem. For example, tuck the patient’s chin forward when eating, and don’t rush meals or 'stuff' food.
Where the ability to swallow has seriously deteriorated, a simple to carry out Percutaneous Endoscopic Gastrostomy (PEG) operation for tube feeding might be advisable, to reduce the risk of food going down the ‘wrong way’ with the resulting risk of Aspirational Pneumonia (a common complication of PSP). Your GP/SLT/ Neurologist/ Dietician should all be involved in giving advice on the decision, which is ultimately made by the family and patient, should the disease progress in this way. A PEG will normally improve the quality and length of life of the patient due to the increased intake of essential food and drink. It does not preclude taking carefully prepared food under supervision by mouth if the risk is judged to be sufficiently low by your medical advisor. Fitting a PEG is a simple and reversible operation.
Q. What about excessive saliva and drooling?
A . PSP, as it progresses, can lead to the ‘weakening’ of the throat muscles, and consequent problems of excess saliva and drooling. A bib, particularly when eating, is sometimes a partial answer. For thicker, more viscous saliva, suction devices may be of help, but need to be used carefully to avoid the risk of infection and of 'worsening' the condition. For problems with drooling, your GP may advise the use of the drug amitryptylene to dry secretions from the mouth, which can be helpful. Botulinus toxin therapy into the salivary glands is being investigated as a further option in a few neurological centres. Your Speech and Language Therapist should always be consulted.
Effects on Intellect
Q. How good are comprehension levels once all speech has gone?
A. This is difficult to assess. Comprehension levels are probably slightly dulled, but the patient's intellect normally remains largely intact, even in the later stages of PSP. The patient should be treated as if they understand all that is going on around them, which they probably do.
Q. Do PSP patients sometimes suffer anxiety, aggression, hallucinations etc?
A . In some cases, yes. Patients can have hallucinations, often due to medication. Advice should be sought from your GP or Neurologist about this and the option of taking a patient off a prescribed drug, if necessary. PSP can also cause changes in behaviour, including greater emotional lability and, sometimes, a more aggressive attitude.
Accessing Care
Q . I s residential nursing care inevitable in the later stages of the illness?
A. The decision on where nursing care should be given should be made by the family, with advice from the GP / Specialist and from Social Services. Factors include the levels of care needed and the level actually available, the suitability of the home and costs involved, since personal care is means tested, balanced by the level of stress placed on the carer, if the patient were to remain at home. It can be a tough and stressful decision for all involved.
Q. How do I access Care in the Community?
A. Go directly to Social Services, look in the local telephone directory for their contact number, or ask your GP.
Q. How can Carers be persuaded that they have not failed in their duty, if they agree to residential and/or respite care?
A. In short, by explaining that their own health and well being is often paramount to the well being of the patient.
Lifespan
Q. Should the patient always be told the full implications of the diagnosis ?
A . This has to be a decision taken by the family. In practice, most patients usually ask to be told the truth about their condition, which allows them to plan for the future. Remember, PSP is a very individual disease and its progress and symptoms vary from patient to patient. For some, its progress is very slow.
Q. What are the criteria for Continuing Care
A. Continuing Care is when the NHS accepts the full cost of nursing and personal care in hospital, in residential care or at home. The criteria for Continuing Care are currently being revised in a new National Service Framework to ensure greater fairness and greater uniformity of awards across the UK. These are based on assessments by qualified nurses of the patients set against the agreed criteria which looks broadly at the level of the nursing component required; and if sufficiently high, an award will be made. Continuing Care is diagnosed for those in the later stages of progressive diseases.
Q. What is the average life expectancy of PSP?
A. Medical journals quote an average life expectancy from onset of the disease of some seven years, but this varies considerably from person to person. We have living patients who joined The PSP Association over ten years ago. With good care, patients with PSP can live well into their seventies and beyond.
