LIVING WITH PCA
Although there is no medication available to treat posterior cortical atrophy (PCA) specifically, patients may find some of the medications available to treat patients with typical Alzheimer’s disease helpful. The medications are called acetyl cholinesterase inhibitors and include donepezil (Aricept), rivastigmine (Exelon) and galantamine (Reminyl). However, these medications are only designed to treat the symptoms of the disease and are not a cure (see this page on ‘drug treatments for Alzheimer’s disease on the Alzheimer’s Society website for more information: https://www.alzheimers.org.uk/about-dementia/treatments/drugs/drug-treatments-alzheimers-disease).
Although individuals with PCA often show relatively preserved insight, they can have profound difficulties in interacting successfully with the visual world around them. The cognitive problems associated with the condition also often lead to a loss of independence, can cause activities of daily living to be compromised and may inhibit the enjoyment of previous hobbies, especially reading. Such problems can lead to depression, irritability, frustration and a loss of self-confidence. Individuals with PCA who are experiencing low mood may benefit from a trial of antidepressant medication.
Supporting people with PCA
Medications available to treat patients with typical Alzheimer’s disease may be helpful to some people with PCA, however these are designed to treat the symptoms of the disease and are not a cure. There is therefore a need for strategies that may help to cope with visual impairment. Here we’ve tried to put together some relevant advice from a number of sources, primarily the Alzheimer’s Society, RNIB and Thomas Pocklington Trust. It is quite general advice for people with visual loss or Alzheimer’s disease, so you may or may not find that it works for you.
The Alzheimer’s Society have produced a factsheet about vision and Alzheimer’s disease. Visuoperceptual difficulties in dementia (factsheet 527) outlines potential perceptual problems and gives some environmental adaptations and tips for minimising visuoperceptual problems.
There is a range of practical visual aids designed to assist individuals with different types of visual impairment which may be of use to people with PCA. These include devices such as talking clocks and watches, mobile telephones with simplified displays or pre-programmable direct-dial buttons and cooking aids such as sensors which beep when a cup is nearly full.
There are a variety of devices designed for people with sight loss that may be helpful to people with PCA. Examples include telephones with large clear buttons, talking clocks and reading machines. There’s plenty of information on the RNIB website about using computers, telephones and other access technology. Their reading and writing pages also have details of talking books, newspapers and other reading aids such as typoscopes. Intuitively, making text larger through magnification or large-print books makes it easier to read. Whilst this is true for some people with PCA, others find that smaller text is easier to read than large text (Crutch et al. 2010).
There is also an increasingly wide range of talking books and audio recordings available either on CD or downloadable from the internet, with a range of titles from romantic novels to biographies and technical manuals. Audio guides are also available for many cultural events including the theatre and museums.
The RNIB website [https://www.rnib.org.uk/] has a range of visual aids and adaptations you may want to have a look at. Please note that not all aids for people with eye-related visual problems will be suitable for people with PCA because PCA also often involves difficulties in spatially orientation and proprioception (knowing where one’s body is in space), but many of our support group members have found off-the-shelf visual aids helpful for a time.
Adapting the home environment
Although written for people with many different sorts of visual problems, the RNIB’s “Living with sight loss” pages may have some strategies that could help. There are suggestions about using colour contrast to make important objects, for example door handles or handrails, stand out. There is also advice about good lighting, which could help lessen some of the visual symptoms. Reducing shadows, using non-shiny flooring and reducing glare and minimising obstacles may help. You may also want to read the booklet entitled “Improve the lighting in your home” produced by the RNIB and Thomas Pocklington Trust. More advice about lighting and design can be found at the Pocklington Trust website.
You may also be interested in this factsheet about adapting the home environment.
You may also be interested in our list of organisations that provide information and support [text/links coming soon].
What to expect - The Stages of PCA document
As mentioned, the experience of PCA differs from one person to the next, but this document attempts to describe how abilities change during the course of the condition. This document reflects the different symptoms, deficits and challenges faced by individuals diagnosed with PCA relative to more typical, memory-led presentations of Alzheimer’s disease. It was developed with people with PCA and their carers and is based on the well-established ‘7 Stages of Alzheimer’s Disease’ framework.
You can access the full Stages of PCA document here, but we recognise that information about later stages of this disease may be a sensitive topic and difficult to embrace. Below we have included information about stages 4-5(?), and information about the later stages is available within the corresponding tab above (or in the full document in downloadable PDF format), should you wish to access it.
Stage 4: Moderate cognitive decline (Mild or early-stage Alzheimer's disease)
At this point, individuals with PCA may require significant help with everyday activities such as cooking, dressing and using appliances. The person retains a clear sense of purpose regarding activities and plans but attempts to achieve these goals are frustrated by frequent, small visual problems and have significant implications for independence and the care/support required e.g.
- Inability to read labels on food packets
- Problems discriminating coins
- Inability to tell the time from an analogue or even digital watch
There may be significant contrasts between what people can and cannot do e.g.
- Dissociation in ability to walk, sit down and stand up (e.g. might be able to walk but not sit down or unable to stand up and walk).
- Being able to see some things but not others (e.g. following moving targets better than localizing static objects; can’t read but can still follow or at least get pleasure from TV or the cinema)
There may be changes in the ability to navigate around unfamiliar and familiar environments: Someone with PCA might be aware that they are at home but have difficulty navigating from one room to the next, or locating the bathroom; some may find a loss in their mental map resulting in problems navigating even familiar environments, (e.g. the home) or problems locating rooms (especially in low light). This might result in getting lost within the home. Problems outside the house might also occur.
Severe visual and other problems may also result in:
- Inability to engage in a number of pastimes such as reading
- An inability to identify even very familiar faces, with recognition of identity dependent upon hearing someone’s voice
- Resorting to eating with one’s hands, or being fed, owing to difficulty manipulating cutlery and seeing the location of food on a plate
In addition, increasing word finding difficulties may mean that more time is required to express oneself, leading to reduced participation in group conversations.
Stage 5: Moderately severe cognitive decline (Moderate or mid-stage Alzheimer's disease)
Vision deteriorates further to the extent that the world is viewed as individual pieces of a puzzle or as if through a fractured mirror, with occasional small details noticed but difficulty appreciating whole scenes or the relationship between different objects. Individuals with PCA may now:
- Require help with most or all everyday tasks, including toileting and personal care owing to problems undoing zips/buttons, positioning the body relative to furniture and seeing and feeling the position of clothing, etc.
- Experience spatial problems not limited to vision, with spatial commands and actions (e.g. ‘turn round’, ‘shuffle forwards’) difficult to comprehend and execute
- Require support and/or supervision when walking, for example because of difficulty detecting the edge of pavements, paths and steps etc., and/or because of a tendency to shuffle, stoop or lean
At this stage, many individuals are registered as blind, meaning they require support in all visually guided activities. Episodic memory and word finding difficulties may also now be prominent, though many individuals retain considerable awareness of their situation. Some people may have problems with multiple senses, e.g. problems with:
- Sensing the relative position of parts of the body (as pronounced as not knowing if one is ‘the right way up’)
- Headache, increased sensitivity to pain and/or sensations in the scalp or other body parts, people with PCA may become more sensitive to the cold and may be prone to experiencing cold hands and feet
- Sense of hearing may change: people with PCA may become more sensitive to certain sounds.
- Feelings of imbalance or instability, particularly when walking
- Little jerky movements in the fingers, arms or other parts of the body
Getting registered as sight impaired
Text/links to factsheets coming soon
Applying for a Blue Badge
Text/links to factsheets coming soon