LIVING WITH PPA
All forms of PPA are caused by slow loss of brain cells due to accumulation and spread of pathological proteins, initially targeting the brain’s language system, for reasons that remain poorly understood. At present, this process is not reversible by treatment and so all forms of PPA lead to a long term deterioration in language and other cognitive and neurological functions. Why pathological proteins accumulate, why they particularly target the language system and how cell changes lead to brain tissue loss and symptoms are all vitally important questions that we need to answer to design effective treatments – the answers will only come through further research. However, it is important not to lose sight of the tremendous advances in understanding PPA that have already been made – advances that have been directly inspired and enabled by patients, their families and supporters.
Because most people with LPA have Alzheimer changes in the brain, this opens up the possibility of using the medicines prescribed for Alzheimer’s disease (Donepezil and related drugs) to try to help the symptoms of LPA. Sometimes, PNFA can be caused by Alzheimer’s changes as well – so it may be recommended that they trial one of these medications. However, most people find that any benefit is quite modest and unfortunately these medicines do not affect the associated changes in brain tissue or the course of the illness, so it is important people do not feel pressured to take them
Speech and language therapy is often recommended. This can help people to use electronic devices and other strategies to help ‘bypass’ their speech difficulty. Written communication will become affected in most people with PPA as the illness unfolds and this can affect ability to type and use electronic aids. Assessment of swallowing and diet is another important role for speech and language therapists.
Understandably, people with PPA can develop a co-existing psychiatric problem such as depression or anxiety. These are treatable problems so it is most important that they are detected and managed effectively, including referral to a mental health professional where necessary. In all forms of PPA, there might be changes in the person’s behaviour, and these problems can often be managed effectively with a combination of medicines and/ or non-medical strategies.
We often see that people with PPA derive enjoyment from tasks and activities that rely less on language. Reading books can become difficult in all forms of PPA, but some people still enjoy watching television and find that they are better able to follow things if subtitles are used. Many people with PPA enjoy listening to music, and sometimes the person’s musical preference can change. In people with semantic dementia (SD), non-verbal puzzles like Sudoku and jigsaw puzzles are often challenging but enjoyable distractions. However, as these tasks rely on specific parts of the brain that can be affected in PNFA and LPA, these might not be suitable activities for everybody.
As the PPA gets worse, other symptoms such as swallowing difficulty, hearing loss and changes in personality and behaviour can emerge, and so the help of speech therapists, audiologists, dieticians and/or occupational therapists can be extremely valuable – ideally, under the coordinating eye of an involved GP or local psychiatry team.
Support groups can be a great source of help, support, information and advice. Because PPA is so rare, there may not be groups that are local to you – although it is worth checking our regional support groups page. People with PPA and their friends and families are always welcome to attend our PPA Support Group meetings in London. Receiving a diagnosis of PPA can be incredibly isolating – for the person who gets the diagnosis, and for their family and friends. It is really important that the right support is available, not only for the person with PPA, but also for their partner/ spouse/ friend. Our support group meetings can be a good place to start.