What is frontotemporal dementia?

Frontotemporal dementia or FTD is a disease where nerve cells in the brain become damaged and prevent the brain from functioning normally. This results in a variety of symptoms that progress over time, but it generally starts with behaviour and language changes.¹ It is not completely understood what causes the damage to nerve cells, but scientists think that it is a build up of abnormal proteins inside brain cells that interferes with normal cellular processes, affecting overall brain function.² Dementia is a very complex and challenging disease for those experiencing it and for their family and friends, and can often be upsetting and confusing. Alongside the distressing symptoms,  the essence of what makes dementia such a difficult disease for everyone involved is the effect on your personality and seeing someone you love become almost a different person because of this disease. This also means there is a lot to consider as someone caring for someone experiencing FTD or as a healthcare professional.

How is FTD different from Alzheimer’s Disease?

The front and sides (behind the ears) of the brain are the areas affected by frontotemporal dementia. The name describes the two areas affected, the frontal lobe and the temporal lobe.⁴ The frontal lobe is responsible for controlling language, short-term memory, judgement, self-control, emotions, planning, organising and voluntary movement among other things, making it key in making up our personalities.¹ The temporal lobe is involved in understanding language, forming speech, learning, memory and processing of auditory information. Therefore the very first symptoms you might see are changes in behaviour and language.

Alzheimer’s disease (AD), another specific type of dementia like FTD, is also a disease caused by degeneration of brain cells. Unlike FTD where behaviour and language differences start occurring first, memory loss is the first sign of Alzheimer’s disease. While behaviour and language changes are also symptoms of AD, these occur later as the disease progresses. Symptoms like delusions and hallucinations are not caused by FTD but can be experienced by those with FTD. Another key difference between FTD and Alzheimer’s disease is when you are diagnosed. Frontotemporal dementia tends to emerge earlier in life, around your 40s to your early 60s whereas Alzheimer’s is more commonly developed later in life over the age of 65. Alzheimer’s causes problems with spatial orientation which is not seen in FTD and tends to affect the whole brain and not just the frontal and temporal lobes like in FTD. While both disorders fall under the larger umbrella of dementia, those with FTD and AD experience different symptoms due to different parts of the brain being affected at the start of the disease. Over time, however, the two diseases may start becoming more and more similar as the damage to brain cells progresses.

Symptoms and disease progression

FTD can be categorised into 2 different types, the more common ‘Behavioural Variant’ and the less common ‘Primary Progressive Aphasia (PPA)’.

Behavioural Variant 

This type of FTD primarily affects the frontal lobe resulting in personality changes, changes in how you behave and control emotions, as well as affecting how you plan and solve problems and focus on tasks. This form of FTD is usually developed in your 50s and 60s but can occur as early as in your 20s. Behavioural FTD tends to be noticed first by people close to you rather than by yourself. This can be difficult as those experiencing FTD may not think they need medical help or refuse assistance, which can be stressful for the family and affect relationships. This may delay someone receiving a diagnosis for FTP and make complying with medical help difficult. If someone doesn’t think anything is wrong, this may have dangerous implications, for example, driving a car.³

Symptoms of behavioural variant FTD include losing the motivation to do things they once liked doing, getting easily distracted, losing foresight, and finding it difficult to make decisions. Another key effect of frontal lobe shrinkage is the loss of your inhibitions, an increase in impulsivity and being less considerate of people around you. Someone with behavioural variant FTD may start making rude remarks or acting against social norms which they wouldn’t have ever done before. Further symptoms may include starting to perform repetitive obsessive behaviours or experiencing new or different sensitivities to temperature, pain and sounds.¹

From reading these symptoms, you may be thinking that spotting FTD early might prove challenging, as we as humans experience change and normal ebbs and flows to our behaviour, motivation and emotions. You might see how this means there are numerousethical and legal considerations in regards to FTD care for friends, family and healthcare professionals. 

Primary Progressive Aphasia 

Primary progressive aphasia (PPA) is a less common form of FTD that primarily affects the temporal lobes which causes problems with language as this lobe is where meanings for words, names of objects and memory and recognition of faces are stored. PPA is further categorised into 2 subtypes depending on which part of the temporal lobe is affected: the semantic variant and the non-fluent variant.¹ 

The Semantic Variant 

The semantic variant occurs in a fifth of FTD cases and causes you to forget the meaning of words. People experiencing this type of FTD will notice first forgetting technical words or words they don’t use very often. This slowly progresses to forgetting common words and words they would normally use every day. You may notice this by someone referring to an object with a less specific word, for instance, asking you to pass that “thing”. Other symptoms include forgetting what common objects or appliances are used for, eating issues and forming obsessions with daily routines. The effect of semantic variant PPA is that you lose the ability to communicate clearly and effectively which may lead to a feeling of isolation and being misunderstood, resulting in a loss of skills and independence.¹

The Non-Fluent Variant

The non-fluent variant describes its symptom in its name; causing problems with speaking. This form of PPA occurs in a quarter of FTD cases and essence makes it hard to get your words out resulting in hesitant, laboured and ungrammatical speech. If you have a non-fluent variant of PPA, you may find your speech becomes slow, and you may say words in the wrong order and with the wrong grammar. You may also experience saying the opposite of what you mean, which means that ethically there is a lot to consider here when caring for someone with this form of FTD. You may notice someone with a non-fluent variant starting to use much shorter and simpler sentences and find conversation difficult or may stop speaking completely. This must be very frustrating and lonely for someone experiencing this form of frontotemporal dementia and results in a feeling of isolation as with the semantic form of Primary progressive aphasia.¹

Later Stage FTD

As the disease progresses it becomes more and more unclear as to which form of FTD a person has with symptoms worsening. This is due to a greater range of symptoms occurring as brain cell damage spreads across to other brain areas. The already existing symptoms become more pronounced in later stages and symptoms may become similar to other forms of dementia like Alzheimer’s disease. These later stage symptoms include forgetfulness, delusions, hallucinations, restlessness, agitation, aggressiveness and losing the ability to recognise friends and family. The progression of FTD as well as life expectancy is unique to each person depending on their age, the presence of other conditions and lifestyle.¹

Legal considerations in FTD care

Advanced Directives and living wills

It is possible to make decisions for yourself in the future should you become unable to make and communicate decisions yourself. This is done by creating a legal document called an ‘advance directive’ or a ‘living will’, which specifies treatments and medical decisions you would like to refuse in the future. These treatments must be named and the specific circumstances made clear to be valid. Other requirements include the document being written and signed by you, being signed by a witness, you must be over the age of 18, and have a good mental capacity. It must also be very clear that no one has forced you or harassed you into making any decisions in your will, as your wishes must be respected and carried out without the influence of others. It may be helpful to talk to a doctor when making a will, about what medical treatments and options you may have. While this may seem daunting, making an advance directive ensures you remain in control of your medical care.⁶

Guardianship 

If you are unable to make decisions for yourself due to losing mental capacity, which may happen as FTD develops, a legal guardian may be appointed to make decisions for you. Your guardian may help decide where is best for you to live, whether that be at home with assistance from social services or at a care home. They may decide if you need to go somewhere to receive medical treatment, work, education or training but they cannot force you to do anything. Guardians must make decisions with your best interest at heart and that is the least restrictive, which makes choosing your guardian important. You want someone with whom you have a good relationship so that they may successfully act as your guardian. An approved mental health professional can become your guardian but only if your closest relative approves, and in most cases the local authority assumes guardianship.⁷

Power of attorney 

If you are over the age of 18 and have the capacity to make informed decisions, you can arrange for someone to make decisions for you about your property, your finances, welfare and any medical care. This may be temporary while you don’t have the capacity due to an illness or lasting, which is called a ‘lasting power of attorney’. You can appoint multiple or just one attorney who can make collective decisions on your behalf or for their own individual things e.g. one manages your finances and another manages your property.⁸

Capacity and consent 

The term capacity has been mentioned throughout this article and describes the very important ability to understand the information given to you by your doctors, use this information to make an informed decision and then be able to communicate it. Those experiencing FTD may lose capacity if they cannot understand information about a medical decision, remember that information and communicate it. A trained healthcare professional will usually assess someone's capacity if it’s not clear that they are able to make their own decisions about their medical care.⁹

End of life Care

A type of specialised medical care called ‘palliative care’ may be introduced when someone is nearing the end of their life. This care aims to effectively assess, manage and prevent illness-related, physical, spiritual and psychological distress. An important collaboration between healthcare professionals and family is required to find all possible ways to make this difficult time less stressful for the person with FTD, by reducing pain and suffering and improving their quality of life as much as possible.¹⁰

Ethical considerations in FTD care 

Autonomy and respect for patient wishes

Autonomy in healthcare refers to the right you have as someone with the mental capacity to independently make your own informed decisions about your medical treatment and care. Autonomy can be a challenging issue when caring for someone with FTD or any type of dementia as it can be challenging to balance respecting their wishes whilst also ensuring their safety and wellbeing.¹¹

Resource allocation and fairness in care 

People with FTD have individual, long term deteriorating and ever changing needs, making optimal care for everyone with dementia extremely challenging. Most of the dementia care budget is allocated to home support with not nearly enough allocated to physiological support. With a limited budget and very high care costs, this leaves many without the care they really need.¹²

Caregiver burden and ethical responsibilities

The majority of those with dementia are cared for by their loved ones at home. This contributes to dementia’s impact being much larger than just the individual experiencing a neurological decline. It can be difficult for people to afford help with caring for their loved ones and to find the right alternative support for their loved one’s own individual medical, physical, social and emotional needs. Along with the emotional difficulty that comes along with seeing someone you love change and lose their sense of self with FTD, you may also have to deal with the burden of care, which may go on for months and years and can be very distressing and tiring. It is not uncommon for people to feel overwhelmed with this task despite wanting to care for their loved ones themselves. 

Summary

With dementia numbers growing worldwide, it is important to consider the ethical implications of this disease which affects your autonomy, behaviour, sense of self, and capacity to make decisions. Ethical dilemmas describe the conflicts we experience about what we value in different social contexts. What may be morally good in one cultural context may not be good in another. In dementia care,  autonomy, beneficence (doing no harm) and justice should all be considered in making good care decisions.¹³