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Frontotemporal degeneration (FTD – also known as frontotemporal dementia) has become more widely recognised and understood in recent years, particularly in its relationship to MND or ALS. So I’m going to put medical descriptions in blue italics. Then I’m going to put my everyday explanations in green. I have tried to keep most of the text green, but sometimes it helps to know some of the medical terms surrounding what is happening. Finally I’m going to put examples of how these things happened in my own experience of caring for my husband in orange. Is it dementia or degeneration?Often it is now called Frontotemporal degeneration rather than dementia to distinguish it from being confused with Alzheimer’s disease (AD). Most people generally think of Alzheimer symptoms and behaviours as soon as they hear the word dementia. FTD and AD do have some similarities, but at the same time they are incredibly different. FTD is a syndrome of progressive changes in behaviour and language due to loss of function of neurones in the frontal and temporal lobes of the brain. Many researchers now say that up to 50 per cent of people with MND/ALS will display some degree of FTD. This means some degree of change in behaviour and/or the ability to make complex decisions. FTD starts in small ways, just little things you begin to notice and over time you start to realise that something is happening to the person you love. Usually it is changes in the behaviour or the personality of the person. Not everyone who experiences FTD have the same degree of changes, some are just slight and seem to happen over a long time, others are radical changes that happen fast. Around one year before Chris began to have trouble speaking and swallowing, I began to notice personality changes. I thought he seemed mildly depressed but he asserted that he was not. He had always loved working with people and food. He would come home tired, but full of funny stories about his day. He was always pulling faces and talkig in an animated way, finding the good, funny and positive in everything. He began to come home from his cafe telling more stories of what had annoyed him in his day. He just wanted to sit and do very little once he was home and had his complaint session. He did still tell some funny stories, but over a period of months I realised that there was a real change happening in his attitude towards many things. As the months went by, if I said anything about it he would become very angry and accuse me of expecting to only share the good stuff. He did not realise that there was a change in him. I had always marvelled that he always saw the good and positive, and I saw this wonderful trait in him slowly dissolving. Early symptomsMost commonly the initial symptoms may include changes in behaviour, difficulty with expression of language and impaired comprehension. These early symptoms can be subtle and it can take a little time before the partner or other close friends or family realise that something in the person has actually changed. Not every case is the same. There is a list of possible symptoms and they often occur in clusters. This means that one person with FTD may display a group of typical symptoms and the next person may display a different group. It appears that one main reason for this lays in exactly which part of the frontal or temporal lobe is being affected. An example may be that the person was always far more concerned with the views and comfort of all other family members, but now they disregard everyone else and even berate them for their own views or needs. Apathy is often the first symptom reported by caregivers and may be mistaken for depression. People experiencing these changes may become self-centered, emotionally distant, withdrawn, unaware of the emotions of others, avoid social contact or neglect previous hobbies and interests. They may develop a lack of concern for their personal appearance and become increasingly unkempt early in the course of disease. Common symptoms/signs of FTD
Executive dysfunctionIn what is known as ‘executive dysfunction’ problems arise with planning, organising, abstracting and prioritising, along with impaired verbal fluency. Most researchers agree that bulbar onset more often results in MND/ALS-FTD compared with those with MND/ALS alone. It can be difficult to assess the problems with verbal fluency as the person is also experiencing speech issues associated with the loss of the function of their tongue. It is interesting however, that FTD is more likely to occur in bulbar onset people. Bulbar onset is when MND/ALS begins with speech and swallowing issues as the nerve to the tongue begins to fail. Most people with MND/ALS go on to develop bulbar problems as the disease progresses. Most people with bulbar onset go on to lose the use of their arms and legs as the disease progresses. Organising is a key word here. This is talking about how the person is able to pay attention to detail and then plan something. Can the person still look at a problem and work out what order things should be done in and what the priorities are? Once they work this out can they still put this plan into action and stick to it? Can they make good decisions based on the details they have been given? My Chris had run café’s for many years and his life revolved around food and making people happy. He just loved planning meals and interacting with people, taking a real interest in them and seeing them leave feeling good about themselves. No matter who tried to talk with him about his steady weight loss and the impact of this, he was adamant that his weight loss was to be expected and nothing could be done to change it. This has to be compared to the person that he was before the disease started. He believed solidly in the importance of eating well, and enjoying to eat. His life revolved around constantly thinking about, planning, preparing and serving food. As time went on and he lost the ability to eat more than a little pureed food and was dependent on being fed through a PEG tube, he would simply refuse feeds. This was despite doctors and dieticians weighing him regularly and talking to him about the connection between his poor eating and weight loss. In the end he began to blame his weight loss on me, saying I wasn’t giving him enough. In reality he would refuse to allow me to give him anywhere near enough. Survival in patients with MND/ALS-FTD is generally shorter compared with those with MND/ALS alone, especially when the executive functions are highly impaired. One of the main reasons for this is the lack of insight and poor judgment. This often results in the person making very poor decisions on how to maintain supportive therapies such as nutrition, hydration and safe swallowing or early PEG placement. Another very common and distressing result of this can be a total lack of insight into the need for safety. People with FTD involvement frequently have a high rate of falls that result in serious injury and at times death as a result. When safety was discussed with Chris he would state that he was going to die anyway so it made no difference whether he died of the disease or by a head injury. Diagnosing FTDNo single diagnostic test exists to confirm or rule out a diagnosis of FTD. This often results in a long and frustrating process of testing for other disorders with symptoms similar to FTD to rule them out. This means that testing can be complicated and if we are dealing with someone with MND/ALS, they already have a terminal illness to come to terms with. When a lot of anger and paranoia are involved testing can become extremely traumatic and cause many problems between the person and those that love them. Changes in the medical professions attitude to MND/ALS and behaviourInterestingly, in the past, before FTD was widely recognised, the following list was commonly reported in MND/ALS patients:
The symptoms on this list were considered to be understandable and normal, rather than symptoms of further involvement of the brain in the disease process and degeneration. For myself, even though I came to understand FTD and what was happening to Chris, it was extremely difficult not to take the lack of empathy, paranoia, anger and lack of insight into the whole situation to heart and feel like a total failure as his wife and carer. How anyone caring for a person with MND/ALS- FTD, without understanding this aspect of the disease, comes out of the situation without severe scarring is beyond me. When executive functions are affected, the person begins to think more slowly than they used to. So health professionals find themselves constantly saying to the person at each visit, ‘We are worried about you falling’, ‘We are worried about you choking’, and the patient has a lot of difficulty understanding the impact of this. When the family is brought in on this they say, ‘My loved one just doesn’t seem to be the same person any more and he doesn’t seem to understand. He does not want to listen to me and does not want to do anything that is being suggested as the best for his health and quality of life.’ For me as a carer, the very worst part was that I could not talk to friends, most health professionals or family about the FTD, as my view was immediately discounted as me just not understanding what an awful thing Chris was going through. Time and again the items on that list were thrown at me until I stopped talking about this side of the horror at all. Many carers find it incredibly difficult to even discuss the behavioural changes they are witnessing and experiencing in their loved one as they feel guilty that they are speaking ill of a person who has a terminal illness. They find themselves explaining away the behaviours. Worse can be that others who are not so close to the situation can add more guilt on top by explaining the behaviours as a normal response to such a terrible situation. Often a person with FTD has enough control of themselves that they can appear to others as still quite highly functioning for the duration of a visit, and can present none of the behaviours the partner or primary carer is experiencing. This often makes it hard for a carer to stand up and say anything. One of the responses from health professionals or other people that distressed me the most was to be told “Oh when someone is ill they always take it out on the person closest to them, it’s perfectly normal”. Chris was the most amazing man. I loved him completely, and all the emotional horror we lived through was because this disease was degenerating the frontotemporal area of his brain. Language variantsGenerally it is understood that people who start with behavioural variant progress to develop language problems. Some people begin with the language problems but progress to displaying the behavioural and executive function symptoms over time. When this is combined with bulbar speech issues of MND/ALS communication becomes incredibly difficult as using speech technology or even simple charts becomes very difficult as they lose the ability to spell, remember words, and put sentence structure together. The person often becomes very withdrawn as their ability to use language to communicate lessens. Eventually, even the ability to communicate simply on their most basic needs becomes difficult for them to articulate using just a few simple words on a chart. Chris progressed into the language issues during his last few months of life. Communication was only possible using charts with words and/or pictures. He would often nod his head whilst saying no, or shake his head whilst saying yes. He could not speak well enough to make a noise that could be distinguished as 'yes' or 'no' so the head movement became critical. He could not grasp the concept of using one sound for yes and a double sound for no. Much of the time I was left guessing his answer even to a closed question. He would say no to a range of questions I would use to follow through a path to what he wanted. When all questions had been answered no, I would have to start again as the problem was that he could not follow the conversation well enough to give the appropriate answer. He also did not realise it was happening - he would accuse that I was the one confused. References used to create this page and recommended further reading:
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If you suspect FTD involvement I would recommend you start taking note of behaviour changes and record it in a diary fashion. This will help you to work out if there are patterns to the behaviour and give you something you can use to discuss with your health care team. Usually it is best to discuss this without the affected person being present as it is very distressing for them to think you even suspect there is something wrong with the way their brain is functioning. This way you can decide with your doctor what approach you are comfortable with. This approach may involve a formal diagnosis which will mean the affected person will be aware of what is happening. |
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This website has been designed to support CALS - people who are caring for someone with MND/ ALS. Katrina Jeffery cared for her husband Chris through MND/ALS with FTD and provides these supports from first hand experience. Copyright 2015. All resources that have been used or quoted provide direct links to the source. |