23 August 2009

Aphasia sufferers: Avoid hospital, if possible!

Five months ago A was admitted to a private hospital about 125 km away from our home for the repair of a mitral valve in his heart – i.e. open-heart surgery. And though it was the aftermath of that major but relatively routine procedure that resulted in my finally assuming official status as his carer, in fact A has needed the help of a caregiver for some time because he suffers from a debilitating condition called primary progressive aphasia. Almost certainly, this condition is also why he experienced traumatic after-effects from his recent surgery. For nine weeks, A lay in an Intensive Care Unit, almost immobile and with only rudimentary awareness of anything happening around him. For most of that time he was dependent on a ventilator, and the tracheostomy performed to admit the tube into his lungs meant he was not able to speak while connected to the ventilator. When the ventilator was occasionally disconnected to force him to relearn how to breathe independently, a 'speaking tube' at the tracheal opening would enable A to talk, but it was weeks before most people could understand anything he tried to say. When he was finally released from Intensive Care, we spent an additional two weeks in hospital and then two more weeks in a rehabilitation facility. And for most of those four weeks after he left Intensive Care, I slept in A’s hospital room at night. Having a personal caregiver in attendance was essential if A was going to benefit from normal hospital treatment and recover enough to be able to come home. To understand why recovering from surgery, or just being in hospital, can be such a challenge for an aphasia sufferer, you need to understand something about aphasia.

Aphasia is a language difficulty that usually results from damage to the brain. Most commonly, a person suffering from aphasia finds it difficult to express himself or herself in words; this form of aphasia is known as ‘expressive aphasia’. ‘Semantic aphasia’ is another form which, as the name implies, affects a person’s ability to make meaning from the language that he or she hears or speaks. Semantic aphasia might look very much like what the average person would recognise as dementia. But in fact, every kind of aphasia is a form of dementia, because that frightening label applies to all degenerative brain conditions. But to say that aphasia is a form of dementia does not provide much useful information about the condition for the patient or the family. In fact, it mainly serves to frighten us. One thing the label does do, however, is convey the seriousness of being diagnosed with a condition that involves clinically observable brain damage.

Even when difficulty speaking is a person’s most obvious manifestation of aphasia, as is the case with A, there will usually be a number of related impairments, not all of which will be immediately evident to anyone who does not spend a lot of time with the aphasic person. With us, for example, some of the earliest ways in which aphasia manifested itself included A’s reduced ability to work with numbers (for example, his inability to balance a cheque book, after a lifetime of managing budgets for major projects), confusion between left and right, and inability to follow simple written instructions (for example, when putting together the half dozen parts of a new office chair, the seat ended up upside down). Some of these behaviours might be normal for some people. But they were not normal for A. So early recognition of the problem depends very much on having a very good knowledge of what kinds of behaviours – linguistic and otherwise – are ‘normal’ for a particular person.

We did not realise five years ago, when A first began to show frustrating changes in his ability to perform many routine tasks, that there might be a legitimate medical explanation for this. Too often I would suspect him of laziness. He was already retired and I hadn’t yet stopped working, and I resented the fact that he seemed to have lost interest in the management of our domestic affairs. I now suspect he had begun avoiding certain practices because he appreciated that he was becoming inadequate to perform in those areas. But he could not understand himself, or explain to me, why the bank records often showed errors in basic addition and subtraction, or why bills that arrived while I was away might go astray (at that time my consultancy work required me to be absent overseas for months at a time). In a man about whom I used to joke: ‘Even his underwear drawer is well organised’, such behaviours were completely out of character. He probably feared he was losing his mind. I thought he just couldn’t be bothered. So for both of us, the year or two prior to diagnosis was a very difficult time and placed a lot of strain on us. When we did finally go looking for answers, it was to find out if maybe A had had a small undetected stroke.

The standard literature on apahsia will say that it is caused by damage to the language centres of the brain, and that the possible causes of this damage include stroke, head injury, tumours, infections or inflammation in the brain. What is not always mentioned in the basic literature – and even in the pamphlets circulated by medical authorities and aphasia organisations – is that sometimes aphasia has no known cause. And this is A’s situation: no known cause for his aphasia can be identified. He did not have a stroke, did not sustain any head injury and experienced no known medical condition sufficient to cause one part of his brain to be deteriorating. Yet for the past three years, annual nuclear scans of the brain have clearly shown gaps in that area of the brain where language-processing ability is located. And that deterioration is progressing. This accounts for the term ‘progressive’ in the name 'primary progressive aphasia'; I guess the term ‘primary’ refers to the fact that in such cases the aphasic condition itself is the primary condition, and is not a secondary result of some other event or condition.

There is always the possibility that a knock on the head in a small traffic accident fifty-five years ago, a fall eight years ago or some unremembered conk on the head in childhood was serious enough, even if unremarkable at the time, to have resulted in long-term residual damage to A's brain. I have also wondered whether some reduction in blood supply to the brain could have played a part. After all, A was born with holes in the heart. Two of these were repaired when he was 33 years old. But his recent heart surgery revealed that, as well as the more recently detected problem of malfunctioning valves, there also remained a small tear in the heart muscle that had not been repaired in the earlier surgery – or may even have been caused by the earlier surgery. In the 1960s, pioneer surgical teams using the new heart-lung machine had about 20 minutes in which to complete an operation. So surgeons then often did not find and solve all of a heart's malfunctions. In their haste to make the repairs to known damaged areas, they sometimes even caused other damage. This leftover hole or tear in one wall of A's heart was probably the reason he experienced occasional heart fibrillations for the next 46 years. But I also wonder: could this have starved some part of his brain of oxygen? Is that why he developed aphasia? Well, interesting as that may be to ponder, 'primary progressive aphasia with no known cause' remains the official diagnosis.

I have said that scans clearly show the extent of tissue degeneration in A’s brain. Aphasia can be diagnosed clinically in the living person, unlike neurologic disorders such as Parkinson’s where patients’ MRI and CAT scans appear normal. Indeed, brain degeneration in persons with aphasia looks very similar to deterioration caused by Alzheimer’s, except that the brain scans of Alzheimer’s sufferers show a more widespread pattern of damage in brain tissue; the 'holes' in the brain are not restricted to the language areas. I’m not certain at what point any form of brain damage becomes clinically obvious. As we never thought of having a scan four or five years ago when symptoms first manifested, we don’t know if a brain scan then would have detected damage. Nor do we know how much an earlier diagnosis would have helped us – or if it would have helped at all. Even more invidious than the relative rarity of this kind of aphasia is the fact of its being ‘progressive’. There is no cure for progressive aphasia. The condition almost always gets worse over time. The person's condition may plateau, but it cannot get better. And this difference between forms of aphasia that have a known cause and the more mysteriously generated progressive aphasia is critical when selecting appropriate therapies (more about therapies in a later post).

In the three years since A’s condition was diagnosed, we have become earnestly engaged in finding out as much as possible about aphasia. We have joined various formal and informal groups of aphasia sufferers, almost all of whom developed aphasia as the result of having a stroke. Some stroke victims can progress from near muteness immediately after their stroke to functional, or at least greatly improved, speech months or even years later. This progress requires hard work and many different therapies, but at least these aphasia sufferers may be able to reverse some of the worst effects of their communication difficulties. The prognosis for people with primary progressive aphasia (sometimes referred to as PPA) is very different. We have only met three other families where a family member suffers from PPA. In two of these families, we met only the partners or children of the aphasia sufferer because the aphasic persons themselves were no longer functioning well enough linguistically or mentally to attend the event at which we met their family members. These families were desperately seeking information and strategies to help them cope with what was happening to the family member with PPA. The third PPA sufferer we have met is a woman aged about 50 who has only recently been diagnosed. But we have not met anyone, or read about anyone, with primary progressive aphasia whose condition improved over time. As A’s gerontologist explained to us gently at our first consultation: PPA sufferers are on a track that only has a downward slope; hard work on the right therapies may help to slow down the rate of language degeneration, but we cannot expect any therapy to change the overall direction of this progression. Eventually, the level of degeneration in language ability must mirror the level of degeneration in brain tissue. We were also warned that A might arrive at a point where therapy of any sort ceased to be effective. Deterioration of language ability may plateau at some stage. If we're lucky, he may already have reached the lowest level of language performance. Or his condition could suddenly deteriorate dramatically, or ‘bottom out’. Hard work and therapy may delay arrival at the level of poorest performance, but ‘delay’ is probably the best we can hope for. There may come a time when A is no longer able to use language at all – he could become mute. And though I won’t elaborate here, I should point out that what applies to speaking skills usually applies in a similar degree to writing skills and any other form of communication in which a person attempts to use a symbolic system to convey meaning. There really is little use in trying to learn sign language, for example, because ‘signing’ what he is trying to say will be as difficult for an aphasic person as speaking, writing or drawing the meaning he or she wants to communicate. There are also related difficulties involving the brain's ability to process information and these, too, may become more serious over time.

All of this was very much in my mind as I witnessed the long weeks of frightening after-effects from A’s recent open-heart surgery – or, more likely, from the massive dose of anaesthetic he received during a complicated eight-hour initial surgery in which he nearly bled to death. A second surgery in week 5 – to clean out a sternum wound infection and re-wire the sternum in a procedure called debridement – required yet another dose of anaesthetic. For weeks after this second surgery, A appeared to have totally lost, not only oral language ability, but also a substantial amount of his mental faculties, memory, physical strength and balance, and even his distinctive personality. Certainly, many of his treating physicians – though thankfully, never his surgeon – seemed to think he would die. Several of them suggested more or less openly that death might be preferable and I should consider withholding further treatment. Maybe I'm just stubborn, but a little voice kept telling me these 'experts' were wrong. So with the wonderful support of family and friends, and 24-hour, expert care from a whole bevy of intensive-care nurses, we plowed on through weeks of anxious waiting.

Now, five months after surgery, A’s recovery has been nothing less than phenomenal – an opinion shared by our family doctor and other medical personnel. I think most of the medical staff who treated A agreed that much of his post-surgical trauma flowed from the fact of his having a ‘compromised’ brain. But I believe more of the treatment he received during the recovery period could have taken better account of the particular needs of a brain-damaged patient. This may not have prevented a wound infection, but insisting that A should move through standard post-operative stages at the same rate as 'normal' patients probably did not help in his recovery. He clearly lacked the strength to get up and walk soon after his operation, for example. Insisting that he be made to do so must have placed great strain on his unhealed sternum. Eventually a more appropriate, slowly-slowly approach was adopted. But it seemed to me that the standard patient management plan in ICU, and in hospital generally, did not allow for this brain-damaged patient's reduced ability to communicate or for his difficulty learning new tasks and relearning supposedly familiar old tasks. For example, one nurse was determined that A should learn how to use a pain button to control the level of his pain medication. She refused to accept my expert opinion that A would not be able to manage this. I explained that A would need to master too many steps (recognise level of pain, remember what has to be done, find the controls on the bed at a time when he still had limited control over his limbs, and finally, push the button). Such a multi-stage task would be difficult for a PPA sufferer to carry out in the best of circumstances, never mind while lying almost paralysed and barely conscious of his surroundings. But this particular nurse would not accept that my knowledge of A's ability might be better than hers. And the aphasic patient's inability to respond quickly to doctors' questions during fast-paced daily medical rounds – or even to understand their questions – is another example of why brain-damaged patients, and their caregivers, often find hospital routines overwhelming and frustrating. It was many weeks, too, before ICU personnel accepted that my presence during all routine treatment procedures was necessary to ensure that Allen understood what was happening. Weeks after he and I had resumed a reasonable level of semi-verbal communication, one ICU doctor even told me he had yet to detect the slightest trace of any alertness in A's responses to him. Though otherwise kind and gentle in his manner, this doctor clearly considered A to be a lost cause almost from the earliest post-operative days.

Then there is the matter of appropriate drug therapy. Inexpert though I am, I did not see why anyone with partial brain degeneration who is experiencing consciousness problems should be given anti-depressants – let alone anti-psychotic drugs, as later happened. For all I know, such treatment may be standard protocol for persons remaining in intensive care for many weeks. But in A’s case, until he was weaned off these drugs, he experienced severe night-time hallucinations and extreme daytime lethargy, which made it even more difficult for him to participate actively in physical and other therapies. Cause and effect? It seemed to me reasonable to presume that this might be so. I objected immediately when anti-depressants were prescribed after the first few weeks of intensive care. The prescribing doctor listened politely, then dismissed my views. I was almost amused to find that doctors had decided A was depressed – this at a time when I was the only person with whom A could communicate. Not even our adult children were yet able to understand anything A tried to say. Then at some point, the anti-depressants stopped and an anti-psychotic drug was substituted – which I only found out by accident in week 10, immediately after A left intensive care. This drug was finally phased out over the next two weeks, just as I began a campaign for this to happen – the resident physician saying he had intended to stop the drug anway. Coincidentally or not, A's nighttime hallucinations began to abate as the drug was withdrawn.

Perhaps the worst outcome of a heavy drug regime was A’s apparent mental and social disengagement, which caused his treating physician to declare him unfit for rehabilitation. Such an official decision would have made it impossible for us to gain access to a public rehab facility, or have insurance agree to pay the cost of private rehabilitation. Luckily, our supportive surgeon disagreed with that assessment and obtained a second, more favorable opinion which resulted in A being referred for rehabilitation. Even in the rehab hospital, however, some therapists seemed to take insufficient account of A's mental state, giving him instructions he could not understand and looking for quicker reactions than he was capable of providing. The hospital also was not able to provide a safe environment for him. A was still being weaned off the worst drug, and he would repeatedly attempt to climb out of his bed or chair, even though he could not yet stand without assistance. Surely, even individuals displaying demented behaviours should have safe access to rehabilitation services. But neither of the two hospital facilities had a movement-detecting alarm system for patients like A, such as I saw in aged care facilities in the USA. And with all forms of restraint being frowned upon nowadays, there was no way to ensure A's safety, especially at night, other than by setting up a bed and sleeping alongside him. I was grateful that both the hospital (post-ICU) and the rehab facility allowed me to do this, though both were obviously uncomfortable with this solution.

Most frustrating for me was the amount of time it took hospital staff in the intensive care unit to treat me, A’s caregiver and the person best able to communicate with him, as a member of the team, and not just a concerned family member. I believe this made many treatment decisions more difficult, especially in the early weeks, and could even have put A’s life in danger in some cases. And since the ICU was a locked facility to which one gained access only if and when the personnel in charge allowed it, contact with physicians had to be handled delicately for fear of being locked out at crucial stages. In the first weeks, for example, whenever doctors were doing rounds or administering certain treatments, none of which A could understand without my careful explanations, I might be refused admission or even asked to leave. Luckily for me, this situation changed dramatically as the weeks progressed and doctors seemed to accept that my presence was a help during both routine and specialised treatments. Especially in the frightening lab-like environment of the intensive care unit, the presence and reassurance of a trusted caregiver during a procedure such as the draining of fluid from the lungs can make a considerable difference to a patient who is mentally confused and unable to recognise or communicate with nurses and doctors.

A is now ‘on the mend’ physically and mentally. His aphasia symptoms had deteriorated dramatically after surgery and that low level of mental and linguistic performance continued throughout most of the months of his hospitalisation. His ability to speak, read and write had virtually disappeared, but gradually he seems to be returning to a level of performance similar to his pre-op period. I don't think this amounts to a reversal of any of A's aphasia-caused problems, but only the alleviation of some aggravated losses that resulted from the trauma of surgery and long recovery. There remain a few strange quirks: for example, he has lost the ability to tell, or ‘read’, time accurately, using a standard clock! But aphasia sufferers, like illiterate adults, learn lots of ways of concealing some of their more embarrassing difficulties, and as a former stage actor, A is particularly good at this. So I suspect that telling time was a problem for him before the operation, but he managed to hide the fact. It may be that only in the hospital ward, where an analog clock was the only timepiece available, did this problem manifest itself. However it happened, this loss of ability is just a small example of the many invisible ways in which life is more difficult for people with aphasia than for the rest of us.

It seems to me feasible that A’s greatly improved physical condition – after all, he now has a perfectly functioning heart pumping blood efficiently all around the body, including to the brain – may well help him to implement more successfully the therapies we already had in place for slowing down the effects of brain deterioration on his ability to use language. Certainly with better health he is demonstrating enhanced motivation for all such tasks. We have even begun attending a weekly aphasia clinic at a university which requires us to travel 150 km in each direction. And as long as A’s annual nuclear scans continue to show that degeneration is more or less confined to the left side of the brain (specifically: ‘asymmetrical left temporoparietal pattern of cerebral atrophy, more parietal than temporal’), we can take some comfort from the fact that he does not have Alzheimer’s. Luckily for us, after thirty-one years together, we manage to communicate quite well without always needing to talk.

Postscript: In recounting our experiences of A's hospitalisation, I have probably focused overmuch on what I consider to be some of the ways that some medical personnel demonstrated insufficient appreciation of the special needs of a patient who suffers from the effects of brain impairment. Other aphasia sufferers and their carers with whom I have shared my story have almost invariably told stories of similar difficulties they experienced while in hospital. But I can't end without also saying how extremely lucky we were to have had access to such a high standard of clinical care for months. Sure, there were occasions when decisions taken or treatments prescribed did not seem to me to be appropriate for someone with A's condition. Even so, every morning as I walked into the ICU the variety of equipment and materials and, most importantly, the expertise available there instilled optimism and reassurance. We will forever be indebted to so many first-rate specialists and nurses, to our surgeon and to many different therapists – for the care and kindness we received during nine long weeks of truly 'intensive' care, and then four more weeks of hospitalisation and rehabilitation.

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About me

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I started this blog in 2009 when I became a full-time caregiver. My husband had been diagnosed a few years earlier with primary progressive aphasia. Over the next four years until his death in 2013, we went on a journey of discovery about this rare condition. My blog is about what I learned, how we both coped and how the journey deepened our love and appreciation of each other. Allen’s journey is over, but mine goes on.