Medicosnext
Can you imagine a situation where your dearest loved ones—your father or mother, or your grandparents— losing their memory and not being able to recognize you and their own close family members? They will not be able to remember what day, date, and year it is, and they will not be able to remember whether they have taken their meals or medicines and where they have kept their belongings. They may have, in addition, hallucination and delusion and suspiciousness and various behavioral issues that will make them not able to sleep and do their daily activities. Gradually, their memory fades away and they will be totally dependent on others for their daily needs and activities. In the following article, this disabling disease of dementia is discussed and how the person with dementia has to be cared for is highlighted, so that all of us will become aware about this disease and will be able to take care of our loved ones in situations where it is needed. This is more relevant as September is the month for spreading awareness about Alzheimer’s disease.
What is Alzheimer’s disease and related dementia? What is the burden of problem?
Alzheimer’s disease (AD) is a progressive neurodegenerative disease of the elderly, in which the brain cells slowly die due to accumulation of abnormal proteins, resulting in dementia and other behavioral problems. Dementia is a syndrome, usually of a chronic or progressive nature, caused by a variety of brain illnesses that affect memory, thinking, behavior, and ability to perform everyday activities.
Alzheimer’s disease is the most common cause of dementia, accounting for an estimated 60% to 80% of cases. Recent large autopsy studies show that more than half of individuals with Alzheimer’s dementia have Alzheimer’s disease brain changes (pathology) as well as brain changes of one or more other causes of dementia, such as cerebrovascular disease or Lewy body disease, a variant of atypical Parkinson’s disease.
Another disease with similar presentation to Alzheimer’s disease is known as Fronto temporal lobar degeneration (FTLD), in which patient presents with behavioral changes first before the memory issues sets in. FTLD includes dementias such as behavioral-variant FTLD, primary progressive aphasia, Pick’s disease, corticobasal degeneration, and progressive supranuclear palsy. In FTLD, nerve cells in the front (frontal lobe) and side regions (temporal lobes) of the brain are especially affected, and these regions become markedly atrophied (shrunken). In addition, the upper layers of the cortex typically become soft and spongy and have abnormal protein inclusions (usually tau protein or the transactive response DNA-binding protein TDP-43).
As per a report of the World Health Organization published in 2019, it is estimated that 50 million people worldwide are living with dementia, which is sure to double every 20 years, reaching 82 million in 2030, and 152 million in 2050. Majority of this burden is observed in low- or middle-income countries, constituting 68% of total cases. The problem looks even more worrisome if we say that every three seconds, someone develops dementia, amounting to 9.9 million new cases each year. In 2015, dementia costs were estimated at US$ 818 billion, equivalent to 1.1% of global gross domestic product, ranging from 0.2% for LMICs to 1.4% for high income countries, which would further increase to US $ 2 trillion by 2030. Due to the impact of this magnitude, the WHO has declared dementia as a disease of public importance.
The prevalence of dementia is estimated to be 6.5 % in those aged above 65. In Nepal, population above 65 years of age constitutes 4.5% of the total population. So, by general calculation, approximately 87,750 people at this moment suffer from dementia. This burden is going to double by the next decade. If we don’t start planning now, it will be too late to tackle the morbidity caused by this disease. Currently, there are just 20 neurologists, just countable geriatricians, and very few old-age care homes in Nepal; it is already a herculean task for them. With the surge of non-communicable diseases like dementia, this ‘double burden’ will have a huge impact on how the economy of the country will progress in the future.
Because of the deep-rooted stigmas about dementia, only 10 % of these people worldwide seek medical opinion; so much so, that dementia is often hidden away, not spoken about, or ignored at a time when the patients and their family needs the most support.
With the increasing life expectancy of 70.2 yrs in Nepal, according to the WHO World Bank Report 2018, population of elderly is slowly increasing in Nepal. But, due to lack of insurance coverage, not-so-strong economic status of the elderly, and the changing family structure; elderly people are often not cared for and left to strive with their problems on their own. Tackling their medical problems themselves by paying from their pocket is an insurmountable challenge.
How does Alzheimer’s disease present? What is the underlying pathology?
The main presentation is the progressive intellectual decline from previous level of functioning and performance, so as to interfere with their ability to function at work and routine daily activities. Usually, the information from family members is the key to the diagnosis of Alzheimer’s disease.
Of the following five symptoms, if two are present, a strong suspicion of Alzheimer’s dementia has to be considered, and further work up has to be started.
• A worsened ability to take in and remember new information (misplacing personal belongings and getting lost in familiar routes)
• An impairment of reasoning, complex tasking, and judgment (difficult to manage routine finances, inability to plan sequential activities)
• Impaired visual-spatial abilities, despite good vision (inability to orient clothing, not recognizing familiar faces)
• Impaired speaking, reading, and writing, if the person was doing it well previously.
• Gradual change in personality
Alzheimer disease is a continuum ranging from preclinical stage, minimal cognitive impairment (MCI), to clinical stage, which is further divided into mild, moderate, and severe stage, depending on clinical symptoms and presentation of patient. Difficulty remembering recent conversations, names, or events is often an early clinical symptom; apathy and depression are also often early symptoms. Later symptoms include impaired communication, disorientation, confusion, poor judgment, behavioral changes, and ultimately, difficulty speaking, swallowing, and walking.
The hallmark pathologies of Alzheimer’s disease are the accumulation of the protein fragment beta-amyloid (plaques) outside the neurons in the brain and twisted strands of the protein tau (tangles) inside neurons.
A healthy adult brain has about 100 billion neurons, each with long, branching extensions, and 100 trillion synapses, in the latter of which information flows in tiny bursts of chemicals that are released by one neuron and detected by another neuron. They allow signals to travel rapidly through the brain, and the information they carry creates the cellular basis of memories, thoughts, sensations, emotions, movements, and skills.
The core pathologies for cellular degeneration are accumulation of plaques consisting of beta-amyloid called oligomers that interfere with neuron-to-neuron communication at synapses. Inside the neurons, tau tangles block the transport of nutrients and other molecules essential for normal function and neuron survival.
Age is one of the risk factors for development of Alzheimer’s disease, as studies have shown increased prevalence of this disease with the aging population. There is some genetic association with earlier onset dementia seen in persons or families with presenilin gene and ApoE mutation. Also, dementia is seen earlier in individuals with Down’s syndrome—a genetic disease where the child is born with extra chromosome. Other risk factors that can predispose dementia, usually through vascular risk factors, are hypertension, obesity, dyslipidemia, diabetes, smoking, use of illicit drugs, and unhealthy lifestyle. So, a healthy lifestyle is of utmost important to get protected from developing dementia in an individual, and the common mantra is, “what is good for your heart is good for your brain”.
How can we diagnose Alzheimer’s disease?
• Obtaining medical and family history from the individual, including psychiatric history and history of cognitive and behavioral changes.
• Asking a family member to provide input about changes in thinking skills and behavior.
• Conducting problem-solving, memory, and other cognitive tests, as well as physical and neurologic examinations.
• Having the individual undergo blood tests and brain imaging to rule out other potential causes of dementia symptoms, such as a tumor or certain vitamin deficiencies.
• In some circumstances, using PET imaging of the brain to find out if the individual has high levels of beta-amyloid, a hallmark of Alzheimer’s; normal levels would suggest Alzheimer’s is not the cause of dementia
At the bedside, common tests that we do to identify cases of dementia are mini-cognitive test, Rowland universal dementia assessment scale (RUDAS) , Minimental score test (MMSE), and Montreal cognitive assessment score (MOCA) tests, which can be applied according to the situation and educational status of the patient, and these tests will help to identify cases of dementia and also will grade severity of dementia and act as objective tools for the screening of dementia cases.
It is important to note that some individuals have dementia-like symptoms without the progressive brain changes of Alzheimer’s or other degenerative brain diseases. Causes of dementia-like symptoms include depression, untreated sleep apnea, delirium, side effects of medications, Lyme disease, thyroid problems, certain vitamin deficiencies, and excessive alcohol consumption. Unlike Alzheimer’s and other dementias, these conditions often may be reversed with treatment. Consulting a medical professional to determine the cause of symptoms is critical to one’s physical and emotional well-being.
Is there a cure? How are patient with Alzheimer’s disease cared for and treated?
This is a million dollar question that has not been answered so far. We do not have a cure of this disabling disease, even with all the scientific advancement done so far. Common drugs that are used, such as Donepezil and Rivastigmine either help to increase the level of acetylcholine at the nerve synapses, or prevent the nerve cells to get destroyed by glutamate toxicity—a medicine like memantine. Lots of research have been done to prevent amyloid deposition in the brain cells, and the recent controversial approval of aducanumab (Aduhelm) by the U.S. FDA is one of the hypothesis-driven treatment of the amyloid hypothesis, but the real benefit to the patient in cognitive benefit is not proven substantially, and its cost and side effects are outweighing its medical benefit. Till the magic drug is available in the corner, the main strategies are taking care of the patient and treating their behavioral issues by a multidisciplinary team of neurologist, psychiatrist, geriatric, nursing personnel, family members, and social workers. Prevention by modifying our lifestyle and getting control of the unhealthy lifestyle like smoking, alcohol intake, sedentary lifestyle, and obesity, along with control of hypertension and diabetes if a person has those underlying diseases, are of utmost importance to get protected from this ghastly disease.
Care can be done in the following strategical steps:
1. Assessment of patient and identifying needs—periodically assessing the level of activity of daily living of the patient, along with identifying the behavioral problems, cognitive problem, physical problems, sensory deficits, feeding problem, and pain will help us to formulate a plan for the dementia patient.
2. Promoting independence, functioning, and mobility—offer graded assistance (as little help as possible to perform ADLs), role modeling, and cueing and positive reinforcement to increase independence, allowing plenty of time for the patient to perform tasks. Use of assisted devices like glasses, hearing aids, and crutches will help to keep them active.
3. Managing behavioral and psychological problems—manage physical problems like pain and constipation, which can exacerbate agitation in the patient, together with maintaining friendly and homely environment, consistency of caring persons, and giving required medication for agitation after consultation with the psychiatrist.
4. Cognitive intervention—making them oriented to time, place, and person, and using television, radio, and family albums household items to promote communication to orient them to current events, to stimulate memories and to enable them to share and value their experience.
5. Nutritional management—assuring proper nutrition, providing soft and palatable food, checking temperature of food before feeding, supporting in feeding if needed, and giving time while feeding will help in maintaining their good physical health.
6. Supporting family and caregivers—the family and caregivers have to be supported by the community and social support system, as taking care of a patient with dementia is a challenging work and carer-burden and frustration is a well-known phenomenon.
Some of the communication skills and caring tips to be followed during care of a patient with dementia include:
• Address the person by the name he or she prefers
• Approach the person from the front
• Speak to the person at eye level
• Speak slowly and calmly, and use short, simple words
• Allow enough time for the person to respond (counting to five between phrases is helpful)
• Focus on the person’s feelings, not the facts
• Use a comforting tone of voice
• Be patient, flexible, and understanding
• Avoid interrupting people with dementia; they may lose their train of thought
• Allow individuals with dementia to interrupt you, or they may forget what they want to say
• Limit distractions during communication (e.g. turn off the radio or move to a quiet place)
• Increase the use of gestures and other non-verbal communication techniques
• Observe the individual to recognize non-verbal communication
• Use gentle care-giving techniques, including: warnings before touching a person or beginning care, apologies for causing distress, and keeping the person covered and warm.
Combating the problem—joining hands together
The WHO has come out with an action plan to combat the problem of dementia. The key strategies include making dementia care a national and social priority, increasing awareness of dementia, decreasing the risk of dementia, providing access to medical care, implementing social support services, developing research and development in dementia, and strengthening the disease surveillance system.
Since Alzheimer’s disease is a progressive disease that has no obvious cure so far, the care of the patient by care givers remains the priority. From individual level and community spreading awareness about dementia, improving our lifestyle and healthy habits, spreading optimism of care, and de-stigmatization of prevailing attitudes are of utmost important. Priority has to be given to the voices of people with dementia and their caregivers; and also integration and coordination of health and social care system has to be done. The government should prioritize the long-term care of the elderly and design and implement national programs to address the issue of elderly care.
Can you imagine a situation where your dearest loved ones—your father or mother, or your grandparents— losing their memory and not being able to recognize you and their own close family members? They will not be able to remember what day, date, and year it is, and they will not be able to remember whether they have taken their meals or medicines and where they have kept their belongings. They may have, in addition, hallucination and delusion and suspiciousness and various behavioral issues that will make them not able to sleep and do their daily activities. Gradually, their memory fades away and they will be totally dependent on others for their daily needs and activities. In the following article, this disabling disease of dementia is discussed and how the person with dementia has to be cared for is highlighted, so that all of us will become aware about this disease and will be able to take care of our loved ones in situations where it is needed. This is more relevant as September is the month for spreading awareness about Alzheimer’s disease.
What is Alzheimer’s disease and related dementia? What is the burden of problem?
Alzheimer’s disease (AD) is a progressive neurodegenerative disease of the elderly, in which the brain cells slowly die due to accumulation of abnormal proteins, resulting in dementia and other behavioral problems. Dementia is a syndrome, usually of a chronic or progressive nature, caused by a variety of brain illnesses that affect memory, thinking, behavior, and ability to perform everyday activities.
Alzheimer’s disease is the most common cause of dementia, accounting for an estimated 60% to 80% of cases. Recent large autopsy studies show that more than half of individuals with Alzheimer’s dementia have Alzheimer’s disease brain changes (pathology) as well as brain changes of one or more other causes of dementia, such as cerebrovascular disease or Lewy body disease, a variant of atypical Parkinson’s disease.
Another disease with similar presentation to Alzheimer’s disease is known as Fronto temporal lobar degeneration (FTLD), in which patient presents with behavioral changes first before the memory issues sets in. FTLD includes dementias such as behavioral-variant FTLD, primary progressive aphasia, Pick’s disease, corticobasal degeneration, and progressive supranuclear palsy. In FTLD, nerve cells in the front (frontal lobe) and side regions (temporal lobes) of the brain are especially affected, and these regions become markedly atrophied (shrunken). In addition, the upper layers of the cortex typically become soft and spongy and have abnormal protein inclusions (usually tau protein or the transactive response DNA-binding protein TDP-43).
As per a report of the World Health Organization published in 2019, it is estimated that 50 million people worldwide are living with dementia, which is sure to double every 20 years, reaching 82 million in 2030, and 152 million in 2050. Majority of this burden is observed in low- or middle-income countries, constituting 68% of total cases. The problem looks even more worrisome if we say that every three seconds, someone develops dementia, amounting to 9.9 million new cases each year. In 2015, dementia costs were estimated at US$ 818 billion, equivalent to 1.1% of global gross domestic product, ranging from 0.2% for LMICs to 1.4% for high income countries, which would further increase to US $ 2 trillion by 2030. Due to the impact of this magnitude, the WHO has declared dementia as a disease of public importance.
The prevalence of dementia is estimated to be 6.5 % in those aged above 65. In Nepal, population above 65 years of age constitutes 4.5% of the total population. So, by general calculation, approximately 87,750 people at this moment suffer from dementia. This burden is going to double by the next decade. If we don’t start planning now, it will be too late to tackle the morbidity caused by this disease. Currently, there are just 20 neurologists, just countable geriatricians, and very few old-age care homes in Nepal; it is already a herculean task for them. With the surge of non-communicable diseases like dementia, this ‘double burden’ will have a huge impact on how the economy of the country will progress in the future.
Because of the deep-rooted stigmas about dementia, only 10 % of these people worldwide seek medical opinion; so much so, that dementia is often hidden away, not spoken about, or ignored at a time when the patients and their family needs the most support.
With the increasing life expectancy of 70.2 yrs in Nepal, according to the WHO World Bank Report 2018, population of elderly is slowly increasing in Nepal. But, due to lack of insurance coverage, not-so-strong economic status of the elderly, and the changing family structure; elderly people are often not cared for and left to strive with their problems on their own. Tackling their medical problems themselves by paying from their pocket is an insurmountable challenge.
How does Alzheimer’s disease present? What is the underlying pathology?
The main presentation is the progressive intellectual decline from previous level of functioning and performance, so as to interfere with their ability to function at work and routine daily activities. Usually, the information from family members is the key to the diagnosis of Alzheimer’s disease.
Of the following five symptoms, if two are present, a strong suspicion of Alzheimer’s dementia has to be considered, and further work up has to be started.
• A worsened ability to take in and remember new information (misplacing personal belongings and getting lost in familiar routes)
• An impairment of reasoning, complex tasking, and judgment (difficult to manage routine finances, inability to plan sequential activities)
• Impaired visual-spatial abilities, despite good vision (inability to orient clothing, not recognizing familiar faces)
• Impaired speaking, reading, and writing, if the person was doing it well previously.
• Gradual change in personality
Alzheimer disease is a continuum ranging from preclinical stage, minimal cognitive impairment (MCI), to clinical stage, which is further divided into mild, moderate, and severe stage, depending on clinical symptoms and presentation of patient. Difficulty remembering recent conversations, names, or events is often an early clinical symptom; apathy and depression are also often early symptoms. Later symptoms include impaired communication, disorientation, confusion, poor judgment, behavioral changes, and ultimately, difficulty speaking, swallowing, and walking.
The hallmark pathologies of Alzheimer’s disease are the accumulation of the protein fragment beta-amyloid (plaques) outside the neurons in the brain and twisted strands of the protein tau (tangles) inside neurons.
A healthy adult brain has about 100 billion neurons, each with long, branching extensions, and 100 trillion synapses, in the latter of which information flows in tiny bursts of chemicals that are released by one neuron and detected by another neuron. They allow signals to travel rapidly through the brain, and the information they carry creates the cellular basis of memories, thoughts, sensations, emotions, movements, and skills.
The core pathologies for cellular degeneration are accumulation of plaques consisting of beta-amyloid called oligomers that interfere with neuron-to-neuron communication at synapses. Inside the neurons, tau tangles block the transport of nutrients and other molecules essential for normal function and neuron survival.
Age is one of the risk factors for development of Alzheimer’s disease, as studies have shown increased prevalence of this disease with the aging population. There is some genetic association with earlier onset dementia seen in persons or families with presenilin gene and ApoE mutation. Also, dementia is seen earlier in individuals with Down’s syndrome—a genetic disease where the child is born with extra chromosome. Other risk factors that can predispose dementia, usually through vascular risk factors, are hypertension, obesity, dyslipidemia, diabetes, smoking, use of illicit drugs, and unhealthy lifestyle. So, a healthy lifestyle is of utmost important to get protected from developing dementia in an individual, and the common mantra is, “what is good for your heart is good for your brain”.
How can we diagnose Alzheimer’s disease?
• Obtaining medical and family history from the individual, including psychiatric history and history of cognitive and behavioral changes.
• Asking a family member to provide input about changes in thinking skills and behavior.
• Conducting problem-solving, memory, and other cognitive tests, as well as physical and neurologic examinations.
• Having the individual undergo blood tests and brain imaging to rule out other potential causes of dementia symptoms, such as a tumor or certain vitamin deficiencies.
• In some circumstances, using PET imaging of the brain to find out if the individual has high levels of beta-amyloid, a hallmark of Alzheimer’s; normal levels would suggest Alzheimer’s is not the cause of dementia
At the bedside, common tests that we do to identify cases of dementia are mini-cognitive test, Rowland universal dementia assessment scale (RUDAS) , Minimental score test (MMSE), and Montreal cognitive assessment score (MOCA) tests, which can be applied according to the situation and educational status of the patient, and these tests will help to identify cases of dementia and also will grade severity of dementia and act as objective tools for the screening of dementia cases.
It is important to note that some individuals have dementia-like symptoms without the progressive brain changes of Alzheimer’s or other degenerative brain diseases. Causes of dementia-like symptoms include depression, untreated sleep apnea, delirium, side effects of medications, Lyme disease, thyroid problems, certain vitamin deficiencies, and excessive alcohol consumption. Unlike Alzheimer’s and other dementias, these conditions often may be reversed with treatment. Consulting a medical professional to determine the cause of symptoms is critical to one’s physical and emotional well-being.
Is there a cure? How are patient with Alzheimer’s disease cared for and treated?
This is a million dollar question that has not been answered so far. We do not have a cure of this disabling disease, even with all the scientific advancement done so far. Common drugs that are used, such as Donepezil and Rivastigmine either help to increase the level of acetylcholine at the nerve synapses, or prevent the nerve cells to get destroyed by glutamate toxicity—a medicine like memantine. Lots of research have been done to prevent amyloid deposition in the brain cells, and the recent controversial approval of aducanumab (Aduhelm) by the U.S. FDA is one of the hypothesis-driven treatment of the amyloid hypothesis, but the real benefit to the patient in cognitive benefit is not proven substantially, and its cost and side effects are outweighing its medical benefit. Till the magic drug is available in the corner, the main strategies are taking care of the patient and treating their behavioral issues by a multidisciplinary team of neurologist, psychiatrist, geriatric, nursing personnel, family members, and social workers. Prevention by modifying our lifestyle and getting control of the unhealthy lifestyle like smoking, alcohol intake, sedentary lifestyle, and obesity, along with control of hypertension and diabetes if a person has those underlying diseases, are of utmost importance to get protected from this ghastly disease.
Care can be done in the following strategical steps:
1. Assessment of patient and identifying needs—periodically assessing the level of activity of daily living of the patient, along with identifying the behavioral problems, cognitive problem, physical problems, sensory deficits, feeding problem, and pain will help us to formulate a plan for the dementia patient.
2. Promoting independence, functioning, and mobility—offer graded assistance (as little help as possible to perform ADLs), role modeling, and cueing and positive reinforcement to increase independence, allowing plenty of time for the patient to perform tasks. Use of assisted devices like glasses, hearing aids, and crutches will help to keep them active.
3. Managing behavioral and psychological problems—manage physical problems like pain and constipation, which can exacerbate agitation in the patient, together with maintaining friendly and homely environment, consistency of caring persons, and giving required medication for agitation after consultation with the psychiatrist.
4. Cognitive intervention—making them oriented to time, place, and person, and using television, radio, and family albums household items to promote communication to orient them to current events, to stimulate memories and to enable them to share and value their experience.
5. Nutritional management—assuring proper nutrition, providing soft and palatable food, checking temperature of food before feeding, supporting in feeding if needed, and giving time while feeding will help in maintaining their good physical health.
6. Supporting family and caregivers—the family and caregivers have to be supported by the community and social support system, as taking care of a patient with dementia is a challenging work and carer-burden and frustration is a well-known phenomenon.
Some of the communication skills and caring tips to be followed during care of a patient with dementia include:
• Address the person by the name he or she prefers
• Approach the person from the front
• Speak to the person at eye level
• Speak slowly and calmly, and use short, simple words
• Allow enough time for the person to respond (counting to five between phrases is helpful)
• Focus on the person’s feelings, not the facts
• Use a comforting tone of voice
• Be patient, flexible, and understanding
• Avoid interrupting people with dementia; they may lose their train of thought
• Allow individuals with dementia to interrupt you, or they may forget what they want to say
• Limit distractions during communication (e.g. turn off the radio or move to a quiet place)
• Increase the use of gestures and other non-verbal communication techniques
• Observe the individual to recognize non-verbal communication
• Use gentle care-giving techniques, including: warnings before touching a person or beginning care, apologies for causing distress, and keeping the person covered and warm.
Combating the problem—joining hands together
The WHO has come out with an action plan to combat the problem of dementia. The key strategies include making dementia care a national and social priority, increasing awareness of dementia, decreasing the risk of dementia, providing access to medical care, implementing social support services, developing research and development in dementia, and strengthening the disease surveillance system.
Since Alzheimer’s disease is a progressive disease that has no obvious cure so far, the care of the patient by care givers remains the priority. From individual level and community spreading awareness about dementia, improving our lifestyle and healthy habits, spreading optimism of care, and de-stigmatization of prevailing attitudes are of utmost important. Priority has to be given to the voices of people with dementia and their caregivers; and also integration and coordination of health and social care system has to be done. The government should prioritize the long-term care of the elderly and design and implement national programs to address the issue of elderly care.
