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Caregiver FAQ

Nearly 400 LBD caregivers listened in on a recent scientific webinar on dementia with Lewy bodies (DLB) aimed at physicians and researchers, hosted by the Alzheimer Research Forum. Before and after the event LBD caregivers submitted over 100 questions on LBD clinical care to the panelists, which included Dr. Ian McKeith, Dr. James Galvin and Dr. James Leverenz who are members of LBDA’s Scientific Advisory Council.  (To view the webinar, click here.)

Due to the tremendous caregiver response, AlzForum turned to LBDA to provide answers to a broad assortment of caregiver questions. We are pleased to provide the first installment of answers to some of the most frequently asked caregiver questions.

 

Quick Links:

Symptoms and Diagnosis
Treatment
Living with LBD
Physician Awareness
Caregiver Information
Genetics
Research
 

SYMPTOMS AND DIAGNOSIS

What is LBD?

Lewy body dementia (LBD) is a progressive neurological disorder.  LBD is an umbrella term for two related diagnoses - Parkinson’s disease dementia (PDD) and dementia with Lewy bodies (DLB). The earliest symptoms of these two diseases differ, but reflect the same underlying biological changes in the brain. Over time, people with both diagnoses will develop very similar cognitive, physical, sleep, and behavioral symptoms.

Do all people with DLB hallucinate? My dad has a recent DLB diagnosis but does not hallucinate, thank goodness. Does this mean his diagnosis is wrong?

This is great question. Not everyone with LBD hallucinates, though many will at some point. Likewise, not all patients will exhibit the other clinical signs associated with LBD. The diagnostic criteria requires a person to have dementia plus only TWO of any of the following: hallucinations, parkinsonism, fluctuating cognitive abilities, REM sleep behavior disorder, a severe sensitivity to antipsychotic medications, or an abnormal result on a brain scan that detects levels of dopamine.

Are the hallucinations/delusions the main difference between DLB and Alzheimer's? And can people presenting with delusions otherwise score quite well on standard cognitive testing, like the MMSE?

The MMSE is a commonly-used test to detect a general decline in cognitive function and memory. Because people with LBD typically fluctuate in their cognitive abilities and often perform well on the MMSE early in the disease, the MMSE is not sensitive enough to diagnose DLB, nor is it adequate by itself to diagnose Alzheimer's disease (AD).

There are a number of distinguishing features that help differentiate DLB from AD. For example, the cognitive profile in DLB features deficits in executive functioning, such as problem solving, abstract thinking, and reasoning, while Alzheimer's features a predominant decline in memory. Hallucinations occur earlier in Lewy body dementia, while they appear later in Alzheimer's disease. Severe sensitivity to antipsychotic medications used to treat hallucinations is common in LBD, but not so in Alzheimer's disease. REM sleep behavior disorder is common in DLB, but not in Alzheimer’s. For a more detailed discussion, please visit LBDA’s webpage on LBD symptoms.

Could you address sleepiness? My husband is very tired during the day. Are there ways to combat these symptoms of the disease?

Sleep disorders like sleep apnea, restless leg syndrome and REM sleep behavior disorder are common in LBDs. Sleep disorders often prevent the person with LBD and their caregiver from getting sleep. Excessive daytime sleepiness, despite adequate rest at night, is also common in LBD. Ask your physician to order a sleep study to identify and treat ALL underlying sleep disorders. It is also important to review sleep hygiene such as use of alcohol, caffeine, chocolate or other substances late in the day. Many foods and over the counter medications can have effects on sleep. If excessive daytime sleepiness is a severe problem, a sleep specialist may recommend the use of medication such as a stimulant to promote daytime arousal.

Does anyone with LBD ever recoup mental capabilities?

There are a number of things that can impact cognition in LBD in the short run. Cognitive abilities fluctuate in LBD, making a person appear to function better on one day, and worse on another. Illness, pain, infection, sleep disruption and other medical issues can impact cognition negatively as well. Some people with LBD respond to treatment with cholinesterase inhibitors (originally intended for Alzheimer's disease) very positively. But because LBD is a progressive disease, there is irreversible long term cognitive decline.

Are we getting any closer to confirming diagnosis prior to autopsy?

Right now, 2 out of 3 cases of dementia with Lewy bodies go undiagnosed, and there are no widely-available tests to definitively diagnose it. There are some very promising biological indicators in development to identify LBD through cerebral spinal fluid, blood, and brain imaging; however these are still several years from being ready for clinical use.

My father passed away from DLB, or so we thought. We donated his brain for research and the autopsy came back as having no Lewy bodies. Do LBD and Alzheimer’s mimic each other?

Alzheimer's disease (AD) and Lewy body dementias are both neurodegenerative diseases and share many overlapping symptoms. The clinical criteria permit correct classification the majority of time however, the criteria are not perfect. Among the most common cause of disagreement between autopsy reports and clinical diagnosis is when an autopsied LBD cases was diagnosed with AD. Less commonly a clinically diagnosed LBD case will really be caused by AD. This highlights the need for more research to develop better clinical diagnostic tests.

How do I find a local neurologist who specializes in DLB?

Family physicians are a great, first-step resource if you are experiencing any cognitive, emotional, behavioral or physical changes. However, neurologists generally possess the specialized knowledge to diagnose specific types of dementia or movement disorders, as do geriatric psychiatrists and neuropsychologists. However, these specialists may require a referral from your primary care physician. Geriatricians, who specialize in treating older adults, are also frequently familiar with the different forms of dementia.

If you have access to a hospital affiliated with a medical school, the hospital may have a clinic specializing in dementia or movement disorders where you may find a high level of diagnostic and treatment capability.

The Lewy Body Dementia Association's Scientific Advisory Council is comprised of leading experts in Lewy body dementias. Click here to learn more.

 

TREATMENT

What is the best treatment for DLB?

Right now, cholinesterase inhibitors are the gold standard in LBD treatment. People with LBD may obtain more benefit from these drugs than people with Alzheimer's disease. Treating sleep problems is also important, as any unresolved sleep disorders may increase confusion or behavioral problems. And very conservative treatment of parkinsonism or hallucinations is important in order to avoid medication side effects. Other symptoms, such as depression and changes in blood pressure regulation, should also be evaluated and treated.

Is DLB responding to any meds at this point? If so, which ones should be considered and what side effects are associated with them?

The symptoms of LBD are often quite responsive to treatment. Information on the most common LBD treatment options can be found at by clicking here.

Side effects vary for each person, but the most typical are as follows: Cholinesterase inhibitors, used to treat cognition, sometimes cause gastrointestinal upset. A recent report suggests that cholinesterase inhibitors may increase the risk of fainting spells (syncope). Levodopa, used to treat movement problems, can sometimes cause or worsen hallucinations. And many people with LBD are very sensitive to medications used to treat hallucinations.

NOTE: Up to 50% of patients with LBD who are treated with any antipsychotic medication may experience severe neuroleptic sensitivity, such as worsening cognition, heavy sedation, increased or possibly irreversible parkinsonism, or symptoms resembling neuroleptic malignant syndrome (NMS), which can be fatal. (NMS causes severe fever, muscle rigidity and breakdown that can lead to kidney failure.)

Is donepezil (Aricept) the best drug to counter memory loss in DLB? If not, what is?

There is no evidence that any one cholinesterase inhibitor is better than another for LBD cognitive symptoms.

Is Exelon likely to exacerbate hallucinations in DLB patients?

No, in fact, that class of drugs (cholinesterase inhibitors) has been shown to reduce hallucinations and other behavioral disturbances.

Does memantine (Namenda) have value for a DLB patient?

While there has been some conflicting data on memantine in LBD, new research from a small clinical trial shows memantine may be beneficial in LBD. Data from a larger trial is currently being analyzed and will provide a clearer picture in the near future.

It has been suggested to me that quetiapine (Seroquel) is the only medication that should be given to my husband who has DLB when he has “bad behavior.” What is your opinion and suggestion for the best medication for these behavioral problems? They make caregiving much harder.

The first course of treatment for LBD, cholinesterase inhibitors, has been shown to be effective in treating hallucinations and other psychiatric symptoms of LBD. However, the effect is not immediate. If hallucinations they are disruptive or upsetting, physicians may recommend a cautious trial of a newer antipsychotic medication.

Your doctor is showing caution for a good reason. Severe sensitivity to neuroleptics is common in LBD. Neuroleptics, also known as antipsychotics, are medications used to treat hallucinations or other serious mental disorders. While traditional antipsychotic medications (e.g. haloperidol) are commonly prescribed for individuals with Alzheimer’s with disruptive behavior, these medications can affect the brain of an individual with LBD differently, sometimes causing severe side effects. For this reason, traditional antipsychotic medications like haloperidol should be avoided. Some newer ‘atypical’ antipsychotic medications like risperidone may also be problematic for someone with LBD. Quetiapine is preferred by some LBD experts. If quetiapine is not tolerated or is not helpful, clozapine should be considered, but requires ongoing blood tests to assure a rare but serious blood condition does not develop. Hallucinations must be treated very conservatively, using the lowest doses possible under careful observation for side effects.

 

LIVING WITH LBD

In what age range does DLB begin?

LBD is most often seen in the over-60 population, though like other neurodegenerative diseases, there is a small percentage of people who experience and earlier onset in their 40's and 50's.

What does the typical progression of DLB look like? Can you please describe the stages of the disease, so I know what to expect as it progresses?

Unlike Alzheimer's disease, which has clearly defined stages, LBD progression is much less predictable. Symptoms often appear in varying combinations and severity, making each person's experience unique. The prognosis is also different for each person and may be affected by your general health or the existence of unrelated illnesses. Because LBD progresses at varying rates for each individual, it is not possible to determine how long someone may live with the disease. The average duration of LBD is typically five to eight years after the onset of obvious LBD symptoms, but may range from two to twenty years. It is important to remember that this is a disorder that progresses gradually over years, not days or months.

My father was just diagnosed with DLB. It came on suddenly, though looking back we see other signs that we didn't realize at the time are a part of the disease. He doesn't know he has DLB. I don't want to tell him. Should I?

That decision may depend on the cognitive ability and temperament of the individual with LBD. While some people may find a dementia diagnosis distressing, a recent study indicates that most individuals actually find some relief in knowing the diagnosis and in understanding how this relates to their changing abilities.

My father has DLB. It wasn't until I went with him to a doctor’s appointment that he got diagnosed (turns out he wasn't telling the whole story to his doctor). What I find most difficult in caring for my father is his thinking he is all right and everyone else has a problem. Patience is a great asset for repeating directions, plans, and whatever comes up. Is there anything else we can do to help him keep focused and slow the disease?

Not everyone with LBD retains an insight into why they cannot do some of the things they used to do. So maintaining a meaningful and structured life may benefit both the person with LBD and the family caregiver. Encourage him to be an active partner with you in a comprehensive treatment program, especially by remaining socially and physically active, as they are both good for overall cognition. And treatment with a cholinesterase inhibitor is highly recommended.

My husband's cognitive abilities go up and down like a yoyo. Is this "normal"?  He was diagnosed three years ago with Alzheimer's, but has since been told he has LBD with Parkinson's symptoms. He is sometimes better now than he was three years ago.

As opposed to calling something “normal” or “abnormal”, it is probably better to comment that the “yoyo-ing” or fluctuating course of your husband’s disease is consistent with the cognitive fluctuation feature of LBD. His partial improvement may be attributed in part to his early treatment when he was diagnosed with AD.

How do I get family members to understand that some of the behaviors from our mom is the disease and not mom?

The best thing you can do is to educate them about the disease. Send them to the Lewy Body Dementia Association's website to learn more about LBD and to hear the stories of other families like yours, posted at LBDA’s online community.

I have a family pattern of DLB. Can I prevent getting it?

Most dementias, including Alzheimer and Lewy body diseases are not genetic. Because research has not yet unlocked enough answers about LBD’s root cause, we do not know yet how to prevent it. Living a heart-healthy lifestyle has been shown to reduce the risk of all forms of dementia, as has staying cognitively and socially active and physically fit.

My wife is now diagnosed with DLB after having had PD for 11 years. I need to know what I am in for. Please describe "rapid decline" regarding future months or years.

Technically this would be a case of PD dementia (PDD). Both PDD and DLB are Lewy body dementias – the difference being largely what the first symptom presentation was. Most individuals experience a gradual disease progression lasting well over 5 years, though a small percentage of people experience the full disease progression in as few as 2 years.

My husband has had two quadruple bypass surgeries, the last one in 1994 at age 60. Months after the last surgery he went back to work but the math he had to do for his job was too much for him. He just couldn't do it. He had to give up a job he loved and had been doing for over 30 years. He also made a mess of our checkbook many times. He was most difficult to live with, as opposed to normally being a kind, loving, and gentle man. He had vascular surgery again four times in 1997 and was off-the-wall difficult to handle. He was semi-conscious for days and used his hands to write in the air. He had a nurse with him 24/7 the first few days. He saw cats in the register overhead, etc. It was also after that that he could no longer do things around the house that he had always done. He just couldn't figure out how to do them. He finally asked me to help. I believe he began having DLB in 1994, most definitely since 1997. Is it possible that his dementia could have begun so long ago?

Sometimes DLB can have a very slow, progressive path. An illness or surgery may reveal LBD underlying symptoms that were not apparent at a clinical level, leading to a new diagnosis. People with LBD also often respond to certain anesthetics and surgery with acute states of confusion or delirium, and may have a sudden significant drop in functional abilities, which may or may not be permanent.

In addition, cerebral vascular disease is commonly co-existent with cardiovascular disease. Open-heart surgery is a tremendous stress for the body and the brain in particular. Many patients have periods of post-operative confusion, most recover but not all do.

It is always difficult to pinpoint exactly when neurodegenerative diseases begin because there is probably a period of several years where brain changes are occurring yet patients do not yet exhibit symptoms.

My father was diagnosed recently with DLB. He was in and out of different hospitals five times, then ended up in a care facility where he got better. So mom took him home and now he doesn’t remember being sick. He seems fine. How can that be?

In all dementia, periods of time (particularly more recent events) may seem less clear or even forgotten. Some of these forgotten events can be quite climactic such as a recent hospitalization. In addition, certain medications can exacerbate LBD symptoms, as do pain, infection, and illness. In addition to addressing other underlying health problems, in-patient care sometimes provides the opportunity for physicians to discontinue certain medications, identify which ones may be problematic, and start them on more appropriate medication regimes. 

 

PHYSICIAN AWARENESS

My dad has DLB and Alzheimer’s. The most distressing thing so far as a caregiver is some doctors’ lack of knowledge about DLB and that DLB is different from other types of dementia when prescribing medication. Is this just my sense?

It can take many years from the time a new disease is defined and for awareness of that disease to make its way through the entire medical profession. The diagnostic criteria for DLB was first published in 1996, and while most neurologists, psychiatrists and geriatricians are becoming more familiar with it, awareness of the disease, its symptoms and treatment issues is still low among generalists and other specialties.

My wife sees a head of a clinical department at an Ivy League medical school for an eye condition. When I told him about her dementia with Lewy bodies, he asked: “What’s that?” Where can I point the doctors so they can catch up on DLB?

The Lewy Body Dementia Association has just launched physician resources on their website. Physicians, nurses and other health care professionals will now find easy-to-read tables and figures on LBD diagnosis and treatment options along with family- and patient-centered publications that can be downloaded or ordered at no cost at www.lbda.org/go/physicians.

I am very much in agreement with the frustrations associated with diagnosis educating physicians! My husband was initially diagnosed with AD, which we treated for six years. Doing more reading, I discovered that he had all the symptoms of DLB. His REM sleep disorder goes back at least 30 years. He also has had no sense of smell for that many years. The Mayo clinic saw him in Scottsdale and agreed with the probable diagnosis of DLB. A well-regarded senior care physician made the statement that "while the universities may be trying to discern one type of dementia from another, they virtually all come down to Alzheimer's." Is that true?

The underlying brain pathology and symptoms are different among the many types of dementia, and there are important differences in treatment as well. People with Lewy body dementias need an early differential diagnosis to provide them with optimum care and to avoid unnecessary exposure to certain medications (like traditional antipsychotic medications, i.e. haloperidol) that may cause severe and sometimes irreversible side effects.

Many patients with LBD also have Alzheimer disease pathology at autopsy however, clinical, cognitive and behavioral features are distinct and research has shown differences in meaningful outcomes such as loss of functional abilities, nursing home placement and death.

How do I tactfully suggest that we have DLB, not Parkinson's and Alzheimer’s?

If your physician is not familiar with Lewy body dementias, providing them with publications from the Lewy Body Dementia Association can provide them with the information they need to make an accurate diagnosis. You can request a physician packet be mailed to your doctor by emailing LBDA lbda@lbda.org.

 

CAREGIVER INFORMATION

What is the best way for caregivers to deal with DLB?

This is a complicated disorder with an often unpredictable course. So there are a number of things that will help. First and foremost, become educated about the disease and be prepared to educate those around you (including some medical professionals). Second, find the support you need for everyday caregiving and locate resources in your community BEFORE you need them. And last, but not least, connect with other LBD families for understanding and support. The Lewy Body Dementia Association's website, www.lbda.org, is a great place to start.

What is the best way to get DLB patients involved in activities to help stimulate them if they do not want to do them?

It is difficult to get people to do things they do not want to do, regardless of whether they have dementia. There can be many reasons for not wanting to participate; one of the more common ones is fear of the unknown. Slowly introducing a new activity in the comfort of a familiar surrounding may reduce anxiety.

Apathy (or lack of interest and motivation) is a common symptom of LBD, as is depression. Talk with your loved one's physician about whether or not there are symptoms of depression or apathy as the patient may benefit from an antidepressant medication.

Which websites are the most reputable for the latest DLB medical information?

The Lewy Body Dementia Association has a wealth of information on LBD for both families and physicians. Visit www.lbda.org. Related dementia and movement disorder organizations can also be found on LBDA’s website by clicking here.

Is there a "beginner's book" for the caretaker and the family? We need to read up on what to expect, what we can do. Thanks!

The Lewy Body Dementia Association lists a number of books on LBD and related dementias on their website, which you can find by clicking here.

 

GENETICS

Is DLB hereditary?

Because DLB is biologically related to Parkinson's disease, from a genetics point of view, what holds true for Parkinson's will hold true for DLB. So, yes, a small proportion of families with DLB will have a genetic connection, but the vast majority of cases are considered 'sporadic,' meaning no genetic causes have been identified.

 

RESEARCH

My husband is 66. He was diagnosed with Alzheimer’s seven years ago. Four years ago after extensive testing, he was diagnosed with DLB with parkinsonian symptoms. He is on memantine (Namenda), carbidopa (Stelevo), selegiline (Zelapar) and the rivastigmine (Exelon) patch. He no longer drives and is having more trouble putting together sentences, dialing a phone, using the remote control, etc. He does mow the grass but misses a lot of it. He is now having much more trouble with grandchildren’s names, etc. Do you think research is getting any closer to reversing his problems?

At this time, we don't know what causes LBD. We do know that naturally occurring proteins misfold in the brains of people with LBD but to date we do not know fully comprehend the steps that cause the proteins to misfold. Until we understand the underlying disease mechanism, we are unable to halt the progression of the disease or reverse it.

Is LBD being aggressively investigated the way that AD is starting to be? Is your research funded by the Alzheimer's funders? Should there be a movement to distinguish LBD on a fundraising level or is that counterproductive due to the disease overlap?

Research into LBD is gaining attention from both the Alzheimer's and Parkinson's disease research communities, as it represents a link between these neurodegenerative diseases. Thus, research into one of these diseases often contributes to the understanding of the others. However, LBD research funding still lags behind, despite having approximately the same patient population as Parkinson's disease.

For more information on how to contribute to LBD research, visit LBDA's website.

When my loved one passes away, should I consider allowing an autopsy to aid in research to LBD?

A brain autopsy is the only definitive way to diagnose Lewy body dementia, but an autopsy alone does not benefit LBD research. Participating in a research study that includes an autopsy is one of the best ways to contribute to the growing body of LBD knowledge.

What research agency do I contact if I am willing to participate in a study about early biomarkers of DLB?

www.ClinicalTrials.gov provides regularly updated information about federally and privately supported clinical research in human volunteers. ClinicalTrials.gov gives you information about a trial's purpose, who may participate, locations, and phone numbers for more details.
 

 

Edited by Dr. James Galvin, member of LBDA's Scientific Advisory Council