Lewy Body Dementia (LBD) is the second most common form of degenerative dementia in the elderly, affecting an estimated 1.3 million people in the United States, yet it is most often misdiagnosed as Alzheimer’s disease. According to experts at the Lewy Body Dementia Association (LBDA), nearly 80 percent of people with LBD received a diagnosis for a different cognitive, movement or psychiatric disorder before ultimately learning they had it. Misdiagnosis impacts patient and caregiver alike. The LBDA’s recent report, Caregiver Burden in Lewy Body Dementias, reveals that people with LBD and their caregivers face barriers to obtaining an early diagnosis and that caregivers experience moderate to severe emotional burden as well as a sense of isolation because so few people know about LBD.
Dementia: Not Just Alzheimer’s
Although it’s not as familiar to many people as other dementias, LBD affects an estimated 1.3 million individuals and their families in the United States. LBD is an umbrella term for two related diagnoses, Parkinson’s disease dementia and dementia with Lewy bodies. 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.
In 1817, Dr. James Parkinson first reported on the “shaking palsy” that “spared the intellect.” In 1912, Dr. Friederich Lewy reported microscopic protein accumulations in the brainstems of autopsied people with Parkinson’s. Over time, it became acknowledged that a large proportion of individuals with Parkinson’s also developed cognitive impairment that progressed to dementia. In 1961, the protein deposits that are now called Lewy bodies were also linked to progressive dementia that did not start with Parkinson’s disease, and by 1990 researchers across the world were using different names to describe a single disease that had motor, cognitive and psychiatric symptoms. In 1996, an international consortium established a single set of diagnostic criteria for the disease now known as dementia with Lewy bodies.
In 2007, researchers and clinical experts in both dementia and movement disorders established a new consensus that Parkinson’s disease with dementia and dementia with Lewy bodies probably share the same underlying disease mechanism and outlined important priorities for the future to lead to better diagnosis and treatment. Because LBD symptoms can closely resemble more widely recognized diseases like Alzheimer’s disease (AD) and Parkinson’s disease, it is under-diagnosed. Many physicians and other medical professionals are not familiar with the symptoms and management of LBD.
LBD’s primary symptom is a progressive dementia that features memory and language impairment and visuospatial and executive function impairment. Additional symptoms vary and can include fluctuating cognitive abilities, visual hallucinations, parkinsonism, rapid eye movement (REM) sleep behavior disorder and severe sensitivity to neuroleptics (medications used to treat hallucinations). Respondents to the LBDA survey reported these frequent early symptoms:
- Memory problems
- Shuffling or other walking problems
- Lapses or fluctuations in attention or alertness
- Driving difficulties
- Hand tremors
Demands On Caregivers: The “Caregiver Burden In Lewy Body Dementias” Findings
The LBDA’s Internet-based survey of family caregivers was designed to better understand the impact of caring for a relative with LBD and was the first of its kind to highlight the plight of LBD families. Between December 2007 and April 2008, when the survey was posted on the LBDA website, 962 people with a relative with LBD completed it. Respondents included both people currently providing care for someone with LBD and people whose relative with LBD had already died. (A note about the responses: The results presented in the report include all respondents for those questions concerning characteristics, symptom onset, diagnosis and physician satisfaction. For the analysis of questions involving current levels of disability and current emotional or behavioral problems of the person with LBD, caregiver burden and resources being used, the results include data for only those people who were still caring for someone with LBD.)
The LBD Caregiver’s Profile:
- 88 percent of survey respondents were women 44 percent were daughters of the person with LBD and 35 percent were wives
- 64 percent of respondents indicated that they were the person who had primary responsibility for care of the person with LBD
- 46 percent of respondents lived in the same household as the person with LBD
- 62 percent indicated they saw the person with LBD daily
In contrast to Alzheimer’s disease (AD), significantly more LBD caregivers are women and are more often the spouse of the affected person (compared to AD, LBD is slightly more common in men than women).
Patient & Caregiver Challenges
Caregivers experienced significant barriers in obtaining a diagnosis for their loved ones. Most saw multiple physicians over more than a year before their relative was diagnosed with LBD and more than
75 percent of those with LBD were given a different diagnosis at first. Given the evidence that early, aggressive treatment with cholinesterase inhibitors may be even more beneficial to persons with LBD than persons with Alzheimer’s disease, these barriers are especially significant. Early diagnosis also provides physicians an opportunity to minimize exposure to medications that may aggravate symptoms, such as traditional neuroleptics, say the experts at the LBDA. It is estimated that almost 60 percent of persons with LBD may experience severe, potentially irreversible reactions to neuroleptics, which suggests traditional drugs, such as haloperidol, fluphenazine and thioridazine, should be avoided.
In addition to its role in good medical care, early diagnosis allows families and caregivers the time to plan for the expected decline, such as preventive steps to improve home safety, given the tendency for recurrent falls and rapid fluctuations in attention. Families also will have time to develop a better understanding of their role in patient care, including assistance with daily activities and provision of social and cognitive stimulation.
LBD is a complex disorder affecting cognition, mood, sleep, movement and behavior, and its symptoms often require a team of collaborating health care providers. In this survey, persons with LBD routinely
saw an average of three physicians for ongoing care–it would not be unusual for a person to receive treatment for different LBD symptoms from a primary care physician plus specialists in neurology, psychiatry and urology. Today’s medical system is not set up to deal with the level of coordination needed between different physicians. More than half of the survey’s respondents had difficulty coordinating the care of different physicians, including conflicting medication orders and symptom management priorities of different physicians and dealing with adverse drug reactions.
Loss of independence often occurs early in LBD, with the inability to manage one’s own medications and finances. Driving is commonly curtailed early in LBD due to visuospatial problems, hallucinations or fluctuating cognitive abilities. LBD caregivers must provide increasing supervision and vigilance as the person with LBD experiences increasing executive dysfunction (affecting judgment and problem-solving skills), changing levels of cognitive abilities from day to day or even hour to hour, early incontinence products, hallucinations and an increased risk for falls due to muscle rigidity or syncope (fainting). Recent studies demonstrate that LBD families need considerable resources and assistance from healthcare professionals and other health-related agencies, possibly even more than families caring for a relative with Alzheimer’s disease.
The Caregiver Burden
Respondents reported medium to high levels of burden brought on by the demands of caring for their relative:
- 38 percent of respondents reported they felt very or extremely burdened on 7 out of the 12 items measuring burden.
- Family members reported the highest burden when describing the impact of providing care on their personal lives, health and emotional well-being.
- One quarter of the sample said they felt that no one understood what they were going through.
- Over 80 percent said they felt somewhat or very isolated because of their involvement in providing care.
Most respondents indicated that their relative with LBD was not currently receiving paid help to assist with care, and most had not received paid help in the past. To help them cope, 23 percent of respondents were attending a support group and 21 percent reported their relative used an adult day services program. Among those respondents whose relative was receiving paid help, a majority was satisfied with the help that they received, but most rated the cost of services as a problem.
A majority of respondents also indicated they dealt with a crisis involving their relative within the past year, often due to behavioral problems or a sudden decline in functional ability. During a crisis, the most frequent place where respondents sought help for their relative was a hospital emergency room. The most common behavioral problems reported by caregivers included talking aggressively, shouting, hitting or attempting to hit someone and having hallucinations and delusions. Inappropriate sexual behavior also was reported. When behavioral problems dramatically increase and require emergency treatment, physical ailments like bronchitis, pneumonia, urinary tract infections or pain are often the cause. Medications also may cause agitation leading to behavioral problems. Falls and wandering are common in LBD and may necessitate a trip to the hospital, as would health emergencies unrelated to LBD. This frequent need for emergency care highlights the importance of education about LBD for emergency room staff and for close medical management by primary care physicians.
The high levels of burden reported by LBD caregivers result not just from the time, effort and energy it takes to provide care, but also from increased emotional and financial burdens. Disrupted sleep, depression and decreased health are also common in dementia caregivers. Social isolation can occur as a result of caregiving responsibilities as well as a lack of emotional support from being in a situation not easily understood by others. Financial burden can result from loss of income, increased medical expenses, and the often unreimbursed costs of in home supportive services, respite care and long term care.
Respondents indicated the types of services and assistance that they would like to have for their relative with LBD and themselves including web based information about LBD and an on-line support group and directories of physicians who were skilled in LBD diagnosis and treatment. Information about nursing homes skilled in LBD care, new medication trials for treatment and local caregiver support groups were also mentioned.
Help On The Horizon
The Lewy Body Dementia Association was formed in 2003 in response to a lack of information on LBD and support resources for LBD families, with the mission to provide LBD outreach and education, raise LBD awareness among the general public and medical professionals, and advance LBD research. Initiatives to be emphasized as a result of the survey include providing physicians with the tools needed to differentiate and treat the four most common causes of dementia (AD, LBD, stroke and frontotemporal dementia), with continuing medical education on LBD to help primary care and other physicians better manage the ongoing care of their patients with LBD and ways to foster better communication among physicians to prevent conflicting medication orders and develop LBD symptom management priorities. For more information, go to lbda.org