American Academy of Family Physicians (AAFP) 2013 Scientific Assembly

September 24-28, 2013; San Diego, CA

Defining Dementia and Alzheimer’s Disease: An Interview With William Sonnenberg, MD

Currently, 5.3 million Americans have dementia, and a new diagnosis is made every 70 seconds, increasing the disease burden. In addition to being the sixth leading cause of death, dementia is a drain on the healthcare system, tripling healthcare costs. In 2010, the total annual cost was estimated to be between $157 billion and $215 billion, with Medicare paying $11 billion per year of this cost. Although older age is the strongest predictor of dementia, a variety of other known modifiable and nonmodifiable risk factors have been reported. These include a family history of dementia; smoking; presence of a variety of diseases, such as diabetes, hypertension, hyperlipidemia, atrial fibrillation, and depression; and low vitamin D levels. Many of these risk factors are more commonly found among older adults, yet a challenge in making the diagnosis in these patients is determining whether their symptoms are indicative of dementia or are a normal part of aging.

During the AAFP 2013 Scientific Assembly, William Sonnenberg, MD, family practice physician, Titusville, PA, led an educational session that provided attendees with insights on how to examine aging patients for signs of cognitive decline or dementia and on how to counsel these patients’ family members on what is considered a “normal” part of aging and on how to cope with neurologic disorders that impair cognitive functioning. Annals of Long-Term Care: Clinical Care and Aging^® (ALTC) had the opportunity to discuss this session with Sonnenberg.

ALTC: You note in your session that the ApoE4 allele is the largest known genetic risk factor for late-onset, sporadic Alzheimer’s disease. It is currently used predominantly in the research setting to identify study participants. Do you think it will eventually become commonly used to screen older adults for their risk of developing Alzheimer’s disease?

Sonnenberg: I feel it will come into use once we find a value in identifying those at risk before they show symptoms of dementia. At the present time, identifying this gene in a patient will only be bad news, but that may change. ApoE4 is involved in lipid metabolism, and there is some evidence to suggest that this gene lowers a protein called Sirt1, which is associated with anti-aging and longevity. Animal studies have shown that some medications may be able to maintain levels of this protein. Should such medications demonstrate similar effects in humans and become approved for use, ApoE4 testing may become a useful diagnostic test. 

Although Alzheimer’s disease is the most commonly recognized dementia, there are many other forms of dementia. Can you describe some of these and discuss what distinguishes one from the other?

A full discussion would take up an entire article, but I would love to give a few brief pearls to help out. The first dementia I want to discuss is Lewy body dementia. This form of dementia is similar to Alzheimer’s disease, but patients tend to have more problems with executive functioning and with figure copying and clock drawing; however, they have better verbal memory. These patients also display bradykinesia and mask-like facies, but there is less tremor than with Parkinsonism. A prominent symptom is colorful 3D hallucinations involving humans or animals.

The second form of dementia I want to discuss is frontotemporal dementia, also known as Pick’s disease. Patients with this dementia have fewer problems with memory, but have considerable issues with disinhibition. They may make inappropriate remarks, such as sexual comments, or display childish behaviors. This form of dementia should be considered in younger patients with dementia. 

Finally, I want to discuss vascular dementia. This dementia is seen more in the setting of hypertension and has a stepwise progression. People with vascular dementia display apathy and poor verbal fluency and preservation.

How can physicians determine whether a patient has dementia or is experiencing cognitive decline as a “normal” part of the aging process?

It is often a question of severity. Forgetting things like where you left your keys is normal and common, but forgetting the names of friends and losing your way home is not. If there is any question, cognitive testing should be undertaken, such as via the Clock Drawing Test and the Mini-Mental State Examination. Generally, symptoms of mild cognitive impairment (MCI) do not affect daily life. Typically, general functioning, vocabulary, and intellectual ability remain unaffected; however, half of patients with MCI will progress to dementia.

In Alzheimer’s disease, there are two abnormal structures: beta-amyloid plaques and neurofibrillary tangles. What do you think of brain amyloid imaging? Do you think this is a promising imaging modality for diagnosing Alzheimer’s disease?

I think it is promising, but not for routine use. While elevated beta amyloid plaques are a feature of Alzheimer’s disease, elevated levels can result from other conditions as well. Therefore, this imaging modality should not be used in patients who are asymptomatic or have a confident diagnosis of Alzheimer’s disease. The Amyloid Imaging Taskforce feels that appropriate candidates are those with unexplained memory problems, those with unusual clinical presentations, and those presenting with dementia before age 65 years.¹ It should also be pointed out that this imaging is not covered by insurance.

Once Alzheimer’s disease is diagnosed, how should it be managed?

The first thing on the agenda is to address safety issues; use of firearms and/or power tools, smoking in bed, falls risk, and driving ability need to be considered. Of these, driving is often the most difficult issue for the patient, family, and physician to address. Indications that driving should be restricted include history of crashes and/or traffic citations. A Mini-Mental State Examination score below 25 or misplacement of hands on the Clock Drawing Test are other clear indications that driving and other safety issues warrant careful consideration.

The next step is to set realistic goals and keep things simple. Goals should be limited to dressing, feeding, and maintaining hygiene. A respectful, gentle approach should be used when addressing these issues. It is essential for everyone to remember that these patients were often great people who took pride in their independence.

The last thing to address is medication. First, physicians should get rid of medications with anticholinergic side effects. Next, they should consider prescribing anticholinergics and memantine; although these can be prescribed individually, I favor using these medications together. 

It seems that the medical foods market is growing with regard to Alzheimer’s disease. What do you think of these foods? Do they have any role in Alzheimer’s disease management?

I am both amused and appalled at the paucity of scientific proof that is being touted as evidence in the marketing of supplements for the treatment and prevention of Alzheimer’s disease. I reviewed the Website of one such product out there, which claims to increase the ketones to the brain as a supplement to glucose by exploiting ketone metabolism. It suggests that ketones are better for the brain because it takes three metabolic steps rather than the 11 required for glucose, and this finding was the extent of the science used to support this product. Nothing was presented resembling a comparison study, much less a double-blinded, randomized, controlled trial. Of course, the supplement manufacturer suggests that patients contact their doctors to get approval before use.

Saying this, I do believe that in the future there will be more evidence for the role of proper nutrition in the treatment and prevention of Alzheimer’s disease and other dementias. Based on current evidence, I believe the Mediterranean diet is the best one to recommend. A recently published study in Epidemiology combined data from 11 observational trials with a randomized control trial. Nine of these studies were found to support the hypothesis that a Mediterranean diet both improves brain function and lowers the risk of developing dementia. The research was conducted at the University of Exter in the United Kingdom. The intriguing aspect of this study was that increased adherence to the diet was associated with better results (ie, there was a dose response).² The other study I presented in my lecture showed a 40% reduction in both Alzheimer’s disease and mild cognitive impairment upon increased adherence to the Mediterranean diet.³

There is one final observation I want to note. Watching nutritional advice unfold over the past 30 years has taught me that when a study finds a benefit to a food item, the benefit is generally lost when that item is put in a capsule, pill, or formula.

References

1.     Carrillo MC, Blennow K, Soares H, et al. Global standardization measurement of cerebral spinal fluid for Alzheimer’s disease: an update from the Alzheimer’s Association Global Biomarkers Consortium. Alzheimers Dement. 2013;9(2):137-140.

2.     Lourida I, Soni M, Thompson-Coon J, et al. Mediterranean diet, cognitive function, and dementia: a systematic review. Epidemiology. 2013;24(4):479-489.

3.     Scarmeas N, Stern Y, Mayeux R, Manly JJ, Schupf N, Luchsinger JA. Mediterranean diet and mild cognitive impairment. Arch Neurol. 2009;66(2):216-225.

Communicating an Unfavorable Prognosis: An Interview With David Ross-Russell, MD

Facing death is a difficult prospect for patients and their families, both physically and mentally. Although these individuals desperately need guidance and information at this time, it can be uncomfortable for healthcare providers to discuss this sensitive topic with these individuals. It can also be difficult for healthcare providers to decide when end-of-life care should be initiated, particularly because medicine strives to save and extend life by treating illnesses and injuries. Determining that end-of-life care is the correct and appropriate course of action may naturally lead to some feelings of defeat; however, initiating such care in a timely manner can significantly improve these patients’ quality of life for whatever time they have remaining, whether that is days, weeks, or months.

During the AAFP 2013 Scientific Assembly, David Ross-Russell, MD, family practice physician, Wallingford, CT, presented an educational session that provided attendees with guidance on how to determine patients’ candidacy for hospice care and on how to discuss an unfavorable prognosis and end-of-life care with them. Annals of Long-Term Care: Clinical Care and Aging^® (ALTC) had the opportunity to interview Ross-Russell about these challenging end-of-life care issues.

ALTC: According to Medicare, a patient is eligible for hospice care when he or she has a medical prognosis that leads to a life expectancy of 6 months or less if the illness runs its normal course. How can healthcare providers make this determination? Are there any particular variables they need to consider?

Ross-Russell: The fact is that it is very difficult to make a definitive prognosis of impending death more than a few days to a week or so in advance without a huge degree of uncertainty. This has been demonstrated in studies of almost every terminal disease. Therefore, providers need to consider the trajectory type of the underlying condition (is it slow and gradual, such as in the case of amyotrophic lateral sclerosis; is it gradual with severe exacerbations, as with conditions like chronic obstructive pulmonary disease; or is it rapid during its final stages, as with cancer), and ask themselves the surprise question: “Would I be surprised if the patient were to die in the next 6 months?” If the answer to that question is no, then there is a high probability that the patient meets Medicare criteria for hospice care. Documenting that to the satisfaction of the recent Centers for Medicare & Medicare Services demands is another problem for the hospice medical directors.   

During your session you noted that physicians tend to overestimate patients’ prognoses in the setting of a terminal illness. Why does this occur?

I believe this stems from two sources. First, I think that many physicians are not aware of what evidence there is on prognosis that can help formulate prognostic estimates. For instance, with cancer patients a decline in functional status below 70 on the palliative performance scale is likely to signal the start of a rapid decline and death occurring within 3 months. Second, I think there is a deep-seated emotional reluctance to address the imminence of death, which causes us as physicians to enter a state of shared denial. Part of that means unconsciously ignoring signals that a patient is nearing the end. 

Is it necessarily a bad thing to express more optimism about a patient’s prognosis than what the clinical indicators might indicate? People beat the odds all the time and it seems that some patients might become extremely distraught if they are made fully aware of their anticipated prognosis.

I have no problem making entirely clear to patients who are willing to discuss prognosis that my estimate is just that—an estimate—and that I have been wrong many times. I usually follow that with an anecdote about a patient who outlived my expectations. But at the same time I stress that I do need to be honest with them as to the basis of my expectation. Not doing so would have an even greater downside. For example, if a patient is given 12 months to live but is rapidly deteriorating 3 months later the patient may develop a much greater sense of failure. Not only that, but we may have led him or her into a set of decisions around treatment, family, and finances that were never appropriate for the stage of the condition.  

What tips do you have for physicians when it comes to breaking bad news to their patients?

I like to teach and use the SPIKES model, which is as follows:

•      Set up the interview: establish a time, find an appropriate space, and decide on participants

•      Perception: determine the patients’ current understanding of his or her condition

•      Invitation: obtain the patient’s consent to discuss his/her prognosis

•      Knowledge: share the news and information with the patient

•      Emotions: acknowledge the patients’ emotions

•      Summarize and Strategize: recap and develop a plan for next steps.

More about this approach can be read online at The Oncologist.