Dementia and Alzheimer’s Disease – Facts, Figures and Proposed Code ChangesSeptember 14, 2021
In this blog we discuss Alzheimer’s disease (AD), proposed codes and modifications to the Tabular for reporting dementia, and a new drug, – Aduhelm, recently approved by the FDA to treat Alzheimer’s disease.
Increased attention to and a renewed focus on treatments for Alzheimer ’s disease (AD) occurred when the Food and Drug Administration (FDA) granted accelerated approval of “Aduhelm,” the trade name for the drug Aducanumaba in June, 2021. Aduhelm is a new type of drug that targets the fundamental pathophysiology of the disease. The drug will be marketed under the name Aduhelm.
Data indicates that the number of people diagnosed with Alzheimer’s disease and dementia has steadily increased; the National Library of Medicine, National Institutes of Health estimates the total number of people with AD dementia in 2050 is projected to be 13.8 million, with 7.0 million aged 85 years or older.
The Alzheimer’s Association currently reports these Facts and Figures:
2021 Alzheimer’s Disease Facts and Figures: At a Glance
Prevalence, Incidence and Mortality
- An estimated 6.2 million Americans age 65 and older are living with Alzheimer’s dementia in 2021.
- More than 1 in 9 people (11.3%) age 65 and older has Alzheimer’s dementia.
- Two-thirds of Americans over age 65 with Alzheimer’s dementia (3.8 million) are women.
- Deaths due to Alzheimer’s between 2000 and 2019 has more than doubled, increasing 145%.
- 1 in 3 seniors die with Alzheimer’s or another dementia.
A Brief History of “Alzheimer’s Disease”
It may be surprising to learn that “Alzheimer’s Disease” was so-named over 100 years ago, in 1910 by Dr. Emil Kraepelin, a German psychiatrist. Kraepelin named this disease after his colleague, German psychiatrist and neurologist Dr. Alois Alzheimer.
The story goes (there are several that vary in details, and some that do not portray Dr. Alzheimer in a flattering light) that in 1901, Alzheimer observed and became obsessed with a patient at the Frankfurt Asylum (a facility for people with mental disorders in Germany). The patient, Frau (Mrs.) Auguste Deter, only 51-years-old, exhibited what was considered strange behavioral symptoms, including a loss of short-term memory.
When Frau Deter died in the Frankfurt Asylum in 1906, Dr. Alzheimer arranged to have her brain and medical records brought to his laboratory in Munich, Germany. There, assisted with two Italian physicians, Dr. Alzheimer dissected Frau Deter’s brain. Using tissue staining techniques, he identified the anomalies amyloid plaques (amyloid beta plaques) and neurofibrillary tangles (tau), markers of AD to the present day. Today, an MRI can detect brain anomalies, but a definitive diagnosis of AD can only be detected after death when the patient’s brain tissue is examined.
Although AD was identified and has been a known disease for over 100 years, there is still no cure. Nearly a century had passed before the first AD drugs were approved for treatment in the 1900’s.
A New Drug, a New Hope for Treating Alzheimer’s Disease – Aduhelm
Aduhelm is the first drug approved by the FDA to treat Alzheimer’s disease (AD) since 2003, “Memantine,” common brand name “Namenda.” Prior to 2003, Tacrine (Cognex) was approved by the US Food and Drug Administration (FDA) in 1993, Donepezil (Aricept) in 1996, Rivastigmine (Exelon) in 1998, and Galantamine (Razadyne and GalantaMind) in 2001. Which drug prescribed for an individual patient is based on the stage of the disease – mild, moderate or severe.
According to the FDA, “Aduhelm is an amyloid beta-directed antibody indicated for the treatment of Alzheimer’s disease. It is approved under the accelerated approval pathway, which provides patients suffering from a serious disease earlier access to drugs when there is an expectation of clinical benefit despite some uncertainty about the clinical benefit of the drug.
Accelerated approval is based upon the drug’s effect on what is called a “surrogate” endpoint—an endpoint that reflects the effect of the drug on an important aspect of the disease—where the drug’s effect on the surrogate endpoint is expected, but not established, to predict clinical benefit. In the case of Aduhelm, the surrogate endpoint is the reduction of amyloid beta plaque.
This pathway requires the company to verify clinical benefit in a post-approval trial. If the sponsor cannot verify clinical benefit, the FDA may initiate proceedings to withdraw approval of the drug.”
- Amyloid beta – Amyloid Plaques, beta-amyloid protein
In an Alzheimer’s brain, abnormal levels of this naturally occurring protein clump together to form plaques that collect between neurons and disrupt cell function. https://www.nia.nih.gov/health/what-happens-brain-alzheimers-disease
In mid-July, 2021, the FDA narrowed the group of Alzheimer’s disease patients who should receive Aduhelm and approved a change in the label to clarify that the drug is intended only for Alzheimer’s dementia patients who are in a mild, or early stage of the disease. This decision was based on clinical trials, which showed no effectiveness on patients with Alzheimer’s disease in later stages.
Biogen, the drug manufacturer has updated the prescription information for Aduhelm, to now state that the drug “should be initiated in patients with mild cognitive impairment or mild dementia stage of disease,” instead of people who have more advanced Alzheimer’s. https://investors.biogen.com/news-releases/news-release-details/fda-approves-updated-aduhelmtm-prescribing-information-emphasize
Although Aduhelm is directed at amyloid beta plaques, another protein normally found in the brain is called “tau,” which is also believed to contribute to or cause AD. The video and website “Identify Alzheimer’s Disease Earlier” by Biogen provides information on progression, pathophysiology, challenges, early detection and diagnosis and intervention. Watch the video (3.04 minutes) for a concise explanation of how the build-up of these two proteins, amyloid beta and tau, form the basis of Alzheimer’s disease.
- Aduhelm is administered as an intravenous (IV) infusion over approximately one hour every four weeks and at least 21 days apart.
- Aduhelm costs approximately $56,000 a year, plus the additional costs of monitoring, including brain MRI’s. What a patient may end up paying will be dependent on their insurance or financial status.
“Alzheimer’s Disease” vs “Dementia”
The Alzheimer’s Association explains that “Dementia is a general term for a decline in mental ability severe enough to interfere with daily life. Alzheimer’s is the most common cause of dementia. Alzheimer’s is a specific disease. Dementia is not.
Learning about the two terms and the difference between them is important and can empower individuals with Alzheimer’s or another dementia, their families and their caregivers with necessary knowledge.” Ref: https://www.alz.org/alzheimers-dementia/difference-between-dementia-and-alzheimer-s
Here are some important concepts regarding dementia and Alzheimer’s disease to bear in mind when reporting the respective ICD-10-CM codes:
- Dementia (F03.-), a general term also known as major neurocognitive disorder, is characterized by a significant decline in cognitive functions such as memory, problem-solving, attention and language skills.
- Alzheimer’s dementia is a type of dementia. Another prevalent type of dementia is vascular dementia (F01.-).
- Alzheimer’s disease is a cause of dementia, accounting for approximately 60-80% of all dementia cases. See codes from categories G30.- Alzheimer’s disease and F02.8– Dementia in other diseases classified elsewhere.
See the Tabular and note that many of the current code categories above are further defined and specified by “behavior disturbance” such as
F02.80 Dementia in other diseases classified elsewhere without behavioral disturbance
F02.81 Dementia in other diseases classified elsewhere with behavioral disturbance
A Proposal for New “Dementia” Codes
Numerous proposed new codes and Tabular modifications for “dementia” and “Alzheimer’s disease” were presented at the March 9-10, 2021 ICD-10 Coordination and Maintenance Committee Meeting, discussed later in this blog. Although these changes and codes were not approved for use effective October 1, 2021 (FY2022), we believe that the information presented was informative and worthy of discussion considering the increasing number of people projected to be diagnosed with AD and dementia. In addition, as you may have seen the terminology used and level of specificity in the proposed code descriptors documented in your health records. This blog should help you prepare for the code changes, if and when approved.
The topic “Dementia: Stage of Severity, Behavioral and Psychological Symptoms” was presented at the ICD-10 Coordination and Maintenance Committee Meeting March 9-10, 2021. The proposal can be found in the March 9-10 Meeting Packet, pages 31 – 48. Pages 35 – 48 show the proposed Tabular Modifications. Here is a link to the PDF https://www.cdc.gov/nchs/data/icd/March-2021-proposal-packet-508.pdf
The proposal was submitted by The National Minority Quality Forum. “The National Minority Quality Forum was founded in 1998 to address the critical need for strengthening national and local efforts to use evidence-based, data-driven initiatives to guide programs to eliminate the disproportionate burden of premature death and preventable illness for racial and ethnic minorities and other special populations.” https://www.nmqf.org/.
In addition to the submitter, per CMS, “The proposal was developed in collaboration with, and has the support of, the following clinical and scientific collaborators: Amita Patel, MD, CMD, MHA, CPE; David S. Geldmacher, MD, FANA, FACP; and Maureen Nash, MD, MS, FAPA, FACP. Additional advisors include Istvan Boksay, MD, PhD; Meenakshi Patel, MD, FACP, MMM, CMD; Sandra Swantek, MD, FAPA; and Ajanta S. Vinekar, MD, FAPA. The American Association for Geriatric Psychiatry https://www.aagponline.org/ has reviewed and supports this proposal.”
The proposal (see references at the bottom of the proposal on the CMS website) explains the rationale for code modifications, as follows:
- “The burden for dementia is high to both patients, whose quality of life is greatly impacted, as well as society in terms of resources required. For example, among individuals age 65 or older, those with dementia have twice as many hospital stays per year and their rate of skilled nursing facility stays is almost four times higher.
- In addition, patients with chronic conditions and dementia use more healthcare services than patients with chronic conditions who do not have dementia.
- Current codes for dementia do not identify the stage of severity and also do not fully identify behavioral and psychological symptoms of dementia (BPSD). Both of these clinical elements are major factors in patient management strategies. Particularly because dementia is progressive, there is a great need for the longitudinal clinical data to capture the stage of severity and the key associated disorders over time to move research and clinical studies forward.”
As we previously noted, ICD-10-CM codes may be reported, in some categories, to indicate “with” or “without” a behavioral disturbance. The proposers contend that there is a need for additional detail on other “key associated disorders,” specifically
- psychotic disorders,
- mood disorders,
- anxiety, and
- agitation, all of which are used by clinicians to determine the patient’s stage of severity.
AD Stages of Severity
There are the three stages of severity that indicate the progression of dementia:
- mild dementia
- moderate dementia
- severe dementia
As explained in the code proposal, the definitions of each stage is routinely used and documented by clinicians who work with and treat AD patients. Professional societies and advocacy groups, including the American Academy of Neurology, the American Geriatrics Society, the Gerontological Society of America, the National Society on Aging, and the Alzheimer’s Association also use and apply these three stages and definitions:
- Mild dementia: Clearly evident functional impact on daily life, affecting mainly instrumental activities. No longer fully independent/requires occasional assistance with daily life activities.
- Moderate dementia: Extensive functional impact on daily life with impairment in basic activities. No longer independent and requires frequent assistance with daily life activities.
- Severe dementia: Clinical interview may not be possible. Complete dependency due to severe functional impact on daily life with impairment in basic activities, including basic self-care.
- Mild Cognitive Disorder aka Mild Cognitive Impairment, characterized by cognitive deficits, often precedes dementia, but clinically is not dementia. The proposal states “This pre-dementia state may be protracted but may also progress to dementia. A proposal to create new subcategory F06.7 with two new codes for mild cognitive disorder was presented at the September 2020 meeting of the ICD-10 Coordination and Maintenance Committee and is being re-presented at the March 2021 meeting. Related changes in the proposal for mild cognitive disorder are factored into the proposal for dementia.”
Behavioral and Psychological Symptoms of Dementia (BPSD)
Bear this in mind for future reference. The presenters of the proposal explain “Although codes exist for dementia without and with behavioral disturbances, there is a need for additional detail on other key associated disorders, particularly psychotic disorders, mood disorders and anxiety. Moreover, within behavioral disorders, there is a need to distinctly identify agitation. Associated disorders in dementia are variously referred to as behavioral and psychological symptoms of dementia (BPSD), noncognitive behavioral changes (NCBC) and neuropsychiatric symptoms (NPS). These are broader than the current coding structure. BPSD can generally be grouped into three main categories: behavioral disturbances, psychotic disorders and mood (affective) disorders.”
- Note that new/revised codes proposed would have provided the stage of a patient’s Alzheimer’s disease, enabling a link to monitoring the usage and effectiveness of Aduhelm and other drugs used to treat Alzheimer’s disease. Although the new/revised codes we discuss in this blog were not approved for use effective October 1, 2021, we suspect that the new/revised codes will be presented again for approval.
The proposed new codes capture both the severity and BPSD of patients with dementia. The full list of proposed codes as they would appear in the Tabular Modifications can be seen in this PDF Pages 35 – 48. Here is a link: https://www.cdc.gov/nchs/data/icd/March-2021-proposal-packet-508.pdf
Most of the numerous proposed new codes and changes are for “vascular dementia,” code category F01 Vascular Dementia. They are best viewed in their entirety in the PDF.
The Food and Drug Administration (FDA) granted accelerated approval as of June, 2021 of “Aduhelm,” the trade name for the drug Aducanumaba. Aduhelm is a new type of drug that targets the fundamental pathophysiology of the disease. The drug will be marketed under the name Aduhelm.
The FDA revised its approval in mid-July, 2022, updating the prescription information for Aduhelm, to now state that the drug “should be initiated in patients with mild cognitive impairment or mild dementia stage of disease,” instead of people who have more advanced Alzheimer’s. https://investors.biogen.com/news-releases/news-release-details/fda-approves-updated-aduhelmtm-prescribing-information-emphasize
Aduhelm is the first drug approved by the FDA to treat Alzheimer’s disease (AD) since 2003.
An estimated 6.2 million Americans age 65 and older are living with Alzheimer’s dementia in 2021.
Data indicates that number of people diagnosed with Alzheimer’s disease and dementia has steadily increased; the National Library of Medicine, National Institutes of Health estimates the total number of people with AD dementia in 2050 is projected to be 13.8 million, with 7.0 million aged 85 years or older.
Deaths due to Alzheimer’s between 2000 and 2019 has more than doubled, increasing 145%.
“Alzheimer’s Disease” was so-named over 100 years ago, in 1910 by Dr. Emil Kraepelin, a German psychiatrist. Kraepelin named this disease after his colleague, German psychiatrist and neurologist Dr. Alois Alzheimer.
Although AD was identified and has been a known disease for over 100 years, there is still no cure.
Nearly a century had passed before the first AD drugs were approved for treatment in the 1900’s.
“Dementia” is a general term for a decline in mental ability severe enough to interfere with daily life. Alzheimer’s disease is the most common cause of dementia. Alzheimer’s is a specific disease. Dementia is not.
Dementia is a general term, and is also known as major neurocognitive disorder.
Alzheimer’s dementia is a type of dementia. Another prevalent type of dementia is vascular dementia.
Alzheimer’s disease is a cause of dementia, accounting for approximately 60-80% of all dementia cases.
The topic “Dementia: Stage of Severity, Behavioral and Psychological Symptoms” was presented at the ICD-10 Coordination and Maintenance Committee Meeting March 9-10, 2021. It includes proposals for new codes and code modifications.
The proposed new codes capture both the stage of severity of the patient’s dementia and any behavioral and psychological symptoms of dementia (BPSD), but were not approved for used in FY2022.