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PPA (Primary Progressive Aphasia)

What is it?

People who suffer from PPA discover that they can't process language. They have trouble "finding words." Both spoken and written communication are affected. Many other causes result in a similar situation, including stroke, head trauma, "normal aging", and early Alzheimer's Disease. (Some very mild problems in thinking of words can occasionally occur as the result of normal aging, but these are never significant enough to disrupt normal communication.) However, at the onset of PPA, the person can otherwise function normally: decision-making, activities and personality are unaffected. PPA is often misdiagnosed by physicians who are unfamiliar with the condition.

PPA is a clinical syndrome. That is, it's defined by a set of symptoms, not by the specific brain disease that is causing the symptoms. An MRI scan may (but not always, especially in the early stages) show brain tissue shrinkage in the area of the left hemisphere that supports language functions. A thorough explanation of PPA and its relationship to other conditions appears on NU Medical's PPA Handbook page.

Some Web sites (including NIH's) refer to PPA as "rare." That classification is slowly changing, as neurologists learn to properly diagnose PPA. PPA patients' caregivers commonly describe multiple occurrences of misdiagnosis by physicians who are unfamiliar with the condition.

PPA is caused by a degenerative brain disease. The most common degenerative brain disease is Alzheimer's disease, and in a subset of individuals (about 20%) this may be the cause of PPA. However, the majority of individuals with PPA have a non-Alzheimer's brain disease, although there is no way to determine this until autopsy (by examining brain cells). At the present time, there are many different theories being pursued to determine what causes these brain diseases to begin, but the exact causes remain unknown. There is no cure for any of the degenerative brain diseases at this time, but medications have been developed that have been shown to be helpful in slowing the rate of memory loss associated with Alzheimer's disease, and NU is currently working on clinical trials to see if these medications are also useful in PPA.

The word progressive means that PPA is not a static condition. How rapidly the condition progresses and what brain areas (other than the language areas) will be affected over time varies widely with each individual. Caregivers new to PPA often ask, "What can we expect?" The answer to that question (and to many others related to PPA) is "We can't tell." What we do know is that the deterioration may progress into motor areas of the brain, affecting such things as movement or swallowing. Or it may move into the areas affecting emotion and judgment, producing inappropriate behavior or bouts of anger.

PPA is a cruel condition. Unlike Alzheimer's Disease, which affects memory and may also result in reduced awareness of the individual's cognitive decline, most people with PPA are aware of the changes affecting them. And caregivers have no way to predict the pace of deterioration or the direction it will take. What is known, unfortunately, is that PPA is progressive and causes a continued decline in thinking over time. And it can lead to reduced functioning over time that can increase susceptibility to other fatal illnesses (for example, pneumonia).

To put PPA in some perspective, a research staff member at NU's medical school (see below) said, "PPA today is where Alzheimer's was twenty years ago."

What's being done?

Northwestern University's Feinberg School of Medicine has a research program, headed by the foremost researcher in the field. The program is described on NU Medical's PPA page.

Useful sites

Here are some additional sources of information, many of which have been suggested by members of the online PPA support group. Some of them also appear elsewhere, but are repeated here for convenience. A site may also appear in more than one category. (The decision of the judges is final.)

Please suggest sites that others would find useful.

When you click on a link, its associated page will open in a new window. When you close that window, you'll return here.

The sites are divided into these categories:

Information & Reference

  • Information on frontal lobe dementia from Northwestern University's Medical Center.
  • The Association for Frontotemporal Dementias is a new Web site (as of January 2004), and many areas are still under construction. The site includes an excellent description of one type of PPA, called Progressive non-fluent Aphasia.
  • Understanding Primary Progressive Aphasia: The affliction explained in a question and answer format, from the National Aphasia Association. The information is somewhat dated.
  • National Library of Medicine (NLM) PubMed articles on PPA: NLM is a service of the National Institutes of Health. The link provides a "hit list" of PPA-related articles. Be aware that much of this information is in medical terminology. It may nevertheless be useful.
  • Northwestern Memorial Hospital?s PPA site: The site describes the PPA program, includes back issues of the Northwestern Alzheimer?s Disease Center?s PPA newsletter, and has a set of questions and answers about PPA. This organization also sponsors quarterly PPA group meetings in Chicago.
  • A university site with a PPA bibliography: This site is at the University of Nebraska at Lincoln. The bibliography is from a book that was to be published in 2000. The articles might be available through NLM's PubMed (see the PubMed reference).
  • The Agency for Healthcare Research and Quality (AHRQ) "The Agency for Healthcare Research and Quality (AHRQ) research provides evidence-based information on health care outcomes; quality; and cost, use, and access. Information from AHRQ?s research helps people make more informed decisions and improve the quality of health care services. AHRQ was formerly known as the Agency for Health Care Policy and Research."
  • The AMA's Program on Aging and Community Health "The AMA's program on Aging and Community Health serves to enable physicians to prevent and postpone disease and disability and to maintain the well-being, independence, and mobility of an aging population."
  • Tips on Choosing a Care Facility "Don't choose a home for today's care needs. Too often people choose a facility for an elderly person based on their care needs today. Choose a care facility that is going to meet your needs tomorrow, six months, a year, two years down the line. If your relative or friend is going to need more help-not less-as time goes on, make sure that the home you choose has the services they are going to need."
  • Check Drug Interactions: You select two or more drugs from the list, and the site shows potential interactions among them, and with foods as well. Disclaimer: "Not all drug interactions are known or reported in the literature, and new drug interactions are continually being reported. This information is provided only for your education and for you to discuss with your personal healthcare provider."
  • MayoClinic.com's drug information page: search for a drug by name or browse by name or category.

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Support Groups

  • The UK-based Pick's Disease Support Group Web site explains and provides support and information about this and related conditions. They also have a support group on Yahoo.

    The support group is described as follows: "The Pick's Disease Support Group is an International Support Group set up by carers of people with various types of fronto-temporal dementia: Pick's disease and Frontal Lobe Degeneration, Dementia with Lewy Bodies, Corticobasal Degeneration and Alcohol Related Dementia. The group meets several times a year in the United Kingdom and produces a newsletter which is always available on the internet, all other newsletters are also available. This discussion list allows carers to communicate with one another in a supportive environment."

  • The PPA support group is a Yahoo group, whose original members came from a contact list in Northwestern Memorial's PPA newsletter.
  • Northwestern Memorial Hospital?s PPA site: The site describes the PPA program, includes back issues of the Northwestern Alzheimer?s Disease Center?s PPA newsletter, and has a set of questions and answers about PPA. This organization also sponsors quarterly PPA group meetings in Chicago.
  • The Well Spouse Foundation "Illness and accident attack without warning and can happen to anyone. No two people are living in the same situation and no two illnesses exact the same toll. Alzheimer's is different from MS, heart disease is different from Parkinson's, stroke is different from accident. But all well spouses face similar problems of anger, guilt, fear, isolation, grief, and financial threat whether they are full-time caregivers or whether their partners have only moderately disabling illnesses. "Well Spouse is a national, not for profit membership organization which gives support to wives, husbands, and partners of the chronically ill and/or disabled. Well Spouse support groups meet monthly. Here, our members can share their thoughts and feelings openly with others facing similar circumstances in a supportive, non-judgmental environment. WS support groups are also an excellent source for information on a wide-range of practical issues facing spousal caregivers. Well Spouse support groups exist or are being formed in many areas of the country." The site has a support group locator, to help you find a group near you.

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Caregiver Information & Services

  • The Alzheimer's Association, a national network of chapters, is the largest national voluntary health organization dedicated to advancing Alzheimer's research and helping those affected by the disease.
  • H.E.L.P. is a not-for-profit Torrance, California-based non-profit service for older adults and their caring families and friends. Since its inception in 1996, it has helped thousands of people meet aging-related legal and care challenges. Its services include public classes, private consultations, and telephone and Internet assistance.
  • The Eldercare Locator Web site is the online portion of a larger, more comprehensive telephone-based service of the U.S. Administration on Aging that "helps older adults and their caregivers find local services for seniors."

    However, to get more specific information, call the Eldercare Locator toll-free at 1-800-677-1116. Its Information Specialists can access to a database of more than 4,800 entries. The Eldercare Locator service operates Monday through Friday, 9 AM to 8 PM Eastern time.

  • The Hoffmann Family Journal: Caring for a Spouse with Early Onset Alzheimer's "Shirley was diagnosed in February of 1995 with 'probable' Alzheimer's Disease. The diagnosis was later updated at the Mayo Clinic in May of 1995 to be 'probable' Pick's Disease. Both are variations of a class of progressively degenerative neurological diseases for which there is no prevention or cure. Shirley was 50 years old when diagnosed, which is much younger than normal for Alzheimer's victims and is referred to as Early-Onset or Young-Onset. She died on August 1st, 1998 at the age of 54. She was cared for at home by her family throughout this period. These pages contain the family's journal and experiences while dealing with the illness. It is our hope that our experiences may benefit others following this same path."
  • Management of Agitation Behavior (in Alzheimer's here, but applies to other situations as well).

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Products & Services

  • Products to help people with PPA: I wouldn't normally list commercial products or services. But Interactive Therapy seems to have their corporate head on straight. The link is to their "For Families" section, but there are also sections for practitioners. If you?re working with a speech therapist or other professional, some of the tools sold here may help both you and the practitioner.

    In particular, Interactive Therapeutics sells communicators, looseleaf products in pocket size and larger, holding cards with common words or pictures organized into useful categories to aid communication. Note that pages contain either words or pictures, not both.

  • The Augmentative and Alternative Communication Centers at UNL. "Augmentative and alternative communication is an area of clinical practice that attempts to compensate (either temporarily or permanently) for impairment and disability patterns of individuals with severe expressive communication disorders." More simply, these folks find devices and methods to help people communicate. One of a number of such places. See the Rehab Institute of Chicago entry for another.
  • The Rehabilitation Instititute of Chicago's Technology Center for the Computer, Environment and Communication "strives to meet the needs of persons experiencing difficulties in the following areas:
    • communicating their needs, wants, and ideas using speech
    • accessing their telephone quickly in cases of emergencies
    • controlling their telephone, lights, TV, VCR, stereo, and/or hospital bed
    • accessing a computer for school, work, or personal use
  • Medical identification jewelry: when you can't explain your medical condition. Medic Alert is the grandmother of all ID-bracelet organizations. It has a personal medical data repository that emergency medical staff can consult. It is a for-fee service.
  • The Alzheimer's Association Safe Return Program " is a national, government-funded program of the Alzheimer Association that assists in the identification and safe, timely return of individuals with Alzheimer?s disease and related dementias who wander off, sometimes far from home, and become lost."
  • The Eldercare Locator Web site is the online portion of a larger, more comprehensive telephone-based service of the U.S. Administration on Aging that "helps older adults and their caregivers find local services for seniors."

    However, to get more specific information, call the Eldercare Locator toll-free at 1-800-677-1116. Its Information Specialists can access to a database of more than 4,800 entries. The Eldercare Locator service operates Monday through Friday, 9 AM to 8 PM Eastern time.

  • The Family Caregivers links page of the Region One Area Agency on Aging. Although this agency serves Maricopa County, Arizona (Phoenix), the links page has some useful national entries. The site notes that it "is part of a national network of organizations established under the Older Americans Act (OAA) to respond to the needs of older adults. There are more than 650 Area Agencies on Aging throughout the United States."
  • The National Association of Area Agencies on Aging (N4A) has an Eldercare Locator to help you find a local Area Agency on Aging.

    The organization is " the umbrella organization for the 655 area agencies on aging (AAAs) and more than 230 Title VI Native American aging programs in the U.S. Through its presence in Washington, D.C., N4A advocates on behalf of the local aging agencies to ensure that needed resources and support services are available to older Americans. The fundamental mission of the AAAs and Title VI programs is to provide services which make it possible for older individuals to remain in their home, thereby preserving their independence and dignity. These agencies coordinate and support a wide range of home- and community-based services, including information and referral, home-delivered and congregate meals, transportation, employment services, senior centers, adult day care and a long-term care ombudsman program."

  • The Caregivers Marketplace™ is a group buying service with many other services. You must enroll, but there is no charge.

    The organization describes itself as: "An innovative approach to easing the financial burdens of caregiving. [It] is the nation's first free cash back and discount service for everyone who gives, gets or needs care. The mission of The Caregivers Marketplace is [...] to provide the community of 27 million Americans currently caring for a loved one at home with education, resources and - of course - everyday savings on the products they use and rely on."

  • MediSaveCanada.com, a site to buy Canadian prescription drugs.

Please suggest links that others would find useful.

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