Primary progressive aphasia (PPA) is a neurological effect specifically associated with certain types of dementia (Alzheimer’s Disease and frontotemporal dementia are the most common underlying forms of dementia in which PPA occurs) that results in linguistic abilities becoming slowly and progressively impaired until they are completely gone.
Tag Archive | Age-Related Illnesses Caregiver Support
Profiles in Dementia: Gene Wilder (1933 -2016)
Gene Wilder was a comedic actor best known for his performances in movies such Willy Wonka and the Chocolate Factory, Blazing Saddles, Stir Crazy, Young Frankenstein, and The Producers.
Wilder got his start in acting in 1961 in off-Broadway productions, but his acting career began to take off in 1967 when he landed a role in Bonnie and Clyde, which starred Warren Beatty and Faye Dunaway.
Wilder’s career peaked in the 1970’s, as he became a household name after masterfully capturing the essence of the title character in 1971’s Willy Wonka and the Chocolate Factory.
Wilder’s performance in Willy Wonka and the Chocolate Factory caught the attention of Mel Brooks, a filmmaker well-known for making movies that are farces or comedic parodies (perhaps because I’m too much on the serious side of things, I have never really gotten Brooks’ movies and don’t find them appealing, but they are very popular), and Wilder was featured in starring roles in the string of hit movies that Brooks made in the late 1960’s and throughout the 1970’s.
Wilder’s last movie role was in 1999, after which the actor began a more private life, with rare cameos in television shows and interviews (the last of which was in 2008 with Alec Baldwin in a Turner Classic Movie biography entitled Role Model).
According to statement from Wilder’s nephew, Wilder had been officially diagnosed with Alzheimer’s Disease in 2013, but had been showing increasingly more obvious behaviors and symptoms of the neurological disease for several years before that.
There have been many critics of Wilder’s desire to keep his diagnosis private because the critics believe that celebrities with a dementia diagnosis will bring more awareness about dementia and will spur more public and political action.
I strongly disagree with the critics and I support Wilder’s desire to keep his diagnosis private.
There is plenty of information – this blog and the books I’ve written with in-depth and practical information about dementia (what it looks like, how to travel through the journey day-to-day with our loved ones, and invaluable information on resources to best facilitate that journey), in addition to other blogs and books that focus on certain aspects of dementia or are devoted to a certain angle of dementia awareness – available to raise awareness.
I also believe that each person, and their families, should have the discretion to choose whether to make a dementia diagnosis and journey public or private.
In my mom’s case, while she was alive, I kept her diagnosis confined to a small group of people whom she and I were close to and who would want to know. I don’t really know what Mama would have chosen (it’s not a question you can really ask), but I knew I wanted to protect her and to maintain her dignity, so I made that choice with no regrets.
It was only after Mama’s death that I chose to make her dementia public, but only because I knew that our journey could help other people and that Mama would have supported that. I haven’t ever given all the details of our journey and I never will (some things just need to stay private).
Gene Wilder died at the age of 83 from complications of Alzheimer’s Disease on August 29, 2016.
Polypharmacy: A Dangerous and Potentially-Fatal Threat to Seniors
Senior citizens, including our loved ones with dementias and Alzheimer’s Disease, are an especially vulnerable part of the human population to the dangerous – and potentially deadly – risks of drug interactions associated with polypharmacy (coexisting multiple prescription medications/supplements/over-the-counter medications use). Continue reading
A Mixture of Degenerative Neurological Changes Drives the Development of Dementia
This blog stresses over and over that not all dementias are Alzheimer’s Disease (Alzheimer’s Disease is but a single type of dementia), but the Alzheimer’s Association has been quite effective at leading most people to believe that all cognitive impairment and subsequent neurological degeneration is Alzheimer’s Disease.
Not only is this general belief false, but it is also potentially very dangerous to our loved ones with dementia. Continue reading
Daylight Savings Time and Its Effects on Health, Sleep, and Our Loved Ones with Dementias and Alzheimer’s Disease
Here in the United States, most of the country started Daylight Savings Time (DST) at 2:00 a.m. on Sunday, March 13, 2016, which moved our clocks forward an hour.
Both the beginning and end of DST are tough changes on even the healthiest among us. For someone like me who has had hardwired sleep challenges all my life, the beginning of DST is particularly hard for me for about a week until my body and brain adjust to the change. Continue reading
Super Bowl 50, Football, and the Everpresent Looming Specter of Dementia
Today, February 7, 2016, the 50th Super Bowl game in NFL (National Football League) history will be played by the Carolina Panthers and the Denver Broncos. Therefore, it is appropriate to discuss the other dark and dangerous side of this football game – and all the ones played before and all the ones that will be played after – before it is played.
Super Bowl games have become extravagant and lavish productions in the last ten years or so, intended to bring into the audience people who normally either don’t have interest in the game or don’t normally watch football. Super Bowl games also represent an obscenely huge financial windfall for the NFL and for advertisers with enough money to pay for the coveted and outrageously expensive advertising spots during the game. Continue reading
Grief in a 24/7 Connected World
Grief is complex and complicated and no two people grieve the same way.
However, the more we’ve become connected in a 24/7 way through technology, the more we’ve become a shiny-happy-people (I am a huge R.E.M. fan, but this song annoyed me beyond words the first time I heard it and continues to do so now) society that focuses on the inane, on fluff, and on the ridiculous and real, palpable, tangible grief rains on that parade of lollipops, unicorns and butterflies.
As a result, it (and the grievers) are prime targets for some of the most judgmental, critical, harsh, and mean things that people can say and do to other people.
Better to grieve alone, in private, keeping the deepest secrets and pain in our souls to ourselves than to have the grief compounded by ignorance, attacks, and accusations.
The Fallacy of “Silver Bullet” Solutions to Dementias and Alzheimer’s Disease
There are no “silver bullet” solutions to the growing epidemic of dementias and Alzheimer’s Disease among the human population.
Almost daily, there are reports – from the internet, which is a numbers game in terms of reliability and accuracy of information (the more hits a site gets, the higher it appears in the search engines), not an indicator of expertise and factual information – of “silver bullet” solutions to these neurological diseases.
I suppose, because of the devastating effects of the journey through dementias and Alzheimer’s Disease that our loved ones experience and we, as caregivers, intimately deal with as we go through the journey with them, that we all our susceptible to a measure of magical thinking that leads us to even remotely consider or believe the most outlandish – and patently false – claims that a solution will reverse or cure these irreversible and incurable neurogically degenerative diseases.
What makes me angry about the “silver bullet” solutions that keep cropping up is that they prey on the vulnerable (and that includes us, as caregivers), giving false hope and, often, reaping a huge monetary reward (if they’re selling the “silver bullet” solution) at our expense.
This conduct is shameful. This conduct is cruel.
How can we as caregivers avoid falling into the “silver bullet” solutions trap?
First, we must educate ourselves and study diligently to learn and know the facts about dementias and Alzheimer’s Disease. We must understand the physiology, neurology, and neuroscience of the brain.
Going Gentle Into That Good Night has many, many articles that detail, from a layperson’s point of view, each of the dementias, including Alzheimer’s Disease, as well as factors in their development and the prominent indicators of each type of dementia.
Second, we must understand dementias and Alzheimer’s Disease in terms of their steps and what to expect in each stage and how to walk with our loved ones with these neurological diseases through each step.
This is not the oversimplified stage progression that the Alzheimer’s Association (which does not include details of exactly what happens in a detailed and sequential way and nor what and how we caregivers successfully navigation through the journey of these diseases).
Without a deep and thorough understanding of how dementias and Alzheimer’s Disease begin (often decades before any overt symptoms appear), progress, and end, we will be susceptible to the false information and misinformation of “silver bullet” solutions, which will do neither our loved ones or us any good, and will only add additional unnecessary suffering to our journeys through these neurological diseases.
Ignoring or avoiding this aspect of our educational process will not make the development of the development and progression of dementias and Alzheimer’s Disease go away. Instead, it will only make the journey for our loved ones and us as caregivers harder, more painful, and more sorrowful than they should be or need to be. The choice is ours.
Third, we need to become experts at discerning credibility and balderdash in the information coming at us.
I see articles all the time (not in just discussing dementias and Alzheimer’s Disease, but everywhere in life) from websites that are clearly not credible – look at the website name first and that will give you good insights into the quality of the information source – (and the information is misleading, at best, and totally false, at worst) presented and endorsed by a surprisingly high number of people as “truth.”
That is because of profound, and in many cases, willing ignorance. We, as caregivers for our loved ones with dementias and Alzheimer’s Disease, cannot afford to be ignorant about these neurological diseases.
There are plenty of snake oil propagators and sales people on the web. They want you to believe them either because they want to make a name for themselves or they want your money. Or both.
It’s that simple. We must be vigilant to not fall for the snake oil propagators and the snake oil sales people. We – and our loved ones with dementias and Alzheimer’s Disease – will be the losers in this equation. Always.
And, fourth, we have to recognize our own capacity for magical thinking. Even as children, we have an innate capacity to believe things that are unrealistic or untrue, but they are how we want them to be.
That is part of being human. While there is an aspect of this ability that feeds imagination and creativity productively, the majority of it leads us to the unhappiness of disappointment, disillusionment, and denial of what’s right in front of our faces.
That does us no good and it does our loved ones with dementias and Alzheimer’s Disease no good. In fact, it does us all a lot of harm in the long run.
We must be willing to confront these neurological diseases head-on, realistically, without the blinders of illusions and false hopes hindering our competent, compassionate, and loving care for our loved ones with dementias and Alzheimer’s Disease.
Our loved ones are depending on us to do this. They deserve our best and most-well-informed efforts on their behalf because they’ve placed their trust and their lives in our hands.
What will we do?
Profiles in Dementia: Rita Hayworth (1918 – 1987)
Rita Hayworth was an American actress whose career peaked in the 1940’s as Columbia Pictures’ most lucrative female lead, with a career that included 61 movies over a 37-year span.
One of Hayworth’s most acclaimed performances was in 1946’s Gilda, starring opposite leading man Glenn Ford. Hayworth also starred in two movies with Fred Astaire, who said she was his favorite dancing partner on screen.
However, fame and fortune could not stave off Hayworth’s personal demons, one of which was alcoholism.
By the late 1950’s, Hayworth’s chronic abuse of alcohol had ravaged the beauty of her younger years and had aged her considerably, making her no longer as attractive to Columbia as a leading lady even though she wasn’t 40 years old yet.
Alcoholism also created havoc in Hayworth’s personal life – she married and divorced five times (to men who in their own rights were not good choices).
By the 1970’s, when Hayworth was in her mid-50’s, the ravages of years of alcohol abuse began to also affect her brain. From 1972 until her death 1987, Hayworth’s cognitive impairment, memory loss, and repetitive outrageous behavior were what kept her name in the news headlines.
Hayworth died in February 1987 at the age of 68. Although she was the first public face of Alzheimer’s Disease (then a relatively-unheard-of form of dementia), there is absolutely no doubt that Hayworth also had alcohol-related dementia (also in 1987, mixed dementias and the many types of dementia were relatively unheard of as well), which probably hastened both her neurological decline and her death.
The Layperson’s Guide to Neural Disorders That Often Lead to Neurodegeneration and Dementia
Most dementias – Lewy Body dementia, vascular dementia, early-onset dementias, alcohol-related dementia, and Alzheimer’s Disease among them – appear seemingly suddenly as primary and distinct neurodegenerative processes without definitive causes (except in the case of genetic inheritance, which primarily occurs in rare dementias like Corticobasal Degeneration, Progressive Supranuclear Palsy, and Fatal Familial Insomnia and some of the early-onset dementias).
However, there are a group of neural disorders, which are caused by the same genetic mutation that affects lipid storage in the body, that often have dementia as a secondary symptom as the diseases progress.
These neural disorders (all these have sphingolipid metabolism dysfunction in common) – which include Niemann-Pick disease, Tay Sachs disease, and Gaucher disease – are characterized by by increased levels of a particular type of sphingolipid.
There is no cure for these neural disorders and they are all fatal (in many cases, during childhood).
Spingolipids are the biological product of a chemical process that creates a protective layer on nerve cell membranes and ensures proper – and protective – cell signaling and are critical to optimal brain function.
The genesis of sphingolipids are long-chain – also known as sphingoid – bases that normally have a length of 18 carbons, although they can also have lengths of 16 or 20 carbons. The length of long-chain bases is determined by serine palmitoyltransferase (STP), a multiprotein enzyme.
In neural disorders like Niemann-Pick disease, Tay Sachs disease, and Gaucher disease, a mutation (known as Stellar) in one of the proteins that makes up STP creates an abnormally high number of 20 carbon long-chain bases, which dramatically interferes with sphingolipid metabolism.
This causes neurodegeneration to occur. In all these neural disorders, much of the neurodegeneration begins soon after birth.
In Tay Sachs disease, neurodegeneration of the brain and spinal cord begins at about six months of age. The average lifespan is four years.
Gaucher disease has three subtypes.
In Type 1 Gaucher disease, symptoms, which include anemia, bone deterioration, and liver and spleen impairment, are non-neurological and do not materialize until middle age. The average life expectancy for Type 1 is 68 years.
Type 2 and Type 3 Gaucher disease are both neuropathic forms of the disease. Neurodegenerative symptoms include abnormal eye movements, seizures, and systemic brain damage.
In Type 2 Gaucher disease, the onset of symptoms is within three to six months of age. Deterioration is rapid; the average life expectancy is about two years of age.
Type 3 Gaucher disease is a slower onset and involving version of Type 2. The average onset of neurological involvement is late childhood into adolescence. Life expectancy ranges from the mid-twenties to, in extremely rare cases, the early forties.
Niemann-Pick disease has four types: Type A, Type B, Type C1 and Type C2.
Niemann-Pick disease Type A occurs in infants. Symptoms include enlargement of the liver and spleen (around three months of age) and a failure to thrive during the first year of life. At one year, widespread damage to the lungs occurs, and there is a progressive loss of neurological and motor function.
Along with Tay Sachs disease, Niemann-Pick disease Type A also has a common eye deformity consistent with neurometabolic disease, known as a cherry spot, that occurs within the macula and is often what initially identifies the two neural disorders.
While most children born with Niemann-Pick disease Type A die in infancy, a few may live as long as four years.
Niemann-Pick disease Type B includes most of the same symptoms as Type A (motor skills are not usually affected), but the onset of symptoms is during adolescence. Most people with Niemann-Pick disease Type B survive into adulthood, but mortality rates climb dramatically between twenty and thirty years of age.
Niemann-Pick disease Type C (C1 and C2 are caused by different gene mutations, but the symptoms are the same) is characterized by severe liver disease, severe pulmonary infections, progressive neurodegeneration, and increasing difficulty with speech and swallowing that deteriorates completely over time.
The onset of Niemann-Pick disease Type C can be at any age, but it is most commonly seen by the age of five. The life expectancy with this type is under twenty years of age when symptoms appear in childhood. When symptoms appear later, the life expectancy is ten to twenty years after symptoms begin.
Going Gentle Into That Good Night 