Tag Archive | Lewy Body dementia

The Layperson’s Guide to Neural Disorders That Often Lead to Neurodegeneration and Dementia

Normal brain cellMost 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.  

Structure of cell membraneThese 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).

Anatomy of a sphingolipidSpingolipids 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.

Chemistry of sphingolipidIn 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.

A cherry red spot on the macula is a common denominator in Tay Sachs Disease and Niemann Pick disease Type 1Along 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.

 

 

Profiles in Dementia: William Shakespeare’s King Lear

William Shakespeare as a young manWilliam Shakespeare, the playwright, was one of the most intuitive and astute observers of the human race. A careful reading of his body of plays – especially the histories and the tragedies – show an author who intimately understood human nature and human folly at their very core manifestations.

In King Lear, one of Shakespeare’s most gut-wrenching plays, Shakespeare gives us an in-depth look at what dementia – and, most likely, based on the symptoms, Lewy Body dementia – looks like in action in his portrayal of King Lear.

From a big-picture standpoint, the play shows in close detail how dementia can destroy a family (and a kingdom), and it shows how family dynamics can hasten the destruction. It also shows how dishonesty with our loved ones with dementia is never acceptable

The summary of King Lear is fairly straightforward. King Lear, a monarch in pre-Christian Britain, who is in his eighties and aware of his own cognitive decline, decides to abdicate the throne and split the kingdom among his three daughters, with the promise that they will take care of him. 

The first sign of Lear’s dementia is his irrational criteria for how he’s going to decide which daughter gets the largest portion of the kingdom: not by their abilities, strengths, rulership experience, but by which one professes the greatest love for him.

His two oldest daughters are duplicitous and try to outdo each other with their professions of love for their father (they don’t love him, but they want the lion’s share of the kingdom).

King Lear’s youngest daughter, who genuinely loves her father and who is his favorite, gets disgusted with the whole thing and refuses to play the game.

King Lear, in a sudden fit of rage, then disowns his youngest daughter completely. When one of her friends, the Earl of Kent, tries to reason with the king, King Lear banishes him from the kingdom.

King Lear’s youngest daughter then marries the king of France and leaves King Lear in the hands of his two devious older daughters.

The Tragedy of King Lear - William ShakespeareBoth daughters are aware of King Lear’s vulnerability because of his cognitive decline and are intent on murdering him so that they can have everything without the responsibility of having to take care of him. They treat King Lear horribly in the process of formulating their scheme to end his life and be rid of him.

The youngest daughter comes back from France to fight her sisters, but loses and is sentenced to death instead.

While she is awaiting execution the two older sisters fight over a man they both want. The oldest sister poisons the middle sister, who then dies. 

The man the two sisters were fighting over has been fatally wounded in battle and he dies (but he reverses the execution order of the youngest sister before he dies). After his death, the oldest sister commits suicide.

Meanwhile, the youngest sister is executed before the reversal order reaches the executioners. And King Lear, upon seeing his youngest daughter dead, dies too.

Woven throughout the plot are signs that King Lear has dementia, that he knows something is cognitively wrong, and we watch him actually go through the steps of dementia throughout the play.

King Lear exhibits deteriorating cognitive impairment, irrational thinking, sudden and intense mood changes, paranoia, hallucinations, and the inability to recognize people he knows.

Lewy Body dementia seems to be evident in King Lear’s conversations with nobody (he thinks he sees them but they aren’t there) and the sleep abnormalities that are brought out in the play.

A few poignant lines spoken by King Lear give us a glimpse:

“Who is it that can tell me who I am?”

“O, let me not be mad, not mad, sweet heaven
Keep me in temper: I would not be mad!”

“I am a very foolish fond old man,
Fourscore and upward, not an hour more or less;
And, to deal plainly,
I fear I am not in my perfect mind.”

“You must bear with me:
Pray you now, forget and forgive: I am old and foolish.”

Everyone around King Lear knows he’s not himself, including his deceptive daughters, who note after he disowns his youngest daughter, how bizarre his behavior was toward someone he loved so much and how quickly his temperament changed. King Lear see

As the play progresses, King Lear’s dementia continues to be revealed in his frequent rages against fate and nature, in his disregard for personal comfort or protection from the elements, and in his eventually having fewer and fewer lucid moments in which he recognizes people and knows who he is.

If you haven’t read King Lear in a while or you’ve never read it at all, it is an entirely different experience to read it now with the knowledge of dementia as a backdrop. It’s even more tragic than we even imagined. 

 

“You Oughta Know: Acknowledging, Recognizing, and Responding to the Steps in the Journey Through Dementias and Alzheimer’s Disease” – Chapter 9 Excerpt

You Oughta Know: Recognizing, Acknowledging, and Responding to the Steps in the Journey Through Dementias and Alzheimer's DiseaseIn this tenth installment of chapter excerpts from the book You Oughta Know: Acknowledging, Recognizing, and Responding to the Steps in the Journey Through Dementias and Alzheimer’s Disease, we look at the ninth step in the journey through dementias and Alzheimer’s Disease.

This post includes an excerpt from chapter 9, which gives extensive information on how to acknowledge, recognize, and respond to the ninth step in the journey through dementias and Alzheimer’s Disease: balance, stability, and falls.

This chapter discusses all of the reasons that the neurological damage of these diseases affect balance, stability, and lead to an marked increase in fall risks and actual falls.

This is the ninth step in the journey through dementias and Alzheimer’s disease.

This chapter also discusses practical, real-time, and loving ways we as caregivers should respond and help our loved ones as we negotiate this step in the journey.

This series begins with the forward to the book and an explanation of why I wrote this book and why you should read it.

The series continues with the inclusion of excerpts from Chapter 1, Chapter 2, Chapter 3, Chapter 4, Chapter 5, Chapter 6, Chapter 7, Chapter 8, and, with this post, Chapter 9.

The steps in the journey through dementias and Alzheimer’s Disease are presented sequentially in the order in which they actually appear in the course of these neurological diseases.

There are no other books that literally walk through each step in sequential order as they emerge in the journey through dementias and Alzheimer’s Disease.

Additionally, there is no other book that discusses:

  1. The process we as caregivers acknowledge each new step – there is an acceptance period that we have to go through
  2. The process we use to guide ourselves and our loved ones with dementias and Alzheimer’s Disease through the recognition phase of each step
  3. The concrete, loving, and practical information on how we should respond and how we can help guide our loved ones’ responses

These are the things that make You Oughta Know: Acknowledging, Recognizing, and Responding to the Steps in the Journey Through Dementias and Alzheimer’s Disease unique and stand alone in the plethora of books about dementias and Alzheimer’s Disease.

going gentle into that good night divider

Excerpt “Chapter 9: ‘I Keep on Fallin’”

“While many of our loved ones with dementias and Alzheimer’s Disease are in good shape physically when these diseases begin to manifest themselves, they eventually reach this step of the journey where there is a great risk of falling. Attached to this risk is the possibility of broken bones – especially hips, which may be so badly damaged that our loved ones become confined to wheelchairs or bed for the rest of their lives – and head injuries, which can be fatal.

The reasons that our loved ones are increasingly susceptible to falls are:

  1. Gait changes

    Gait changes are common as dementias and Alzheimer’s Disease progress in our loved ones. One of the most characteristic gait changes is shuffling. This is especially pronounced in our loved ones who have Lewy Body dementia and Parkinson’s Disease, but it becomes a feature of all dementias and Alzheimer’s Disease by this step of the journey.

    Shuffling is when our loved ones don’t pick their feet up off the floor to take steps, but instead slide them across the floor to move forward. One of the inherent dangers in this is catching the front part of shoes on the floor – especially carpet – and falling forward.

    Shuffling can be both a result of neurological impairment – not remembering how to take normal steps to walk – and muscle weakness from lack of use.

    The most important thing we can do for our loved ones with dementias and Alzheimer’s Disease to help minimize shuffling is to have a physical therapist on our care teams to help regain muscle strength and work on gait normalization. Additionally, if possible, we should be helping our loved ones maintain muscle strength and walk with them daily encouraging them, both by example and by instruction, to use a normal stepping gait to walk.”

“You Oughta Know: Acknowledging, Recognizing, and Responding to the Steps in the Journey Through Dementias and Alzheimer’s Disease” – Chapter 7 Excerpt

You Oughta Know: Recognizing, Acknowledging, and Responding to the Steps in the Journey Through Dementias and Alzheimer's DiseaseIn this eighth installment of brief excerpts from each chapter in the book You Oughta Know: Acknowledging, Recognizing, and Responding to the Steps in the Journey Through Dementias and Alzheimer’s Disease, we look at the seventh step in the journey through dementias and Alzheimer’s Disease.

This post includes an excerpt from chapter 7, which provides a comprehensive look at how to acknowledge, recognize, and respond to the seventh step in the journey through dementias and Alzheimer’s Disease: sleep changes and disruptions.

This chapter shows that changes to sleep patterns and sleep disturbances, which includes sundowning, are all part of the seventh step in the journey through dementias and Alzheimer’s disease.

This chapter also discusses how this step impacts our loved ones and us as caregivers and the practical, real-time, and loving ways we as caregivers should respond and help our loved ones walk through this step in the journey.

This series begins with the forward to the book and an explanation of why I wrote this book and why you should read it.

The series continues with the inclusion of excerpts from Chapter 1, Chapter 2, Chapter 3, Chapter 4, Chapter 5, Chapter 6, and, with this post, Chapter 7.

The steps in the journey through dementias and Alzheimer’s Disease are presented sequentially in the order in which they actually appear in the course of these neurological diseases.

There are no other books that literally walk through each step in sequential order as they emerge in the journey through dementias and Alzheimer’s Disease.

Additionally, there is no other book that discusses:

  1. The process we as caregivers acknowledge each new step – there is an acceptance period that we have to go through
  2. The process we use to guide ourselves and our loved ones with dementias and Alzheimer’s Disease through the recognition phase of each step
  3. The concrete, loving, and practical information on how we should respond and how we can help guide our loved ones’ responses

These are the things that make You Oughta Know: Acknowledging, Recognizing, and Responding to the Steps in the Journey Through Dementias and Alzheimer’s Disease unique and stand alone in the plethora of books about dementias and Alzheimer’s Disease.

going gentle into that good night divider

Excerpt “Chapter 7: ‘Don’t Know If It’s Day Or Night’”

“Changes and disruptions in sleep are the next step in the journey our loved ones go through with dementias and Azheimer’s Disease. Included in this step is a phenomenon called sundowning, which we’ll explain the logic and science behind.

But first we need to talk about the science of sleep. All humans have a 24-hour internal clock that is known as our circadian clock (the term circadian rhythm refers to any biological process that completes a 24-hour cycle).

This clock, shown below, is a complex and coordinated system of neurology, hormones, environmental factors, and routines that are established from the time we are born.

circadian rhythm sundowning melatonin going gentle into that good night

Everyone’s circadian clock is unique, but each follows the general pattern shown above. In fact, the clock shown above is the ideal and the circadian clock that humans basically followed until the Industrial Revolution took place in the late 18th and early 19th centuries.

Since the full transition into the Industrial Revolution, human life and the adherence to this natural circadian clock has been altered and challenged because one of the side-effects of the Industrial Revolution was the development of artificial lighting (gas in the 19th century and electricity in the 20th century), which enabled lighting to be available 24 hours a day.

This was the byproduct of greed that served the captains of industry well (instead of limiting work hours to daylight hours only, artificial lighting enabled factories, foundries, mining operations, etc. to operate on a 24/7 schedule), but the human race definitely got the short end of the stick here.

Because the body is designed genetically, neurologically, hormonally, and environmentally to function in sync with the 24-hour circadian clock shown above, disrupted sleep and sleep deprivation has a chaotic effect on the body, even in otherwise-healthy people.

Time and again, science and medicine have shown a significant increase in accidents and serious injuries among shift workers who work at night. This includes not only production workers, but also professionals such as medical personnel. There is also a considerable amount of evidence that shows night shift workers are much likely to be injured or killed in driving accidents because they have a higher incidence of falling asleep behind the wheel going to and from work.

The most disruptive shift to the human body is the graveyard shift (usually 11 pm to 7 am). By the time these workers start their shift, the body is fully prepared (the hormone melatonin relaxes the body and mind for sleep beginning around 9 pm) to sleep. Forcing the body to do the complete opposite of what is it naturally designed to do is often counterproductive and very destructive to human health.”

Mother’s Day 2014

mama mother's dayToday is the second Mother’s Day since Mama’s death. Grief still lingers and hovers over me, punctuated even more by packing up for the impending sale of my house and subsequent move.

Because packing has brought the inevitable sifting and sorting, which entails opening boxes, containers, and drawers that have sat unopened for a while. And I’ve come across a lot of memories in the process and the tears that they are just memories now have fallen quite frequently.

While some who read this blog knew my mama, most of you don’t. So in honor of Mother’s Day 2014, I would like to briefly introduce you to this wonderful and beautiful lady that I’m honored to have known as “Mama.”

Mama was one of the most intelligent people I’ve ever known. Though beset by a hearing loss – that worsened with time – all her life, she was an excellent student and she loved learning.

Although Mama had completed medical technology training shortly after she and Daddy married, she yearned to go back to college and get more education. At 48, she did just that, ending up with a bachelors of science degree in biology and a bachelor of arts  degree in English. Her cumulative GPA was 3.5, despite the fact that she struggled through two required, but dreaded, math courses. We – she and I – worked together and got her through both of them with a C.

Almost right up until the time of her triple-dementia – vascular dementia, Lewy Body dementia, and Alzheimer’s Disease – diagnosis, Mama was taking classes of some kind. Her last formal classes were sign language classes, which Mama persuaded me to join so that we could learn and practice together.

Mama was also a voracious reader. When Mama was in elementary school, she’d spend every Saturday at the public library in Greenville, SC where she made the pronouncement to the librarians there that she was going to “read every book in the world.” They laughed, but Mama never gave up on that unattainable goal.

Mama, in spite of all the odds against her, was, as an adult, hopeful and optimistic about life. She enjoyed life and made the most of her time on this earth.

Mama had a whole lot of love and she generously poured it out on everybody who intersected with her life and who responded to it. I’m not sure Mama ever met a stranger; of the five of us, she was, even more so than Daddy, the most likely to introduce herself to someone and make them feel welcome in any setting.

Mama cared very deeply about humanity and often cried tears for the most vulnerable among us – children and the elderly – when she learned of hurts, sorrows, and oppressions that had befallen them.

Mama also loved all the four-legged friends that accompanied her and us as a family throughout her lifetime. She also cried tears for them when it was time for her and us to say goodbye to them.

Mama was the enthusiastic cheerleader in our family. Whenever any of us expressed a desire to do or try something, Mama was right behind us encouraging us to go for it, assuring us that she’d be with us all the way.

Sometimes if we were too reluctant or refused to do something and Mama believed it was something we needed to do, she’d give us an “or else” ultimatum. My only jump ever, tear-laden though it was, off a diving board into the deep end of a swimming pool when I was 9 was the result of one of Mama’s ultimatums. 🙂

mama-entertaining-bday-2003Mama had a fantastic sense of humor that always had the edge of mischief around it. She laughed easily and often and her blue eyes sparkled with joy most of the time, although dementias and Alzheimer’s Disease took more than their fair share of that away as they progressed.

Mama was always up for adventures with her family and her friends. When she and I were both in college at the same time, we arranged our schedules so that our Tuesday and Thursday classes were done by noon.

At least once a week, we’d go to lunch together, then go to Baskin-Robbin’s and get three-scoop sundaes to take to the movie theater to eat while we caught the afternoon matinee of whatever new movie was out.

On days we didn’t go to the movies, we’d either spend the afternoon walking and talking out at Wrightsville Beach (North Carolina) or Fort Fisher or in downtown Wilmington or we would go bowling or go to shoot pool (Mama never quite mastered the technique of shooting pool, but she was a pretty decent bowler back in the day).

I cannot tell you how much I treasure those memories, nor how much I miss those times. When we traveled together in subsequent years, we’d always spend a lot of time exploring together, until the last few years, when Mama’s energy and heart was winding down and she simply couldn’t manage long exploratory walks and strolls.

It broke my heart when it happened. I guess I believed she’d be enthusiastically keeping pace with me, taking two steps to every one of mine (her legs were shorter), until the day she died.

Mama was also a talented writer and storyteller. One of the memories I’ve encountered as I’ve been packing is the reminder of how much she wrote over the years of her life. 

One of those pieces of writing reminded me, though, that Mama experienced a lot of grief during her life, including the worst grief, I believe, of her life after Daddy’s death in October 1998.

She wrote these words six months after Daddy had died:

“This is the eve of the sixth month since my husband’s death. I have come a long way since that fatal day in October. I need to pause and take stock of where I’ve been and what I’ve learned since that time…

…At first the pain of my loss was indescribable and unrelenting. I was locked into my grief, unable to think or act on anything. When everyone [us kids] returned to their daily routine, I was in an abyss of hopelessness. The pain had even paralyzed my tear ducts…

…While sharing activities with others or hearing some interesting information, I could hardly wait to tell my husband and had the shock of realizing that would never again happen. When writing, I kept expecting him to knock on the office door and I’d look up and see that crooked grin and hear him say: ‘Just checking on you.’

I found myself talking to him about my problems and asking him how I’d ever solve them without him. I’d fuss at him for keeping every key he had ever possessed and I had no idea what they fit. Or I’d rage at him for saving every rubber band, paper clip, and ad infinitum.

I’d tell him he was right, I was the messiest person alive and I had to change, but I’d show him that I could. At night, I’d reach over to touch him, but only felt his robe that I left on his pillow. Those were the times the flood gates opened.”

Reading Mama’s words about her grief after Daddy’s death reminded me both of my grief after he died and my grief since her death. Somehow Mama’s words reflected much of what I’ve experienced since her death. It’s not in my face all the time, but it still hits me like a ton of bricks out of the blue more often than not.

mama-bday-2003I miss you, Mama, just like I miss Daddy. I’m a little lost at times with both of you gone and I’m often struggling with the idea of being all alone in the world, humanly-speaking.

This Mother’s Day will be bittersweet like last year’s was and all the ones in the future will be until I see you again. For you now, it is just the blink of an eye. For me, it’s a little bit longer than that.

I love you, Mama! Until we see other again, sleep well.

Going Gentle Into That Good Night – The Book is a finalist in the SeniorHomes.com Best Senior Living Awards 2014

Thank all of you who have voted so far for my book, Going Gentle Into That Good Night BookI wanted to pass what I learned on, so I wrote Going Gentle Into That Good Night .

It’s a finalist in the SeniorHomes.com Best Senior Living Awards 2014 books categories.

A panel of judges will determine the SeniorHomes.com expert winner, but your votes will determine the People’s Choice winner. Voting is open until May 12, 2014. So, if you haven’t had a chance to vote yet, I’d certainly appreciate you doing some research into my book and giving me a vote if you think it’s a good offering in this category.

Going Gentle Into That Good Night – the book – is a finalist in the SeniorHomes.com Best Senior Living Awards 2014.

In God’s Country: Northeast Tennessee and Mama

This is a remembrance of Mama (written at the blog I created for the memoir I wrote about our family), who would have been 85 today. She fought with strength, dignity, and bravely in all the journeys of her life – including vascular dementia, Lewy Body dementia, Alzheimer’s Disease, and congestive heart failure – and left an example showing us, her kids and grandkids, how to walk and endure our own journeys. Miss my mama and love her dearly. ♥

Fields of Gold: A Love Story - The Book

Eighty-five years ago today, about nine months before the Great Depression began, in Flag Pond, Tennessee, Muriel Foster Ross – my mama – was born to Samuel and Ennis Foster.

A doctor and both grandmothers were there attending the long and arduous birth that was taking a fatal toll on Mama’s mother and led the doctor, upon delivery, to dismissively say “that baby’s going to die” as he handed an underweight and frail Mama to her paternal grandmother, Grandma Foster, and turned his attention to trying to save Ennis Foster’s life.

Grandma Foster countered, “This baby’s going to live!,” and put Mama in the warming bin of the oven in the kitchen – a primitive incubator. Grandma Foster hovered over Mama through that afternoon and night, feeding her and wrapping her up in fresh towels every few hours.

By the next morning, Mama was stronger, had color, and it was…

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