Where's the Rage?

“Where’s the rage? Why is the medical community so unaware aware all of those LBD drug issues? Why do I have to be the one who stands against the system and demand the care my wife needs? What happens to those who don’t have someone as assertive—and knowledgeable—as I am to stand up for them? Why don’t the professionals KNOW? They are supposed to be the ones with the training.”

Bill came up to me after our presentation, asking these questions—obviously feeling the rage himself and wanting to know why it wasn’t endemic. Actually, medical personnel ARE better trained than they were ten years ago. In 2002, most primary physicians had not even heard of LBD. Now they usually know about it although they may not be able to identify it or may still believe that since “all dementias are treated alike”, it is not necessary to know just what kind you have.

Spreading the word about any new disease is a long, painful process. I read somewhere that it takes 20 years for a disease to become known to the general public—and, I suspect, to be more than mentioned off-handedly in medical schools. LBD was only identified as a disease in 1996. That means it still has at least four years of relative invisibility. Often more.

I mentioned this to Bill. “But Lewy bodies have been known for many years,” he returned. Yes, since 1912, but then, only as something that was present with Parkinson’s disease. Japan’s Dr. Kosaka finally connected Lewy bodies with dementia in the mid 1980’s. But it was considered no more than a “rare disorder” until a group of specialists got together and agreed upon a set of diagnostic criteria in 1996.

In 2003, LBD awareness got another big boost. Jim Whitworth and four other caregivers founded the Lewy Body Dementia Assn. They felt Bill’s rage and wanted what he wanted—more awareness about LBD in the medical community and more support for LBD caregivers. The LBDA is almost nine years old now and it has become a strong national organization. Its efforts to advocate for awareness and caregiver support have had results, albeit, not always for the individual caregiver to see. 

In 2005, dementia and movement specialists agreed that dementia with Lewy bodies (DLB), which starts with dementia, and Parkinson’s disease with dementia (PDD) were closely related, with similar causes and cognitive symptoms. Lewy body dementia became an umbrella term for both. Until then, half of our LBD loved ones were not recognized as having those same drug issues. Research shows that drug sensitivities actually do get worse for the PD patient when dementia appears. Another step forward.

In 2008, our first book, Riding a Rollercoaster with Lewy Body Dementia came out, the first comprehensive, easy-to-read book about LBD. In it, caregivers often found the help they needed to take their concerns to the medical community. In 2010 A Caregiver's Guide to Lewy Body Dementia replaced this book.  We are honored to have this place in the history of LBD. We continue to teach, and, like Bill, our focus is on the medical community--especially the hands-on caregivers, the ones who care for our loved ones when we no longer can. If you want your caregivers to be more Lewy-savvy, refer their supervisors to us--we'd love to provide them with some training!  

Some of the latest advances have been in the area of earlier diagnosis. As of 2009, Mild Cognitive Impairment (MCI) has been divided into two types, amnesiac and non-amnesiac. The latter includes less—or no—memory loss along with the decline of executive functions (planning, organizing, etc.). However, it still doesn’t include impairment of social functions, i.e., the problems that delusions bring very early in the LBD journey. We still have a ways to go with this!

Most recently, in 2011, LBD became one of the 100+ Compassionate Allowance diseases, cutting application time for SSA claims down to as little as a few weeks—instead of the months, or even years that it was previously. This doesn’t change the medical community’s awareness of LBD’s problems, but it sure does help caregivers who are running out of funds.

Yes, Bill, things are changing. More people, more medical personnel, even primary physicians, are becoming aware of LBD, even if they still don’t really know how to identify it—or treat it. And yes, the progress is awfully slow—much slower than we’d like. We’ll have to keep on doing our own research and being strong advocates for our loved ones for a while yet. 

Nearing the End of the LBD Journey

We received a question recently about how end-stage Alzheimer's (AD) and LBD differ, and what to be concerned about. By the time a person reaches end-stage dementia there’s so much damage that it’s all very similar and you may not see much difference. These are some of the differences you might see along with some suggestions about care:
Cognition degeneration:Both AD and LBD are degenerative dementias. That is, cognitive abilities will gradually decrease over time.

o   LBD’s characteristic fluctuations between awareness and confusion continue even into this late stage, albeit, few and far between. It is not unusual for patients to know their family members just before death. Look for these and take advantage of them for a final goodbye. However, remember that by now, your loved one will not be able to communicate well.

·         Communication: Eventually any dementia patient will lose the ability to communicate through the normal channels of talking and facial expressions. However, there’s research saying that comprehension is the last ability to go, and so continue talking to your loved one and assume understanding. Remember that touch continues to be important, as does a loving tone of voice. In addition, don’t talk about them or argue with others in their presence anymore than you would if they were responding. 

o   LBD weakens facial muscles, thus talking and facial expressions become difficult often well before the end stages. Add LBD’s larger share of confused thinking.  The result is that much earlier than with AD, LBD folks may stop trying to communicate through normal channels. Behavior becomes the main form of communication.

·         Acting out behaviors: Consider acting-out behaviors the body’s call for help. They usually become more intense as normal channels of communication fail. Look for physical and/or environmental reasons for the behavior: pain, too much stimulation (light, sound, etc.) See previous blogs, and our book, The Caregiver’s Guide to Lewy Body Dementia.

o   LBD affects thinking early on, thus acting-out due to delusions can be a very early, sometimes first symptom. LBD related symptoms such as hallucinations and acting-out behaviors will increase as communication becomes more difficult. However, they may change in form. With degenerating health, agitation and restlessness may be the most common “acting-out” behaviors.

·         Sleeping. People with any kind of dementia tend to sleep more and more as the end nears—20 hours a day is more the norm than not. If your loved one is restless or agitated at this stage, consider this “acting-out behavior” rather than a sleep problem.

·         General health: Eventually any degenerative dementia will cause a body to become incapacitated and die.

o   LBD is more than a cognitive disorder. As already mentioned, its effect on muscles makes communication difficult. It can also weaken other muscles, and it can affect physical health right from the beginning. Therefore, complications like pneumonia, urinary tract infections or falls can be life threatening.  Good patient care and early detection of problems becomes very important. Consider increased acting-out a signal that something is not right. Check for UTI’s, constipation, or other signs of discomfort. If you can find and remove the irritant, the restlessness should decrease.

·         Life expectancy: If there are no complications, a person with dementia can live for many years before enough brain cells have died to shut everything down—often 20 years or more.

o   LBD patients usually do have complications. Therefore, life expectancy is short: 2 to 7 years after diagnosis. However, LBD is seldom diagnosed until well into the disease process. Life expectancy from the first LBD symptom remembered by spouse or family would likely be similar to AD’s 15-20 years—perhaps longer with good care.

Can You Tell Your Story --Briefly?

Here's a challenge for you!  Can you tell GE Healthcare how dementia (or any other neurological disorder) has touched your family in only 140 characters? They want stories “to inform their campaign from the grassroots.” Go to http://www.mindonlinecampaign.com/ and give it a try.  Let your story be heard. We did.

Jim wrote:  My wife had Lewy body dementia, was diagnosed with AD, and got acting-out drugs that made her life worse. Now I teach LBD awareness.

 (133 characters. Spaces and punctuation marks count!)

Helen wrote:  My sister had Parkinson’s but no dementia, had surgery, and began having scary delusions—a forerunner of Lewy body dementia.

(124 characters)

What’s your story?  Go submit it and then, come back and share it here.

Oh, and while you are at it, consider becoming a follower of our blog. We plan to discuss more about acting out in future blogs and you won't want to miss them. Thanks to all who read, follow or comment on our blog.

Tools for teaching and learning about Lewy Body Dementia

The Many Faces of Lewy Body Dementia

Some say that if you put Alzheimer’s disease (AD), Parkinson’s disease (PD) and schizophrenia in a bag and shake them up, you’ll get Lewy body dementia (LBD). But that’s not all; add sleeping disorders and autonomic nervous system dysfunctions and you’ll be closer to describing this multifaceted disorder. However, today we’ll only talk about the first three and save the others for later.

LBD is similar to Alzheimers, in that it is a loss of cognitive abilities. However, AD folks tend to lose memory skills first. They forget words and things. LBD folks lose executive skills first. They become unable to think, do sequential tasks, plan, or make decisions, judgments or choices. Even more important, LBD folks may have severe drug sensitivities to drugs that Alzheimers folks can usually take with comparative safety.

Concern: These two dementias often occur together. Since AD is most common, someone with both disorders will most likely be diagnosed first with Alzheimers. Thus LBD’s severe drug sensitivities may not be discovered until the damage is done.

LBD is related to Parkinsons. There are two types of LBD. Like PD, both are caused by Lewy bodies in the brain. When dementia starts first, this is called Dementia with Lewy bodies (DLB). They may never have major mobility problems and are the ones most likely to be diagnosed with Alzheimer’s disease. People who develop PD first and then go on to develop dementia symptoms are said to have Parkinson’s disease with dementia (PDD).

Concern: Drugs that improve mobility decrease cognition. When a person has PD, they usually see a movement specialist who may view the dementia as an unavoidable symptom of advanced Parkinsons rather than a possible side effect of the PD drugs. When given a choice, caregivers will almost always chose cognition over mobility for their loved ones, even when it means more work for them. They may not get this choice with a movement specialist.

LBD is sometimes compared to schizophrenia because perceptual dysfunctions like hallucinations and delusions are so common and because they tend to very start early in the disease process. In fact they may be the first dementia symptoms, although they are seldom recognized as such until other more cognitive symptoms such as the loss of executive skills appear. One big difference is that people with schizophrenia often hear voices telling them what to do, while LBD hallucinations almost never include voices at all.

Concern: When acting-out behaviors start before more recognizable dementia symptoms, people may see a psychiatrist first. The behavior management drugs most used by psychiatrists are those most dangerous to LBD folks. Even one dose may cause serious, permanent motor or cognitive problems.

The bottom line is that when any of the above symptoms are present, everyone involved should be aware that LBD could also be present, no matter what a person’s diagnosis. If you are wrong, no harm is done. If you are right, you may avoid the damage that can come with LBD’s drug sensitivities. You may also be able to identify “inappropriate behavior” that is alienating co-workers, family and friends as possible early LBD related acting-out behavior. Naturally, you should share your observations and concerns with your loved one’s physician and if appropriate, ask for a referral to a dementia specialist.

Adding up the Statistics: 60% of All Dementia Patients Have Lewy Body Dementia!

We still hear Lewy body dementia refered to as a "rare dementia."  However, in his recent article, Confronting 100 Years of Confusion about Lewy Body Dementia, Dr. James Leverenz, University of Washington, Seattle, states, “… up to half of Alzheimer’s disease patients have the “Lewy body variant” [Lewy bodies in the brain upon autopsy] … and most Parkinson’s disease patients develop dementia….” Here are some statistics:

• 60-80% (let’s say 70%) of all dementia patients have Alzheimer’s. (Alz. Assn.)
• 20% of all dementia patients are diagnosed with a Lewy Body Dementia. (LBDA.org)
• Many  Parkinson’s patients develop dementia but never see a dementia specialist and are not included in the LBD statistics.

For the percentage of all dementia patients with LBD, we can add: 

• the approximately 35% of dementia patients with both AD and LBD, per Dr. Leverenz,
• the 20% of those diagnosed with LBD,
• and a very conservative 5% for the unidentified number of Parkinson’s patients with undiagnosed LBD.

That's a conservative 60%. We can safely say that LBD is no longer rare.