My Loved One has PSP or CBD: What's That?

by AbbyFitz

PSP and CBD are two diseases you'll likely never hear about unless someone close to you is diagnosed. Learn more about them as I share my mother's story.

After two years of strange behavior, even weirder symptoms, and many misdiagnoses, my mother was finally diagnosed with both CBD and PSP.

I had never known anyone who suffered from these diseases, and I really couldn't find a whole lot of information online. The basic information I was able to find was just that, basic.

I did learn that both progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are rare. PSP affects about 6 out of every 100,000 people, and only about 3,000 people have CBD in the US. Somehow, as rare as lightening striking twice in the same spot, my mother is afflicted with both.

I wanted to write something to share my mother's story about these diseases since I really couldn't find out much at the beginning. I want to give those who care for loved ones a place to hopefully find answers to some of the same questions I had.

These rare disorders are deadly, but nearly unknown by the general public. Unless lightening strikes in your life, you'll probably never hear about either disease. It's time to change that. It's time to find a cure.

*I am not a medical doctor. This article is not meant to make a diagnosis. It is written to tell my mother's story and what I've learned through my own research. Please talk to your physician about any concerns you may have about PSP or CBD

My Loved One is Suffering, but from What?

Question Vanishing

What is Progressive Supranuclear Palsy?

PSP is a rare neurological disease in which the brain slowly deteriorates over time. It's in the same family of diseases such as Alzheimer's, Lou Gehrig's disease, and Parkinson's, but far less known about by the general public and most of the medical community.

Progressive Supranuclear Palsy affects the part of the brain that controls walking, balance, vision, speech, swallowing, and behavior.

It's thought that the disease might be more widespread than it is, because so often it's misdiagnosed as some other disease. Some experts think that for every one person who is correctly diagnosed as having PSP, there are three others that are misdiagnosed as having something else.

PSP has no cure, and will eventually lead to death. More research is needed to understand this disease and how to fight it. 

What is Corticobasal Degeneration?

CBD is a neurodegenerative disorder that causes nerve cell loss and shrinkage of the brain. It affects multiple areas of the brain, which is why it can have such wide-ranging symptoms.

When corticobasal degeneration first begins to show itself, the patient is most likely diagnosed with Parkinson's because of the movement problems. However, as the disease progresses more symptoms present and it becomes clear that it is not PD.

CBD has no cure and leads to death, but is not the cause of death. Eventually patients will become immobile and pneumonia and other infections will be easy to contract. These are usually what causes death.

There are no objective tests that can be given to diagnose CBD. It can only be diagnosed by the symptoms the patient has. An examination of the brain after death is the only way CBD can be positively determined.

Video: Actress Patricia Richardson Shares Information about PSP

The Road to PSP and CBD Diagnosis: My Mother's Story

These diseases are so rare, most doctors have no clue what's really wrong.

My mother's journey into CBD and PSP has been a long and winding one. Looking back through the years, she had shown symptoms that we really didn't know were symptoms at the time.

An occasional fall, usually backwards, and weakness in her left leg, were just chalked up to clumsiness or old age. Then her mental ability took a nosedive and she began having problems swallowing. It wasn't so easy to explain her symptoms away anymore. 

At first she would just go blank when trying to think of the right words to say. Soon, it became an all-out forgetfulness of what she had just done or said.  Finally, I knew it was time to see someone about it. Their diagnosis? My mother had dementia.

Still, there were more symptoms, My mother had complained of chronic dizziness and headaches for months. Her primary care doctor said her ears were clear, no infection, and no inner ear problems. There were times, though, she stumbled around the house like she was drunk. Also, watching her move was like watching a movie in slow motion. Everything from her body movements to her speech was slow. Sometimes she'd just look at you with a blank expression, but have a freaky look in her eyes.What was going on?

Articles about How to Deal with Dementia Symptoms:

Having a parent diagnosed with dementia can be devastating. When people think of dementia, they often think of memory loss. However, some symptoms of dementia are rarely discussed.
A loved one's struggle with dementia can be heartbreaking and stressful. Here are a few things I've learned that's helped me cope with caring for my mother.

Another Doctor is Stumped

Alien LimbConfused, her primary physician referred her to a neurologist. After a boatload of tests, he told me she had Alzheimer's. I was relieved in a way because it was comforting to know that whatever was wrong with her had a name. However, as time passed and I researched further, it became clear to me that she didn't suffer from Alzheimer's.

I talked to her neurologist about what I thought, and he agreed that she didn't have typical Alzheimer's dementia. He decided to try and treat it as if it were Alzheimer's anyway, and prescribed different memory medications that are helpful to dementia patients. Nobody was more hopeful about their effectiveness than my mother, but, sadly, none of them worked. He finally told me that he didn't know what was wrong and there was nothing else he could do. 

And so we lived with whatever it was for months. I was convinced there was something more sinister than Alzheimer's at work here. Then in May of this year, she was hospitalized for pneumonia. It was there, watching her in the hospital bed, that I noticed still more strange symptoms. Her left hand seemed to do things on its own. Even more odd, when she was eating she would stare straight ahead and not look down at her plate.

After she was released, I noticed that she more or less would drag her left leg at times. Someone always had to be near her when she was walking down steps or an incline. She'd almost always fall because she would never look down. In fact, when I took her to appointments and they'd do the usual neuro tests, she could only follow the doctor's finger with her eyes from side to side, not up and down. She just couldn't move her eyes downward or upward.

My Mother Recieves an Accidental Diagnosis

In July of 2014, I took my mother to the Mayo Clinic to follow up on an unrelated health condition she had had the previous year. I wheeled her in for her checkup that day in her wheelchair because by this time she was so unsteady on her feet she'd fall. Her neurologist was shocked because at her appointment a year earlier she had walked in on her own without any assistance. My mother's mental state had also declined, too, and she had trouble answering simple questions.

Up to this point, I had explained to any doctor who would listen the laundry list of symptoms she had. I felt as if he might be able to help her, so I told the Mayo doctor everything that had happened in the past year. He was so concerned, he set her up for testing the very next week.

And so, at last, after all her psychological tests were performed, I was told she had not only PSP, but CBD, too. She also had a more aggressive form, because she'd gone from being independent, driving, and working out at the gym, to her present state in just a year.

The Mayo Clinic doctor also told us there was no cure, and that the diseases would ultimately kill her. 

Do You Know Someone with PSP or CBD? Have you Even Heard of Them?

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No.
ologsinquito on 11/03/2014

No, I've never heard of these conditions.

WriterArtist on 11/02/2014

I am hearing for the first time.

Yes.
Lois on 11/07/2016

Yes - my mother

Books that Explain More About PSP:

Killing Mother: Progressive Supranuclear Palsy

Progressive Supranuclear Palsy is a rare brain disease affecting only a few thousand people in the U.S. each year; yet, the trajectory of the illness compares with other termina...

View on Amazon

He Lives On: A Man's Struggle with Progressive Supranuclear Palsy: A Daughter's Struggle to Heal

This book records the emotional struggle a daughter faces during the journey of illness she encountered with her sick father. During the course of two years, the family faced mi...

View on Amazon

21st Century Progressive Supranuclear Palsy (PSP) Sourcebook: Clinical Data for Patients, Familie...

This comprehensive ebook provides authoritative information and practical advice from the nation's health experts about Progressive Supranuclear Palsy (PSP), also known as Steel...

View on Amazon

Condemned by Progressive Supranuclear Palsy: Virulent Cousin of Parkinson's Disease

The aim of this book is to acquaint the reader with the humanistic aspects of the little known neurological disease known as Progressive Supranuclear Palsy and to draw attention...

View on Amazon

Tears In My Coffee: Living With Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP) is a rare degenerative brain disease. The disease impairs movements and balance. Many people with PSP also experience changes in mood, behav...

View on Amazon

Symptoms of Progressive Supranuclear Palsy (PSP):

  • Loss of balance while walking
  • Feeling of dizziness
  • Inability to move the eyes, especially downward
  • Slurred Speech
  • Swallowing problems (easily chokes on saliva, liquids, and food)
  • Light sensitivity
  • Problems with memory, reasoning, and decision making

Symptoms of Corticobasal Degeneration (CBD):

  • Stiffness, shakiness, jerkiness, slowness, and clumsiness in the arms or legs
  • Speech problems
  • Difficulty articulating one's thoughts
  • Problems controlling facial muscles
  • Walking and balance problems
  • Dementia
  • Alien limb (an arm or leg will move on its own uncontrollably)
  • Sensory loss

Is PSP and CBD Fatal?

They don't kill, but the complications do.

My first question to the neurologist at the Mayo Clinic was will these diseases kill my mother? He explained to me that the diseases themselves are never what kills a patient. It's the complications that arise from them that do.

Usually, CBD and PSP patients will die of two things: pneumonia and injury.

CBD and PSP sufferers contract pneumonia because of the difficulty they have swallowing. Bits of food or liquid get aspirated into the lungs, and pneumonia can set in quickly.

My mother has already had pneumonia once. She was fine laughing and talking to guests at her birthday party one day, the next day she was falling out of her chair because she was so weak. It's important to take your loved one to the doctor or ER as soon as they start acting off. Pneumonia can kill quickly.

The other thing that most patients die from is head injuries they receive from falls. Until my mother started taking medication, she fell on a regular basis. In 2014 alone, I have taken her to the ER no less than five times. Fortunately, the worst injury she sustained was a gash to her chin that took 12 stitches to close.

Is PSP or CBD Hereditary?

Watching my mother suffer, I couldn't help but wonder if I was looking at myself in 30 years.

I was very concerned about the possibility of either of these diseases being hereditary. The doctor assured me that while these diseases are not well understood by the medical community, one thing they are confident about is that it is not genetic.

I thought back through my mother's family history. No one I could think of had had anything like this. I asked her brothers and sisters if their grandparents or aunts and uncles had had any PSP or CBD symptoms. My grandfather had had Parkinson's, but that was the only neurological problem anyone could think of.

While I'm not too worried about PSP or CBD being in my future, I am planning ahead. Being the sole caregiver for my mother is difficult. I only have one child, and I don't want to burden him and his future family. I am making a point to have disability insurance in place that covers aides to care for me.

Are there Medications to Help People with CBD with PSP?

Medications are out there that will only treat the symptoms of PSP.

Up until the time I took my mother to the Mayo Clinic, we'd tried any medicine a doctor wanted to try because we were so desperate for any alleviation. None of them helped at all.

The neurologist told us there were medicines that helped a small percentage of patients with PSP, but it would only help her balance problems, not her mental abilities, and would probably only be effective temporarily. At this point, I couldn't see any harm in trying, especially if there was nothing else that could be done anyway.

The doctor prescribed Sinemet three times a day. After two weeks I was amazed. Before, I couldn't leave her alone for fear of her falling. Her balance is, I'd say, 75% better. I'm glad to report there's been no falls.

Unfortunately, I know the medicine won't work forever and her disease will begin progressing again, but until then, she'll enjoy a better quality of life.

Other Medications that Have Been Helpful to PSP Patients:

  • Mirapex
  • Elavil
  • Botox

How Long Does my Loved One Have?

I've learned this is difficult to pin down.

From talking to my mother's doctor and in doing my own research, I've learned the typical life expectancy of someone with either disease is 5-10 years after onset of symptoms.

But this is confusing for me. Looking back, my mother has been showing symptoms, albeit mildly, for about seven years. Which symptoms are they talking about? There's no real hard and fast answer it seems.

I know eventually PSP and CBD sufferers become bedridden and lose their ability to speak and eat. In my view, it's probably at this point that pneumonia would be fairly easy to develop and would be what's finally fatal.

My philosophy is, you can't really put a time line on anyone's life. Things happen when things happen. A seemingly healthy person might die in an accident tomorrow, while someone with cancer might hang on for years. I think it's all about taking things one day at a time and living life.

Facts about PSP:

  • PSP is only 1% as common as Parkinson's
  • No one knew PSP existed until 1964
  • Patients usually begin showing symptoms in their 60s
  • 6 out of every 100,000 people will develop PSP
  • PSP occurs more often in men than women
  • Actor Dudley Moore had PSP

Facts about CBD:

  • CBD's alien limb symptom was dramatized by Peter Sellers in the movie Dr. Strangelove
  • About 3,000 people suffer from CBD in the U.S.
  • CBD was not discovered until 1968
  • CBD affects women more than men
  • Age of onset is usually around 60 years old
  • Can affect people in their 40s, but rarely

Tips for Caregivers:

These are just things I've learned in dealing with my mother's illness that might be useful to others.
  • CBD and PSP patients benefit from physical, occupational, and speech therapy. It keeps them strong and mentally stimulated. Medicare won't pay for ongoing therapies. Talk to your doctor about it. Sometimes they can work around it.
  • Because my mother can't communicate her feelings well, it's become a guessing game as to what's really going on with her from a health standpoint. I've began looking at her as I would a baby who can't tell me what they feel. I use my own judgment depending on her behavior.
  • Cut food up in bite-sized pieces and use a thickening agent for liquids to cut down on choking.
  • These brain diseases can cause depression. I try to get my mother out of the house at least once a week, and more if she's up to it, to give her a change of scenery. Also, I've found it's good to let her do something around the house, even if she does it wrong, to make her feel useful. So far, she's been able to avoid taking anti-depressants.
  • Don't be afraid to ask for help from friends or neighbors. Sometimes, I've just got to get out and away. I have a small handful of people I can call on when I need to get out, and it's a lifesaver.

Where can I Find More Information?

PSP and CBD awareness is growing, thanks to CurePSP.  Here I've been able to find a lot of information about these brain diseases. There's also a loving online community to provide support to caregivers and patients.

There's a listing of support groups that meet in person. Unfortunately, there are none near me. If you're a caregiver, I think this would be a great source of support.

CurePSP also sends out a monthly newsletter and resource material for caregivers. I've found these to be really informational, since there is not a whole lot of information I've been able to find online.

Also, be sure to ask your physician about any support they may offer. The Mayo Clinic where my mother was diagnosed assigned us a social worker that was very helpful in making sure we had everything we needed.

Updated: 11/02/2014, AbbyFitz
 
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ologsinquito on 11/03/2014

Abby, I've never heard of these conditions and I'm sorry that your mother is suffering from them. I also wish you had more help caring for her. I'll put her on a prayer list.

AbbyFitz on 11/03/2014

Thank you @mira. It's frustrating when it seems there are no answers, especially when the outcome isn't good either way

AbbyFitz on 11/03/2014

Thank you@writerartist. Faith is the only thing to hold on to at times like these. It's the only thing that will get a person through.

Mira on 11/03/2014

These diseases are tough, and misdiagnosis (erring on one side or the other) are a sad truth. I'm glad that Sinemet works somewhat, and I hope this article will help people take better care of their dear ones.

WriterArtist on 11/02/2014

On times like this, it is so difficult to hols oneself, especially if one's loved one has encountered these rare diseases whose symptoms are difficult to analyze. Moreover, when there aren't any cures, I have found that faith in Almighty gives some hope and strength. Hope you and your mother find the strength to endure the tough experiences ahead. Keep yourself calm and composed, all things pass - good or bad.

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