Rapid Progression of CBD?: This is my first... - PSP Association

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Rapid Progression of CBD?

AllieBBird
AllieBBird

This is my first post, so I hope this isn't a tired worn-out subject. My husband, like so many, had a long delay in his CBD diagnosis, including two spinal fusions and a lot of other shenanigans starting back in 2014. He has been moderately healthy until this spring (his last surgery was in February) when his balance became so bad he couldn't walk and his arm so rigid he really can't use it. He was admitted to the hospital two weeks ago, and they diagnosed him right away, but really seemed unconvinced that his recent rapid progression was consistent with the disease. As I read this site I seems like some have seen this sudden, almost weekly worsening of symptoms, but I wanted to ask - is this a pattern many see? If so, has your loved one's decline remained consistent or most often finding a new plateau? I am getting soft-deflection answers from the medical team, but I need to decide what to do about my job and how to best help our two teenage daughters. Any experience, understanding it is just a small sampling, would be helpful.

Gratitude to this community, I am glad to have found it.

33 Replies
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General anesthesia from his last surgery could have hmmm "fast fowarded" his progression a bit. If you search for general anesthesia on the site you'll find many posts about it. Being put under poses a slight risk for anyone, persons with neuro issues can be more at risk. If the surgeon had known there was a possible Dx of CBD he would have explained the increased risks. When my father, also CBD, had surgery for a blocked bowel the surgeon made it clear there was a possibility of 1) remaining at the same baseline 2) having the baseline drop 3) never make it off the operating table. I opt'ed for the surgery anyway since without it Dad would have gone sepsis.

Ron

Welcome.

Many have experienced a sudden decline in their loved one. This can be after surgery or when they have a urine infection or other infection.

They often make some improvement but not back to their previous level. We call this the new normal. A plateau can then continue for a long time - or not !

I'm afraid it is true that there is no time line but the pattern is progressive.

You do need to plan for the inevitable decline though. Others will tell you . My own thoughts - You need to discuss "end of life " wishes , get power of attorney, contact hospice and make sure that you do anything you want to do NOW

Keep your sense of humour - use this site for support and to rant !

This site was my lifeline and I hope you find it so.

love from Jean x

The medical team doesn’t know what will happen. That’s why they are giving you evasive answers. Everyone has a different progress with theses neurological diseases. The anesthesia probably didn’t help. Getting a proper diagnosis is hard. Most people go for years without one. He may improve or he may not. All you can do is wait and see.

Welcome.

My husband has CBD - diagnosed in 2012 - but he had obviously had it for several years before diagnosis. Everyone’s experience is different so it is such a difficult disease to plan for but in my experience these sorts of sudden declines are fairly common sadly. Two years ago my husband went from being able to walk to being totally bed bound almost overnight. However, he also had times when the progress of the disease was incredibly slow. The other thing is that very few medical professionals truly understand the disease, which is not surprising as it is so rare. This means it can be a challenge when you talk to them about treatment and as Jeff says, it is a challenging illness to diagnose.

I have found this site and the PSPA incredibly helpful. I would echo Jean’s advice to get everything sorted and enjoy what you can do. Thinking of you in these tough times and hope you find a way forward. X

Hi AllieBBird and welcome!

Your journey sounds a lot like mine was before my husband was diagnosed. I feel for you! I know how distressing and frustrating and exhausting this is for you. Talk to your 2 girls and tell them a bit of what to expect - that his brain is not working properly, that you need their support. If you can do so, I would think about taking a month's compassionate leave, and rethink again at the end of the month.

Go with your gut instinct, and be his advocate. Insist on talking to someone who has a sound knowledge of CBD. They may still not be able (or wish) to give you a clear picture. An MRI may indicate the amount of degeneration in the brain. Our neurologist explained it fully to me, but not to my husband. He was unable to give me a timeframe. The ward doctor said 12 months, and I disagreed with him!

So keep a diary, watch his progress, stay close to your husband!

Try and keep in front of the steps down that you notice by anticipating what his needs may be. Watch for signs of infection which may develop during the day between ward rounds. If you feel something is wrong, or has been missed, alert them! Don't take no for an answer!

Our hospital reviewed each case weekly. I found requests for help, such as increases in care, soft diet, thickened fluids, came far too slowly and his progression was completely outside their understanding. Let others care for him so you can be his wife and give him love and affection and be by his side!

My husband's journey began around 2012 with diabetes, dry eyes, hearing loss, frequent night trips to toilet, muscle spasms especially in back and down one side, heart problems which were improved with a Pacemaker in 2013, dry flakey skin, ongoing reduced ability to exercise, and Parkinsons diagnosis. His mobility decreased rapidly from January 2016 and he was admitted to hospital when he was suddenly unable to sit up unaided. There were also signs to me he may have had a small stroke or TIA. One happened in hospital and was check by xray, but showed nothing, but was marked by another downturn. Over his last 3 months he lost interest in eating and the ability to do most things unaided, had pneumonia two or 3 times.

His decline was of major concern to me over the whole of that time. He died in June 2016 aged 77. Like your husband, he was only diagnosed with CBD when he went into hospital.

This site was a godsend and kept me sane and focussed when everything was crumbling around me. The doctors were unaware of what was really wrong with my husband- until I insisted on a neurologist being involved!

You will find my posts if you search for CBD. You will find other posts too, some of them showing a slower decline. I have tried to document symptoms my husband had to find if they are specific to CBD. There do seem to be common threads.

My husband did not seem to be aware of how sick he was, and did not feel it necessary to have that end of life discussion. We had a POA in place but it was not activated by the ward doctor as he considered my husband to have a "sound mind", and doctors could get a yes/no answer from him daily each time they spoke - even though I found him unable to function or focus later in that day. We had a joint bank account and joint assets, so nothing was frozen on his death. So I was able to manage, but it was a constant concern while he was ill, that nothing was in place and added to my tension and frustration.

I hope your husband finds that plateau and gives you more time together.

Big hug - which I know will grow as others read your post,

Jen xxx

enjoysalud
enjoysalud in reply to honjen43

"Let others care for him so you can be his wife and give him love..........." Wise words and wise advice.

Welcome AllieBird; Glad you found us :-)

As all have said, there's no one consistent pattern to the degeneration. The first symptom I noticed with hubby's CBD was speech loss - and that's not a common first symptom! He did have the common experience of plateau-step-down-plateau etc. He has now passed away after approx 5 years with the disease; however more and more we are seeing members on the site whose loved ones are living more than 10 years with the disease.

Check in often, even if just to "vent" 😉

XX. Anne G

Thank you all. We do need to sort out our legal affairs. My husband is aware of the information about his disease, but he is strangely detached which has sometimes led me to be a little slow myself in pushing to get things done. But I suppose that is all part of the disease itself. Lovely. Well, it is reassuring that he isn't completely devastated. Thank you all!

honjen43
honjen43 in reply to AllieBBird

I remember my husband showing great delight that he did NOT have Parkinson's! He did not ask what CBD was and did not enquire further. His attitude was similar to your husband's, detached as if it isn't happening to him.

His reaction did not really surprise me as my husband had faced many unpleasant situations over which he had no control in his early childhood. His 'normal' reaction was to shut himself off and ignore it - head in the sand like. I think by the time CBD caught up to him, his intellect was already showing signs of being incomplete. He did not discuss it with me. His only sign of concern was close to the end when he asked me if I would be all right.

Big hug

Jen xxx

Hi AllieBBìrd

I was diagnosed with cbd in 2015. Over the years I have found the decline not being linear. In that I could go months even a full year without any detereation. However recently things have progressed at an alarming rate. In that I have had some major dips over last few months. Went to sleep 1 evening woke up next morning and my right side had come more alien. Shuffle was twice as bad right arm movement really rrestricted.

honjen43
honjen43 in reply to Brian219

Sorry to hear that, Brian.

Hope you have some family to give you a hand.

I have noticed that determination and invention seem to go a long way towards keeping the body active, no matter what is thrown in the way.

Go well, my friend!

Hugs

Jen xxx

Brian219
Brian219 in reply to honjen43

Hi

I wanted to explain more in my original repĺy. But it takes me so long. Yes I have all family helping. I try and stay focussed but find it so upsetting the journey I am putting my family on. It is 1 horrible disease.

Thank you for your kind reply.

Brian

Hidden
Hidden

It's certainly how the disease has progressed with my mum. Months sometimes pass without change & then suddenly we'll see rapid deterioration that's notable day to day. Then she'll plateau again for a while at a new lower norm. Unfortunately everything that involves the brain seems unpredictable, so I wouldn't look for patterns or specifics. Seems to be from observing mum who's had CBD since 2010 & was diagnosed in 2013/4 that it progresses different in everyone ( for example a completely stiff arm was one of my mum's first symptoms)

Hi how old is your husband. My husband was dx at 55 had symptoms for approx 2 years xx

He is 54, I am 47. It all is going by so fast right now...

I'm so sorry it's so awful....I just hope my husband has a fast demise so he does not suffer xx

Where are you in our world xx

We live in the US, in Philadelphia. Lot's of great medical care at least.

AllieBBird,

My heart breaks as I see new people join this forum, but I’m glad you found us. This site has been a godsend for me as a caregiver for information, and mental and emotional support. I had very similar questions with my wife when I joined this site in 2017. She too was misdiagnosed for many years, however with this disease the correct diagnosis would not have changed the outcome.

I naively kept thinking that the symptoms would plateau, but it seemed like each month a new skill/ability would be taken away. When I look back, she was 52/53 when she noticed and was concerned with cognitive abilities declining, but they seemed minor at the time(our general physician dismissed her concerns). She passed away last year at 59. We worked with physical therapists (voice, occupational, and memory). They seemed to help a little at first, but the cognitive and physical declines continued. We would continue to adjust to compensate for the lost of those abilities.

I hope I’m not too blunt about your path ahead. It was not until I began reading and sharing here that I recognized the reality of the situation that we were in.

The most important thing for you and your family is to cherish the time you have with him, despite the difficulties and hardship. I pray for wisdom, comfort and strength for you and your family as you go through this time.

Blessings,

Bobby

AllieBBird
AllieBBird in reply to Beads0122

Honestly Bobby, that is incredibly helpful to hear. I feel like the grim reaper when I catalogue all that has changed (he seems to understand the information, but most of the time he doesn't internalize what it means.) He is a musician and has been moving forward with booking gigs this fall and winter, which just seems to be inviting pain, but I am not ready to push back, we are only two weeks into having the diagnosis and still gathering information. I have been at his therapy sessions, and see issues (well, he can't walk, use his left arm/hand and his breath control for singing or speaking is very weak) that make me think some of his ambitions are unrealistic. I also see his cognitive issues evolving, but he doesn't seem to have insight into this piece at all. This is a cruel disease.

Beads0122
Beads0122 in reply to AllieBBird

AllieBBird,

I think if you did a search on this forum the words “cruel disease” would be found in nearly every thread of comments. I believe that it is important for you and your husband to gather as much information as you can to prepare. I initially thought my wife didnt internalize what the disease meant, but when she started to try to tell me her final wishes, it was clear to me she did. When she passed, it made it easier for me and our grown children. I’m sorry if I am being too negative. I do suggest that you encourage your husband to pursue his passion as long as he possibly can.

Everyone is slightly different, but there are always common aspects. My wife’s loss of the use of her left arm and hand was early, but she could walk nearly to the end. She was a runner and would say that when she ran, she felt “normal”. She ran a marathon 10 year before she passed, but could barely walk around the block 5 months before she passed. Her voice slowly got quieter until she could barely get a word out. This was painful for her because she loved to talk with family and friends.

I hate this cruel disease,

Bobby

honjen43
honjen43 in reply to Beads0122

So sorry for your loss, Bobby! That is so young. My husband was 77.

Hugs

Jen xxx

It’s just hard to know. I’ve had a bit of the opposite experience with my mom (CBD). She has had a very slow decline (15+ years) but she has been at end stages for a long time (fully bedridden - last 2 years), to the point that I’ve had to adjust resource planning. We frankly never thought she would last this long with the condition and the secondary complications from having this condition. It’s been draining for us all.

Northstar1
Northstar1 in reply to Ettavb

Hello sorry to hear about your mum. How old is she xx

Ettavb
Ettavb in reply to Northstar1

Thank you, she is now 81

My husbands progression was slow and steady for about 6 years, but then he fell in October and had to have a partial hip replacement. The general anesthesia really did a number on him and accelerated all of his symptoms. He died two days before Christmas. I feel if not for the anesthesia we would have had at least another two years or so. I didn’t know and obviously the doctors didn’t know that the anesthesia would exacerbate his symptoms the way it did.

honjen43
honjen43 in reply to Cade08

Am sorry for your loss, Cade08. We try so hard to keep them safe!

I have seen a number of posts that suggest anaesthesia has considerable influence on the brain - maybe more on a more susceptible brain? My husband had 2 surgeries with anaesthetic from 2005, as well as exploratory investigations. Always wonder how they affected him on his path through CBD.

Hugs

Jen xxx

aliciamq
aliciamq in reply to honjen43

I've been reading about anesthesia till my eyes cross. Surgery itself or anesthesia can and does accelerate the processes in the brain that lead to decline. Still no concrete answers. Avoiding hypothermia (in the operating arena?) seems to make a difference in older mouse studies ~ and the addition of geriatric specialists to the team reduces POCD by 40%. And Luck. I am still putting the info together and wish I could speak about it with fluency, but cannot. POCD = Post Operative Cognitive Decline . My husband is having a quick urological procedure done in the morning --- Cross your fingers for him everyone🐁Please and Thanks!!

honjen43
honjen43 in reply to aliciamq

I had spinal anaesthetic when I broke my leg many years ago. The frigid temperature in the operating room on 2 separate occasions is something I still remember!

Gotta keep those bugs at bay somehow!

Hugs

Jen xxx

Cade08
Cade08 in reply to honjen43

Thank you. It’s been rough as I feel it is my fault that he fell in the first place. I was in a hurry and not paying close attention. But he didn’t blame me, bless his heart, and he always had such a good attitude about everything. It made taking care of him so much easier. I wouldn’t want him back the way he was, but I just miss him so very much.

honjen43
honjen43 in reply to Cade08

I know and I understand! I often wonder if I could/should have reacted differently.

No matter what, we did our best for our loved ones and could not do more! I miss my man too!

Hugs

Jen xxx

aliciamq
aliciamq in reply to Cade08

We just went through it with my dad - It was horrible what the whole mess of drugs and surgery did to him. Sad. Sad. Sad.

Hi friend..I'm do sorry about your hubby...sounds like he has been through soooo much and you as well!! My beautiful sister Linda is 69 and I'm pretty sure we / she is in the final stages of this insidious disease CBD...she was officially diagnosed in 2015 after many MRI'S a d CT SCANS..at first they thought it was epilepsy,then Parkinsons because CBD is so rare and hard to diagnose. Being her baby sister (i'm 63 and she is 69) I've read and tried to educate myself ( and her husband) about what shes going through and what to expect from this time in her journey... I've read CBD is sometimes called the European Parkinsons.. It mostly effects left handed British women( we live in Orlando and we aren't British... Although we love our British friends) Lindas diagnosis was cortico basal degeneration with alien limb syndrome...she hasnt been able to use her left hand for almost 5 years..my brother in law is her soul care giver IN her home,he will keep her home as long as he can...he feeds her,bathes her,dresses her...shes forgetting how to walk,her words are very jumbly...i could go on and on but just know you are not alone and it's hard,hard and awful but we will do whatever it takes because we love them sooooo much!! I'm pretty sure what my beautiful soul of a sister is experiencing now is terminal agitation...not not sleeping ( neither is he) shes constantly in motion in her chair...up down up down over and over..now when I say up I dont mean she gets up...she tries to get up... crossing and uncrossing her legs and now her tounge has started lolling to the left...its her left side brain that is dying...but she is sweet as pie..no tears ever and very pliable. I'll say you want some pudding and she lights up!!!!!

honjen43
honjen43 in reply to robiric

Hi robiric I have learned somewhere that the right side of the brain controls the left side of the body! There is a crossover in the system somewhere!

I used a website on Alzheimer's to track the damage in my husband's brain. I found a picture that showed where all the different centres of activity are.

I sat beside my father in 1997 when he was believed to have had a stroke and then a brain tumour was discovered. He was losing use of his left side and his speech, and finally his ability to swallow.

I thought that would be the end of my experience with brain disease - until I realised my husband seemed to be having problems with moving and lifting his feet! The rest of his story is in my posts.

I take my hat off to all you lovely people who have found this site. And I continue to try to add to the wealth of information here.

My heart goes out to you all! And the number with these rare diseases here just keeps growing. Information on CBD was hard to find when I joined in 2016.

Big hugs to you all

Jen xxx

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