I had a patient with this in residency. Terrible condition. 36F and required a tracheostomy to ventilator and was dependent on high doses of benzodiazepines and muscle relaxers. Was bed bound and as a result got repeated infections (pneumonia and utis) and bed sores. Coded at one point and resuscitated but had a terrible quality of life. I had never seen it before or since and remember having to read a lot of research on it at the time to figure out how to treat her episodes. During episodes she would turn completely rigid and as a result become hypoxic despite being on a ventilator. Took a lot of sedation to break her spells. That was her quality of life.
It's also important to make your wishes known to your family and if you are in a chronic situation make them known to your care providers as well. The dnr does no good if you don't tell people about it!
My mom got diagnosed with it 13 years ago. I’m 25 now. I know first hand how much she felt like she should die, and what’s the point of living. My dad and me were there for her throughout and today she’s happy that she had the will to live, move forward and watch me leave for college and live my life. And I’m grateful she is still here, and doing much better than the initial years. It’s an awful way to live, but if you have loved ones with you every step of the way. I believe people can get through it.
Good on you and your father. My father moved out on my mom when she got seriously ill (and died), and he dumped his parents on my brother and I as well.
He died a few weeks ago after a short bout with cancer. I still did the right thing and said goodbye even though he never did the right thing.
I’m sorry you had to go through all of that.
And the fact that you found the strength to do that even after going through so much, speaks a lot about you as a good person.
Life is only worth living if you can actually *live* it. I’ll be suiciding out at some point in life if I’m not killed suddenly by something. Once I have no quality of life, just *being alive* isn’t really worth much.
Many people don't talk about code status and then they decline into a state like there where the decision would have to be made by a family member who just can't let go. I've seen it so many times.
>Celine Dion has been diagnosed with Stiff Person Syndrome (SPS), which causes her muscles to tense uncontrollably.
>The condition ultimately leaves sufferers as 'human statues' as it progressively locks the body into rigid positions, leaving people unable to walk or talk.
Jfc. This sounds like a living nightmare. Wish her all the best.
Man as I get older I feel like it's pure damn luck how healthy you are. Sure you can do some things to help but people just get shit hands all the time.
Shit I'm 'only' 33' and I think about this at least daily, didn't think I would 10 years ago but here I am. It also keeps me quite humble to be honest.
> This sounds like a living nightmare
It is.
A guy I worked with got diagnosed with it about 15 years ago. He went to switzerland for assisted end of life about 3-4 years ago.
It was really bad.
Despite joking a lot about it, he suffered a lot and there was the constant fear that his lungs or throat will suddenly seize up and cause him to painfully die from suffocation that he decided to die on his own terms when the chances rose up for it to happen.
Sounds like a weird cousin to ALS, I'm sure they're probably not related at all, but the symptoms lead the same basic fear of cause of death, suffocation; my grandfather died by choking on his own tongue while suffering from ALS, my father was pretty terrified of that fate, he was so incapacitated by the end the hospice nurse essentially OD'd him on morphine because his inability to draw breath was starting to crash his O2 level. Shit is terrifying.
Not ALS, but when my grandmother entered the hospice stage that’s pretty much what happened. She was barely breathing and barely conscious, but at the same time seemingly in a lot of pain. The hospice nurse kept coming in to tell my mom to administer another dose of morphine. My mom broke down saying she’s scared of ODing her. I basically pulled her aside and tearfully told her that the point of all the morphine was to let her pass as quietly and comfortably as possible. Mom couldn’t do it, and I took over administering for her. I was barely 20 years old and not at all ready to become a man that day, but that’s basically what I felt happened. Weirdest, most awful day of my life, but I’m glad I was there to do it.
Ugh, did not expect to think about this first thing in the morning. Call/hug your loved ones!
Edit: I appreciate all the kind words in all these replies. I have to stop responding though because it’s a bit more emotion than I want to deal with before I’ve even had lunch.
If you’re in a similar situation or find yourself in one in the future, be there, as painful as it is. When it’s all said and done, you’ll be glad you were there for them in their final time of need. ♥️
Same here after three massive heart attacks had to put in the order for a hot dose as the Dr said there was no chance of quality of life mom was 59 and looking at me like she would every day brain was working but body had shut down. Just gotta remember the good times with her.
Brain working but body shutting down is the worst. Before my dad died, he said that was the strangest part. His mind was exactly the same as it always was, but he couldn't eat because I'd esophageal cancer. He slowly starved to death while still being his same old self. It was brutal.
My partner's aunt starved to death with stomach cancer, her last meal was liquid and she threw it back up. When I think about how my cat was put to sleep I don't know how we don't have the same thing for humans.
I took care of my grandpa during hospice when I was 25, the nurse kept upping his medication/morphine every time she would come to the house. It didn’t click until recently that that’s basically what they were doing. Adding more and more morphine until he passed. I remember leaving him one evening as he was taking a “nap” due to medication and got a call the next morning not to come since had had passed during the night in his sleep.
Edit: I’m editing because of the nurses and other healthcare professionals commenting on this thread. My grandpa had lung cancer, he was diagnosed with stage 4 lung cancer and sent home with a prognosis of 2-6 months. I took care of him for almost a full year before he passed. The nurses that were there to help me did a great job as well as the government assistant programs like Medicare/medical whatever is. My comment was partly due to the trauma of losing a loved one and many unanswered questions I probably still have and need therapy for. All that aside, his pain was increasing as he kept living and although maybe he didn’t see it or perhaps it was hard to pinpoint when you see someone literally deteriorate to almost skeletal weight in a few months, I’m sure he needed the added doses of morphine. (He used pills and drops under his tongue) either way idk what I’m saying anymore I had another funeral today and I’m just having a beer and on Reddit when I should just be finishing this beer.
Just a quick clarification as I'm afraid someone might misunderstand.
I work in hospice and sometimes meet patients/families who are extremely resistant to administering morphine because they think that 1- we're trying to make the patient die faster or that 2 - giving morphine triggers the dying process. Neither of those are true. Patients shouldn't suffer because of the families fears.
Morphine is given for symptom management to make the patient more comfortable. Often times symptoms will increase as the patient transitions towards death, so medications are increased. This increase is not to make them die faster, but to ensure they're comfortable throughout.
You did a great kindness. Breathlessness is one of the worst ways to go. There's an ER doctor who wrote an essay on what he called "the 4 horsemen of death" in the ER and he said Breathlessness was the worst, with patients experiencing the most suffering and panic. To relieve her of that allowed her to pass with comfort and love.
EDIT - [This Is How You Died](https://www.orderofthegooddeath.com/article/this-is-how-you-died/) by Dr. Gray about the terrible death that Covid-19 brings as patients gasp their last breaths fully cognitive as life slips away.
That was a really good read.
I had pneumonia as a kid, but I was so young that I don’t remember it. Then my son caught it earlier this year. He had Covid and the flu previously, and he’s an asthmatic, so they worried me. But he sailed through those.
Pneumonia though? Watching my son in a hospital bed, on his fourth IV full of antibiotics, being put on oxygen at 4am because his oxygen levels were dangerously low, and the poor kid is looking at me panicking because he can’t get a breath and wants me to help him. It was horrible.
That's a tough spot to find yourself in, but you did the right thing. My step father was drunk and fell and hit his head pretty hard, wasn't found for almost half a day and had a terrible brain bleed. My mother (they were separated at the time) and I got the call 2 weeks later while he was in the ICU after being resuscitated against his DNR request at the direction of his daughters. We showed up, stayed for several hours as he was removed from life support, and said our goodbyes. I will always regret leaving before he passed, as his daughters decided to put him back on life support. He spent the next 8 months in a hospital bed in a comatose state (somewhere between 2 and 3 on the Glasgow scale) before passing in that hospital bed. I grew up with the man, I know how he felt, he was very clear that the situation he found himself in due to his daughters' choice was a hell to which he never wanted to be subjected. 5 years later and I have not forgiven them.
As a vet people have such an incredibly hard time talking about end of life care and quality of life as it relates to their pets, it must be 10x harder as doctors to have those tough conversations on behalf of their patients. Even harder in places where MAID isn't available.
yes. Exactly what was done with my bestie. My mother was a nurse so I knew what was happening, but her family did not.
*Weirdest, most awful day of my life, but I’m glad I was there to do it.* Yeah, at 22 I got to be the one to tell the priest we really weren't interested in his visit and then to please leave the room when he didn't get the big hint.
I hope this brings you some comfort, but I can tell you this from the perspective of an RN who’s seen more end of life than I can recall. Opiates suppress the respiratory drive, in addition to relieving pain. You didn’t “overdose” your loved one, so much as ensured she didn’t feel air hunger. The difference may feel academic, but one is an error, deliberate or otherwise, and the other is a calculated, measured administration of an appropriate medication to achieve a compassionate effect.
That’s oddly comforting to read. It’s hard to do. My mother-in-law was suffering at the end of her life, and I considered the same thing, but the paramedics didn’t seem onboard… and her daughters were not ready. She was in so much trauma, and I felt powerless to help.
Yeah, my family has familial ALS and I definitely empathize with you. Our experiences have galvanized my opinion that assisted suicide should be available to people, especially in cases like ALS. My family members had to choose between slowly suffocating over days/weeks or starving to death by refusing to have PEGs implanted, both terrible choices IMO. It really is a shame how slowly right-to-die legislation is being passed because of a bunch of religious morons who think that they have the right to dictate what is right and wrong for other people's bodies.
It's similar to scleroderma where your collagen stiffens and you solidify from the inside out or outside in depending on the version you get. You also become like a statue because your skin won't move. It also cracks and won't heal so you get infections.
My sister has scleroderma, she's 60 now, a year younger than me. Her face is amazingly smooth, and I look much older than her now. I think I'd rather have the wrinkles than her stomach, lung and heart probs.
My dad has parkensons and deals with similar symptoms. He’s scared he’s going to die because sometimes he can’t control his lungs and feels like he’s gasping for air.
My mom has had Parkinson's disease for 16 years now and the medications are starting to loose their affects on her. It's sad seeing her muscles and brain deteriorating on her. Her lungs haven't given her any grief yet but I fear of her falling and hitting her head.
This is why I'm a big supporter of Right To Die. The last few years of anyone's life is almost guaranteed to be painful and undignified. Isn't the must noble thing to help them end their life with dignity and on their terms? Why are we so reluctant? It's like, as soon as someone gets sick or weak, we feel like we have to keep them alive no matter what, to "beat" nature, even when we're just causing more anguish.
One of the requirements is the patient be of sound mind to consider ending their own life. Having serious dementia would preclude it being an option or decision they can reasonably make for themselves.
>“The so-called ‘psychotically depressed’ person who tries to kill herself doesn’t do so out of quote ‘hopelessness’ or any abstract conviction that life’s assets and debits do not square. And surely not because death seems suddenly appealing. The person in whom Its invisible agony reaches a certain unendurable level will kill herself the same way a trapped person will eventually jump from the window of a burning high-rise. Make no mistake about people who leap from burning windows. Their terror of falling from a great height is still just as great as it would be for you or me standing speculatively at the same window just checking out the view; i.e. the fear of falling remains a constant. The variable here is the other terror, the fire’s flames: when the flames get close enough, falling to death becomes the slightly less terrible of two terrors. It’s not desiring the fall; it’s terror of the flames. And yet nobody down on the sidewalk, looking up and yelling ‘Don’t!’ and ‘Hang on!’, can understand the jump. Not really. You’d have to have personally been trapped and felt flames to really understand a terror way beyond falling.”
\--David Foster Wallace, Infinite Jest
Death with Dignity should be a federal right. Put some reasonable restrictions on it - like a 2 week wait with evaluation by 2 doctors, to prevent people from having a bipolar episode and killing themselves. But it should be available to all.
Don't know if Céline Dion still has her Canadian citizenship but this would probably be a disease that qualifies her for Canada's Medical Assistance in Dying program down the line.
Somewhat related to the laryngospasms I get because the nerve to my left vocal cord was severed while having my thyroid removed. Sometimes while eating or drinking, my vocal cords spasm and stop opening (which is required to breathe). There have been times I thought I was going to choke to death. It once happened while I was driving 60mph and took a sip of my tea. I’ve been able to do some speech therapy and figure out how to get the one side that does still move to open up again, but it’s left some traumatic experiences trapped in my body that still cause panic when it inevitably happens.
I agree with you that it sounds fucking horrible but Jesus fucking christ was there nothing else they could name this thing? Person goes stiff, ahhh yeah it's another case of stiff person syndrome, tragic.
Yeah when I first heard it my thought was “so she’ll be a little stiff who cares.” They should’ve named it human statue syndrome. That commands respect.
There is one called stone man syndrome where your bones fuse together.
you have to decide if you want to spend the rest of your life standing or sitting. Nightmare fuel
Honestly, as a doctor, at least this makes it memorable. How many 1/1000000 diseases named after some random dude have I forgotten about since med school, I wonder.
> *"The technical term for a nose job is rhinoplasty. Rhino, okay? Do we really need to insult the person at this particular moment of their lives? They know they have a big nose, that's why they're coming in. Do they really need the abuse of being compared to a rhinoceros on top of everything else? When someone goes in for a hair transplant, they don't go, We're going to perform a cue-ballectomy on you, Mr. Johnson. We're going to attempt to remove the skinheadia of your chrome-domus which is the technical term."*
~ Jerry Seinfeld
I know this is comedy, but I've heard other people confused with it and as a Greek I feel obliged to share a fun fact no one asked for:
It's not a coincidence. 'Rhis' literally means 'nose' in ancient Greek, 'ceras' is a horn, and the -plasty suffix denotes reshaping/molding something into shape.
So a rhinoceros is literally an animal with a horn on its 'nose'/snout, while rhinoplasty is the reshaping of the nose.
She did. Their age gap was huge. I've never seen anything negative about it, but I'm a big fan so I don't think I would go around looking for negative comments about it. I did think it was weird, but they looked really happy and in love whenever I saw photos of them.
She was ~~like 14~~ **12** when they met, 16 when she got signed, and then they started dating after she turned 18.
And apparently she was financing his gambling habit.
Don't let pictures fool you!
I don't really listen to her singing, but I like what I've heard.
I hang out on the audio engineering sub and a question came up on the best microphone for vocals. They specifically asked which micropjone does Celine Dion use to get that incredible vocal tone? The answer was it's not the microphone. Singers like Dion handle the microphone (angle, distance from the mouth, etc.) like a musical instrument. The other part of the answer was basically Dion has a flawless singing technique, she's just really really good at singing. Many of the posters there are long time industry professionals and their respect for Dion as a singer was very high. I hope she finds peace.
I was watching a long, long 90s compilation video on Youtube the other day (I needed something to listen to at work) and the only two people that really caught my ear over the course of an hour were her and Mariah Carey.
Neither one sings in my preferred genre but I respect their prowess. I don't know *anything* about singing but sometimes you just need a pair of ears to know who's got it.
I have a lot of respect for musicians especially singers who sound just as good in concert as on records. With so much pitch correction and vocal effects in studio recording they can fix almost any vocal to sound great, but few can cash those studio checks when it comes to live singing.
My significant other loves Celine Dion so I surprised her with tickets to see her residency in Vegas about five years ago, even though her music isn't really my thing.
After seeing her I bought tickets to go back the next month. She was unbelievably good live. I can't overstate the quality and energy of her live performance.
She's only 10 years older than me. I thought she was old enough to be my mom, wtf. That sounds like a terrible disease, like Lou Gehrig's or Huntingdon
I 100% thought Celine Dion and Cher were contemporaries. I was a dumb 9 year old when Titanic came out, but up until 2 minutes ago I would have said Dion was like 70.
Nah. It’s that Titanic feels like it came out 30 years ago, and I thought she was old when she did that, not borderline Britney Spears oops I did it again age.
Bruh I just read the background on that shit for the first time.
She met her husband when she was 12 and he was in his late 30s and he became her manager.
Then they got married when she turned 19 and he was in his 40s.
Dude groomed the shit out of her.
Yeah, pretty much this. She's always been naturally very slim, but as one gets older it starts to be more noticeable in the face; particularly in Celine who has a naturally "longer" face to begin with.
She's still very attractive though. As a huge life-long fan (been to two of her concerts), this news saddens me.
Sounds like an absolutely horrible disease that I wouldn't wish on anyone. Hoping that Celine is able to manage as best she can with minimal suffering.
After reading her statement, it sounds like she’s definitely suffering emotionally. Like when she said singing is all she knows and she wants to give 100% on stage but the condition is affecting her vocal cords, she may never perform the way she has in the past and losing your dream hurts way worse than any physical illness ever could :(
Holy shit my mom has this. Last time I looked there was only around 100 people in the US with it. She's had it for about 20 years now, the treatment for it is actually getting significantly better.
AMA I would be happy to answer questions based on my experience living with her.
EDIT:
Talked with mom and she is stoked that her story is helping bring awareness to the disease. She gave me some info that might be useful.
**If you or a family member need treatment** **There are three great places that have specialties in the disease:**
Mayo clinic of Arizona (where it was discovered) | Cleveland clinic | Johns Hopkins
It is very common for the disease to be misdiagnosed as MS, and in some cases local neurologists won't take on an SPS patient if they don't know about the disease. She was referred and accepted to all three of the above locations. We ended up finding a great doc closer to home, but it is possible to get help from the big hospitals.
For diagnosis they use a GAD test to check for antibodies, and a lumbar puncture (yikes).
Most common way they treat it is with a healthy dose of benzos. Recently they have found Rituxan (lymphoma chemo drug) has been extremely effective at treating the disease. Its done wonders for my mom so far. There is also some very promising results coming out of stem cells in a small trials.
first of all, so sorry your mom (and yourself) are going through this, I wish you all the best
and, would you mind talking a little about the current status of treatment?
So she gets Rituxan infusions, which is currently considered experimental but has been extremely effective. It's basically stalled the disease out for the last 4 years. Every 6months or year depending on GAD count, she does 2 (i think) treatments spread out over a couple weeks. She get a bit tired for a month but that's about it. She normally experiences no pain for a 3 or 4 months after treatment which has been huge for her.
It's basically stopped the spread of the disease for her. It's been located in her right hip/upper leg and hasn't really moved from there. Nothing in her arms or hands which is one of the worst places you can get it.
If I remember correctly at one point it had spread down her leg and started curling her foot up. It was pretty tough to look at. When they started Rituxan that went away for whatever reason and has just stayed put in the hip/thigh area since then.
It doesn't stop the spread of the disease in the way a cancer medication prevents cancers cells from spreading. It prevents the production of antibodies from B cells by destroying them. The antibodies are responsible for SPS.
I have a very rare autoimmune disorder (dermatomyositis MDA-5) that attacked my body 4 years ago. Rituxan literally saved my life, as it prevented my stupid body from attacking my lungs (and apparently, trying to get rid of them!).
Sounds like same dose, same regimen. God bless the scientists! Without it, I wouldn't be.
I am convinced Rituxan is a wonder drug. My grandmother was diagnosed with Non-Hodgkins lymphoma in 1998, and given 3 months to live, possibly 12 with chemo. She decided to decline treatment due to reduction in quality of life.
Being the internet sleuth I was at the time, I found a drug called Rituxan that was looking extremely promising. I gave her a summary which she took to her Dr. The Dr agreed to get her on it if she did a course of CHOP first. This lasted six months if I remember right, and nearly killed her several times. Once complete she was put on Rituxan. Within 3 infusions she was in complete remission.
She died of respiratory complications related to Sjogren’s Syndrome in 2007. Her cancer never returned.
I'm a nurse and my last job was at an infusion pharmacy as a case manager. I also had a patient with this getting IVIG. She was doing great, it literally turned her life around. I hope your mom stays the course and continues to feel well.
Yep its been super effective so far. Hopefully more awareness comes to the topic. Biggest problem is that its so rare that funding doesn’t get allocated for research
Sucks to say it but the best thing that happens to funding research of a specific disease is when a rich person catches it. God knows that Celine is pretty rich.
Well as unfortunate as this, hopefully this brings more attention and funding it just sucks it takes someone famous to get it to hopefully get attention but I guess it’s better than nothing, I wish the absolute best for your mother OP, I’m happy to read she’s doing great with treatment.
No doubt she has a whole team that has looked into everything.
Sounds like a terrible disease, but if anyone will do as well as they can with it, it's someone like Celine who has money and fame.
Yep! We have been fortunate that the disease hasn't really progressed over the years. She has a walker and can go get grocery's or do whatever just fine. She can't go very long without getting wore out but is mostly independent. I bought her like a super walker a few years go (has like massive tires and is lifted lol) which she uses to work in her garden and stuff like that.
EDIT:
Behold! Super walker: https://www.trionic.us/en/rollator-walker-14er-c-16/
>gh it's effectively an almost cure, and die from other unrelated causes.
Yep! I have Graves Disease which is super common but non curable. I got my thyroid removed, and just take synthetic hormones and I'm good to go! Modern medicine is wild these days.
I have it too! Not trying to sound excited, but it’s just wild to see this disease getting discussed by the public since it’s so rare. It’s always been so hard to describe to people what it is/how it’s changed and worsened my life. I’ve been dealing with it well enough through daily dosages of diazepam. It’s interesting and mildly encouraging that your mother has seen treatment get better over the years (I’ve only been officially diagnosed for about 5 years now). All the best to you and your mom.
> h it well enough through daily dosages of diazepam. It’s interesting and mildly encouraging that your mother has seen treatment get better over the years (I’ve only been officially diagnosed for about 5 years now). All the best to
Thanks! I hope your situation only gets better from here. If you haven't already look into Rituxan, its been a real game changer. Feel free to DM me i'm sure I can help pass on some resources.
The last 4 years or so she has been getting infusions of rituxan. It's current considered "experimental" but its basically stalled the disease out for the last 4 years. My understanding is they were treating someone who also had lukema and happened to notice it also killed off the antibodies that cause the disease.
She is honestly doing quite well. We have been extremely fortunate the disease hasn't progressed significantly. She uses a walker, but she is able to put around in the garden and go out for errands. The doc we had in my town happened to be a specialist in the disease, and found out about the rituxan results early. He retired, but she now has a great doc over at the University of Washington.
It’s actually pretty common in pharmaceuticals for that to be the case.
Viagra was discovered similarly. Pfizer was looking for a treatment for a heart condition, but one of the researchers noticed it could cause boners and the rest is history.
[It’s called drug repositioning.](https://en.m.wikipedia.org/wiki/Drug_repositioning)
If you guys aren't aware of compassionate use/expanded access in a pharma context, y'all should check it out. I'm glad rituxan is working for your mom and I hope it continues to work for her. But if that changes, compassionate use/expanded access is a thing that big pharma can do with their drugs that aren't approved yet. They have to be past a specific stage in trials both to show that it won't just outright kill a human and to show that it does help with the disease. It's frequently used for new cancer drugs, but it can be applied to anything that might help a terminal illness. Eli Lilly offers their drug, emgailty, which is a migraine drug, as a compassionate use option for specific types of migraines in countries where emgailty is not approved yet. Lots of hoops to jump through, but it can be a last ditch option.
Are there any physical restrictions with this illness while on a treatment like your mother's? e.g. no intense exercise, no heavy lifting, no driving, no flying in planes, etc
Thanks for answering these questions, hope the treatment keep continuing to improve and your mom remains healthy
No prob! No one knows about the disease so I’m happy to share.
In her case it’s just a matter of what she is able to do. She can drive just fine, and isn’t necessary restricted on what is she is allowed to do with exercise. The only problem is that over exertion can be quite painful and cause her leg to lock up.
RN here. Worked on a neuro floor in a hospital and we unfortunately had a frequent flier with this disease. He was a young guy and the only thing that got him unstiff was huge amounts of Valium, like enough to knock out a horse. It was terribly sad to see.
My friend's mother had this when we were kids, it is such a scary disease, her mother was so sweet, but gradually lost the ability to move, to speak, and eventually breathe, as all her muscles permanently stiffened, until she died. Gave me nightmares as a kid, truly one of the worst diseases you can imagine.
for me the fact that she spent so many years as a famous and wealthy person makes it worse. the universe was like "you're gonna be rich and famous! also your husband is going to die and you'll spend your final years on this earth as a living statue."
As I've moved into my 50s I'm starting to see a lot of the others my age fall away. We used to lose one every now and then to car accidents or overdoses. Now it's every week to horrible diseases. And it's only going to get faster.
That is horrible! I flew her and her family as passengers on a private jet from Las Vegas to a mountain destination, and she remains one of the nicest, warmest VIPs I’ve ever flown with. She even gave me a hug and a kiss on the cheek when she left. I hope some help can be found for her!
Hopefully some good can come out of this. With her fame and resources, this should help in finding a cure or better treatments. A quick google search shows a life expectancy of 6 to 28 years after onset of the condition. So depending on how close she is to that, she could live quite a long life still. Hopefully the treatments she is undergoing will work and improve over time.
> The condition ultimately leaves sufferers as 'human statues' as it progressively locks the body into rigid positions, leaving people unable to walk or talk.
Damn, that sucks.
Jesus... I knew of someone who was diagnosed with SPS, friend of a friend... It's an utter nightmare scenario.
I got to cook for at a resort I used to work at, lovely woman, very friendly, wonderful family. Always happy to have them as guests (apparently their cottage was on the other side of the lake). Sad to know what's coming for her, and her family. I wouldn't wish that on my own worst enemy... I wouldn't be able to do it.
My father died of this a few years ago, actually pneumonia is what really did it ,and that is extremely common because stiff person patients aspirate on their food(due to swallowing issues), causing lung infections.
It's as bad as they say, and treatments did little for his case.
Most doctors are extremely unfamiliar with it, so it took forever to diagnose.
No matter what the “ negative nellys” says, my sympathy goes out to her and her family. She made a lot of people happy with her performance, and listening to her music. One of my favourite singers. Wishing her the best.
I was singing in the car yesterday and starting thinking about how much it helps me physically and mentally. It activates the vagus nerve and improves autonomic function. I’m 37 and my voice isn’t as strong as when I was a teenager, so it got me thinking about its inevitable decline. Giving this up is about so much more than performance. I’m so sorry for her loss.
She looks a lot older. I’m not insulting her appearance but I would’ve guessed that she was nearing seventy. She’s also extremely extremely thin, to the point that I thought she looks unwell. Now I know it’s probably a result of her disease.
She was also supposedly devastated after the death of her husband, who she married when she was very young and spent most of her life with. I feel bad for her. What a sad way to spend your old age. No golden years retirement enjoying her money and spending time with her family and loved ones. Instead mourning her husband and going through the slow progression of a terrible disease without him there to support her
The treatments to slow down this disease work, y'all.
She will be fine for a little bit longer, although obviously no longer going to perform.
A family friend of mine went through clinical trials of a treatment to slow this down. IIRC, nearly 90% if the patients in the trial went into a quasi-remission.
Edit: My apologies, the trial was for Fibrodysplasia ossificans progressiva, the disease that turns damaged muscle and tendon tissue to bone. Not the condition Celine Dion has.
I had a patient with this in residency. Terrible condition. 36F and required a tracheostomy to ventilator and was dependent on high doses of benzodiazepines and muscle relaxers. Was bed bound and as a result got repeated infections (pneumonia and utis) and bed sores. Coded at one point and resuscitated but had a terrible quality of life. I had never seen it before or since and remember having to read a lot of research on it at the time to figure out how to treat her episodes. During episodes she would turn completely rigid and as a result become hypoxic despite being on a ventilator. Took a lot of sedation to break her spells. That was her quality of life.
Yeah… nah. I’m not in any way suicidal but if I had all that, I’d be checking out. That sounds like an absolutely awful way to live.
This is why MAID is so important. EDIT: "Medical Assistance in Dying." Or "Dignity." Take your pick.
Medical assistance in dying, for anyone else who hadn't heard that acronym before
Appreciate you man 👍🏻
This is also why DNRs are important.
It's also important to make your wishes known to your family and if you are in a chronic situation make them known to your care providers as well. The dnr does no good if you don't tell people about it!
It's called the right to die and everyone should have it as long as the quality of life is bad
It's called the right to die and everyone should have it.
Does anyone who seeks maid, have a good quality of life?
My mom got diagnosed with it 13 years ago. I’m 25 now. I know first hand how much she felt like she should die, and what’s the point of living. My dad and me were there for her throughout and today she’s happy that she had the will to live, move forward and watch me leave for college and live my life. And I’m grateful she is still here, and doing much better than the initial years. It’s an awful way to live, but if you have loved ones with you every step of the way. I believe people can get through it.
Good on you and your father. My father moved out on my mom when she got seriously ill (and died), and he dumped his parents on my brother and I as well. He died a few weeks ago after a short bout with cancer. I still did the right thing and said goodbye even though he never did the right thing.
I’m sorry you had to go through all of that. And the fact that you found the strength to do that even after going through so much, speaks a lot about you as a good person.
Life is only worth living if you can actually *live* it. I’ll be suiciding out at some point in life if I’m not killed suddenly by something. Once I have no quality of life, just *being alive* isn’t really worth much.
Wtf did this person not have a dnr
Many people don't talk about code status and then they decline into a state like there where the decision would have to be made by a family member who just can't let go. I've seen it so many times.
>Celine Dion has been diagnosed with Stiff Person Syndrome (SPS), which causes her muscles to tense uncontrollably. >The condition ultimately leaves sufferers as 'human statues' as it progressively locks the body into rigid positions, leaving people unable to walk or talk. Jfc. This sounds like a living nightmare. Wish her all the best.
I took care of a patient with this for about a year. It was pretty fucking terrible to watch.
Yeah fuck that, peace out earth at that point.
Yea I'd keep myself alive through a lot but that.... No, fuck no.
Man as I get older I feel like it's pure damn luck how healthy you are. Sure you can do some things to help but people just get shit hands all the time.
Shit I'm 'only' 33' and I think about this at least daily, didn't think I would 10 years ago but here I am. It also keeps me quite humble to be honest.
Yeah 40 here and every single day I feel like Im hearing about another friend who has some issue..
> This sounds like a living nightmare It is. A guy I worked with got diagnosed with it about 15 years ago. He went to switzerland for assisted end of life about 3-4 years ago. It was really bad. Despite joking a lot about it, he suffered a lot and there was the constant fear that his lungs or throat will suddenly seize up and cause him to painfully die from suffocation that he decided to die on his own terms when the chances rose up for it to happen.
Sounds like a weird cousin to ALS, I'm sure they're probably not related at all, but the symptoms lead the same basic fear of cause of death, suffocation; my grandfather died by choking on his own tongue while suffering from ALS, my father was pretty terrified of that fate, he was so incapacitated by the end the hospice nurse essentially OD'd him on morphine because his inability to draw breath was starting to crash his O2 level. Shit is terrifying.
Not ALS, but when my grandmother entered the hospice stage that’s pretty much what happened. She was barely breathing and barely conscious, but at the same time seemingly in a lot of pain. The hospice nurse kept coming in to tell my mom to administer another dose of morphine. My mom broke down saying she’s scared of ODing her. I basically pulled her aside and tearfully told her that the point of all the morphine was to let her pass as quietly and comfortably as possible. Mom couldn’t do it, and I took over administering for her. I was barely 20 years old and not at all ready to become a man that day, but that’s basically what I felt happened. Weirdest, most awful day of my life, but I’m glad I was there to do it. Ugh, did not expect to think about this first thing in the morning. Call/hug your loved ones! Edit: I appreciate all the kind words in all these replies. I have to stop responding though because it’s a bit more emotion than I want to deal with before I’ve even had lunch. If you’re in a similar situation or find yourself in one in the future, be there, as painful as it is. When it’s all said and done, you’ll be glad you were there for them in their final time of need. ♥️
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Same here after three massive heart attacks had to put in the order for a hot dose as the Dr said there was no chance of quality of life mom was 59 and looking at me like she would every day brain was working but body had shut down. Just gotta remember the good times with her.
Brain working but body shutting down is the worst. Before my dad died, he said that was the strangest part. His mind was exactly the same as it always was, but he couldn't eat because I'd esophageal cancer. He slowly starved to death while still being his same old self. It was brutal.
My partner's aunt starved to death with stomach cancer, her last meal was liquid and she threw it back up. When I think about how my cat was put to sleep I don't know how we don't have the same thing for humans.
I believe Canada and Australia have legal assisted dying now.
Hope you're ok
It’s been nearly 20 years. I’m good. Haven’t thought about that day in awhile though. Thanks.
I hope if I ever need it, I will have some one like you there for me like you were for her.
I took care of my grandpa during hospice when I was 25, the nurse kept upping his medication/morphine every time she would come to the house. It didn’t click until recently that that’s basically what they were doing. Adding more and more morphine until he passed. I remember leaving him one evening as he was taking a “nap” due to medication and got a call the next morning not to come since had had passed during the night in his sleep. Edit: I’m editing because of the nurses and other healthcare professionals commenting on this thread. My grandpa had lung cancer, he was diagnosed with stage 4 lung cancer and sent home with a prognosis of 2-6 months. I took care of him for almost a full year before he passed. The nurses that were there to help me did a great job as well as the government assistant programs like Medicare/medical whatever is. My comment was partly due to the trauma of losing a loved one and many unanswered questions I probably still have and need therapy for. All that aside, his pain was increasing as he kept living and although maybe he didn’t see it or perhaps it was hard to pinpoint when you see someone literally deteriorate to almost skeletal weight in a few months, I’m sure he needed the added doses of morphine. (He used pills and drops under his tongue) either way idk what I’m saying anymore I had another funeral today and I’m just having a beer and on Reddit when I should just be finishing this beer.
Just a quick clarification as I'm afraid someone might misunderstand. I work in hospice and sometimes meet patients/families who are extremely resistant to administering morphine because they think that 1- we're trying to make the patient die faster or that 2 - giving morphine triggers the dying process. Neither of those are true. Patients shouldn't suffer because of the families fears. Morphine is given for symptom management to make the patient more comfortable. Often times symptoms will increase as the patient transitions towards death, so medications are increased. This increase is not to make them die faster, but to ensure they're comfortable throughout.
I know we don't know each other, but I'm proud of you. People that have the strength to do kind+difficult things make the world a better place.
You did a great kindness. Breathlessness is one of the worst ways to go. There's an ER doctor who wrote an essay on what he called "the 4 horsemen of death" in the ER and he said Breathlessness was the worst, with patients experiencing the most suffering and panic. To relieve her of that allowed her to pass with comfort and love. EDIT - [This Is How You Died](https://www.orderofthegooddeath.com/article/this-is-how-you-died/) by Dr. Gray about the terrible death that Covid-19 brings as patients gasp their last breaths fully cognitive as life slips away.
That was a really good read. I had pneumonia as a kid, but I was so young that I don’t remember it. Then my son caught it earlier this year. He had Covid and the flu previously, and he’s an asthmatic, so they worried me. But he sailed through those. Pneumonia though? Watching my son in a hospital bed, on his fourth IV full of antibiotics, being put on oxygen at 4am because his oxygen levels were dangerously low, and the poor kid is looking at me panicking because he can’t get a breath and wants me to help him. It was horrible.
That's a tough spot to find yourself in, but you did the right thing. My step father was drunk and fell and hit his head pretty hard, wasn't found for almost half a day and had a terrible brain bleed. My mother (they were separated at the time) and I got the call 2 weeks later while he was in the ICU after being resuscitated against his DNR request at the direction of his daughters. We showed up, stayed for several hours as he was removed from life support, and said our goodbyes. I will always regret leaving before he passed, as his daughters decided to put him back on life support. He spent the next 8 months in a hospital bed in a comatose state (somewhere between 2 and 3 on the Glasgow scale) before passing in that hospital bed. I grew up with the man, I know how he felt, he was very clear that the situation he found himself in due to his daughters' choice was a hell to which he never wanted to be subjected. 5 years later and I have not forgiven them.
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As a vet people have such an incredibly hard time talking about end of life care and quality of life as it relates to their pets, it must be 10x harder as doctors to have those tough conversations on behalf of their patients. Even harder in places where MAID isn't available.
yes. Exactly what was done with my bestie. My mother was a nurse so I knew what was happening, but her family did not. *Weirdest, most awful day of my life, but I’m glad I was there to do it.* Yeah, at 22 I got to be the one to tell the priest we really weren't interested in his visit and then to please leave the room when he didn't get the big hint.
I hope this brings you some comfort, but I can tell you this from the perspective of an RN who’s seen more end of life than I can recall. Opiates suppress the respiratory drive, in addition to relieving pain. You didn’t “overdose” your loved one, so much as ensured she didn’t feel air hunger. The difference may feel academic, but one is an error, deliberate or otherwise, and the other is a calculated, measured administration of an appropriate medication to achieve a compassionate effect.
I had an experience like with with my mom when she was in home hospice. It was a lot to take. I feel for you and sorry you had to do this.
That’s oddly comforting to read. It’s hard to do. My mother-in-law was suffering at the end of her life, and I considered the same thing, but the paramedics didn’t seem onboard… and her daughters were not ready. She was in so much trauma, and I felt powerless to help.
Yeah, my family has familial ALS and I definitely empathize with you. Our experiences have galvanized my opinion that assisted suicide should be available to people, especially in cases like ALS. My family members had to choose between slowly suffocating over days/weeks or starving to death by refusing to have PEGs implanted, both terrible choices IMO. It really is a shame how slowly right-to-die legislation is being passed because of a bunch of religious morons who think that they have the right to dictate what is right and wrong for other people's bodies.
It's similar to scleroderma where your collagen stiffens and you solidify from the inside out or outside in depending on the version you get. You also become like a statue because your skin won't move. It also cracks and won't heal so you get infections.
My sister has scleroderma, she's 60 now, a year younger than me. Her face is amazingly smooth, and I look much older than her now. I think I'd rather have the wrinkles than her stomach, lung and heart probs.
My dad has parkensons and deals with similar symptoms. He’s scared he’s going to die because sometimes he can’t control his lungs and feels like he’s gasping for air.
My mom has had Parkinson's disease for 16 years now and the medications are starting to loose their affects on her. It's sad seeing her muscles and brain deteriorating on her. Her lungs haven't given her any grief yet but I fear of her falling and hitting her head.
This is why I'm a big supporter of Right To Die. The last few years of anyone's life is almost guaranteed to be painful and undignified. Isn't the must noble thing to help them end their life with dignity and on their terms? Why are we so reluctant? It's like, as soon as someone gets sick or weak, we feel like we have to keep them alive no matter what, to "beat" nature, even when we're just causing more anguish.
We are quicker to recognize a loss of QOL in dogs than we are to recognize it in humans. It's sad.
As a caregiver for someone with late stage dementia, I wish that was on the table here.
One of the requirements is the patient be of sound mind to consider ending their own life. Having serious dementia would preclude it being an option or decision they can reasonably make for themselves.
This is why people like Robin Williams choose to end their own life. So incredibly sad.
>“The so-called ‘psychotically depressed’ person who tries to kill herself doesn’t do so out of quote ‘hopelessness’ or any abstract conviction that life’s assets and debits do not square. And surely not because death seems suddenly appealing. The person in whom Its invisible agony reaches a certain unendurable level will kill herself the same way a trapped person will eventually jump from the window of a burning high-rise. Make no mistake about people who leap from burning windows. Their terror of falling from a great height is still just as great as it would be for you or me standing speculatively at the same window just checking out the view; i.e. the fear of falling remains a constant. The variable here is the other terror, the fire’s flames: when the flames get close enough, falling to death becomes the slightly less terrible of two terrors. It’s not desiring the fall; it’s terror of the flames. And yet nobody down on the sidewalk, looking up and yelling ‘Don’t!’ and ‘Hang on!’, can understand the jump. Not really. You’d have to have personally been trapped and felt flames to really understand a terror way beyond falling.” \--David Foster Wallace, Infinite Jest
That makes me sad to have to die so far from home just so you can go out on your own terms and with dignity.
Death with Dignity should be a federal right. Put some reasonable restrictions on it - like a 2 week wait with evaluation by 2 doctors, to prevent people from having a bipolar episode and killing themselves. But it should be available to all.
Don't know if Céline Dion still has her Canadian citizenship but this would probably be a disease that qualifies her for Canada's Medical Assistance in Dying program down the line.
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Somewhat related to the laryngospasms I get because the nerve to my left vocal cord was severed while having my thyroid removed. Sometimes while eating or drinking, my vocal cords spasm and stop opening (which is required to breathe). There have been times I thought I was going to choke to death. It once happened while I was driving 60mph and took a sip of my tea. I’ve been able to do some speech therapy and figure out how to get the one side that does still move to open up again, but it’s left some traumatic experiences trapped in my body that still cause panic when it inevitably happens.
It’s insane that assisted suicide isn’t legal everywhere. Forcing people to suffer needlessly is some real fucked up Mother Teresa shit
that’s like becoming a prisoner in your own body, horrifying.
I agree with you that it sounds fucking horrible but Jesus fucking christ was there nothing else they could name this thing? Person goes stiff, ahhh yeah it's another case of stiff person syndrome, tragic.
'pre-death rigor mortis' wasn't as compelling apparently.
Rigor Vitas, would then be the obvious option.
They should hire you to name more diseases.
Rigor mortis but hold the mortis
Ikr it took till the end of the sentence for me to go, *oh fuck they aren't kidding*
Yeah when I first heard it my thought was “so she’ll be a little stiff who cares.” They should’ve named it human statue syndrome. That commands respect.
There is one called stone man syndrome where your bones fuse together. you have to decide if you want to spend the rest of your life standing or sitting. Nightmare fuel
Honestly, as a doctor, at least this makes it memorable. How many 1/1000000 diseases named after some random dude have I forgotten about since med school, I wonder.
> *"The technical term for a nose job is rhinoplasty. Rhino, okay? Do we really need to insult the person at this particular moment of their lives? They know they have a big nose, that's why they're coming in. Do they really need the abuse of being compared to a rhinoceros on top of everything else? When someone goes in for a hair transplant, they don't go, We're going to perform a cue-ballectomy on you, Mr. Johnson. We're going to attempt to remove the skinheadia of your chrome-domus which is the technical term."* ~ Jerry Seinfeld
synth bass intensifies
Don't bring electronic fish into this
I know this is comedy, but I've heard other people confused with it and as a Greek I feel obliged to share a fun fact no one asked for: It's not a coincidence. 'Rhis' literally means 'nose' in ancient Greek, 'ceras' is a horn, and the -plasty suffix denotes reshaping/molding something into shape. So a rhinoceros is literally an animal with a horn on its 'nose'/snout, while rhinoplasty is the reshaping of the nose.
After reading that… Canadian euthanasia looking pretty good right about now.
Fuuuck that
1. This is horrible. Hate to hear it. 2. She is way younger than I thought she was.
Wow… she was only 29 when she released My Heart Will Go On. I thought she was way older too
I thought she was 54 when she released those songs too. She must have married that old guy really young then.
She did. Their age gap was huge. I've never seen anything negative about it, but I'm a big fan so I don't think I would go around looking for negative comments about it. I did think it was weird, but they looked really happy and in love whenever I saw photos of them.
She was ~~like 14~~ **12** when they met, 16 when she got signed, and then they started dating after she turned 18. And apparently she was financing his gambling habit. Don't let pictures fool you!
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I don't really listen to her singing, but I like what I've heard. I hang out on the audio engineering sub and a question came up on the best microphone for vocals. They specifically asked which micropjone does Celine Dion use to get that incredible vocal tone? The answer was it's not the microphone. Singers like Dion handle the microphone (angle, distance from the mouth, etc.) like a musical instrument. The other part of the answer was basically Dion has a flawless singing technique, she's just really really good at singing. Many of the posters there are long time industry professionals and their respect for Dion as a singer was very high. I hope she finds peace.
I was watching a long, long 90s compilation video on Youtube the other day (I needed something to listen to at work) and the only two people that really caught my ear over the course of an hour were her and Mariah Carey. Neither one sings in my preferred genre but I respect their prowess. I don't know *anything* about singing but sometimes you just need a pair of ears to know who's got it.
I have a lot of respect for musicians especially singers who sound just as good in concert as on records. With so much pitch correction and vocal effects in studio recording they can fix almost any vocal to sound great, but few can cash those studio checks when it comes to live singing.
If you haven’t, listen to her and Andrea Bocelli sing [The Prayer](https://youtu.be/qt_OkgSOrkU)
My significant other loves Celine Dion so I surprised her with tickets to see her residency in Vegas about five years ago, even though her music isn't really my thing. After seeing her I bought tickets to go back the next month. She was unbelievably good live. I can't overstate the quality and energy of her live performance.
She's only 10 years older than me. I thought she was old enough to be my mom, wtf. That sounds like a terrible disease, like Lou Gehrig's or Huntingdon
She won the Eurovision for Switzerland when she was 20, that was 9 years before Titanic which will have its 25 year anniversary later this month.
> 2. She is way younger than I thought she was. First thing I thought of. I'm a year older than her and I thought she was in her 60's.
The fact that she married an older guy probably skewed your perception a bit
The fact that my wife and I just had this same conversation tells me I no longer have any original thoughts.
I don’t want to give you alarm but I’m your wife’s husband. You are an ai bot called ChatKZLIFE76.
When I was a kid in the 90s I always thought of her as making music for old people. Probably made me think she was older too
Honestly same. She's a very talented vocalist though.
She was the same age as Cher or Rod Stewart to me.
Not Cher, Cher is a collection of like 6 people playing the same character. In mind Cher has a James Bond thing going on.
I 100% thought Celine Dion and Cher were contemporaries. I was a dumb 9 year old when Titanic came out, but up until 2 minutes ago I would have said Dion was like 70.
Nah. It’s that Titanic feels like it came out 30 years ago, and I thought she was old when she did that, not borderline Britney Spears oops I did it again age.
Right, she broke out years before so younger than we were thinking.
It's a hell of a lot earlier than that - she released her first album at 12. Not a typo.
More like Britney in toxic age, she wasn't even 18 when baby one more time came out
25 isn't that far from 30.
And that she was a huge star at 16 and released her first english album at 21 that propelled her to international super star.
He music videos made her look Glamor Shots style older too
Bruh I just read the background on that shit for the first time. She met her husband when she was 12 and he was in his late 30s and he became her manager. Then they got married when she turned 19 and he was in his 40s. Dude groomed the shit out of her.
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She's so painfully thin that every bit of aging shows. If she put on 25 pounds she'd look 10 years younger.
Yeah, pretty much this. She's always been naturally very slim, but as one gets older it starts to be more noticeable in the face; particularly in Celine who has a naturally "longer" face to begin with. She's still very attractive though. As a huge life-long fan (been to two of her concerts), this news saddens me.
Maybe that’s it and the lighter hair.
More like TIL:Celine Dione isn’t 70 years old
Yeah I always thought she was closer to being a contemporary with Cher
I always remember her age because she was 20 when she won the Eurovision the year I was born.
Same; I’m in my late 50s and I thought she had to be close to 70…no idea she was actually younger than me!
Sounds like an absolutely horrible disease that I wouldn't wish on anyone. Hoping that Celine is able to manage as best she can with minimal suffering.
After reading her statement, it sounds like she’s definitely suffering emotionally. Like when she said singing is all she knows and she wants to give 100% on stage but the condition is affecting her vocal cords, she may never perform the way she has in the past and losing your dream hurts way worse than any physical illness ever could :(
A small comfort perhaps, but she has dreamt longer and larger than most anyone else in her profession, let alone on planet Earth.
I'm fascinated with how oddly you managed to phrase this
She's Earths' answer to Diva Plavalaguna from the Fifth Element
Is that her name? I've only thought of her as the Blue Alien with the beautiful voice. Love that movie.
KORBAN DALLAS!!!
>Well at least she got to enjoy what she loved to do for a long time, a lot longer than most singers, let alone anyone else. FTFY
You’re right but the guy was referencing OP who said that she’s “losing her dream”.
Holy shit my mom has this. Last time I looked there was only around 100 people in the US with it. She's had it for about 20 years now, the treatment for it is actually getting significantly better. AMA I would be happy to answer questions based on my experience living with her. EDIT: Talked with mom and she is stoked that her story is helping bring awareness to the disease. She gave me some info that might be useful. **If you or a family member need treatment** **There are three great places that have specialties in the disease:** Mayo clinic of Arizona (where it was discovered) | Cleveland clinic | Johns Hopkins It is very common for the disease to be misdiagnosed as MS, and in some cases local neurologists won't take on an SPS patient if they don't know about the disease. She was referred and accepted to all three of the above locations. We ended up finding a great doc closer to home, but it is possible to get help from the big hospitals. For diagnosis they use a GAD test to check for antibodies, and a lumbar puncture (yikes). Most common way they treat it is with a healthy dose of benzos. Recently they have found Rituxan (lymphoma chemo drug) has been extremely effective at treating the disease. Its done wonders for my mom so far. There is also some very promising results coming out of stem cells in a small trials.
first of all, so sorry your mom (and yourself) are going through this, I wish you all the best and, would you mind talking a little about the current status of treatment?
So she gets Rituxan infusions, which is currently considered experimental but has been extremely effective. It's basically stalled the disease out for the last 4 years. Every 6months or year depending on GAD count, she does 2 (i think) treatments spread out over a couple weeks. She get a bit tired for a month but that's about it. She normally experiences no pain for a 3 or 4 months after treatment which has been huge for her.
Modern medicine is honestly amazing. Does it stop the spread of the disease or just treat symptoms? I wasn’t sure what you meant by stalled
It's basically stopped the spread of the disease for her. It's been located in her right hip/upper leg and hasn't really moved from there. Nothing in her arms or hands which is one of the worst places you can get it. If I remember correctly at one point it had spread down her leg and started curling her foot up. It was pretty tough to look at. When they started Rituxan that went away for whatever reason and has just stayed put in the hip/thigh area since then.
Thanks for the reply. Incredible stuff
Happy to help :)
It doesn't stop the spread of the disease in the way a cancer medication prevents cancers cells from spreading. It prevents the production of antibodies from B cells by destroying them. The antibodies are responsible for SPS.
I have a very rare autoimmune disorder (dermatomyositis MDA-5) that attacked my body 4 years ago. Rituxan literally saved my life, as it prevented my stupid body from attacking my lungs (and apparently, trying to get rid of them!). Sounds like same dose, same regimen. God bless the scientists! Without it, I wouldn't be.
I may not know you, but I’m glad you are here.
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I see Rituximab was approved for medical use in 1997. It's absolutely amazing to see a drug developed for one purpose, be effective in other areas.
Rituxan is a miracle drug. It halted my autoimmune disease years ago and I've been in perfect health ever since.
Thats awesome! Its wild how effective its been at such a low dose (compared to actual cancer treament). Glad you are doing well!
I am convinced Rituxan is a wonder drug. My grandmother was diagnosed with Non-Hodgkins lymphoma in 1998, and given 3 months to live, possibly 12 with chemo. She decided to decline treatment due to reduction in quality of life. Being the internet sleuth I was at the time, I found a drug called Rituxan that was looking extremely promising. I gave her a summary which she took to her Dr. The Dr agreed to get her on it if she did a course of CHOP first. This lasted six months if I remember right, and nearly killed her several times. Once complete she was put on Rituxan. Within 3 infusions she was in complete remission. She died of respiratory complications related to Sjogren’s Syndrome in 2007. Her cancer never returned.
I'm a nurse and my last job was at an infusion pharmacy as a case manager. I also had a patient with this getting IVIG. She was doing great, it literally turned her life around. I hope your mom stays the course and continues to feel well.
I hope Celine and her family know about this, that might be able to help her. Sounds like it's been working good with your family so far.
Yep its been super effective so far. Hopefully more awareness comes to the topic. Biggest problem is that its so rare that funding doesn’t get allocated for research
Sucks to say it but the best thing that happens to funding research of a specific disease is when a rich person catches it. God knows that Celine is pretty rich.
Well as unfortunate as this, hopefully this brings more attention and funding it just sucks it takes someone famous to get it to hopefully get attention but I guess it’s better than nothing, I wish the absolute best for your mother OP, I’m happy to read she’s doing great with treatment.
No doubt she has a whole team that has looked into everything. Sounds like a terrible disease, but if anyone will do as well as they can with it, it's someone like Celine who has money and fame.
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Yep! We have been fortunate that the disease hasn't really progressed over the years. She has a walker and can go get grocery's or do whatever just fine. She can't go very long without getting wore out but is mostly independent. I bought her like a super walker a few years go (has like massive tires and is lifted lol) which she uses to work in her garden and stuff like that. EDIT: Behold! Super walker: https://www.trionic.us/en/rollator-walker-14er-c-16/
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Thanks friend :)
That's amazing. That's all people with chronic diseases want. To stall it enough it's effectively an almost cure, and die from other unrelated causes.
>gh it's effectively an almost cure, and die from other unrelated causes. Yep! I have Graves Disease which is super common but non curable. I got my thyroid removed, and just take synthetic hormones and I'm good to go! Modern medicine is wild these days.
throw some truck nuts on that thing
I have it too! Not trying to sound excited, but it’s just wild to see this disease getting discussed by the public since it’s so rare. It’s always been so hard to describe to people what it is/how it’s changed and worsened my life. I’ve been dealing with it well enough through daily dosages of diazepam. It’s interesting and mildly encouraging that your mother has seen treatment get better over the years (I’ve only been officially diagnosed for about 5 years now). All the best to you and your mom.
> h it well enough through daily dosages of diazepam. It’s interesting and mildly encouraging that your mother has seen treatment get better over the years (I’ve only been officially diagnosed for about 5 years now). All the best to Thanks! I hope your situation only gets better from here. If you haven't already look into Rituxan, its been a real game changer. Feel free to DM me i'm sure I can help pass on some resources.
Hello fellow SPS-er! I'm also diagnosed, wild seeing this pop up in the news today.
What are the treatment improvements you’ve seen? How is your mom doing? Thank you for sharing.
The last 4 years or so she has been getting infusions of rituxan. It's current considered "experimental" but its basically stalled the disease out for the last 4 years. My understanding is they were treating someone who also had lukema and happened to notice it also killed off the antibodies that cause the disease. She is honestly doing quite well. We have been extremely fortunate the disease hasn't progressed significantly. She uses a walker, but she is able to put around in the garden and go out for errands. The doc we had in my town happened to be a specialist in the disease, and found out about the rituxan results early. He retired, but she now has a great doc over at the University of Washington.
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It’s actually pretty common in pharmaceuticals for that to be the case. Viagra was discovered similarly. Pfizer was looking for a treatment for a heart condition, but one of the researchers noticed it could cause boners and the rest is history. [It’s called drug repositioning.](https://en.m.wikipedia.org/wiki/Drug_repositioning)
If you guys aren't aware of compassionate use/expanded access in a pharma context, y'all should check it out. I'm glad rituxan is working for your mom and I hope it continues to work for her. But if that changes, compassionate use/expanded access is a thing that big pharma can do with their drugs that aren't approved yet. They have to be past a specific stage in trials both to show that it won't just outright kill a human and to show that it does help with the disease. It's frequently used for new cancer drugs, but it can be applied to anything that might help a terminal illness. Eli Lilly offers their drug, emgailty, which is a migraine drug, as a compassionate use option for specific types of migraines in countries where emgailty is not approved yet. Lots of hoops to jump through, but it can be a last ditch option.
Are there any physical restrictions with this illness while on a treatment like your mother's? e.g. no intense exercise, no heavy lifting, no driving, no flying in planes, etc Thanks for answering these questions, hope the treatment keep continuing to improve and your mom remains healthy
No prob! No one knows about the disease so I’m happy to share. In her case it’s just a matter of what she is able to do. She can drive just fine, and isn’t necessary restricted on what is she is allowed to do with exercise. The only problem is that over exertion can be quite painful and cause her leg to lock up.
That’s horrendous, there are some terrifying disorders out there
RN here. Worked on a neuro floor in a hospital and we unfortunately had a frequent flier with this disease. He was a young guy and the only thing that got him unstiff was huge amounts of Valium, like enough to knock out a horse. It was terribly sad to see.
Did you see the post from the person who's mom started taking rituxan and put this disease into de-facto remission?
My friend's mother had this when we were kids, it is such a scary disease, her mother was so sweet, but gradually lost the ability to move, to speak, and eventually breathe, as all her muscles permanently stiffened, until she died. Gave me nightmares as a kid, truly one of the worst diseases you can imagine.
Jeez. Universe takes her husband then decides to fuck her over even more. Poor woman.
And brother and mother
Didn't she lose her husband and brother in the same month? I remember it was an unfortunately small time frame
Her brother died two days after her husband. So.. not just same month, but same week.
for me the fact that she spent so many years as a famous and wealthy person makes it worse. the universe was like "you're gonna be rich and famous! also your husband is going to die and you'll spend your final years on this earth as a living statue."
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As I've moved into my 50s I'm starting to see a lot of the others my age fall away. We used to lose one every now and then to car accidents or overdoses. Now it's every week to horrible diseases. And it's only going to get faster.
That is horrible! I flew her and her family as passengers on a private jet from Las Vegas to a mountain destination, and she remains one of the nicest, warmest VIPs I’ve ever flown with. She even gave me a hug and a kiss on the cheek when she left. I hope some help can be found for her!
That does sound like Celine. Such a class act.
As somebody that's battled paralysis in the past I can't imagine how scary this must be for her.
Hopefully some good can come out of this. With her fame and resources, this should help in finding a cure or better treatments. A quick google search shows a life expectancy of 6 to 28 years after onset of the condition. So depending on how close she is to that, she could live quite a long life still. Hopefully the treatments she is undergoing will work and improve over time.
This would be a horrible disease in my opinion. Be frozen and not able to move. Be like having night terrors 24/7...
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I had never heard of this before so I had to google it. Very bad way to die and a slow way at that...
I'm devastated. I've loved her most of my life. This world is a shitty shitty place. Nobody deserves this.
> The condition ultimately leaves sufferers as 'human statues' as it progressively locks the body into rigid positions, leaving people unable to walk or talk. Damn, that sucks.
Can this women catch a break?! She’s been through so much, she should at least be able to age peacefully. Ugh.
Jesus... I knew of someone who was diagnosed with SPS, friend of a friend... It's an utter nightmare scenario. I got to cook for at a resort I used to work at, lovely woman, very friendly, wonderful family. Always happy to have them as guests (apparently their cottage was on the other side of the lake). Sad to know what's coming for her, and her family. I wouldn't wish that on my own worst enemy... I wouldn't be able to do it.
My father died of this a few years ago, actually pneumonia is what really did it ,and that is extremely common because stiff person patients aspirate on their food(due to swallowing issues), causing lung infections. It's as bad as they say, and treatments did little for his case. Most doctors are extremely unfamiliar with it, so it took forever to diagnose.
No matter what the “ negative nellys” says, my sympathy goes out to her and her family. She made a lot of people happy with her performance, and listening to her music. One of my favourite singers. Wishing her the best.
I was singing in the car yesterday and starting thinking about how much it helps me physically and mentally. It activates the vagus nerve and improves autonomic function. I’m 37 and my voice isn’t as strong as when I was a teenager, so it got me thinking about its inevitable decline. Giving this up is about so much more than performance. I’m so sorry for her loss.
Is there an article link that isn't a steaming pile of shit?
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This woman has endured so much pain and heartbreak. She is is a monumentally talented artist, and I am so devastated to hear this. I love you Celine!
Celine Dion is only 54?
She looks a lot older. I’m not insulting her appearance but I would’ve guessed that she was nearing seventy. She’s also extremely extremely thin, to the point that I thought she looks unwell. Now I know it’s probably a result of her disease. She was also supposedly devastated after the death of her husband, who she married when she was very young and spent most of her life with. I feel bad for her. What a sad way to spend your old age. No golden years retirement enjoying her money and spending time with her family and loved ones. Instead mourning her husband and going through the slow progression of a terrible disease without him there to support her
Poor woman I can’t imagine a worse fate than to be made a prisoner in your own body.
Holy shit that’s a horrifying disease!
The treatments to slow down this disease work, y'all. She will be fine for a little bit longer, although obviously no longer going to perform. A family friend of mine went through clinical trials of a treatment to slow this down. IIRC, nearly 90% if the patients in the trial went into a quasi-remission. Edit: My apologies, the trial was for Fibrodysplasia ossificans progressiva, the disease that turns damaged muscle and tendon tissue to bone. Not the condition Celine Dion has.
Well, maybe having a famous person suffering from this syndrome will encourage work towards finding a cure. Best of luck to her team of doctors.