Céline has Stiff-Person Syndrome

[scielle]

Interesting podcast interview with Tara Zier:

She talks a bit about how Celine’s announcement “pushed awareness forward by 50 years in a day”, not just for the general public but also the medical community, how good it’s been that her coming forward gave the SPS patient community the opportunity to talk about it & have people listen.

Edited June 20, 2024 at 09:49 PM by scielle

[PuraVida]

Interesting podcast interview with Tara Zier: https://open.spotify...5Wo1qUsy0BtlP75

She talks a bit about how Celine's announcement "pushed awareness forward by 50 years in a day", not just for the general public but also the medical community, how good it's been that her coming forward gave the SPS patient community the opportunity to talk about it & have people listen.

 

That's great news, not least because it's seemingly way more common than was previously thought. 1 or 2 in 100,000 would mean that there are possibly two dozen other people in the Vegas region who have this along with Celine.

[Céline RO]

That's great news, not least because it's seemingly way more common than was previously thought. 1 or 2 in 100,000 would mean that there are possibly two dozen other people in the Vegas region who have this along with Celine.

 

It’s crazy to think just how many people with SPS each of us probably has in their proximity.

[Ukrfan]

That's great news, not least because it's seemingly way more common than was previously thought. 1 or 2 in 100,000 would mean that there are possibly two dozen other people in the Vegas region who have this along with Celine.

 

When Celine made her SPS announcement, there were a few big threads on Reddit, and so many comments mentioning their relative/patients have SPS. I thought to myself that it seemed too many incidents for a "1 in a million" disease

[PuraVida]

It’s crazy to think just how many people with SPS each of us probably has in their proximity.

 

I know! It makes me wonder if the disease is becoming more common in the population over the years due to environmental factors like pollution of various kinds. Personally, I had never heard of SPS before Celine made her announcement.

[scielle]

Dr Piquet says here that “since the end of the documentary, there has been significant progress”.

[ewh12]

Dr. Piquet explains SPS in this short video from UC Anschutz

 

[PuraVida]

Before the last week or so, I didn't know about the "positive stress" trigger that SPS had. So if Celine is feeling too good, happy, or positively stimulated, that is a bad thing. It's such a balancing act. Similarly about how she mentioned not enough physical activity is bad, but also too much. She has to moderate every way of being.

[stone]

I think she also feels everything so deeply, happy, sad, angry, excited, surprised, etc. I wonder if having such heightened emotions all the time plays into it.

[scielle]

ABC News: Celine Dion raises awareness of Stiff-Person Syndrome

"As singer Celine Dion opens up about her battle with Stiff-Person Syndrome, other influencers share their realities of living with the condition."

 

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Edited June 24, 2024 at 02:55 PM by scielle

[Jeanette]

Before the last week or so, I didn't know about the "positive stress" trigger that SPS had. So if Celine is feeling too good, happy, or positively stimulated, that is a bad thing. It's such a balancing act. Similarly about how she mentioned not enough physical activity is bad, but also too much. She has to moderate every way of being.

 

For me, this new information about being over stimulated even through positive, happy emotions and feelings, is the most worrying factor for me in how she can pull off a return to performing. I mean, isn’t that why she loves what she does! I was previously worried about the fact that noises and lights could be a stimuli, but then thought perhaps these aren’t triggers for her when we started seeing her out and about at other artists shows and she seemed to cope fine with it but now just don’t know what to think as performing and good, positive feelings and emotions obviously go hand in hand, particularly for her.

 

I honestly really want nothing more for her and us of course, that she can make a comeback to the stage but at what cost to her health and what would a comeback even look like? Are we to expect she’s going to have to find a new way of singing, is that what she means when she says “my voice is being rebuilt”. The BBC interview kinda intimated that to me, while the NBC interview gave me more of a feeling she’s coming back with an even better, healthier voice than recent years, with the big notes fully intact and restored. I also feel concern that to come off as if she’s achieved this voice rebirth, she’ll just resort to extreme use of playback which I was happy to see she had kinda culled a bit in recent tours, since Rene’s passing anyway. Not blaming Rene for the heavier use of it in the past (he obviously was, rightly so, protective of her voice) but she definitely opted for it less in recent tours and any songs she struggled with live were dropped rather than lipped to a previous live performance which I’m sure was probably the previous solution, years gone by.

 

Talking of dropping songs, many of these were newer recordings and so when she said about finding new ways to get through shows previously, i.e, lowering keys and in some cases dropping songs, if you think about it, these songs she struggled with should have been more plausible for her to pull off as they were recorded during her vocal health issues, when the clear belts of her earlier days was in all honesty a distant memory apart from the odd recording, and whether due to stylistic changes in the music industry or that she just couldn’t pull off the huge notes anymore because of her situation, it still worries me that she couldn’t manage those songs. Would adapting changes in her voice to accommodate her new limitations and capabilities, if that’s what she means, actually work?

[scielle]

Some initial reaction from an SPS patient: https://www.instagram.com/p/C8oav3gN-Ay/?utm_source=ig_web_copy_link

[scielle]

We've written about Dr. Richard Nash (of Colorado Blood Center Institute) in this thread before, so it's interesting to see him quoted in this article referencing Celine's doc.

Dr. Nash specializes in stem cell transplants, including for SPS, and has done research alongside Dr. Amanda Piquet (in fact they recently did a workshop/ presentation together which I had posted about in this thread).

Makes you wonder if Celine also consulted with him re. a possible HSCT and decided/ was advised against it.

 

https://www.scrippsn...ss-to-condition

 

“Now "I Am: Celine Dion", a documentary on Dion which came out Tuesday, details her journey with the diagnosis.

“It’s really directed attention to this disease type,” said Dr. Richard Nash, a member physician with the Colorado Blood Cancer Institute. He has also conducted research on autoimmune diseases like stiff person syndrome.

“Treatments aren't terribly effective, so oftentimes when patients present with stiff person syndrome, the natural history of the disease is that it continues to progress,” he said.

[…]

Doctors say Celine Dion being open about her diagnosis has also brought more attention to the research being done on the incurable disorder.

“We’ve seen this with other disease types where an individual who is a celebrity or well-known within the community acts as a spokesperson for the disease type or an organization that is kind of interested in developing research,” Dr. Nash said.

Dr. Nash is involved in numerous studies and research looking at treatments for this condition.

“We started exploring or investigating the effects of transplant for patients with autoimmune disorders,” he said.

Dr. Nash was part of a study that found that after patients with stiff person syndrome received a stem cell transplant, they saw clinical improvement in the stiffness index and walking time, for example. The study was published in January.

“This is research, so we are still trying to explore what the benefits are of the treatment, and then there are risks associated with the treatment as well,” Dr. Nash said.”

Edited June 26, 2024 at 07:30 PM by scielle

[superstar85ca]

Isn't it amazing that just before Celine announced she had Stiff Person Syndrome that it was pretty much unheard of? Now everyone it seems is aware of it, even calling it SPS now. The awareness she has given to this disease is so important and amazing. Celine is a gift in so many ways!

[scielle]

The doctor from Montpellier who was in the press re. Celine some time ago is back at it -

 

Google translate:

 

“The IHU of Montpellier Immun4cure launches an appeal to Céline Dion to finance medical research concerning her illness: "If someone today comes with 10 million euros saying make me a biomedicine, I will do it", says the director of the university hospital institute Christian Jorgensen.

The professor was already in contact last year with the star's teams but "there was no follow-up, it was still very complicated to bring her to Montpellier". “The entourage of stars is always a little strange, a little vague, not very concrete,” he testifies. "These are not necessarily people to whom we can propose a scientific development plan, so I don't think that Céline Dion herself had a concrete proposal but perhaps with the press, it can happen as far as 'to her."”

 

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[CelinesDIVO5]

The doctor from Montpellier who was in the press re. Celine some time ago is back at it -

 

Google translate:

 

“The IHU of Montpellier Immun4cure launches an appeal to Céline Dion to finance medical research concerning her illness: "If someone today comes with 10 million euros saying make me a biomedicine, I will do it", says the director of the university hospital institute Christian Jorgensen.

The professor was already in contact last year with the star's teams but "there was no follow-up, it was still very complicated to bring her to Montpellier". “The entourage of stars is always a little strange, a little vague, not very concrete,” he testifies. "These are not necessarily people to whom we can propose a scientific development plan, so I don't think that Céline Dion herself had a concrete proposal but perhaps with the press, it can happen as far as 'to her."”

 

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Well, that’s a bold statement. “Give me 10 million Euros and I’ll come up with the medicine.” And to just demand that it be given by a celebrity, simply because they may have the money is just unhinged lol.

[scielle]

Well, that's a bold statement. "Give me 10 million Euros and I'll come up with the medicine." And to just demand that it be given by a celebrity, simply because they may have the money is just unhinged lol.

Yeah, he rubs me the wrong way. Perhaps there was good reason Celine's team didn't follow-up!

[Ajax]

Yet he seems to know a lot and is saying that they manage to control Lupus etc and it works the same way.

[Jeanette]

Still not watched the doc yet by the way, waiting until the weekend so I can give myself time afterwards to process it all. So perhaps I should reserve some of my observations until then but I was literally just thinking again about the fact she’s been dealing with this for 17 years and I have to think surely if it was a constant issue, she wouldn’t have hesitated in giving it all up to live her life with her kids, without the stresses and constant worry of whether she could pull off live show after live show and recording sessions etc. After all, she always lived by the mantra that she could give it all up tomorrow, that she didn’t need it and at that time, I 100% believed that. I do think her mindset on this changed after Rene’s passing hence why she threw herself into a hectic schedule, as she says it was to show the world she was strong but I believe a big part of it is that she really needed us, the fans. We in turn gave her our unconditional love and she connected with us more than ever before and I feel that’s possibly one of the main reasons why she’s striving to get back on stage now.

 

Anyway, I’m waffling again, I’m like Celine, I go right off context 😂 But just to say that I believe her symptoms if present all those years surely presented themselves sporadically, possibly at stressful times, and while I know that her lifestyle as a singer/entertainer would always carry with it some form of stress, I’m referring more to particular instances i.e. the cancellations of numerous shows in 2014 (just before everything was cancelled indefinitely), we were told this was due to inflammation of the throat muscles, if I’m not mistaken, this was obviously also during Rene’s 2nd cancer battle and possibly a terminal diagnosis was known by then. The start of the Courage Tour, viral issues causing problems with her throat, could have been just unfortunate timing to get sick but also could very well have been due to the realisation that she was embarking on her first BIG world tour without Rene’s guidance and the stress of can I actually pull this off given what we now know.

 

There’s obviously many examples but I do also wonder how much of her issues with her voice over the years actually lie with SPS and how much of it is just purely due to her voice degrading with age, overuse and other general factors, such as stress etc, could she now just be looking back at all previous vocal issues and putting everything down to her now diagnosis and thinking it’s been SPS all along. I guess these are answers we’ll never know, she’ll probably never have a definitive timescale herself as to the onset of this disease. I’m glad it seems she’s doing better now, both physically and mentally as this diagnosis and the whereabouts and whenabouts can really prey on the mind and make her question everything about a huge chunk of her career.

 

[scielle]

An interview with Dr Piquet:

 

https://youtube.com/watch?v=c1C0U393DZw&si=srSA9jgIhkUGp8DH

 

https://www.cbsnews.com/colorado/news/celine-dions-donation-uchealth-university-colorado-aurora-hospital-aims-help-find-cure-stiff-person-syndrome/

 

“In Celine Dion's case, she suffered symptoms for more than a decade before she met Dr. Piquet two years ago and was finally diagnosed with SPS.

"She happened to find us, and it was a great relationship, and we worked really hard with her managing those symptoms, getting her on a good treatment pathway," Piquet said. "There are no FDA-approved therapies for this disease. We often use immune therapies, and symptomatic therapies to manage the disease. We will also do things like physical therapy, massage therapy, and in Celine's case vocal therapy to help manage the symptoms."

[…]

Without a doubt, SPS is a difficult disease to live with let alone understand. Yet Dr. Piquet is confident Celine Dion's generous contribution to her research will make a world of difference.

"With Celine bringing public awareness to this disease, we're going to get there faster," the doctor said of finding a cure.“

 

Edited June 26, 2024 at 11:05 PM by scielle

[Nmj]

Neurologist Dr Susie Bash.

 

[jpatdeleon09]

1719434234[/url]' post='2453924']

Still not watched the doc yet by the way, waiting until the weekend so I can give myself time afterwards to process it all. So perhaps I should reserve some of my observations until then but I was literally just thinking again about the fact she's been dealing with this for 17 years and I have to think surely if it was a constant issue, she wouldn't have hesitated in giving it all up to live her life with her kids, without the stresses and constant worry of whether she could pull off live show after live show and recording sessions etc. After all, she always lived by the mantra that she could give it all up tomorrow, that she didn't need it and at that time, I 100% believed that. I do think her mindset on this changed after Rene's passing hence why she threw herself into a hectic schedule, as she says it was to show the world she was strong but I believe a big part of it is that she really needed us, the fans. We in turn gave her our unconditional love and she connected with us more than ever before and I feel that's possibly one of the main reasons why she's striving to get back on stage now.

 

Anyway, I'm waffling again, I'm like Celine, I go right off context �� But just to say that I believe her symptoms if present all those years surely presented themselves sporadically, possibly at stressful times, and while I know that her lifestyle as a singer/entertainer would always carry with it some form of stress, I'm referring more to particular instances i.e. the cancellations of numerous shows in 2014 (just before everything was cancelled indefinitely), we were told this was due to inflammation of the throat muscles, if I'm not mistaken, this was obviously also during Rene's 2nd cancer battle and possibly a terminal diagnosis was known by then. The start of the Courage Tour, viral issues causing problems with her throat, could have been just unfortunate timing to get sick but also could very well have been due to the realisation that she was embarking on her first BIG world tour without Rene's guidance and the stress of can I actually pull this off given what we now know.

 

There's obviously many examples but I do also wonder how much of her issues with her voice over the years actually lie with SPS and how much of it is just purely due to her voice degrading with age, overuse and other general factors, such as stress etc, could she now just be looking back at all previous vocal issues and putting everything down to her now diagnosis and thinking it's been SPS all along. I guess these are answers we'll never know, she'll probably never have a definitive timescale herself as to the onset of this disease. I'm glad it seems she's doing better now, both physically and mentally as this diagnosis and the whereabouts and whenabouts can really prey on the mind and make her question everything about a huge chunk of her career.

 

Just watch it tonight. No need to prepare for anything besides tissue. It’s better that you’re not prepared because that brings out the emotions, no expectations. Just watch it without distractions.

Edited June 27, 2024 at 05:18 AM by jpatdeleon09

[Ajax]

Well, that's a bold statement. "Give me 10 million Euros and I'll come up with the medicine." And to just demand that it be given by a celebrity, simply because they may have the money is just unhinged lol.

 

Documentaire sur Céline Dion : l'IHU de Montpellier lance un appel à la chanteuse pour financer la recherche contre le "syndrome de la personne raide" (francetvinfo.fr)

[PuraVida]

Neurologist Dr Susie Bash.

 

https://youtube.com/watch?v=TRd3L16XfW8&si=LxDfzxlmjJjK4tvo

 

That news anchor asking "So there's no cause of the condition?" Everything has a cause!

[Ajax]

Documentaire sur Céline Dion : l'IHU de Montpellier lance un appel à la chanteuse pour financer la recherche contre le "syndrome de la personne raide" (francetvinfo.fr)

And more

 

BFM "Pour elle, 10 millions d'euros ce n'est pas grand chose": l'IHU de Montpellier lance un appel à Céline Dion (msn.com)

LE PARISIEN Céline Dion : l’IHU de Montpellier lance un appel à la chanteuse pour financer la recherche sur sa maladie (msn.com)

Edited June 27, 2024 at 09:58 AM by Ajax

[PuraVida]

Still not watched the doc yet by the way, waiting until the weekend so I can give myself time afterwards to process it all. So perhaps I should reserve some of my observations until then but I was literally just thinking again about the fact she's been dealing with this for 17 years and I have to think surely if it was a constant issue, she wouldn't have hesitated in giving it all up to live her life with her kids, without the stresses and constant worry of whether she could pull off live show after live show and recording sessions etc. After all, she always lived by the mantra that she could give it all up tomorrow, that she didn't need it and at that time, I 100% believed that. I do think her mindset on this changed after Rene's passing hence why she threw herself into a hectic schedule, as she says it was to show the world she was strong but I believe a big part of it is that she really needed us, the fans. We in turn gave her our unconditional love and she connected with us more than ever before and I feel that's possibly one of the main reasons why she's striving to get back on stage now.

 

Anyway, I'm waffling again, I'm like Celine, I go right off context But just to say that I believe her symptoms if present all those years surely presented themselves sporadically, possibly at stressful times, and while I know that her lifestyle as a singer/entertainer would always carry with it some form of stress, I'm referring more to particular instances i.e. the cancellations of numerous shows in 2014 (just before everything was cancelled indefinitely), we were told this was due to inflammation of the throat muscles, if I'm not mistaken, this was obviously also during Rene's 2nd cancer battle and possibly a terminal diagnosis was known by then. The start of the Courage Tour, viral issues causing problems with her throat, could have been just unfortunate timing to get sick but also could very well have been due to the realisation that she was embarking on her first BIG world tour without Rene's guidance and the stress of can I actually pull this off given what we now know.

 

There's obviously many examples but I do also wonder how much of her issues with her voice over the years actually lie with SPS and how much of it is just purely due to her voice degrading with age, overuse and other general factors, such as stress etc, could she now just be looking back at all previous vocal issues and putting everything down to her now diagnosis and thinking it's been SPS all along. I guess these are answers we'll never know, she'll probably never have a definitive timescale herself as to the onset of this disease. I'm glad it seems she's doing better now, both physically and mentally as this diagnosis and the whereabouts and whenabouts can really prey on the mind and make her question everything about a huge chunk of her career.

 

So I counted and she cancelled 57 shows during her second residency from 2011-2019 due to her own health reasons (i.e. not counting her hiatus during Rene's cancer battle)...That's about 6 shows a year if you want to look at it that way. Usually it meant she cancelled 2-4 weeks worth of shows whenever she decided to do it.

 

Not horrendous, given that she performed nearly 430 shows for that second residency.

[Nmj]

This lady has had SPS, since birth.

 

[PuraVida]

I wonder how long the IVIG will be effective for Celine. By the way, I found this:

 

Immune globulin costs $75 to $80 per gram. Thus, for a patient who weighed 70 kg, the cost of the drug would be at least $10,500 per month or $31,500 for the full three-month course of treatment.

[Nmj]

I wonder how long the IVIG will be effective for Celine. By the way, I found this:

 

Immune globulin costs $75 to $80 per gram. Thus, for a patient who weighed 70 kg, the cost of the drug would be at least $10,500 per month or $31,500 for the full three-month course of treatment.

 

I often wondered if it can stop working? Is it a type of thing your body can used to? Or is it consistent? I hope it continues to work for her.

[Ukrfan]

I often wondered if it can stop working? Is it a type of thing your body can used to? Or is it consistent? I hope it continues to work for her.

 

In around 30% of patients, it stops helping so effectively in an average of around 2.6 years since the beginning of the treatment.

 

Here:

 

This is a large study in 36 patients with SPS demonstrating that monthly maintenance IVIg therapy offers long-term benefits in 67% of patients for a median 3.3-year period. Because 29.1% experienced diminishing benefit over time due to disease progression, the study highlights the need for more effective therapies.

Edited June 27, 2024 at 01:32 PM by Ukrfan