Céline has Stiff-Person Syndrome

[scielle]

Anyway, according to this (though source looks shady), Celine's team contacted this hospital research center in Montpellier. From what I can gather, they sequence antibodies?

https://www.midilibr...de-11202988.php

 

So seems this is the doctor her team supposedly reached out to: https://www.rgnmed.c...stian-jorgensen

Checks out; it appears his focus is on rheumatology and innovative stem-cell based therapies. But, with anything requiring research, feels like any real solution would be years away.

Edited May 19, 2023 at 12:10 AM by scielle

[Zofia]

 

Anyway, according to this (though source looks shady), Celine's team contacted this hospital research center in Montpellier. From what I can gather, they sequence antibodies?

https://www.midilibr...de-11202988.php

 

 

Thanks for sharing, it's very interesting, although quite vague. CART-T is an already well-known method in oncology, but in the literature there is no trace of its application in the treatment of SPS (at least I can't find it). Doesn't seem like a very advanced search... I admit, I'm a little afraid there's desperation in these maneuvers.

On the other hand, here is a recent article which presents the fairly complete panoply of current and developing therapies: https://nn.neurology.org/content/10/3/e200109

[scielle]

Thanks for sharing, it's very interesting, although quite vague. CART-T is an already well-known method in oncology, but in the literature there is no trace of its application in the treatment of SPS (at least I can't find it). Doesn't seem like a very advanced search... I admit, I'm a little afraid there's desperation in these maneuvers.

On the other hand, here is a recent article which presents the fairly complete panoply of current and developing therapies: https://nn.neurology...nt/10/3/e200109

Thanks.

Yeah, agreed, it does smack a bit of desperation....

And it's pretty telling how limited the research in this field is by the fact that the same 5 or so doctors show up in most research papers, and basically all articles on SPS reference just a handful of the same studies.

Edited May 19, 2023 at 12:38 AM by scielle

[Zofia]

Yeah, Celine has a gigantic misfortune to fall - with all her money, which in another case could have saved her - on such a rare and little known disease.

On the other hand, as other patients have been repeating since December, without her misfortune there would be no chance for them. Céline will serve as a booster even for the researchers, we can already see it, even if it will not perhaps be she who will benefit from all the results...

[scielle]

Thanks for sharing, it's very interesting, although quite vague. CART-T is an already well-known method in oncology, but in the literature there is no trace of its application in the treatment of SPS (at least I can't find it). Doesn't seem like a very advanced search... I admit, I'm a little afraid there's desperation in these maneuvers.

On the other hand, here is a recent article which presents the fairly complete panoply of current and developing therapies: https://nn.neurology.org/content/10/3/e200109

 

Upcoming lecture from the same doctor:

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And from a recent interview with him: https://www.neurologyadvisor.com/topics/neuromuscular-disorders/stiff-person-syndrome-rare-progressive-neurologic-disorder-marinos-dalakas-md/

“Is enough being done to develop effective treatments for this patient population?

 

Dr Dalakas: I am not aware of any medications or treatments currently in development for SPS. I know that researchers in Europe and Japan are interested in developing more treatments for SPS. Neurologists who are involved in the SPS Foundation are very active in discussing the possibility of setting up trials for certain drugs that might be helpful for this patient population. It is a difficult process in finding sponsors and companies willing to research and develop drugs for so few patients with such a rare disease.

Edited May 19, 2023 at 01:56 AM by scielle

[scielle]

And the other doctor behind much of the research. Believe he’s a pioneer of HSCT but has recently retired:

[Katie]

https://www.goodmorningamerica.com/wellness/story/mom-fights-teen-daughter-battling-stiff-person-syndrome-99454110

[Ukrfan]

https://www.goodmorningamerica.com/wellness/story/mom-fights-teen-daughter-battling-stiff-person-syndrome-99454110

 

Thank you for the link.

 

Interesting. I think they also put Celine on steroids, and it helped for some time (hence her May video where she said she was better). But I doubt it is the long-term solution.

[comingback]

Thank you for the link.

 

Interesting. I think they also put Celine on steroids, and it helped for some time (hence her May video where she said she was better). But I doubt it is the long-term solution.

 

 

Yes, when I saw her face in the video one year ago, I automatically thought on a steroids therapy. Because her face was fuller (it's called Moon face). But one year later, the steroids don't seem to be enough and she has a stem cell treatment. I really hope she is OK.

 

 

https://www.youtube.com/watch?v=vh0RCsdgptU

Edited May 19, 2023 at 02:52 PM by comingback

[scielle]

Yes, when I saw her face in the video one year ago, I automatically thought on a steroids therapy. Because her face was fuller (it's called Moon face). But one year later, the steroids don't seem to be enough and she has a stem cell treatment. I really hope she is OK.

 

Quite possible, though I suspect they'd have tried IVIG first (or rituximab or plasmapheresis). From what I've read, doctors seem to advise against HCST unless the patient is unresponsive to less invasive and risky therapies. Dr Burt (who authored the seminal research in the area, including papers cited in links above, and who did Selma Blair's transplant for MS) has abruptly wound down his transplant clinic. (I didn't dig into all the drama around that but seems he's no longer taking new patients and his last study was halted prematurely.) Other doctors seem to only recommend going that route as a last resort.

 

I wonder when she actually got her diagnosis. It all seemed quite fresh and overwhelming in the Dec video. They may have been trying all sorts of things between fall '21 - fall'22 before actually having a diagnosis.

Edited May 19, 2023 at 04:52 PM by scielle

[smw]

[Zofia]

From what I've read, doctors seem to advise against HCST unless the patient is unresponsive to less invasive and risky therapies. Dr Burt (who authored the seminal research in the area, including papers cited in links above, and who did Selma Blair's transplant for MS) has abruptly wound down his transplant clinic. (I didn't dig into all the drama around that but seems he's no longer taking new patients and his last study was halted prematurely.) Other doctors seem to only recommend going that route as a last resort.

 

 

It is true that HSCT is controversial. Some doubts are raised by the authors (rather skeptical) of an important study "Stiff person syndrome spectrum disorders; more than meets the eye" https://www.sciencedirect.com/science/article/pii/S0165572822001102?fbclid=IwAR0yz5QMIVzBZcoZM9v7DfS1ZeF47DABL4suNG4dtBOxKYRPtiSP4pHLxnY

Firstly, only the fairly accurately profiled classical phenotype seems to respond well to this method, so it is not universal. Secondly, according to them, the promising results of the first attempts were not entirely relevant: “As the aim of this treatment is to restore immunologic tolerance through intense lymphodepleting conditioning, patients pre-HSCT transplant regimens included immunosuppressant therapies that are used to treat people with SPS. Therefore, it is difficult to attribute the clinical improvement to the auto-HSCT and not to the immune suppressive therapies themselves.” Thirdly - but this concerns all immunomodulatory therapies, therefore also IVIG, SCIG, TPE and Rituximab - the recommended timing is not always established: should this treatment be initiated immediately after the appearance of the first symptoms or wait until aggravation? “Studies are needed to determine if a proactive treatment approach, initiated shortly after initial symptom onset, results in better outcomes as compared to a reactive treatment approach, in which immune therapies are initiated only after a worsening of symptoms and disease burden despite attempted symptomatic interventions.”

Finally, they advise against the method “due to the lack of persistent and definitive clinical benefit and the risk of development of serious adverse immunological events”.

 

All these objections do not prevent many patients from dreaming that they are qualified for the HSCT, so much they need hope (a bit the same for Baclofen pumps, officially discouraged and praised by those who benefit from them and finally owe them pain relief)…

[A new day...]

I am feeling completely down after reading this. I can’t translate the medical terms so I’m just going to paste it here after using Google translate but it does NOT sound good at all.

 

Celine Dion develops an antibody that targets an enzyme that completely disrupts her muscles. There is no treatment today for this. On a platform like ours, you can try to find one. This requires sequencing the antibody and constructing a specific CAR-T, and testing it.

 

CART-T cells are modified T lymphocytes already used in immunotherapy treatments for certain cancers. They are part of the strategies tested at the Montpellier IHU against autoimmune diseases.

 

"Our platform is open to that, we can contact us and we can offer tailor-made, put ten researchers on a particular problem, we are able to do it", specifies Christian Jorgensen. In this case, he "was contacted" by Celine Dion's entourage, but did not have a direct link with the singer. “For the moment it is on stand-by”, he explains again.

 

Source: [link] https://www.midilibre.fr/2023/05/18/lentourage-de-celine-dion-a-contacte-le-chu-de-montpellier-au-sujet-de-sa-maladie-du-syndrome-de-lhomme-raide-11202988.php[/link]

[scielle]

I am feeling completely down after reading this. I can’t translate the medical terms so I’m just going to paste it here after using Google translate but it does NOT sound good at all.

 

Source: [link] https://www.midilibre.fr/2023/05/18/lentourage-de-celine-dion-a-contacte-le-chu-de-montpellier-au-sujet-de-sa-maladie-du-syndrome-de-lhomme-raide-11202988.php[/link]

 

Scroll up a bit, I put up a link to the doctor’s profile a few days ago and his bio seems somewhat fitting (but yeah, I think the few of us who had read that also felt it sounded pretty desperate...) Based on a couple videos of him I watched in these past few days, seems his research is very early stage, so whatever he and his team could concoct, an actual treatment would probably be years away.

[PuraVida]

Seems a proper medical breakthrough would be 3-10 years away. I do hope progress is made, no doubt Celine will be a big part why of why it will have happened.

[CelinesDIVO5]

Wait. Is that actually Celine’s doctor? Or is he just speculating?

[Zofia]

I am feeling completely down after reading this. I can't translate the medical terms so I'm just going to paste it here after using Google translate but it does NOT sound good at all.

 

 

 

Source: [link] https://www.midilibr...de-11202988.php[/link]

 

 

 

I'm not sure if it's comforting or not, but in his first two sentences there's nothing new. In the first, he explains in very few words the pathogenesis of SPS, in the second, he affirms the current status of this disease: there are people who have gone into remission but no one is considered "cured".

The following is rather about its scientific activity whose usefulness for patients with SPS has not yet been confirmed; fortunately it is not the only possible treatment, on the contrary, it is a rather experimental line of research.

 

Personally, what worries me a bit are rumors of contacts between Céline's entourage and doctors who previously had nothing in common with SPS or even with neuromuscular autoimmune diseases in general (dr Jill R. Schofield de Denver and now dr Christian Jorgensen). It seems to me that the standard treatment is proving somewhat disappointing in Celine's case... but maybe I'm wrong, maybe they're just looking for a better solution than a "temporary improvement".

[Ukrfan]

I'm not sure if it's comforting or not, but in his first two sentences there's nothing new. In the first, he explains in very few words the pathogenesis of SPS, in the second, he affirms the current status of this disease: there are people who have gone into remission but no one is considered "cured".

The following is rather about its scientific activity whose usefulness for patients with SPS has not yet been confirmed; fortunately it is not the only possible treatment, on the contrary, it is a rather experimental line of research.

 

Personally, what worries me a bit are rumors of contacts between Céline's entourage and doctors who previously had nothing in common with SPS or even with neuromuscular autoimmune diseases in general (dr Jill R. Schofield de Denver and now dr Christian Jorgensen). It seems to me that the standard treatment is proving somewhat disappointing in Celine's case... but maybe I'm wrong, maybe they're just looking for a better solution than a "temporary improvement".

 

I think Schofield does specialize in autoimmune diseases, but not SPS itself, so yeah, I agree with you. It is a bit strange.

 

As for the treatment part, it is also possible that it helps, but not enough for her to return to stage. I mean, for most patients, the treatment simply makes it easier / less painful to live, only a tiny % was somewhat able to return to their previous life. I'm afraid Celine and her team's expectations are too high. It will never be as before.

[Zofia]

I think Schofield does specialize in autoimmune diseases, but not SPS itself, so yeah, I agree with you. It is a bit strange.

 

 

Exactly, that's why I'm talking about “autoimmune neuromuscular” diseases and not just “autoimmune”.

 

I'm afraid Celine and her team's expectations are too high. It will never be as before.

 

Absolutely. It's quite common that people who function well professionally and economically cling to their life before, find it more difficult to accept the irreversible nature of the situation and take longer to move on to the next stage. They know their self-agency and resent its loss… Patients with SPS say it themselves: this disease is particularly cruel to very active people; those who always spent their time on a sofa adapt better - also because their level of expectations is much lower.

That's why I hope so much that Céline is surrounded as best as possible… and that she will succeed in moving from the stage of "restitution narrative" to that of "quest" without going through that of "chaos" ((this useful terminology comes from an excellent book by a Canadian sociologist, Arthur Frank, "The wounded storyteller: body, illness, and ethics").

[scielle]

Wait. Is that actually Celine's doctor? Or is he just speculating?

 

He's just a stem cell researcher her team supposedly reached out to... though a scrupulous doctor wouldn't disclose that to the tabloid press!

 

I think Schofield does specialize in autoimmune diseases, but not SPS itself, so yeah, I agree with you. It is a bit strange.

 

One of the conditions she specializes in is Ehlers-Danlos syndrome, which is what Sia has. Maybe it's a personal recommendation.

 

But recall we're getting all this from shady sources that have reported all sorts of incorrect things about Celine in the past, so it's all to be taken with a grain of salt.

 

On a separate note, I was trying to find out more about IVIG and landed on a video of a girl who uses it for... cystic fibrosis. Full circle.

Edited May 20, 2023 at 11:41 AM by scielle

[scielle]

So I found this blog: https://thestiffman.com/

It's someone with SPS who has been documenting his recent treatment - chemo and both a stem cell and bone marrow transplant... Apparently it's a very experimental procedure and only something like 10 people have had it (in Denver & Seattle). You're supposed to quarantine for 100 afterwards because the immune system is basically starting at zero. I've only read a few of the entries and I think it's early days but he seems to be doing a bit better?

It's all so brutal though. I wonder if Celine would put herself through something this experimental.

 

I think this might be the trial Darrell Cherry (who writes the above blog) is participating in: https://clinicaltrials.gov/ct2/show/NCT00716066

Phase 2, taking place in Denver & Seattle, HSCT and bone marrow transplant to tackle a number of diseases. Apparently only 3 participants with SPS have been selected: https://nbcmontana.c...-rare-condition

Edited May 20, 2023 at 12:00 PM by scielle

[comingback]

.

 

Edited May 20, 2023 at 12:36 PM by comingback

[comingback]

. Edited May 20, 2023 at 12:38 PM by comingback

[comingback]

I wish she would give some updates here and there: Hey guys, I'm in Denver and I have .... therapies. It's hard, but I'm OK...etc.. Edited May 20, 2023 at 12:46 PM by comingback

[LukeD]

A colleague of mine told me a few days ago that they were diagnosed with multiple sclerosis.

 

Also two days ago I learnt that a very beloved family friend who has been battling cancer for years is very unwell as the cancer spread to the brain.

 

These in the context of Celine dealing with SPS. It is too much. I feel like I want to cry all the time. Life is so unfair, only fair thing about it is that it can be unfair to anyone.

Edited May 20, 2023 at 01:34 PM by LukeD

[nuts2you]

You have to wonder what her Bothers and sister's think of all the stuff that has happening to there Youngest family member..

[Zofia]

I wish she would give some updates here and there: Hey guys, I'm in Denver and I have .... therapies. It's hard, but I'm OK...etc..

 

Yes, we would like to know... but no patient owes us anything. It is we who have obligations towards them. The weight they have to carry is sometimes overwhelming. For us, Céline's drama is one more worry in our lives, for her - it's her whole life that is in crisis. She owes us nothing, we owe her our support.

[scielle]

She may not be talking to Claudette but I wonder if Céline connected with Jimmy, given his experience living with disability.

[scielle]

Personally, what worries me a bit are rumors of contacts between Céline's entourage and doctors who previously had nothing in common with SPS or even with neuromuscular autoimmune diseases in general (dr Jill R. Schofield de Denver and now dr Christian Jorgensen). It seems to me that the standard treatment is proving somewhat disappointing in Celine's case... but maybe I'm wrong, maybe they're just looking for a better solution than a "temporary improvement".

 

Perhaps while in Colorado, rather than seeing Dr. Schofield, she was actually there to be treated by Dr. Nash. The Colorado stay was confirmed by Claudette (if you consider that a confirmation...) but the doctor name was purely from Closer, which of course is known for publishing all sorts of conjectures on a regular basis.

But, now that Dr Burt's HSCT program at Northwestern is no more, it seems Dr. Nash's in Colorado (along with fellow researchers in Seattle, part of the same study) may be the only program currently doing HSCTs for SPS? https://bloodcancerinstitute.com/clinical-trials/autoimmune-diseases.dot

I've come across several blogs/ posts from people currently undergoing this treatment with him for either MS or SPS. Dr. Nash's protocol is more aggressive than Dr. Burt's, as it uses very high dose chemo so it really knocks people out for a long while.

 

If Celine's medical team first started her on pharmacological remedies and possibly steroids in late '21/ early '22, before even having the SPS diagnosis, then maybe she got diagnosed mid-22 and they tried IVIG (which I gather doctors recommend for 3-6 months to see if the patient is responsive), and if that didn't quite work maybe she actually did get a transplant early this year. The subsequent 3-month quarantine required would explain her absence, even in any kind of videos, for LA.

But if that's the case, I just can't imagine how they would expect her to get back on stage by August. And even if she did have it and it was successful, as others mentioned, it may make SPS less painful but not be enough to allow her to return to performing...

 

Purely speculating on all of this, of course!

[wjd]

The speculation is really good to read, as in the absence of actual news, it is providing balanced views on this awful situation, backed up by credible sources.

 

So thank you to the likes of scielle for continuing the discussions, and hoping for news from Celine very soon!