Superficial Siderosis

Life Expectancy of Superficial Siderosis Patients

Superficial Siderosis Life Expectancy

It’s human nature to wonder How long will I live? When you’ve been diagnosed with a life-changing ultra-rare disorder like superficial siderosis, thoughts like this can consume you. Superficial Siderosis Life Expectancy, four words make it intensely personal. The early impressions I found online were dark; there is no cure. The day we learned about superficial siderosis, I read one single sentence in a very early report, the damage is irreversible and fatal. My heart dropped. The word fatal is a definite attention grabber. However, five years into this journey, we have first-hand insight with a much clearer understanding of the nature of this disorder.

The Toll On Your Body

If you review the possible combination of symptoms that may assault you, it should become clear while you may suffer body and soul, none are life-threatening in an immediate sense. However, there is no doubt the toll on your body is punishing. The possibility of dementia is alarming, but in reality, only a tiny percentage of our community will ever face dementia.

Symptoms will progress so slowly during the early years they make you crazy with frustration, but it does take time. Twenty-two years passed after Gary’s tumor removal before his symptoms progressed to where he had to change his life radically. There does seem to be a point of symptom acceleration (To read about Garys’ current condition see Inescapable Realities). Slow progression eventually evolves in response to a patients’ hemosiderin deposition. The likelihood you will develop multiple serious superficial siderosis related health problems down the road is strong if you have a heavy build-up over large areas.

Repairing The Bleed Source Early

Some will have their bleed source repaired early, so they have a chance to escape heavy hemosiderin build-up. Five years into this journey, the anecdotal evidence is overwhelming; even if an active bleed is repaired, the damage will continue if hemosiderin deposits remain in place. With only one feasible chelation therapy option available, whether due to cost or inability to tolerate the medication, not everyone will have an opportunity to remove the iron.

Turning To The Expert

Let’s reconsider the question, Life Expectancy of Superficial Siderosis Patients? In 2015 this was the only answer Dr. Levy was able to give our community-

...does superficial siderosis lead to an early death? We don’t know the answer to that. We don’t know if superficial siderosis causes earlier mortality. The reason we don’t know that is because we haven’t studied enough patients over time to be able to see that.

– Dr. Michael Levy 2015, SS Webinar

Four years have now passed with time spent observing multiple patients. Dr. Levy offered us an updated opinion during his 2019 Superficial Siderosis Presentation to the SSRA.

I always get this question, so I really just want to address it right now, which is, does Superficial Siderosis lead to early death? The answer is probably not. We don’t see an early mortality directly related to the superficial siderosis, but it is profoundly disabling. Patients do end up completely deaf sometimes, and they can be disabled to the point that they’re in a wheelchair, all day long even. If you consider those circumstances, which increase the chance of blood clots and that really increase the ability to live a full life, then, yes, superficial siderosis is not a friendly, easy, mild disease. This is one of those difficult conditions that’s hard to live with. While it may not cause an early death, it certainly makes life very difficult to live.

Dr. Michael Levy, December 2019

Gary has learned first-hand just how disabling this disease can become. We see it every time we read an update from fellow patients whose journey we’ve followed along with these past years. Depression and hopelessness are real enemies, along with feelings of isolation or loss of independence. Constant reminders of what this disorder brings to the unlucky few diagnosed with superficial siderosis.

First published December 2017 Updated December 2019
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Living With Superficial Siderosis

Living with Superficial Siderosis is the informational site of the Superficial Siderosis Alliance. Contact the editor: admin@livingwithss.com

26 Comments

  1. Can I get some information sent to me on superficial siderosis I am newly diagnosed and cant get any help.

  2. Hi,

    I had a csf leak and regular scans. The superficial siderosis did not appear on my scan until January 2023. The surgical repair was completed October 2023. Therefore, it is a very mild site. I am currently asymptomatic. Would it be necessary to take medication even in my case? Thank you.

    1. Hi J, yes both are common in SS. Gary has dropped from 230 to 152lbs. He requires a combination of stool softeners and prescription laxatives to make his bowels move. Without the medication he would not have a BM for up to none days. In the later stages it is recommended patients be given a daily dose of the liquid used to clean someone out before a colonoscopy. 

  3. Hi, my husband robin was diagnosed with ss Siderosis in 2008. He’s in a wheelchair lost his speech his hearing as well as a permanent caffiter. Suffering with terrible constipation. Has a feeding cup with a straw which he struggling to put in his mouth and I have to remind him how to suck up the straw. Can no longer wash himself I do everything for him. He has recently started sleeping an awful lot. I am worried that his life maybe coming to the end.

    1. I havent yet been diagnosed with this but i have alot of the signs and systems of this and have for years but as of lately some have been getting really bad so i googled what part of my brain controls some of my systems and it came up with this, i am now scared and more curious

    2. Hi kath how is your husband doing? My mother has SS . She has really declined the last 3 weeks. She’s in the hospital with double pneumonia but she no longer has pneumonia but currently is in a wheelchair and cannot do anything for herself . She has lost most of her vision and hearing and has a hard time speaking. She Also has dementia. I hope you are receiving help. My mom lives in assisted living but will need more services when she’s discharged from the hospital. 

  4. Can SS be diagnosed from nasal fluid?(I have fluid from 1 nostril) I would like to avoid lumbar punctures and other spinal invasions as I have Tarlov Cysts due to connective tissue weakness.

    1. Hello Sue, a lumbar puncture will confirm if you have fresh blood products in your spinal fluid. Establishing a diagnosis of superficial siderosis only requires a noninvasive MRI scan. The radiologist should employ susceptibility weighted imaging (SWI). The SWI is an MRI sequence that is extremely sensitive to venous blood, bleeding, and the presence of hemosiderin.

    2. Hi Sue, I apologize for not seeing your question earlier. SS can be diagnosed with an SWI MRI imaging sequence, so nothing invasive is required. A lumbar puncture or CT Myelogram might be needed to locate an active bleed site if your doctor suspects you may have one. At that point you can discuss the pros and cons of the procedures with your neurologist. Sometimes additional conditions may need to be taken into consideration.

  5. Does anyone use a champion for rare disease who has SS in Scotland? If so can you let me know the name of the person and also how useful would they be for my Scottish brother in law who has SS?
    Many thanks for any heads up peoples!  Best regards, Val ?

    1. Hi Val, I don’t know, but I will ask the SS patients I have the contact information for in Scotland. If Anyone can help you, I will give them your email address so they may contact you.

      Rori

  6. I suffered a brain hemorrhage due to a AVM rupture 3 years ago and my last MRI showed mild case of superficial but my doctor says it’s ok so I’m just wandering if that’s correct or do I need to worry and check out more resources.

    1. Hi Joy, there are different variations of Superficial Siderosis so your MRI should be reviewed by someone qualified to recognize the type. I would suggest contacting Dr.Levy via MGH and asking him to review your scan.

      1. My husband just received an MRI of the brain that shows siderosis in multiple areas. This was an incidental finding by an ENT and has been deferred to our PCP for review who has been in touch with our local Neurology group to discuss as the PCP has never seen this on a scan before. So I am waiting and googling and going crazy wondering what the true clinical implications of this scan are and worried even more as my husband must be on a blood thinner for afib (that is currently not well controlled—efforts are in process to address this aspect).
        How do I be in touch with Dr Levy or another neurologist who specializes in this area so we can start the process of diagnosis, if clinically appropriate of course, and then be able to make decisions about treatment?

  7. Hi All,
    My Dad has just been diagnosed with SS. I am desperately trying to find out more about this drug that’s available. Can you tell me any more please?
    Thanks
    Danny

      1. My mother was just diagnosed with SS as well and I would love information about the drug that’s available too! Thanks!

        1. Hi Ellen, Deferiprone is an iron chelator that can cross the blood-brain barrier. It enters the spinal fluid within 10 minutes and peaks at 1 hour on an empty stomach and 2 hrs if a person has eaten. After 4 hours, it’s done, for the most part, exiting the body through the kidneys and urinary system. It is the only option available to try to reduce iron deposits. Some people will begin to see a slight reduction after as little as a year. In others, it may take two or three years. There are only a few cases to date where iron has been completely removed after ten years.

          Deferiprone is classified as off-label use, so private insurance coverage (if you are in the U.S) often requires an appeal from the neurologist. From personal experience, it does seem to slow the speed of progression. For now, the first step continues to be determining if there is still an active bleed, and if so, finding the location and repairing the bleed site. Otherwise, the chelator will try to remove the new iron first. In my husbands’ case, he was put on a blood thinner for life without the benefit of having his active bleed repaired. This proved to overwhelm the chelator, and his iron deposition has increased to extreme levels. I’d be happy to discuss this further with you if you’d like. Send me a note, and I will reply via email.

        2. My mother was diagnosed with this recently. I would love more information about it. She is already having hearing loss and motor skill problems. She has an appt with a specialist soon hopefully will know about treatment and had an angioplasty and lumbar puncture. We just need info and also support from people. 

  8. There is a drug called DEFERIPRONE Has anyone worked with it or used it and how are you doing so far?

    1. Hi Terry, We have personal experience with Deferiprone. Gary has been taking it for chelation five years now. It was holding his iron build-up and his progression flat-lined for a few years, but he has been put on blood thinners for life now so new, more substantial deposits are building again. The deferiprone is being overwhelmed until he can get his active bleed repaired.

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