It’s human nature to wonder How long will I live? When you’ve been diagnosed with a life-changing ultra-rare illness 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 damage is irreversible and fatal. The word fatal is a definite attention grabber.
The day we found out about superficial siderosis I read that one single sentence in a very early report and my heart dropped.
Three Five years into this journey and we have first-hand insight with a much clearer understanding of the nature of this disease.
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. There is no doubt the toll on your body is punishing. The possibility of dementia is alarming, but in reality, only an extremely small percentage of our community will ever face this issue.
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 the stage where he had to change his life radically.
There does seem to be a point of symptom acceleration.
For us, the changes during the past three years have been intense. (To read about Garys’ current condition see Inescapable Realities) Slow progression eventually evolves in response to each patients’ free-iron load and location in their brain or spine. If you have a large wide-spread hemosiderin deposit the likelihood you will develop multiple serious superficial siderosis related health problems down the road is strong.
A lucky few will have their bleed repaired early so they have a chance to escape heavy hemosiderin build-up. Five years into this journey and the evidence is overwhelming; even if an active bleed is stopped damage will continue if hemosiderin deposits keep releasing toxic free-iron molecules into your cerebrospinal fluid. 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.
This brings us back to 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 the true enemies. Constant pain, feelings of isolation, and loss of independence are constant reminders of what this disease will bring to the unlucky few, the one in three million diagnosed with superficial siderosis.