The Role Of Chelation In Superficial Siderosis
There is some confusion when it comes to understanding chelation therapy and superficial siderosis. Deferiprone (Brand name Ferriprox) is a prescription medication whose primary purpose is treating people who have transfusional iron overload due to Thalassemia syndrome or Sickle Cell.¹ Deferiprone was designed to bind to iron in the bloodstream and remove it in a process called Chelation therapy.
Superficial siderosis patients do not have too much iron in their bloodstream.
They have iron deposits (hemosiderin) stuck to surface areas of their brain (most often the cerebellum) and spine that build due to blood infiltrating into their central nervous system. An accident, surgery, stroke, or another event allowed blood into their spinal fluid. The average person will naturally absorb and remove this blood. However, a rare few people will not be able to clear it. Free-iron is toxic to nerve function and the underlying neural tissue.
There are many other prescription iron chelation medications on the market. However, studies have demonstrated deferiprone is the only prescription chelator (at this time) with the ability to cross the blood-brain barrier and enter the cerebrospinal fluid, so it has a chance at removing iron.
Will Ferriprox Make Me Feel Better?
Honestly? Many superficial siderosis patients will tell you it doesn’t make you feel better. Some feel it does. In Gary’s case, he experienced increased fatigueOverview The brain plays a crucial role in regulating sleep ... More once he had been on chelation therapy for a while. If you let yourself become too anemic chronic exhaustion is a common complaint.
Why do you feel so tired when you take Ferriprox?
Superficial siderosis already causes fatigue. Don’t forget, deferiprone is primarily for people with transfusional iron overload due to Thalassemia. SS patients have normal blood iron levels. At this time, there is no way to target where the deferiprone goes to work. Bloodstream iron will always be the first target before making its way to where it’s really needed to work. Unfortunately, it reduces the bloodstream iron levels that your body needs to function at peak levels.
Gary follows a reduced iron diet five days a week while taking his medication and cycles off the deferiprone two days a week. He loads up on iron-rich foods on his medication off days, so he doesn’t become too anemic. However, even by following this schedule, there has been no escaping the fatigue or staying slightly anemic.
You also need to be aware there is a chance of severe side effects. All SS patients on chelation therapy with Ferriprox have regular blood tests run to track their neutrophil levels and liver function. There have been rare instances of patients developing agranulocytosis. Agranulocytosis is a deficiency of a type of white blood cell whose purpose is to fight off infection. If your neutrophil level drops too low, you need to cycle off the medication until it stabilizes. If neutropenia issues develop, you will not be able to continue chelation.²
Will Ferriprox Cure Me?
No, Ferriprox will not cure superficial siderosis. It will try to remove the toxic free-iron molecules that are unable to remain bound by a protein called ferritin. You may be wondering why you would take a costly medication that will make you feel tired and still not cure you?³ At one time, medical professionals thought by stopping the bleed, they were preventing the progression of the symptoms. We now know this to be untrue. As long hemosiderin is present, your neurodegeneration will continue. It will progress more slowly but, make no mistake, your nerve function and surrounding tissue will continue to deteriorate progressively.
It takes years for hemosiderin deposits to show any signs of reduction. Some patients have had their iron reduced after three-five years or, at the very least, no increase. A few reports have been received on the success of removing the iron found on their brain after ten or more years. Some people, unfortunately, will show no change. There are no answers to why it works for some and fails for others.
Taking a chance
Why even try Ferriprox? We say why not. The decision to start deferiprone comes down to the quality of life. How do you see your future? We aren’t young (we’re grandparents), but we are a long way from giving up on life. It is our choice to be as proactive as we possibly can. The odds are not in our favor if we choose to do nothing. Win, lose or draw; we want to fight superficial siderosis.
¹http://www.ferriproxtotalcare.com/docs/APOMedGuideandPIFerriproxTCP.pdf
²http://www.ferriproxtotalcare.com/docs/APOMedGuideFerriproxTCP.pdf
³May 19, 2015 post in our Facebook group Dr. Levy reported: “To all SS patients on Ferriprox or thinking about Ferriprox, I want to take this opportunity to clear a misconception about the expectation of this drug. Ferriprox is an iron chelator, a small molecule that binds iron. The unique property of Ferriprox that makes it suitable for SS is its ability to dissolve into the spinal fluid and chelate iron on the surface of the brain and spinal cord. While Ferriprox is chelating the iron in an SS patient, it should not make the patient feel better in any way (except psychologically maybe?). Remember, symptoms from SS are due to damage to the outer layer of the brain due to iron toxicity from the adjacent siderosis layer. As long as the siderosis is present, the damage it causes continues even while the iron is being chelated. SS patients should expect to continue to progress in their SS disease until the iron is gone and healing can start. In my experience, it takes two years in most patients to begin to see reductions in the siderosis extent by MRI. Those brain areas that have been cleared can begin to heal while neighboring siderotic brain tissue is being damaged. Since I’ve started using Ferriprox in 2010 (the majority since 2012 when the drug was FDA approved in the US), only a few patients have managed to clear all or most of their iron, and in those patients, we saw the most clinical improvement.”
