Understanding Chelation Therapy

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Ferriprox-deferiprone

There is some confusion when it comes to understanding Ferriprox and superficial siderosis. Ferripriox (Deferiprone) is a prescription drug whose primary purpose is treating people who have transfusional iron overload due to Thalassemia syndromes.¹ Deferiprone was designed to bind to iron   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 areas of their brain (most often the cerebellum or spinal cord) that forms as the result of blood infiltrating into their central nervous system from a trauma. An accident, surgery, stroke, etc., but somehow blood was introduced into their spinal fluid. The average person will naturally absorb and remove this blood. A rare few people will not be able to remove it. Free-iron is toxic to nerve function and ultimately tissue damage.

There are many other prescription iron chelation medications on the market. Studies have shown Ferriprox as the only prescription drug (at this time) with the ability to cross the blood-brain barrier and enter the cerebrospinal fluid so 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 fatigue 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,  Ferriprox (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 Ferriprox goes to work. Bloodstream iron will always be the first target before any remaining Ferriprox makes it’s 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 Ferriprox two days a week. On his medication off days, he loads up on iron-rich foods, so he doesn’t become too anemic. Even by following this schedule there has been no escaping the fatigue or always staying slightly anemic.

You also need to be aware there is a chance of severe side effects. All SS patients who are 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. What it will try to do is remove the toxic free-iron molecules which were 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 a bleed they were also preventing the progression of the symptoms. We now know this to be untrue. As long hemosiderin is present to release free-iron molecules into your cerebrospinal fluid your neurodegeneration will continue. It will progress 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 lucky patients have had all iron disappear after three-five years. A few show a significant reduction or at the very least no increase. Some people, unfortunately, show no change. There are no answers, as of yet, why it works for some and fails for others.

Taking a chance

Why even try Ferriprox? We say why not. The decision to start Ferriprox (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.

In Part 2 I outline sources  Ferriprox is available from and some of the resources out there.

UPDATED Nov 13, 2019

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.”

About Rori Daniel

Living With Superficial Siderosis began as a way to keep family and acquaintances updated after my husband Gary was diagnosed with Superficial siderosis in 2014. We invite you to join us as we share the details of our life, finding care, and the search for answers of how to navigate this extremely rare disorder.

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