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