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 this iron and remove it in a process called Chelation therapy. It’s my understanding if a person suffers from iron overload they feel better when chelation lowers iron levels.
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 in the cerebellum or spinal cord) that are 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. This iron is toxic to nerve function and ultimately can end in nerve death.
There are many other prescription iron chelation medications on the market. Studies have shown Ferriprox as the only prescription drug, at this time, able to cross the blood-brain barrier so it might have a chance at removing hemosiderin (iron) deposits.
Will Ferriprox Make Me Feel Better?
Honestly? No. Most superficial siderosis patients will tell you it doesn’t make you feel better. In our personal experience, you will experience increased fatigue once you’ve been on chelation therapy for a while. Especially if you let yourself become too anemic. Chronic exhaustion is the principal complaint. On the positive side, there have been very few reports of adverse effects from patients we’ve spoken with.
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 trying to chip away any hemosiderin stuck to your brain or spine. It removes the iron 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 hemosiderin (iron) deposits. 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 as there is hemosiderin your neurodegeneration will continue. It will progress slowly but, make no mistake your nerve function will continue to deteriorate progressively.
It takes three years or longer for hemosiderin deposits to show any signs of reduction. Some lucky patients have had all iron disappear after three-four years. A few show a significant reduction or at the very least no increase. Some people, unfortunately, show no change. I don’t know what variables might be different between these groups. Diet, lifestyle, body chemistry, age, gender, who knows?
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 MAY 4, 2018
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.”