Finding Help for Superficial Siderosis
May 21, 2015 Living With Superficial Siderosis
We suggest you print out all the information and research available. Organize this into a folder you can present to every doctor at your initial appointment. You need to become familiar with all of the possible clinical symptoms and see which ones match your history. No one experiences all the symptoms thank goodness. Every case is specific to the individual.
Make a plan
Your first step after diagnosis should be to find out if you have an ongoing bleed. Lumbar puncture is the most reliable and accurate procedure for determining if there is blood in your CNS. If it comes back positive, then the consensus is you will need to consult a neurosurgeon to see if there is a surgical option to have the bleed ablated.
Repairing an active bleed will stop blood cells from entering the CNS. Cerebellar Bergmann cells, microglia, and superficial astrocytes will all attract blood cells that will break down and just keep adding to the hemosiderin build up. If your MRI has noted SS, then you already have hemosiderin deposits present. Dr. Neeaj Kumar, of the Mayo Clinic, has reported excellent results with repairing ongoing bleeds surgically.
Hemosiderin deposits will continue to be problematic. Neurotoxic to nerve function patients continue to slowly progressively decline. Talking with others in our SS group we’ve found some folks go many years with slight symptoms while others progress at a more rapid rate. It may be some were able to have their bleed stopped before there was a massive build up, and others still have ongoing bleeds that are not repairable. It is important to remember, the rate of decline is unique to every individual.
Prepare for roadblocks
Our neurosurgeon was looking for a tumor when he sent Gary in for a new series of scans in 2014. Finding answers for the symptoms Gary was experiencing was exhausting. One small paragraph at the end of the radiologist report would alter our lives forever.
“IMPRESSION: Diffusely abnormal decreased signal of the meningeal surfaces more pronounced about the brainstem, cerebellum, and basilar cisterns. There appears to be mild associated cerebellar atrophy. The MR findings, as well as the clinical report of dizziness and hearing loss, are consistent with superficial siderosis.The abnormal decreased signal is observed primarily on the T2-weighted series correlating with the pial and ependymal surfaces most pronounced about the brainstem, basilar cisterns, and cerebellum. Findings correlate with hemosiderin deposition in a pattern of superficial siderosis. Etiology for superficial siderosis is presumed to include episodes of subarachnoid hemorrhage although not always clinically evident. Sensorineural hearing loss and cerebellar findings, by a report, are often presenting symptoms and appear to correlate in this case.”
When our neurosurgeon called to give us the scan results he happily informed us all the scans were clear. No sign of tumors. We should proceed with our ENTs plan; Gary should go to an otolaryngologist because of his rapidly declining hearing. Gary is nosy. He popped the MRI disk we had picked up from the radiologist and read the report himself. Hmmm? What is this superficial siderosis the report references?
We called and spoke to the office nurse. I brought her attention to the radiologist’s findings, and then I read her a short rundown of the symptoms. When she relayed our concerns to the doctor his response was, “I’ve never heard of that and it’s so rare what are the chances he even has that?” I only bring this up because this neurosurgeon had been a professor at a well-known university. If a former university professor was not familiar with Superficial Siderosis, then chances are neither is your doctor.
The bottom line is it is challenging to find a neurologist or any physician who is not only willing to accept an SS patient but also is prepared to take the time to research.
We have no idea who thought initially to try and remove hemosiderin deposits. Earlier chelation therapy trials must have failed. The problem was no drug could cross the blood-brain barrier into your spinal fluid.
Dr. Michael Levy of Johns Hopkins oversaw a 90-day pilot study testing the safety and efficacy of Deferiprone (Ferriprox®), a lipid-soluble iron chelator that was discovered to cross the blood-brain barrier, on ten patients. The results of this study were promising enough to expand with additional candidates for an extra year. The research is now ongoing with results expected sometime in 2016. UPDATE:
December 2017 Study published December 28, 2017.
In a May 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.”
Chelation therapy is still a very new option that has strict requirements of its own. Our neurosurgeon suggested we needed a neurologist. Our PCP was willing to begin chelation but after research decided to refer us to a Hematologist.
In our area, it would be a 12-month wait before a local neurologist would accept a new patient, so we searched until we found an excellent one 100 miles away. We found Dr. Allison Hennigan to be a kind and most importantly interested physician.
That is what you need to look for in a doctor. Our hematologist and neurologist have both emailed Dr. Levy for advice. Dr. Levy freely gives his time to answer any physician who contacts him. If your doctor tells you there are no options, find a new one. If your doctor says you there is nothing to worry about, find a new one.
Do not settle.