Our first suggestion is to print all the information and research available. Organize your printouts and present a copy to every doctor during your initial appointment. You will need to familiarize yourself with the possible clinical symptoms. No one person will experience all the symptoms thank goodness. Every case is specific to the individual.
Talking with others in our Facebook superficial siderosis group we’ve found some folks go many years with only slight symptoms while others progress at a more rapid rate. The reason why is unclear, maybe some patients were able to have their bleed stopped before there was a massive build up while others still have ongoing unrepairable bleeds. 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 MRI series in 2014. Finding answers to 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 he informed us all of Gary’s scans were clear with no sign of new tumors; we should proceed with our ENT’s plan. Gary was to travel to an otolaryngologist because of his rapidly declining hearing. Gary being nosy, popped his copy of the MRI disk into his laptop and read the radiologist report himself. Hmmm? What is this superficial siderosis the report references?
I spoke with the neurosurgeon’s nurse about the radiologist’s findings, and read her a list of superficial siderosis symptoms I found on the Dave Hill Blog site. When she relayed our conversation to the doctor, his response was, “It’s so rare what are the chances he even has that?” This neurosurgeon had been a professor at a well-known university. If a former university professor had never seen a case involving superficial siderosis, then chances are neither has your doctor.
The bottom line is it is challenging to find a neurologist who is both willing to accept a superficial siderosis patient and prepared to research treatment.
There was a 12-month wait before our local neurologist would accept a new patient, so we found an excellent one 100 miles away. Dr. Allison Hennigan was a kind, and more importantly, interested physician. Be prepared to travel if needed.
Do You Have An Active Bleed?
Your next 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 still blood in your spinal fluid. If it comes back positive, then the consensus is you may need to consult a neurosurgeon about trying to have the bleed ablated.
Repairing an active bleed will stop new blood cells from entering your subarachnoid space. As individual blood cells travel through your subarachnoid space, they begin to rupture through a process called Hemolysis. The bursting of cell walls creates a heme overload that triggers the Bergman glia and microglial cells to fight back. Glial cells now manufacture ferritin to capture and store the free-iron. For some unknown reason, the ferritin becomes overwhelmed. Unable to contain all of the free-iron the excess is left floating in spinal fluid throughout the subarachnoid space surrounding your brain and spinal cord. This free-iron settles and forms hemosiderin deposits.
If your MRI report states superficial siderosis is present, then you have hemosiderin deposits present. Dr. Neeaj Kumar, Mayo Clinic, has reported excellent results with repairing ongoing bleeds surgically. Unfortunately, the existing hemosiderin deposits will continue to be problematic. Neurotoxic to nerve function, patients will continue to decline slowly as long as the deposits remain in place.
We have no idea who first decided to try and remove the hemosiderin deposits. Early chelation trials must have failed. The problem? No drug could cross the blood-brain barrier. Dr. Michael Levy, Johns Hopkins, was a member of the research team conducting a 90-day pilot study testing the safety and efficacy of Deferiprone (Ferriprox®), a lipid-soluble iron chelator that was able to cross the blood-brain barrier, on ten patients. The results of this early study were promising enough to expand the study with additional candidates. This extended study published the results December 28, 2017. You may download a copy of this study on our website.
In a May 19, 2015 post to 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 considered a very new treatment option that has strict requirements of its own. Our neurosurgeon suggested we needed a neurologist to oversee treatment. Our PCP was willing to begin chelation once we discovered finding a neurologist was going to be delayed but decided to refer us to a Hematologist.
In the end, we found some very engaged physicians. That’s 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. Never settle.