Our first suggestion is to print all the information and research available. Organize your printouts and present a copy to every new doctor during your initial appointment. Next, you need to familiarize yourself with the possible signs and clinical symptoms and note any you may be experiencing. Every case is specific to the individual, so no one experiences every symptom, thank goodness.
Looking back on discussions in our Facebook superficial siderosis group, we’ve found some people go many years with mild 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 searched for a tumor when he sent Gary in for a new MRI series in 2014. Our journey to finding answers to the symptoms Gary was experiencing was exhausting. But, 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 and the clinical report of dizziness and Hearing Loss will affect 95% of Superficial Siderosis patien... More 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. Hearing Loss will affect 95% of Superficial Siderosis patien... More and cerebellar findings, by a report, often present symptoms and appear to correlate in this case.
When our neurosurgeon called, he explained 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?” Our neurosurgeon had been a professor at a well-known medical 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.
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. A lumbar puncture may help confirm if there is still blood in your spinal fluid, but it will not work if your bleed is intermittent. A CT Myelogram is now the preferred method to locate an active bleed from a dural defect. If a bleed source is located, you should consult a neurosurgeon about having the bleed ablated.
If your MRI report states superficial siderosis is present, then you have hemosiderin deposits present. The Mayo Clinic has reported excellent results with a complicated bleed repair. Unfortunately, the existing hemosiderin deposits will continue to be problematic. Neurotoxic to nerve function, patients will continue to decline slowly as long as the hemosiderin remains 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 was a member of the Johns Hopkins research team conducting a 90-day pilot study testing the safety and efficacy of Deferiprone (Ferriprox®). This lipid-soluble iron chelator was able to cross the blood-brain barrier on ten patients. The results of this early study were promising enough to expand the research with additional candidates. This comprehensive study was published on December 28, 2017. You may read and download a copy of this study on our website.
“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.“Dr. Michael Levy, May 19, 2015 post to the Facebook support group
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 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 there is nothing to worry about, find a new one. Never settle.