Research Day

Waking up for the Big Day I woke up to a bad headache, probably a bit of nerves and excitement about going to the best hearing institution in the United Kingdom. After all, I’m a musician and what’s the fun in music without having any hearing? It’s been a difficult journey but reaching out on Twitter to the Joint Royal College of Physicians Training Board (JRCPTB) had put me in contact with a doctor who is doing her PhD on the audio-vestibular (hearing and balance) components of superficial siderosis. My sole intention of contacting the JRCPTB however, was not that of getting help for myself, but an attempt to make it mandatory or at least advisory for ENT doctors/surgeons and audiologists in the U.K, to screen for superficial siderosis if a patient has had previous head/spinal trauma or surgery and now has unexplained hearing loss. Helping others to avoid this

read more….

Is Neuroprotection The Answer?


Can The Progression Of Superficial Siderosis Be Slowed? The holy grail of any rare disorder is discovering the cure. With Superficial Siderosis, so far, chelation therapy has offered the only solution for removing the iron. Where does this leave a patient who can’t physically tolerate chelation or can’t afford the cost? Where is the hope? Are We Looking For The Wrong Answer? Watching the progression of someone in the later stages of Superficial Siderosis is heartbreaking. While we now have eight volunteers who have generously committed to leaving their brain for future study, those answers will be years away. As a patient community, our end game will always be discovering a way to stop the devastation. Should our priority shift to finding out how to slow cell degeneration? To identify treatment options safely tolerated by every patient, whether or not they are currently on chelation therapy that will be neuroprotective

read more….

One More Family

Be Counted

Our superficial siderosis Facebook family is a tightknit group. We share our problems and successes; we listen and learn from each other. It doesn’t matter that we’ve never met because, along the way, it began to feel like we know each other. Still, no matter how our group has grown these past years, there will always be one more diagnosis, and one more family learning someone they love has superficial siderosis.

read more….

Combating Neural Damage


The holy grail of neurodegenerative disease research is discovering a pathway to healing—reversing neural damage, either through regeneration or surgical reconstruction. Our nerves control multiple biological functions. The complexity of cranial nerves has made regenerative therapies exceptionally challenging. The olfactory nerve (I) has become a favored model for the study of neural regeneration through the rewiring of axons or surgical transplantation.¹ It contains afferent fibers and is the only identified cranial nerve that may regenerate depending on the type and degree of underlying damage. Cranial nerves are divided by responsibility; afferent nerves control communication by collecting sensory information, while efferent nerves control motor functions. The physical makeup of each is distinct; efferent neurons have short dendrites with a long axon, while afferent neurons have long dendrites and a short axon. Cranial nerves may contain solely efferent fibers, afferent fibers, or a combination of both— making the current methods of repair,

read more….

Introducing Mass General Research Institute

Facebook giveaway 1

Rare Disease Week kicks off for the SSRA with a trip to our Chief Medical Advisor, Dr. Michael Levys’, new research home with the Mass General Research Institute. What better way to celebrate than a giveaway! With a few clicks or taps, you can be entered to win some really sweet swag from the good folks at Massachusetts General Hospital. You have a chance to win an Official MGH Men or Woman’s zip-front fleece jacket, MGH Ball Cap, or an official grey scarf embroidered with our SSRA logo. There will be several winners so why not enter? The contest details are below. Mass General is the #1 Hospital on the East Coast and named #2 in America by U.S. News and World Report. They are the only hospital in America to be recognized across all 16 specialties assessed by U.S. News. Let’s talk about Dr. Levys’ new facilities for a

read more….

Is Your Chelation Working?

C4CBA4AB C98A 4846 AE91 D9A9BC35092D e1576509405451

When the two-year study on the efficacy of Deferiprone was published one finding stood out. Patient-participant physicians submitted MRIs during several stages of the study; differences in MRI equipment, software, and magnification produced data that was difficult if not sometimes impossible to use. To correct this situation the research team began a project developing a software tool for use with the OsiriX DICOM viewer that would not only measure the exact amount of iron deposits with a 3D visual but answer the question is your chelation working? With high performance and an intuitive interactive user interface, OsiriX is the most widely used DICOM viewer in the world. It is the result of more than 15 years of research and development in digital imaging. It fully supports the DICOM standard for an easy integration in your workflow environment and an open platform for development of processing tools. It offers advanced post-processing

read more….

The Pathway To Healing


What if research could identify a way to give your body a fighting chance? Kick-start the path to healing? Ataxia, sensorineural hearing loss, and myelopathy; These are the real enemies you confront because of superficial siderosis. Yes, free iron wreaks havoc, but this mix of symptoms will rob you of your independence and your wellbeing. The damage at the cellular level creeps along annoyingly slow for many years until one day you realize, “What’s happening with my body? To my life?” The goal of surgery is clear. To stop an active bleed from creating a heavier or more widespread iron build-up. Newly diagnosed patients are often confused by the chelation process. Will it make the symptoms better? Will it make me better? The purpose of chelation is to remove the iron and stop your cell damage from progressing. It won’t address the damage already done. In theory, if you remove

read more….

The Results Are In

Image 12

“Does Deferiprone provide a clinical benefit to the superficial siderosis patient?”   If you remember, the April 2017 edition of The Neurology Journal gave us an early peek at what the study findings might be in the final revision of Two-year Observational Study Of Deferiprone In Superficial Siderosis¹. The study was released this past December 28th, and the results have been published. This blog post is our cliff note version of the study, keeping in mind, we’re not medical researchers or doctors.   Initial recruitment included 48 participants.   Nine withdrew from the study because their insurance wouldn’t cover the off-label use of Ferriprox and one for other reasons. 38 people began the investigation, and over the course, two dropped out because of neutropenia concerns, four dropped for cost issues and one left for unrelated health reasons. 31 people completed the study. Each participant submitted a current neurological exam, a

read more….

Superficial Siderosis Webinar Transcript

File Jul 28 1 05 10 PM

Superficial Siderosis Webinar Transcript Presented by Dr. Michael Levy   To view the recorded webinar you may watch here Download a copy of the transcript and slides  here Click on the individual slide to view larger   Hi, my name is Dr. Michael Levy. I’m an assistant professor at Johns Hopkins University in Baltimore and a neurologist. I see patients with Superficial Siderosis of the nervous system. This webinar was really a joint effort with my patients on Facebook, who recruited me to present a twenty to thirty minute overview about Superficial Siderosis. Today I’ll go through just kind of the broad strokes of the pathophysiology of the disease, at least the parts we understand. We’ll work out the treatment options that are available. First, what is Superficial Siderosis? In broad brush strokes, Superficial Siderosis is an iron overload condition of the brain and spinal cord. Patients with Superficial Siderosis

read more….

Superficial Siderosis Webinar

File Jul 28 1 04 42 PM

Superficial Siderosis Webinar In this webinar, Dr. Michael Levy, The Johns Hopkins Hospital, Department of Neurology explains just precisely what Superficial Siderosis is. Listen to an explanation of the known causes, how your doctor will make the diagnoses and what your treatment options are in this easy to understand 30-minute presentation. To view a written transcript of this webinar click here To download the transcript and slides in .pdf form click here     Huge thanks to James Hines for coordinating this webinar and Dr. Micheal Levy

read more….