The Superficial Siderosis Patient Registry

sspr landing

  Your Superficial Siderosis community advocates, the Living With Superficial Siderosis website and The Silent Bleed -Your Superficial Siderosis Charity have joined together in a grassroots effort to support the launch of a patient-powered registry. You are being asked to take part. Before you agree to participate in this volunteer effort, it is important that you understand what this registry is about and what will be done with the information that you provide.  The disadvantages we fight include the geographic distance between our community members and finding interested researchers or healthcare industry partners. Superficial Siderosis patient member groups on social media have been our primary means of communication. Recent Facebook group discussions led to the realization our Superficial Siderosis community needs a patient registry accessible through a web-based platform. After some consideration, it seemed the most cost-effective way to a Superficial Siderosis patient registry was to build the first one ourselves. This website takes advantage of internet security protocol, registration requirements, and restricted member login for access to profile and survey areas. Our personal information will be stored the same as yours. No information will ever be offered for sale. No identifying information will ever be disclosed without your express consent.  There are three registration…

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Decoding The Superficial Siderosis MRI

MRI Superficial Siderosis5

Tracking Your Hemosiderin   There has been a definite increase in people diagnosed with superficial siderosis. The improvements in neuroimaging have resulted in advanced iron sensitive 2-D and 3-D MRI techniques. Thankfully you can now be diagnosed in vivo and, if you’re fortunate, early enough to do something. Researchers have now identified three branches of superficial siderosis, each with a unique clinical presentation and pathology. Infratentorial Superficial Siderosis (iSS) Type1 Classical is the superficial siderosis which affects our group. Clinically it presents with hearing loss, ataxia, myelopathy and slow progressing neurodegeneration. Infratentorial Superficial Siderosis (iSS) Type 2 Secondary will show classic hemosiderin staining on the MRI but will not present with any clinical symptoms or degeneration. The newcomer is Cortical Superficial Siderosis (cSS). Hemosiderin deposition is limited to cortical sulci of the cerebral hemispheres. The cerebellum, brain stem, and spine escape deposits. Cortical Superficial Siderosis (cSS) has different clinical symptoms and causes. It seems to be age related with a connection to cerebral small vessel disorders.¹ Annual MRI   Gary is in his fourth year now of chelation therapy. His first dose of Ferripriox was in July 2014 and his original hematologist always made sure he ordered annual MRI scans of his head and spine.…

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Tips For Developing Great Patient-Provider Communication Skills

Patient Provider Communication Skills

Communication must be a two-way street Rare disease patients often battle a variety of complex clinical symptoms. A visit to your specialist often results in as much confusion by the end of your appointment as at the beginning. Healthcare providers are often under pressure to keep patient face time to a minimum, so clear communication is a vital step in understanding your plan of care. Your doctor needs accurate information to help you. It’s essential patients with short-term memory problems or mild cognitive impairment pre-plan for what they need or expect to learn during their appointment. Gary keeps a small daily notebook where he records his vitals, notes about medication or symptom changes, fluctuations in blood pressure, pain levels and new questions. Without this health diary, he is unable to remember significant details or forgets essential questions whether an appointment is a few months down the road or tomorrow. He brings his notebook to every regular office or hospital visit. Physicians find details with dates and times very helpful. It also helps if you can tell the story of events leading up to a specific incident. I was doing my usual ____ late in the afternoon, and then I was doing…

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20 Strength Exercises When You’re Fighting Mobility Problems

bands exercise

  Finding a user-friendly exercise program for patients diagnosed with related ataxia symptoms isn’t easy. For example, Gary is limited to lifting 10lbs so he doesn’t cause fresh bleeding into his CNS. Standing or sitting with free weights is a no-no; even without the lifting limit, you wouldn’t want to be the spotter with his balance problem. Before his nerve block, getting down on the floor to do planks, sit-ups or leg lifts would bring an instant migraine. Exercising was a minefield. Gary’s appointment for his balance and gait evaluation gave us the opportunity to ask the kinesiology department these questions. He needed a low-impact strength program designed so it: Could be done at home Wouldn’t trigger headaches Balance problems wouldn’t interfere Muscle tone could be strengthened or maintained   The physical therapist built a program with ten strength exercises, each set targeting the upper or lower body using resistance bands. If you’ve never used a resistance band during exercise, I think you will be surprised with their efficiency. The therapist first demonstrated each activity and then had Gary repeat it. Most are done sitting on a chair. A few of the more advanced exercises require holding on to a…

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The Struggle With High Cost Medications

copay struggle

Life with a rare disease or chronic illness has a way of taking over; juggling responsibilities, appointments, kids, and family. Take your meds on time. Eat something. Try to work. Order your meds. Unless you’re lucky enough to have perfect drug coverage the last one is always a heartbreaker. People who battle chronic illness often struggle to cover their high-cost co-pay share of prescription medications. Newly diagnosed patients are often not aware of the one bit of information that may help ease some of the expense. There are programs out there who offer copay assistance specifically for individuals who have drug coverage but are faced with covering high out-of-pocket costs. Often programs are often offered by the specific drug manufacturer, but there are also many non-profit organizations who will work with your pharmacy or physician, so cost doesn’t keep you from filling your prescriptions. Every day, millions of chronic disease patients are forced to go without the medication and treatments that can drastically change the quality of their lives. In fact, 3 out of 10 Americans cannot afford to cover the expensive medications they need—even with insurance. – For example when Gary first began his chelation therapy with Ferriprox our…

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Becoming A Better Friend To The Chronically Ill


Becoming A Better Friend To The Chronically Ill   If you are fighting a battle with a rare disease or are chronically ill, you are familiar with the challenges you face daily. Sometimes the same can’t be said of your friends, family or acquaintances. This blog post is for everyone who knows someone fighting a battle, not just with superficial siderosis but any rare or chronic condition. No one ever intentionally wants to hurt someone’s feelings or cause them distress. Intentional or not it happens. I hope you can take something from this post and help yourself and your friend by becoming more aware, present and mindful. Learning to Listen Sometimes people need to practice the art of mindful listening. Engaging in a conversation with someone who has a rare disease or chronic condition requires particular skills. It helps if, in the beginning, you allow them to control the flow of conversation. Take a moment to think about your questions. “How are you?” is often the first thing people will throw out in a passing conversation. It’s an easy question, but subconsciously it sends the message: ” I know you’re ill, but I don’t want to hear all the gory details.”…

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Five-Two Diet Plan Update

Five-Two Plan

Some of our earliest blog posts explained Dr. Levy’s suggested medication dosing schedule and diet recommendations. We dubbed this the Five-Two Diet Plan so we would have an easy way to write about it.

Two and a half years in and we can report our high/low iron foods cycle diet plan is a success at reducing Gary’s ferritin level. His body seems to have leveled out naturally and hovers in a channel now.

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Following The Low Road: Low Iron Diet

UPDATE DECEMBER 2017: Gary has been following this diet plan for 30 months now. His serum ferritin level now stays between 11.2 and 9.9. His lowest level recorded was 8.8. He cycled off his Ferriprox for a week, and we iron loaded his diet until he was back in the mid 9 level.



Life Expectancy of Superficial Siderosis Patients

life expectancy

Life expectancy, on the surface a straightforward question. It’s human nature to wonder “How long will I live?” A life-changing illness and thoughts like this can consume you. Superficial Siderosis Life Expectancy, two words and it becomes intensely personal. The early impressions were dark; no cure, the damage is irreversible and fatal. That word fatal is a definite attention grab. The day we found out about superficial siderosis I read that statement and my heart dropped. Three years into this journey and we can add some personal insight into understanding the nature of this disease. If you study possible combinations of symptoms that could assault you, it should become clear while your body and psyche will suffer none are life-threatening in an immediate sense. It is a fair assumption the toll on your system is punishing. The thought of autonomic problems or dementia is alarming, but only a small percentage will ever face these issues. We know that in the early stages superficial siderosis moves slowly   Symptoms creep along driving you crazy, but it does take time. Twenty-two years passed after Gary’s tumor removal before his symptoms progressed to where he had to change his life radically. There does seem to be a…

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Your Life As A Germ Magnet

compromised immune system

Germ Magnet Immune System Life as a germ magnet. The average person will stroll through life never giving much thought to living with a weak immune system. A person with Superficial Siderosis will often find out the hard way that caution should be the word of the day. Every day we’re bombarded with invisible bugs. You think you’ve taken care to avoid those unseen dangers, but life as a germ magnet just about guarantees something nasty is bound to take hold. Last winter is the perfect example. Our daughters family was suffering from a particularly nasty flu bug. We had, in all honesty, been avoiding their house, but we found ourselves answering a plea from one of the granddaughters. A stopped up commode. Gary walked in, plunged the offending toilet into submission, and walked straight to a sink in another room. He thoroughly washed his hands and arms. I stood ready with the hand sanitizer and slathered him down. We drove home and within three hours Gary was down, 103-degree fever, and a very nasty case of the flu. I was unaffected even though I had also been in the bathroom cleaning up behind him.   We learned an important lesson…

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Hemosiderin in the Cerebellum

Multiple Angioma Cerebellum Sagittal Siderosis

Putting A Face On The Enemy We’ve all read the descriptions of how hemosiderin deposits (Iron Salts) form. Do you sit looking at the MRI scan results wondering just where we are supposed to be looking? Nothing explains it better than a picture. When you look closely at this photo of hemosiderin in the cerebellum, you can see how thick the deposit is. It makes sense why researchers think it will take years for your iron buildup to begin to lessen. Deferiprone has to journey from your stomach to the upper G.I tract and into your bloodstream. Next, what doesn’t bind to the iron from your last meal will travel through the blood-brain barrier and into your spinal fluid. The molecules target free iron from the hemosiderin and help carry it out of your body via your urine. The process is indeed a wonder of modern medicine.  

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