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. 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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The Common Thread Searching For The Unexplained

common thread

Diagnosis of superficial siderosis is rare. It’s so unique in fact if you searched for information on one of the national or international rare disease databases until the last few years it wasn’t listed.  We should be glad so few will face a diagnosis of superficial siderosis, but you can’t help but wonder. Is there a common thread to be found? I find myself thinking about this subject a lot. Dr. Levy has said they don’t know why some people are overwhelmed by the blood infiltration. An unlucky spin of the wheel. We’re not medical professionals but what if we brainstorm a little and toss around a few ideas. First, let’s talk about what we do know. Superficial Siderosis is not hereditary; you aren’t born with it. Superficial Siderosis is not contagious; you can’t spread it or catch it.  Age or gender do not play a significant role in the diagnosis; patients were diagnosed, in the past, at an older age due to the slow nature of the progression, but newer MRI technology is making earlier detection possible. All confirmed superficial siderosis patients had experienced unnatural blood infiltration into their central nervous system; trauma, surgery, aneurysm or stroke that caused long-term bleeding.…

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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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Staying Woke:

Superficial Siderosis Awareness

Get Woke   I like listening (ok, eavesdropping) to my granddaughters when they find time to get together. Our grandkids ages range from a first-year college student, high school, elementary to toddler. Conversations can be eye-opening. You learn a lot: who’s salty, why something’s lit, and being woke. If you follow social media, you see it everywhere. It stays with me, this word. Woke. People are willing to be engaged.   It’s Up To Us   There’s a significant message in “being woke” for the Superficial Siderosis family. We are obligated to spread awareness. Whether for ourselves or an advocate for a family member or friend. Those who suffer from this ultra rare condition need all the support we can give. The Silent Bleed squad has been busy providing flyers, selling wristbands and hosting fundraisers over in the U.K. Here in the states, we hope our blog will help spread awareness, provide a little education, and some of our insights.  What Will You Do?     Not everyone can start a charity or find time for a blog. When you’re chronically ill, getting out of bed some days is a big deal. We just want you to spread the word.…

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Railroad Spike: Chronic Headache Pain

chronic headache pain

Living With Chronic Headache Pain   Chronic headache pain has been in our lives for years. Our Superficial Siderosis community has many fellow sufferers. On good days the pain is a dull throb pecking away, neck, eyes, and top of your head. The not so good days it feels like a hot railroad spike through the base of your skull sprinting up to your eyes. Pain that radiates into every part of your head creating excruciating agony.   It’s A Headache Not A Marathon   Gary has always tried to control his pain with over-the-counter pain medications, sitting quietly with his eyes closed and keeping his neck still but this hadn’t been working the last eight months. The severity is increasing along with the duration. This June an unusually debilitating headache took hold and wouldn’t ease up for 76 DAYS. He agreed to a trip to the Shreveport VA emergency room and even though they did the best they could it didn’t help. They ran multiple tests, warned him of the dangers of too much acetaminophen and treated him for a tension headache with a lovely shot of something they said would make him feel so much better. His pain eased…

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Testing, Testing, Hope

Test Hope

The Whirlwind Test Tour We’ve been doing the whirlwind testing tour with our many specialists these past few weeks. Not only was it time for our quarterly neurology follow-up but we found ourselves eagerly waiting for the first MRI and hearing tests since starting on Ferriprox this past year. Our health insurance network is being changed from a PPO next year to an HMO, so we suffered few weeks of panic after we received notice from BCBS of Texas. HMO networks are not popular with doctors in our rural area. Seven specialists and one PCP currently provide care for Gary, and no one was in an HMO network. UPDATE: Every doctor but the neurologist joined an HMO network before January.   First Stop   Our first stop was the audiologist. Dr. Lee had mailed us a copy of a  magazine article this summer from one of her professional publications. It outlined audiologists now being asked to screen patients who present with a sensorineural hearing loss. Identify case history, determine if an MRI and neurological referrals were necessary. The article then describes a diagnosis of a rarely seen neurodegenerative condition called Superficial Siderosis, its effect on hearing loss and the importance of early diagnosis.…

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Rethinking Cholesterol Medication

myelin axon nerve cell

Rethinking Statin Use   Are you a long-term user of popular statin drugs on the market? Studies are convincing it’s time to rethink cholesterol medication use if you’re in a high-risk, neurodegenerative patient group. If you are already fighting nerve degeneration, there is evidence your cholesterol medication may be contributing to peripheral nervous system damage. 25% of the adult population in the US, over the age of 45, is now on a prescribed statin. The popularity of cholesterol-lowering medication was rolled out with both an advertising blitz and a push from industry drug reps. Physicians initially thought statin drugs had few side effects. For the high-risk individual who has heart disease, statin drugs can be a life-saver, lowering bad cholesterol by as much as 50 points.    Does My Body Need Cholesterol   Cholesterol has become synonymous in the public mind with heart disease. Cholesterol is bad, bad, bad; an ingrained mantra pounded into our subconscious. If your arteries become blocked and you suffer a heart attack, then yes, you have a problem. For people with neurodegeneration, cholesterol plays a vital part in trying to maintain healthy nerve function. 25% of cholesterol resides in your brain as Myelin Sheaths.  The parts…

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Navigating The Iron Food Road

MRI Color Enhanced

Finding The Iron Content Of Everyday Foods   I’ll be honest – this is a tough one – no, you don’t have to follow the 5/2 diet cycle while on Ferriprox. Gary does because we’re trying to get our money’s worth. In March of 2014, when first diagnosed, the radiology report noted there was only “mild associated cerebellar atrophy.” Working, driving or walking a straight line were all still possible. Chelation therapy using Ferriprox started a year ago in July 2014. We know the deterioration continues even though you are on the medication. Ferriprox needs years to work on trying to remove the iron accumulation. The MRI in February of 2015 (11 months later) shows “prominent volume loss in the superior aspect of the cerebellum.” We probably could have guessed the latest report ourselves. Gary’s symptoms were getting worse so you can understand we feel the need to help this process along. The recommended daily intake of iron   A 57-year-old man needs an average of 8 mg of dietary iron daily to stay healthy. If you check out the Iron Content Database you’ll see the average fast food double meat cheeseburger (355 gm total weight), vegetables and condiments contain a total…

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Whole New Ballgame: ReSound Linx²

The two-week update on the ReSound Linx² hearing aids The question most asked, “Do you still like the new hearing aids?” Short answer, yes. But there are so many features we’ve just barely scratched the surface. Classified as Smart Hearing Aids, they can be set to work in tandem with your smartphone with an app you download. This app allows your phone to work as the control for both the preset and customized audio settings. 90% of the time close captioning is no longer needed when watching T.V., but since this is a case of  Sensorineural Hearing Loss, there are always problems with tones. Some voices are at a pitch that is just impossible to understand. The first two weeks sound levels were set at a 60% level. Our audiologist explained your brain needs time to process all the new sound. The first two days certain sounds were deafening with some distracting clicks (the dog’s tags or change in the pocket). That leveled out during the week. When we went back for the two-week follow-up, the most significant complaint was sound had become muffled and not as clear as the first few days. Since it didn’t take long to get used to…

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The Decision To Choose Chelation Therapy

Ferriprox-deferiprone

The Decision To Choose Chelation Therapy Superficial Siderosis Diagnosis, Chelation Therapy, and Deferiprone Researching on your own can be tricky. Superficial Siderosis is a serious diagnosis and the early research papers we found scared us to death. At first glance, the prognosis is horrendous so when we came across Pilot Safety Trial of Deferiprone in 10 Subjects With Superficial Siderosis-Michael Levy and Rafael Llinas ¹ we started looking into chelation as a therapeutic option. I printed out every reference I could find online about deferiprone. You or a family member are diagnosed with Superficial Siderosis, an unheard of medical condition. Everyone around you is scratching their head? Quick, run to the internet. Research papers are for physicians and researchers, not intended for use by lay people. I will tell you if you read enough of them you can begin to form a simple understanding of the data. We found the contact information for Dr. Michael Levy and carried our folder to our primary care physician. He studied everything for a few days and agreed with us. This brand new drug therapy was looking like our only choice. Deferiprone is a drug that only recently received approval in the U.S for treating Superficial Siderosis…

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