A Day In My Life

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My Life With Superficial Siderosis In 2016 I was diagnosed with Superficial Siderosis, a rare degenerative neurological condition which is caused by bleeding into the cerebrospinal fluid over a sustained period of time. I had a brain tumour removed from the back of my head in 1998 when I was eight years old; this is believed to be the cause of my Superficial Siderosis. The blood which leaks into the cerebrospinal fluid eventually overwhelms the body’s capability to remove it completely, leaving iron deposits on the surface of the brain and spine. The iron is toxic to the nerves of the central nervous system (CNS) and causes all sorts of mostly irreversible, damage. In 2018 I underwent an operation to seal the back of my head. It appears to have stopped the leakage of blood into my cerebrospinal fluid. The issue I have now, like most people with Superficial Siderosis,

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Combating Neural Damage

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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,

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The Decade that Was

End of a decade

The eve of a new year is a time for reflection. When I look back on our lives at the end of 2009 I’m reminded of how carefree we felt. A long wished for grandbaby joined our lives, dreams we would hike the Grand Canyon from rim to rim together, crawfish boils, Halloween parties, vacation trips, outdoor adventures camping, and hiking. Life was a nonstop activity. 2019 find us saying goodbye to the decade that was. A decade that began filled with promise is ending with changes so profound, if someone had given me a peek into the future, I would have never believed. It’s ironic this new decade begins with 2020. Our road ahead may be bumpy but we are determined to tackle this new year with 20-20 vision. 2019 is ending with so many new possibilities I am feeling hopeful once again. The vision for Living with Superficial

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Small Victories

Small Victories

I wrote an earlier piece on livingwithss.com detailing Gary’s intermittent swallowing problem. The Danger Of Dysphagia outlines some serious risks you may face if you choke while eating or drinking. Gary has been evaluated on a regular basis since that first baseline test. Steps In the Right Direction Earlier this summer, Gary had been referred to an endocrinologist for a lump he was feeling on the front of his neck. He had a past history of thyroid cysts so after X-rays and a scan they decided a biopsy was needed. Fast forward through the nail-biting, the lump was benign, but he was referred to an otolaryngologist closer to home for an opinion on surgery. Superficial siderosis can bring so many different symptoms the merry-go-round of specialists many of us visit can leave us exhausted and confused. When you stumble across that rare physician who is both caring and delivers an

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The Ripple Effect Of Chronic Illness

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The role of becoming a caregiver will alter your life. Most will tackle this task with laser focus. There are no second thoughts or questions. Someone we love- spouse, parent, or child is depending on us and we will not fail them. We become advocates for their healthcare, educating doctors about their rare disease or chronic condition. We help with paperwork, transportation, medication, and everyday care. Balancing life as a caregiver I took part in a rare disease caregivers survey last year and I was slightly taken aback by one of the questions concerning the “burden of care”. Never once had I considered what I do on a daily basis as a burden. Gary and I have been together through everything for the majority of our lives. If our roles were reversed he wouldn’t have hesitated. We’re lucky because some in our community don’t have this support. 69% of the

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Making Our Voice Heard

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Superficial siderosis is an ultra-rare condition with a small patient population. Our community knows all too well how difficult the journey to a diagnosis and then care. Too often hope dwindles leaving you to wonder how we can make our voice heard when our numbers are so small? A disease is classified as rare in the United States if there are fewer than 200,000 confirmed cases. Fewer than 2,000 and you join the exclusive ultra-rare class. Superficial siderosis is classified as ultra-rare — lucky us. There are 7,000 recognized rare diseases and conditions, and in the U.S. alone one in ten will be diagnosed. Global Genes estimates there are over 300 million people worldwide fighting a rare disease. When you think about it, our voices might be small but together as a rare disease advocate community we can’t be ignored. Once life on the doctor-go-round slowed, I found myself searching

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Surviving The Neurology Ride

Surviving The Neurology Ride

A visit to the neurologist often reminds me of stepping up to ride the tallest most twisty ride in the park. You start off slowly inching along, anticipating a thrilling experience with just a hint of fear. Then the bottom falls away and down you go. Sometimes the ride is exciting, everything you expected. Often the anticipation and hype don’t live up to your expectations. Do We Expect Too Much? Gary just finished with his last neurology visit for this year. We’ve had an outstanding year as far as finding solutions to some of his problems. We’re lucky to have been assigned an indulgent doctor who listens to my oft-times long-winded list of concerns. I am aware I can be a high maintenance caregiver at times, but without constant prompting, Gary often rambles through triage sessions. Why does it always feel like it’s never enough? Superficial siderosis is a slowly

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Focused Neuro-Otological Review of Superficial Siderosis of the Central Nervous System

Information Download

iSS can be associated with significant neurotologic and cerebellar morbidity; the recurrent SAH variant is frequently clinically symptomatic, has a shorter latency and greater neurotologic disability. In these cases, a thorough search and management of a covert source of bleeding may stop clinical progression. The frequency and clinical course of radiographic iSS after traumatic and post-aneurysmal SAH is largely unknown. Detection of radiographic iSS after trauma or aneurysm bleeding suggests that the slower clinical course could benefit from an effective intervention if it became available. The use of cochlear implants is a valid alternative with advanced hearing impairment.

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Countdown To The Last MRI

Our Chelation Journey

Our Chelation Journey This past week has seemed like a bad episode of Mission Impossible. We found ourselves desperately racing the clock trying to schedule what might be Gary’s last MRI of his brain. Strangely enough, this month marks the four year anniversary of his journey into chelation. If you remember from Summertime Neurology Blues during our trip to the neurologist, we found out the hematologist only ordered an MRI series of his back. No brain. We couldn’t fault the hematologist since ordering an MRI series for Superficial Siderosis isn’t something he would ordinarily be doing. There might have been some pressure on our end to get them done. Usually, neurology would order the scans but since we travel between three states and two VA hospital systems for care the lines between who is responsible for what is sometimes blurred. Our hematologist in Louisiana likes to remind us he is

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Decoding The Superficial Siderosis MRI

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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

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The Common Thread Searching For The Unexplained

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Superficial siderosis is ultra-rare. In fact, if you searched 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 often find myself thinking about this vey topic. Dr. Levy has said they don’t know why some people are overwhelmed by the blood infiltration. An unlucky spin of the wheel? I’m not medically educated but what if I brainstorm 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 at an older age due to the slow nature of

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Life Expectancy of Superficial Siderosis Patients

life expectancy

It’s human nature to wonder How long will I live? When you’ve been diagnosed with a life-changing ultra-rare illness like superficial siderosis thoughts like this can consume you. Superficial Siderosis Life Expectancy, four words make it intensely personal. The early impressions I found online were dark; there is no cure, the damage is irreversible and fatal. The word fatal is a definite attention grabber. The day we found out about superficial siderosis I read that one single sentence in a very early report and my heart dropped. Three Five years into this journey and we have first-hand insight with a much clearer understanding of the nature of this disease. If you review the possible combination of symptoms that may assault you, it should become clear while you may suffer body and soul, none are life-threatening in an immediate sense. There is no doubt the toll on your body is punishing. The

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Railroad Spike: Chronic Headache Pain

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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

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Testing, Testing, Hope

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The Whirlwind Test Tour We’ve been doing the whirlwind testing tour with Gary’s many specialists these past few weeks. Not only was it time for his quarterly neurology follow-up but we found ourselves eagerly waiting for the first MRI and hearing tests since Gary began Ferriprox this past year. Our health insurance network is being changed from a PPO to a HMO next year, 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 not one was in an HMO network. UPDATE: Every specialist but the neurologist joined the HMO network before the January deadline. 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

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Rethinking Cholesterol Medication

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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

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Whole New Ballgame: ReSound Linx²

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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

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