Orangeberry Newbie #82

Orangeberry Newbie #82

Sunday, 8th December 2013

Readers - Please note that listed prices are accurate at the time of posting and are subject to change. Availability and prices may differ from country to country.

Authors - If you would like your book(s) to be included in this weekly listing, please click HERE

The Big Book of Harry by Andrew R. Gabriel

Lulu Marketplace

Genre – Children’s

Rating – G

The Reported and The Girl Minus The Super Man! by S. C. Rhyne

Amazon US

Smashwords

Genre – Erotic

Rating – X

Love Me or Leave me by Kristine Mason

Amazon Kindle US

Genre – Contemporary Romance

Rating – PG16

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Orangeberry Newbie #77

Orangeberry Newbie #77

Sunday, 17th November 2013

Readers - Please note that listed prices are accurate at the time of posting and are subject to change. Availability and prices may differ from country to country.

Authors - If you would like your book(s) to be included in this weekly listing, please click HERE

Blind View Drive by Jerry Gufferd

Amazon Kindle US

Genre - Transgressive Fiction

Rating - R

They say that laughter is the best remedy in life—and if that truly is the case, then Blind View Drive is exactly what the doctor ordered. A hilarious psychedelic adventure full of sex, drugs, and karmic vendettas, it’s sure to leave you in stitches while making you acutely aware of the side effects of the human condition. 

The story centers on Chance, an existentially driven young man who seems to have a personal score to settle with the universe at large. His life is filled with a vengeance against societal norms, religion, and any other type of rule or boundary, and he uses everyone and everything around him to satisfy his twisted self-interests. 

Most shocking of all is Chance’s controversial view of justice. It allows him to take karma into his own hands to right the wrongs of his grandfather and carries readers into a darkly comedic world where they are forced to take a closer look at society’s moral and ethical constructs and to reassess the concepts of truth, decency, and fairness that they blindly accept as granted in their own lives. 

In the same distended vein as acclaimed writers Charles Bukowski, Hunter S. Thompson, and Chuck Palahniuk, maverick author Jerry Gufferd delivers a compelling work of transgressive fiction that steps outside of convention to vividly depict the absurdities of life, challenging readers to discover—and question—the fallacies of the world we live in. 

Based on extremely exaggerated events from Gufferd’s life, Blind View Drive will push and pull you in unexpected directions and speak to those raw, gritty parts of yourself that you’ve worked so hard to keep hidden.

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10 Years Down, A Lifetime More to Go by Pandora Poikilos

On 4th November 2003, my life changed. The signs that appeared a few months before weren't that easy to miss but when you aren't sure, your level of hope is so much higher than when you are officially diagnosed.

There was the weight gain and I'm not talking about a mere five pounds. Clothes I could wear today would mysteriously tighten overnight and I would need more loose fitting clothes. Then, the headaches came. The first one creeped up on me as I went for my usual evening jog. 

I don't feel so good. 

"It's probably something you ate," my friend said. We left it at that, I went home and had an early night. When morning came, the headache was so crippling I couldn't get out of bed. I eventually made it to a doctor. Migraine, he said. It's hereditary. 

It happens, no big deal. 

Two weeks later, I lost vision in my right eye. If you have ever seen the horror movie The Grudge and have an image of the ghost's eyes going all the way to the sides of her eyes, then yes, that's what I looked like. The irony of the situation was that my first car was being delivered that very week. 

So, not a migraine. 

The doctor ordered a CT scan. As it progressed, he was convinced I was having a stroke. He freaked. I freaked. We rushed to a hospital across town for an emergency MRI. 

No stroke, no tumour. Very cool.  

We spent the evening in Tesco's after making an appointment with an eye doctor. The scans were clear. Life was good at that moment. Days later, that eye appointment was the beginning of my winds of change. 

"Your eye nerve has ruptured." 

Yes, and my favourite colour is blue. 

I wasn't sure what this meant, why was he being so matter of fact? Why were there more people coming to verify what the first person saw? 

Okay, so? 

"Chances are this is the condition you have, Benign Intracranial Hypertension. You'll display all the signs of a tumour, but you do don't have a tumour. That's why it's called benign." 

A fake tumour? Just when you thought you've heard of everything. 

Nothing made sense after this. I was referred to a neurologist. It was in a hospital I hadn't heard of in a place I had no idea of getting to. I remember asking my ex, if he could take me. I couldn't see and it would be difficult. He said he had to work and that I could manage just fine because I still had one working eye. 

Not as easy as it seems, trust me. There is a reason why you have TWO eyes, and I assure you balance is one of them. 

Cue, French friend who strangely was more familiar with the roads than I ever was. Looking back, I remember so many things about this early morning trip that I didn't expect to.  I remember telling him not to be late, and he wasn't. I remember he was working on a University assignment about monkey brains. We ate egg sandwiches that didn't taste so right from the hospital cafeteria. I remember coming out from the neurologist's room after the diagnosis was confirmed, looking at him, not sure whether I should cry like a baby or pretend that everything was going to be okay. 

It's just an incurable brain condition, there's bound to be a cure very soon. 

We prepared for my first lumbar puncture. My friend hugged me, and told me everything was going to be okay. These are words, I've held on to all these years. I've held on to the friendship as well. He's married and lives on a different continent now but him being there for me, on this particular day of my life, is not something I am likely to forget. 

Once the lumbar puncture was finished and I was discharged, I grew up. My so-called first love ended and I was dumped within days because he wasn't expecting something so serious. 

Yea, I was totally expecting to have an incurable brain condition that would change my life. Who doesn't? 

Keeping a job was a bigger challenge. Between meds, concealing the condition, and shuttling myself for lumbar punctures, I struggled for seven years to find middle ground. Why conceal the condition? Would you really want to hire a person who has a brain condition you haven't really heard of? 

No, really, think about that for a second. Be honest. 

Google (it was a new tool back then) helped a lot during this time. I managed to compile a file about the condition and join online groups who helped me understand the condition better. Cue, Pixel, an old and dear friend who helped a long the way. Together the file of information grew. He's stuck around as well although we don't speak or meet as much as we would like to. 

I always had hope that some day they would find a cure in my lifetime. Going into my tenth year, I am not so sure anymore. Has the fire died? A little bit. I know breakthroughs are being made everyday, and maybe it will happen, but still. Maybe it won't. 

I had a VP shunt in September 2010. This happened at a time in my life when I thought I was broken beyond repair. I had no job. Living in a place I didn't want to. And was still no where near understanding the condition than when I had first heard about it. I was in therapy. I felt weak in every possible way. 

That was when I started this blog. This was my place to think, vent and share. In a little while, this blog will be crossing half a million hits. That's an achievement to me, no matter what anyone else says. 

If anything, at this point in my life, that is the biggest lesson an incurable brain condition has taught me. 

People will laugh and mock what you have achieved. Let them. If you've crossed the road for the first time by yourself, and you're proud. So be it.  When I first wrote my book, Excuse Me, I still get comments that the book didn't deserve to be published and people laugh at what a terrible writer I am.

Sure. I finished writing the book within six months of my first brain surgery. If you don't like it, it's okay. I achieved my lifelong dream, not yours.

Life with an incurable brain condition teaches you many things. You learn not to take things and people for granted. Every aspect of your life has a Plan B. And then a Plan C, and sometimes a Plan D. 

You learn that small minds and small issues are just that, small. They may hurt and sting for awhile but your every waking hour with an incurable brain condition is spent climbing mountains. You have bigger things on your mind than dealing with he said, she said, they said. 

Lastly, and I think this is the most important of all. The people who stick by your side and who love you despite the baldness, the pain, and the scars. Love them a bit more everyday. Not everyone is lucky to have people who stick around. 

They won't always understand. I get it. 

But when you're down, out, in pain and your memory is failing, you need that little bit of hope to keep you going. I met Peas a month before my first brain surgery. Imagine what a buzzkill that was. 

I'd really like to date you. I'm also having brain surgery, you cool with that? 

I don't think I would have made it through the past three years without him. He's been my rock when my legs felt mushy, my surfboard when the waves got rough, my eyes when I couldn't see and most of all he's my guiding light when this strange road of Incurable Brain Condition gets a little too dark. 

That's my 10 years. In a nutshell. Now here's to the rest of my life. You coming? 

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Rare Disease Day 2013 - Info & G!veaways

On this day, millions of patients and their families will share their stories to focus a spotlight on rare diseases as an important global public health concern.

“There are nearly 30 million Americans—and millions more around the world—affected by rare diseases,” said Peter L. Saltonstall, president and CEO of NORD.  “Everyone knows someone with a rare disease.  But, while many of these diseases are serious and lifelong, most have no treatment and many are not even being studied by researchers.  This leaves patients and families without hope for a better future.”

On Rare Disease Day, people with rare diseases around the world promote awareness of the challenges of living with a rare disease.  The global theme for 2013 is “Rare Disorders Without Borders.”  

World Rare Disease Day was launched in Europe in 2008 and last year was observed in more than 60 nations.  It is always observed on the last day of February.  On that day, patients and patient organizations will post stories, videos and blogs online and host events to raise awareness of these diseases, which are often called “orphans”.

This year, the observance has special significance in the U.S. because 1983 is also the 30^th anniversary of the Orphan Drug Act, which provides incentives to encourage companies to develop treatments for rare diseases, and of NORD, which was established by patient advocates in 1983.

 “More than half of the people who have rare diseases are children,” Saltonstall said.  “Challenges faced by patients and their families include delayed diagnosis, few treatment options, and difficulty finding medical experts.  Many rare diseases have no approved treatment.  Insurance may not cover treatments that aren’t approved.  Also, treatments for rare diseases tend to be more expensive than those for common diseases.”

In 1983, the Orphan Drug Act was passed by Congress to create financial incentives for companies to develop treatments for rare diseases.  Since then, more than 400 orphan drugs and biologics have been approved by the Food and Drug Administration (FDA).  It is estimated that approximately 15 million Americans benefit from these products, but that still leaves millions more with diseases for which there is no approved treatment.

For more information about Rare Disease Day activities in the U.S., go to www.rarediseaseday.us.  For information about global activities, go to www.rarediseaseday.org).

*******

In conjunction with Rare Disease Day, Pandora Poikilos' books will be free from 28th February 2013 till 15th March 2013. To download your free copy, please click on the links below.

Excuse Me, My Brains Have Stepped Out is free via KDP Select (Kindle) from now till 3rd March.

Dora's Essentials - Examining Anxiety is free via Smashwords from now till 15th March.

Genetically Modified Foods vs. Sustainability is free via Smashwords from now till 15th March.

Pandora's Reading Room (Short Story compilation) is free via Smashwords from now till 15th March.

a Rafflecopter giveaway

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I Have A Brain Condition, What's Your Excuse?

By Pandora Poikilos

In case you haven't heard, September 2011 is awareness month for Intracranial Hypertension (IH). I was diagnosed with this rare neurological disorder in November 2003. In the past week with every opportunity I got, I shared this message on Twitter - RT If you hope for a cure for "#RareDisease #IntracranialHypertension #Cancer #BreastCancer ... #IHAwareness #IHope4ACure" Someone was annoyed enough to tell me that if I was diagnosed with a rare condition that I had to accept it and move on. Hoping for a cure wasn't going to change my situation and I wasn't being realistic. Now, there's a shocker. So, let's recap. 

What are the facts about IH?  Intracranial hypertension is a neurological disorder which literally means that the pressure of cerebrospinal fluid (CSF) within the skull is too high. “Intracranial” means “within the skull.” “Hypertension” means “high fluid pressure.” Main symptoms are headache, nausea and vomiting, double vision and other symptoms. If untreated, it may lead to swelling of the optic disc in the eye, which can progress to vision loss. There is no known cause. There is no cure. (Sourced from IHRF.org)

Pause. 

Forget the facts.  

How does IH make me feel? Most days I feel double my age. I have a VP Shunt in my head, a tube that runs from my brain to my body. I feel scared. I feel cursed. And I feel abnormal. On some days, I tear around like a raging bull doing as much as I can. Others see it as a good day, determination they call it. I see it as a mask to do as much as I can before a bad day catches up with me and I can't do anything but lie in bed and wish away this dreaded condition. Some days it's easy to tell people how I feel and on other days I feel like a broken tape recorder.

Pause. 

Forget the facts. Forget how IH makes me feel. 

Now, look in the mirror. Yes, you. Take a long hard look at your reflection. Imagine pain eating away in places you never realised made so much difference. IH is treated with lumbar punctures. Your lumbar is at the small of your back. A needle is inserted into your lumbar to drain your excess spinal fluid. It hurts, a lot. I underwent lumbar punctures for seven years. I stopped counting after 10 times.  Then, on top of that pain - imagine disorientation. You have to hold on tight to your memory, you never know when the pressure gets so high you can't remember daily events. Imagine, losing your hair. First, to medicines and then to brain surgery. 

Imagine feeling helpless. You cannot see what's eating at you. But you know it's there, everyday. This invisible force tearing you up from the inside out. You feel blackness surround you, your eyes fail you. You need help with simple everyday tasks. Imagine, the pain you feel when you jam your hand between a door. The pain from brain surgery is easily ten times worse but a VP Shunt is a choice between sight and sanity or ongoing crippling symptoms driving you mad. You stop looking in the mirror. When all this stops. Take a deep breath. 

Then it starts, all over again. IH is a lifelong condition. There is no cure. How would you feel?

Pause. Think about it. 

Do you fight? Do you say and do nothing? Do you hope? 

In the meantime, I say again and again until the day it becomes real ...  
I HOPE FOR A CURE (#IHope4ACure). Love and light, always. 

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What Dreams Are Made Of

Some weeks ago, I posted that I was Moving On To The Next Chapter and promised to keep everyone updated on what this new chapter was. Well, here it is ... my very own fiction novel - Excuse Me, My Brains Have Stepped Out. Take a sneak preview of some of the chapters, here.

Edited and compiled from some of the blog posts made at Peace from Pieces with many more newly added chapters, this comes after a tremendously long trail of rejections, one VP shunt surgery, seven years of lumbar punctures, a very rare medical condition and the realisation that comes with getting your priorities in order. To say, I am thrilled to bits is truly an understatement. 

For now, there are no critics who can take away this moment from me and no pin that is going to burst this bubble. I have arrived to the point where I know what dreams are made of, I am now a published author! What next, hmmmm, bestseller list maybe? Second novel and more articles, for sure. 

In the meantime, to the publisher (Heart Press) who said yes, to the editor (Sonia Rumzi) who has poured much time and effort into nitpicking the raw material to make it an even better read for everyone, to the readers of this blog who have made this possible and to the family and friends who cheered me on, my utmost heartfelt appreciation to each and everyone of you.

And still, my cup runneth over.

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My Grief, My God

By Pandora Poikilos

Grief is a small word that carries with it extensive emotions we are all familiar with. If we have not been introduced to grief then we haven't yet grown. The past two days have been a little rough, staying positive and keeping my mind occupied with daily tasks has been intruded with a little lump on my back. It bothers me that the lump comes as though it is in a straight invisible line from the surgical wound (from my VP shunt) that I have on my chest and it bothers me even more that it hurts, a lot. It has reintroduced some of the pain I used to feel just after surgery and yet, after such a major surgery, it is a waiting game to see when and how the lump can be removed. Yes, where is all that positive thinking when you need it the most?

A small part of me feels like grief is trying to slide itself through my window again, no matter how tightly I have shut it. That, this lump may mean something bigger and my biggest fear of having a second shunt surgery may materialise. Then, there's this other bit inside me that insists on having faith in God and in the belief that everything will be fine. How can it can get any worse from having brain surgery? A cartwheel of emotions indeed.

Am I fearful of grief? A little. I've had one too many long walks with it to know grief walks hand in hand with loss. It leads you on a path of self-discovery and it builds your character but with so much that has happened, I fear that I may not be able to handle anymore. I met grief at a very young age. At the age of three, I lost one parent to a bad heart and another to indifference. I grew up being repeatedly told that I was never good enough, that I would never amount to anything more than a street sweeper, that the mistakes I made were so grievous, I wasn't worth standing up for. Everybody else was worth listening to, I wasn't. On more than one occasion, I've been forgotten from a piano class or after school activity and this often meant waiting alone, hoping that I wasn't that invisible. So, yes, grief was a regular bedside companion the many nights I cried myself to sleep thinking one day, all this will be just a dream, that I too, would have a Daddy Warbucks somewhere out there.

When I was six, grief had me very confused. If anything, grief taught me grown ups make mistakes too and nobody, and I mean nobody is perfect. I had something precious taken from me. Something that a million dollars would never be able to restore. I would feel guilt, shame and blame for a long time before realising that the sexual impropriety I had experienced was not my fault. It would make me more aware of people who think they can make you feel small just so they can gloat in the sensation of awe they have, for themselves. And it would teach me that self-pity is a dish best served with stupidity because you will never move yourself forward emotionally and mentally.

Grief came and sat by my side when at the brink of receiving a much awaited internship, I was diagnosed with a rare and incurable condition, Pseudotumor Cerebri. Half of it seemed like a joke. And the other half, was a mental and emotional state I could not cope with. My first lumbar puncture. My first sense of losing control. There were days when I would rather sit still just so I wouldn't have to show people that I couldn't see from my right eye. The days when my memory would get so mixed up, I have had to accustom myself to writing things down. The excruciating seven years of lumbar punctures that had me thinking, if this is what it meant to move forward in medicine, somebody, somewhere must be slacking off their research. Grief enveloped me when my first, real four year relationship ended because the condition was too difficult for him. Talk about transference.

Holding the pieces together and trying to put up a strong front often meant more pieces falling apart and grief being a full time companion. Grief lingered through the betrayals of friends who stayed to gain for their benefit and left when the benefits wore out. It was a much needed wake up call I do not wish on anyone but hope that each of us will learn, it is true, the best of friends will stay when the rest of the world walks out. Grief was a constant bystander in so many situations when I've tried to explain my medical condition and was instead greeted with, "it's your brain, so you must be crazy."

Even as I prayed for a non-eventful VP Shunt surgery, grief held my hand ever so tightly when I thought about how monstrous I must look and how I wished I hadn't taken so many little things in my life for granted. A very close friend once told me, that if I've had to deal with all this, there must be some grand reward tucked away somewhere. That God can't allow so much to happen and not bless me with anything good.

It took me awhile but I realised the reward wasn't tucked away anywhere. It was right in front of me. My blessings are outnumbered, in the few people that are living proof that there is such a thing as unconditional love. In the fact, that my writing journey is far from over and in the simple knowledge of knowing, there are more blessings to come.

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Can You Carry Me?

By Pandora Poikilos

It has been exactly 99 days since I've had my surgery. For all the moments when I tell myself "seize the day and life is normal", I am given a few more small reminders that say, "Easy now, it's not so normal and not so much seizing." Peas and I have accustomed to calling my not-so-crowning glory, Stubbly and are now used to the days when Stubbly rules all. For instance, some days when getting out of bed is the biggest chore or when I feel so rotten that I need to keep telling myself all this is just a passing cloud that I'll be laughing at 10 years from now.

If you're up to it, you're up to it
This is probably the best advice ever given, by my neurosurgeon. (Well, for someone operating on my brain, it is good to know he has a wise line or two to impart.) Before the surgery, I had a long list of "when can I do this" and "when will it be okay to do that", which ranged from getting back to my computer, reading, exercising, laundry and so on. With continual stress being put on "not carrying heavy things and being careful of not stretching my right arm" as that's where the shunt tube runs down, he said for everything else, if you're up to it, you're up to it. You're the only one who knows. That is so true. I think on some days people who know me must think I'm utterly mad or just plain lazy to be lying down as much I do but try having a head wound that's healing, gives you a vibrating feeling when cold and has you in so much pain you can't bear to stand. So yes, now my rule is very simple. I start out my day with a set of things I want and need to do. But I also tell myself, if Stubbly gets in the way and I can't finish this list, the world won't end because of me.

The world really won't end because of me
A very close friend told me once, "Don't give yourself so much credit. You're not that famous and you're not that well known". Of course this was when a horribly bad untrue rumour was being spread about me and he came to the rescue of my very bruised heart and ego. But it's true. Abraham Lincoln died and the world moved on. Gandhi died and the world moved on. Princess Diana died and the world moved on. We all do some really great things and still we all make some really grave mistakes. We are not perfect. I am no exception to this rule. Yes, maybe (and hopefully), I'll one day become a well-known writer or even finally have my book published but I can only do what I can, the best I can before I too must move on and yes, the world will move on. Life's like that.

You just need to accept it
Like everyone else, I had materialistic dreams too. A well-known, well-traversed journalist or a high flying entrepreneur who would have her own kids, live a comfortable lifestyle and have enough for luxuries to look the part. Instead, I'm very bald, I have a very carefully planned expenditure plan that the slightest overspending might trigger a tsunami effect and I have a tube running from my brain to my stomach. But I have the ability to make my thoughts known by my writing, I have the opportunity to source out more writing jobs and more importantly, I have love. People who understand me, people who don't push me in the ways they want to go. People who accept and rarely expect. Do I have everything I want? Not at all. Do I have everything I need? Absolutely. I know things won't be easy. There'll be more days when I'm not sure what's happening with my body. Having my own child will also mean more monitoring by my neuro than my gynecologist. But if I do not accept these unique differences in my life and make the best out of them, what else is there to do?

People will say what they want to say
I read this line somewhere, "Funny thing about people, they'll believe that God is dead and Elvis is alive". Yes, somehow and I'm no professional but some people get their wires crossed badly. They'll believe something so silly for the sake of satisfying their comfort zone than to open their mind just a wee bit more and question what has just been said. When I was first diagnosed with PTC and the word got out (as it always conveniently will) people used to ask me "when are you going to die". Even when blog readers ask, my answer stays the same, "we are all going to die". It's like writing a sentence - the language, alphabets, length and meaning might be different but it'll have to end in a full stop at some point. People will have a million ways of looking at things, when they haven't gone through it. You can't please everyone. Really. Love the ones who love you, move on with everyone else.

Appreciate the pain you have
Now, on some days Stubbly gets so bad that I almost feel like I'm in a scene from Gulliver's Travels where my head is tied down with very thick rope and they are little people running all round my head banging and constructing away. The pain I felt as I came out of surgery and the pain I feel on the bad days I currently have, I do not expect everyone to understand nor do I expect people to run around my whims and fancies, although some quiet would be a great help. However, when someone says, "I have a bad headache" I may not snicker but then again, I may not be the best one to seek sympathy from either. It gets me very hot and bothered when I see some people who have controllable medical conditions like certain types of diabetes carry on like nothing is wrong because give me one day, just one, when there is a way to control CSF (brain) fluid or to know what triggers its increase, I'll try it all, with a smile on my face no matter how painful it is. Yes, I may seem very selfish when I cannot relate to pain that is much less than what I am going through but I also know that it is best to appreciate the pain that you do have because it might just be worse.

Don't take life for granted
I might be exaggerating but this phrase has easily been said a million times over. In different situations, in different languages, by different people from different generations and yet, we each only realise the value of this lesson when we are ourselves are faced with a shattering loss that life crudely offers us. I could go on but let me just allow you one last scenario about how precious life is. Imagine a bright eyed five year old playing with you and he circles your neck with grubby cheesecake coated fingers, rubs your nose with his and says, "Can you carry me?" Your heart breaks, more than once when you think of how you'll have to say no to him. That he's just a little too heavy for the tube in your body to bear, that you've just had a VP Shunt, that this is a major surgery, yes, there'll be some intense explaining that needs to be done. He'll either not understand what you tell him or he'll forget in less than five minutes and run off on his next conquest and come running back to you with another hug and even more love. He's a child with so much to give and a very short memory but how will it make you feel?

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Thank You!

When Peace from Pieces started out in May 2010, the theme was always set on inspiration and was meant to be a resource to keep me writing while I waded through some very deep waters.

On 24 July, when it recorded 524 hits, I thought, "when will I ever be able to get it to move along and have blog comments and hits like other blogs I had seen". Ah well, 20,000 hits later I am thankful for all of you who have taken the time to read, comment and lend your support to this blog.

The last significant milestone we hit was on 22 November with 15,000 hits.

But a lot has happened since then.

There's a new e-publication in town - Essentials For Your Dream Wedding

There's a traveler who is sharing his thoughts, dishing out the real deal - Rough Guy'd Travels

Then, there's a cause that needs much support and only 2 minutes (or less) of your time - A Pledge For Responsible Social Media Users

There's the upcoming event that needs much more blabbing and sharing - Rare Disease Day 2011

and of course, to the Netizens who blog and the world who reads them, we listed our first 100 Blog-A-Licious Blogs.

Here's to more thoughts being jotted down, more inspirations shared and of course more hits, views, reads and comments to come. I thank you all very, very much! Happy holidays!

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Rare Disease Day 2011

Millions Around World To Observe Rare Disease Day

The last day of February has been designated as World Rare Disease Day to call attention to rare diseases, which affect nearly 30 million Americans and countless others around the world, as an important global public health concern.

“People with rare diseases remain a medically underserved population in every country,” said Peter L. Saltonstall, president and CEO of the National Organization for Rare Disorders (NORD), which is sponsoring Rare Disease Day in the U.S. “This day will bring together patients and families around the globe who are dealing with some very challenging issues.”

The coalition, being coordinated by NORD, includes patient organizations, professional medical societies, government agencies, medical researchers, and pharmaceutical and biotechnology companies.

Rare Disease Day 2011 activities in the U.S. will include creating a “video encyclopedia” of two-minute videos about many of the nearly 7,000 rare diseases. Also, patients and others will help NORD create a database of physician experts on various rare diseases. In addition, state and municipal proclamations in honor of the day will be sought, and there will be a drive to enlist support for a new Rare and Neglected Diseases Caucus in the U.S. Congress.

A rare disease is one that affects fewer than 200,000 Americans. According to the National Institutes of Health (NIH), there are nearly 7,000 such diseases affecting nearly 30 million Americans.

“More than half of the people who have rare diseases are children,” Saltonstall said. “Challenges faced by patients and their families include delay in getting an accurate diagnosis, few treatment options, and difficulty finding medical experts. Many rare diseases have no approved treatment. Insurance may not cover treatments that aren’t approved. Also, treatments for rare diseases tend to be more expensive than those for common diseases.”

In 1983, the Orphan Drug Act was passed by Congress to create financial incentives for companies to develop treatments for rare diseases. Since then, more than 350 “orphan” (for rare diseases) drugs and biologics have been approved by the U.S. Food and Drug Administration (FDA). FDA estimates that from 11 to 14 million Americans benefit from these products, but that still leaves more than 15 million Americans with diseases for which there is no approved treatment.

This will be the fourth annual Rare Disease Day. The concept was launched in 2008 by EURORDIS, NORD’s counterpart in Europe. Last year, EURORDIS asked NORD to host Rare Disease Day in the U.S. In 2010, Rare Disease Day was observed in 46 nations around the world. The observance is always on the last day of February.

For more information about Rare Disease Day go to www.rarediseaseday.org

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VP Shunt / Rare Disease Day

I take a deep breath as I write this post.

I started Monday New.s after the quote from Eleanor Roosevelt, "Do one thing everyday that scares you." in the spirit of trying one completely new thing every Monday, whether it scared me or not, as long as I learned something from it.

At the time, I knew my surgery was in place and I knew it would be life altering. But nothing prepared me for what was to come. I will write more about it, soon but in the meantime, here's a link to the little gadget that has me going, a VP Shunt and that qualifies for my Monday New.s from 20 September to 4 October 2010. Nothing I would recommend you to try, but if you've had to and if you'd like to share, please go right ahead.

On this note, please also pass the word around or lend your support about Rare Disease Day - Alone we are rare. Together we are strong.

February 28, 2011
http://www.facebook.com/RareDiseaseDay.US

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Pseudotumor Cerebri (Benign Intracranial Hypertension)

Sourced from Mayo Clinic

Pseudotumor cerebri occurs when the pressure inside your skull (intracranial pressure) increases for no obvious reason. Symptoms mimic those of a brain tumor, but no tumor is present. Pseudotumor cerebri can occur in children and adults, but it's most common in obese women of childbearing age.

When no underlying cause for the increased intracranial pressure can be discovered, pseudotumor cerebri may also be called idiopathic intracranial hypertension.

The increased intracranial pressure associated with pseudotumor cerebri can cause swelling of the optic nerve and result in vision loss. Medications often can reduce this pressure, but in some cases, surgery is necessary.

Pseudotumor cerebri symptoms may include
- Moderate to severe headaches that may originate behind your eyes, wake you from sleep and worsen with eye movement
- Ringing in the ears that pulses in time with your heartbeat
- Nausea, vomiting or dizziness
- Blurred or dimmed vision
- Brief episodes of blindness, lasting only a few seconds and affecting one or both eyes
- Difficulty seeing to the side
- Double vision

The exact cause of pseudotumor cerebri in most individuals is unknown, but it may be linked to an excess amount of cerebrospinal fluid within the bony confines of your skull.

Your brain and spinal cord are surrounded by cerebrospinal fluid, which acts like a cushion to protect these vital tissues from injury. This fluid is produced in the brain and eventually is absorbed into the bloodstream. The increased intracranial pressure of pseudotumor cerebri may be a result of a problem in this absorption process.

In general, your intracranial pressure increases when the contents of your skull exceed its capacity. For example, a brain tumor typically increases your intracranial pressure because there's no room for it. The same thing happens if your brain swells or if you have too much cerebrospinal fluid.

The following factors have been associated with pseudotumor cerebri
Obesity. Pseudotumor cerebri occurs in about one person per 100,000 in the general public. Obese women under the age of 44 are nearly 20 times more likely to develop the disorder.

Medications. Substances that have been linked to pseudotumor cerebri include:
- Lithium
- Oral contraceptives
- Tetracycline
- Steroids or discontinuation of steroids
- Excess vitamin A

Health problems. The following conditions and diseases have been linked to pseudotumor cerebri
- Head injury
- Kidney disease
- Lupus
- Lyme disease
- Mononucleosis
- Underactive parathyroid glands

As many as 10 percent of the people with pseudotumor cerebri experience progressively worsening vision and may eventually become blind. Even if your symptoms have resolved, a recurrence can occur — months or even years later.

While you might first discuss your symptoms with your family doctor, he or she may refer you to a neurologist or an eye specialist for further evaluation.

What you can do
Because appointments can be brief, plan ahead and write a list that includes:
- Detailed descriptions of your symptoms
- All the medications and dietary supplements you take
- Questions you want to ask the doctor
- What to expect from your doctor

In addition to a physical exam, your doctor may also check your neurological health by testing your:
- Reflexes
- Muscle strength
- Muscle tone
- Senses of touch and sight
- Coordination
- Balance

Eye exams
If pseudotumor cerebri is suspected, a doctor specializing in eye disorders will look for a distinctive type of swelling — called papilledema — in the back of your eye. You will also undergo a visual fields test to see if there are any blind spots in your vision.

Brain imaging
CT or MRI scans can rule out other problems that can cause similar symptoms, such as brain tumors and blood clots.

Spinal tap (lumbar puncture)
A lumbar puncture — which involves inserting a needle between two vertebrae in your lower back — can determine how high the pressure is inside your skull.

Pseudotumor cerebri treatment typically begins with medications to control the symptoms. Weight loss is recommended for obese individuals. If your vision worsens, surgery to reduce the pressure around your optic nerve or to decrease the intracranial pressure may be necessary.

Medications
- Glaucoma drugs. One of the first drugs usually tried is acetazolamide (Diamox), a glaucoma drug that reduces the production of cerebrospinal fluid by at least 50 percent. Possible side effects include stomach upset; fatigue; tingling of fingers, toes and mouth; and kidney stones.

- Diuretics. If acetazolamide alone isn't effective, it's sometimes combined with furosemide, a potent diuretic that reduces fluid retention by increasing urine output.

- Migraine medications. Medications typically prescribed to relieve migraines can sometimes ease the severe headaches that often accompany pseudotumor cerebri.

Surgery
- Optic nerve sheath fenestration. This procedure cuts a window into the membrane that surrounds the optic nerve. This allows excess cerebrospinal fluid to escape. Vision stabilizes or improves in more than 85 percent of cases. Most people who have this procedure done on one eye notice a benefit for both eyes. However, this surgery isn't always successful and may even increase vision problems.

- Spinal fluid shunt. Another type of surgery inserts a long, thin tube — called a shunt — into your brain or lower spine to help drain away excess cerebrospinal fluid. The tubing is burrowed under your skin to your abdomen, where the shunt discharges the excess fluid.

Symptoms improve for more than 80 percent of the people who undergo this procedure. But shunts can become clogged and often require additional surgeries to keep them working properly.

Complications can include low-pressure headaches and infections.

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