Author: sug_97

Walnut-Crusted Chicken Salad with Blueberry Balsamic Vinaigrette – “Anti-Inflammatory”

Published: 28 March 2019
Written by sug_97
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Categories: Health & Fitness Resources, Recipes

 

INGREDIENTS:

Blueberry Balsamic Vinaigrette:

    • ½ cup fresh blueberries
    • ¼ cup aged balsamic vinegar
    • 2 teaspoons honey
    • ¼ cup avocado oil or light olive oil
    • ¼ tsp sea salt
    • ¼ tsp ground black pepper
    • ¼ teaspoon onion powder
    • ½ tablespoon whole grain Dijon mustard

Walnut-Crusted Chicken:

    • 2 cups walnuts
    • ½ tsp onion powder
    • ½ tsp garlic powder
    • ½ tsp sea salt
    • ½ tsp ground black pepper
    • 2 eggs, whisked with 1 T water
    • 2 large boneless, skinless chicken breasts
    • 2 TBSP olive, coconut or avocado oil

Salad:

    • 2 cups kale, washed, ribs removed, and roughly chopped
    • 4 cups 50/50 Spinach/Spring Mix salad greens
    • 8 radishes, thinly sliced
    • ½ cucumber, sliced into half moons
    • ¼ red onion, thinly sliced
    • ½ cup fresh blueberries
    • 1 medium carrot, shaved with a vegetable peeler or grated with a cheese grater
    • 1 medium avocado, sliced

DIRECTIONS:

In a blender, combine all dressing ingredients. Blend on high speed about 20 seconds, or until dressing is smooth and emulsified. Taste dressing and add more honey if it is too tart. The sweetness may vary depending upon the ripeness of your blueberries and the quality of your balsamic vinegar. Set aside at room temperature.

Place walnuts in a food processor and pulse until walnuts are ground, resembling course breadcrumbs. Avoid the temptation to leave the food processor on high, as this can result in a nut butter. Mix ground walnuts, onion and garlic powders, salt and pepper in a medium bowl or pie plate. In a separate medium bowl or pie plate, whisk together eggs and water.

Cut each chicken breast in half by thickness by laying the breast on a cutting board and pressing down on it with the palm of one hand. Using a chef’s knife in your other hand, hold the blade parallel to the cutting board and slice through the thickness of the breast, using a sawing motion. Repeat with the other chicken breast. You should now have four fairly thin chicken cutlets. Pat them dry with a paper towel.

Heat your chosen cooking oil on medium heat in a large, nonstick skillet. If your skillet is not large enough to accommodate all four cutlets, use half the oil at a time and work in two batches. Dip each cutlet in the egg wash, then the walnut mixture, pressing the walnuts onto the chicken. Place chicken in heated pan and cook approximately 3-4 minutes on each side, until walnut coating is deep golden brown and chicken is cooked through. Remove to a CLEAN cutting board to rest. Allow to rest and cool for five minutes, then cut into ½-inch slices.

While chicken is resting, assemble your salad, by combining all the ingredients except avocado in a large bowl. Divide the salad into four bowls and top each bowl with a sliced walnut-crusted chicken cutlet and ¼ of the sliced avocado. Drizzle prepared vinaigrette over chicken and salad and serve immediately. Leftover dressing can be stored in the refrigerator, but will be best if you let it return to room temperature before serving.

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When A Chiari Woman Passes Away

Published: 21 February 2019
Written by sug_97
1 Comment
Categories: Expressive Writings, The Fight of a Chiari Angel, To Family/Friends

When a Chiari woman passes away it changes so much for so many.

It leaves a hole in the hearts of the Chiari community because, even as dysfunctional as we are sometimes, we know we’re all in this together!

  • We know what it’s like to have conditions that so few understand, including our doctors.
  • We know how humiliating it is to watch our bodies change and our muscles deteriorate as we fight just to hold up our heads, even for just a few hours a day.
  • We know what it’s like to have our symptoms and pain consistently dismissed as psychosomatic.
  • We know what it’s like for doctors to say how easy the surgery will be only to find out that they had no clue of what they were talking about.
  • We know the heartbreak we feel when those we love the most choose to believe our doctors instead of us.
  • We know what it’s like to be told that the surgery cured us, while everything in our heads and necks seemingly rebel in disagreement.
  • We know what it’s like to have to fight for every aspect of our treatment, from imaging to specialist, and still have help denied to us.
  • We know what it’s like to have to find strength through the pain each day and still try and carry on the best that we can for those that we love so much. We long more than anything to get back to who and what we once were. To run and jump and enjoy life with those we love most, but the pain is too overwhelming. The truth is that those times that we did try left us in even more pain; so, we are forced to learn to choose what we do wisely.
  • We know the genetic nature of what we have, even if our doctors don’t, and we all long to figure it all out and fix this very broken medical system that relies on information that is centuries old, before our children and their children are forced to face what we face.
  • We know what it’s like to live in fear of leaving all those we love, as they are our reason for living.

It leaves a hole in the hearts of her family that should be respected.

To them she is so much more than a Chiari Woman, she is my wife, my mother, my daughter, my grandma, my sister, my aunt, and the love of my life.

Chiari didn’t just rob their loved one, it’s robbed the entire family for years.

They too have longed for what was and often cried behind closed doors for all that likely would never again be.

Her husband has had days where he was so petrified at the thought of losing her. Sometimes that fear manifested as anger and frustration, but it wasn’t her that he was really mad at; He was mad at the Chiari that was taking his wife from him. He regretted those words from the very minute that he gazed upon her eyes as she heard them. He couldn’t make her forget those words, they pierced her soul too deeply, so he internally committed to just try and show her why he loves her. Despite his frustrations with the situation, he admired this amazingly strong woman who was facing more pain than he could ever understand. He’s cried out to God privately for this soul mate that he committed to so many years ago. He feared losing her and he did not know how he could ever hold everything together like she did, but he had to try because there was so much was on his shoulders. He never talked much about the weight he carried in it all, because he knew what she was facing was already more than she could handle. So, he learned to grieve as silently as he could – to understand what he could, to empathize as he could, to remain as strong for her and the family as best as he could.

Her children have learned to grieve in silence as well. They learned to face life and all the obstacles they faced without making waves at home. How could they? They saw the pain that flared when things had stressed her in the past. They didn’t want to cause that again. They longed having the mom that they used to have, the mom with the strength to climb every mountain with them, the mom that made every challenge in life seem conquerable. They admired her strength and never understood how they could admire so much in her yet hate the fact that she wasn’t always there for them anymore. Even when she so desperately tried to be there the brain fog often dominated and took over the conversation entirely, this conversation that she probably wouldn’t remember for long. They resented her for it but knew that it wasn’t her fault. Like everyone else that loved her the most, they said so many things that they regretted. They knew that they often came across like they hated her, but the real truth was there was no one in the world that they longed for more. They’ve always hated seeing her in pain, yet her pain served as a constant reminder of the future that they feared in their future. They remember when she told them about the hereditary nature of the genes passed on to them, with tears in her eyes. Would they have what she had? Would they become as symptomatic? Would they have the courage to fight it as valiantly as she did? Should they have children one day? The reasons for concern were endless.

Depending on when she became symptomatic, her parents have likely spent endless days and nights in hospitals and emergency rooms. They’ve spent years learning all they could and even learned to recite her diagnoses with detailed explanations because they’d became so accustomed to these conditions that so few doctors could pronounce, much less treat. For years they made sure to always have her complete medical chart in hand with official documentation, in case they were challenged by a medical professional or anyone dared to call their daughter “a drug seeker” or “psychosomatic.” They spent countless hours trying to dull the tortuous pain they saw her go through, and spent many more hours researching creative home remedies to give her a sense of relief, even if it was only momentary. There were times they prayed for God to just go ahead and take her, so she wouldn’t be in anymore pain; and then felt guilty for even thinking it. Then one day the screaming stopped, the pain was gone, but with it so went their daughter.

As Chiarians, we all deal with thoughts of death more than “normal” people and “normal” families. We go from surgery to surgery knowing that this might be the surgery that ends our fight altogether, yet we hold on to hope that it will be the surgery that helps us to be all that we’ve yearned to be once again. Our community has found strength in our unity. As we remember those we’ve lost and face our fears about our morbidity, let us continue to unite to change this for us and our future generations that we love so much.

*This article is dedicated to all the Chiari Angels and their families, as well as those that are still fighting the fight. Hold your families close; forgive quickly and as often as necessary. We never know how much time we have and we usually never really know how much we’re truly loved until it’s too late.

**Note: This article was not to leave out all of our valiant Chiari brothers fighting the fight, it was just easier to write from one point of view.

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My Husband, My Caretaker, My Hero

Published: 14 February 2019
Written by sug_97
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Categories: Expressive Writings, Inspirational, To Family/Friends

Like any marriage, we’ve had our rocky moments. We’ve both showed our ugly sides more than we like to admit. I’m not sure when he changed, but somehow along the way in our 27 years of marriage, my husband morphed into this amazing man who is EXACTLY what I need in every way!

My husband doesn’t have the same responsibilities that most husbands have. He works full-time to provide for his family and does more than his fair share of household chores. But my husband has a wife with several chronic conditions. He has a wife that has been in some level of pain almost every day for over 18 years! He’s seen me through ten surgeries since we’ve been married, eight of them being in a five-year period. That is enough to exhaust the strongest of men, but exhausted or not he has stood by my side through all of it.

I first became symptomatic following a car accident. We were both 29 with three young kids (ages 2, 5 and 8). I was initially diagnosed with double whiplash, but when that failed to get better, I was left with no reason for why I couldn’t hold up my head. It felt like my neck lacked the strength to hold up my head for more than an hour at a time and on some days, not even that. To add insult to injury, I went without a diagnosis for over a decade. It couldn’t have been easy to stay by my side when all the doctors were saying that they couldn’t find anything wrong with me.

Over our decade without answers, I continued to decline. I started having severe cognitive decline and memory loss. I’d have periods of time where I lost my ability to walk and use fine motor skills (so I couldn’t write or pick up small things). He helped me walk to my classes and even helped me do my homework when I couldn’t write. He didn’t have the answers, but he knew that I desperately needed them, and he was determined to stay by my side even though by doing so it was putting an unfair burden on him. He didn’t have the help-meet that he needed in life. He just had this sick, scared wife, with no means to any end to her suffering.

When I was finally diagnosed, the choice for decompression was an easy one. It was 2010 and I was now 39 years old. I had lost 10 years and the possibility of getting my life back and engaging as a wife and mother again was something that I couldn’t pass up. We never expected this “easy surgery” to take the turn that it did. We knew that there was a chance that it would be unsuccessful at relieving all of my symptoms, but never in a million years did we think that decompression would open Pandora’s Box on my need for surgeries. From 2012-2015 I needed seven additional surgeries. Each surgery had its own recovery and complications. And with each additional surgery, I became increasingly dependent on my husband for help, but not once did he complain. This was affecting his life too, but not once did he concentrate on that. Not once did he think of it as being unfair to him to have to care for me, even though it really was. I’m now covered with scars on my left side from my neck to my groin, one night he raised my pajama shirt and gazed intensely upon them. He started kissing them. When he got to the scars covering my stomach, I pushed him away and with tears in my eyes, I told him that I wasn’t comfortable with him touching me there. He looked me in the eyes and told me that he didn’t see me the way that I see myself. He said that when he sees my scars, he’s reminded of all that I’ve had to fight through and what a strong woman he’s married to. “I wish that you could see yourself the way that I see you,” he exclaimed. All my insecurities just melted with his words of affirmation.

This man who vowed to love me for better or worse, richer or poorer, in sickness or in health, really meant it, and those vows were continually put to the test every day of his life. And he wasn’t seeing the polished-up version of me that others tend to see. He saw the frustrated me, the defeated me, the hopeless me. The me that went from one pair of pajamas to another. The me that gained over a hundred pounds on nerve meds that ultimately made me worse. The me that started to stutter and slur and hated myself for it. The me that drooled in front of people and whose nose ran uncontrollably when I sat down to eat. The me that lost control of both her bladder and bowels and that had to wear a diaper for years. The me that no longer could wipe herself or shower without his help. The me that took so much of my pain and frustrations out on him, when he’d take all of it upon himself in an instant if he could save me from it. Not only does he not complain or criticize, but he became my biggest cheerleader in life!

He listened to me as I read studies to increase my knowledge on what I’m facing. He lets me yell to him about the incompetency of doctors. He’d make small jokes to help me make light of the seriousness at hand. When he forgot things, he’d say that he was having a “Chiari moment.” I’d remind him that he wasn’t the one with Chiari and he insisted that it was sympathy pains. When he developed a herniated cervical disc and we looked at his MRIs together, the first words that came out of his mouth were, “well, I don’t have a Chiari Malformation,” as he pointed at the cerebellar tonsils. I often feel so unworthy of his love, yet if you talked to him, he’d tell you how unworthy he is of my love.

There’s no denying his real role in my life; he’s my hero!

*This article is dedicated to my husband, Johnny (my hero and the love of my life) and to all the other couples trying to hold a marriage together through this crazy fight we face.  

This Disease Called A Blessing

Published: 28 January 2019
Written by sug_97
1 Comment
Categories: Expressive Writings

They keep telling me I’m a blessing.

That I’m lucky to be alive.

That although I’m sick I’m blessed to be here every day.

I’m blessed to spend time with my kids.

And although people tell me this everyday like it is some sort of affirmation, I don’t feel blessed.

I don’t feel blessed when I walk to my car and my heart beats so hard and so fast that I feel like I’m the star of the movie Blow.

But I am always reminded at least my heart beats… a blessing.

I don’t feel blessed when my five-year-old rubs my back every day as I throw up every speck of food I ingest.

But at least I have food…. blessed.

I don’t feel blessed that I can no longer provide for myself financially because even getting dressed is a chore.

But at least I have people whose lives I can greatly burden with my illness…. blessing?

I don’t feel blessed when I hear my beautiful kids playing and I can’t drag my body out of bed to play with them.

But at least I have my hearing so I can listen…. blessing?

I’m often frustrated that I have to take 9-15 pills a day, as I throw up the sour taste of the meds.

I don’t feel blessed, but I’m told I am because I have access to health care.

I’m supposed to feel blessed when my angels fall asleep in the car, but I don’t because I’m 99% sure carrying them in the house will kill me.

All these blessings but everyday feels like hell.

I’m alive. I’m breathing. But I can’t touch any of the things that make me happy.

All these blessings and all I can think is that I’d trade all these blessings in for one last day.

Give them all up, just for one last day that I can feel normal.

That I wake up and I don’t want to scoop out my eye balls and pull out my brain just for a little relief.

That I wake up and feel energized. That I can cook my kids’ breakfast and smile and laugh.

That I live just one more day without this sickness.

I’ll trade all my blessings just for one day of no disease.

One day for my children to remember me laughing and hugging. Not vomiting. Not crying. Not laying on the floor asking them to quietly play around me because my head will explode.

I don’t want my blessings anymore.

They’re supposed to be some beautiful gift from God.

I can still see and hear all the things and people I love. But I cannot participate.

And somehow that’s a gift.

And I cannot be appreciative of them while they rob me of who I am.

I’m sick of this disease called a blessing.

 

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Breaking The Cycle

Published: 9 January 2019
Written by sug_97
3 Comments
Categories: Expressive Writings, Inspirational, To Family/Friends

I’m in an abusive relationship. It’s not a romantic one at least, not in the traditional sense of the word.

When I fell in love with her, she reminded me of a goddess. She was beautiful and kind. She never took no for an answer. She was unstoppable. She was an inspiration. To me and to everyone around her. She made me feel the highest high.

As time went on, I watched my goddess like creature slowly turn to stone. She suddenly became cold. She wanted me to stop caring about everything: my friends, my family, my hobbies, my career. She just wanted me to stay inside with her and listen to her. She wanted all my attention.

It started with bashing other people. Telling me why they didn’t deserve my time. She blanketed her insecurities by saying I was better than dealing with other people’s problems and to put myself first which really meant her and her illnesses.

Once I isolated myself from the people that I once would have laid my life down for, she turned it on me. There was no more self-love. Instead she instilled self-doubt. I wasn’t good enough to have friends and family, and love and happiness. I deserved her miserable and toxic company. All this emptiness was my fault and now I must live with it and her forever.

After she attacked my mind she started on my body. I had bruises everywhere. She’d trip me and push me into the walls. I was so sore and tired. I couldn’t eat. I lost so much weight.

All the confidence she once gave me was gone. I didn’t care anymore what I looked like. I didn’t do my makeup or change my clothes for days. Why should I? She is the only one who loved me and now she’s turned on me. I’ll stay in my sweat stained shirt forever. Maybe it will keep her away from me so I can be in peace.

It didn’t. Clothed or unclothed, it didn’t matter where I was, what time it was, or who I was with. She was always in my ear whispering about how much I’ve changed and how ashamed of me she was. Her presence made me vomit. She gave me headaches that lasted for days. I wanted to kill her.

I started to hate her. I hated her for making me hate myself. And every time I would get close to telling her to stay away from me forever, I would get flashbacks of that goddess. The girl who laughed in the face of fear. The girl who made me feel the best I’ve ever felt. I know she’s still there. I can’t give up on her.

When did she get so callous? Maybe if I can track down what triggered her abrupt change, I can help her get back to herself. What made her so abusive?

I need her to get back to who she really is. Who is she anyways? She is me. I am her. She is the reflection in the mirror that I refuse to look in the eye. She is my body before and after this disease. She is my greatest love and my mortal enemy. But can I live without her? How can I end this cycle? I can’t. I can’t walk away. I must continue to be destroyed and only hope that goddess will reappear. That her glow will radiate into my soul and warm me. That I can look at her and see love and not despair; that I can love this broken diseased soul that makes me who I am.

 

Brain Under Pressure – Understanding Intracranial Hypertension [Archived]

Published: 26 October 2018
Written by sug_97
1 Comment
Categories: Comorbidities, Ehlers-Danlos Syndromes, Empty Sella Syndrome, Etiological Co-Factors, Hydrocephalus, Idiopathic Intracranial Hypertension, Intracranial Hypertension

INTRACRANIAL HYPERTENSION (IH) AND IDIOPATHIC INTRACRANIAL HYPERTENSION (IIH) ARE CONNECTED, BUT ARE NOT THE SAME THING AND THEREFORE SHOULD NOT BE USED INTERCHANGEABLY.

Intracranial Hypertension (IH) means high pressure inside the skull. Intracranial Pressure (ICP) is measured in millimeters of mercury (mmHg). Most scholars agree that on average, “normal pressure” should be between 5-15 mmHg and that 20-25 mmHg is when the ICP crosses the line into being IH. Pressure can be brought on by several different means: space-occupying masses such as hydrocephalus and cranial cysts/tumors; cranial edema (Encephalitis); trauma; stroke; aneurysm; certain infections/diseases (Meningitis), liver failure[1], kidney failure[2]; or as a side-effect of certain medications (such as: Tetracycline[3][5], Sulfasalazine[4], Lithium[5], excess amounts of Vitamin A, steroid use[6], growth hormone treatments[6], and the hormonal Intrauterine Device (IUD), “Mirena”[7]); however, sometimes the cause of the pressure is completely unknown. When an etiological cofactor exists, it is considered Secondary Intracranial Hypertension (SIH); when no other cause is identified, it is known as Idiopathic Intracranial Hypertension (IIH) or Primary Intracranial Hypertension (PIH).

“Idiopathic Intracranial Hypertension (IIH) was first noticed in 1893, by the German physician Heinrich Quincke, who named it Serous Meningitis. As its absence of space occupying masses/lesions began to draw more thought, it was renamed Pseudotumor Cerebri (PTC) by Max Nonne in 1904. Sometime later, the term “Benign Intracranial Hypertension” began being used interchangeably with Pseudotumor Cerebri, to describe the fact that while it is sharing some of the same characteristics that a cranial tumor would cause, it is benign (not harmful), but arguments were made against it in that blindness is not indicative of being benign.”[6] The name finally settled as “Idiopathic Intracranial Hypertension,” which means IH of an unknown cause. No matter what you choose to call it, the pain and damage remains the same for those who have it.

 

UNDERSTANDING IDIOPATHIC INTRACRANIAL HYPERTENSION
IIH is a neurological disorder where the cerebrospinal fluid within the skull is elevated, without the presence of a space-occupying mass, edema (brought on by things such as trauma, infection, or disease), or any adverse reactions to certain medications. Studies show that IIH is more common amongst women between the ages of 20 and 50,[8] and there is a slight increase amongst those that are overweight. Some studies also suggest a connection between obstructive sleep apnea and transverse cerebral venous sinus stenosis.[9] Amongst the general population, IIH is believed to exist in 1/100,000 (0.00001). Amongst those that are 10% above their ideal body weight, the numbers increase to 13/100,000 (0.00013), and rising to 19/100,000 (0.00019) in those 20% above their ideal body weight.[10] Although doctors often tend to pass this off as merely a side effect of weight gain, the increase is slim and seems to decrease as the percentage of weight gain above ideal weight continues to rise above the 10% margin. Additionally, the weight factor excludes men and children under the age of 10, which may simply be because women are more likely than men to have comorbid conditions that would lead to Intracranial Hypertension. Studies show that the women to men ratio for Chiari Malformation is believed to be 3:1 and those with both Chiari Malformation and Ehlers-Danlos Syndromes 9:1[11]). However weight is not irrelevant with IIH, the overweight/obese patient population report finding improvement of some symptoms when weight loss of 5-10% of one’s overall body weight, when accompanied by a low-salt diet[12]. 

 

UNDERSTANDING THE IH/IIH CONNECTION: THE MONRO-KELLIE DOCTRINE
The association between IH/IIH and Chiari Malformation, appears to be a malicious intricate pathological circle. The cranium (skull) consists of brain matter, cerebrospinal fluid, and both venous and arterial blood. A hypothesis, referred to as the Monro-Kellie Hypothesis (or Monro-Kellie Doctrine), states, “The sum of volumes of brain, CSF, and intracranial blood is constant. An increase in one should cause a decrease in one or both of the remaining two.”[13] Therefore, if there is an abundance of cerebrospinal fluid (IIH or hydrocephalus), both cranial blood volume and brain matter should be forced to deplete. This depletion is usually directed in the path of least resistance – through the foramen magnum and into the spinal canal. When the cranial brain matter closest to the bottom of the skull (cerebellar tonsils) goes through the foramen magnum and into the spinal canal (an Acquired Chiari Malformation), it blocks the flow of cerebrospinal fluid, which in turn, continues to raise intracranial pressure.

 

SYMPTOMS OF INTRACRANIAL HYPERTENSION
Intracranial Hypertension (IH) can be either acute or chronic and comes with a variety of symptoms, many of which can help distinguish IH pain from typical pain associated with Chiari Malformation. A typical Chiari headache originates at the back of the skull (at the occiput), but IH headaches are usually described as pressure at the top of the head, that radiates downward. Headaches tend to be worse when laying down (which is opposite of low pressure headaches that are often relieved by laying down). Those that suffer from IH, often report waking up from sleep with a bad headache, and often a slight incline can help alleviate the headache pain. Pulsatile Tinnitus occurs when you hear a ringing in your ears that coincides with your heart beat. The tale-tell symptom of IH involves the damage done to the optical nerves.  Papilledema is when the optic discs swell in response to the increased cranial pressure.[14] Symptoms of Papilledema include: headaches behind the eyes, blurred vision, fleeting vision, dimmed vision, double vision, visual obscurations, decreased peripheral vision, and photopsia. Another source of IH damage is seen in the pituitary gland and is known as Empty Sella Syndrome (ESS). As the high intracranial pressure (ICP) tries to take over, cerebrospinal fluid finds its way to the sella turcica and starts filling it with spinal fluid (partially or completely)[15]. The intruding CSF attempts to envelope this depression in the sphenoid bone, and squeezes the pituitary gland, flattening it until it appears “empty.” While some initially suffer no symptoms of the damage done to the pituitary gland, most eventually develop a variety of hormonal issues, known as hypopituitarism.

 

DIAGNOSIS CRITERIA
Diagnosis of Intracranial Hypertension usually begins with investigating either the headaches or the vision problems. The least invasive test is having a neuro-ophthalmologist check behind your eyes for Papilledema. It is not considered conclusive in testing for IH, but it is essential in determining the extent of the damage to the optical nerves. Magnetic Resonance Imaging (MRI) of the brain can be useful in showing signs of Intracranial Hypertension. In cases where one or more space-occupying masses exists, further imaging and often biopsy may be required. The type of mass, its exact location, and the amount of damage that it is believed to be doing, will be used to determine the best treatment. If imaging gives an indication that the intracranial pressure is high, but no space-occupying mass exists, additional testing is usually necessary to confirm, some of which can be potentially be dangerous for those with Heritable Disorders of Connective Tissue (HDCT), such as Ehlers-Danlos Syndromes (EDS). Lumbar punctures (LP), also known as a spinal tap, are often used to test the opening CSF pressure, but by puncturing the dura (which is thinner than normal with Connective Tissue Disorders), the risk of a CSF leak is high. When an LP causes a CSF leak, the first indication is usually a post-dural-puncture headache (PLPH) and eventually, the intracranial hypertension will decrease, as the leak causes intracranial hypotension.[16] CSF leaks can escalate very quickly and can be difficult to identify and treat; therefore, we recommend that LPs be done only when absolutely necessary, and that they be done only under fluoroscopy, by qualified surgeons that fully understand the likelihood of Connective Tissue Disorders, the symptoms of leaks, and have a plan of action should those symptoms occur. Sometimes, ICP can fluctuate and have high spikes that cause problems, rendering LPs useless unless they are done at the precise time. When these spikes are suspected ICP monitoring bolts might be the better option, but still poses a risk of leaks.[17] 

 

TRANSVERSE SINUS STENOSIS (TSS)

Transverse sinus stenosis (TSS) occurs when there is a narrowing of the transverse sinus (dural venous sinus), which in turn can compromise cerebral venous outflow. TSS is common in idiopathic intracranial hypertension (IIH). Depending on the study that you are reading, it is proving to be present in 65-100% of those diagnosed specifically with IIH. Its direct connection seems relatively obscure, and there is no indication of its prevalence in intracranial hypertension (IH), but it is worth looking for and treating if found. While scholars remain undecided as to whether TSS is a cause or consequence of IH, if it does prove to be a cause of high pressure, IIH will likely no longer have an idiopathic element to it and it will become another etiology of Intracranial Hypertension. TSS can often be undetectable with standard Magnetic Resonance Imaging (MRI). The correct procedure would be Magnetic Resonance Venography (MRV, with the ATECO technique [18]), specifically looking for signs of stenosis, to include looking for fistula(s) and aneurysm(s). The lack of a fistula or aneurysms however, does not exclude the possibility of a TSS existing (remember it’s being found in 65-100% of those with IIH). Even with MRV, TSS can often be misinterpreted as “flow-related artifacts.” [18] Because the prevalence of TSS in IIH patients is high (some studies call it “universal”) [19], we recommend that all IIH patients have a MRV with the ATECO technique done before surgical treatment and that venous stenting be considered as a viable surgical treatment.

 

TREATMENT OPTIONS
Treatments for Idiopathic Intracranial Hypertension usually starts with weight loss and/or medicinal options; Diamox (Acetazolamide) and Topamax (Topiramate) are most frequently prescribed. Those with IH/IIH should avoid consuming caffeine, as it can increase pressure and therefore is counter-productive to treatment measures. Diamox is a carbonic anhydrase inhibitor and Topamax can also inhibit carbonic anhydrase, but is an anticonvulsant, often prescribed for the treatment of neuropathy and seizure disorders. Both are believed to successfully lower the production of cerebrospinal fluid. Topamax can also help suppress the appetite, which can help with weight loss, but it also comes with many side-effects like all nerve meds do. When medication fails to decrease ICP, a Ventriculoperitoneal Shunt (VP Shunt) or Ventriculoatrial Shunt (VA Shunt) are surgically placed to drain cerebrospinal fluid straight from the ventricle. Shunts are known for failing and often need a multitude of revisions. Venous stenting is not a new procedure, yet it is not readily offered. While there are studies indicating that the successful reduction of intracranial pressure can help with TSS. Stenting is not only a surgical treatment for the stenosis (which could significantly reduce the possibility of a life-threatening aneurysm in patients with a connective tissue disorder), but it is also a surgical treatment for intracranial hypertension as it “improves CSF resorption in the venous system.” [18] Therefore, it seems illogical to shunt (just dealing with the pressure) and leave such a potentially life-threatening condition untreated. [20] Studies are indicating as high as a 94% of patients being cured of all IIH symptoms as a direct result of venous stenting. [18] While all surgeries pose a risk of complications, and the statistics for stenting are likely inflated and skewed (like that of decompression surgeries), these statistics on stenting are definitely encouraging!

Intracranial Hypertension is a complex issue that should be explored whenever a Chiari Malformation exists, before a decompression surgery is performed. When both Intracranial Hypertension and Chiari Malformation are found to co-exist, the treatment should be in consideration of the correlation of the two, as they both are pathological co-factors of one another. Failure to recognize and treat Intracranial Hypertension before or soon after decompression surgery, will increase the likelihood of a failed decompression. While a decompression surgery can lower Intracranial Hypertension, as cerebrospinal fluid is once again allowed to flow, if space-occupying masses or a case of Idiopathic Intracranial Hypertension (where too much cerebrospinal fluid is being created) are left untreated, those problems will still exist after decompression surgery and the high pressure is likely to cause the cerebellar tonsils to fall once again.

*Revised October 2018

 

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References:

1 Jalan, R. “Intracranial Hypertension in Acute Liver Failure: Pathophysiological Basis of Rational Management.” Seminars in Liver Disease., U.S. National Library of Medicine, Aug. 2003, <www.ncbi.nlm.nih.gov/pubmed/14523680>.

2 Chang, D, et al. “Benign Intracranial Hypertension and Chronic Renal Failure.” Cleveland Clinic Journal of Medicine., U.S. National Library of Medicine, <www.ncbi.nlm.nih.gov/pubmed/1525975>.

3 Holst, Anders Vedel, et al. “A Severe Case of Tetracycline-Induced Intracranial Hypertension.”Dermatology Reports, PAGEPress Publications, 31 Jan. 2011, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4211491/>.

4 Sevgi, E, et al. “Drug Induced Intracranial Hypertension Associated with Sulphasalazine Treatment.” Headache., U.S. National Library of Medicine, Feb. 2008, <www.ncbi.nlm.nih.gov/pubmed/18070060>.

5 Kelly, S J, et al. “Pseudotumor Cerebri Associated with Lithium Use in an 11-Year-Old Boy.”Journal of AAPOS : the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus., U.S. National Library of Medicine, Apr. 2009, <www.ncbi.nlm.nih.gov/pubmed/19393521>.

6 Aylward, Shawn C. “Intracranial Hypertension: Is It Primary, Secondary, or Idiopathic?”Journal of Neurosciences in Rural Practice, Medknow Publications & Media Pvt Ltd, 2014, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4173226/>.

7 Etminan, Mahyar, et al. “Risk of Intracranial Hypertension with Intrauterine Levonorgestrel.”Therapeutic Advances in Drug Safety, SAGE Publications, June 2015, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4519742/>.

8  “Pseudotumor Cerebri Information Page.” National Institute of Neurological Disorders and Stroke, U.S. Department of Health and Human Services, <www.ninds.nih.gov/Disorders/All-Disorders/Pseudotumor-Cerebri-Information-Page>.

9 Thurtell, Matthew J., et al. “An Update on Idiopathic Intracranial Hypertension.” Reviews in Neurological Diseases, U.S. National Library of Medicine, 2010, <www.ncbi.nlm.nih.gov/pmc/articles/PMC3674489/>.

10 Wani, Irfan Yousuf, et al. “Complete Ophthalmoplegia: A Rare Presentation of Idiopathic Intracranial Hypertension.” Annals of Indian Academy of Neurology, Medknow Publications & Media Pvt Ltd, 2015, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4683894/>.

11 Henderson, Fraser C., et al. “Neurological and Spinal Manifestations of the Ehlers–Danlos Syndromes.” American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 21 Feb. 2017, <www.onlinelibrary.wiley.com/doi/10.1002/ajmg.c.31549/full>.

12 Thurtell, Matthew J., and Michael Wall. “Idiopathic Intracranial Hypertension (Pseudotumor Cerebri): Recognition, Treatment, and Ongoing Management.” Current Treatment Options in Neurology, U.S. National Library of Medicine, Feb. 2013,<www.ncbi.nlm.nih.gov/pmc/articles/PMC3554852/>.

13 Mokri, B. “The Monro-Kellie Hypothesis: Applications in CSF Volume Depletion.” Neurology., U.S. National Library of Medicine, 26 June 2001, <www.ncbi.nlm.nih.gov/pubmed/11425944>.

14 Schirmer, Clemens M, and Thomas R Hedges. “Mechanisms of Visual Loss in Papilledema.”Journal of Neurosurgery, <www.thejns.org/doi/full/10.3171/FOC-07/11/E5>.

15 “Empty Sella Syndrome Information Page.” National Institute of Neurological Disorders and Stroke, U.S. Department of Health and Human Services, <www.ninds.nih.gov/Disorders/All-Disorders/Empty-Sella-Syndrome-Information-Page>.

16 Panikkath, Ragesh, et al. “Intracranial Hypertension and Intracranial Hypotension Causing Headache in the Same Patient.” Proceedings (Baylor University. Medical Center), Baylor Health Care System, July 2014, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4059569/>.

17 Abraham, Mary, and Vasudha Singhal. “Intracranial Pressure Monitoring.” Journal of Neuroanaesthesiology,  <www.jnaccjournal.org/article.asp?issn=2348-0548;year=2015;volume=2;issue=3;spage=193;epage=203;aulast=Abraham>.

18 Ahmed, Wilkinson, et al. “Transverse Sinus Stenting for Idiopathic Intracranial Hypertension: A Review of 52 Patients and of Model Prediction.” American Society of Neuroradiology, July 2011. <www.ajnr.org/content/32/8/1408.long>.

19 Riggeal, Bruce, et al. “Clinical course of idiopathic intracranial hypertension with transverse sinus stenosis.” American Academy of Neurology, 2012. <www.ncbi.nlm.nih.gov/pmc/articles/PMC3589184/>.

20 Patel, et al. “Evaluating and treating venous outflow stenoses is necessary for the successful open surgical treatment of arteriovenous fistula aneurysms.” Society for Clinical Vascular Surgery, Volume 61, Issue 2. February 2015. <www.sciencedirect.com/science/article/pii/S0741521414014116>.

 

 

Palliative Care: An Essential for EDS & Chiari Families

Published: 23 October 2018
Written by sug_97
No comments
Categories: Advocacy Resources, Chiari Action Network (C.A.N.), General Information

When I was first diagnosed with Chiari Malformation, I believed everything that my neurosurgeon told me. I was originally diagnosed with a Chiari 1 Malformation. I was told that it was congenital and due to my mother either using drugs or not getting proper prenatal care, which was crushing to hear, but not all that unlikely since I was born in the early 1970s. Little did I know, that these assumptions weren’t based on my findings, but on what textbooks have said since before the advent of the MRI in the early 1980s. Sadly, the percentage of Chiari patients that are given these same faulty assumptions remains near 100%. It didn’t take much time after my decompression surgery for me to realize, that it wasn’t quite as easy or cut-and-dry as it had been made out to be. When you have a connective tissue disorder, that is the root cause behind your Chiari diagnosis, the risk of postoperative complications is high, especially when pathological conditions went undiagnosed and untreated when the decompression was done. The difficulties of having a genetic mutation that can cause problems throughout your entire body, can make a patient seem like a hypochondriac, and that is exactly what most doctors think when they have a subpar understanding of Ehlers-Danlos Syndromes. When something goes wrong structurally, it can cause a cascading effect that manifests throughout the body. We go into the Chiari fight understanding that something is wrong with our brain and neck, but when it crosses over to problems with the autonomic nervous system (for instance), we don’t realize that the continued compression on our brainstem is why our hearts are now beating so fast, why we are now so short of breath, or why we are having fainting spells. We find ourselves wishing for someone, or a panel of experts to help us navigate through it all. While it is still far from the panel that we really need, Palliative Care can offer help with some essentials.

What is Palliative Care?

Palliative Care (pronounced “pal-lee-uh-tiv” care) is a subspecialty of medical care, where an interdisciplinary team of professionals (both medical and social) are committed to helping provide “relief from symptoms and stress” for patients with serious, life-altering illnesses, and their families. [1] Palliative Care is “supportive care,” by professionals committed to you as a patient with a serious illness! Your Palliative Care Team generally consists of a Palliative Doctor, a nurse, pharmacist, social worker, nutritionist, and a chaplain [2] (all as needed). Together, they will seek to:

  • Ease symptoms and/or help control pain to relieve suffering
  • Help improve your quality of life
  • Help coordinate with your care team
  • Assist with stress, fears, anxiety, and/or depression in the patient, caregiver, and/or family
  • Help you create a plan for end-of-life care (directives)

 

Benefits to Palliative Care:

  • Both Chiari Malformations and Ehlers-Danlos Syndromes are relatively complex. Both conditions can spur a vast array of symptoms. One of the objectives of your Palliative Team is to help you find a way to alleviate symptoms and/or help you with coping mechanisms through them.
  • Because Palliative Care is designed for patients with “serious” medical conditions, being accepted as Palliative Care patient, means that they recognize the seriousness of our condition. For those of us that have spent years/decades with our symptoms being dismissed, this alone is no small thing.
  • The CDC Guideline for Prescribing Opioids, as well as many of the state laws regarding the same, have exemptions for palliative patients (along with cancer and end-of-life patients). Doctors can still decide to discontinue prescriptions, taper your prescriptions, or require an “opioid contract,” but it will be 100% by their choice, and not due to CDC Guidelines and laws. Although, you may have to tell them that. [4]

 

Misconceptions Surrounding Palliative Care:

  • While it is outlined in the U.S. Department of Heath & Human Services (NIH) under the “National Institute on Aging” (NIA), there is NO AGE CRITERIA to qualify as a Palliative Care patient. [1]
  • While Palliative Care is frequently listed beside Hospice Care and/or End-of-Life Care, there is no national requirement for a patient to be in an end-of-life situation (or even an expected early demise). [1] Hospice care falls under palliative care, but palliative care expands beyond the scope of just hospice. Palliative care may begin at any stage of a serious illness. [2]
  • You can continue treatments (even curative treatments) with your personal doctor/specialist, while receiving palliative care. There is no “incurable condition” requirement for a serious condition under palliative care. [2][3] In fact, a patient can qualify and receive Palliative Care whether their illness is curable, chronic or life-threatening. [2]
  • You do not need to wait until your condition reaches a certain level of severity, you can start with Palliative Care at any stage of your illness. In fact, Palliative Care works best when it begins as early as possible in the illness, as some symptoms may be avoidable or more manageable if addressed early.
  • Palliative Care does not replace or override your pain management doctor, or any of our doctors for that matter. Instead, we give them permission to discuss our case with our medical professionals, so they can help coordinate our care, especially in regard to our pain/suffering and quality of life care.
  • Your Palliative Care Team will not consist of the medical specialists that we specifically need, but they can help navigate you to the type of doctor you may need and discuss these recommendations with your doctors.

 

Problems Surrounding Palliative Care:

  • Primary Care Doctors (who are supposed to be offering referrals to Palliative Care for their patients with serious medical conditions) often fail to fully understand the spectrum of Palliative Care. Because of their faulty understanding, most of us are never offered Palliative Care, and when we request it, we are often told that it is equivalent to hospice care or set aside for hospice patients, and therefore they believe that we do not qualify. THEY ARE WRONG!
  • Our medical professionals often fail to recognize just how “serious” of a condition and life-altering Chiari and EDS really are (on us as patients and on our families).
  • Not all insurance companies cover Palliative Care. Because some insurance companies fail to see the seriousness of our conditions, qualifying may be difficult. Private insurance and HMOs are more likely to offer Palliative Care. Different states have different policies regarding the level of Palliative Care (if any) they offer. [2] If cost concerns are an issue, the social worker on your Palliative Team can assist you in ways that might help you qualify. [3]

 

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References:

1. “What Are Palliative Care and Hospice Care?” U.S. Department of Health and Human Services, National Institute on Aging, 17 May, 2017. <https://www.nia.nih.gov/health/what-are-palliative-care-and-hospice-care>

2. “Frequently Asked Questions About Hospice and Palliative Care.” American Academy of Hospice and Palliative Medicine. <http://palliativedoctors.org/faq>

3. “Handout for Patients and Families.” Center to Advance Palliative Care. <https://getpalliativecare.org/handouts-for-patients-and-families/>

4. “CDC Guideline for Prescribing Opioids for Chronic Pain.” Centers for Disease Control and Prevention, National Center for Injury Prevention and Control, Division of Unintentional Injury Prevention. 29 Aug, 2017 <https://www.cdc.gov/drugoverdose/prescribing/guideline.html>

Finding Hope In The Seemingly Hopeless Chiari Fight!

Published: 17 October 2018
Written by sug_97
1 Comment
Categories: General Information

Despite the pain we face on a daily basis, that our doctors so often ignore…

Despite the anger that builds towards a medical system that is relatively
clueless about our conditions…

Despite the frustrations we face when those we love fail to understand what we’re going through…

… Despite it all, hope remains!

Purple-flower-HOPE_AS185193152.jpeg
“They tried to bury us. They didn’t know we were seeds.”

There is little easy about the Chiari fight (and that of its co-morbid conditions), but it is easy to lose hope! The thing about that however, is if you look at the complexities of our bodies and the conditions we face, one thing is for sure, we weren’t created for easy! Coping with conditions like Chiari Malformations, Ehlers-Danlos Syndromes, and their many ugly friends can be a very daunting task that we all have to face. There are many challenges, including chronic pain that can be severe, fatigue that can be debilitating, the fight with doctors for proper diagnosis and treatment, the fight with loved ones to be understood and supported, and the general lack of awareness of these conditions in the public. It is easy to lose sight of the light at the end of the tunnel. But, even with all the hardships we face, there is a lot to still be hopeful about.

Education & Research 
One of the most frustrating aspects of dealing with Chiari and its comorbidities is the lack of education within the medical community. There is little more disconcerting than walking into a doctor’s appointment and being asked to spell your condition, so the doctor can Google it. These appointments often end in tears because the doctor has absolutely no clue about our condition, but this is improving. Just ten years ago, few of the doctors we encountered had heard of Chiari Malformation, we had to explain it to almost every doctor we came across, regardless of their specialty. This is happening far less now. While there are still many misconceptions that medical professionals tend to have about these conditions, many have a general level of familiarity now. That means we are making real progress and the future is looking brighter. In a recent webinar on Chiari, Complex Chiari and Craniocervical Instability, Complex Chiari expert, Dr. Paolo Bolognese shared the following:

In reference to the association between EDS and Complex Chiari, Dr. Bolognese shared that he stumbled upon the association in 2002 during office visits, when he saw a half dozen patients in a 2-week period diagnosed with both. After consulting colleagues and experts in genetics and connective tissue disorders, Dr. Bolognese and his colleague, Dr. Milhorat, conducted a pilot study of the association and published their findings in a major neurosurgical journal in 2006. These findings were met with much skepticism among even the world’s top Chiari experts. In 2017, however, at the XXIX ASAP Conference on CM1, Dr. Bolognese submitted a questionnaire to these top experts. 63 experts from 4 countries responded. 77.5% agreed that there is an association between EDS and CM1. 99.5% agreed about the existence of Complex Chiari. On this fight to get other experts to see the connection, Dr. Bolognese said, “Science does not have a linear behavior. It goes in accelerations, stops, accelerations, stops, some steps backwards, some steps forward. So, it took 12 years to convince most of the leading experts in our field. It will take 12 more years to convince the ‘regular’ neurosurgeons. It will take 12 more years to convince primary care physicians.”

While this delay in translating research and scientific discovery into medical practice is frustrating for us as patients, there are also some great reasons to be hopeful. First, there are doctors who have dedicated their careers to helping us find answers and improving our quality of life in the meantime. These doctors are willing to put their professional reputations on the line and face the critics to make improvements in our treatment outcomes. Second, the experts are being convinced, and as more experts come to believe in what we, the patient community, already know to be true, it is more likely that younger generations of doctors and researchers will want to get involved in these areas of research. While many of us are suffering and fighting for care by physicians who fully understand the complexities of our conditions, progress is being made that will likely revolutionize the future of patient care.

In addition to this little snapshot into the experts’ world, anyone who has spent any time in online support groups over the last decade can tell you that patients are getting diagnosed sooner, tested for comorbid conditions more often, and are having better surgical outcomes because they are better informed and are asking all the right questions of their doctors. This trend is likely to continue as patients now have better access to pertinent research information through a wide variety of educational materials offered online by Chiari and EDS non-profit organizations. It is finally OUR time and each of us can do our part to make the most of it, by educating ourselves and sharing information with others, as well as supporting the organizations that make this information available to us.

Support
Thanks to the worldwide availability of the internet and social media, support is easier to access and is more abundant than ever before. Patients and caregivers alike can now find a wide variety of support groups for these conditions on a variety of social media platforms. Groups range from small and intimate, to large and very active, and there is a fit for practically any personality. There are groups for women only, men only, teens only and parents only. There are groups focused on laughter and those focused on hobbies like gaming or crafting. Some groups focus on support, while others focus on information, and some do an excellent job at both. There are international groups where you can interact with other patients from around the world, and there are local groups where you can potentially meet people in your own town or surrounding area. There are also numerous in person support group meetings where you can meet fellow patients, attend educational lectures, participate in awareness activities, and much more. Interacting with others who are enduring similar struggles is not only an opportunity to feel less alone and more validated, it is also an opportunity to help others and even potentially form lifelong friendships.  

Awareness
The patient community has made great strides in raising awareness for Chiari and related disorders. Between individuals sharing awareness materials on social media, patients reaching out to local media to share their stories and organizations hosting awareness walks, much needed attention and information is getting out to the public. While we still have a long way to go in making Chiari a household name, we are making progress toward it. You can join in these efforts by getting intentional about awareness! What has helped you, might help others!

Creating Our Own Hope
We have been a neglected community for far too long. We have been ignored, dismissed, and sometimes even abused. We have had doctors laugh in our faces, call us crazy, and even accuse us of making things up. It often leaves us feeling hopeless, like there’s nowhere left to turn, but we are standing up and demanding better care. We are demanding that our healthcare professionals educate themselves and study the most recent research. We are demanding that our voices be heard and that we are taken seriously. Although there has been some progress, our fight is far from over. It is just beginning, and we refuse to sink into the shadows because we are mighty warriors who will fight for what we need! When necessary, we will create our own hope! As the old Mexican proverb goes, “They tried to bury us. They didn’t know we were seeds.” Our community is growing every single day and there is a need now, more than ever, to stand up not only for ourselves, but those among us who are vulnerable and need us to do it for them. Please, whether you are a patient yourself, a caregiver, a spouse, family member or friend to someone who is suffering, find your inner warrior and join us in this fight!

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How Much Are You Worth?

Published: 15 October 2018
Written by sug_97
2 Comments
Categories: Advocacy Resources, Chiari Action Network (C.A.N.)

When a person suffers from a chronic condition, we sometimes equate our value to how we feel. Chiari, Ehlers-Danlos, CSF Leaks, Chronic Fatigue Syndrome, etc. all cause pain. Sometimes we tend to carry that pain along with us as baggage. If we carry our self-value as related to pain, we are more likely to let healthcare professionals bully us and eventually, all this baggage compromises the relationships that matter most to us. Well, it’s time to look deep inside ourselves and find our inner strength.

Take a step back and pretend for a moment that a loved one had the conditions you have. Imagine knowing all the struggles they have fought. Imagine them hoping for a diagnosis so that they could have a name to fight. Imagine them enduring countless appointments and procedures. Imagine them in pain and struggling to go to important events, so they are not letting people down. Imagine them doing hours and hours of research to find help. Now, stop for a moment and ask yourself a question. Would you call that person strong or weak? Really stop and think about it. They aren’t famous and probably not wealthy. I know what I would call them. I would call them the epitome of strength! I hope you would consider a loved one like that to be a super strong person too. Wouldn’t you have respect for them? Wouldn’t you wonder how they kept functioning and be in amazement of their abilities? Now stop again. This person we imagined as a loved one is now back to being YOU!!!

That’s right! YOU, with these chronic conditions, feeling as battered and bruised as you do, are like Superman or Superwoman. You have done more while enduring pain, than most people do that are completely healthy!

Now, how do you value your life? You should value your life as a beautiful person that finds strength deep down inside. You should find joy in small things and accept love that is given to us as someone that is deserving of such love. Let them love you and cherish the special moments you have in life. Value your health and fight for what you feel is the right treatment. Don’t be afraid to ask questions of your medical professionals and fight to get back and keep whatever quality of life that you can, because you matter! But we must learn to fight right! Not with fists or harsh words, but by articulating the extent of our pain at every level allotted to us!

When your doctor provides substandard care, he subjects himself/herself to a formal complaint. Misdiagnosis or delayed diagnosis that leads to incorrect treatment, delayed treatment, or no treatment at all, can cause you harm and is complaint worthy. Overprescribing, underprescribing, and failure to prescribe adequate treatment for your condition(s) are also grounds for a formal complaint.

 

Preparing to make your complaint(s):

• Calmly articulate your condition(s) and how difficult treatment has been on you and your family thus far.

• Explain the treatment options you have been presented with, the options you have tried and why they should not be considered efficient treatment for you, and why you feel that the option being declined to you is the best course of treatment for you personally.

• Directly ask the recipient at each level for help and express your desire to resolve the issue at their level.

 

Preparing to send your complaint:

• Write the department and/or division head and request a meeting with him/her about it.

• Write the members services of the hospital or medical group that you are a part of and request an internal review.

• If the internal review failed to bring about the action that you need, request an external review.

• File a complaint with the state medical board that the doctor is in. Be sure to provide all supporting evidence that you have. They will enter your complaint into database and review your case to determine if disciplinary action should be taken against your doctor. Even if the state medical board doesn’t agree with you, it will go on record and might be reviewed again when/if additional patients make similar complaints about that doctor.

• Contact your state legislators asking for their help. While they usually wont respond to you directly, they have state aides that are armed with lists of resources to help. If state laws are a reason behind the lack of treatment you are getting, let them know how these laws are having a negative impact on the quality of care that you are receiving in their state.

• Contact your federal legislators asking for help at a national level. You might feel intimidated, like they are not going to care about what is happening with a sick constituent, but the truth is that you are not the only patient experiencing these problems, and their ability to hear from multiple patients with similar problems can raise red flags. Federal legislators are officials paid to represent your state at a federal level. If federal laws are a reason behind the lack of treatment you are getting, let them know how these laws are having a negative impact on the quality of care that you are receiving.

• Write letters to all government agencies, such as the Centers for Disease Control (CDC), Federal Drug Administration (FDA), etc. They might not do anything specifically about your doctor, but they are responsible (at least in part) for many of the rules and regulations governing aspects of our care. Let them know how you’re doing under the policies their responsible for. 

• Write the United States Attorney General and the President of the United States and let them hear directly from you on how these laws, rules and regulations are affecting your health care.

The bottom line is, everything has been accomplished for Chiarians, has been done by us working together. They might not care about one letter, but how about tens of thousands? Let’s unite and fight for our rights to proper medical care, including proper pain relief, while we can!

You can do this! Together we C.A.N. do this!

 

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Overview: Chiari Malformation [Revised]

Published: 4 October 2018
Written by sug_97
3 Comments
Categories: Acquired Chiari Malformation, General Information

CHIARI MALFORMATIONS (PRONOUNCED: KEE-AH-REE) ARE STRUCTURAL DEFECTS IN WHICH THE CEREBELLUM, THE HIND PART OF THE BRAIN, DESCENDS BELOW THE FORAMEN MAGNUM INTO THE SPINAL CANAL.

While Arnold Chiari Malformation (Type 2) was first identified in the late 19th century by the Austrian pathologist Hans Chiari, much of the current medical knowledge has developed since 1985 with the expanded use of Magnetic Resonance Imaging (MRI). The number of patients diagnosed with Chiari malformations continues to increase, and with that increase Chiari Malformation is getting some of the attention the condition has always demanded.

Chiari malformations (“CMs”) are neurological disorders in which the cerebellum extends out of the skull and into the spinal canal, which in turn blocks the flow of cerebrospinal fluid, puts pressure on the brainstem and spine, and may result in varying degrees of nerve compression. Once thought to occur in 1 in 1000 people, it is now believed to be much more frequent of an occurrence. A 2016 pediatric study found it to occur in 1 in 100 children[1]. However, since the most common type (Type 1) tends to become symptomatic during late teens and early adulthood, it is likely to be much more common when adults are factored in. Females are more likely than males to have a Chiari Malformation (at a ratio of 3:1), and significantly higher amongst those with both Chiari Malformation and Ehlers-Danlos Syndrome (9:1)[2]. We affectionately refer to those that live with this condition, including the attendant pain and frequent disregard from the medical community, as Chiarians (regardless of whether they have had surgical intervention or not).

While some Chiarians are symptomatic throughout their lifetime, the vast majority of Chiarians (those with Type 1) develop symptoms in their late teens or early adulthood. Those symptoms can range from mild to crippling, and can become severe enough to cause paralysis (often associated with syringomyelia) or death.


WHAT CAUSES A CHIARI MALFORMATION?

Multiple factors have been identified which can either cause or attribute to Chiari malformations. Although they too were once thought to be rare, Acquired Chiari malformations are now being diagnosed in increasing numbers. A brief overview of what each of these labels entail, together with a summary of the different types of CM’s, is provided below:

  • Congenital Chiari, is believed to be caused by a posterior cranial fossa hypoplasia (PCFH)[3], which can also be caused by a connective tissue disorder such as Ehlers-Danlos.[4] While the cerebellum continued to grow in utero, the posterior skull failed to grow proportionately. Problems resulting from this size discrepancy continue and eventually the overcrowding of the hindbrain squeezes the cerebral tonsils downward into the opening of the spinal canal (cranial constriction). While the herniation of the cerebellar tonsil(s) can take place during gestation or after birth, because the cause is 100% congenital, and the process most likely began in utero, it is usually considered a Congenital Chiari Malformation when the only pathology found is a small posterior cranial fossa. In one large study, they found those with a Chiari Malformation and no associated etiological/pathological co-factors, with only slightly over 52% having a small PCF. When other co-factors were present, the number of Chiarians found with a small PCF plummeted, and therefore it should be considered acquired until proven otherwise.[5]

  • Acquired Chiari can have one or more possible pathological co-factors; any of which can result in the descent of the cerebellar tonsils. Many Chiarians often mistakenly conceptualize an Acquired Chiari Malformation as being brought on only by trauma; however, “acquired” is an antonym for “congenital,” so an Acquired Chiari Malformation in medical terms is one that a person was not born with. While this can include Acquired Chiari malformations resulting from trauma, it can also include Acquired Chiari malformations resulting from a variety of other medical conditions:
    • Heritable Disorders of Connective Tissue (HDCTs), most commonly Ehlers-Danlos Syndromes (EDS), make the tonsils more prone to prolapse below the foramen magnum.
    • Multiple conditions are known to create a pushing/pulling effect that can result in a tonsillar herniation. These conditions include: Intracranial Hypertension (IH), Atlantoaxial Instability and Craniocervical Instability (AAI/CCI), Tethered Cord Syndrome (TCS), and Intracranial Hypotension (cerebrospinal fluid leaks), Hydrocephalus, and a variety of cysts and brain tumors.[2]

    Special care should be taken when any of these co-morbid conditions exist in conjunction with a Chiari Malformation. Before the consideration of decompression surgery, a plan should be developed which addresses each possible comorbid condition before decompression. This can reduce the likelihood of complications and/or a failed decompression surgery.

SEVEN TYPES OF CHIARI MALFORMATIONS WORTH DISCUSSING (asterisks “*” indicate commonly known types)

Chiari Zero:  The lower part of the cerebellum (the cerebral tonsils) are blocking the foramen magnum, but are not descended through. Because of the cerebellum’s position, it blocks the flow of cerebrospinal fluid and all the effects of that blockage are comparable to Type 1.

Diagnosis Requirements: Symptomology; MRI showing no herniation but the low-lying tonsils that are pressing against the top of the foramen magnum; MRI showing a syrinx (despite the name, Chiari Zero is classified under Syringomyelia and not Chiari Malformation – so a syrinx is technically required for diagnoses). [6][7]

Treatment Options: With few symptoms, non-surgical treatments might be recommended. When a syrinx is present, a decompression is often recommended before the syrinx has a chance to further develop and cause additional damage to the spine. However, even when a syrinx is present, all pathological cofactors should be explored and addressed prior to decompression surgery.

Chiari 0.5: In cases of Chiari 0.5, the lower part of the cerebellum (the cerebral tonsils) are descended through the foramen magnum, but descends < 5mm (which is the measurement that some doctors use to define Chiari). Usually labeled “tonsillar ectopia” on radiology reports, the symptoms and effects of the obstruction are generally the same as those experienced with Type 1 or Chiari 1.5.[3]

Diagnosis Requirements: Symptomology; MRI showing a herniation of < 5mm, unless already properly diagnosed with a Type 1 or Chiari 1.5; presence of a syrinx is not “required” for diagnosis, but as with Chiari Zero, it illustrates that it is causing a problem obstructing the flow of cerebrospinal fluid and may be relevant when deciding between various courses of treatment.

Treatment Options: The same as Type 1 or Chiari 1.5, respectively.

*Chiari Malformation Type 1: The most common type of Chiari Malformation, Type 1 is diagnosed when the cerebral tonsils descend below the foramen magnum. Medical professionals unfamiliar with current research surrounding Chiari Zero and Chiari 0.5 (and the symptomology surrounding the blockage of cerebrospinal fluid), believe that a tonsillar herniation of less than 5mm is simply a tonsillar ectopia and only diagnose a Chiari Malformation when the descent is > 5mm. However, the 5mm requirement is controversial, and many doctors now base their diagnoses not solely on measurements, but rather on symptomology and a combination of other factors, including cine MRI’s, a patient’s symptoms, and other relevant factors.[6] Many people with a Chiari Zero, Chiari 0.5, or Type 1 can be asymptomatic for a lifetime: one large study found that approximately 30% of those with a CM measuring between 5-10mm were asymptomatic.[8] If symptoms develop, they often present in adolescence or early adulthood. Anecdotal evidence supports the proposition that once symptoms start, the symptoms often progress rapidly until the damage is stopped surgically.

Diagnosis Requirements: Symptomology; MRI indicating at least one herniated tonsil (without the brainstem descending as well).[9]

Treatment Options: Prior to surgery any/all comorbidities should be explored and treated especially if you are found to have a normal sized posterior fossa. However, if you have classic Chiari 1 Malformation with a small posterior fossa, the risks of surgery should be weighed against the severity of symptoms and the impact that symptoms are having on the patient’s quality of life. It is often recommended to treat mild symptoms with medication, with surgical options typically reserved for cases in which symptoms cause more serious medical and quality of life problems. However, symptoms do tend to progress, and studies have shown a correlation between successful decompression surgery and the amount of time between the onset of any symptoms and surgical intervention[10]. See “Decompression Surgery” below.

Chiari 1.5: This type of CM (often referred to as a “Complex Chiari”) is often acquired as opposed to congenital.  Chiari 1.5 should be the diagnosis when the tonsil(s) and all/part of the lower brainstem (the medulla oblongata) has descended past the foramen magnum. This is usually indicative of another comorbid condition pushing the brainstem downward from above or pulling downward from below.[5][11][12]

Diagnosis Requirements: symptomology; MRI indicating at least one herniated tonsil AND a downward displacement of all/part of the brainstem; without the other radiological findings associated with Type 2.

Treatment Options: Treatment options can vary significantly from patient to patient depending on the cause of the Chiari 1.5. While a variety of medical options might initially be used to treat symptoms, it is extremely important that all possible causes and/or comorbidities are thoroughly investigated and treated prior to the consideration of decompression surgery. Failure to identify and treat any such conditions can increase the likelihood of a failed decompression and further complications such as brain slumping, increased cervical instability, etc.

*Chiari Malformation Type 2 (also known as Arnold Chiari Malformation): Type 2 involves a herniation of the cerebellar tonsils and lower part of the brainstem (the medulla oblongata). Unlike in Chiari 1.5, in Type 2 the fourth ventricle is usually herniated, all/part of the cerebellar vermis (the tissue connecting both halves of the cerebellum) is missing or herniated, the corpus callosum (nerve fibers connecting both hemispheres of the brain) is fully/partially absent (agenesis), and it is almost always accompanied by a myelomeningocele (the most serious form of Spina Bifida, a congenital neural tube defect where the spinal canal does not close properly).[13][14][15]

Diagnosis Requirements: While a myelomeningocele is usually evident and diagnosed at birth, a brain MRI should confirm the radiological aspects of Type 2.

Treatment Options: Myelomeningocele is usually treated surgically at birth. If other related problems develop, such as hydrocephalus and/or tethered cord, they are often dealt with surgically as they become problematic. While some with Type 2 are decompressed, anecdotal evidence reflects a general trend of an increased failure rate with decompression surgeries as compared to those with Type 1. Because of this, some neurosurgeons choose not to decompress those with Type 2.

*Chiari Malformation Type 3: Type 3 is a serious type of Chiari Malformation involving herniated cerebellar tonsils, brainstem, and fourth ventricle. However, in most cases of Type 3, a sac forms out of the back of the skull (encephalocele) that contains brain matter from the cerebellum and the meninges. Type 3 causes severe neurological problems that are evident at birth and has a high infant mortality rate.[16][17]

 *Chiari Malformation Type 4: Type 4 is the most severe type of Chiari Malformation, but does not involve a hindbrain herniation (and therefore arguments have been made that it is not a Chiari Malformation). Instead, it consists of an undeveloped or underdeveloped cerebellum. Most infants born with Type 4 die in infancy.[16][17]


SURGICAL INTERVENTION 

Decompression surgery is currently the only available means of attempting to stop the progression of symptoms of a congenital chiari (with no other pathological cofactors), but decompression is not a cure (not even close). Statistics show that up to 69% of decompressed patients find some measure of relief from surgery (usually headaches)[18]. Most neurosurgeons will give only a 50% chance of helping each individual symptom. Some of the symptoms are irreversible once they develop. Recent studies show that there is a correlation between early surgical intervention and positive post-surgical outcomes.[19] However, we cannot over emphasize the importance of your doctors taking time to find, diagnose, and treat co-morbid conditions BEFORE decompression surgery. If they are not willing to consider comorbidities, they are probably not the doctor for you!

 

 

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*Original version released January 2018, revised October 2018.

 

References:

1 Eltorai, Ibrahim M. “Rare Diseases and Syndromes of the Spinal Cord” Cham: Springer International Publishing: Imprint: Springer, 2016. Page 43, 15.2, <www.springer.com/us/book/9783319451466>.

2 Henderson, Fraser C., et al. “Neurological and Spinal Manifestations of the Ehlers–Danlos Syndromes.” American Journal of Medical Genetics Part C: Seminars in Medical Genetics, 21 Feb. 2017, <www.onlinelibrary.wiley.com/doi/10.1002/ajmg.c.31549/full>.

3 Sekula, Raymond F, et al. “Dimensions of the Posterior Fossa in Patients Symptomatic for Chiari I Malformation but without Cerebellar Tonsillar Descent.” Cerebrospinal Fluid Research, BioMed Central, 2005, <www.ncbi.nlm.nih.gov/pmc/articles/PMC1343586>.

4 Stagi, Stefano, et al. “The Ever-Expanding Conundrum of Primary Osteoporosis: Aetiopathogenesis, Diagnosis, and Treatment.” Italian Journal of Pediatrics, BioMed Central, 2014, <www.ncbi.nlm.nih.gov/pmc/articles/PMC4064514>.

5 Milhorat, Thomas H., et al. “Mechanisms of Cerebellar Tonsil Herniation in Patients with Chiari Malformations as Guide to Clinical Management.” Acta Neurochirurgica, Springer Vienna, July 2010, <www.ncbi.nlm.nih.gov/pmc/articles/PMC2887504>.

6 Isik, N, et al. “A New Entity: Chiari Zero Malformation and Its Surgical Method.” Turkish Neurosurgery., U.S. National Library of Medicine, <www.ncbi.nlm.nih.gov/pubmed/21534216>.

7 “JNS JOURNAL OF Neurosurgery OFFICIAL JOURNALS OF THE AANS since 1944.” The Resolution of Syringohydromyelia without Hindbrain Herniation after Posterior Fossa Decompression | Journal of Neurosurgery, Vol 89, No 2, <www.thejns.org/doi/abs/10.3171/jns.1998.89.2.0212?url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org&rfr_dat=cr_pub%3Dpubmed>.

8 Elster, A D, and M Y Chen. “Chiari I Malformations: Clinical and Radiologic Reappraisal.”Radiology., U.S. National Library of Medicine, May 1992, <www.ncbi.nlm.nih.gov/pubmed/1561334>.

9 Wilson, Eugene. “Chiari.” CEDSA Home, <www.cedsa.org/index.php/59-quick-reference/73-chiari.html>.

10 Hindawi. “Surgical Management of Patients with Chiari I Malformation.” International Journal of Pediatrics, Hindawi, 28 June 2012, <www.hindawi.com/journals/ijpedi/2012/640127>.

11 Kim, In-Kyeong, et al. “Chiari 1.5 Malformation : An Advanced Form of Chiari I Malformation.”Journal of Korean Neurosurgical Society, The Korean Neurosurgical Society, Oct. 2010, <www.ncbi.nlm.nih.gov/pmc/articles/PMC2982921>.

12 Malik, Amita, et al. Chiari 1.5: A Lesser Known Entity. Annals of Indian Academy of Neurology, <www.annalsofian.org/article.asp?issn=0972-2327;year=2015;volume=18;issue=4;spage=449;epage=450;aulast=Malik>.

13 Wolpert, Samuel M, et al. “Chiari II Malformation: MR Imaging.” American Journal of Roentgenology, <www.ajronline.org/doi/pdf/10.2214/ajr.149.5.1033>.

14 Yumer, M H, et al. “Chiari Type II Malformation: a Case Report and Review of Literature.”Folia Medica., U.S. National Library of Medicine, <www.ncbi.nlm.nih.gov/pubmed/16918056>.

15 Kim, Irene. “Chiari II Decompression in Patients with Myelomeningocele in the National Spina Bifida Patient Registry (NSBPR).” <http://spinabifidaassociation.org/sbworldcongress/wp-content/uploads/sites/10/2017/04/B.4-Kim-Neurosurgery.pdf>.

16 “Chiari Malformation Fact Sheet.” National Institute of Neurological Disorders and Stroke, U.S. Department of Health and Human Services, <www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Chiari-Malformation-Fact-Sheet>.

17 “Chiari Malformations.” NORD (National Organization for Rare Disorders), <www.rarediseases.org/rare-diseases/chiari-malformations>.

18 14 Aliaga, L, et al. “A Novel Scoring System for Assessing Chiari Malformation Type I Treatment Outcomes.” Neurosurgery., U.S. National Library of Medicine, Mar. 2012, <www.ncbi.nlm.nih.gov/pubmed/21849925>.

19  Siasios, John, et al. “Surgical Management of Patients with Chiari I Malformation” International Journal of Pediatrics, Article ID 640127, Hindawi, 2012, <www.hindawi.com/journals/ijpedi/2012/640127>.