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Awareness Pumpkin 2023 Contestant 2: Ethan and Amy D (mom)
Author: sug_97
Awareness Pumpkin 2023 Contestant 1: Haley E.
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September Is Chiari Awareness Month
It’s hard having a chronic illness that isn’t all that understood.
As patients, we have to fight on absolutely every level!
Before diagnoses, we fight for someone to hear us when:
- We explain to them that our neck is to weak to hold up our head.
- We’re trying to hold our heads up with our hands when laying back isn’t an option.
- Our necks start spasming to the point that we feel like we’ve been internally decapitated.
- We have to ride in the front seat to try and minimize the car sickness.
- We suddenly can’t balance to walk.
- Our eyes start twitching beyond what could ever be considered normal.
- We aren’t able to do what we could just a short time ago, or even a few hours ago.
- That we want to scream and cry because of the pain, but we know it will only make it worse.
- We go to say something and can’t find the right word because it just isn’t in our memory bank at that moment.
- We spontaneously can’t read because we have double vision, blurred vision, or our eyes wont stop jerking around to focus, yet an hour later we’re fine.
- We explain that doctors not knowing what’s wrong doesn’t mean that nothing is wrong (even when they say nothing is wrong).
Around diagnoses, we fight to:
- Process the magnitude of what we’re facing.
- Learn all we can so we’re prepared for the important decisions before us.
- Find the right doctors who are knowledgeable and trained in our condition(s).
- Fight to be stoic when we know that it’s not just our bodies enduring all of this – something is breaking in our souls and we’re fighting to not let it change us for the worse.
When our doctors continue to dismiss our symptoms, we need our friends and families to understand:
- That we’re still the same wife/husband, mother/father, sister/brother, aunt/uncle, and/or friend that you’ve known and loved for all these years, and we need you now more than ever!
- That decompression is not a cure! In fact it typically fails to relieve symptoms over the long term nearly 50% of the time when pathological conditions aren’t treated beforehand.
The Important Questions to Ask Your Neurosurgeon [Revised]
Most Chiarians go to see a surgeon with an expectation of them being knowledgeable in their field. However, while they might be a neurosurgeon, their knowledge of Chiari and its comorbid/pathological conditions might not rank high in their practice. Make the most of your initial appointment by interviewing them and what they really know about Chiari Malformations. Be cautious of inflated success rates. Chiari decompression in general offers a just over a 50% success rate (which means it has a nearly 50% failure rate). Surgeons that claim a 100% (or near 100% success rate) are usually not basing their success on how their patients feel afterward, it is based on if they were successful with the aspects of the surgery:
Removal of the occipital bone ✓
Opening the dura and adding the patch/graft ✓
Laminectomy ✓
Cauterization/resection of cerebellar tonsils ✓
WE DESERVE BETTER THAN THAT!
HERE IS A LIST OF CHIARI QUESTIONS WE RECOMMEND ASKING AT YOUR FIRST NEUROSURGERY APPOINTMENT:
General Questions:
- How do you define a Chiari Malformation?
- What do you believe causes a Chiari malformation?
- Are all Chiari malformations from a small posterior fossa?
- Do I have a small posterior fossa? If yes, how big is it? If size is unknown, was my posterior fossa measured? If not, why not? How did you come to the conclusion that I have a small posterior fossa?
- How common do you believe Acquired Chiari malformations to be?
- Do you always recommend decompression surgery for all of your patients with herniated cerebellar tonsils? Why/why not?
- In an average month, how many Chiari decompressions do you perform? How many tethered cord releases? How many craniocervical fusions? What percentage of your practice is spent treating patients with these connective tissue related conditions?
- Looking at my brain scan, is any part of my “brainstem” herniated (below the posterior fossa)? If so, does that make me a Chiari 1.5?
Intracranial Hypotension (low pressure) Questions:
*Article to help you understand CSF Leaks & Intracranial Hypotension prior to your appointment.
If you have SYMPTOMS OF LOW INTRACRANIAL PRESSURE and/or suspect a cerebrospinal fluid leak, we recommend asking the following questions:
- S.E.E.P.S.
- Looking at my brain scan, do you see any Subdural fluid collections?
- Looking at my brain scan, do you see an Enhancement of pachymeninges?
- Looking at my brain scan, do you see an Engorgement of my venous structures? Should we do an MRV to make sure?
- Looking at my brain scan, does my Pituitary appear to be enlarged?
- Looking at my brain scan, does my brain appear to be Sagging?
- Looking at my corpus callosum:
- Does there appear to be a depression?
- Is there an inferior pointing of the splenium?
If he/she answers affirmatively to any of the above S.E.E.P.S. questions, ask:
- What should be done to find/repair a potential leak?
- Are you aware that it is common for CSF Leaks to not show up on MRI?
- Are you willing to do a CT Myelogram and/or a digital subtraction myelogram, if I develop symptoms of a leak and none can be found on MRI?
- Are you aware that it can often take multiple epidural blood patches to try and seal a leak, and sometimes when a blood patch fails to work, a surgical dural repair might be necessary?
Intracranial Hypertension (high pressure) Questions:
*Article to help you understand Intracranial Hypertension prior to your appointment.
If you have SYMPTOMS OF HIGH INTRACRANIAL PRESSURE, we recommend asking the following questions:
- Looking at my brain scan, do I have cerebrospinal fluid in my sella turcica (Empty Sella Syndrome)?
- Looking at my brain scan, do you see any evidence of my optic nerves are swollen (papilledema)?
- If so, should I be referred to a neuro-ophthalmologist?
- Looking at my brain scan, do my lateral ventricles appear small or flattened?
- If so, do I need to have my pressures checked?
- If yes, are you aware of the risks of developing a CSF Leak from a lumbar puncture?
- What are the symptoms of a CSF Leak, should one develop?
- What is your plan of action if I should develop these leak symptoms?
- Are you aware that it is common for CSF Leaks to not show up on MRI?
- Are you willing to do a CT Myelogram if I develop symptoms of a leak, and none can be found on MRI?
- Should a leak be found, are you aware that it can often take multiple epidural blood patches to try and seal a leak?
- If so, do I need to have my pressures checked?
Tethered Cord Questions:
*Article to help you understand Tethered Cord: Sorry, Coming Soon.
If you have SYMPTOMS OF TETHERED CORD, we recommend asking the following questions:
- Looking at my brain/cervical scan, does my brainstem appear to be elongated?
- Looking at my cervical scan, does my spinal cord appear to be stretched?
- Looking at my lumbar scan, does my conus reach my mid/low L2?
- Looking at my thoracic and lumbar scan, does my spinal cord appear to be pulling to the back, or one particular side?
- If so, should we do a prone MRI to see if it has actually adhered to that side?
- Looking at my lumbar scan, do I appear to have fatty tissue inside the epidermis?
- If the answer to any of these questions is affirmative, do you suspect that I have a tethered spinal cord?
- If so, should we plan for a Tethered Cord Release before or soon after decompression surgery, so the likelihood of a failed decompression is reduced?
- If I have urological issues, can I get a referral for urodynamic testing to rule out any other potential causes of my urological issues?
Craniocervical Instability (CCI) & Atlantoaxial Instability (AAI):
*Article to help you understand CCI & AAI prior to your appointment.
If you have SYMPTOMS OF CRANIOCERVICAL INSTABILITY or SYMPTOMS OF ATLANTOAXIAL INSTABILITY, we recommend asking the following questions:
- Looking at my brain/cervical scans, what are the measurements of my clivoaxial angle and Grabb-Oakes?
- Do these measurements meet the diagnostic criteria for Craniocervical Instability?
- Looking at my flexion and extension imaging, how many millimeters of translation are there between flexion and extension?
- Does Chamberlain’s Line cross my odontoid? If so, does it cross at a level that would indicate Basilar Invagination?
- Looking at my rotational imaging, what is the percentage of uncovering of the right and left articular facets on rotation?
- Do the percentages from my rotational imaging meet the diagnosis criteria for Atlantoaxial Instability?
IF A DIAGNOSIS CRITERIA IS MET IN ANY OF THE ABOVE, WE STRONGLY RECOMMEND THAT YOU WAIT ON DECOMPRESSION AND PURSUE THE TREATMENT OF SAID CONDITION(S) AND THAT OF EHLERS-DANLOS SYNDROME, AS EACH OF THESE CONDITIONS CAN BE PATHOLOGICAL TO AN ACQUIRED CHIARI AND EACH IS A STRONG INDICATOR THAT A CONNECTIVE TISSUE PROBLEM EXISTS.
*The questions in this article will periodically change as we are able to expand our recommended questions.
*Original version released September 2018, revised 2023.
Obtaining Your MRIs on Disk
Hospitals and imaging centers in the United States are required to give you a copy of your imaging if you request it.
Many hospitals and imaging centers will give a copy of your MRI on disk or flash drive immediately after your appointment, but they do this as a courtesy and not as a requirement. (Keep in mind that you will still have to wait for a copy of your radiology report and might need to ask for a copy of that separately.)
Those that refuse to give you a copy immediately following your appointment must have procedures in place on how to obtain them. It will generally involve you, or your caretaker with medical power of attorney, contacting the facility’s records department and filling out a specific request form. Many states allow them to charge a minimal charge (usually no more than a few dollars), but most facilities will give at least one free copy. Some can have it ready in a matter of hours after making the request, while others can take up to two to three weeks. Some are willing to mail it to your home and others will require you to pick it up at their Patient Records Dept. (Different countries have different requirements, but most modern countries have procedures in place to request a copy.)
In the United States, records of imaging are legally allowed to be destroyed after a period of time (usually 7 years or less, depending on the state). For this reason, we recommend that you get a copy of all available MRIs and keep a copy for yourself and because disks are so easily destructible, we encourage you to back all of the contents up onto a hard drive and then store the disk away for safe-keeping. Never send anyone the original disk. Make copies as needed and send the copies.
Understanding Your Head and Neck Pain
You Might Have More Than A Migraine If…
High-Pressure Headaches
Those that suffer from high pressure tend to feel pressure behind the eyes (often mistaken for sinus headaches) and report feeling like their “head is going to explode” from the pressure. High-pressure headaches are generally characterized by being worse when laying down – often awaking in the middle of the night or first thing in the morning with a headache, and the headache tends to dissipate to some degree after being upright for a period of time (and that period of time is different for everybody). Caffeine generally exacerbates high-pressure headaches.
- For more on Intracranial HYPERtension: http://chiaribridges.org/brain-pressure-understanding-intracranial-hypertension/
- For a list of common high-pressure symptoms:: http://chiaribridges.org//glossary/symptoms-of-intracranial-hypertension/.
Low-Pressure Headaches
Those that suffer from low-pressure headaches tend to report feeling like there is an invisible pressure pushing down from the top of the head, often making it feel like your “head is going to implode.” Low-pressure headaches are characterized by being worse when upright and relieved by laying down. Low-pressure headaches are typically a sign of a cerebrospinal fluid leak (CSF Leak). The longer that the leak has existed, the less obvious the positional element is – meaning the patient can be upright longer before they feel the pressure at the top of their head, and they tend to need to lay down longer before getting any measure of relief. Caffeine often helps relieve low-pressure headaches.
- For more on Intracranial HYPOtension & CSF Leaks: http://chiaribridges.org//cerebrospinal-fluid-leaks/
- For a list of common low-pressure symptoms:: http://chiaribridges.org//glossary/symptoms-of-intracranial-hypotension/
Occipital Headaches
Chiari headaches are felt at the occiput – at the base of the back of the skull and upper neck. They are generally tussive in nature, where they are exacerbated by valsalva maneuvers, which generally include: coughing, sneezing, heaving, laughing hard, or bearing down (like with a bowel movement or childbirth). These maneuvers reduce cardiac output (the amount of blood coming from the heart with each heartbeat), which in turn affects the attempted flow of cerebrospinal fluid, and it increases vagal stimuli.
- For more on Chiari Malformation: http://chiaribridges.org//chiari-malformation/
- For an expansive review of the name and definition of Chiari Malformation: http://chiaribridges.org//whats-in-a-name-chiari-malformation/
Connecting the Three Headaches
- Untreated high pressure can cause cranial leaks (which leads to low pressure) – often accompanied by cerebrospinal fluid leaking through the nose or ears.
- CSF leaks can sometimes seal on their own leading to rebound high pressure (which is temporary) or continued high pressure if they originally had high pressure.
- Untreated high pressure can push the cerebellar tonsils down into the foramen magnum where it blocks the flow of cerebrospinal fluid, leading to occipital headaches (often diagnosed as a Chiari 1 Malformation) AND in return, the blockage of cerebrospinal fluid further increases intracranial pressure.
- Untreated low pressure can cause the brain to sag and as it sags the cerebellar tonsils can get lodged into the foramen magnum where it blocks the flow of cerebrospinal fluid, leading to occipital headaches (often diagnosed as a Chiari 1 Malformation).
- Untreated spinal leaks can create a suctioning or pulling down effect where the cerebellar tonsils can get lodged into the foramen magnum where it blocks the flow of cerebrospinal fluid, leading to occipital headaches (often diagnosed as a Chiari 1 Malformation).
- All of the above is most common in patients with a connective tissue disorder such as Ehlers-Danlos Syndrome.
Major Problem Regarding Our Diagnoses & Treatment Options:
- Doctors and radiologists alike, tend to see the herniated tonsils and assume a small posterior fossa.
- Most do not check for high pressure or low pressure, even when directly asked and symptoms are present.
- When a posterior fossa decompression is finally offered, the high or low pressure is often left untreated which leads to a failed decompression.
- By the time sufferers get a name to go with their symptoms, we jump at the opportunity for relief.
The “Bobble-head Sensation” – When It Feels Like Your Neck Can No Longer Hold Up Your Head
While most of us experience this feeling either intermittently or continuously, it is generally related to structural instability issues:
- Craniocervical Instability (CCI, also known as Syndrome of Occipitoatlantialaxial Hypermobility) involves vertical hypermobility (back and forth sliding) of the craniocervical junction (interface between the occipital bone and the 1st and 2nd vertebrae), where the neck is no longer properly supporting the cranium. This condition can be dangerous as it often involves brain stem compression that can lead to a vast array of symptoms of Dysautonomia (dysfunction of the Autonomic Nervous System – ANS).
- For more on Craniocervical Instability and Other Related Disorders: http://chiaribridges.org/craniocervical-instability-related-disorders/
- For a list of common CCI symptoms: http://chiaribridges.org//glossary/symptoms-of-craniocervical-instability/
- For a list of common AAI (a condition commonly seen with CCI) symptoms: http://chiaribridges.org//glossary/symptoms-of-atlantoaxial-instability/
- Subaxial Instability (SAI; also known as Cervical Instability) involves hypermobility of the C2/C3 to the C7 intervertebral discs. This condition (like most conditions involving the cervical spine) is a major cause of muscle spasms (in the neck and throughout the body at any point below the disc issues. When these neck spasms occur, they can cause the “Bobble-head sensation” where it feels like your neck can no longer hold up your head. This disc degeneration can lead to paralysis as discs compress the spinal cord.
Important Questions to Ask Your Neurosurgeons: http://chiaribridges.org/important-questions-for-your-neurosurgery-appointment/
Originally written 10/2019
Updated 12/2022
Christmas Presence
Making homemade stockings and cutting flowers for wreaths.
Baking treats and devouring them with hot cocoa by the tree that we spent hours decorating.
Shopping for just the right gifts and wrapping them meticulously, so those I love know just how special they are.
I remember all the traditions that we did together as a family before my symptoms hit hard.
Now, I am reduced to shopping the internet for gifts, but still, I do the best I can to find something special.
I usually start out trying to help decorate, just to end up on the couch watching everyone do what I can no longer do.
I sit here and watch despite the pain I feel from the few decorations I put on the tree because I so want to stay a part of things as much as I can, while I can, even if it’s nothing more than a shadow of what I once was.
Do they know how much I still long to be a part of it all? How much I long to be a part of them?
I sit here and as I ponder how much things have changed and all that I am now, I find myself stuck in my head.
Do they see how stuck I am?
Do they see how afraid I am, that each holiday might be the last that I have with them, and they have with me?
I’ve got to get out of my head and be present.
The best present that I can give them really is just me, fully engaged in showing them that they’re the best that has ever happened to me!
Guidelines for Nonprofessional Opinions (NPOs)
If you decide to post your MRIs for Nonprofessional Opinions (NPOs) at WTF, please make sure that your post/images adhere to the following guidelines. Requests that do not meet our guidelines will be removed by an admin.
PLEASE MAINTAIN 100% PRIVACY TOWARDS THOSE TRYING TO HELP YOU OR SOMEONE ELSE.
We have very strict privacy rules in this group. All images and comments given should be kept in the group or used privately by the owner of the image. Some members that give NPOs are okay with their input being shared, but it is the responsibility of the owner of the image to get permission from the person giving the NPOs before sharing them and all names should be edited out of all screenshots. Violating this rule will most likely result in an IMMEDIATELY removed/blocked from the group/site.
IMAGES SHARED MUST BE YOURS OR BELONG TO SOMEONE IN YOUR IMMEDIATE FAMILY.
Members are not allowed to share the images of their friends outside of this group. Violating this rule will be considered a breach of our privacy rules and will result in the member being IMMEDIATELY removed/blocked from the group/site.
WE DO NOT ALLOW PHOTOGRAPHS OF MRIs.
Photographs of images often contain glares, slants, and other problems that can compromise the image. We want you to get the best information possible here and we don’t want people going on a wild goose chase because the images were compromised in the first place. That will only make your doctors further disregard what you bring up to them.
IN GENERAL, WE PRIMARILY PREFER SAGITTAL VIEWS:
- Sagittal views are slices taken from one side to the other, viewing it from the left perspective. While sagittal is our preference, other views can be helpful as well:
- Coronal views for instance are slices taken from the front to the back (so the left is really the right and vice-versa) and if you find the right slice, it can show if one cerebellar tonsil is lower than the other (it is also better for showing scoliosis).
- Axial views are slices from the top to the bottom, so they can be instrumental in finding leaks (especially when contrast was used), but since a leak can be cranial or spinal and can happen anywhere along the way, you have to go through them closely one slice at a time.
ALL IMAGES MUST BE PROPER MIDSLICES:
When we talk about midslices, we’re NOT talking about the middle slice on the disk (although it is often close to that), we’re talking about the image showing the middle section of your head/neck/spine.
What you need to look for:
- A midslice of the brain (example) should clearly show an unobstructed view of three bones: the clivus, the occipital bone, and the odontoid process.
- A midslice of the cervical spine (example) should show the same three bones, but they will be slightly higher on the image.
- A midslice of the thoracic spine (example) should show the vertebrae and full spinal cord. When scoliosis is present, you will see the spinal cord clearly at one point and then almost disappear (and sometimes you will see it come back into clear view – depending on the location of the curves of the scoliosis).
- The spinal cord does not go as low as the vertebrae in the spinal column. The point in which it ends is called the conus medullaris and from there what continues down into the lumbar region is called the filum terminale. A midslice of the lumbar spine (example) should show the point where the spinal cord ends (conus medullaris) and the filum terminale begins.
*We do make one exception to images being midslice and that is if there is the presence of a mass (cyst/tumor) or lesion (any of which can be anywhere in the brain and may not be visible in the midslice). If that is the only image given, that is the only thing that we will be commenting on (we will not pretend that it is a midslice and comment on it as though it was).
T2 WEIGHTED IS PREFERRED, BUT T1 IS PERMITTED AS WELL:
- On a T1 weighted image, the cerebrospinal fluid (CSF) is dark gray or black.
- On a T2 weighted image, the cerebrospinal fluid (CSF) is light gray or white. Having the CSF show up as white creates a better contrast that makes it easier to see certain things (such as intervertebral disc issues).
Image Size:
Small images make it hard for us to see what we’re looking for and even harder for us to try and mark what we see. Therefore, all images submitted should be cropped into a minimum of 500-800px. To accomplish this, please open the images full screen on your PC and either save them to size or use your snipping tool to snip them while they are large. Please DO NOT simply enlarge the picture as it reduces resolution that can compromise what can be seen.
NPO Markup with the Admin Think Tank:
The Nonprofessional Opinion (NPO) Request Form is required for all markup requests with the Admin Think Tank (no exceptions). Once you’ve submitted the form, you will be added to the waiting list (which is in our Facebook group), and an admin will contact as your name gets close to the top of the list.
