Six Weeks Post-Op

Please consider donating to my medical expense fund.  Surgery is expensive.  So is recovery!  http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820   Thank you so much to everyone that has donated!  This wouldn't be possible without your help!!!

Things are continuing to improve, just very gradually.

I've had two unexpected improvements from surgery:
1. My digestion and food sensitivities have improved a lot!  I seem to tolerate gluten okay, at least small portions.  I can also tolerate small amounts of dairy without a problem!
2. My hormone regulation has improved.  (I formerly had amenorrhea for 1 1/2 years, but that has resolved.)

I don't know how to explain either of these things really, but I appreciate being a little more normal.

Post-surgical issues are still improving gradually.  This includes my POTS symptoms, my muscle tightness, my surgical pain, my muscle weakness, my endurance, etc.

The POTS symptoms are the most challenging, functionally.  These include:
-unstable blood pressure
-unstable heart rate
-lightheadedness
-palpitations
-shortness of breath
-tightness in chest
-extreme fatigue
-exercise intolerance

These symptoms affect my life a lot.  The mornings and early afternoons are still when my POTS symptoms are worse.  Also, if I overdo my exercise or activity level, it may cause an episode later that day, or not until the following day.  I don't get any immediate feedback from my body that I am overdoing it.  So I have to carefully measure and track the amount of activity I have each day, and the amount of time spent upright.

Surgical pain and muscle tightness are still daily (and nightly) issues, and will probably remain issues for some time...especially as I try to become gradually more active.  The pain is worse when I sit up and when I move.  I also have trouble with bumpy rides in the car.  I don't get out much yet.  So I also have to find a balance between increasing activity, and pushing my body too hard.  Sometimes, I overdo it, and the pain becomes too much.

I finally got my bone growth stimulator today!  It is an electromagnetic device that I wear over my collar.  I am to wear it for four hours a day, for several months.  I will continue to wear it until I have x-ray confirmation that there is a successful bony fusion.

Thanks for still caring, after all this time. 

I have Chiari; why should I be concerned over EDS or Tethered Cord?

Compiled by Robin Armstrong Griffin

When I hear a member in the support groups is diagnosed with Chiari the next question they often ask is why then should I concern over Ehlers-Danlos Syndrome (EDS) or Tethered Cord (TC)? Isn't chiari just caused by a small skull so the brain is forced down?

Chiari is a hind brain herniation of the cerebellar tonsils/lower brain down out of the skull and into the neck.  It is caused by a malformation of bones within or near the skull or the pulling from below by Tethered Cord Syndrome, (explained below). In other words, chiari is a symptom caused by a malformation/underlying condition of something else. And a small skull is not the only condition that can be present to cause it, which many doctors feel is the case. So knowing the true underlying condition is key to the proper treatment of your Chiari.

Ehlers-Danlos Syndrome (EDS) is a connective tissue disorder. Connective tissue acts like "glue" in the human body, holding everything together. Collagen, a protein that adds strength to the connective tissues, is not produced correctly in an individual with EDS. The resulting weak connective tissues are too weak to hold the organs, ligaments, joints, etc.of the body in place. Ehlers-Danlos Syndrome is currently separated into 6 major types. Hypermobility, Classic, Vascular, Kyphoscoliosis, Arthrochalasis and Dermatosparaxis. More and more individuals with Chiari are being found to also have EDS and/or Tethered Cord (TC). (The EDS and/or TC being the CAUSE of the hind brain herniation/chiari.) Although at risk for any type, Hypermobility or Classic are seen the most, some the more rare Vascular type.

Individuals with Ehlers-Danlos Syndrome (EDS) are at risk for cranio-spinal instability (CSI), which often results in cranial settling. In cranial settling the tissues in the neck are so weak (due to EDS) that the cervical bones are not held in place. Slumping or collapsing onto itself occurs. These usually leads to abnormal angles/malformations of various bones and this can lead to both anterior and posterior brain stem compression. Many chiari surgeries (Decompression) fail because only the posterior brainstem compression was addressed. And since most have an MRI laying down the true picture isn't seen because the patient wasn't upright for gravity to reflect to true image. Hence the importance of upright MRI's. These malformations force the cerebellar tonsils out of the skull into the spinal column through the small hole at the base of the skull called the foramen magnum, a phenomena called cerebellar herniation, AKA Chiari. When the cerebellar tonsils are out of place, they take up space normally allotted for the cerebral spinal fluid (CSF), the brainstem, and all the arteries and veins leading through this area to the brain. Blockage can be both posterior and anterior. The cerebellar tonsils are the lowest part of the cerebellum, which controls the sensory perception and motor control systems of the body. The brainstem controls the autonomic nervous system, or everything your body does without you having to think about it, heart rate, respiration, blood pressure control, etc. Damage to these systems is very serious. CSI may also allow the Odontoid bone at the front of the neck to angle into the brainstem (Retroflexed Odontoid), causing even more concerns, such as Basilar Invagination or Basilar Impression. (Brain Stem Compression).

Postural Orthostatic Tachycardia Syndrome (POTS) is a type of dysautonomia. It is also commonly seen with individuals who have EDS and EDS with Chiari as a result of brainstem concerns. In addition to this, an individual with EDS and instability is at risk of subluxation (partial dislocation of bones) of the neck or abnormal sliding of the skull in flex/extension, further compromising the brainstem, tonsils and vertebral arteries. Making this life threatening. When you flex and extend your head, your skull should shift up to 1-2mm, with instability your skull can exceed that, (Stephens' is 8mm) further endangering the individual and for some is even life threatening.

As shared from Dr. Clair Francomano at Harvey Institute of Genetics. "The term "Chiari Malformation" is used to describe the situation where the cerebellum, a structure at the base of the brain, is extending through the opening at the base of the skull, which is called the foramen magnum. The extension through the foramen magnum is called "herniation." This can result from cranial settling, which "pushes" the cerebellum through the opening, or tethered cord, which pulls the cerebellum from below. The conventional definition of a Chiari Malformation states the cerebellum tonsils must protrude at least 5mm below the foramen magnum. However, patients with less extreme herniations can also experience significant neurological symptoms involving the brain stem and cerebellar functions.

A wide variety of neurologic symptoms include headaches, neck pain, tinnitus, swallowing difficulties, visual disturbances, a sensation of pressure behind the eyes, autonomic nervous system dysfunction (POTS, neurally mediated hypotension), sleep disturbances, brain fog and memory issues may result from Chiari Malformations.

Another complication of hereditary connective tissue disorders is called occult tethered cord. In this condition, a band of connective tissue called the filum is wrapped around the base of the spinal cord and exerting pressure on the nerves that go to the legs, pelvis, bladder and bowel. This condition may cause numbness in the legs and pelvis area as well as severe incontinence as well. Because of the hyperextensibility of the connective tissue in patients with this disorder, the filum may become stretched out over the years and is not visible on an MRI. This is why it is called "occult", it cannot be seen using current imaging techniques. Diagnosis is usually made based on clinical findings and also the consequences of tethering which may be seen on a cervical and brain MRI, affecting the angles between specific landmarks in the spine and skull (Milhorat TM et al. Association of Chiari Malformation type 1 and tethered cord syndrome: preliminary results of sectioning filum terminale. Surgical Neurology 72: 20-25, 2009). At this time, it is not known why this condition occurs more frequently in patients with hereditary disorders of connective tissue."

The problem is if you have EDS and it isn't known and you have decompression, ESP with laminectomy, it adds instability. This can greatly effect the success of the decompression because you've added more instability and only addressed the posterior issues and not the anterior issues often seen with cranial settling. Often times those with EDS & Chiari need fusion done at the same time or known to be needed at a later time or tethered cord release done first or soon after. Many also chose to wear cervical collars to help with the instability symptoms and pain. The decompression alone often treats only a part of the issue, not all and even if CSF flow is restored posterior the pain and neuropathy issues still remain due to cranio-instability and continued brainstem compression in other areas or tethered cord still pulling the tonsils from below. Thank you for reading!

Resources: Dr. Clair Francomano, Harvey Institute of Genetics and www.MedicalZebras.com

1 Month Post-Op

Please consider donating to my medical expense fund.  Surgery is expensive.  So is recovery!  http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820   Thank you so much to everyone that has donated!  This wouldn't be possible without your help!!!


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This has been a really interesting time.  Last Monday, we had to let go of our sweet dog, Jango.  He lived a very happy life, but was in a lot of pain at the end.  I am glad that we could at least relieve his pain.  He was a wonderful spirit, full of joy, and we miss him dearly.

As part of the healing process from this loss, we decided to get a new puppy.  He is a delight, and helps to fill the emptiness that we are feeling.  His name is Dexter.  He is an 8-week old Coton, and he loves to cuddle!

I got my stitches out on Thursday.  What a relief!  The stitches were very embedded after a month in my head, so it took quite a while to work them out, but it feels so much better now.

My dysautonomia is my biggest problem at this point.  My body is still very unstable and unpredictable.  My heart rate jumps up, my blood pressure drops, or I become suddenly short of breath or have heart palpitations.  I also experience reduced consciousness at times...particularly in the early hours of the afternoon.  I am on several medications for this, but they don't always help.

I am exercising daily to try to improve the symptoms of dysautonomia.  I have to exercise sitting down, so the recumbent bike is perfect.  But I have to be very careful.  If I overdo it one day, the next day may be a very sick day with low blood pressure and reduced consciousness.  So, I have to be very careful about exercising the same amount every day, and barely ever increasing.  At this point, I can do 11 minutes, twice a day.  This will probably take months to improve, with a lot of hard work and persistence.

The pain is still being managed with Tylenol.  I'm taking 1500 mg of acetaminophen a day, only 3 tablets (when 8 is the daily maximum).  This keeps most pain manageable.  I've still had a few migraines that require separate migraine medicine.

The muscle spasms are still the biggest cause of pain.  I still cannot tolerate any muscle relaxants.  So, I'm using heating pads, topical creams (China-gel), and massage to relieve the pain.  The pain still feels very similar to migraine pain, but in a slightly different location than a normal migraine.  I can usually tell them apart now.

My eyes were injured during my 16 hours under anesthesia.  I got large corneal abrasions across each eye.  The intense pain only lasted a few days.  But I still get burning, dry eyes many times a day.  I'm not ready to wear my contact lenses yet.  I believe they are healing, but not healed yet.

I also developed large areas of numbness on each thigh from surgical positioning.  The numbness was complete at first--no sensation for temperature, sharp/dull, or pressure, besides very deep pressure.  Gradually, the feeling returned, but these areas became hyper-sensitive to touch and quite painful.  These areas are still healing.  The pain is decreasing.

Ready for the good news?  My digestion seems to be improved since surgery.  I am having less food sensitivities and less digestive disturbance and pain.  This is an unexpected improvement!

My swallowing is still improved, but no longer perfect.  I still have aerophagia at times with large amounts of water.  It is usually mild, currently.

So, given the complexities of this recovery, here is what daily life is like for me right now:

I need to sleep about 11 hours each night.  That's about 11:00 pm to 10:00 am.  I wake up once each night.  As long as I can get back to sleep, I'm fine.  If something disturbs my sleep, my dysautonomia symptoms will flare the whole day.

My mornings are a bit better recently.  If I take my morning meds right away, including my salt and extra fluids, my blood pressure is usually pretty good, and my heart rate is mildly elevated.  I need to spend the first few hours of the day mostly horizontally.

After lunch, I seem to have a flare of autonomic symptoms.  My heart starts racing, my blood pressure drops, I become short of breath, and I experience reduced consciousness, or even temporary loss of consciousness.  We manage this as well as we can with medication, salt, and elevating my legs above my heart.

On a good day, these symptoms will improve by dinner time.  I can sometimes eat dinner at the table, something I could never do before surgery.  Sitting up in a chair has always worsened my symptoms.  So, I think this is a really good sign for my long-term recovery.  I also get my exercise on the recumbent bike at this time of day.  I still sometimes get shortness of breath or an elevated heart rate, but I usually stay fully conscious.  I can spend some time upright during this window.  I still have to rest frequently.

I still get frustrated by my symptoms, especially on bad days, where I feel like I'm not getting better at all, or when I spend a lot of time really out of it.  But overall, I am satisfied with my progress so far.  I just have to keep reminding myself that recovery takes a very long time, and this is just the beginning.  I know there are good times ahead for me.  A new puppy helps!  I really look forward to being able to move back in with my husband, even though my mom and step-dad are taking such good care of me!

Thanks to everyone for caring and supporting me.

Pics from surgery

Please consider donating to my medical expense fund.  Surgery is expensive.  So is recovery!  http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820   Thank you so much to everyone that has donated!  This wouldn't be possible without your help!!!


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Here's some photos of hospital and home recovery.  As always, I can't seem to control the order of the photos.  So it's more of a collage.  Recovery is slow, but I do see some tiny improvements overall.  I'll update more tomorrow, after my stitches finally come out!!!

3 weeks post-op

Please consider donating to my medical expense fund.  Surgery is expensive.  So is recovery!  http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820   Thank you so much to everyone that has donated!  This wouldn't be possible without your help!!!

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Today marks 3 weeks since my operation.  Yesterday, I got out of the house for the first time--went to Portillo's for dinner with my mom and stepdad.  There's a lot of sensory overload in the outside world, but it was really good to get out.  This would have been inconceivable just one week earlier, when I was barely conscious all day, wondering if I would ever be well enough to get out of the hospital and get back home.

Today, my mom washed my hair--carefully, with the bandage covering up the incision.  The stitches are still in for one more week.  Can't wait to get them out!

I've been gradually building up the amount of active time each day.  I have to be so careful, because if I overdo it, especially in the early hours of the day, I pay for it later with reduced consciousness.  But I have started getting some things for myself (some of my food and water refills).  I've also gradually built up my time on the recumbent bike.  Today I rode for 11 minutes, two times.

My tachycardia is improving very gradually.  But my blood pressure has been stable since discontinuing all muscle relaxants on Tuesday.  That means I've been conscious all day for three full days!  :)  I'm not saying I'm 100% alert all day, or that I would make a great conversationalist at noon.  But, I'm awake, aware of my surroundings, and able to respond when called all day.  (This is with the help of Midodrine and Florinef, along with salt pills, to keep my blood pressure up.)

The muscle tightness in my neck and shoulders is still problematic, and quite painful.  I haven't had a full-blown migraine since Sunday.  Just migraine-like pains from the muscle contractures.  I'm trying to arrange to have dry needling sessions in May to alleviate this.  Until then, it's heat packs, icy hot, and massages.

I've been noticing that I sometimes have started swallowing air with my water again.  I'm trying to ignore this fact for now.

The most difficult thing has been that my beloved dog, Jango, has become very sick, suddenly.  He has some neurological symptoms, and he frequently cries out in pain.  I have cried most of the day today, because it is so hard to watch my happy little buddy become so miserable.  We know there isn't much time left for him.  Watching Jango in pain gives me even more appreciation for my caregivers.  Watching someone you love suffer is so painful.  And my caregivers are amazing!  I am so lucky to have such wonderful support.

I am also grateful every single day that I survived this whole ordeal.  This hospital stay did some damage to me, physically and psychologically.  But healing is possible.  I will get stronger.  The psychological wounds may take a bit more time to heal...

Sorry to end on a down note.  I am an emotional wreck.  But I am so happy to see little improvements in myself each day.  Recovery is slow, as expected, but improvements are still noticeable.

Unpredictable Recovery

Please consider donating to my medical expense fund.  Surgery is expensive.  So is recovery!  http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820   Thank you!!!

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It's not like this is my first major surgery.  But it seems like it, since my body is reacting so differently this time.  So many unexpected complications and problems.  The primary unexpected problem has been adverse reactions to medications that I tolerated only 3 years ago.  I have developed severe episodes of hypotension/loss of consciousness after taking several medications.  The medications that have caused this problem in the last 3 weeks are: Morphine, Oxycontin, Flexeril, and Zanaflex.  Basically, the good pain meds and the muscle relaxants are all out.  That makes recovery very challenging!!!  But these episodes are so scary, for me and for my family, that I am scared to take anything for pain and muscle spasms.

It is really hard to face this recovery without pain meds or muscle relaxants.  The muscle contractures and spasms are so severe that they are triggering migraine-like pain and actual migraines.  So, I'm in a lot of pain most of the time.  I use heat on my neck and shoulders, along with topical creams and massages.  If anybody wants to give me a massage, any day of the week, I welcome it!  I wish I could afford daily massage therapy.  It helps reduce the pain dramatically.  But, of course, insurance doesn't cover my massage therapy.  Also, when I am well enough to go back to Milwaukee, I have a physical therapist that does dry needling therapy on my muscle contractures.  I'd really like to get back to her as soon as possible.

The last complication to deal with is the POTS symptoms.  I wake up with my heart going 105 bpm.  It peaks around 140 standing, 120 sitting.  It's worst in the mornings, then gradually decreases throughout the day.  By the evening, my heart rate is at a happy 72 bpm, and I can finally catch my breath.  I'm taking Florinef and Midodrine for my POTS.  And I'm using the recumbent bike to try to improve my conditioning.  I also take salt and high fluid intake throughout the day...all just to get my cardiovascular system to calm down.  The POTS will take months to improve, but I've gotten better before, and I can do it again.

I haven't ever had to recover from this major surgery without pain meds and muscle relaxants before.  So, that is my new challenge.  It is a long, long road.  So, I'll just keep going.  My family has been amazing with their support.  I am so incredibly lucky to have them to help me through this.  Thanks to them, my attitude has remained quite positive most of the time.  As long as I can remain conscious throughout the day, I feel like I can get through anything.  Although I really hope to not get another migraine that reaches a 10 on the pain scale.  That would bring anyone down.