Friday, April 29, 2011

Alexis' Chiari Surgery Video 3-14-2011

Zipperhead Chiari in Children :( Alexis is in this video

http://youtu.be/uo9yez08H0c

Living with Chiari Malformation~Several Symptoms Alexis is having & what to look for!

Chiari Malformation

Chiari I Malformation is defined by a volumetrically small posterior cranial fossa (the compartment in the back of the skull), which leads to overcrowding of the cerebellum and brainstem. This causes a blockage of the flow of cerebro-spinal fluid through the foramen magnum, making it difficult for the fluid to flow around the brain and in and out of the skull. In many cases, the cerebellar tonsils are pushed downward through the foramen magnum. However, this is not a necessary part of diagnosis.

Chiari is generally considered congenital, although certain things like trauma and spinal taps have been shown to cause an acquired form of Chiari. Chiari has come to be better known with the advent of the MRI, leading to more people being diagnosed.

The symptoms of Chiari are caused by raised intra-cranial pressure and compression of the cerebellum and brainstem. Again, it is important to note that symptoms are not caused by herniation of the cerebellar tonsils, thus the length of herniation does not correlate with the severity of symptoms. Chiari symptoms may arise at any time in a person's life. Sometimes, symptoms are brought about by trauma (like surgery, injury, or childbirth), by growth spurts, or by illness. Other times, it is unknown what causes symptoms to set in. The following symptom list is from chiarione.org, and is the best compilation I've found. Please note that these symptoms are reported by patients, so they may be symptoms of related conditions in addition to strictly Chiari symptoms.

* Headache (esp. if daily or at lower back of head)

* Painful tension in neck

* Fatigue

* Migraines

* Dizziness

* Visual disturbances/loss of vision/spots in vision/double vision

* Tingling/numbness in the extremeties

* General imbalance/clumsiness

* Memory loss

* Intolerance to bright light/difficulty adjusting to light change

* Vertigo from position change or sudden standing

* Difficulty walking on uneven ground/feeling ground under feet

* Poor motor skills

* Pressure/pain in the neck

* Pressure/pain behind the eyes (soreness in the eyeballs)

* Back pain

* Neck spasms

* Insomnia

* Ringing in ears (like the tone heard in a hearing test)

* Tingling/crawling feeling on scalp

* Intolerance to loud/confusing sounds

* Decreased sensation to touch in extremeties

* Decreased sensitivity to temperature

* Pain & tension along ear/eye/jawline

* Difficulty swallowing/lump in throat/sore throat/swollen lymph nodes

* Spontaneous vertigo

* Hand tremors

* Poor blood circulation/cold hands & feet

* Sinus/mucous problems

* Sleep apnea

* Pressure in ears

* Nausea

* Difficulty reading/focusing on text

* Depth perception problems

* Burning sensation in extremeties/shoulder blades

* Fluid-like sound in ears (like water running)

* Loss of sexual interest/lack of sensation in pelvic area

* Pulling sensation while sitting/standing

* Intense itchiness w/profuse sweating

* Gag reflex problems/lack of gag reflex

* Pressure/tightness in chest

* Loss of bladder control

* Frequent urination

* Dehydration/excessive thirst

* Dizziness

* Loss of smell/confusion with sense of smell

* Dry skin and lips

* Sudden/abrupt changes in blood pressure

* Hiccups (in higher frequency/severity)

* Low body temperature/sudden changes in body temperature

* Strangling feeling

* Floating sensation

As you may be able to tell, the variety of symptoms is astounding. This is one of the major causes of misdiagnosis and underdiagnosis. Also, many doctors are not well-educated about Chiari, especially about the most recent definitions and standards for diagnosis. Many neurologists are unaware of the condition or its severity. Many radiologists will note Chiari as an "incidental" finding if they are not well-informed. Also, even many neurosurgeons may not believe Chiari is the cause of the patient's symptoms. In particular, they may be using the former standard that the cerebellar tonsils must be at least 5mm below the foramen magnum. Doctors now know that the length of the herniation does not correlate with symptom severity. In order to get diagnosed and treated properly, it is best to go to a neurosurgeon with a great deal of experience with Chiari.

The treatment for Chiari is called a decompression surgery. This consists of a craniectomy, a laminectomy, and/or a duraplasty. A craniectomy is where they remove part of the base of the skull to enlarge the foramen magnum, which is the passageway between the skull and spine, to make more room for the flow of cerebro-spinal fluid. A laminectomy may also be done, where they remove the arch of one or more vertebra, also to open up the posterior fossa space and decrease crowding in the hindbrain area. Additionally, sometimes a duraplasty is done, in which the dura (the lining of the spinal cord) is opened and replaced with a patch, allowing for extra space for the flow of fluids.

Sunday, April 24, 2011

Alexis in her new brace! Right before Easter

Alexis in her pretty Ice Age Blue brace. She doesn't feel so pretty in it. And not looking forward to the warmer weather as she is already hot in it. Today was the warmest weather we have had since last summer and she was melting in the heat in the brace!
I hate having to being the one to say, Yes, you have to wear this thing!
Still praying for a cure.

Tuesday, April 19, 2011

April 17th Incision Photo's 34 days Post surgery

Aunt Phyllis and Uncle Harold...and one of our favorite Nurses, Eric

Alexis was busy working on a sweet card for Aunt Phyllis. And then they showed up for a visit. She brought Alexis a pillow pet. And she still sleeps with it to this day :)
We were so lucky to have them visit while we were in Spokane.

Love you Aunt Phyllis and Uncle Harold!!!!!!

ART Sleep More ART!!! WoooHoooo!

One of the many things that Alexis loves, is ART! This was one of the 1st things that brought a Smile to her face.
She made a few projects while she was in the hospital! They have an awesome program there.

Monday, April 18, 2011

Signs and Symptoms of Syringomyelia

Signs and Symptoms

Dissociated sensory loss - pain and temperature are disrupted whereas light touch, proprioception, and vibratory sensation are often unaffected. Sensory disturbance is almost always asymmetric in a cape-like over the shoulders and back. Pain is frequently felt in the neck and shoulders.

Central Cord Syndrome: Centrally located syrinx, that injures the fibers located centrally (cervical) before the fibers located laterally (thoracic, lumbar, and sacral, in this order) in the spinal cord. A cervical syrinx often results in early symptoms (sensory loss and motor weakness) of the more distal muscles of the fingers and hands followed by late signs in the proximal muscles of the shoulders, trunk, and later the legs. A thoracic syrinx may cause motor weakness and sensory disturbance of the trunks followed by the legs.

Brainstem symptoms and signs: When the syrinx extends into the brainstem (syringobulbia), symptoms are common early on in the process. These usually consist of dysfunction of the lower cranial nerves with coughing, aspiration, and dysphagia.

Scoliosis: May result from unequal weakness of the spinal muscles. Young age, atypical curve, rapid curve progression, and back pain associated with scoliosis should alert one to the possible role of syringomyelia.⁹

Chronic pain: is associated with a variety of chronic pain syndromes, particularly in posttraumatic syringomyelia, in which there is an initial traumatic injury followed by later spinal cord injury secondary to syrinx distention; however, while it is nearly impossible to determine if the pain is caused by the syrinx or the primary pathology, it appears that treatment of the syrinx rarely results in resolution of the pain, which suggests that the pain may be related to the initial spinal cord injury.

Quick Facts About Chiari Malformation:

Quick Facts About Chiari Malformation:

Affects more than 300,000 people in the US; 30% of children born with spina bifida are also born with a Chiari Malformation.
Causes a wide ranging, diverse set of symptoms. Research has shown that 95% of patients experience at least 5 symptoms.
Diagnosed using combination of MRI, neurological exam, physician’s judgment, and other tests; patients often go 5 or more years before being properly diagnosed.
A study of over 300 Chiari Malformation patients found that 57% had at one time been told by a doctor they were suffering from a mental or emotional problem.
Many patients undergo serious brain surgery in an attempt to stop symptoms from progressing.
Research has shown that surgery can improve symptoms for about 80% of patients, but fails about 20% of the time.
Chiari can place a tremendous physical, mental, emotional, and economic strain on patients and their families.

Chiari I malformation: Diagnosis made on MRI. Syrinx associated with Chiari I malformation is usually treated with posterior fossa decompression. If the syrinx does not resolve, one would consider (re-explore the posterior fossa and expand the decompression; consideration of subtle craniocervical instability; consideration of benign intracranial hypertension; consideration of shunting the syrinx directly; others).

Sunday, April 17, 2011

Shannon and Syd's visit!

Shannon and Sydnie came a few times to see Alexis. Syd made the cutest card! Thanks you guys, we love you tons!!!
It's a bonus to get to see you when we are in town for our several visits! HUGS! :)

Visit from Grandma Jan and Grandpa Ed

Grandma Jan & Grandpa Ed. This was a busy day...we had Travis & Russ visiting, and my Mom there as well.

And then the last picture is the removal of the bandage. And our first look at Alexis' incision! OUCH!!!!!! My poor sweet girl!

We love you so much, Alexis!

March 17th week....oncology floor

The view is from Alexis' room 316. It was a nice view. I slept right below this window when I wasn't in the chair next to her.
The picture of the gifts....just a few of some Alexis received in the hospital.
And Alexis' only source of food while in the hospital for the most part! :)

Room 316

Wednesday, April 13, 2011

The Look of FEAR!!!!!

Need I say more....she's scared!

Mommy's point of view from Chronically Ill Child...

So, I wanted to update a little bit.   Yesterday was a visit to Shriner's Hospital for the Body cast that they are making for her.
Yes, we went with the hard cast for now to see how it does. We still may go with the Spine Cor brace. I'd rather have Alexis in that so she is more movable and comfortable! And I don't want her muscles to Atrophy! And being in a hard brace is difficult to move the way you normally do.
I am sure this summer will be NO FUN being in a HARD Plastic brace!

So, yesterday was quite the event. Alexis ended up vomiting. We were at my Good Friend's house. And so happy to see her. Then Alexis started getting sick :(
Now, if this was just a normal kid, no worries. But since Alexis just had surgery on her brain, and they cut open the Dura....that is another story!

We put in a call to the Neuro. And about an hour or 2 later, they said, it should be ok. But they have some things they can do to help her.

I chose to wait since she seemed to be ok.   Shortly after that call. Alexis was back vomiting again.
I got ahold of some of the mom's who were able to guide me and tell me their thoughts!
So, I made the decision to bring her into the Sacred Heart Children's ER.

We got there and there must have been 20-30 people ahead of us. I thought, Oh great! This will be a long time to wait.
But they had me sign a sheet stating the nature of the visit.   And then another form. As I was filling out the 2nd form, they read the 1st and that quick, they said, we need to get this little girl back right now.

I could hear them talking in the back ground and mentioning a shunt! I Was a little scared.

I was the only one there with Alexis. Yet I had to keep my cool for her!

They said they wanted to do a CT (cat scan) of her head. To make sure there was no blood on her brain!

Now, Alexis was terrified of the MRI. And we had to put her to sleep for that. She was so scared to do the CT!
But she did great. It was fast.

No blood on the brain! WHEW!!!!!!   They think that the bump on the head was the cause of all this. But who knows.

She stayed home today. And as she was doing her math fractions, she was grabbing her head. She said, Mommy my head is hurting!

OH MY!!!!!!!!
What else....there is no manual out there on how to help your child who is chronically sick!   I don't know what to do and how to help her.
And I sure as heck don't want to ask just anyone for help! Meaning the Dr.'s here. They don't know about this disease. And they don't know how to help.

There is No Neuro in Pullman....and it is a drive up to Spokane for the help!

I AM EXHAUSTED! Being a mommy is a lot of work.   And I've been doing my best to give Alexis the ABSOLUTE BEST CARE!
When it comes to the brain, and spine, I don't take chances.   I KNOW kids are fast healers and resilient. But my Kid isn't just any kid.   Trust me, I've been researching this info trying to do the right thing.
It's Not as simple as ya'll might think. So please, bear with me. I'm doing my best. :)

I think I hit my breaking point last night. I am totally and completely worn out. I wish I had several friends who live out of town closer to me ~!
Mommy's need support, too!