What’s that? Oh, it’s just my hand…

So, this is pretty confusing for most to understand so I will try my best to explain this strange phenomenon to you all. I have migraines. When I get migraines, I lose a large portion of my vision almost every time. Yup, vision…gone. It’s really annoying and hard to explain to people.

Have you ever gotten “floaters” or “seen stars?” If you have, multiply that experience by 25,000 and that’s my vision during my migraine. It’s like a giant burst of colorful squiggly moving worms crowding my visual space and impeding my vision. Sounds fun, right?

So what are these amazingly wonderful migraines called? They are called ocular migraines. These cause vision loss or blindness lasting less than an hour, along with or following a migraine headache according to WebMD. Generally, these happen with only one eye being affected. For me, I am lucky enough to have it affect both of my eyes (sometimes simultaneously).

Ocular migraines are rare. They actually only occur in one out of every 200 people diagnosed with migraines. Many doctors suggest these ocular migraines are caused by other conditions. My doctors and I have not confirmed any other possible reason for my migraines besides my syringomyelia and/or my Chiari type 0.

Here are some of the symptoms of the ocular migraines:

·         flashing lights

·         blind spots in your field of vision

·         blindness in the eye

·         vision loss

·         headache (lasting from four to 72 hours)

o    affects one side of your head

o    feels moderately or very painful

o    feels worse when you are physically active

·         nausea

·         vomiting

·         unusual sensitivity to light or sound

As with any migraines, mine has a tell-tell aura. So what’s an aura? These early symptoms, also called a prodrome, are early indicators that a migraine will be visiting you shortly. Many people have auras with their migraines.

Here are some of the symptoms of an aura:

·         blind spots or scotomas

·         blindness in half of your visual field in one or both eyes (hemianopsia)

·         seeing zigzag patterns (fortification)

·         seeing flashing lights (scintilla)

·         feeling prickling skin (paresthesia)

·         weakness

·         seeing things that aren't really there (hallucinations)

My aura? Well, it’s not one of those listed above. My aura is when I look at my hand and discover it feels foreign to me. It appears I lose a little bit of my proprioception. Proprioception is the unconscious perception of movement and spatial orientation arising from stimuli within the body itself.

I can’t explain this feeling very well. All I can say is that I just don’t feel like my hand is mine. I feel it, I can touch it and my senses are there, but my mind does not register it as being mine. This is something my doctor first informed me I would lose if I were to have a shunt placed to drain my syrinx (cyst in my spinal cord – syringomyelia).

The shunt would not remedy the symptoms I have already developed from the spinal cord damage associated with the syringomyelia, but rather would HOPEFULLY prevent further symptoms from developing. So…not really a guarantee things would be better. I guess that’s why I opted to forgo the surgery to place the shunt. If I were to have the shunt placed, I would completely lose my proprioception. So what does that mean for me?

Some symptoms of Proprioceptive processing deficits include:

·         Clumsiness

·         A tendency to fall

·         Minimal crawling when young or difficulty crawling

·         Difficulty manipulating small objects (buttons, snaps),

·         Eating in a sloppy manner

·         Resistance to new motor movement activities

·         Constantly jumping, crashing, and stomping

·         Loves to be squished and get “bear hugs”

·         Prefers tight clothing, loves rough-housing, may be aggressive with other children

·         Bumps into things often

·         Moves in a stiff and/or uncoordinated way

·         Doesn’t know how hard to push on an object

·         Misjudges the weight of an object

·         Breaks objects often

·         Rips paper when erasing pencil marks

·         May tire easily

Obviously, these are not things a 31 year old mother of (soon to be) 5 children would want to lose. I want to be able to dress my babies and I have enough trouble with onesie snaps without a proprioceptive processing deficit!

 

For me, I’ve learned to live with my migraines and, although they are NOT fun, I know they are just part of life for me. This is just something I have lived with since I was in seventh grade. Thirteen years old with vision loss…that was scary! Luckily, my mother got me into the doctor and they let me know it was ocular migraines. Now I can even work through them for the most part. 

 

If you, or a loved one, have been diagnosed with migraines, please feel free to leave me a comment. Although this blog is dedicated to my life with Syringomyelia, I realize I am not defined by that disorder. I have other issues, sometimes linked to syringomyelia, I hope to share with you all, as well.

 

Thanks for reading. Go forth and be well.

 

Sources for blog entry:

http://www.webmd.com/migraines-headaches/guide/ocular-migraine-basics

http://www.thefreedictionary.com/proprioception

http://sensoryprocessingmadesimple.com/the-proprioceptive-system-and-sensory-processing-disorder/ 

Rare Disease Day - February 28th

February 28^th is known as Rare Disease Day. This day is coordinated by EURORDIS to raise awareness amongst the general public and decision-makers about rare diseases and their impact on patients’ lives. Wondering what makes a disease “rare?” A disease or disorder is defined as rare in the USA when it affects fewer than 200,000 Americans at any given time.

So in the spirit of Rare Disease Day, I wanted to update you all on my rare disease, syringomyelia. I know many of you have been followers of my blog since I was diagnosed in 2011, but some of you are new and don’t have as much information about Syringomyelia as you perhaps would like. This blog entry is dedicated to letting you all know about this rare disorder and spread awareness.

Syringo-MY-Whatia?

Syringomyelia is pronounced (sear-IN-go-my-EEL-ya). We often call Syringomyelia SM for short. SM is the buildup of fluid inside the spinal cord due to many causes. It can be caused by a blockage due to a Chiari Malformation (CM), trauma, tumors, and other causes. When you are unsure of the origin of the Syringomyelia, they call it “idiopathic.” I have Idiopathic Syringomyelia.

What makes Syringomyelia “rare?”

Duke University conducted an analysis which estimates between 100,000 - 200,000 people in the US may have syringomyelia.

What are the symptoms of Syringomyelia?

Symptoms develop slowly over time, but can come on suddenly after a fall or minor trauma. Sensation may be affected first. Some common symptoms include:

·         Loss of sensitivity to pain and temperature

·         Numbness and tingling

·         Bowel and bladder function may be affected

·         Scoliosis

·         Pain in your neck, arms and back

·         Muscle weakness and wasting (atrophy)

·         Spasticity

·         Paralysis (in severe cases, quadriplegia)

·         Loss of reflexes

·         Stiffness in your back, shoulders, arms and legs

·         Muscle weakness and spasms in your legs

·         Facial pain or numbness

Scoliosis may be the only symptom in children. Often people with scoliosis undergo spinal MR imaging because of an atypical left-sided thoracic curve.

Many individuals suffer from chronic pain and some will develop neuropathic pain syndromes. This type of pain is difficult to treat. A large percentage of people have headaches which can be severe.

How is Syringomyelia diagnosed?

To diagnose syringomyelia, your doctor will begin by asking about your medical history and doing a complete physical examination.

If your doctor suspects syringomyelia, you'll likely undergo a magnetic resonance imaging (MRI) scan of your spine and spinal cord. An MRI is the most reliable tool for diagnosing syringomyelia.

How do I cope with having Syringomyelia?

Living with Syringomyelia and the complications it brings can be very challenging. For me and others I know with the disorder, coping has been very difficult. I am not able to do the things I have in the past nor can I have new adventures (like skydive) due to the disorder. Sometimes it feels like a life sentence without the possibility of parole. Chronic pain affects my everyday life and sometimes keeps me from my loved ones. This breaks my heart and I wish it were different.

I was diagnosed in 2011 and I started searching for support almost immediately. I needed to know that someone was out there who understood what I was going through both physically and emotionally. My family was a great support, but no one had ever dealt with the disorder before my diagnosis. I found several groups on Facebook, through the foundations supporting syringomyelia, and through friendships with others affected.

If you have been diagnosed with Syringomyelia, or any other rare disease, I encourage you to find support. You may not want to reach out to strangers, but I urge you to find a way to cope. This disorder is not easy to live with and it’s important to know, you don’t have to do this alone.

Portions of the above information were gathered by visiting these websites:

http://csfinfo.org/education/patient-information/what-syringomyelia-sm/

http://www.rarediseaseday.org/

https://www.conquerchiari.org/education/syringo-faqs.html

http://asap.org/index.php/disorders/syringomyelia/

http://www.mayoclinic.org/diseases-conditions/syringomyelia/basics/symptoms/con-20034245

http://www.mayoclinic.org/diseases-conditions/syringomyelia/basics/tests-diagnosis/con-20034245 

CRUSHED IT! $700 for a CURE!

September is over and so ends the 2013 Crush Chiari campaign for Chiari and Syringomyelia awareness sponsored by the Chiari and Syringomyelia Foundation (CSF). This year was my first to participate as a fundraiser and although I know I drove most of you crazy with my incessant posting, I wanted to share my gratitude with you all for your support. The funds donated through the jars have been tallied and donated to the fundraising site. Those of you who supported me in the Crush Chiari campaign raised $700 to go for a cure! You helped me exceed my goal by $200 and have allowed me the opportunity to be one of the top fundraisers for the cause!

The overall Crush Chiari goal was $15,000 and, although we did not reach that goal, you all helped us raise a lot of money for a good cause. As of this evening CSF has raised $1,665 (11.1% of their goal). Please know that YOUR support helped the foundation raise over 42% of that money by supporting me through my fundraising page.

I want to especially thank the locations who allowed me to place my donation jars at their locations, those of you who donated directly on the #CrushChiari website, those who gave through the donation jars and, most especially, those of you who spread the word by teaching others about these conditions. The support I was given last Wednesday for the wear purple for a cure day was so humbling for me! Those of you who wore purple showed how much you truly cared about me and my desire to find a cure for those of us with this condition. The flood of purple did the trick because every time someone would see those of us matching in our purple attire we could explain how we matched due to the Chiari Awareness Month and we could tell them about Chiari. That was what the whole campaign was about. Thank you for helping me realize my vision!!

I’d like to take this time to thank my family for their dedication to helping me raise money for this campaign despite all of the setbacks we encountered this month. Justin Hair, Ginger & Jim Powell, Amber Love, and Stephanie & Aaron Powell have been the epitome of family during this time. They helped me drop off jars, pick them up, pester you all with posts about awareness and donated not only funds, but their time to support me in this cause. They did this even as I underwent my open-heart surgery last week because they knew how much this campaign truly meant to me. Their support should most definitely be recognized!

The following businesses allowed me to place jars at their locations raising $151.80 collectively for a cure:

Cowboy's General Store

Dismuke’s Pharmacy

Luling Tire & Automotive

NAPA Auto Parts - Salt Flat Feed & Mercantile

Stanley Theater Collectibles

Taqueria Durango

Texas Lutheran University Bookstore

The Watermelon Shop

Plans for Future Fundraising and Awareness for Chiari and Syringomyelia:

Unite@Night, June 2014:

I will be participating in the Unite@Night Walk with the CSF Foundation in June of 2014. I have not selected a date for this walk, yet; however, I would LOVE for you to all participate with me. Here is information from their website about the walk:

“The Chiari & Syringomyelia Foundation’s (CSF) unite@night is a one-mile casual evening walk in various locations around the country and in Canada during the month of June. This walk will bring together people who are suffering with devastating effects of Chiari malformation, syringomyelia, and related disorders such as, but not limited to: Ehlers-Danlos Syndrome, hydrocephalus, intracranial hypertension, dysautonomia, and more.”

You can find more information on their website here: http://www.csfinfo.org/walk-information1/.

Crush Chiari, September 2014:

If you would like to participate in next year’s #CrushChiari campaign by allowing me to place a jar at your business, please send me a message. You can place a jar at your business for any length of time you wish. If you would like to participate only in September, that is fine; however, some have asked to allow the to keep one of my jars at their location year round to raise as much money as possible. I would be happy to make a jar for your location. It won’t cost you anything except counter space and the funds go directly to a great cause!

Conquer Chiari Walk Across America, September 20, 2014:

I plan to do a national walk in September next year to spread awareness about Chiari and Syringomyelia. I will be attempting to set up a walk in the Austin Area. Anyone who wants to help is welcome to contact me. We will begin the process of finding locations in Austin in January. Here is more information:

“The Conquer Chiari Walk Across America is an annual fundraising and awareness event comprised of a series of local walks held on the same day.”

To find out more information about this please visit the website: http://www.conquerchiari.org/ccwaa/ccwaa-main.html.

I don’t plan to give up fighting for a cure for any of these conditions. Chiari and Syringomyelia are a part of my life, despite my utter distain for them, and I want to help others who also have to deal with these situations to have a running start. Raising awareness and funding research is the best way I know to do this at this time. I will continue to fight until a cure is found…and maybe then, I’ll fight to ensure everyone can get the cure no matter his or her financial background. I’ve got a lot of work ahead of me…care to join?

Thanks again for your support! 

Crush Chiari, Not Candy!!

I have talked so many times about raising money for Syringomyelia and Chiari awareness and yet never have…until this September during Chiari Awareness month. I decided I wanted to put my money where my mouth was and donate my money and time to an amazing cause and spread the word about these neurological conditions.

If you wish to donate, please visit: http://csf.kintera.org/crushchiari/mistihair. 

For those of you visiting my blog for the first time, I wanted to share some facts with you about Chiari and Syringomyelia so you have some understanding of what people like me go through daily. Many of us with the condition are unable to have any treatment besides pain management (which doesn't always work). I am not a candidate for the decompression surgery for Chiari (where they remove a portion of your skull to release the pressure) or a shunt for Syringomyelia (where they drain the fluid out of the syrinx [cyst] inside the spinal cord).

About Chiari:

Chiari malformation (kee-AH-ree mal-for-MAY-shun) is a condition in which brain tissue extends into your spinal canal. It occurs when part of your skull is abnormally small or misshapen, pressing on your brain and forcing it downward. 1 in every 1,000 will be diagnosed with a form of Chiari Malformation.

Symptoms of Chiari Malformation include:

Neck pain; Unsteady gait (problems with balance); Poor hand coordination (fine motor skills); Numbness and tingling of the hands and feet; Dizziness; Difficulty swallowing, sometimes accompanied by gagging, choking and vomiting; Vision problems (blurred or double vision); Speech problems, such as hoarseness; Ringing or buzzing in the ears (tinnitus); Weakness; Slow heart rhythm; Curvature of the spine (scoliosis) related to spinal cord impairment; Abnormal breathing, such as central sleep apnea, characterized by periods of breathing cessation during sleep; and several other symptoms.

About Syringomyelia:

Syringomyelia( sih-ring-go-my-E-lee-uh) is the development of a fluid-filled cyst (syrinx) within your spinal cord. Over time, the cyst may enlarge, damaging your spinal cord and causing pain, weakness and stiffness, among other symptoms. 8 in every100,000 will be diagnosed with Syringomyelia.

Symptoms of Syringomyelia:

Muscle weakness and wasting (atrophy); Loss of reflexes; Loss of sensitivity to pain and temperature; Stiffness in your back, shoulders, arms and legs; Pain in your neck, arms and back; Bowel and bladder function problems; Muscle weakness and spasms in your legs; Facial pain or numbness; Spinal curvature (scoliosis); Numbness and tingling; Spasticity; Paralysis (in severe cases, quadriplegia).

You can find more information by visiting: www.csfinfo.org and www.ASAP.org. As I stated earlier, not all of us are able to have treatment besides pain management. I firmly believe that the money we are able to raise in the Crush Chiari campaign will go to fund research and awareness for doctors and scientists. 

For those of you who have already helped me by putting a jar in your business, donating from your own pocket, or helping me spread the word...please know I am so grateful for you and your help! If you cannot afford to donate, please help me spread awareness. I could really use your help in spreading the word! 

Again, if you wish to donate, please visit: http://csf.kintera.org/crushchiari/mistihair. 

The Big Day

It has been quite some time since I have written in this blog, I apologize; however, things have been completely crazy. My son was born in January and I find myself not having much time to update things as I once had. I wanted to update everyone who was interested in how the labor and delivery went since it was not a “normal” birth by any means thanks to my Syringomyelia. Thanks for reading!

I went to the doctor for my weekly checkup on January 16 and discovered that since my blood pressure was high for several days, I should be admitted for observance for at least 24 hours. Following my 24 hours my blood pressure stabilized, but my symptoms were still present. I saw spots and had a massive headache. They noted that in my lab results I had protein in my urine and was in fact diagnosed with Pregnancy Induced Hypertension (PIH). After several emotional conversations with the doctors, nurses and my case manager, we decided it was in the best interest of the baby and me to stay in the hospital until he was delivered.

On Saturday I started feeling very poorly. I started cramping quite a bit and began having strong contractions. They were coming very fast and I was scared because I was only 35 weeks. We were planning to have the c-section moved up to the following Sunday to allow for my son to grow more and for his lungs to mature. They gave me medication and after several hours they stopped the contractions and I was relieved! They stated they would go ahead and complete a second 24 hour urine test the following day beginning at 6am.

The next day I spent feeling the same symptoms as before. The headache had not subsided and it felt as though everything was becoming more prominent. In the afternoon, while completing the urine test, I discovered that there was a considerable amount of blood in my sample. I called the nurses and they told me to get into the bed and hooked me up to monitors. I didn’t feel strong contractions so I asked if I could take a shower and they agreed. At that time I went to the restroom and discovered even more blood this time and started to panic. The nurses came in once I called and said they needed to call the doctor. When the doctor saw the blood, she examined me and discovered that there was even more blood. She said that the amount of blood I had was not considered normal and I needed to have the baby that night. She informed me that I had a placental abruption.

I went into surgery at 9:30pm and was terrified. The doctors, my husband and I decided that it was safer to have the baby via c-section with general anesthesia due to the Syringomyelia. The c-section was necessary due to the placental abruption and the discovery that my son had turned into a breech position overnight (which was likely what caused all the commotion the night before). During the c-section, my anesthesiologists were very careful about intubating me because of my Chiari type 0. A person with a Chiari should not have their heads hyper extended and they were very careful with me to ensure they did not extend my head too far.

When the doctor pulled my son from me, they discovered that the anesthesia had caused problems for him. His heart and breathing stopped and they had to revive him. For this, I am grateful that I was asleep; however, my being asleep is what caused these terrible issues to have occurred in the first place. The doctors got him stabilized and moved me into recovery and him into the NICU. My goal was to hold my son first, and that was no longer a possibility. I did not know that he was taken to the NICU until I was awake (obviously) and I had a very difficult time with this fact. I was not able to see him until the following morning at about 10am. I pushed myself so hard to pass all their physical tests in order to see my sweet son. Following delivery, I had an uneventful stay myself, aside from some stray bleeding from the placental abruption and the obvious pain from delivery. All my pain was masked because I could not have my little boy in the room with me. I was released four days after delivering and did not anticipate the NICU pediatrician would let my son go as we had hoped. We were pleasantly surprised when he stated that he would allow him to come home with us!

I spent 8 days in the hospital. None of these days were related to my Syringomyelia or Chiari malformation. I am not certain of what decision I would have made if I were to do this all over again. I realize that my son was affected by the anesthesia; however, he is here, healthy and in my arms just as I had hoped and prayed, despite the way he entered the world. I have had a strong relapse into the world of pain and discomfort caused by Syringomyelia following the birth of my son. I did not have nearly as many symptoms and felt almost “cured” while pregnant with him. Now I am back to the way I was before my pregnancy. After many doctor visits and MRI’s later, it has been decided that there is nothing anyone can do to minimize my symptoms. For now we are only treating the pain and hoping that one day a medication will help slow the degradation process. It’s not an easy thing to have, but it was the hand I was dealt and I will try to fight this as long as humanly possible – cure, or no cure.

A Rare Glimpse of Vulnerability

I’d like to give you a rare glimpse into my true emotional side of my diagnosis. I try very hard to be positive most of the time but my husband was there last night to witness what a true emotional breakdown is when talking about the fears of what is to come in the future with this disorder. The truth? I’m scared.

So much is unknown. All the research that I have read is merely speculation of what “could” happen to me because each case is completely unique. The possibilities range from no symptoms (which is obviously not my path) all the way to death. I guess it just troubles me when I think about how devastating my diagnosis really was in March 2011. I really want to just forget about my diagnosis and live life as a normal adult, but my symptoms don’t allow me to do that these days.

I have the delivery of my son coming up and I’ve had what they call a patient care conference at the hospital. My case is quite rare and the room was full of doctors and medical professionals offering me some sort of insight into what my delivery might be like in the coming months. They discussed that there is not a lot of research for women with my diagnosis who have become pregnant. Luckily, I have a support group that is amazing and full of women who also conceived when diagnosed with Syringomyelia and/or Chiari. One doctor (anesthesiologist) felt that I could handle a natural birth, while my obstetrician and perinatologist both recommended a c-section delivery. The meeting went on for an hour and a half discussing both the pros and cons of both sides. In the end, we all agreed that a c-section would be the best choice for me. The next decision will come from the type of anesthesia I will be given, whether it is general (completely asleep) or an epidural.

Yesterday proved to be a difficult one for me. I realized that I am less than two months from bringing my son into this world and I am not sure how it will all play out for me physically. My greatest fear is to not see my son being born due to a possible general anesthesia delivery. The other major fear is that I may not be able to fully care for my son the way most mothers are able due to my physical constraints. What the doctors and I completely agreed upon is that my son is in wonderful health and that my concern is not on how HE is going to handle delivery but more so, how I am going to handle it with my condition. We discussed that pregnancy does not worsen my condition, but potentially a natural delivery could.

Colin, our new son, is doing amazingly well. He is growing quickly and, as of our last ultrasound, weighed 3lbs 5ounces at 28 weeks. This is approximately one and a half pounds larger than the other babies in the same week. We will be 30 weeks tomorrow and will have seven weeks until our little Colin comes into our family. Through all of the fears, I am more anxious to meet my little boy and bring him into our already loving family full of wonderful children. Colin will be our final child and will give us two girls and two boys. I’m truly blessed even through all of the pain and anguish the diagnosis brings me.