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Allison's Story

From: Allison Ruggles, allisonruggles@hotmail.com

Sent: June 26, 2009

My story really begins when I was cured.  But first a little background.

Compared to many, my journey to diagnosis is quick.  I have a few minor neurological symptoms in 2004, slurred speech and facial numbness that quickly fade.  A trip to a neurologist for unrelated back pain reveals nystagmus during the basic neurological exam.  This leads to a MRI.  After a couple of tense weeks believing I have a brain tumor, I am diagnosed with a CCM in the brainstem – specifically the Pons.   

It is February 2005. 

The CCM is surrounded by healthy brain tissue.  That plus my healthy state makes the conservative approach the only option.  2005 is really about follow-up and education.  My life is 95% normal but I am not working.  Like all of you, I find the Angioma Alliance and eventually volunteer as the registry nurse for the bio bank.  

In 2006 my CCM begins acting up again.  New neurological problems include vision problems (nystagmus, oscillopsia, double vision), facial numbness, short term memory loss, unsteady gait, and slurring of speech.  The MRI confirms that the lesion expanded in size and intensity indicating recent bleeds.  It begins impacting my personal life, I have a couple of car accidents limiting my driving to good days, I can no longer run, fatigue and memory loss became issues.  Appointments are missed.  Children are left waiting at school.  Fine motor skills are going and we begin to lose dishes to slippery fingers.  The doctors say exercise will not cause bleeds but exercise wipes me out. 

Bleeds again in late 2006 and early 2007 start to turn the tide against the conservative approach.  My gait is impacted.  I sometimes stop talking in mid sentence. I am feeing tired all the time and have to lay down quite frequently. The frequency of the bleeds is problematic.  The CCM is no longer surrounded by healthy tissue but has bled to the surface of the Pons.  2 neurosurgeons agree that surgery is warranted, another thinks it will be inevitable, but would defer for now.  The decision is risk of debilitating stroke versus risk of surgery.  The scale tips in favor of surgery.  A date is set and that is when my story really begins.   

On April 4, 2007 just over 2 years after my diagnosis I have surgery to remove a CCM from the Pons.  Entering from the base of my skull, and through the fourth ventricle, the surgeon moves up the brain stem to the point where my CCM had bled to the surface of the Pons. 

The six hour surgery is deemed a success, the surgeon is confident he got all of the CCM and this is backed up by subsequent MRI’s.  As a sporadic, this is my one and only CCM. 

Post surgery I am conscious and in neurological ICU.  My chief concerns are whether they got the CCM and if the tissue donation was processed correctly.  I don’t ask and they don’t mention the new deficits.  I touch my face a lot and people are having trouble understanding me.  I cover one eye in order to see. 

If you are a fellow brainstem you know already the tangle of nerves that originate and pass through the brainstem.  This, “expensive real estate”, as they call it does not react kindly to any intrusion, be it a hemorrhage or a surgeon’s scalpel.   

In my case several cranial nerves, or perhaps more precisely, the nuclei or functions of those nerves are impaired.  It is not the speech center of my brain, but rather my facial muscles that are impacting my ability to speak.  After surgery I have bilateral facial paralysis.  No smiling, no blinking, and no facial expressions.  My mouth won’t allow me form words correctly.  The fifth nerve is compromised on day 2.  That controls my jaw.  My husband later says I sounded like a bad ventriloquist.   

The sixth nerve controls my eyes and my right eye turns inward.  My eyes no longer track together.  I cannot blink and, like an old roll-up blind, my eye lids remain completely open, offering no protection to my corneas.  I am wide eyed at all times and they tape my eyes shut at night. 

My balance is severely compromised.  This becomes more apparent over the days and weeks to come.  I have no problems with strength.  My left and right sides are fine.  I could walk but I can’t walk.  Ataxia and fine motor skills are a problem.  Early on these balance problems are severe enough to cause challenges transitioning from bed to wheelchair. 

3 days in ICU and then another week in the neurological medical surgical floor.  They give me a swallow test.  While my throat is okay to swallow, my mouth is not up to the task of controlling the flow of liquid back to my throat.  For weeks I am relegated to nectar consistency drinks and pureed food.  

Massachusetts General Hospital is ready to discharge me and I am ready to go home but I am not really ready to go home.  My next stop will be a rehabilitation hospital. 

I spend the next 3 weeks at Spaulding Rehabilitation Hospital in Boston on their brain injury unit.  My sleep and wake cycles are still greatly impacted by either the surgery or the steroids, causing me to be confused at times.  This corrects itself over time.  Each day is filled with physical therapy, occupational therapy, and speech therapy.   

My time at Spaulding is very difficult.  It is hard to be away from my family.  A brain injury rehab floor is very noisy. My combination of deficits for sight, speech, and mobility leave me feeling very isolated.  I do recover enough jaw and mouth function eventually to drink regular liquids.  I learn to speak slowly to better enunciate even though I cannot purse my lips.  I learn techniques for dealing with my balance challenges.  The therapy is beneficial but it is really hard to be away from my husband and daughters. 

On May 3, 2007 I returned home.   

Over the coming months I slowly improve.  I gain enough mouth function to eat solid food.  I progress from a wheel chair to a walker in the house.  We had temporarily moved our bed down to a 1st floor den.  I am able to safely navigate stairs and we return to the second floor master bedroom. 

Through the summer I need someone in the home with me.  My daughters are teenagers and when they are out of school they help me.  My friends and other family members also take turns coming over.  When fall approaches I am still not quite ready to be on my own.  My husband takes a leave of absence and by November I am ready to be on my own within the home.

Through all of this I become depressed about losing my independence although I think it is temporary.  They increase my antidepressant. 

My facial paralysis does not resolve in the months after surgery.  This is especially problematic for my eyes.  My corneas are constantly exposed because I cannot blink.  We use eye drops during the day and lubrication each night before taping my eyes shut.  It’s a losing battle and my corneas are at risk.  In June they insert gold weights into my eye lids.  This lowers the lids to a normal level, allows me to manually blink and to close my eyes at night without tape.  With the weights and continued drops and lubrication my corneas begin to heal. 

During the year we consult Massachusetts Eye and Ear Infirmary (MEEI) on facial reanimation.  They harvest from the Gracilis muscle in your leg and implant it in your face.  By connecting this implanted muscle to your fifth cranial nerve, patients regain the ability to smile initially through clenching the jaw and later more naturally. 

The doctors do not want to proceed until they are sure the facial paralysis will not resolve itself naturally.  On April 7, 2008 I have the procedure, a bilateral Gracilis transfer on both sides.  The first time these particular doctors performed the transfer on both sides in one operation.  It took 4 doctors and 12 hours to complete the operation. 

The muscle transfers work.  It takes a while for the muscle and nerve to begin working together.  3 months later I am able to barely raise the corners of my mouth.  My first smile in 15 months!  Over the next few weeks I get more function and more control. I still have to work at it but what a huge improvement.  These doctors are amazing. 

Another major problem for me is paralysis in my right eye muscles, causing it to turn in and resulting in double vision.  After a lot of visits to MEEI, I get prism for my glasses improving my vision.  Eventually in 2009 I get a Botox injection to my eye muscle and this pretty much straightens my right eye improving my straight ahead vision. My right eye turns inward but not all the way so I am due next week for another injection. If this does not work I will have eye surgery.

Recently I went to Wisconsin to try this machine called the Brainport Balance Device. It gave me back my energy and some balance but not enough to walk unassisted. I can't believe what this machine did for me in just 6 weeks.  It was more effective than two years of rehab. This technology is amazing and when it gets FDA approved it will help people with balance problems. 

I still require a walker or assistance when I am walking.  Balance is still a problem and unfortunately I have more falls then I would like to admit.  I cannot drive or walk to the end of my driveway to get the mail.  We have modified our home and over time I have become more independent and able to cook meals and work in the house.  Thanks to online shopping I can take care of groceries, birthdays and Christmas.  Instead of jogging with my husband I ride beside him on a three wheel bike.  My near term goal is to move to fore arm crutches.  My facial paralysis remains, the facial re-animation has done wonders but I still struggle to eat discreetly. 

Today I am more than two years removed from my surgery. I haven't given up hope. There are times I just sit and cry but I realize there are others in the world worse off than I am. I must continue on this journey knowing I was born to help others.  My family and friends have been through a lot. I am so thankful they are still by my side. My garage is filled with Tupperware and casserole dishes, the remains of a years worth of meals made and delivered by our family and friends.  My husband and daughters have been through the most. I love them and only hope this experience will draw us closer together. 

I know what is important and I thought I knew it before my brain surgery.  I will never take life for granted.  I also have become much more faithful.  There are days I swear at god for putting me through this but I know he is there and one day I will have the answer. Technology is going to help all of us one day very soon and this is exciting so I continue on for those who can't.

Allison