Sophia's First Stroke

April 2015

In April of 2015, shortly after Easter, my precious six year old angel asked me to join her in her bedroom to watch a movie together.  We agreed on The Road to El Dorado, made popcorn, and settled into her bed.  Before starting the movie, Sophia left the room and returned with her daddy's Easter basket.  As she picked through the candy I watched as she happily munched on the chosen pieces and I noticed she cared more for the candy than starting the movie.  She asked me to get my phone and film her as her online persona "Ninja Kitten". 


I started filming and asked what she was doing, she proceeded to explain her robin egg eating process as she addressed her imagined million YouTube fans.  I will explain more about Ninja Kitten later.


As we chatted I informed her she had daddy's basket, asking how she planned to explain where all his robin eggs went.  She of course immediately stopped stealing his candy and ran to return his basket to the kitchen.  I stopped filming and when she returned, she gleefully hopped into bed and told me to start the movie.  


And then it began....


Her tiny voice said "Hey, I can't make a fist."

I didn't quite understand.

Then the panic started "Mommy! I...I can't make a fist! Mommy!" I sat up and saw her holding her left hand with her right.

"Mommy help me! What's wrong with my....", and then the screaming started.

I didn't understand what was happening, I was trying to hold her, comfort her, but she started panicking and got off the bed screaming "No, no...what is happening! Help me mommy, I think I'm dying!"


More screaming. 


"Mommy help me I think I'm dying help me!"


I tried to calm her down as I stopped her from rushing out of the room, she was hysterical. I was confused, scared, and shocked. I think I'm dying? What does that mean? And then I saw her face.  The left side of her face looked like it was melting.  First I saw the eyebrow slightly droop, followed by her cheek relaxing and appearing to sag, then her lips began to pull down and left her mouth slightly open as the left corner pulled down.  She fell silent and as her shoulder dropped, the life in her eyes faded away just a bit.  She stared at me, but it seemed like she was staring through me.


A Demand for Answers

Immediately when my husband saw her he knew it was a stroke.  We rushed her to the emergency room in our small town, her face had recovered from the drooping and the doctor on call stated that they couldn't take images because their machine was down.  He said that the left side numbness she described was probably just the arm falling asleep from leaning on it wrong, he gave her a dose of Tylenol and sent us home.  Upon leaving, staff at our hospital advised us to travel to Lubbock to obtain images should she still have trouble with that side.


We watched her closely but noticed she used the left arm less and less.  Her face had returned to normal but that arm seemed to just hang from the shoulder.  Sometimes she would use it but it was only when we mentioned it.  I watched her play a video game  with her brother and noticed she was only using the right hand to control the entire XBox remote controller.  I would toss waded paper to her far left and she wouldn't lift that arm in any attempt to grab it. 


When I showed my husband he agreed we would take her to her primary pediatrician in Lubbock which is about 40 minutes away.


I asked her what was wrong with her left arm, questioning why she didn't use it.  "Ghoulia? Oh, I named her Ghoulia." Ghoulia is a zombie character from the Monster High series, which she loves. I asked why she named it Ghoulia and she said in a scary voice, "Because she's dead.....but still alive!!!" and then used her right hand to lift it on top of her brother's head saying it wanted his brains.  Good...her sense of humor isn't changed. 


On the way to Lubbock, I videotaped her saying her name. I figured if I kept her saying the same sentence it would be easy to compare changes in her facial muscles.


When we saw her primary care provider she saw no significant signs to warrant an ER visit for an MRI.  Sophia had a second episode which recovered again.  But since no immediate signs were showing the MRI was scheduled the following week.  Her doctor is a wonderful compassionate woman who immediately believed the symptoms we were telling her and due to Sophia's complicated medical history she agreed that imaging should be done.  We  were told to come back if the signs returned. 


On our drive back home I repeatedly told my husband that I had a bad feeling, I knew something was very wrong and told him that I intended to return to the hospital and go directly to the ER.  We stopped at the grocery store and my husband quickly ran inside. In the car, Sophia started saying she was getting "that feeling again".  


I  went home and packed a suitcase for my son for three days and for myself and Sophia for three days. Because of the history she has had I knew what to expect should she be admitted to the hospital, I knew I needed  to prepare for several days.   We returned to Covenant Medical Center in Lubbock and I went straight to the emergency room where I demanded that she get an MRI and began showing the doctors the videos I had taken. I was told a long time ago that pictures and videos speak louder than words for doctors.   They ordered an MRI, and as expected, they came back telling us she had had a stroke, probably several.  She was admitted into the ICU .


MoyaMoya

While in the ICU we were told by the doctors that the strokes were most likely the result of a blockage of blood flow in the brain, possibly a clot or tumor.  In order to gain further knowledge, Sophia would need to be sedated for an angiogram.  

The day of the procedure the doctor told me that it would be awhile before she was awake from the angiogram and we should just relax, get something to eat, and return to the room, but added "Unless something goes wrong, we could be done in five minutes.  But I'm sure it will be awhile for us to complete this."  

I was with her as they took her into the operating room and began her sedation.  When I left the room I met my husband and my son In the hallway. We sat in the waiting area near the operating room to discuss where we should go while we waited.  Within minutes the doctor came down the corridor and told us to stop.

Something had gone wrong.  They discovered at the start of the angiogram that there was no blockage, but her right carotid artery had completely closed and she appeared to have a very rare disease called MoyaMoya.

MoyaMoya is typically characterized as the narrowing of blood vessels in the brain which results in limited blood flow and eventual brain damage from the lack of oxygen to those areas of tissue. There is no cure.

We were told neurosurgery would visit us to discuss our options.

When we met with the neurosurgeon, he explained the typical treatment to help MoyaMoya patients was a surgery called revascularization. The skull is opened and blood vessels are "planted" into the brain in hopes that six months later those blood vessels would take "root" and offer a new source of blood supply to the brain.  He was a skilled surgeon and confident in his abilities but due to Sophia's complicated medical history it was best to air lift her to Texas Children's Hospital in Houston where there was a MoyaMoya specialist and a better support system than what was available in Lubbock.


When she woke from sedation she was behaving a little differently. A little more aggressive.  


 The staff informed us that Texas Children's Hospital was sending their private jet to transport her.  In the meantime the hospital wanted an additional MRI of her brain to send with her.  She asked to not be sedated and promised to remain still.  She had  been through countless imaging procedures and the machines no longer scared her.  We went to radiology and during this particular image she began to panic.  The nurse had added Versed in small increments to her IV line to keep her calm but she had a paradoxical effect.  The nurse added more which became apparent was just enough to tip her over the edge.  She became very angry, panicky, and eventually violent.


By the time we returned to the ICU room she was at the beginning of a full meltdown.  She began screaming and flailing her limbs and gradually became angrier.  She became verbally aggressive, saying things that were hurtful and not typical of her at all.


Then the screaming began again followed by pleas for help and that sentence again, "Help me mommy! I think I'm dying!"


This behavior was the precursor to a massive stroke which completely paralyzed her left side.  This time the paralysis did not recover And ran on the entire left side.  She could not lift her or move any part of that side. She continued to scream. The staff came in and attempted to sedate but her little body wasn't going down, they tried different medication and finally she was under again.  It was decided that due to her violent episodes it would best for her safety and the safety of the flight crew that she remain sedated.  She was intubated and we were transported to Houston. 





Texas Children's Hospital


From the moment we arrived we were assured we were in the place to get answers.  But as the teams of specialists studied her imaging for days they began to agree not to perform the brain surgery.  


Why? Because they were baffled.  Sophia's imaging showed severe damage to the right side of the brain.  These images revealed that the extensive damage done from the strokes would result in her permanent loss of use of her left side.  She would never walk again. Never use her left arm again. She wouldn't even be able to hold her own head up. 


So why were they baffled?

As they observed the recent images of Sophia's brain, in comparison side by side, they showed her brain healing itself.


As these doctors took repetitive images and compared the images side-by-side, they were baffled because Sophia began to revascularize her own brain without the surgery. 


They were baffled because her brain began to show new blood vessels forming in the brain on the right side and they could not understand how. 


We were told that her brain was doing in days what only surgery can do and would take four to six months.


They decided not to perform the surgery since they couldn't explain what was happening.  It became a concern that if they did open up her brain the shock could force the brain to do what the images showed it should be doing...just be dead tissue.


Eventually they decided to move her out of the ICU to the neurology floor for further observation.


The regular imaging continued as they spent time teaching us how to care for Sophia in a partial paralyzed state.  How to bathe her, feed her, care for her, and move her with the left half of the body paralyzed. 


As the days progressed teams of doctors would come into the room and openly discuss her situation.  They would talk to us about what the plan would be for her rehabilitation down the road. 


Sophia became very upset after so much time of listening to dozens of doctors talk about the things she would never be able to do again. She started to speak up and at one point told the doctors "If you have nothing positive to say while you're here, then discuss everything in the hallway, because I can hear you.  If you're only going to talk about what I can't do then I don't want to hear it. Why don't you let me try to walk. Let me try to move. Let me get out of this bed. Unhook me from all this equipment and please just let me try." 


Of course the doctors repeatedly told us it was foolish and a risk to her health should she fall and there were regulations we needed to follow.  At one point when they left the room Sophia told her dad and I to go get her some Playdough.  When I brought it to her she asked me to open it and place it in her right hand. She then moved the Playdough to her paralyzed left hand and began to use her right hand to push each finger of the left-hand into that Playdough. I asked her what she was doing and she told me she's trying to make the left hand feel the Playdough so that it can do this on its own and she was going to work at it because the doctors didn't want to help her try so she would do it herself.


This didn't just stop with her use of her left hand.  She set three goals for herself.  For her leg, she wanted to run again. For the left arm,  be able to touch each fingertip with her thumb. And for her paralyzed face, she wanted to say a tongue twister. 


While much of the staff thought it was cute, most of them told us it was unrealistic and went back to teaching us how to bathe, her how to feed her, how to care for her being completely paralyzed on the left side.


But within a week, Sophia was able to stand and take a few steps with a support belt.  Her progress was miraculous.


They eventually moved her to the in-house rehabilitation floor where she and I stayed for many weeks.


Eventually it was time for her to be discharged. The staff were amazed at her improvement and her recovery but it was time to be sent home and followed up with our local doctors. 


When we contacted our Lubbock neurologist she stated she was closing her practice and was moving back to Chicago to be with her your mother. She also told us not to come back to Lubbock because the only remaining four pediatric neurologists had already been contacted and refused to take Sophia's case because "she was out of their league" or too complicated to take on as a patient.  I was suggested we move to a bigger city where there's a better support team and not to come back to Lubbock.


We didn't know what to do but we prayed as always.


*Check back as we add more details to this section and share more about her time recovering at TCH, the great staff, and her support team she worked with throughout her recovery.

The Naming of Doctors

I should take this moment to talk about Sophia’s “naming of the doctors” habit. 


Sophia has always asked us to tell her the truth about her medical condition because she has been In hospitals and surrounded by doctors her entire life, many times hearing every word they say.  These conversations included many stating she would not live out the week, not live to be more than two years old, four years old, she would be a vegetable eventually...things children shouldn’t hear. 

 


She asked us a long time ago to always tell her the truth when it came to her health.


After seeing literally hundreds of medical personnel in her lifetime, it became increasingly harder to remember the name is each doctor and specialist. 

Sophia would listen intently to her father and I discuss her medical situation and ask “Which doctor is this now?” And then started responding with nicknames she gave them in her little mind.  “Oh, you mean Dr. Bad Breath” or “Oh, you mean Dr. Baldy.” or “Dr. Bowtie.”


We soon found it easier for us all to use her labels because we knew who she was talking about.

Cook Children's Hospital

We heard of a great neurology team at Cook Children's Hospital in Dallas but after contacting them to take Sophia's case we were told that they were not interested and it would be six to nine months before we could get an office consultation.


My sister had recently moved to Dallas and had been helping us through all of this and offered her home for us to stay at so that if anything else happened with Sophia we would be closer to a larger hospital which had a larger support team. 


The second day we were in Dallas Sophia suffered another minor stroke and we took her to the emergency room at Cook Children's Hospital in Fort Worth.  Upon discharge they ask us to follow up with our own neurologist and since we had none at the time, we we're pleased to tell them they had to see Sophia in clinic now that she had been seen at their emergency room.


I won't say it's a coincidence that these things happened, because God always has a plan.


When Sophia and I arrived at the appointment with the neurologist he told Sophia "I heard about you. I've read about you and I've seen your images. Let me tell you something little girl, you're not doing the things that the medical books say you should be doing. So why don't you tell me how are you doing this. How are you holding your head up and moving when you shouldn't be able to?"


Of course Sophia smiled and confidently said "Well, because I'm awesome that’s how!" 


He asked "I've been hearing a rumor Sophia, that you can stand on your own.  Is that true? Because I've seen your images and you shouldn't be able to do that, but I heard that you can."


Sophia replied "You want to see what I can do? I'll show you what I can do." 


I told her to make safe choices as she locked her wheelchair and swung open the wheelchair legs. She stood up, took about 11 steps, turned around, and walked back to her wheelchair, sat down, raised her arms,  and said "Ta-da!"


After some silence, this neurologist told her, "I know you scare a lot of doctors, but you don't scare me.  I don't know what is happening, but if you trust me, I'm willing to work at finding answers together."


Sophia looked him over and asked “Do you promise not to use me like a lab rat? And always tell me the truth?”  He replied that he would and she immediately said “Ok then, I shall call you Dr. Stoic...after Stoic from the movie How To Train Your Dragon, because he is the bravest man I know!”


Over the next year we made regular trips back to Fort Worth, sometimes as often as once a week, so that Sophia can participate in the stroke clinic at Cook Children's Hospital and receive therapy.  She regularly has imaging done of her brain. These images take about five hours under sedation and they are deep imaging that looks inside the blood vessels in the brain.


They have come to the conclusion that Sophia has MoyaMoya like symptoms but not actual MoyaMoya. The stroke team has concluded that Sophia has a "unknown genetic vasculopathy" which is another way of saying they don't know exactly what it is that she has, because it can't be identified. What they do know is that the blood vessels within her brain or slowly narrowing. They don't know why and they cannot stop it. 


There is no cure for Sophia's condition. Sometimes the imaging shows that there is no more additional narrowing. Sometimes the images show that there has been an increase in the narrowing. 


Every day is a gift. We hear that all the time, but for Sophia it truly is. We are constantly reminded that it any moment she could suffer another stroke and there's nothing that can be done. The next stroke could put her in a vegetative state, permanent paralysis, or end her life.  As her mother, this is harder than you could possibly imagine. But our son once told us that God gave her to us for a reason. That God chose us to be her family because we were the right family for her and we absolutely believe that.


During the holidays in 2016, Sophia began suffering minor strokes again. New imaging showed that the left carotid artery artery and the right posterior artery were closing more.  The need for an alternative blood supply source outweighed the risk of surgery.


The dural revascularization surgery was performed on February 6.  There was an chance that she would not survive the surgery without a stroke possibly killing or permanently paralyzing her. 


Once again this little girl came through miraculously. One of the neurosurgeons stated "This little girl has God on her side."


We prepared for weeks of recovery time.  But by day two, she was off pain medication. By day three she was up and walking around the nurse’s station. 


Sophia returns to Dallas in August to take more images to see if the revascularization  surgery was successful.


About a year ago my husband heard me repeatedly telling the story of Sophia's miraculous journey to everyone I could.  He was very upset because he couldn't understand why I was retelling the most horrific time of our lives to people with a smile and repeatedly telling it without crying.  


It wasn't until he heard me telling a group of mothers at Sophia's troop meeting that he realized I was telling the story because Sophia story is a testament to God's Grace. 


I told him later that  I feel I must tell it, I have to tell it.  In a world where things are so bad and ugly, when there is so much loss of faith, people needed to hear Sophia's story so that they could hear a true testament to God's Grace and His Glory revealed in Sophia's story.  


This is why we tell her story as much as possible so that you know prayers are heard. So that you know God's Grace exists today. So you can see it, and retell it in this little girl.


Please read the continuation of her stroke story in the section “Believe with Sophia”.