Ellie's Story

Ellie's Story

As most of you know, our 20 month old Ellie has had quite a lot happen to her (and to us) in the past couple of weeks.  I’ve given little updates on facebook but that is far from telling the entire story of what’s been going on with her.  Also people have asked me, “Was she previously healthy?”  “Did you see signs that anything was wrong prior to her having a stroke?”  so I’d like to back up and tell her story from the beginning.

Starting when Ellie was still in my belly, the doctors have monitored her for problems deemed to be minor and not likely to cause any issue.  In utero she was monitored for hydronephrosis (enlarged kidneys) but by the time she was two months old she had an ultrasound that indicated the problem had corrected itself.  At two weeks old her pediatrician indicated that she had a heart murmur and referred us to a pediatric cardiologist.  The cardiologist found that she had a PFO which is a small hole in the heart that actually a good amount of the populations have naturally and usually poses no problems.  The cardiologist also found that she had a small amount of thickening of the aortic and pulmonary valves but that her heart was functioning normally and so he just wanted to keep an eye on things to make sure that it didn’t ever become an issue.  Obviously as a parent these things felt concerning to us but the doctors reassured us that she was fine and these were minor things that would likely never pose a problem.  

Since about 3 months old, Ellie has had days (and sometimes weeks) were she has been incredibly unhappy.  Days where they will cry all day and night.  I had taken her to the pediatrician many times because I just didn’t know what was wrong.  I always figured they would find that she had an ear infection or something but generally her pediatrician had been unable to find anything wrong with her.  Because he could find nothing wrong with her, it was believed that she must have stomach problems.  So we tried all kinds of different diets with her in an attempt to figure out what foods might be bothering her stomach.  When she was really little she had blood in every stool which we finally discovered was a result of her being lactose intolerant so that seemed to fit with the theory that she must have stomach issues.  Unfortunately no diet we ever tried seemed to really help things.

One evening this past January, we were getting Ellie ready for bed when her lips suddenly turned dark blue for no apparent reason.  It was startling to us and the next day I took her to the cardiologist.  The cardiologist did an echo of her heart and said it was functioning perfectly.  He said he didn’t know why her lips turned blue.  We were told just to dismiss it as one of those strange, unexplained occurrences.  

So other than an episode where her lips turned blue and her fussiness, there was no indication that anything was seriously wrong with her.  Just like her older sister, she was a little slow to meet her milestones at first but by 18 months old she was completely caught up and showing signs of even being ahead on some milestones.  She appeared to be a perfectly normal 20 month old--- healthy, active, and smart.

The morning of Tuesday, July 22 started out like most mornings around here.  I rushed to get the girls and I ready for the day and then left the house to drop Payton off at preschool.  After we dropped Payton off at school, Ellie and I went to the grocery store to get food for the week.  At the store, Ellie sat in the shopping cart right in front of me.  She pointed out items she knew the names of like “Nanas” (bananas) and “Boon” (balloons) and we played peek-a-boo and laughed together.  The car ride home takes a total of about 3 minutes and there was no indication that anything was wrong. 

 

When we got home, I got her out of her car seat and carried her into the house like I always do.  I then stood her up on her feet, expecting her to walk off and find a toy to play with.  Instead she collapsed to the ground, crying.  I thought to myself, “Wow, this is a lot of drama, she must be really tired or something”.  So I lifted her back up and again put her on her feet.  Once again she collapsed and was crying even harder.  I still didn’t realize anything was truly wrong so I said, “I have to bring in the groceries so you sit here and watch Signing Time” (her favorite DVD) and I put her into the sitting position and turned Signing Time on.  Instead of staying in a sitting position, she again flopped forward onto her stomach and continued to cry.  The fact that she didn’t care that her favorite show was on TV definitely set off the warning alarms and made me realize this might be more than her just being tired and dramatic.  I went to the kitchen and got some fruit snacks and said, “Ellie, want some candy?  Come here and I’ll give you some candy”.  She was lying on her stomach crying and she kept trying to get up but she couldn’t.  I put her on her feet and then backed up to encourage her to walk to me to get the candy.  She was able to take a couple of steps and then fell forward.  I then carried her to the table, had her stand holding onto the bench, and gave her the fruit snacks.  That’s when I noticed that her left arm was hanging completely limp by her side.  I tried to get her to use that hand to get the fruit snacks but it just appeared dead.  At this point I called Charles, who was at work, and explained what was going on.  His advice was for me to take her to the ER immediately.

A lot of thoughts went through my head on the way to the ER.  Part of me was really scared because I felt like there was something really wrong with her and the other part thought there was just no way something could be seriously wrong with her and that maybe her initial fall had dislocated her shoulder or something.  When I got to the ER, I parked in the drop off location and rushed her instead, telling the front desk lady that I felt something was really wrong with my baby and therefore couldn’t go park my truck in the parking garage.  When they checked us in and they asked me what was wrong, I told them her arm wasn’t working, that it was completely limp.  So, they triaged us as a child with an injured shoulder and everyone who entered our room was expecting to be dealing with a shoulder injury.  Only the thing was, Ellie couldn’t even sit up independently.  She literally couldn’t even hold her head up on her own.  Her head and body would flop to her left side anytime they weren’t supported.  She was crying the majority of the time but also couldn’t seem to keep her eyes open.

Once they had asked a million questions and realized this didn’t appear to be a shoulder issue, they performed a CT scan on her head.  They also sent the PICU doctor to see us and he said, “Not being able to move your arm is not normal and it’s not a good sign”.  After the CT scan, they tried numerous times to get an IV into Ellie but after jabbing around on both her hands, both her arms, and both of her feet, they were unsuccessful.  This was incredibly hard to watch because I knew how painful it must be for my baby.  

The CT results showed a large area on the right side of her brain that the radiologist reported to be “atrophy” and what the PICU doctor believed to be “gliosis” (old scarring).  The PICU doctor explained to us that anything could have caused this--- that it could have occurred in utero or maybe the result of a head injury at some time.  The doctor’s theory was that this old scarring may have triggered Ellie to have a seizure that must have occurred during the 3 minute ride home from the store.  He said this was obviously just his guess on what had taken place and he wanted to do an MRI which would give a much clearer picture of things.  The problem was, with no IV they couldn’t sedate Ellie in the way they deemed the safest.  So we were placed in PICU (pediatric ICU) for the night and told they would get anesthesia to work on the IV and perform the MRI the next day.  

Wednesday they were able to perform the sedated MRI on Ellie.  Having your baby go under anesthesia is a scary thing (we had no idea just how scary until a few days later!) but she did fine.  Early that evening we were back in our PICU room when the PICU doctor came in and said, “Give Ellie to the nurse, I need both of you to come into my conference room.  I have bad news”.  My stomach dropped.  This was one of the worst and scariest moments up to that point in my life.  When your child gets MRI results back and the doctor says, “I have bad news” your mind immediately goes to the worst.  The doctor then started with, “This isn’t cancer, it’s not a tumor, but it appears your daughter has some type of rare, neurological disease.  And we don’t know what it is.  It could be MoyaMoya, a disease we have a little experience in dealing with, or it could be any number of rare diseases.  It could be something with treatment options or it may be a progressive disease with no options.  In these type of situations I can’t tell you what I would do if this were my own child because that hits too close to home, but if your daughter were my niece and I were giving advice to members of my own family, I would say you need to go to Texas Children’s Hospital where they have specialist who have experience with these type of rare diseases”.  

So the decision was made to have us transferred that night to Texas Children’s Hospital in Houston.  When they told Charles that Galveston EMS would transfer us, he sent a text to our friend Joe who is an EMT and asked, “You working tonight?”  To which Joe said, “Yeah, are we transporting Ellie?”  So Joe was able to put in a request to be the one to transfer us.  It was definitely nice to have a friend ride in the back of the ambulance with me and to talk and take my mind off things as we drove to Houston.  They strapped Ellie’s car seat to a stretcher and she slept in her seat on the ride to the hospital.

Once at Texas Children’s we were placed in a room in the Progressive Care Unit, which is a unit in between a regular room and PICU so that the patient is monitored closely but not quite as intensive as PICU.  We met with doctors late into the night.  A neurologist looked at our MRI and came in and said, “I see from the MRI that your daughter has had a stroke and older strokes too”.  And we were like, “No, we don’t think she had a stroke.  UTMB thinks she had a seizure, they assured us she didn’t have a stroke.”  To which she said, “Ok well it looks like a stroke to me and I conferred with another neurologist who also thinks she had a stroke but I will see what radiology says”.  

The next day the chief of neurology met with us and said she had looked at the MRI and so had radiology and Ellie had most definitely had a stroke and had signs of having had other strokes in the past (possibly in utero since we insisted she had never shown signs of any type of weakness before this).  She then scheduled an MRA for the next day which is an MRI where they inject dyes into the brain to get a better look at the blood vessels.  The sedated MRA went fine.  

When the results were in, the neurologist and an entire group of doctors and residents, met with us and told us, “We have a diagnosis, your daughter has Moyamoya disease”.  She showed us the MRA pictures of Ellie’s brain.  The left side of her brain had normal blood flowing through the arteries but the right side there was nothing.  No blood going through the arteries.  With moyamoya the arteries close themselves off.  In an attempt to compensate for this, the body creates new tiny, tangled vessels (the word “moyamoya” is a Japanese term meaning “puff of smoke” which is how these new vessels appear on an MRI) but these vessels are too small to transport blood efficiently which is why people with moyamoya then start having strokes.

The neurologist explained that the treatment for Moyamoya is brain surgery to help revascularize that side of the brain.  I asked based on Ellie’s MRA what she felt her prognosis might be.  And she told me, “To be completely honest, all the arteries I would be concerned about closing off are already completely damaged.  Looking at this MRA I would say your daughter should be severely impaired.  And yet she isn’t.  Her young brain has created new pathways to compensate and she is functioning normally.  Therefore I would say she has a good prognosis.”  

Late that night (these people work crazy hours!) the neurosurgeon came by to talk to us for the first time.  Dr Lam explained again about Moyamoya and about potential surgical options.  She let us know that she wanted to perform an angiogram either Friday or Monday.  She explained that with the angiogram Ellie would need to be sedated and they would be sending a catheter up through her vessels which poses a small risk of dislodging a new clot and causing another stroke.  The angiogram would allow her to get an even clearer picture of Ellie’s arteries prior to surgery.  

The angiogram didn’t take place on Friday so they told us they would be moving us to a regular room on the neurology floor for us to just hangout for the weekend.  The weekend felt pretty long.  Ellie wanted to be constantly active but they had her on IV fluids so it was difficult to keep up with her while also pushing her IV pole around.  During the week there are volunteers who open up a playroom for the kids but not on the weekends.  So we took many walks down the hall to see the fish in a fish tank and to let her play on some toy cars and bikes they have for kids to ride in the hallways.  Thankfully our nurse, Angela, was wonderful and encouraged us to also take her to the next building over to see the model trains and for a couple of short visits to the playground (which unfortunately was miserably hot with little shade and no breeze).

Monday arrived and Ellie had to suffer through yet another day of having nothing to eat or drink.  I know my baby lost some weight through this entire ordeal and she didn’t have any to lose to begin with.  I had no idea that this Monday would stick in my mind for the rest of my life.  The angiogram went well and once Ellie had woken up in recovery they allowed Charles and I to be with her.  Ellie pointed at us and said, “Mama, Daddy!  Mama, Daddy!”  She was clearly alert and happy to see us.  The nurse then gave her an apple juice which she sucked down.  Both neurosurgeons were there and they started discussing with Charles and I the results of the angiogram.  The nurse left the room and I stood at Ellie’s side while listening to the surgeons.  Suddenly Ellie looked at me with this really blank look on her face, stuck her tongue out and stopped breathing.  I grabbed her and sat her up but she didn’t breath.  I yelled, “She’s not okay!  She’s not okay!”  The surgeons and Charles ran over.  Ellie’s face started turned really white.  Her oxygen monitor started alarming and her oxygen level dropped to 54.  The nurse ran in from the other room and yelled at me to “Move!”  I stepped back and she grabbed the bag resuscitation devise, placed it over Ellie’s face and started giving her breaths while saying, “Ellie breath!  Breath!”  Thank the Lord after just a few resuscitation breaths, Ellie started to breathe again on her own. 

She then seemed okay again and although we were shook up, we were relieved that she was okay.  About 30 minutes later, Ellie got upset about something and started to cry.  But she cried out several times and didn’t take a breath in.  Her oxygen plummeted to about 70, the nurse told her to “Breath!” while massaging her chest and thankfully she did start breathing again without needing to be resuscitated.  Another 30 minutes past, she got upset again and exactly the same thing happened, again with her regaining her breathing with encouragement.  The doctors then put in orders for Ellie to be transferred to PICU for the night.  Charles and I were very rattled after seeing our baby stop breathing and so we spent the majority of the night just staring at her, watching her breath in and out.  Plus there is no place for a parent to rest in a PICU room anyway.  

By morning we were relieved that Ellie had made it through the night without any other issues but we were very scared about the brain surgery she would need to have on Wednesday and the possibility of this happening again when she went under anesthesia.  They decided to keep Ellie in PICU until her surgery and for her to return to PICU after her surgery.  

PICU at Texas Children’s Hospital is pretty much the saddest place on the face of the earth in my opinion.  It is massive--- tiny hallway after hallway, with little rooms all filled with two patients per room.  Full of very sick children, all crying and screaming in pain or in fear.  Parents are welcome to stay with their child but there is no place for them to rest, just a couple of chairs to sit in.  And then if you need anything, such as to use the restroom, you have to find your way through the maze of tiny hallways…. past all the sick screaming children….. to a door that locks people out, through a washroom, through another door, through another small hallway, through another door, through the family waiting room, through a final door, and out to the main hospital and visitor waiting area.  I still pray for our roommate while we were there…. A young boy of maybe 12 years old on life support.  

The second day we were in PICU, after not having slept in many, many hours, the nurse asked me if I thought Ellie would sleep better next to me in a regular hospital bed instead of the hospital crib they had assigned to her.  I had no idea this was even an option otherwise I would have requested it nights ago.  So once they got us a regular bed, Ellie and I were set and were finally able to get a little sleep.   I would lie next to Ellie and prayed over and over, “Please God don’t let me lose her.  Don’t let me lose any part of her.  I adore the person that she is and I’m so scared of losing her, of losing any part of who she is”.  The fear about her upcoming brain surgery was gripping but I could also feel all the prayers being lifted for us and they gave me a strength I never would have had on my own.  I was more terrified than I have ever been in my life and yet I had many moments of feeling peace that everything was going to be okay. 

Wednesday, a little over a week after all of this began, was the big day for her brain surgery.  We were very concerned, especially about how she would react to anesthesia, but we knew she absolutely needed this surgery so all we could do was put our trust in God and trust in the surgeons and anesthesiologist.  Every 30 minutes or so during surgery we would get a call from the nurse and I would absolutely panic but every time it was the nurse saying that everything was going just fine.  After about 2 hours, one of the two neurosurgeons came out to meet with us.  Again I was so afraid it would be bad news but he said he was finished with the dural inversion and that everything had gone well.  That Ellie had needed a blood transfusion during the operation but that was to be expected.  And that the other neurosurgeon was closing her back up.  Probably an hour after that we were called back to see her.  While we were so thankful the surgery had gone well, we were still scared because recovery was when Ellie had stopped breathing during the angiogram.  We walked in and she looked so pale and little lying there.  She opened her eyes and in a very hoarse voice said “Mama, Dada”.  She started to get upset so the nurse had me pick her up and hold her.  She had bandages over the incision area and a hat on top of the bandages.  We told the nurse how scared we were that she might stop breathing and she said, “Well this is the place to be.  We deal with that at least once a day, sometimes several times a day so we’re prepared for it”.  Thankfully Ellie did just fine and after about an hour we were taken back to PICU.

Ellie was in pain throughout the night and all they would give her was regular infant Tylenol.  They said they don’t like to give narcotics right after neurological surgeries.  It was so hard to see her in so much pain.  She would try to sleep but every 10 minutes or so she would wake up crying and hyperventilating.  At one point I asked if they would give her IV Tylenol instead since my dad said it’s more affective and they did but said insurance wouldn’t approve more than 1 dose of it because it’s expensive and she’s capable of taking regular Tylenol.  The next day she was in pain and had no interest in eating.  The few bites of cereal that we convinced her to eat came right back up.  Other than the pain and nausea she was stable so by afternoon we were moved out of PICU and back to the neurology floor.  Once on the floor they had zero issue with prescribing a hydrocodone medication and by dinner she was clearly in much less pain and was able to keep down some peas that she ate.  Unfortunately before bed her stomach was upset again and she vomited up a few grapes we had given her.  

We stayed 4 days after surgery for her to recover and thankfully they were fairly uneventful days.  A couple days after surgery Ellie was up walking and playing.  Occupational therapy and physical therapy both came to evaluate her and work with her.

On Sunday, a little less than 2 weeks after this all began, we got to leave the hospital and go home.  We are so thankful to be home and so thankful that Ellie is doing well.  We attribute Ellie doing so well to all the many, many people who offered up prayers on her behalf.  We are so grateful to everyone and for all of the support we have been given.  Today marks two weeks since Ellie’s stroke and tomorrow will mark a week since her surgery.  We still have to give her pain medications around the clock (she even wakes up at night in pain) but as long as we keep up with her medications she seems comfortable.  We are waiting on a call from occupation and physical therapy so we can get her started in an outpatient program through UTMB.  The main lingering affect she has from her stroke is that she still doesn’t have full use of her left hand.  We have also noticed that her walking is incredibly slow and aren’t sure if that’s just because she’s still sore from surgery or if it’s a neurological affect.  But overall she is doing great!  We will visit her neurosurgeon in 1 1/2 weeks to check on the incision and then in probably about 6 weeks we think she will have another angiogram to see if the revascularization is starting to take place.  And also at that time she may undergo surgery on the other side of her brain.

There are times when this all still seems so surreal.    You don’t ever think your perfectly healthy baby could suddenly suffer a stroke and be diagnosed with a rare neurological disease.  Two weeks ago if someone had suggested that my healthy, smart 20 month old could have a brain disease that had already permanently damaged over 1/3 of the right side of her brain, I would have said they were absolutely crazy.  But the thing with little ones is that their brains are constantly changing and remapping themselves and until the stroke her brain had fully compensated for the damage done to it.  Her diagnosis explains the only symptoms she previously had---- blue lips because her brain wasn’t getting enough oxygen and it’s likely the fussiness was in response to headaches she was getting since that’s a complaint that most people with Moyamoya have.  There is no cure for Moyamoya.  It is something she will always have and will need to take certain precautions, like taking Asprin every day to prevent a stroke, but thankfully if these surgeries work she should be able to live a long, normal life.  Unfortunately they don’t work immediately because it takes several months for the revascularization to take place.  So if yall would, please continue to pray for Ellie who remains at an increased risk of having a stroke and who still has to undergo one more major brain surgery in the fairly near future.  We have seen the power of prayers and we are so thankful that our Ellie is doing so well now.  Thank you all and thank God for our little miracle!