Emily meet HLH - Part 2

Picking up from Thursdays blog (and this is REALLY long so i apologize) ~ 
Things were going great after she was taken off the chemo.  Sure she would have these weird, unexplained fevers...low grade (99 - 100) but she was acting just fine.  She was playing with no questions asked, eating like any other child but she just had this fever.  It would last 1-3 days and on school days...give her a little Motrin and she was good to go!  No one was the wiser that she had this little fever.  Unbeknown to us, this was a sign that the disease was acting up.  This went on for a year - on and off.  In May of 2010, Emily was in Kindergarten, she started getting a swollen gland.  she had a little cough but nothing major and again, a low grade fever.  Given her (regular) doctor seemed to be OVERLY cautious by always threatening to send her back to the hospital if her oxygen level was 96 even,  I opted to seek out a new pediatrician.  I wasnt going to be all the way upfront with her at the beginning because I wanted Emily to seek "normal" help.  Her doctor at the hospital insisted we treat her as a "normal" child and since i didnt feel she was getting medical attention as that, i sought out a new one.  This doctor was so sweet, a little older but super sweet.  She did a strep test on her and gave her some medication and said it should clear things up within a couple days.  Well, after 2 days, her glad was swollen twice the size and now she has a fever of 102!  After going back to see her she sent us to the ER.  There they started doing labs on her and getting her medical history and everything that comes with going to the ER.  After waiting there for what seemed to be forever, the ER doctor pulled the curtain and asked to speak with us privately.  My stomach in knots wondering what could possibly be wrong...its just a swollen gland.  He took us to the desk and said he got the lab results back and just a quick look he thinks her disease is back!  I could not believe what i was hearing.  Really?  I mean this is 1 in a million to even get it and when its infectious you do the treatment and then your done..your healed...you go on with life as normal!  Bryan and I went back to our "room" and pulled the curtain and began to cry...trying to not allow Emily to see me because I didnt want to worry her but all i kept thinking was Lord, I cant do this again!  I am NOT strong enough to handle this and now that  i have a baby!  I rode with Emily in the ambulance...again..to Childrens as Bryan followed us there.  They admitted us once again but this time was different. After a few doctors saw her, we saw a surgeon.  This was different and a doctor that we havent seen ~ considering we feel as though we have seen someone from every area of the hospital almost!  I believe they did an ultrasound and said that they would surgically removed it considering they have no idea what it is.  When its out, they would then test it for Lukeumia and Lymphoma to rule those two things out.  They said the surgery would only take about 45 min and they would cut in the fold of her skin so when it healed it wouldnt be too noticeable. Surgery day came, Bryan took the day off and waited there with me and we waited and waited and waited.  I seem to remember it took about 2 hrs before they came to tell us anything.  They sent her back up to her room and we met her there.  She had a draining tube coming from her neck and she was in so much pain.  She is so strong though!  she did NOT like the test of the medicine they were giving her so she opted to deal with the pain instead of take the medication.

The next day, they came and took the tube out.  They said she was doing wonderful and they would have the results back soon.  They did say the surgery took longer because it was the size of a small apple and it was in the middle of the main veins in her neck which made the surgery more delicate.  A while later we rec'd the news ~ NO lukeumia and no lymphoma however, it was a new disease called Kikuchi Disease! Ironically this is usually found in Asian woman - 20 years and older.  Well Emily did not meet either of these however it is the Histyocytic family ~ so again...its her disease!  After spending about 9 days in the hospital things were great and we were sent home!

Emily returned to school for the last few days of school!  We started noticing though that her eye lid (same side as her surgery) was sagging a little.  We didnt think to much of it until Fathers Day (just a few short days after school was let out) when her eye lid was sagging so much that it looked like it was half closed!  This was obviously concerning and moreso because we were planning on leaving in just about 3 weeks to go to Disney World!  The next morning it wasnt any better.  My parents came over, my mom stayed with Benjamin and my dad went with me to see the surgeon.  After seeing him he didnt like it and thought maybe it was something where she should have an MRI done.  First he sent us to a wonderful Opthamologist .  The drive was 15 min.  In that short time, her eye lid went back to normal but now her eye was off track and she was now seeing double.  We were sent back to the hospital for an MRI.  Because Emily has to be put completely out for one and she couldnt do it with just plain sedation, they opted for a a CAT Scan but admitted us so we could get the MRI done the next day.  The scan showed things were fine but still wanted the MRI.  After being in the hospital for 12 days, canceling our trip to Disney World and postponing it to December, after seeing all the Pediatric Neurology team and then onto the Chief of Neurology for Adults, after videos and pictures were taken of her to be presented at the Case meeting they were having, her doctor (who she sees regularly) said what he felt.

Steroids!  She was back on steroids!  So July 2010, she started back on steroids and started a 3 month course of IV chemo treatments again.  We were also sent to Children's Hospital of Cincinnati for a second opinion.  

They do feel this is a secondary case of her HLH (which is just as rare) and they are calling it Sarcoid.  The doctors in Cincinnati felt the same after a long 5 hour doctor appt!

The steroids didnt waste anytime showing its ugly self on my precious baby.  My once petite little thing was now getting bigger and bigger as every day passes.  The clothes that were just bought no longer fit, the stares of others are beginning again and I question again, am I really strong enough to do this all over again with her.  

She was a flower girl in a cousins wedding days after her last IV chemo treatment.  Everything went well!  She started school a couple days after we returned and she was loving 1st grade!  Around the 15th or so of September 2010, she developed a cough...no fever just a cough.  Not a big deal.  I questioned myself as to if she should be seen but the fact that she was acting just fine...I didnt give it another thought.  Little did I know what was going on in her body.

September 29th was a warm day...warm enough for her to be outside running around, playing with her friends, wearing her shorts and t-shirt.  It was dinner time and she said she wasnt feeling good.  Strange since she was just playing her little heart out outside!  We felt her and sure enough, she had a fever but this time it was about 101-102.  We gave her tylenol and she went to bed.  Bryan checked on her at night and said her breathing was labored a little but not enough to panic, she had a vaporizer going in her room now for a few days because of the cough so she should be fine in the morning.  I said if she had a fever in the morning I would be taking her to the doctor.  Morning came, Bryan checked on her before leaving for work.  He reported back that her breathing was labored and that i should take her to the doctor that day no matter what.  My alarm was set to go off in an hour...


At 7:00 am on September 30, 2010, my world as I knew it changed!  I heard her get up and I laid there because she always liked to "wake me up", I heard her turn her nightlight off, go into the bathroom and call me.  As soon as she called me, I knew she was throwing up.  When I got to the bathroom, how I would have given my right arm to have her only be throwing up! She was sitting on the toilet and just looking at me.  I honestly thought she was still sleeping and tried to wake her ~ even to the point of gently hitting the side of her face.  It was almost like she was in slow motion and when she looked at me ~ it was as though the looked thru me.  She had lost all control of bodily functions so i had to somehow get her in the tub to get her cleaned up and clean the bathroom up.  It was hard since she had gained so much "weight" from being back on the steroids for almost 3 months and not to mention, she had no strength.  When sitting in the tub, she kept falling over.  Somehow I managed to clean her up, get her out of the tub and literally drag her to her room.  I managed to get a pull up diaper almost on her in case she "lost control" again and got pants on her.  Her heart was racing and it seemed like i could see it beating almost out of her chest...almost like it was going to explode.  I called Bryan at work and left a frantic message, Ben was still sleeping.  I called Bryan again, this time he answered. I was crying and said how she cant walk, her heart is racing and she isnt making any sense when she speaks.  He immediately left work and I called 9-1-1.  Thankfully we live literally a min. from the police station and they were there immediately.  They quickly took her vitals, got a mini medical history and when they heard she was on chemo and had a rare disease they wasted no time in rushing her to Childrens.  When i say they wasted no time, they literally took her downstairs, put her in the ambulance and left before i got Ben downstairs with a diaper bag, my purse and opened the garage. 


At the hospital we were in a room with what seemed like a million doctors and nurses.  I sat on the bed a little ways from Emily while they worked on her, trying to keep Ben occupied while Bryan was giving different information out and trying to keep Emily calm.  She was so worried about being late for school and what she was buying for lunch.  It seemed like an hour or two and they finally took her to a room in ICU.  They had her sedated and was placed on a vent at the highest settings.  They informed us that she was in septic shock and her body was shutting down.  They also said if it would have been just a few min. longer, she would have been in cardiac arrest.  She was diagnosed with Legionella Disease and Aspergillus Pneumonia. Remember in the previous post when she spent less then 24 hrs on a vent and i now know how the Lord was preparing me for the future?  Well this was the future and she spent 20 days on a vent. 


It was the hardest thing in the world walking into her room knowing we came so close to losing her and now she lay lifeless with more IV's & tubes coming out of her then I ever thought imaginable.  She had around 10 IV poles (it literally covered an entire wall behind her bed) and each pole had about 3-4 medicines coming out of it.  When they had to change places for the IV's they would poke her in her arms, legs, feet...wherever they could find a spot. As the days went by,  they started lowering the sedation medication (although when i would leave at night, they would give her a little more because it would take nearly an hour to calm her down and reassure her i would be back in the morning) during the day and she would open her eyes and try to mouth words.  Ever try to figure out what saying is saying when there is a vent that is taped to there mouth?  It was so hard but all she kept asking for was to go to the play room.  She didnt realize how sick she was. We took lots of pictures so we could show her when she was better just how bad it was this time.  She begged and begged me to buy "Camp Rock 2" DVD and the soundtrack.  I did and she would laugh and insist on it being turned up so load you could hear it nearly throughout the floor.  Her one dr would come in and laugh and sing with her sometimes too!  


She earned the trust of the doctors and nurses and they decided to take the restraints away from her.  They were so impressed that a child this young would just leave the vent in place and not touch it, while as adults, we would be trying to remove it first chance we got.  She received a feeding tube and she was doing well.  The day came during rounds when everyone was in her room and I got there just in time.  Her doctor looked at me and said "well, if she has a good night tonight, tomorrow we will be removing the vent".  I was nearly in tears that she was so much better and better enough to get off the vent.  Bryan and I decided to not tell anyone in case something happened.  The next day, I walked in and they all had big smiles on their faces telling me it was a good night and they are getting ready to take the vent out.  Now, ICU it is a HUGE  no-no to have your cell phones on.  I didnt care, I stood in the hall while they were getting ready to take the vent out, I had Bryan on the phone and the resident looking at me telling me play-by-play of what was happening (I could have been in the room but i wouldnt have been able to handle seeing them take it out). As soon as it was out, I heard a little cry and i heard her say "Mommy" ~ I cried as i had not heard my babies voice in 20 days. Bryan told his boss he was taking a half day and he was up to the room to see her.  Two days later, I received a phone call from the hospital, frantic, I answered it and the nurse said immediately that nothing was wrong but Emily was crying.  I got on the phone with her and she said "they let me pull my tube out and i want cereal".  Her nurse got back on the phone and informed me they allowed her to pull her feeding tube out and that they were moving her to a regular room!

We spent a week in a step-down room, where they were weaning her from her medications from ICU.  This was such an emotional roller coaster too!  So weepy and laughing hysterically the next.  We then were moved up to our favorite floor (5-west) where we saw old friends and it was nice to be "back home" or in a familiar place with more familiar faces.  We spent about a week there and then moved again to the rehab side.


What I didnt realize nor take into consideration is how fast your muscle shut down when you have been laying for so long.  Emily had to learn to sit, stand, walk, ride a bike, get dressed, every normal thing you and i take for granted...she had to learn to do it all over again.  She was doing so well too that they gave her a day pass and we were able to leave the hospital and surprise Grandma for her birthday.  We then went to the mall for a little shopping and then back to the hospital. She did so well that they released us 2 days before Thanksgiving.  It made the holiday that much extra special. Unfortunately, since this was just a couple weeks before our trip to Disney (remember, we postponed it from July to December) we now had to postpone the trip again ~ this time we tried to play it safe and we postponed it for a year ~ December 2011!


The put a picc line in when she was in ICU and she came home with that along with about 8 medications.  We had a nurse come once a week for a few weeks to change the bandages on her line.  She continued to go to out patient Physical and Occupational therapy twice a week for the next two months.  She finished up in January of 2011 ~ a month earlier then they anticipated.  She still takes 8 pills a morning and 3 pills at night.  She is still on the steroid that was started back in July 2010 and hopefully in February she can be rid of that!   


Since this last incident with Emily, we have learned that she will have to remain on chemo the rest of her life.  Any of the other medications, I not sure of right now, but I will be happy if and when we can start eliminating some of them ~ i know Emily will be as well.  Emily is such a strong person, takes everything with a grain of salt and just thinks this is "normal".  So normal that she plays that she is getting a CAT scan in the pool.  It doesnt bother her to have blood drawn, in fact, she insist they do it in the hand because it doesnt hurt as bad.  When kids say mean comments to her about her weight or call her that "bad F" word (F-A-T), she laughs it off while my heart breaks and i try to hold the tears in.  Her strength amazes me and i have learned so much from her.  All of the trials she has faced, she still continues to go on with a smile on her face, love in her heart and a gift of thinking of others.


**Picture was taken just before being admitted back into the hospital in May 2010 for the swollen gland