Emily meet HLH - part 1

Well, today is the day that I open up about Emily and her ordeal.  Today is the day that I become raw and allow you into the emotional roller coaster that we have dealt with since she was 2. Let me introduce you to HLH.

Emily was a "normal" toddler!  Sure she got alot of ear infections but many kids do.  Sure she had her bouts with a cold, bronchitis but dont all kids get sick?  Around January  15, 2007, Emily became ill with bronchitis. She didnt receive any medication and was sent home from the doctor. That was a Thursday...by Monday morning she was worse and I took her to a different doctor.  He did an x-ray and said it was now pneumonia and gave her some medication to take for a week and to come back to see him in two weeks.  By Sunday, she was lethargic & her breathing was scary.  I took her back to the doctor first thing Monday morning and he rushed her by ambulance to Children's Hospital of Michigan. We spent a week there and after several breathing treatments and some IV fluids, she was doing so much better!  We were sent home on Super bowl Sunday ~ only for her to take a turn for the worse.  By Tuesday, she was more lethargic then before and her breathing was beyond scary this time.  We went back to the doctor and again, another ambulance ride.  They immediately were taking blood from her a few times a day, they opted to do a bronchial scope ~ which meant they had to put her out.  This was hard, seeing my little baby laying there and not moving.  She did fine with that but found out she had a staph infection, influenza A and pneumonia.  We were moved to a private room! They did countless x-rays, blood draws and then they did a lung biopsy (she still has 3 scares on her side from this).  Again, nothing new showed up.  It was becoming the middle of February and we were no where closer to finding out what was wrong with her.  She was now throwing up, wasnt eating and had lost 6 pounds ~ not great when you consider she was only 26 lbs when she was admitted! She had had a blood transfusion and was now getting nutrients via the IV. Finally they opted to do a bone marrow biopsy but warned us it would take about 2-3 days before they had any results.  The Lord was with us because not even a few hours later a team of doctors came in and with smiles on their faces said "We know whats wrong with her".  They said it was Hemophagocytic Lymphohistiocytosis".  Ok seriously?  Are you speaking English to us ~ that is what I wanted to ask them.  They had smiles, we had smiles and everything was going to be great because they said they would start treatment in the morning!  

That night, it felt like such a relief to finally have an answer and then I googled HLH.  Bryan was home (on the phone with me) and he was doing the same thing.  Unfortunately, most of the information out there is about the genetic form ~ which is deadly!  This disease is 1 in 1 million that get it!  Usually kids that have this, do NOT live past 6 years old and they also have a 50/50 chance of survival after a bone marrow transplant.  Thankfully,  Emily has the infectious form.  That is good news, however, the treatment wasnt much fun.  They started her on oral chemo and steroids the next day (a Tuesday) ~ she was 19 lbs. By Friday, she was 35 lbs and could barely walk to the nurses station.  That Friday night, she was doing so good and since I was "living" at the hospital all this time, Bryan came to stay with her.  I was still working at this time and I went into work right after i left the hospital.  I got home and into bed about 3:00 am.  By 8:00 am, my world would change.  Bryan called me and said "I need you to come here right away, Emily has taken a turn for the worst and I need you to call your parents and have them call my parents." After getting to the hospital I found out Emily had coded on the floor.  She was unconscience, she had a small seizure and was now in ICU.  When we finally got to see her, I thought my world was about to end.  Seeing my baby just lay there, lifeless with cords coming out of her, IV's and on a vent ~ I thought i was going to be sick.  Little did I know, the Lord was preparing me for what was to come later down the road! She had some MRI's and CAT scans too and it was also mentioned she had Vasculitis (narrowing of her blood vessels in the front of her brain and the side of her neck).

Less then 24 hrs later, she was off the vent and sitting in a chair!  Because of her being in ICU, I could not longer stay with her.  This was especially hard on her when it came time to leave at night.  It was determined that she now had hypertension due to the steroid/chemo mix and she now had to start on blood pressure medications ~ 3 of them! She is a fighter though, because 7 day s later we were on our way home ~ March 16th ~ 2 days before her 3rd birthday! She was sent home with about 10 different medications to be taken daily and multiple times daily.

The follow up appts seemed like it was non-stop and I should have just rented a private room since we were there so much!  We were literally there every week and sometimes twice a week for different doctors appts.  After a couple months, it was now down to just 2 doctors ~ both of which we could see at the same appt. time!  She would continue to have many CAT scans and MRI's ~ putting her out for her "naps" now became a routine for her and "old hat".  

Just a month later, after a follow MRI, her doctor did not like to see that the chemo she was on wasnt doing what he had hoped for.  He switched her to a different chemo but this would be administered once a month but IV form.  This meant on the day of the treatment we would wait for the call to come in when a room was ready.  Then we would go and get admitted, settled in and begin the IV fluids.  Once her urine level got to a certain number they could start admitting the medication to help her stomach, then the chemo, then the medication and then 8 hrs of fluids.  This would sometimes be a very long process....the shortest time i think we stayed was about 16 or 18 hrs!  This went on for 6 months.  Her doctor then put her on another chemo that she would take orally ~ twice a day ~ for 18 months.  Still, continued with MRI follow ups.   

That October 2007, after her last IV treatment, I was laid off and found out I was pregnant with Benjamin about 5 months later!  This threw us a little curve ball as he was unexpected (especially since i have PCOS) and because i could NOT touch the chemo.  Bryan would dispense a weeks worth of medication (14 syringes) on a plate and twice daily i would bring the plate down for Emily to take one!  

In December of 2007, she rec'd a special ~ early Christmas gift ~ no more steroids!  After being on them for 9 months, she is now off all her medication but the chemo!


May of 2009, she was off all medication.  Clinically she was "healed".  Her labs were looking good, we were down to dr. appts about every 5-6 months and we were heading in the direction of thats it!  We reached the end of the rainbow.


But then....
~ check back  tomorrow as I finish the story.  I didnt realize it was going to be so long so I decided to make it a 2 part!   


See you tomorrow