Saturday, March 31, 2012

This is going to be one long post....

So our IVIG, Cytoxan, and Remicade infusions went well.  Sunday we were discharged.  We loved our nurse.  We were sent home on a G1 diet until he started tolerating a full diet with no diarrhea. 

Monday Morning
Monday morning Gary had a well child check up scheduled.  I thought why not.   Kindergarden round up was Thursday so it would give me the perfect opportunity to chat with him about what to do.  I had or do I have no intention of any kind of immunizations at this point.  So the whole point became a complete act of God.  As if He knew.  I woke Gary up for a shower.  He had crawled into bed with me at about 230 am with a temp of 102.0.  Thought nothing of it because he has been running fevers.  It aggrevated me because as a mother, you know.  You know something.  But tylenol and by morning as was ok.  I dressed him.  Gave him his medications.  Put him in the car.  He was quiet on the way.  Too quiet.  He moved very little.  Asked very little questions.  Not Gary.  Gary is usually full of anxiety.  He is about planning his day and what comes next to benefit him.  What five year old isn't?  Check in at the pediatrician, blood nose.  Ok.  No biggie.  Odd to me but whatever, kids get bloody noses.  Weight check, bp check, temp check.   101.4 Clearly not feeling well.  He shook  from the high fever.  Our awesome pediatrician look him over.  We talked about him not being ready especially being in the middle of new medications.  On the table Gary looked awful.  Our pediatrician asked questions.  Asked if he had vomited.  I said no.  He said take him home, let him ride out the side effects of the chemotherapy.  Ok.  Pure honestly.  The moment I scoped him up, I scrambled for my phone calling Children's Hospital as I walked out the door.  Maybe I should have shown my pediatrician the respect of letting him know I was not comfortable with any of this.  Only ten minutes into the car ride, he vomited.  I was on my way to the Emergency Room at Children's.  There was a little blood in the vomit but I though oh, bloody nose. 

In the ER he was running a fever.  They put him right back in the room.  The rheumatologist showed up a lot quicker than normal.  Gary was very sick.  He acted funny with his movement.  They felt confidant it was a reaction the IVIG infusion he recieved over the weekend.  A harmless, well, bothersome, but nothing they could do anything for condition that would have to resolve on its own.  Aspectic Menigitis.  So benadryl and tylenol were given by mouth because we figured we were going home.  Until his labs came back. 

The two of many doctors that had chatted with me entered the room with a copy of the labs.  The news was broke.  My sister and her husband were sitting at my sides.  It became a roller coaster ride.  The works spinal tap spilled for her mouth like black sludge.  My outter tough shell shattered into a million pieces, allowing the tears to instantly spill from my eyes.  A lumbar puncture.  I wanted to run.  I wanted to leave.  There was no oxgyen.  But I had a job to do.  I called my husband over facetime.   "We just got some bad news."  I explained he about the labs are elevated, like his white cell count was at 31,000.  Inflammation markers and one in particular that called for the spinal tap, were all elevated.  I cried.  But I knew it was game time.  I knew in an emergency room there is little time to prepare.

What seemed like a whole lot of people dressed into contamination gear filed in.  It was very overwhelming.  Doctors, nurses, and the four of us made for one full room.  Gary cried in fear.  He begged us to tell him what was going on.  I couldn't.  I didn't know what to say.  Oh, your about to have a very large need poked deep inside your back.  Yeah, not good to tell a five year old.  Thank heavens for my sister.  She remained almost stoic.  So still,  so calm, because she had too.  She looked at Gary and said he was having a test done and everything would be ok.  I could only weep.  I could not look at him without fear.  I was not helping.  The sedative they were trying to give him through yet another IV,  was not kicking in.  A lot people standing around in the room, waiting for this five year old who comically enough would not shut up.  The senior doc in charge of the procedure said until he is not making sense we need to keep pushing sedation.  Three vails later at his max, it was go time.  The annoucement of what was about to happen was chilling.  "We are here with Gary Richard birthday.  We will be preforming a lumbar puncture".   Ok why don't you just sock me square in the nose while you say it.  Maybe a quick knee in the stomach.  We began to hold him.  You can only image how important it is to keep a five year old still during something like this.  Of course he was panicking.  Hot, screaming, panicking.  We had an oxgyen mask on his face to help him feel like he wasn't. 

When I was a kid, we would love to rough house and put our siblings knees up to there chest and lay on top of them.  I would often feel like you couldn't breathe.  I can only venture to guess that is the way he felt.  5 adults had him.  It wasn't working.   The doctor who seemed in charge knocked the nurses out of the way.  He said he had it.  He pick Gary up, bent him in half with force, opening his spine.  Gary was in misery.  The man was shaking from muscle fatigue trying to keep him in place.  It was pure choas.  I was of course by his face, at the head of the bed, so I had full view of the procedure.  The doctor performing the puncture shook.  The large need shook.   It was very difficult.  So many things moving, yet this need to be done.  It really did.  Bacteria meningitis is something they can't miss.  The needle finally went in.  It was awful.  It was just bad.  They filled five tubes with his spinal fluid.  After it was all said and done, the sedation took full effect.  The room emptied, and he sucked on his "fresh air" high as kite.  He was calm, tired, laying flat.  They immediately began running antibiotics.  There was no time for results. 

That night, once we got up into our room, he was still sick.  He ran high fevers all night.  He sleep a little. He placed his hands behind his head and just layed there.  The next morning he seemed better.  The fevers however were still low grade hanging out.  The next morning he felt better.  His labs improved so much.  The discharged with a diagnosis of a reaction to IVIG.  No more IVIG.  They were a little hestitant but confidant.  So Wednesday afternoon we arrived home and he was doing fabulous.  He ate dinner.  Slept well.  No worries.  Thursday morning I got a call.  I know Children's number and was expecting it to be the scheduler because I had just called her to check what was scheduled.  I could hardly believe the words the rheumatology fellow spoke.  He had a late growing lab come up positive.  Please return to the ER.  I floated, looking around my house as I spoke to him, "You are playing a joke on me, he is just fine."  The voice replied it would never joke about something this serious.

I ripped my clothes off and started making calls in the shower.  I called my husband, then my sisters.  I called my Mom.  My sister backed her bag back up and came over.  I had no clean laundry.   No food.  No gas.  We had just spent five days there.  I was not prepared.  I knew I had to go so I did a lot of pacing around my house.  Gary was feeling fine at this point, which made it all that more difficult to go back.  I stalled.    I was afraid to tell Gary but since the antibiotics they ran on Monday, this kid is new.  He is happy.  He is euphoric.  Part of me is nervous he is too happy.  Like is this effecting his brain.   We did it though.  We packed up.  Made a stop at his sisters school.  I wanted to explain to her face to face what was going on.  She cried.  But there was nothing I could do.  I had to go.  I had to leave her there crying.    Tears won over a blood infection in her brother.  We came into the ER.  They expected us.  I promptly asked what the labs looked like.  The intake nurse immediately looked it up.  He tested positive in his urine and blood for a gramm rod negative bacteria.  At the time he identified it as ecoli.  A ecoli infection of the blood.  Everyone was surprised how well he was doing despite what his labs were doing.  I promptly reminded everyone that he had already recieved 48 hours worth of antibiotics.  They were clearly already doing there job.  But with a patient who is immune supressed, we are in dangerous territory.  He had an xray done of his tummy.  It was and still is the focus of the culpret of this bacteria.  Weeks ago when Gary had the colonscopy done, they saw ulcers.  I have a feeling it is not what they saw, but what they didn't see.   The Xray however didn't show any preforations or abcesses.  The more anitbiotic they give this child, the more super power he seems to have. 

I wish I had more opportunity to update this,  I will tell more later.  We are so tired. 

Saturday, March 24, 2012

Cytoxan, Rituxan, Remicade

Gary turned five years old on Wednesday.  I knew on Tuesday he needed to come in.  I knew after they did not admit us during the ER visit, that we were living on borrow time.  I called and asked we spend his birthday at home.  No one ever called back so I took that as a yes.  By Thursday, the diarrhea was still getting worse.  I made the call.  They had us come in on Friday for a clinic and planned to be admitted. 

It was good timing because on Thursday night, he ran a low grade fever, again.  We got to meet our new doctor in clinic. She did a question and answer session, and catch up.  She could see he was miserable.  He needed to go to the bathroom a few times during the clinic visit.   She said I am glad you brought him back in.  He needs to be here. 

Things happen quickly.  I will have to get use to her new style.  I like her aggressive approach.  Right from the start of getting this whole thing put back on a different track,  we stopped tacrolimus.  So far that is a total of Cell Cept, Tacrolimus, and Enbrel.  The plan was to admit him for a Remicade infusion.  Remicade is used to treat IBD and JDM, so like I posted on the JM Mom's Board, it suddenly became a might as well, we need to do something.  Of course they put him on Nothing by Mouth.  I told them please don't do that.  Run a high dose of steroids then don't let him eat.  We got water, broth, and a popsicle.  This morning he was up graded to a GI Limited diet.  Mash potatoes, crackers, Pediasure, banana's, rice.  Oddly, once given the clearance to eat he only had a graham cracker and the Chocolate Pediasure.  The chocolate pediasure filled his tummy I think.  I let the nurse know however, within 20 minutes he went 4 times.  She didn't seem concerned so.  He has been sleeping a lot.  He slept 14 hours, and is napping now. 

The plan now is to run Cytoxan and IVIG.  We should be able to get out tomorrow.  Then come back in two weeks for Rituxan and Pamidronate.  Our pamidronate infusions she moved to once a month.  Cytoxan will be once a month.  Remicade will be in another two weeks, then 4, then move to 8.  She does not know how long to run the Remicade.  She has been communicating with one of the doctors that came back in June.  They recommended the Enbrel shots which we noted no marked improvement or difference.  So they think starting Cytoxan again is a good idea with the Rituxan. 

This is all a lot.

This will be hard to take him home.  Not knowing.

Gary's veins are irritated by all these infusions.  He screams when saline is flushed through.  We always stop to make sure his IV hasn't blown.  But it is just that his body is irritated.  How do I let them keep giving him all this stuff.  I see pink eyelids.  I see it.  I see the JDM troll in the back of the dark woods rolling on the ground laughing and celebrating with evil.  Well I am standing at the edge of those woods.  Coming to get him with a baseball bat in hand.  A beating is in order.  That is how.

Thank you Summer for your note : )  I know you asked a few questions, I don't have the note you posted in front of me to answer. 

Monday, March 19, 2012


Gary is doing good. After a visit to the ER last week for a fever that wouldn't break on Wednesday, he seems to be bouncing back.

101.4, 102.2 even with tylenol, we called to see what they wanted us to do. The kids were just arriving home from school when we packed up. Elexis was just as upset as Gary was about having to leave. Elexis was turning ten the next day. I couldn't tell her whether or not we would be returning home. We can only be twoo careful however. The hospital is dreaded. I am sure it is for most people. However, when the effect of distraction because something outbod your routine is happening that tends to distract you. When you have been doing this for three years, that whole concept only plunges you into the unpleasantness of the ER visit. Gary wasn't sick per say. It was just the pesky fever that kept going up despite treating it. An emergency room isn't the best place for an immune suppressed kid to be but we had to do what we had to do. Not sure that is playing it entirely safe especially since it was like a scene from The Walking Dead (a tv show a few members of my family watch that I can't because it is too intense, zombies, blah). When they finally got us back to room, the rheumatologists intern who had just seen us from the week before popped in to say hello. She was on her way out for the day. We talked about what was going on. She said they were talking about admitting us, and getting the medications going that we were waiting on starting until he recovered. After an IV start, and one nasal swab later, labs looked so much better than they did the week before. The 5ml of steroids are bringing those labs down nicely. Not to mention it is also knocking out the heliotrope rash. Inflammation markers are still very elevated, a few started to trend down. The ER doctors came in, went through the usual student doctors. A mirage of questions, me educating them on what they are dealing with. Pleasantly, the student doctor knew of JDM, and DM as well. His neighbor, while growing up had JDM, and so did the father of this child. I always thought that was very rare but it is said to be genetic. The father I am familiar with. I remember reading about him. He was a famous football player in the seventies. Suddenly he became very tired. Running the football field became a huge challenge for him, ending his career. Anyway, another lab came back from the nasal swab. It tested positive for a virus, the coronavirus. Basically it is the common cold virus that is respiratoryish. It can cause many things. They asked if we wanted to go home since we knew what was causing this fever. I wish that made things easy. I knew getting that diagnosis was my chance to escape. There is nothing they can do for a virus but with a child who has what he has and its all complicated, you would think they would want to keep an eye on things. The rheumatologist who was attending did not even bother to come talk to us. They got the go ahead to release us. Our whole ER ordeal took about seven hours. We had spent enough time in the hot zone. I was feeling like the best thing to do was to get the heck out of there before we picked up something else. After sharing a bathroom with a little girl who looked terrible and another kid who practically puked at our feet, yeah, done. Gary was in the bathroom about ten times. I had no choice but to run out of there. My favorite part of the ER was by the time we got there, the fever broke. Ugghhh!

The next morning I called rheumatology to see if I was suppose to hold the methotrexate shot because of the virus. They called back and said yes. Later that day one of our doctors called to say we were suppose to be admitted. The wanted to get to work on stopping the diarrhea that had returned. Somehow, the ER didn't get the message. Neither did the rheumatologist on call. Everything happens for a reason.

One of the plans after the Thursday meeting is to bring us in. In order to give Gary immediate rest and healing to his colon, they want to check him in and put him on TPN, while on NPM. That means for a few days he wont be allowed to eat, and given nutrition by Iv. Uh, no. They want to start remicade which I am comfortable with of course but not the whole TPN thing. We have been on that before and it is difficult. Very difficult. I mean, I only will approve this if he just doesn't get better and it is completely necessary. I asked her to give me a few more days. i told her want this virus to cycle outbofbour house. Yep, we all ended up getting sick. Sneezing, congestion, nose burning, ect,. Give him a little more time to improve. Let the steroids keep working. The flare has come under control. He stopped having blood in his diarrhea. Let's just see. If I don't like what is going on, then I will bring him him. She was completely agreed. She said they are willing to let me drive this.

Where is the car headed? Well, he seems to have had a good day yesterday. The day before he did go 17 times. It wasn't all diarrhea but he still has one set of active bowels. The next day he went 8 times so big improving. I still am getting the feeling we are living on borrowed time however. I had hoped to make it too our original appointment on the 27th. On Wednesday he turns 5. No birthday in one of his least favorite places. We will see by the end of the week.

Saturday, March 10, 2012

Where To Begin

I wish I could start this with telling you about our March infusion.  I wish I could say it was all easy peasy.  but it wasn't.  Monday was our clinic appointment.  We saw our rheumatologist for the last time in clinic.  She is retiring in April.  Everything at that point seemed fine.  Gary had run low grade fevers three different times in February.  We treated with tylenol and no other symptoms besides the obvious and same old diarehha.  By Tuesday things took a turn.  The fevers took a turn for the worse.  He woke up at 8am.  Came down stairs and went to back to bed on the couch till 1pm.  I kept checking on him.   I noticed his face swelling in his sleep, and a rash coming in.  When he finally woke up, I gave him lunch.  He ate a little bit then complained of headache.   I found the tylenol and noticed he was warm.  A fever of 102.  Before the tylenol could digest he vomited explosively.  I called and left a message with the nurse.  I assumed they would tell me not to come in for his infusion the next day.  But his doctor wanted him in.  Fever or not.  He felt much better after vomiting and even ate a little dinner that night and kept down his steroid dose.  But at 10pm another fever.  At this point I had a choice.  Give him more tylenol and sleep one last night in my bed or head to the ER.  We are veterans of this.  We have been here before.  So I decided  to give him tylenol.  If the fever broke then we would get up early and head to the hospital.  If it didn't then we would head out at midnight.  The fever broke.  We slept the night, well barely for me anyway.  The next morning we were admitted.  Things started to unfold.  During the clinic appointment it had been determined that Gary would need a colonscopy and endoscopy.  A level of inflammation was detected in a stool sample.  It was a level that was suppose to be 100 but came back to be 2500.  With that they said it was worth investigating further.  He was already scheduled to be admitted for IVIG and pamidronate so they were able to get him in for the colonscopy and endoscopy. 

The endoscopy and colonscopy was really hard.  After the labs came back crazy high, we got comfortable for an overnight.  Something is not right.  As soon as we checked in it was deteremined that he was scheduled so nothing by mouth was ordered.  The day before he was vomiting so he was really thirsty.  My day and his day went from bad to worse. After two hours of begging for something to drink, he was finally cleared for clears.  Chicken broth, jello, sprite, or clear candy.  All mixed with Miralax to clean him out for the colonscopy and endoscopy.  On top of already having diarreha plus giving him benadryl and the IVIG.  Made for an interesting time.  Imagine being sleepy but need to go to the bathroom.  He was miserable.  The misery sent him into a breakdown.  He awoke from a nap.  He was mad.  He kept standing on the side of his bed screaming, calling me names.  He threatened to pull his IV out.   He would not let the nurses touch him.  They needed to get vitals.  It was impossible.  As we came towards the end of the IVIG he became even more agitated.  Despite running it over a ten hour rate, stopping the infusion half way and giving him benadryl, he still had a reaction.  A large headache, hives rash all over his chest and unnatural irritation.  We abruptly stopped the IVIG.  They tried to run more benadryl but he was very intolerant.  I told them to turn off the IV, he had enough.  He was suppose to get pamidronate after that but he clearly had enough.  The IV was flushing fine but every time they tried to even push a flush, he responded.  Everytime we turned it off, he fell aspleep.  It was agreed to let him sleep through the night.  He had a procedure in the morning.  The day before he had been throwing up.  His body had enough.  He slept the night away.  Well, in between vital checks at which point they detected irregular heartbeat.  Turned out to be nothing.  Then his Bp dropped too low.  The nurses didn't like it but the doctors said it would be ok.  For the most part, his BP is on the high end.  So being too low was a nice change.

We woke up the next morning at about 730.  We were scheduled to be transported to the surgery center for the procedure at 9am.  I was releaved when they came early.  After midnight before he was under NOTHING BY MOUTH at all until after the procedure.  So 8:15 rolled around and they came and got us.  Thank you Lord.  The quicker they got him in the better.  I had not taken the time to tell him what was about to happen.  I didn't know how.  How do you make a five year old understand?  I decided to wait until we were in the prep room.  I explained they were going to give him medicine to make him sleepy.  He was going to take a nap.  The bad IV from the night before was removed before so I was thankful to hear they were going gas him before starting a new IV.  He cried.  He cried a lot.  He told me he was scared.  Several times he needed to use the bathroom.  It was hard to talk to nurses and doctors in between running him to the bathroom.  He broke my heart with begging me, "Mommie, please.  I scared.  I soo scared.  Don't let them put me to sleep."  He cried for his Aunt and Uncle.  Thank the Lord again to them for coming again!  It made it easier.  It was time to wheel him into the procedure room.  He wept.  He wept as they placed the gas mask over his face.  I stayed as close as I could to him.  I remained positive and non chalant so he would not be scared.  I acted as if it was no big deal.  I refused to take my game face off.  As the gas took affect, his eyes rolled into the back of his head.  His knees fell to the outside of his body and he was quickly asleep.  The doctors looked at me and said, "ok Mom, give him a kiss".  I blew a kiss, he was out of my reach.  I couldn't bring myself to actually place my lips on his skin for fear the tears would flow in front of them.  I was not going to let that happen.  Game face.  Quickly out the doors with my sister and my brother in law.  I marched.  I marched straight faced.  I found a chair, sat,  and barried my face in my hands, letting my emotions flow. I kept it to five minutes.  After my sister said something to make me laugh, we went for coffee.  An hour later, we got the page to return to the procedure doors to meet the doctor.

The results of the scopes did find he has ulcerations in his colon.  Blood in the diarreha makes sense now.  It is why that is on hold.  Throat and stomach look normal.  The ulcerations in his colon are on the deep side but not crazy.  They took biospies.  They also took a lot of blood to test everything.  They want to make sure we are covering everything.  Infections, bacteria, viruses, ect,. 

After we talked to the doctor he told us he was back in his room.  We bolted across the hospital to get over to the room.  He wasn't there.  He was still in recovery.   We were told to wait in the room.  I couldn't.  I couldn't sit there while my child woke up after begging not to put him to sleep.  I had to be there.  The nurse sent us back to where we were.  It was hard getting back there into the procedure room.  They would not let me back there until he was waking up.  They put me in a room the was for people going into procedures.  I sat alone for 45 minutes.  Finally there he was.  Crying in recovery.  Not much but he survived!  : )  We didn't spend much time in there and got back to our room.  Back in his room he slept.  He didn't even bother to eat.  He just slept.  I decided that we would come back for the other infusion we skipped the night before.  He had been through enough.  I had enough and so did he.  The doctors were fine with that.  We wanted to wait for him to wake up to make sure he was eating and going to the bathroom.  He woke up in a good mood and immediately started eating and drinking. 

We started packing up because there was no use sitting around waiting for test results.  We noticed Gary's face was pretty swollen while slept in recovery.  When he woke, the infamous JDM rash set in hard.  His eyes were purple pink, and his lips were huge.  I was concerned put thought it was from the fact he had a breathing tube down in throat.  That maybe that trauma was contributing.  We were packing bags, and getting ready to get out of there when the nurse said, "the phone is about to ring, please pick it up, its the doctor."  She explained that they had done tested his blood for levels of his medications.  One lab was in, the cell cept or MMF.  It read 0.  It meant that he is not absorbing his medications.  She asked us to hang out a little longer until they could talk, and figure things out.  The bright pink eye shadow he was wearing suddenly started making sense.  High labs, bright JDM rash, vomiting, he is flaring.  An hour later, after I stopped spinning they came in.  I immediately told them he needed to be pulsed with steroids.  I told them I felt his JDM is flaring.  He is no longer on cellcept.  They also discontinued the enbrel shots.  Deciding to pull him off of what we can right now is better.  The next morning we were going to determine what to do next.  Since tests weren't going to be in till next week, they upped his steroid dose back up to 5ml.  From 1ml to 5ml.  After the intravenous pulse he looked a little better.  We had opted to skip his methotrexate shot the night before because at the time we thought he was "sick".  It is looking less and less like that is what is happening.  That it is in fact, JDM.  Since we were working on our third day in the hospital, we went ahead and did our pamidronate infusion.

So now we wait for Monday, possibly Tuesday.  He is doing better but everything is in the air.  I hate hanging.
Our nurse flushing his iv in his foot. She was awesome!