entry has been made.

well, e got his first infusion yesterday. i have got to say that emla cream is nothing short of amazing. we have had some toruble with the nurse that was supposed to come out here, so i took him to st. joe's until we can find someone that is on board with the way we choose to access his port. when it was time to lay him on the table he got really nervous...he knows that usually means a lot of poking to get an iv in. plus, there was a nurse (that he has never met before) holding him down with her "hug" and she was wearing a mask. we were soon able to distract his apprehension and anxiety by singing "head, shoulder, knees and toes" and he didn't even notice when sara accessed his port with the needle. in fact, he didn't notice she was doing anything at all. it was great.

now i just need to get comfortable with all of this so that i can go ahead and start giving him the infusions.

port surgery went really well!

i was pretty nervous before e's surgery. i think it was the thought of my child having to be put out. and the fact that he was being cut open. and he just happens to have this disorder that keeps him from clotting well. so, yeah...cutting him open was frightening. however small the cut was, it was still cutting him open to me and that was unsettling, to say the least.

i got evan to the hospital at 8. his procedure was at 11, but he needed to be there early for an infusion. they also wanted to take his factor level an hour after the infusion, but before the surgery itself, so that they knew he was alright. that's a great idea in theory, but evan has got some of the hardest veins to find. so we ended up with two pokes by the nurses, then called in the iv team (i live mani...she's awesome). but even she had trouble with him and had to poke him twice. we finally got him infused, at 10:15. so it didn't quite leave an hour before the procedure (and lab time) to get the factor level back. it was decided we would go ahead and take a level after 15 minutes.

well, in an effort not to blow e's iv that had been put in his hand, they needed to find another vein to get the blood from. this time, it was the foot. this poor kid. mani got a little cathedar in him and was planning to milk him like a cow to get the blood to come out into the tube. but (in a strange irony) his blood clotted right away and we couldn't get the blood. the hemotologist tok that as a good sign and gave the ok for him to go ahead with the procedure.

***side note for other hemo parents, e's usual dose is 500 IU, but for this we had to go with 750.


the actual procedure itself took just about an hour. it was weird not being able to be with him. even though he was asleep and wouldn't have known if i were there or not. dr. coughlin (the surgeon) came in to tell michael and i that he did great and that we could go back to see him soon. when we got back there he was waking up from anesthesia and was crabby. but he drank juice right away and ate some graham crackers. he was connected to a bag of saline, as he would need fluids for the next 24 hours just to make sure he has enough in his body.

about thirty minutes or so after we went back to see him, we were moved to our room in the children's hospital. we stayed there for two nights, and were able to go home at 8pm on friday night. it was really nice to be home.

now we're on to the next part of this port...which is actually using it. e will start his prophy treatment around 02.18 or so. a nurse from hemophlia of the sunshine state has already come to the house to show michael and i how to use the port on a mannequin. we'll start the prophy treatment with a home nurse, and then transition to me doing them. i never in a million years thought i would be sticking my own child with a needle.

and here are a few pictures from our stay at st. joe's. i feel funny making posts without pictures. so here they are.

well, we're going to put in a port.

i have been waiting to get lab results back before i posted. we have been doing normal level checks and at the last one (after the picc line was pulled) we found that e's level was at 0.6%. when all of this started when he was two days old, he started out at 6%. he was 5.9% a few months later, and then down to 3% at around 1 year old. right before the head bleed he had labs done and he was at 1.1%.

as other hemo parents know, the doctors are always checking inhibitor levels too (well, every six months or so). evan has always come back with a negative inhibitor, but sara (our hemo nurse) is thinking that he has one that just isn't detectable by their measurments.

so michael and i took evan to a consult appointment with the hemotologist on monday where we discussed reasons for the lower levels and what our course of action is. it's speculated that evan does in fact have an inhibitor, but that it is so small that it is not showing up on their tests.

*side note: for those that arent in the hemo community and don't know what an inhibitor is, it is a protein that the body produces that essentially goes and attacks the factor that his body is making, which makes the amount of factor in his body much lower than it should be.

so the reason for the port is for us to be able to give evan prophy treatment somewhere over the course of the next six months up to a couple of years to try and trick his body into not going and attacking the factor anymore. IF (and i say that as a very big if...but only because i'm learning to stop being too optimistic about this disease) the prophy treatment works, then e's levels should be able to go back to their original levels (somewhere between the 3% and 6%) and we would be able to stop giving him the injections three days a week.

if the levels don't change and we are still showing an indetectable inhibitor, then we predict that we will keep evan on prophy treatment indefinitely.

michael and i are taking e to the surgeon's office tomorrow for his consultation and we may be able to get him in for surgery as early as next week on wednesday. this means we'll be in the hospital for three or four nights, so we should be home for the weekend. it's going to be a definite challenge trying to make sure noah is taken care of and brought to school and karate while i'm gone with e. this disease really does affect the whole family...and i wish there was some way i could keep home life normal for noah through all of this. but, he's a pretty cool kid and adjusts pretty well (and really loves his grandparents) so he'll probably be affected by it far less than i am worrying that he will be.

so tell me about ports.

it looks like we may be going that route, and i'm curious about how long it took you to learn how to access it. also about the types of tricks you use to keep your little one preoccupied and what the actual surgery is like.

i am completely speculating at this point, as we are still awaiting some lab results, but i am a big fat planner and would like to know the dirt.

for other hemo parents (well, non-hemo ones too)

i just ordered some really cool padding for the bathtub for e to keep him from hurting himself if (well, when really) he hits his head on the tub. i thought i would share the link in case anyone else is interested.

i also bought an inflatable spout cover from babies-r-us. it was a lot softer than the other spout covers. they offered plastic ones that seemed too hard for his head if he were to hit it. it was only $2 too, which was a very nice bonus =)

and i figured i would add a new picture because i am a shameless AW.

what a long, strange trip it's been

yeah, no...this is not a post about the greatful dead. in fact, i'm not even a dead fan but i was looking through some cd's yesterday and i guess my husband has a couple of copies of that album and the title just seemed to fit the way i have been feeling the past week.

so, since my last post we have been discharged from the hospital. it was found on sunday that evan did not have an infection in his picc line afterall. the first blood culture was probably contaminated and gave a false positive. the second and third cultures grew absolutely nothing. now those that know me know that i'm not really all that in to god (in fact, i don't really know how i feel about god and religion...but that's another post for another time, i suppose) but i swear that there was some sort of divine intervention that lead us to the hospital for when evan had his seizures. and i am so, so thankful that we were there when it happened.

so we spent sunday night and the greater part of monday at the hospital, just waiting to get the all clear from the neurologist to go home. e had an eeg on monday morning and it came back normal, so we were told it as alright to go home. at first, evan was terribly cranky and just not himself. i was worrying that this whole episode had somehow changed him. and i was feeling sad about it. and worried. for those that don't know him, he is just the most laid back kid ever. he's always happy and cheerful and just the sweetest thing ever. seems like he got the very best qualities of both michael and i and shows those traits all the time. anyway, after 24 hours of not knowing who this child was, michael and i thought that maybe it was the increasae in the antiseizure meds that was causing the difference. so we called and spoke to the on-call neurologist about decreasing his dosage from 3ml's back to 2ml's, since the seizure were related to the fever he had. the doctor said to keep the 3 at night but to give him the 2 in the morning, so we took that to mean that we could change them both back to 2. and, sure enough, that brought our sweet angel back to his normal self. seriously, it has made all the difference in the world.

so we were able to enjoy our christmas together, at home, as a family. it was the greatest gift i have ever gotten. honestly. i know its silly to say, but i couldn't have gotten a more special gift. ever. i went from thinking that i was going to lose a child, to thinking that my child was going to be a different child, to having him back to himself and loving everyone and everything around him. and that was something that meant more to me than i could ever really put in to words.

a friend of mine once told me that once you have children you give hostages to fortune...and that they will age you in dog years. and boy, was she ever right.

oh, what a long weekend.

you know, the funny thing about this disease is just when i think we're out of the woods with something bad, something else happens.

on friday afternoon evan had a fever of 101.4. usually i'd just give him some tylenol and let it do its thing, but since he has the picc line any temperature over 100.5 means he needs a blood culture done in order to rule out infection. so i called sara (our wonderful hemo nurse) and let her know and she told me i should bring him in. i didn't realise this meant we would be admitted for at least 48 hours. picture me hearing this in the middle of a public hallway and starting to cry. i really need to work on this emotional shit.

so we are brought up to our room and the nurses take blood cultures and urine samples and start pre-emptive antibiotics. standard care, i suppose...and i'm still infusing him every 12 hours, so i am figuring we will just be here for the next couple of days and then be done. his cbc comes back and the nurse explains that his white cell count looks good and that she doesnt think he's fighting an infection, so we are probably going home on sunday.

the next morning after the shift change, the new nurse breaks the news that there is bacteria starting to grow in his culture, and that it probably means a 10 to 14 day stay here. dammit. it's christmas and we have an older son and what are we going to do? we're supposed to go out of town, but that can at least be put on hold for now. and i immediately start crying. again.

i manage to stop the tears rathar quickly and focus on the fact that my son isnt feeling well and needs to get better. his temp has been in the 101 to 103 range over the past 12 hours (even after having tylenol) and if he's going to be sick somewhere, i suppose this is just the right place.

fast forward about six hours to the scariest moment i have ever had in my life. michael, noah, evan and i ware playing in the playroom. i stepped out to run back to our room to grab e a snack and some milk and i come back to noah scurrying about putting on his shoes. he tells me that michael left with e because something is wrong with him. i figure this is noah jsut being dramatic, so i walk out calmly to go find them. michael is holding evan at the nurses station and when i get up to see him, evan's eyes are rolled up and to the left and his face (and lips especially) are blue.

my baby stopped breathing.

michael handed him to the nurse, who rushed him back to our room. she laid him on the crib, tilted his head back and gave him the breath of life. it took a few seconds, but he started to breathe on his own again and his color started to come back.

i am trying so hard to be a mother to both of my boys at this moment. the oldest one, who is bawling his eyes out because he just watched his brother stop breathing and turn blue. and the youngest one, who just stopped breathing.

and all i wanted to do was puke.

immediately after this happened, evan sat with michael and was just not himself. his eyes would only look to the left. he wouldn't respond to me when i called his name. all he would do is chew on a paci in a way that he never had done before. finally after about 10 minutes he was showing signs of getting back to normal.

when the nurses determined that he had a seizure, they paged his hemotologist and rushed him down for a ct scan. all within about 30 minutes the ct scan came back normal and showed no sign of hemmoraging in the brain. which, of course, is good. but it doesnt explain why in the hell he had a seizure.

so, after all the drama of the evening, michael told me to go home and rest. not that rest was really possible, but i still have a ton of shit to do. laundry to fold. presents to wrap and put under the tree. more crying to do. so i left. reluctantly, but i went.

about forty-five minutes after i got home, michael called and told me that evan had another seizure. same thing as before, but that he was alright. i threw my stuff back together, got int he car and made it all the way back to st. joe's in about 20 minutes.

when i got back, evan was all doped up on atavan and completely asleep. he looked so sweet, and all i could do was press my lips against his cheeks. and, of course, cry. he was moved to the picu where he was to spend the night. michael had me leave, so i drove three minutes down the road to my sister's house and spent the night there.

i came back to hear that evan had an uneventful night, and i was pretty thankful for that news. we spent the day wandering around the picu, seeing things i would prefer to not see again any time soon. eventually we were moved back to the observation unit where we started out on friday. the hemotologist came in and told us that there is no bacteria growing in evan's second blood culture, so they now believe that there is no infection in the picc line. but, since the ct scan showed that his hematoma had reabsorbed into his brain that we should be able to stop the factor and take the picc line out. if he still needs the antibiotics we can give those orally.

so, that is pretty much where we are at right now. except he still has a high fever. it was 103 about two hours after he was given tylenol. the fevers are worrisome, because they are what the doctor believes are causing the seizures. so here i sit, in a bed next to my baby, thinking about sleep but pretty sure it won't really come because i am scared out of my mind that his oxygen levels are going to plummet and they will have to get him breathing again. and then my mind drifts to the "what if" part...and i don't really need to be there right now.

but at least i'm not crying.