the calm before the storm?

i realise this is pre-emptive worry, but i wouldn't really be me if i weren't worrying for nothing about something. so, cold and flu season is almost here. it's a real bitch by itself, but throw in a kid with a port and it becomes a raging one. so i find myself trying to enjoy the days of playing outside and sending him to the gym daycare with the other children before i find myself wrapping us all up into a plastic bubble so that we aren't living out of st. joe's this winter with small fevers that hopefully haven't turned in to port infections.

i have decided that i am going to be a bit more commanding about this, though. i spoke with kathy (a nurse from hoss) about the possibility of doing the first 48 hours of antibiotics at home while we waited for cultures to come back. for non-hemo parents, this would be the first 48 hours you are mandated to stay in the hospital at the first sign of a fever (which, i should tell you is only allowed to get to 100.5...oh yeah, and you can't leave until you are fever free for 24 hours). she said that, while it is up to the doctors in your practice, it is not unheard of to allow parents to administer these antibiotics at home while you wait for the cultures to come back. so i am going to try and strong arm the doctors to follow this plan with me. but i am going to go in ahead of time and try to sweet talk them first (are you reading this, sara!?!). i figure, if i am capable enough to stick a needle into my son's chest every other day then i am more than capable of keeping it in there (with the biopatch on) and hooking up some antibiotics every 8 hours. i would even push it slowly over 30 minutes if they were unable to lend me a machine. i would also promise not to bathe him or allow him to run around like a wild beast with a needle sticking out of his chest.

you see, it's not that i don't appreciate the doctor's perspective. i do. truly. however, something i learned last year when we were in and out of the hospital three times over two months, i realised that noah gets pushed to the side. and while he would never begrudge his brother the attention, devotion and care he needs from his parents, it definitely wears on him. he worries for his brother. he misses his mom and dad. he misses being home. and being without him for so long...not being able to show him attention and affection daily like i would if i were home is really trying. and one thing i am trying so hard to do with this disease is to keep things as normal as possible. for everyone...evan, noah, michael, and myself.

who knows, though...maybe i need to redefine normal.

sometimes i forget i am here.

well, i mean...i forget that i have this place. i don't know if thats a good or bad thing. on the good side, it means that things with e have been good. nothing really to report other than we are still doing prophy three days a week. it still feels emotionally taxing at times. sticking your kid with a needle has come to be the easy part. its the mixing and the thoughts that swirl around in my brain while i am getting things ready that are tough. its a constant reminder that my idealistic thoughts of parenthood with regard to him will always remain just that...idealistic. and really, i didn't have any delusions of grandeur or anything. i just never imagined having a child with a chronic illness. my therapist referred to the situation once as me having a "sick child." and while i never use this term, the conversation stuck with me. he is sick. he will always be sick. nothing, well now anyway, will change it. and it sucks. it makes me mad. it makes me cry. and most of all, it makes me wish there was something i could do to change it. not just for him and our family, but for every family that suffers from this little known illness. it doesn't get the same sort of press as cancer or hiv or diabetes. but it is just as taxing and scary. i find myself worrying all the time if he is bleeding and i don't know it, or if some parental decision that we make will be the right one. the constant worry and second guessing is so trying. and i am not the most patient person in the world.

on the bad side, my not being here makes me feel like i have alienated a population of people that regularly check in on this site. its weird to me that people from all over check this place out...as far as europe and asia. and my lack of updating reminds me that there may be moms and dads suffering from the same anxiety that i suffer from, with nobody that truly understands it. when e was first diagnosed i wanted nothing more than to quietly separate my thoughts and know that i wasn't alone in my fears and frustrations. and i wanted someone to tell me that everything would be alright. and while i cannot promise that...because, really, who can...i feel like i can offer some sort of relief in knowing that doing the best you can will yield good results.

and, while i am at it, i should say to all you other parents facing the same sort of obstacles that we are...if you ever need to talk. or cry. or vent. i would totally be willing to listen.

hey! help a hemo out =)

so, our spring walk for bleeding disorders is coming up at the end of next month. my goal this year is $3000. i know it sounds like a lot, and its such a strange time to be giving any money away, but the hemophilia foundation does such great things for families and research and any amount that you can give to help out would mean the world. so, if you want to donate you can here. i promise you'll feel good about where your money is going!

our son, the medical anamoly.

so we got e's blood results back from his lab draw this week. first, his factor level was above 1% after 72 hours which i am told is what they want to see. so, yay. the two tests he got (the lupus anticoagulant and the mono test) were hopefully going to solve the mystery of the decreasing factor level. but...its not to be. both tests came back negative.

so. back to the drawing board.

currently, evan is under the care of the oldest doctor in the practice (i'm going to guess he's about 70?) and sara (the nurse, not me) said that he told her he has never seen this before. i assume he means the levels decreasing, a positive test for an inhibitor and no medical explanation.

it was decided that we would try immuno-supression for the week. i gave him his regular 1250 friday dose, and will be infusing him with 1000 units every day for the next seven days. that puts us out to next saturday. then the following monday i will take him back to st. joe's where they will draw more pre-infusion blood and do the mixing study again to see if he still comes back positive for an inhibitor.

michael and i are thinking that if he does come back positive for the inhibitor that we are going to consult a second opinion. i feel weird saying that, because i love the practice that we go to. and i love sara so much too. and i hope this sort of thing wouldn't hurt their feelings, but i feel like maybe we need to see if someone else can help us solve this mystery.

poor boy is only 21 months old and i already long for the days of the 5.9% levels.

i've never been a fan of speculation

and i have come to find that so much of medicine is just that. speculation. and waiting. one of the doctor's in e's hemotology practice brought it to our attention that if his levels have been decreasing that there has to be something going on...even if he has consistently tested negative for inhibitors. so i took him for some bloodwork a couple of weeks ago where we drew blood for a mixing study. i say we drew blood because i drew it from his port. it is so weird how i am now capable of doing small medical procedures for my child that i never dreamed i would be responsible for.

anyway, the mixing study results came back positive for some sort of inhibitor activity. or, at least, i think that's what they said. there is so much that i don't understand right away and i tend to get my facts confused.

so, back to the lab tomorrow. for more bloodwork. this time it is not to be drawn from the port. they are going to run tests to see if evan is positive for a lupus anticoagulant or if he has been exposed to the mono virus. both of these things, i have been told, can affect his factor levels in a natural way. i was also explained what they were (the short, peripheral-hemophilia-additives-for-dummies version) and we are hoping that the results show that he is positive for the lupus anticoagulant. the reason for this is that there is a possibility for the body to correct itself on its own and for his levels to go back up into the moderate (or maybe back up into the mild) range. it wouldn't be a definite, but a maybe. and that's always better than a no. if he is found to have been exposed to the mono virus, then it is a definite no-way-in-hell can his levels ever go back up to where they once were.

and you know, i feel so many times like i have a handle on the tension of this disease. and then we go looking at stuff like this and i realise that i don't. even though this isn't a life or death kind of situation, it delivers stress through my body just the same. and often times that stress leads to anger, which i try so hard to fight because its sort of a wasted emotion in terms of this disease. at this stage in the game, anyway. plus, who do i even get mad at? myself? my child? some spirit that may or may not exist? nature? science?

i guess i try to steer my mind in a new direction. a direction that is more positive for everyone. and i think about the lovely people...people that i dont even know...that are working their asses off to figure out how to cure this shit. and i smile at the thought of them. and wish there was something i could do to help. and i can't wait for the day when e's disease is cured. because hopefully i will be alive and able to hug and kiss these people and let them know just how thankful i am.

but for now, i guess i'll just keep going about my life of remembering anti-seizure meds twice a day, making sure the helmet is always on, accessing ports, and packing up a bag full of medicine and needles that would make a day clinic look like an amusement park.

I did it!

well, i did it. i accessed e's port today. the nurse was here and asked if i still wanted to do it...i sort of hesitated and she said "yep, you can do it." so i got everything ready, and i didn't even screw up putting on my sterile gloves and keeping them sterile. and then i accessed him. it was such an amazing thing. i was scared and nervous that i was going to mess up something, or hurt him...but it went so well! he laid there and we sang songs as i infused him...and he didn't make a peep when he saw me with the needle. this is such a huge deal for us...it means that we can travel without worry now. it means that if something bad happens and he needs to be infused right away, i can do it myself. and, of course, it is just another step on this crazy hemophilia journey that i seem to have conquered.

when e was first diagnosed, i was so scared...and so, so sad. i honestly mourned my idealistic views of his life for a long time. hell, i still mourn from time to time. but its hurdles like this that make me realise we are creating a life for him that is full of capability and hope and independence. as a mom, i work so hard to acchieve that last one...independence. and i have done a hell of a job with noah, and have been scared that i would coddle evan too much to allow him to be like his big brother. but after i find that i can handle the tough parts of this disease, i realise that i can teach him to handle them too.

and, for your viewing pleasure, here are some more recent photos of him. he's growing too fast =(

giving daddy "mwah's"


lounging with daddy on the couch


snuggling with "wowah" and "so cozy" (his blanket)


acting silly wearing noah's army dress up goggles

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.