Dec 8, 2015

0, 4, 27, 28

well there are officially four colemans and our birthdays are officially all within 6 weeks of each other. yikes! levi emory decided he was too snuggly in my tummy to meet his grandparents and great grandparents that had traveled to meet him, so he made his debut one week past my due date and exactly one day after everyone left town! he has filled our last couple of months with little rays of happiness in the middle of some of our most weary times.

i started writing a blog post awhile ago - mostly about levi. i didn't think it would be possible for my heart to love another little boy so much. every parent says that, and they somehow make room, i know. i still wondered though, but our hearts have swelled to let in another little person. so in light of how much i love this new little one, who is 10 weeks old, and growing much too quickly on me already, most of this post is, once again going to be about isaac, and is going to be SO massively long, and i think i will still not even begin to tell you what the last couple of months have been like. i'm giving you the abridged version still! forgive me in advance please for any crazy spellings and things that just generally make no sense whatsoever. i'm sure there will be a few.

our story begins back in july. since then we have been having varying degrees of trouble with isaacs shunt (the line that drains the swelling from his brain down into his stomach cavity) - so after 6 or so trips to the doctors office. maybe more? and a trip to the ER, with irritation/infection and finally swelling and puss (sorry) along the line, and also isaac not being able to wear his hearing aid for two weeks because it was causing even more irritation, we finally decided that we just needed to do a surgery to remove the shunt. the hope was that once it was removed, isaac's body had MAYBE just maybe learned to adapt and he wouldn't even need it anymore, which would have been awesome. the surgery ended up happening quite quickly after a few middle of the night scares, so on daniels birthday we woke up bright and early and whisked our family off to the OR.

after a weeks hospital stay it was decided that isaac was shunt dependent. we learned this quite quickly because he would throw up and become irritable with the slightest pressure change. so by the end of that week a new shunt was placed, a bit unconventionally - on the other side of his head and with the valve in his chest to avoid potential irritation from things like little isaac hands plus his hearing aid band. we had a few hiccups right after surgery, but we finally pushed to go home. isaac was so sad and constantly asking us to leave and hadn't moved in nearly two weeks in his hospital bed, so home we went! he has always done better recovering at home, so we had hoped that being back and reuniting our family would perk him up again and be the extra boost he needed to heal quickly. only this time he didn't seem to make much positive progress. in fairness, he had also never been in the hospital for 11 days either, so we kept thinking that maybe it was just taking longer, and maybe he was feeling a bit depressed after a two surgery stay. so we tried extra hard to be peppy and give him things to look forward to each day, but without much progress.

after just a week, i woke up to give him a squeeze and he was absolutely burning up. he ended up having a fever of 104.7, so we got him to the pediatrician, who tried to refer us to our childrens hopstial - which was FULL (our month was full of little crazy stories like this one)! by this time isaacs fever has passed 105 and we were told we would need to sit in the lobby of the hospital until a room became available. i'm getting pretty good at working some magic, so that wasn't an okay answer. i made a few different calls and isaac had a room by the time daniel got him to the hospital. after a very long night and lots of testing, the uti we thought isaac had ended up being a kidney infection (sad news for someone with one kidney that only functions at 40% anyways) and THAT ended up being a blood infection. and a particularly nasty strain of one at that. anything to us that has to do with blood is automatically freaky, so this was scary news. but we had excellent doctors that week and we got an antibiotic that seemed to knock it out quickly and completely.

one more 5 day hospital stay under our belts, that ended with us putting isaac under anesthesia once again so we could put in a PICC line to administer his antibiotic intravenously. its not the bubblegum tasting kind. this stuff was pretty intense. we had literally run out of veins after a month of poking him and adding multiple IV lines, so the PICC - as much as i have been dreading the day, has been a lifesaver this past month! for two more weeks at home, every 8 hours i would start the hour long process of washing hands/putting on gloves/cleaning the PICC line/saline/administering antibiotics/wash hands again/put on new gloves/saline/heprin. (have i mentioned that im going to be a nurse someday?) we wrapped up the day before thanksgiving and that was seriously something to be thankful for!! after all that, we thought that surely now, isaac would be feeling better and begin to recover. but that would just be too easy, right?

pretty much since we got home from his shunt surgery, he would occasionally throw up. but he had so much going on, it made sense that his body would just be in shock. then after the blood infection it became a little more of a regular occurrence (every 3 or so days). so we talked to doctors and nurses and blamed the antibiotic. thats a lot for such a tiny body! we put him on a probiotic and started to actively work towards restoring his gut health. but things didn't get better, and even got worse. things on that front have continued to worsen even in spite of all of the doctors we've talked to and all the things we've tried. then this past sunday he woke up and seemed very sick. we made calls. we spent an entire afternoon trying to collect a urine sample to check for another UTI, and then we started off our monday by visiting the pediatrician to talk thru whats been happening. we all had theories, and we left with another antibiotic and a nebulizer that isaac calls 'the scary hippo'. it is shaped like a hippo to be more fun, but the extra loud vibrating noise and smoke coming out of the end seemed to convince isaac that it wasn't something he wanted to have anything to do with. so with our new ideas and a bit of hope, we left! and tried to talk isaac into thinking the hippo was okay...

which brings us to today. this morning after daniel went to work i got isaac up and he greeted me with vomit down my shirt (a very common occasion lately. we don't stress anymore). as today wore on he became more and more irritable and lethargic and complained nonstop about his forehead and eyes hurting. we watched christmas movies and snuggled in between all of the doctor calls to try and distract him, but one thing became very clear: after a month of documenting EVERYTHING. every bite he's taken. how long he sleeps. when he throws up. when hes happy. when hes sad. we finally are sure of whats going on...all this time in the back of our minds we've been thinking 'his shunt already isn't working' but we didn't know for sure. and his surgeons office was never concerned when we called because of how infrequently he was throwing up. we've exhausted every other non-surgical option and this is the only one left and he is starting to show other, very clear symptoms now, too: lethargic. irritable/irregular behavior. 'projectile vomiting'. pressure in his head/severe headaches. i realized too, that for all of my charting this past month, i never put two and two together to realize that almost 100% of the times he has thrown up has been when there has been a change in elevation (mostly us picking him up after he's been laying down, or when he wakes up in the morning). it seems simple thinking about it now. we had just so hoped that this wasn't the problem...

so this is where we stand. isaac has spent almost half of a year working towards getting his shunt working again, and its still not. he has quickly moved from being stable, to severe bone marrow failure. his platelets in particular are floating right around 20k and we are transfusing him to keep his hemoglobin in a safe range every couple of weeks. he has lost more than a pound and a half since his surgery 6 weeks ago (super significant for us). he has digressed in physical skills - he hasn't walked since the day we took him in for surgery. his speech has become sloppier. overall, the kid just needs a break. my heart just hurts so much for him. especially at the age he's at - old enough to know that things are scary and happening that he doesn't like, but not old enough to reason with him, or to explain why they're happening. or to tell us how he's feeling or what hurts.

we've thought a lot in the past several months about what the future will hold for isaac. and for us. we are all pretty traumatized. it's hard to share with other people what we're feeling and going through.
life right now is a low. we have moments of happiness, but overall, its a season of getting 4-5 hours of interrupted sleep. drinking coffee until it comes out our veins and going to bed feeling like things are just getting worse. and trying to love on a sweet newborn in the middle of it all. i'm still hopeful for a future for isaac. but if you haven't already figured it out - things aren't going well right now. his body isn't healthy. its easy to think sometimes that i can control things with him. if we feed him healthy enough or just see the right specialists that things will go better, or at least he'll be okay just a little longer. i like to know that things are under control, and things just aren't anymore. there is very little that i feel like i can contribute towards making isaacs body better. we will do what we can for every moment, but i am reminded more and more that his days aren't up to me really. each morning, i watch him looking sicker and i feel so aware of my limitations. isaac was always our little gift. if you don't believe in god and are reading this, it might sound ignorant, or just weird, but i am (more/mostly/most of the time) trusting god for what isaacs life will look like, however long or short. and i don't think i've really wanted to trust him in a long time, but i'm out of other options, and it feels like a good place to be.

if we don't have much longer, i won't let myself live with regrets about any of this. i am finally able to look in the mirror and say that i am a ridiculously incredible mother to the two little people i am called to parent and love. i am so far from perfect, but i do the best i can. there are other moms that would be more structured, or remember to change diapers more often, or could maybe survive on less caffeine, or keep stacks of dishes out of her sink - maybe i can work towards that in a different life season, but not right now.

so our very next step is an MRI first thing in the morning. a lot of what our month is going to look like will depend on how things go tomorrow. we have a very capable surgeon here, but isaac is extra complicated, and we really need doctors that will look at him holistically, especially as he gets sicker, so we are praying for definite direction. if we don't feel great about how aggressively or seriously they are treating this shunt situation, we will probably be packing up our bags for ohio before the end of the week - providing they are able to take us there. so i would imagine, and honestly hope the way things are right now that we have a shunt revision in our very near future. pray for us if you think of it. we need some clear direction this week!

Sep 17, 2015

{an update on the coleman clan, which is mostly isaac}

we are expecting the arrival of little levi coleman any day now, so i thought i would give one more 'just isaac' update before we become a legit family of four.

we've had an exceptionally full past six months with isaac and have added a few new steps to our daily routine, so i'll update as well as i can. every post anymore is titled 'another update'! my brain begins to lack creativity by the time i finish typing up everything. i always think i want to come up with a fun, witty little title, but who has time for that?!

we made another trip to cincinnati at the end of may, as i had mentioned in my last post, and traded isaacs ng feeding tube out for a more permanent g tube. that was a little more of a learning curve than i had expected - learning how to clean it and take care of it and how to check the balloon (if you don't have to know, don't even worry about it :p) and to change out the tube. its been four months now and we've just established what i think is the best nightly routine that takes the least amount of time. up until a few weeks ago we would wait until he fell asleep to get his feeding tube running, and if he was having an off night i could be waiting up at 1 in the morning for him to fall back asleep or fishing around under his covers for his tube extension trying not to wake him up, so what we have going now is SO much easier, i can't believe it took us this long to decide that the best idea would be to just get everything going at bedtime and let him be apart of the process.

the original thought was that i really didn't want him knowing that he would be fed even if he didn't eat during the day. i worried that he would start to rely on the g tube and start thinking eating was a hassle, but so far that hasn't been the case and i think he actually likes helping with that part of bedtime now. so g tube for the win! we were so reluctant to put it in all these years, but it is such a huge load off of my plate to not have to stress over how little he is eating every hour of the day!

along with the g tube, we also did the surgery that is hopefully helping with his kidney reflux - i say hopefully because we actually need to have a VCUG (x-ray study that checks the reflux) done to see if it was actually a successful surgery. we had a wonderful surgeon, but isaacs reflux was pretty intense, so we knew going into it that it might not work. we should know that in the next few months though - whenever we actually decide to get the study scheduled. we just like to pack in as many proceedures as possible if you haven't noticed, so we don't have any more days at the doctor than we have to. but that will happen relatively soon...

he also had a growth hormone study (finally!!! and successfully!!) done on that trip. we tried doing this same study about a year and a half ago, and ironically, after a few hours, were told that isaac was too small and they weren't able to get enough blood for it. i just have to laugh at that one. but this time it worked just BARELY. his vein blew at the very tail end of the 6 hour study and we had a mess of blood everywhere (sorry) but they were able to get JUST enough to confirm that isaac's body doesn't make enough growth hormone (no one was surprised, but we had to know for sure). by mid june we were able to start growth hormones with him. its a shot that we give him in his leg every night. it takes both daniel and i to give it - one person to hold isaacs leg still and one person to give the shot. every night we tell isaac it will make him big and strong. he is becoming braver about it all the time. the trickiest part is that you have to hold it in his leg for 5 seconds, so especially at first, that seemed almost impossible. we all had frayed bedtime nerves for awhile, and i have always been squeamish around needles, so its taken me up until about the past two weeks to get the nerve to be quick about it and not overthink things. we've had a few nights where i've had to ask friends to help me when daniel's been gone - and vice versa. i'm sorry friends! because, fun fact, your body (or a kids body) makes growth hormone when you sleep, so you want to follow that same natural rhythm with this. so it really has to, or should be, a bedtime thing. so there is a little glimpse at our crazy nights. between that and the g tube. we've been working on going on more daytime and brunch sort of dates as you can probably imagine ;)

a great piece of news is that in a few of my last updates, isaacs blood counts were dropping quite quickly and we thought he would need a bone marrow transplant this summer - we were bracing ourselves for july and had started looking for bone marrow donor matches. but as we continued to get blood draws, his platelets jumped back up - they've been up and down since, still in a downward trend, but for now we are in a safe zone. his hemoglobin (red blood /// carries oxygen thru your body) however started dropping pretty significantly once his platelets increased and at the end of june we had to take isaac in for a transfusion that boosted his hemoglobin.

the difference in isaac to daniel and i was actually pretty incredible. his skin had started looking a tinge yellow and jaundice before that point and immediately afterwards his cheeks flushed and his skin turned warm and rosy and his energy levels popped back up and made his moods more consistent - i think he had just been crashing energy-wise before that and would just become incredibly irritable when his body was tired (just like a toddler anyways, right?) so it was really great to see how much it helped him! the bummer is that you really don't want to have to transfuse a kid that needs a bone marrow transplant a lot. theres not a specific number of times that would be considered okay, but the more you do, the more likely that the donated blood will interfere with a transplant. basically less is more, but obviously, he needed it and we aren't going to deny him blood! (kind of funny, because come transfusion day the blood bank actually did...long story, but it eventually all worked out!)

that transfusion lasted about two and a half months and he just needed another this past week - it ends up being an all day affair so i'm glad we were able to get it done before levi got here and it was also just in time for him to start preschool with an extra energy boost.

the very last big thing is that with a little brother on the way and his hemoglobin dropping - we decided to put isaac on androgens. its a route that we didn't think we would have taken two years ago, or really even six months ago, but with a baby on the way, plus isaac still being so small (even though he has gained 2 pounds and has grown an inch and a half since june between his g tube and growth hormones. crazy progress for us!) and also just as daniel and i think about the transplant process with him where he is developmentally and being able to communicate feelings and hurts and understanding what would be happening to him, we're just not ready to let him go into bone marrow failure without a fight right now. there are pros and cons to everything, but a lot of studies with androgens and fanconi anemia have been really positive and we were able to get a lot more info over the summer at camp that made us decide it was an option we wanted to pursue. they only have about a 50/50 shot at working, but if it does, it should boost his hemoglobin, which would mean hopefully no transfusions for awhile, or much fewer and farther between, and it could potentially help stabilize his kidneys and just keep him overall a bit healthier for an undetermined amount of time. it takes about 3 months before we will know if they are even going to work for him, which would put us at about mid december. if they do work, it could buy us six months of some stability, or a year, or 3 years. everyones body responds so differently theres just really no way of knowing. but we are used to living in limbo at this point, so we'll take it month by month and see what happens!

even as things have become a lot more complicated for isaac medically, i feel like we're in a really sweet place with him. he is as feisty and stubborn as ever and sometimes hurts my feelings and makes me cry (truth) but he is also walking and talking and exploding with moments of sweetness and hugs and kisses that make up for it all. as he gets older, we've realized that isaacs disease is becoming, to daniel and i, less about what it does to us, although lets be real, it adds a whole lot of crazy, and more about how we just hate this for him. he's understanding more and as everything in you as a parent wants to protect your kid, there are just so many things we can't protect him from. so we keep trying to make all of the in betweens special and filled with a little extra fun when we have the energy. i'm so thankful god has given us nearly 4 years with him so far. i also think another little person in the house is going to be a really good thing for all three of us :)

hopefully i'll not let another 6 months pass before i at least post a family photo!

{photos above: the bottom picture is how isaac will now pose every time i say i want to take a picture for him. arm up against something + legs crossed. he is olan mills picture perfect}

May 11, 2015

{happy 3 1/2 years little bean}

i feel like i'm forever saying 'thank you' all for the prayers and your sweet words we've received the past few months! we are so blessed by all of the support from friends and strangers alike. and are always humbled to hear how isaac has touched people. i just wanted to give another update. we don't have anything really big to report on the medical end of things - and that's a good thing in my mind :)

this past month we celebrated isaacs half birthday on april 3rd. we've always done half birthdays. a year just seems to long to go without making a big hoorah of things. this year was a bit more low key for us. just daniel, me and isaac - we made sure the day was filled with a lot of his favorite things - kite flying, (he actually just really likes the idea of flying kites more than actually doing it. mary poppins has romanticized the idea to him, i'm pretty sure...) airplane watching, picnicing at the park and lots of family time and snuggles.

overall things are going really well with his NG tube (the tube in his nose. or tube that WAS in his nose. he pulled it out this past weekend somehow). we had a lot of really long, partly sleepless nights the first week and a half or so just trying to get a good system worked out, but i think we are finally into a sort of rhythm. the hope was that he would tolerate the extra formula at night first off, and also that he would gain weight. the first has proven to be true, and he IS gaining the tiniest bit of weight - just very slowly. i think we've upped him about 6 oz in a month and a half? it changes a bit depending on the day, but for isaac, thats actually pretty significant. every extra ounce represents countless missed hours of sleep from mom and dad. we don't take it for granted :)

we have a big summer celebration (doctor week) this week. i think after that we'll know a lot more and have a better course of direction for the year. the hopes with this coming trip are: positive, or at least hopeful results from isaacs bone marrow biopsy, successful surgeries (two of them, one to replace his NG tube with a more permanent G tube in his tummy) and the second to hopefully help with all of the reflux going into his kidneys. then hopefully by the end of the week we'll have enough answers from all of our appointments that we will be able to plan, at least a little of what this year will look like for us - and also whether or not we'll be looking at a bone marrow transplant in the next few months.

oh. one more big family announcement. isaac is not only going to be a big brother, but i'm already half way thru pregnancy number two!! our house is going to be full of boys! and so far he is looking healthy. we can't know anything for sure until after he's born, but so far we're really encouraged and excited. i'm pretty sure we have a name, but we're going to try to keep it a surprise this time around.

so LOTS going on. i'll post a better update sometime next week.

much love from the coleman clan!

Mar 28, 2015

{cincinnati & a change of plans}

one of the things i wanted to do more of this year was updating my blog again. its been on my list of things to sit down and do. more little, fun updates. well this one isn't that. daniel and i actually tag teamed writing this one, so if there are bits that don't sound quite like me - its probably him! he did most of the actual medical update pieces. he's better at simplifying those things. 

this past week we drove up to cincinnati - they have a really incredible childrens hospital and we've started transferring some of isaacs care there this past december. the original plan was to knock out a bunch of annual testing at once to cut down on some of the frequent specialist follow-ups we have at home. so while that did happen, we also just about doubled our expected time in the hospital as we kept finding out things that were unexpected. and just as a disclaimer, each of the doctors we met with were absolutely fantastic. they took their time with us and were kind and concerned and they all worked together to help us decide what steps we need to take for isaac, which meant the world to us. so all that said. all of the x-rays, mri's, blood draws and talks we had this week led us to some of the following, sort of in order of when we learned them:

  • Isaac's pituitary gland is irregular. This was no surprise for us, as this gland is responsible for growth and Isaac is off-the-charts small for his age (even for FA). But an MRI showed amorphous tissue formations where the gland should be. Testing is underway to see just how dysfunctional it is so we'll know how much to counteract it with medicine/treatment.
  • Isaac needs to get more calories in him. Even though his small size may be largely due to growth hormone deficiency, he still needs more nutrition for sustained growth. And with all of the eating issues inherent to FA, it'd be almost impossible to get him to eat enough orally. Sooooo, we're going down the path of a feeding tube. He's got a temporary NG-tube going into his nose now, which will be testing his body's absorption of supplemental feeding over the next few weeks. Once that's proven, he'll likely have a G-tube surgically installed in his tummy. As much as we'd hoped to NOT have to go this route, I think it's going to relieve pressure on Isaac's eating and make his nutrition more consistent and trackable.  
  • Isaac's ability to empty his bladder isn't great. Instead of pushing urine OUT of his body, some urine is taking the path of least resistance & going back UP to the kidney (called reflux). May not sound like a huge deal, but...
  • Isaac's kidneys are in worse shape than we thought. He was born with 2 fused kidneys on the left side & they're only functioning at about 40% right now between both of them. The reflux that's occurring is dangerous because it can bring bacteria up to the kidneys and damage them with infection. Kidney preservation is key to a successful bone marrow transplant, so we're considering surgery options that can help reduce the reflux and protect the kidneys. But, surgery would be risky right now because...
  • Isaac's blood counts are not looking good. His platelets have dropped significantly in just the last couple months, and his other blood counts are trending downward as well. Our FA team is very concerned and want to monitor him closely over the next couple months to determine if a bone marrow transplant will be necessary sooner than later. We can never exactly predict timing with this, but the time frame we are bracing for at this point is any time in the next month to a year. Because of those concerns...
  • We conducted HLA-typing tests & are currently searching the registry for a genetic match for Isaac. Bone marrow transplants have a much higher chance of success when the donor has very similar genetics to the recipient. We did a preliminary search in Minnesota in 2013 that didn't show any GREAT matches, so we're hoping for some better options this time. We should know more in the next day or two!
so thats some of the nitty gritty. i don't know that we've ever talked on here - for real, about what a bone marrow transplant means for our family. and its all still so fresh, i'm not really feeling like i can give a statement just yet on how we're handling things or what the next few months are going to look like, because it changes all the time. the night we got his results from his blood test we cried ourselves to sleep and the next day it was back to business mode, and trying to keep isaac from being too overwhelmed by all of the tests we had to do. so more on that later. the last year and a half though, have been incredibly peaceful, so i'm grateful we had so much time without having to think about a lot of this. and in the midst of all of the crazy - we still managed to make the time outside of the hospital fun for isaac. we watched movies and saw the aquarium and the zoo and visited cousins. so with that said...more to come soon.