Tuesday, May 29, 2012

Reaccess

Keegan's port is accessed seven days a week for his daily IV medications and TPN.  On Sundays, we take the needle out after his morning meds, and he gets a break until bedtime.  We had a wonderful day swimming yesterday while he was deaccessed.  Keegan and Audrey swam like little fish.  After bathtime though, it was time to reaccess Keegan's port with a new needle.

I usually get the needle in on the first try.  Gray and my dad help Keegan hold still while I do a sterile dressing change.  Usually it is very quick, and Audrey sits in the corner of Keegan's room playing.  She's pretty used to the whole routine, as is Keegan, and it doesn't bother either of them too much.

Last night, I didn't get the port the first time.  I had to change gloves and hold pressure over his port for a bit before I could try again.  That really upset Keegan, which in turn really upset Audrey.  She has gotten too old and too smart to watch Keegan go through these trials week after week.  She loves him too much to see him upset or in pain.  

I don't know how much longer I can keep this up.  Both my beautiful children are so brave and strong, but it is wearing on them.  That is something that I can't stand for very long.  There is a limit to what this family can endure, and I fear we are approaching it quickly.  

Thank God these two love each other so because He knows how much I love them.



Sunday, May 27, 2012

Still hanging on

Just a quick update to say Keegan is still hanging on at home and still TPN-free for the moment.  His labs this week continued to show a steady decline.  However, the medical team is in agreement with us that there is nothing to do now but wait.  Even though we know what is coming, we don't know exactly when the other shoe will drop.  That is maddening, surely, but wasting energy worrying about it does no one any good.  Instead, we are trying to immerse ourselves in his therapies and appointments and fill our one free morning each week with something fun.  

By the end of next week, we may very well be in range of a blood transfusion and back on TPN.  Any other child (or adult for that matter) would have been transfused a long time ago, but our thresholds for transplant patients are lower, as each blood transfusion introduces new antibodies that could make later organ or tissue matches more difficult.

Today marks two weeks outpatient and four weeks since his last fever.  That makes us extremely anxious that the next one is around the corner.  We just have to wait for Keegan's body to show us what's next.  He has another rheumatology appointment on the 8th, where we are hoping to get more information from his last genetic work-up.  If he flares before then, we will just continue to pray that we can treat his flares as successfully as we have before.

We are hoping for another "normal" week for Keegan and a quiet start to our summer.  Thank you as always for continuing to keep Keegan in prayer.  Every week we are blessed with a "quiet" week is a blessing, and we are so very grateful.

Monday, May 21, 2012

Farm Day

Saturday we were blessed with another fun day courtesy of Texas Scottish Rite Hospital, where Keegan sees his rheumatologist and developmental pediatrician.  It was the hospital's annual Farm and Ranch Day!  We met up with my friend and Audrey's godmother, Marci, and her son, Noah, to see lots of animals, ride tractors and fire trucks, and play on the playground.  Another day of great memories with the kids!

Petting the bunnies

Freshly hatched chicks

Guns up, Noah!

"Umm, Bubby?  Are you sure you know how to drive this thing?"

BIG fire truck, little Noah

Daddy and Audrey

Petting one of the Sheriff's Departments horses, Sleepy

Mooing at a cow in the milking demonstration

Little ladybugs

Peek-a-boo!

I have a picture of Keegan on this very slide from almost exactly a year ago at the annual transplant picnic.  What a year it's been.

"Audee" and "Nono" - already good friends.

This picture of Marci and me is courtesy of Keegan and his new fascination with my camera.
He's pretty good!  
I'll have to do a post soon of his pictures.

Can't quite see it, but Noah had a football painted on his face.
Audrey had a ladybug on one cheek and a sparkly heart on the other.
Keegan let the girl do one stroke on his face before demanding we wash it!

Friday, May 18, 2012

Nice week

Here we are breathing down the door of being home almost a week, and I have to say I'm relieved...surprised, but relieved.  Keegan had labs drawn Monday and Thursday.  While they could have been better, they could have absolutely been worse, and he has managed to stay off TPN for the entire week!  From the trend in the labs and how Keegan's acting and looking, I don't know how much longer he will be pump-free, but we are certainly enjoying it right now.  He needed some time to even out and see what he could do.  It certainly has been nice for all of us though to have a break this week, but we are ending it with fingers crossed and prayers said that the trend in his labs is not indicative of a looming flare or need for a blood transfusion.

A "break" unfortunately doesn't mean we haven't had the usual chocker-block full schedule of therapies and clinic appointments.  Still, we managed to have a nice week overall and have some fun at the park on our one free morning.  Sure love these precious faces smiling and enjoying themselves.















Saturday, May 12, 2012

Home

Keegan was discharged home today. I think it's safe to say we are all uneasy about how long it will last, but that is surely no reason to stay in the hospital. For tonight, we are setting our short-term goals at longer than 2 weeks! Labs Monday and more as we know it. Thanks again.


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Thursday, May 10, 2012

Shooting for Saturday

Keegan continues to be his usual enigma-wrapped-in-a-riddle self.  There was an error in his TPN last night, so after much discussion (including with the transplant cardiologist on service directly calling our room to go over his decision...yeah!!), we did not run anything except his antibiotics overnight.  We held some of his diuretics today to see how he would do.  Low and behold, you stop the diuretics, and he blows up like a little balloon.  Fluid status is and has always been a problem with a solitary kidney.  It's so hard to find his "happy place."  His kidney lab values did get a little better today, so we are more optimistic that we were not dealing with him simply being on the verge antibiotic toxicity.  Big relief.

So tonight will be the last dose of antibiotics.  The plan then is to see how he handles the first 24 hours off them, play with his fluid status a bit as we are able, and watch his labs closely for another looming flare.  If all is calm tomorrow, we are all banking on a Saturday discharge mid-day after all his labs and vitals have come in.  Then he will have the weekend off his TPN and get some trough-level labs on Monday morning.  This plan errs on the side of caution and experience and respects the fact that it's more emotionally harmful to leave and come back right away.  Very reassuring, to say the least.

We also had our meeting with Keegan's rheumatologist, immunologist, and transplant cardiologists today. I really struggle to put all that information into words, yet alone out on this blog.  The ultimate plan for right now is to stay the course on his regular dose of steroids and anakinra.  His chromosomal microarray labs should be back in the next three weeks.  Those will hopefully tell us if we are on the right track with the IL-1 blockers, or if we should switch gears.  The caveat being that the microarray could also tell us nothing at all.  However, if they indicate that the next "big drug" we have been discussing is really the next best option, we will have a lot of work to do to get Keegan ready for that transition (more to be explained later, if and when that choice is made).  This line infection has put us all on edge because even though the cultures may be negative, we know from past experience that the bacteria can "hide out" on his line and reappear later.  If it's going to do that, we would like to see it happen in the next few weeks while we wait on the results of the microarray.  Then we will have to discuss the need to put a new, clean port in before starting drugs that put him at a greater risk of sepsis.

Now, if the microarray doesn't give us as much guidance as we hope, there will be another set of genetic immunodeficiences that we will be running to look for answers.  The kicker is that if he has one of these combined immunodeficiencies, the next "big drug" we are considering would be the absolute wrong thing to try.  All of this assumes that we can continue to control flares during the waiting with steroid pulses.

(Are you still with me?)

The general idea we got from the rheumatologist and immunologist is that it seems most likely Keegan has some sort of underlying native immune disorder that he would have had whether he was transplanted or not.  The fact that we are following protocols for solid organ transplant patients and he has not rejected his heart means that this dysfunction is coming from a different part of his immune system altogether.  Additionally, the proof that his GI problems are caused by an autoimmune response supports the theory that it is something he has, rather than something we induced.  Whether or not science and technology has a way to find out where that problem is and what to do about it is another story entirely.

I'm sure that none of that made sense to anyone other than me.  In fact, maybe it doesn't even make that much sense to me!  BUT, the general gist for now is that we stay the course and see what he does while we wait on these last bits of information.  This was the perfect time to get these heads together though because we needed to know (1) everyone was on the same page and (2) what is being done to save time and protect Keegan while we wait.

No matter what happens, this is shaping up to be a long, hard summer for our Bug.  Tonight, we are trying to keep our sights on the short-term goal of getting him home on Saturday.  I know we say it all the time, but thank you once again for every prayer and well-wish sent Keegan's way.  We certainly could not do it without our faith and without the encouragement of our family and friends.  Thank you!

Wednesday, May 9, 2012

Brewing?

It's been an interesting couple of days around here.  My hives continued to worsen and spread to my face, so now Keegan's not the only one on steroids.  They do seem to be less angry today though, so hopefully the meds will nip it in the bud quickly.

Keegan has us all scratching our heads a little.  He has been running a low-grade temperature in the mid-99s every afternoon for the last three days.  Very, very unlike his usual mid-96s.  We also had to put him back on heart monitors because his heart rate has been climbing slowly and steadily.  He has been extremely pale and less energetic than usual.  His labs, however, are rather stable, except his kidney function.  The antibiotics he is on can be very harmful to the kidneys.  He was also peeing and drinking water like nobody's business the last few todays.  Today though both have taken drastic declines.  Everyone seems to feel that he would have tanked by now if he was going to do it.  You just never know with Keegan.

The round of antibiotics is over tomorrow around noon, but we will stay inpatient at least through Friday afternoon to see how he does the first 24 hours off them.  Tomorrow will also finally be our big team meeting with all the doctors at 3pm.  Discharging after that meeting seems more reasonable too, in case our care plan changes due to our decisions then.  We are thankful the transplant cardiologist today is being so cautious with these last few days.  There are no guarantees though, and if Keegan sticks to this bimonthly flare pattern, he is definitely due for another one.

So for right now, we are watching all his signs - labs, vitals, output, and activity level - probably just a little too closely.  It's so hard not to though.  We're praying that if he is brewing something that it declares itself here quickly.  Keegan's throwing us enough curve balls to feel pretty anxious at the plate.  Only time will tell, and we are trying not to get too worried.

We would really appreciate some extra prayers for Gray, me, and the entire medical team tomorrow.  We ask for clarity, wisdom, and guidance in making our decisions.  Also, we pray that everyone in attendance can act and speak in Keegan's best interest and with a true sense of collaboration and cooperation.  Thanks so much.  We will keep you updated!

Bust a move, Audrey!

Hide-n-seek in the bathroom of all places.

Happy birthday, Mamie!

Sunday, May 6, 2012

Quiet Weekend

Keegan had a pretty quiet weekend in the hospital.  He had visits from Jenni and Mindy (his friend from the ICU, Tucker's mom) on Friday, one from Mamie, BD, Audrey, Alex, Chum, Poppy, and Pops on Saturday, and one from our friend Stephanie and baby Alexandra today.  (Kicking myself repeatedly for not getting any pictures during those visits!!)  He's slightly popular these days.  ;)

We did our best to keep him busy when he felt well and maintain a pretty normal schedule.  In between, there was a lot of medications, TPN, IV drips, lab draws, and vitals.  Keegan seems to be feeling pretty good most of the day, but he certainly has his moments where he slows down.  It sure makes Gray and I nervous that he's not showing us everything that's going on in his little body.  Only time will tell on that one.  I also broke out in another lovely case of stress-related hives.  Just to make our weekend a bit more interesting, I suppose.  Sigh.  We are still hoping that Keegan will be discharged late Wednesday afternoon if all continues to go according to plan.


Here are a few pictures from the last few days to make up for the brevity of this post.

Keegan has played in this little house in the playroom since he could sit up on his own.
It makes me smile that he is still so attached to it.
Here he says he is "building a birdie house" (from a Mickey Mouse Clubhouse episode).

Legos during a not-feeling-so-hot time.

In addition to Lego and marble towers, we've been blowing lots of bubbles and playing lots of Mr. Potato Head.

This was a highlight of today.
Daddy brought Keegan's kite up to the roof of the parking garage.

Sometimes you just gotta get a little creative when you're stuck inpatient!

Thursday, May 3, 2012

Waiting, waiting

I'm being a bad blogger, I know.  I apologize to all of Keegan's faithful followers and to my future self who looks to this blog to remember medical details that have since fled my mind.  I just don't have the energy in me to hash it all out in writing at the moment, but I will do my best to cover the major points.  Please forgive me in the meantime.  The beginning of the week was quite tense, but we find ourselves just waiting and biding time on just about everything right now.

It seemed earlier this week that, in a not so surprising manner, the different specialists involved in Keegan's care were not not communicating well with each other or with me and Gray.  The whole right hand-left hand communication issues are exponentially more exasperating when they involve your child's medical care than in just about any other possible situation.  There wasn't as much disagreement as there was simply assuming what other doctors would say or do, instead of all getting together to discuss a single plan.  Additionally, the head of rheumatology has been tasked with making the decisions on which drug to start and when.  However, she is not on service at Children's and hasn't been in town for awhile.  After months of saying we need to make these changes and seeing his disease process escalate in the face of an infection, we are quickly losing patience with not having a plan or at least a next step settled.  It isn't as urgent as we would like it to be anyway because we can't do anything at all until we are finished with the entire course of treatment for this infection, which won't end until next week.  That's where the rest of the tension arose over the last few days.

Again in a not surprising in the least manner, the transplant team decided (without our input and in fact quite against our stated wishes) that we could handle the 24-hr a day of IV medications, nutrition, and central line lock therapy from home because Keegan was no longer symptomatic of the infection.  I was trying to bite my tongue and allow the logistics to speak for themselves, all the while fuming inside that our needs, comfort, and wants were being flat out dismissed.  Ultimately, that's what happened.  Some of the protocol for treatment is not available for use outside the hospital setting, and the level of antibiotics in Keegan's blood has been swinging dramatically and in a very unstable manner each day.  Both of which will necessitate that we stay inpatient until next Wednesday at the earliest.  I could write an entire book on this issue.  When it is all said and done, this is where he needs to be now - no matter how much we wish he didn't.  I simply wish that this fiasco had been the "a-ha" moment that it could have been for this team. Instead, I will likely end up sitting on my frustrations until the next issue simply because the proper outcome occurred, not because of the proper motivation for reaching it.

So that leaves us once again with the waiting.  Keegan is feeling much better and appears outwardly extremely stable despite it all.  The steroid pulse is over, so we are just dealing with the lovely readjustment phase to his baseline dose now.  Tuesday was pile of goo/emotional train-wreck day.  Yesterday was swinging between giddy goofball and energizer bunny, and today was bouncing-off-the-walls crazy.  Ahh, the stages of Keegan on a steroid pulse.  Who knows what tomorrow will bring, but for now, we will be stuck riding them out inpatient.  When he feels well, it is a challenge to keep him entertained in the hospital, but we are doing our best.  While we do that, we just have to wait out his blood cultures, the remaining 6 days of antibiotics, and attempts to rally the troops to put an actual treatment plan for his immunodeficiency.  Just a few things on our plate.

There is no way we could possibly say thank you enough for all the prayers and support sent Keegan's way.  Words simply fail us when we try to explain how much each of you mean to us on this journey.  Thank you so very much.

Audrey spent a good part of the day entertaining us yesterday.
She's our "spirited" child, according to her teachers.

This bit of sweet adorableness spirited? No! ;)

Calling out for the "fishies" in the hospital garden.

Our little mignon - starting to feel a little better each day.

Tuesday, May 1, 2012

Silent scream

I managed to keep my cool today, but I have been silently screaming all day.

I'm mad.

Mad at many things both controllable and not.

The cultures all grew out our old friend from a few years ago, staph epi.  So far, yesterday's culture is still negative.  We will run IV antibiotics for 10 days from the first negative culture.  I promise to explain more when I can calm down.  Honestly, we are getting mixed information from transplant, infectious disease, and haven't seen rheumatology at all since we've been here.

The big question is whether or not we will have to pull his port and put a clean one in.  Usually, it's a three-strike rule, but with the bigger drugs we are discussing, the risk of sepsis from a looming bacteria may be too great.  These are all things we need to decide but can't get everyone together to talk about yet. Despite the fact that we are inpatient.

Mad.

Deep breath.


Thankfully, Keegan is very stable through it.  We managed to keep him from having a full MAS flare in response to the infection, and the last dose of pulse steroids are tomorrow.  What a huge blessing that the steroids were enough to calm without making the infection worse!

Not feeling too hot, but he managed to get down to the garden for a bit today.

I sure hope I have more information to share tomorrow, as well as the presence of mind and energy to share it.  We are beyond grateful for and humbled by every prayer for Keegan's safety and recovery.  Keep them coming....they work!  Thank you so much.