Tuesday, May 31, 2011

Tuesday

There was an all-out conference in our hospital room this morning with at least 10 members of the transplant team and other doctors all meeting with us at once (which highly agitated an already wired Keegan, who promptly fell asleep the minute they were gone...at 11:30am in the morning).  Lots of stuff was discussed - planning, theories, treatments, etc.  I will try to go into more detail tomorrow as a little more of it gets set into place.  It looks like Keegan will go to the cath lab on Friday and hopefully to surgery for a new central line next Monday, which is Audrey's birthday.  Then, we would theoretically be able to start the steroid pulse, monitor him for a few days, and be home for her birthday party.  That is still our goal, and I think the team is on board with trying to make it happen.

Other than a very early nap, Keegan seemed to do pretty well today.  They took him off of isolation.  He went to the playroom for about 20-30 minutes in the early afternoon, which just about wiped him completely out for the rest of the day.  You could tell he wanted to play but was struggling.  His heartrate was in the high 160s/170 while he was walking around.  Whew!  Maybe a little much for the first day out of bed, but he it was worth it to see him have some fun.


No improvement on the GI side, but we started his very, very minimal tube feeds up again.  He has been afebrile for 48 hours off the IV tylenol and 24 hours off the antibiotics.  We are praying he stays that way, too.  No improvement at all on the bone marrow side, a little worse in fact.  If it doesn't improve drastically over the next two days, Keegan will get another blood transfusion before the cath.  We will have a consult with the wound care team tomorrow because one of his incisions in his chest does not look like it's healing as well as would be expected (perhaps from his bone marrow/platelet issues).  They have ordered it to be treated with a betadine bath twice a day for now, which is oh-so-fun.  He still freaks if you so much as lift his shirt, and every time we have tried to take his blood pressure on that arm today, he has had an absolute cow.  It has me worried.  Praying it's just sensitive for now....

I will update more about "The Plan" tomorrow.  We were very, very impressed to see so many people gathering to work diligently for answers for our Bug today.  Even if there are a lot of questions left, I am confident our team has not stopped looking for the answers.  Such a huge weight off our shoulders.

Thank you again for your prayers.  I can see that God hears them and answers them when I watch my little fighter do something like go to the playroom for a bit...so huge for him but otherwise normal for another kid his age.  He is our miracle, and we are continually blessed by your love for him.  Thanks again.

Monday, May 30, 2011

Recap

Keegan had a good, quiet day today with a short energy spurt in the morning, a nice nap, and about as much energy as one can have without leaving a hospital bed this afternoon.  The energy spurts go as quickly as they come, so we know he's not feeling all that great.  Nor has he asked to get out of bed yet.  I'm sure it's coming soon, which will be hard if they don't take him off of isolation.

We removed all the dressings from Keegan's surgery and that pesky peripheral IV that had been placed in his shoulder.  All the sites have stopped bleeding and look good.  You wouldn't know that the spot in his ribcage where the port was removed was hurting him at all until you try to (1) lift his arm, (2) pick him up under his arms, or (3) try to lift his shirt in that general area.  Other than that, he is healing well from the surgery overall.  He looks like he's been in a street fight though from all the bruising.  The surgical team had a horrible time trying to get IV access on him.  Hence the IV in his shoulder and the need to get a central femoral line in his leg.  It looks like they tried every arm and foot and even around his collarbone for a line, and every one of those sites is bruised to no end because of some platelet issues he's been having.  His left wrist, in particular, is almost solid black, poor thing.

All of Keegan's cultures, from blood to stool to urine and even the port that was removed itself, have come back 100% negative for bacterial and viral infections.  We have held his IV tylenol for a little over 24 hours at this point with no fevers.  The markers in his bloodwork that indicated inflammation have come down dramatically.  We may never truly know the cause of the fevers, but at least they led us to the CT scan that found the bad port.  Keegan has been unable to keep his potassium levels up the last two days, so we have had to supplement that via IV quite a bit.  At one point, his levels actually dropped after a dose!  Low potassium levels can cause arrhythmias, so we watch it very closely.  

Keegan's bone marrow is still very suppressed, but he has not met any of the transfusion/support levels we set for him.  He will have two other surgeries coming up over the next two weeks though, so that may need to be addressed soon.  His GI symptoms have reached a new level of bad.  Really, really frustrating since no one really knows what to do with it anymore.  He spends alot of time curled in a ball from the stomach pain.  We've also had to go back to the maxed out level of blood pressure medication he was on before this weekend.

Dr. Guleserian, Keegan's cardiothoracic surgeon, wants his heart catheterization done before we put another central line in.  She thinks (and we firmly agree) that we need a good map of his heart to prevent this from happening again.  This whole thing just doesn't add up.  It's not unusual for the artery to be mistakenly accessed during a line placement, and the surgeon who put it in back in December is one of the best we have on staff here.  If the surgeon had put it in the artery at the very beginning, he should have known it right away (the bleeding would have been hard to stop; the blood is a completely different color in an artery than a vein at that place in the body, and the contrast used to check placement should have "backwashed" out by the arterial pressures).  However, if it had eroded from the vein to the artery, Dr. G should have seen what's called an A-V fistula when they flushed contrast into the vein after removing the bad port.  What we are concerned about now is some type of "architectural" anomaly that made it easier to miss the vein.  Dr. G said it appears the vein they targeted takes several 90-degree turns that it shouldn't.  Only a cath would show us that in clear detail.  

We will talk to our main cardiologist tomorrow about a plan, but we anticipate going to the cath lab mid- to late in the week.  Assuming we don't find anything that needs to be treated from that, he would go to surgery in the days afterward for a new central line (unclear if it will be another port or a broviac this time).  After the line is in, then he will start the steroid pulse as long as fevers haven't returned.  We will stay inpatient for 2-3 days to monitor his response to the steroids before going home.  It is fairly certain we will not be home for Audrey's actual birthday, but our greater goal is to be home for the little family party we were planning on the 11th.  The steroid pulse will last 4-6 weeks, followed by another colonoscopy and possible bone marrow biopsy.  If we see improvement in those scope results, we would make a trip to Boston for intestinal rehabilitation.  If we don't see improvement, we will have to regroup and may still make a trip for a second opinion at least.  

So, that's what's been going on here since the surgery.  We are breathing a sigh of relief and trying to recover from such a crazy weekend.  Spending time with this little Ladybug in Keegan's favorite spot of the hospital garden certainly helps with that!


Sunday, May 29, 2011

On the floor

Keegan made it to the recovery floor just after noon today. He is doing very well considering what he's been through. He had some good stretches of energy an was in a fairly good mood. I will update with more tomorrow, just wanted to put up a quick update.

Thank you again for your prayers. Have a safe holiday weekend.




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Saturday, May 28, 2011

Doing well

Just a quick note to say the surgery went well, much smoother than anyone was really expecting, I think.  Keegan is doing well too.  I am pretty confident that he will go back to the cardiac floor tomorrow afternoon after his femoral and arterial lines are removed.  The best news is that we don't think that he suffered any neurological complications so far from the line coming out.  He has had a few energy spurts of sitting up in bed.  He recognized all of our family members, asked for Curious George, and counted to 5, and colored for a bit.  I definitely think that he feels better than he has the last few days.



Cultures on the port will be pending for 48 hours, and we will have to wait and watch to see if the fevers come back.  Keegan is on scheduled IV tylenol right now, so unless it gets so high that he spikes through, we may just be masking them.  I don't have a lot more information than that right now.  The plan is still to tease out the remaining infectious avenues, treat what we may find, and then proceed with the steroid pulse.  He will need another central line placed sometime soon, but the team thinks we need to give his heart a rest from that right now.  His PICC is still in and working.  We have to keep his arm immobilized with a stiff "no-no" sleeve to keep it working, but it doesn't seem to bother him.

This was a very difficult day for us, but your prayers and reassuring encouragement lifted us up time and again.  We are forever grateful.  The Lord is faithful, and we felt the presence of His angels surrounding Keegan today.  This was a big hurdle to overcome, but unfortunately, it does not shorten our journey.  We still have a long one ahead, but we are thankful for your support through it all.  Thank you, thank you, thank you.

Out of surgery

Keegan is in the CVICU. Went very well. All were pleased. Will update again soon. We know it was your prayers that carried him through. Thank you so very much.


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10:35am

They are beginning removal of the line now. Had to go in through his ribcage, which was what we anticipated would happen. They placed two more central and arterial lines that we did not plan on. He is stable & not on bypass at this point. Next update should be in about one hour.


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In OR

Keegan has been in the OR since 8:30am. All surgeons should be there and starting diagnostics & planning by now. We will get updates from OR as they go along. First update should be within an hour once they decide with what method they will proceed.

Thank you for lifting us up in prayer today. Keegan was struggling last night. His labs indicate his body is tired & stressed. You can tell from this sweet face how he feels. Please pray for his safety, his surgeons, and that this is what he needs to get better. Thank you.










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Friday, May 27, 2011

Surgery postponed to tomorrow

You may have seen our updates to Twitter or Facebook that Keegan's surgery will be postponed until tomorrow morning at 8am.  His transplant surgeon, Dr. Kristine Guleserian, will be coordinating and leading the surgery to remove his port.  She has been planning, researching, and assembling her team all evening.  They are of the opinion at this time that it has been in the artery for some time, if not from the beginning, and that waiting 12 hours to fully prepare was more beneficial than rushing him to surgery tonight.

Dr. G has asked for the leading vascular surgeon at UT Southwestern, along with their chief interventional radiologist to assist her in this surgery.  Our chief of interventional cardiology (i.e. head of the cath lab), Dr. Zellers, will also be assisting.  Our favorite cardiac anesthesiologist, Dr. Turner, will be there, and the bypass team will be ready if needed.  This is not something that anyone can ever really remember seeing in a pediatric case, so they are trying to be as prepared with a plan as they can before getting to the OR in the morning.  There are about 5 different ways they could go in to try to get this catheter out.  On a scale of least invasive to most, the most likely way they will go in is #4 on the list.

Brother-sister bonding while waiting to hear about surgery

The surgeons believe that the port may have been placed incorrectly from the get-go back in December.  Keegan was very swollen coming off dialysis at that time, making placement a bit tricker.  However, other options could be that the vein was accidentally punctured during placement, or the catheter truly did erode through the vein into the artery.  They are prepared to repair any damage they detect after removing the port.

Our biggest risk and concern from this surgery is a stroke.  The catheter can develop a fibrin sheath, a clot of lipids, or a blood clot in it.  All of which can break off as the catheter is removed.  The team will use as many precautions as they can, but there is not a lot they can do to stop it if it's going to happen.  We won't know if any neurological complications have happened until they try to wake him up.  We believe this line has been throwing clots already because there were two areas of infarction in his spleen shown on the CT scan, along with the mini-stroke he had back at the end of March.

Trying to make Keegan laugh and feel better

We have no idea how long this surgery will take.  He will be in recovery afterward in the cardiovascular ICU for at least a few days.  We are still chasing every possible source of infection down (including culturing the port once it's out), but when this is all said and done, we still are planning to proceed with the steroid pulse.

That's all the actual information I have to share tonight.  My brain can hardly comprehend the technical stuff.  I have not let my heart start to process it yet.  I simply can't.  I have to be present physically, emotionally, and spiritually for Keegan.  My sheer ability to understand and come to grips will all of this has pretty much disappeared.  I don't know what else to do but soldier on.  We have one hell of a fighter on our hands and an awesome God who will never leave us.  Tonight, I have to place my hope and trust in the Lord, that He will raise us up and out of this depth.  That He will surround Keegan with his strongest angels to cover him with their mighty wings.


So many of you have offered your prayers and support - people we know and those we have never met.  Please keep them coming.  They may be all we have right now.  Thank you.  We will post tomorrow to the blog as the surgery progresses.

For He will command His angels concerning you 
to guard you in all of your ways.
They will lift you up in their hands,
so that you will not strike your foot against a stone.
Psalm 81:11-12

Surgery

Keegan will be going into emergency surgery this evening.  His CT scan showed that the catheter of his port has eroded from the vein it should be in into the subclavian artery.  We don't exactly know how this happened.  All we know is that it is very dangerous and must come out now.  This explains so much about the bleeding problems he has been having and could also be the source of his fevers.

Surgery is consulting with our transplant surgeon, the cardiology team, and all of Keegan's other doctors right now.  Once a solid plan is in place, he will go back for surgery.  This could all go somewhat easily, but there is a lot of potential for this to become a major surgery.  Because it is in an artery, it cannot just be pulled out like it should be if it were properly in a vein.  He will be at a very high risk of stroke during and immediately the removal.  If there is some kind of damage that has allowed this erosion to occur, that may need to be repaired.  There are too many questions and not enough answers right now.

Please, please, please pray for Keegan right now.  I'm not ashamed to ask for you to literally stop what you are doing and pray now.  Please spread the word and spread the prayers.  Thank you so very, very much.  We will update here as things progress tonight.  Thank you again.

Thursday, May 26, 2011

Already?

I had a gut feeling that we were gearing up for a long fight.  I guess I was hoping it wouldn't start until next week!  Keegan woke up with 102.9 degree fever yet again this morning.  This is the fourth episode of fever this month, continuing a cycle that started at the beginning of April.  The last few, he has been lethargic but in a pretty easy-going mood.  Not this time, he is moaning in his sleep, is very puffy, very lethargic, and rather irritable.  He is retaining almost an entire kilo of fluid since this past Sunday (2.2 lbs, which is roughly 1/6 of his body weight in 4 days).

Instead of taking him to the ER, the transplant team had us bring him to clinic.  There was a lot of discussion and a lot of waiting.  These episodic fevers are throwing a bit of a twist into our picture and making it even more difficult to start the steroid pulse he so desperately needs to help his GI system.  We resubmitted a urine culture, even though the one from Tuesday was negative.  We drew an initial set of labs from his PICC, and when things looked worse than Tuesday, Keegan was once again admitted to the hospital.  The fevers haven't stopped spiking yet, but they're not going above 102 right now.



Tomorrow, he will have a full body CT scan.  We are looking for any signs of infection that we have missed through cultures and a few other things.  We are also running stool cultures again.  I would not be surprised in the least if he tests positive for c-diff, since he has been on very strong antibiotics for almost a month now.  Antibiotics to treat an unknown and possibly nonexistent infection that have probably left his already fragile GI system at risk for a mean opportunistic infection.  C-diff is usually a hospital-acquired bug you pick up while on antibiotics because they tend to wipe out all the "good" bacteria in your gut.  If he is positive for c-diff, it will be because of us treating the fevers, not a cause of the fevers. It would definitely postpone Keegan's heart catheterization if it is positive.  The cath will require the doctors to get to his heart via the femoral artery in his leg; any kind of GI infection would be dangerous in that situation.

Keegan's doctors have also now consulted with the Infectious Disease (ID) doctors that specialize in immunocompromised patients.  They have ordered quite a few more lab tests.  There aren't a lot of things that cause intermittent, recurring fevers, but we are tracking down every zebra we have to at this point.  IF we cannot identify a source for these fevers by the beginning of next week, we will likely consider them to be an inflammation response and proceed as planned with the steroid pulse.  We need to be as confident as we absolutely can be that there is no infection before doing that because although steroids help fight inflammation, they will feed an infection and make it much worse.

Needless to say, the team has already warned us that this does not look like it will be a short inpatient stay.  We are starting it off completely exhausted and utterly exasperated with the way things have just seemingly declined so quickly with Keegan over the last few months.  We are praying and hoping beyond all we can put into words that the Lord will see fit to provide some clarity, guidance, and healing for our Bug.  We are ready to be home as a family again.  Especially in time for this precious girl's first birthday...



We have been so humbled today by the outpouring of prayers, encouragement, and support for our family.  You give us strength to keep up the fight.  Thank you so much.  More tomorrow as we learn it.

Difficult

The best word I can find to describe things right now.  Difficult.

Difficult to process.  Difficult to understand.  Difficult to explain.  Difficult to accept.

Without getting into too much detail right this moment, a lot has changed in our picture in the last few days.  Keegan's bloodwork for graft-vs-host disease came back negative.  That pretty much puts us back at square one with Keegan's GI problems.  Which couldn't come at a worse time because they are getting so bad.  Today was particularly difficult for Keegan from a GI standpoint.  I had started to say that the only thing that could get worse at this point would be the volume of output.  That was one of the main reasons we kept his g-tube feeds at such a low rate.  Well, the volume has been crazy lately for no apparent reason.  When will I learn to keep my mouth shut?  Unfortunately, we are now just guessing what to do next, but all signs seem to indicate a steroid pulse.  That was already our plan, so we will stay the course with that for now.

Keegan's bone marrow is still struggling across the board.  White count, hemoglobin, and platelets are all low.  His hematologist is working closely with our transplant team as we start the steroid pulse next week.  We are all rightfully concerned how his weak marrow will tolerate this pulse, but we are trying to be as proactive as possible in supporting him.  The doctors have raised his threshold for support and transfusion.  They have vowed to jump in as soon as we meet those levels.  We have now quadrupled his blood pressure medication, but it is still high.  I don't know what comes next on that end.  He is still bleeding into his central lines, too.

The bigger development this week (and by far, the hardest to come to terms with for us) was learning that Keegan has developed two types of donor-specific antibodies in his blood.  Antibodies are proteins your body forms in response to antigens, i.e. things it detects as being harmful or foreign.  You develop antibodies in response to vaccines, each time you get a cold or virus, etc.  Well, Keegan's heart is not his own.  His body has figured that out and formed antibodies against it.  These antibodies are in the beginning stages, so they are weak.  We know they are in his blood, and next week during his cath, we are hoping to find out that they have not attached themselves to the vessels of his heart yet.  Metaphorically speaking, his body has created a weak army to fight his heart, but we are unsure if this new army has been told exactly where the battlefield is.

It is probably easy to figure out from that description that these antibodies increase Keegan's risk of rejection and transplant coronary artery disease (TCAD) exponentially.  We were already planning to take him to the cath lab next week to check his ventricular pressure, but now, this cath has taken on the utmost importance.  We are praying and hoping and begging that these weak antibodies have not started to do their "job" yet.  Detecting these antibodies is of utmost importance in being vigilant to screen Keegan for rejection and TCAD, but we wouldn't and couldn't necessarily do much more to prevent them from getting stronger.  The first steps when TCAD is detected is to put you on rapamune (the immunosuppressant Keegan is already on) and increase steroids (which we were already planning to do next week anyway).  Right now, Keegan is scheduled to be admitted Wednesday for the cath, and depending on what we find and how he is doing, the steroid pulse could begin as early as Thursday.  He will stay inpatient for the beginning of it.  It shouldn't be too long of a stay, but Keegan's never been one to follow those estimates.

For now, we, as a family, are defeated.  If we weren't before, we certainly are now.  The wind has almost all but left our sails at this point.  I wish I could say we are ready for this battle.  Every bone in my body is telling me that this is just the beginning of not just a battle, but a war.  We will get up tomorrow and fight the same way we always have.  We will not give up.  I just wish that we felt ready to face the dawn.  I pray that Keegan is.  I pray that we all are.  This is the most difficult situation we have been in since his birth and transplant.  There are so, so many issues Keegan is dealing with.  We are honored to have a team of doctors here to help us put on our best armor.  And we are humbled that so many have joined us and stand behind us as we ready to face such a difficult tide once again.

We can only ask for your prayers and support at this time.  Thank you doesn't even begin to cover it.

I was pushed back and about to fall,
but the Lord helped me.
The Lord is my strength and my defense;
He has become my salvation.
Psalm 118:13-14

Sunday, May 22, 2011

Best laid plans

Keegan has rewritten this legendary poem..."the best laid plans of mice and men always go awry."  It doesn't even surprise me anymore.  Although, the disappointment remains.

Keegan's blood cultures are negative, and he has been fever free since Saturday morning.  No line infection.  Thank the good Lord.  However, a strain of bacteria was identified in a urine culture that was sent out, so now he is being treated for a urinary tract infection that he almost certainly does not have.  Try to follow me here because this is a touch complicated.

  • When Gray arrived at the ER with Keegan, they drew the obligatory blood cultures from his PICC line and bagged him for urine.  This is the standard protocol that we are familiar with.  The ER nurse did not clean him before putting the urine bag on.  It is not necessary for a standard urinalysis and didn't immediately set off any red flags in Gray's mind.  If he had been told or expected that a true urine culture was going to be sent to the lab in addition to the urinalysis (which is just a breakdown of the cells and proteins in your urine, not for identifying any bacteria), he would have insisted on a thorough cleaning and likely an in/out catheterization for a good, accurate sample.  That's just the way a culture should be done.
  • Keegan's urinalysis from the ER that morning was fairly normal and not indicative of an infection.  When the team walked in yesterday and said his urine culture was positive, we were all obviously very surprised and yet not in the least bit convinced that it was true.
  • It is almost certain that given the particular bacteria identified and the failure to follow protocol, this was a contaminated sample, and Keegan does NOT have a urinary tract infection.  However, the bacteria involved is very, very dangerous and not what causes your everyday UTI.  If by some chance it truly is in Keegan's urinary tract, it could invade his kidney and his bloodstream quickly if not treated.  
  • Even though we are 99% sure that it's not accurate, we are obligated to treat him for it because it is just too risky for us to be wrong.  We need to have a clean sample from him at least 24 hours before his heart catheterization, and we want to give him enough time off the antibiotics for his bone marrow to recover in time for the steroid pulse.  
SO...Keegan's heart cath and steroid pulse have been cancelled for this week.  We are planning on rescheduling his cath for the following Monday or Tuesday and then remaining inpatient for a few days to monitor him through the first phase of the steroid pulse to treat his graft-vs-host disease.  

I know this is the right thing to do.  That does not mean that I like it in the least bit.  We thoroughly questioned the transplant team this morning and debated with them the merits of waiting or proceeding per plan.  They are still of the opinion that Keegan's fevers are inflammation-related in response to the many disease processes he is battling right now.  Unfortunately, they do not think the fevers will stop spiking until we can get that under control.  I hate putting off this course of treatment even a minute longer than we are forced to.  Keegan is showing us in many ways that his body is struggling under the weight of it all.  His bone marrow, his blood pressure, his kidney function, his GI problems, and now recurrent fevers.  We have already had to double his blood pressure medication after just a few days.

If we wait, something will always crop up.  I fear that at some point we will let it get so far ahead of us that this hill will become a mountain.  He may be struggling now, but he is stable.  I am terrified of not being able to say that.  We are all very aware that Keegan's kidney is not going to hang on forever.  What if it gives up before we address his other issues?  The logical part of my brain obviously grasps that all that won't happen in one short week.  The mom part of my brain, however, wants to fix my son, wants to stop his pain, and wants to do it NOW.

For tonight, we are home.  For this week, we will proceed with Keegan's usual course of therapy visits, clinic appointments, labs, and dressing changes, with an extra visit for a repeat urine culture.  We are praying that we stay home through this week and that Keegan is as ready as he can be for his cath and treatment starting next week.  Beyond that, we are sincerely praying that Keegan will tolerate this plan and be home by Audrey's birthday.

We are running on empty.  We would certainly appreciate your prayers, encouragement, and support for our Bug at this difficult and nerve-wrecking time.  Thank you once again.  Truly.  Thank you.

Friday, May 20, 2011

Round 3

It appears we may be destined to spend every weekend in May in the hospital with Keegan.  This will be our third weekend, only one left to go.  Bleh.

Keegan woke up around 3am.  He wasn't fussy or upset, just restless and awake.  I left him for awhile, hoping he would go back to sleep on his own before my usual 4:30am pump/formula/diaper check.  When he was still up at 3:45am, I went to check on him and prayed I wouldn't find his PICC line messed up somehow.  I could feel his body radiating heat before my hand even reached him.  103.3 degrees.  Blood pressure and heart rate were sky high.  Dang it.  I quickly packed him up, and Gray rushed him to the Legacy ER while I waited for my parents to come sit with Audrey.

Cultures and labs were drawn.  Chest x-ray.  Urinalysis.  IV antibiotics started.  And then, of course, this...

Ambulance ride #12

Keegan is now inpatient downtown on the cardiac floor.  We are running the obligatory 48 hour course of antibiotics and waiting for cultures to be cleared.  His labwork is not indicative of a bacterial infection.  A virus is not super likely considering the fever just took a 5-day hibernation before spiking back up, nor is Audrey sick with viral-like symptoms.  The most likely situation is that it is inflammation-related from the rest of Keegan's issues.  He had a pretty rough day with a fever that just would not break until about 4pm.  He still obviously doesn't feel well, but he actually perked up a bit just a few moments ago to play in bed with some cars.


Truthfully, this is probably the best place for him to be right now.  Keegan's blood pressure spiked so high yesterday, that his team called in some medication last night for him to be given right away.  The blood pressure and continued bleeding from his central lines mean we are going to have to get a good read on the pressures in his heart and test for rejection.  So, assuming the cultures stay negative over the weekend, Keegan will go to the cath lab at 8:30am on Monday morning for a heart catheterization, angiogram, and heart biopsy.

We aren't sure what, if anything, we will find from the heart cath and biopsy.  It is very likely that we will start the steroid pulse we had been planning early next week, and Keegan would stay inpatient while we monitor his initial reaction to that.  There are a lot of "what if"s and "what's next" questions floating around right now, but honestly, we will just have to wait them out.  We did find out that the pathology lab here was able to use some preserved blood to run the test we needed to confirm the graft-vs-host disease and started working on that earlier this week.  We're not sure when that will be finished, but at least it's one item on the to-do list that can be crossed off.

All of this is to say that we don't exactly know much of anything right now, and we probably won't know much of anything over the weekend.  But we have a plan to start off the week, which is always a good way to start.

And just because I miss her and think she's the cutest doll baby that ever lived...

"I looooove cereal bars."  Sticky goodness.

(Sorry for all the iphone pics lately.  New computer, haven't figured it all out yet with my camera.)

Thank you once again for your prayers, love, and support.  We need you now more than ever.  This is uncharted water for us and for our transplant team.  We are beyond exhausted on so many levels.  But we are hopeful and praying that together, by placing our trust in our merciful Lord, we will bring healing to our Bug soon.

Come to me, all you who are weary and burdened, and I will give you rest.
Take my yoke upon you and learn from me, for I am gentle and humble of heart,
and you will find rest for your souls.
Matthew 11:28-29

Thursday, May 19, 2011

PICC and BP

A jumble of letters today.

Keegan successfully had a PICC line placed in his right arm on Tuesday.  So far, it is working and still in place, but it is also bleeding into the line when not in use.  It is not bleeding into the dressing though, so I guess that's an improvement.  As long as it doesn't clot off, I guess it's not too much of a problem.  His port site is definitely bruised and swollen.  I really hope this break will be what it needs to heal properly.

In other news, his blood pressure has been climbing and climbing for the last month.  As of yesterday, it is officially in the range it was the days before he went into complete renal failure at Thanksgiving.  To say that Gray and I are concerned is the understatement of the year.  Walking on a thin sheet of ice, not knowing if it will hold or shatter beneath your feet?  Sounds about right.  He is lethargic but still urinating.  We are waiting on word from the transplant team as to what we need to do from here.

We would greatly appreciate your prayers as we keep up this balancing act.  Thanks so much, friends.

Monday, May 16, 2011

PICC

Keegan will have a PICC line placed tomorrow morning.  This is a more temporary central line that will come out Keegan's arm.  It is placed via the interventional radiology team, not surgery.  It will require anesthesia however (I believe this will be the 35th time for him but not exactly sure).  Keegan does not have a good history with PICCs.  Neither of his previous PICC lines have lasted longer than a week, and that was before he could walk independently!  Needless to say, I'm not very confident that this will provide his port with the break that it obviously needs.

We accessed his port last Thursday night, and we had to replace the dressing due to bleeding yesterday.  This evening, it already looked like this.


Seriously?!  I have no other words.  Frustrated doesn't even begin to describe my feelings at this point.  We obviously need to try a PICC and give the site around his port some time to heal.  I just pray this is the answer we are seeking.  His transplant team  thought we should use the next two weeks to try to troubleshoot this line problem before we start the GVH treatment.  I just hope we can find an answer for him.  He is going to need central access for quite some time, so we have to keep trying.

Other than having a really bad day from a GI standpoint, Keegan seems to be feeling better from whatever virus he had.  We will check in at the hospital tomorrow morning at 9am and are hoping to be home by late afternoon.  After that, I'm praying we are home for the next 12 days before starting the steroid pulse.  Not much else to say tonight.  Thank you for prayer tomorrow.  We will let you know how it goes!

Saturday, May 14, 2011

Update & Graft-vs-Host Disease

I'm so very creative with my post titles, huh?

Keegan's had an ok day.  He struggled with his fever all night, but regularly scheduled tylenol has kept it down today.  We are out of the first 24-hour period with no growth on the blood cultures.  We should be able to go home tomorrow late afternoon if the blood cultures stay negative.  We would assume this was a viral infection at that point.  Although it has been suggested that it is theoretically possible that the fever could be part and parcel of this graft-vs-host reaction.  We'll have to just wait and see.

I promised an explanation of the graft-vs-host issue yesterday.  I'm at somewhat of a loss as to how to explain it though.  Start at the beginning I suppose.  Generally GVH is seen as a complication from a bone marrow transplant.  It is, by it's very definition, a condition where the graft (donor cells/organ) attacks the host (transplant recipient).  It can happen in solid organ transplants, but it is extremely rare in just heart transplants.  The intestinal biopsies showed all the classic signs of GVH, but it will take a very specific blood test to confirm this diagnosis.

The hospital's chief pathologist has confirmed that we have preserved blood samples from both Keegan and his donor from pre-transplant.  We will take a very large sample of blood from Keegan now to compare to both the preserved samples.  If we find specific cells called leukocytes from Johnston in Keegan's blood, it will be a definitive confirmation of GVH.  We believe that this same test was run on some tissue from a heart biopsy we did in Boston two years ago.  We were told then that they had detected a very large amount of "donor cells" in Keegan's heart.  Many more than they would have expected to see at 18 months post-transplant.  Our cardiologist there said they figured it meant he wasn't at much risk of rejection, but they didn't know what else it could mean.  Well, now we know.  The diagnosis is further supported by his skin and liver issues.  

The treatment for intestinal GVH calls for a steroid pulse over the course of 4-6 weeks with antivirals and antifungals to prevent infection during a very immune-suppressed period.  The combination of all this would depress almost anyone's bone marrow, and since Keegan's is already not in great shape, we agreed that the pulse would need to be started while inpatient for constant monitoring.  There is no guarantee that the steroid pulse will work.  GVH can be a persistent little you-know-what.  But that is where we will start.  "Stopping" the GVH does not mean that Keegan's GI problems will be fixed.  It is just the first step.  We will never stand a chance of rehabilitating him to the point of getting off TPN or even tube feeds unless we stop the process of him attacking himself.  When the initial steroid pulse is over, we will repeat his colonoscopy and also perform a bone marrow biopsy to see if it was successful in stopping the GVH.  If not, we try something else.  If so, we can then hopefully still head to Boston for treatment and rehabilitation.

We were planning on admitting Keegan this coming Monday to do the big blood draw and start the treatment.  The plan was to start treatment immediately, even though the bloodwork would take at least a week to perform.  Even if the GVH could not be confirmed, the steroid pulse is still our best shot at stopping the cell death process occurring in Keegan's intestinal tract.  All of this is now on hold for at least two weeks while we give Keegan sufficient time to recover from whatever he is fighting right now.

Nothing ever goes as planned with Keegan.  He never "read the book" on what is expected from normal heart transplant patients.  In true form, his port is bleeding from the site yet again this evening.  We have once again consulted with hem/onc.  They have advised that his port likely is need of some decent rest for a minimum of a week to stop this bleeding.  There is also a test we will probably want to run to rule out a reaction to the heparin we use to keep his line open.  We will run his TPN tonight and discuss it with our team in the morning.  If they agree with hem/onc, we may have to wait until Monday to secure a PICC line for his TPN.  I really hope there's an easier answer than that.

I sure miss my baby girl and am so tired of our family living like this.  I feel like I'm letting Audrey down by not giving her the security and familiarity of a family unit and normal schedule.  Every time we even remotely start to resume our little version of normal, we somehow end up right back here.  Keegan may be used to it, and Audrey is certainly being taken care of and treated like a little princess by my parents.


Still, I long for us to be a family again.  Having this upcoming treatment and pushing back a trip to Boston just compounds that stress because I know it will be a long-time coming before we are well and home together for any length of time.  I pray that she will understand it one day.  I pray more than anything that we finally will be able to find healing for Keegan from this long journey, so we can truly be a family again when it is all said and done.  I struggle daily to lay it at His feet.  This valley is not as deep as it could be, but when you're in it for so very long, it gets increasingly difficult to see the way out.  Sometimes I feel that if I keep trying to carry these burdens myself, it will somehow justify them.  I know that is foolish, but it doesn't make it any easier.  For tonight, I am thankful for His love that carries us through the valley... no matter how long it may be.

The Lord is my shepherd.  I lack nothing.
He makes me lie down in green pastures;
He leads me beside quiet waters,
He refreshes my soul.
He guides me along the right paths, for His name's sake.
Even though I walk through the darkest valley,
I shall fear no evil, for You are with me.
Your rod and staff, they comfort me.
Psalm 23: 1-4

Three days

A lot can happen in three days.  Keegan is back in the hospital for one thing.  Dang it.

The last two days he has been rather lethargic.  He was refusing to get out of bed in the mornings, spending most of the morning cuddled in the chair.  Last night, he was very restless and seemed uncomfortable.  By 6am, I could tell he felt kinda warm, and his respiratory rate was very high.  Since he finally had fallen asleep, I let him stay there until about 8:15am.  He had a temp of 101.9 by that time.  A quick set of vitals showed his heart rate and blood pressure were very high.  Keegan has to go to the hospital for IV antibiotics for any temperature over 100.5 degrees because he is (1) a transplant patient and (2) has a central IV line.  We called his transplant team, and they asked us to bring him to the ER at the main hospital, not the one across the street from our house.  Of course by the time we got there, his fever had magically disappeared on its own.

The ER was waiting for us, and they immediately drew a set of labs and blood cultures and put orders in for the two IV antibiotics.  His labs didn't look terrible.  In fact, his red blood cell counts and platelets had gone up a touch.  His ANC was just barely in the normal range, which for a healthy person would be fine but indicates an infection of some kind for Keegan.  (He has a condition called congenital neutropenia, meaning he normally does not have enough infection fighter cells in his blood.  For him to get to even the bottom threshold of normal levels indicates that he is responding to something.)



Keegan was actually in a very good mood all day.  He wasn't fussy or upset, just very chill and friendly in his ER room.  The fever stayed at bay almost the entire day without any tylenol.  However, anyone who knows him could tell something was brewing.  The child that is usually a tornado of activity only let his feet touch the ground once all day, to stand on the scale in the ER.  Around 4pm, the child who doesn't nap anymore virtually fell asleep sitting straight up.  We got to a room on the cardiac floor around 6pm, and he was ok until around 9pm when the fever came back full force.  He finally crashed again around 11:15pm with some tylenol on board.

We are desperately praying for this to be a virus of some kind.  Keegan cannot afford to have a line infection now (well, no one ever wants a line infection but still...).  We lost his last port to an infection, and it wasn't pretty.  It wouldn't be as terribly scary if he wasn't completely dependent on that line for the only thing keeping him going right now - TPN.  The blood cultures have to be negative for 48 hours, so we are here inpatient with the antibiotics going until that time.  I have to say that I'm absolutely petrified and will be consumed with guilt if his line is infected.  We won't know for sure where it came from, but I was the last one to touch his line.  I had to reaccess his port with a new needle last night.  I keep going over it in my mind, wondering if I didn't clean his site well enough or clean the hub long enough before hooking his TPN up.  I couldn't handle knowing I caused him so much discomfort and threatened his well-being in that way.

The other bad part of Keegan being sick is that it delays a new course of treatment we were about to start for his GI problems.  It appears from his scope biopsies that Keegan has intestinal graft-vs-host disease.  We are waiting on some blood work to be performed to confirm the diagnosis.  We believe a similar test was run on some biopsied heart tissue back in Boston in 2009, and we are trying to get our hands on that as well.  If confirmed, this would be an extremely rare case of a complication from transplant.  We had been planning to admit Keegan for a few days next week to start a steroid pulse for treatment.  I promise that I will explain this all as best as I can in a post tomorrow.  If the steroids work, it wouldn't necessarily improve his GI problems, but it would stop his body from attacking his intestinal tract.  Once that process is stopped, we would still likely need to travel to Boston for treatment and rehabilitation from that standpoint.

Again, I will explain it more tomorrow, but his team said today that he needs to be at least two weeks out from whatever illness he has now before we can even start the pulse.  I hate that.  To have answers and a treatment plan so close and then yanked away so quickly.  Ahhhhh!!!  Anyway, it's super late, and I need to shut my eyes for a few minutes before the next medication is done.  I just took Keegan's temperature and it's come down to 99.9 finally.  Looking like a long night here.

Do I need to say how much we would appreciate your prayers for our Bug?  Probably not, but we do.  So much more than I can express in words.  Thank you again.  

Famous

How nice to open the most recent fundraising letter from Children's and see Keegan's face smiling out at us!  It was another well-written version of Keegan and Johnston's story.

You can read it by clicking HERE.

Ironically, we also got the paper version of this newsletter in the mail, complete with the enclosed auto-generated letter asking for money...

"Dear Gray and Madeleine,
Happy endings like Keegan's happen because big-hearted people like you provide ongoing financial support...."

You have NO idea, Mr. Children's Foundation President....you have NO idea.  ;)

Wednesday, May 11, 2011

Home again, home again

Just a quick post tonight to say Keegan was discharged late Monday afternoon from the hospital.  (Yes, I'm well aware that today is Wednesday.  Thank you.)  The bleeding from his port and into the line has slowed but not stopped.  Keegan's blood counts came up but weren't great when we left.  His energy is up and down, and his color is plain awful.  He will have labs drawn tomorrow or Friday, so I guess we will see where he is then.

We still are not entirely sure what happened with his port.  Our best guess is a combination of three things: trauma to the site from him being an active toddler with a big ol' needle in his chest, a temporary bone marrow depression (when his platelets, red blood count, and white blood count all dropped for some reason), and an increase in his vascular pressures.  Who knows why his bone marrow took such a dive there from Wednesday to Friday.  It's Keegan...there's no explaining it.  The vascular pressure increase is likely a combination of things, but mostly the finger ends up pointing at his kidney.  His blood pressure has gone up over the last month, indicating his kidney isn't too happy (more likely his kidney than his heart at this point).  He is being pumped full of fluids every day that his kidney has a hard time eliminating.  We knew there seemed to be a lot of back pressure in that line, but it wasn't until Sunday night that it all came together.  There was an air bubble clamped in Keegan's line just before we hooked TPN up.  The nurse noticed that it was throbbing with Keegan's heartbeat.  Crazy.  Unfortunately, there's not a lot we can do about it.  We intentionally left his needle alone to give it as much time to heal as possible, but he will be due for a new one tomorrow.  Hopefully, it will be more manageable with time and not be such a huge problem.

I don't mean to be intentionally elusive, but we did get partial results from Keegan's intestinal biopsies back this week.  We have not made any firm decisions about what to do with them just yet, but the wheels they are a-turnin'.  I had a very long call with several of Keegan's doctors  this afternoon.  Ironically, his cardiologist and hematologist will have the most say over this plan.  We have consulted with them, his GI doctor, the hospital's chief pathologist, and his team in Boston.  There are still many questions to be answered and a few more blood tests that need to be drawn.  It may require us to have a brief intentional inpatient stay for treatment.  Over the next few days, we should have a clearer picture of this plan, and once I have a better understanding of it all myself, I will discuss it more here.  The greatest thing about it is that it is much more likely now that we will be here at home for at least another month, especially for Audrey's birthday.  That's a huge weight off our shoulders.  We will still probably be headed to Boston for management afterward, just not right away.

For now, we would appreciate your prayers for our wisdom, clarity, and guidance, as well as Keegan's doctors', as we make these plans and learn as much as we possibly can to help Keegan.  I can't deny my excitement that we may possibly have an answer or at least a feasible plan to help Keegan after almost four long years of not knowing anything at all.  However, we are still learning, planning, and consulting, and there are no guarantees that any treatment we try will work.  We simply are asking for prayers that we can make the best decisions possible with what we know now.  Thanks so much, as always.  We are so beyond humbled and blessed by your support.  More soon!

Sunday, May 8, 2011

Maybe tomorrow

Maybe tomorrow Keegan will get to go home.  We are still having some strange issues and continued bleeding from his port but not nearly as bad as before.  We have polled everyone from surgery to oncology looking for ideas as to what could possibly be happening with this.  No one seems to know for sure.  We are going to try a smaller needle tomorrow, but as long as his blood counts are stable tomorrow, I think we will be discharged.

The longer we watch his line, it seems fairly obvious that there is some kind of pressure anomaly.  There is an immense amount of back pressure in the line that shouldn't normally occur with a port.  The line goes directly to his heart, which leads us (as parents, not cardiac specialists) to think there may be a pressure issue with his heart.  His echo on Friday looked stable from his other most recent echos, although echos aren't always the best way to measure ventricular pressures.  Tomorrow, we are going to run some ideas past his transplant team to see what they may think about it.  Not sure what it would mean or if any of it pans out logically, but we will see.

We were told last week that the results from the electron microscope and the final biopsy reports from Keegan's scopes should be back by tomorrow too.  That would be so convenient to discuss it while we are here....which means it surely won't be ready.  A girl can hope though.

It wasn't the Mother's Day I would have preferred.  Today was also my amazing mom's birthday, which we weren't able to celebrate as we had hoped either.  But Keegan is stable and that is a good enough present for both of us, I think.  My parents and sister were able to bring Miss A up for a quick visit.  Having both my kiddos to hold again was the best part of my day.

Audrey with Keegan's picture on the wall of the cardiac inpatient floor

Being inpatient on Mother's Day certainly puts a different perspective on the meaning of this day.  The halls of this hospital are filled with women who are living the best and worst parts of being a mother to their children.  I know beyond a shadow of a doubt that I was meant to be Keegan's mother, and I know beyond all reasoning that his journey and trials are part of a bigger plan God has for us.  Keegan makes me want to be a better person, not just a better mother.  He has shown me the most difficult parts of being a mother, and Audrey has rounded out my rougher corners, reminding me that it's not always going to be like this.  I have had to hand my son over to surgeons too many times.  I have sat helplessly at his side and begged the Lord to let me take his place.  But I have laughed harder than I ever thought possible.  I have been overcome with joy through tears beyond my expectations, and I have become stronger than I ever would have been without one very small little boy in my life.  

Today may have been Mother's Day, but I think it better to thank my children today.  I am only a mother because of their births, but I am a mom and a better person because of their lives.  Keegan and Audrey, I love you to the moon...and back.


Saturday, May 7, 2011

Still Here

Keegan is still inpatient.  We were able to get his port working last night after busting out a clot that was in there, likely from all the bleeding.  That allowed us to use it to run his TPN overnight, but it didn't address the bleeding issues he was having when nothing was running through the line, i.e. when his TPN was done for a few hours.  So, the decision was to stay inpatient tonight and see what happened after his TPN was done at 4pm. 

We were told to let him act as normally as he would during the day to recreate the situation of the last several days.  That was the easy part!  There was a mistake in the lab, and his blood levels weren't checked today.  His color wasn't too good, but his energy levels were back to normal.  By bedtime, there wasn't a ton of blood in the dressing, but there was blood halfway down the line of the extension where the needle was.  It still flushes and draws back fine though.  So, we're running his TPN again tonight, and we will call the trusty hem/onc nurses for some advice in the morning.  Then, we'll decide what to do from there. 

On a positive note, we heard an interesting theory today that makes me even more anxious to get his full biopsy results back!  It sure sounds promising, but I never keep my hopes up for long on that end.  Too much disappointment.

While we were trying to keep Kegan entertained and running around, we enjoyed some sunshine outside in his favorite part of the garden.



Meanwhile, Audrey enjoyed the first dip in the pool of the season.  Shhh!  Don't tell Keegan!

So, one more day down.  Not the way I wanted to spend my Mother's Day (or my mom's birthday that happens to be tomorrow too!), but it's really just another day anyway.  Hopefully, we will be home by late afternoon tomorrow.  Thanks again for continuing to faithfully lift Keegan up in prayer.  We are humbled by your support for our Bug.

Friday, May 6, 2011

Port

So, you may have noticed via our twitter feed or FB updates that Keegan has been inpatient since last night because of an issue with his port.  I will try to make a very long story short.  We've been having some issues on and off with Keegan's port for about a month now. 

Wednesday evening before bed, however, we discovered this:

By the time we got him to the ER per our medical team's instructions, the whole dressing was covered in blood.  It took a lot of work from the hem/onc nurses, but we went home.  The line worked with his TPN overnight and through the morning.  But by bathtime yesterday, there was more blood, and we had to pull that needle out too.


We were told to go back to the ER...again.  At that point, no one was comfortable running TPN through it, and surgery said they needed to see him.  We can troubleshoot a line as best as we can from the outside, but surgery has "ownership" of the line as the ones who put them in and take them out.  Of course, that has to be downtown, not at the Legacy campus.  Since Keegan's body is dependent on the TPN, his electrolyte, vitamin, and blood sugar levels started to get very low.  We put a temporary IV in his arm to run a glucose drip with some extra vitamins.  Then he took his 12th ambulance ride to be admitted downtown.  By 2am, we were cozy back on the floor with his "girlfriends."

We came in to support his nutritional needs while his line was not working and obviously, to fix the problem with his port.  We ran a few tests, including a dye study and an echo, over the course of the day but had increasing problems with it all day long.  Surgery finally showed up about 20 minutes ago.  Per the tests run today, the port is intact and in the proper place.  The surgical team wants to try a few more things tonight and call the hematology/oncology team in the morning for more help.  If we still are not satisfied with its performance, we will discuss taking him into surgery to troubleshoot or replace the line. 

In the meantime, today his blood levels dropped unexpectedly.  We will redraw labs tomorrow.  Please pray this is a temporary issue and not indicative of something bigger.  As always, we appreciate your prayers for safety and healing for our Bug.  We did get a few tidbits of information from his biopsies today, but we are hoping the results from the electron microscope will be back by Monday to shed some better light on what we've seen.  More tomorrow as we learn it.  Thank you again.

Monday, May 2, 2011

Still waiting

Just a quick update to say that we are still waiting on Keegan's biopsy results.  Pathology hadn't released anything by 5pm this afternoon.  Frustrating to say the least.  Hopefully, they will be ready tomorrow.  Not that I'm getting my hopes up for results that will be helpful.  I just want something in my hands so that I can move on.

Our desire to get somewhere where he can be treated or at least seen with fresh eyes is increasing exponentially at this point.  He had a very rough weekend.  A few ounces of lemonade on Saturday tore him up for the rest of the weekend.  Just lemonade.  Really?  Then last night, we had another port issue come up.  We were delayed in getting Keegan's TPN hung because of the "big announcement."  (Not going to soil my son's blog with that name.  Sorry.)  His line would not draw back or flush around 11pm when we went to hook him up.  We made a few calls to the hospital and ultimately decided to try to reaccess him ourselves.  After calling family for back-up, we plucked Keegan out of bed and did the quickest needle change ever.  It took a little bit of manipulation, even with the new needle, but we got it working without a trip to the ER.  I still have no idea what happened.  Just one more crisis to reinforce the need to get Keegan some help...and quickly.

This choice is not going to be easy.  Boston has the positives of familiar faces and not having to start over from scratch.  Only two years to cover, rather than almost four.  His transplant team there has been advising us for months on his immunosuppression issues, so there will be little frustration in turning that responsibility over to them.  Cincinnati is the top-ranked GI program in the country for many, many years and has the thumbs-up endorsement from some good friends who are treated there.  Just yesterday, we had someone refer us to a program in Omaha that sounds promising too, so the decision is certainly not getting easier as days pass. 

All that being said, we are still waiting on biopsy results.  We would greatly appreciate your prayers at this crucial time.  Prayers for clarity and wisdom as we make big decisions that will affect our family for years to come.  Prayers for compassion and insight from doctors looking at Keegan's issues for the first time and hopefully doing all they can to do it quickly.  And as always, prayers for healing for our precious Bug, if that is His will.

Lots more to do tonight.  Just wanted to post a quick update.

And of course, some random pictures just for fun!

Colored bubbles...fun & messy!

Couch potatoes

He's hugging her!  I promise!

Being a trooper before his scopes last week

Peek-a-boo!

Mama's garden helper