Tuesday, August 21, 2012

One week

The "how-long-Keegan's-been-home" clock has restarted and is now sitting at a week.  His labs last week looked better, and Monday night was his last dose of IV antibiotics.  Overall, he is doing pretty well and has gotten back into the groove of therapy and appointments.

I will get back to the blog in earnest here soon.  It's been a joyous week so far for me.  Stitches in my hand from a shattered salad dressing bottle.  Colonoscopy tomorrow to follow up on some issues that the gallbladder removal didn't seem to solve.  Fun times.

For now, please forgive me, and enjoy these snapshots of K & A dancing in the rain.

Something I think I need to get better at myself.

Monday, August 13, 2012

Looks like...

Keegan's home.

Our schedule for tonight:
10:45pm - Start TPN
11pm - Go to sleep
1am - Give IV benadryl and start IV antibiotics; change diaper
1:20am (if I'm lucky) - Go back to sleep
3am - Stop antibiotics; flush and lock line with heparin; check diaper again.
3:20am - Maybe go back to sleep
5:30am - Gray's alarm goes off
6am - Hopefully drag myself back out of bed to run
8am - Keegan (please, please, please) wakes up
8:45am - Stop and flush TPN
9am - Give IV benadryl; start antibiotics....etc....etc....etc....

So looks like for the next week or more I will not get more than 2 hours of sleep in one stretch.

Let me contain my enthusiasm.

Sometimes....maybe,just maybe...it's not so much of a relief to be home, rather than inpatient.

Certainly helps so much that waiting for us at home was:
- More insult from drought/heat to our already cracked foundation so that Keegan's bedroom door now will not close;
- Statements from United Healthcare that our $12,000 deductible (yes, you read that correctly) that had previously been met as of April was now miraculously UN-met.  Not even not met but rather at a big fat whopping $0.  Along with a bill for an insulting amount from my GI doctor.

Welcome home, Harrisons.

Welcome *sigh* home.

Sunday, August 12, 2012

Weekend update

Today was better than yesterday, and for that, we are grateful.  Keegan walked twice and went to the play room for 45 minutes.  Much better than yesterday's 10 minutes.  He is more even-keiled today also.  I'm sure it helps that his heart rate finally came back into his normal range this morning.  Keegan's antibiotic levels have been within target range the last two draws.  The first dressing change on the line went well yesterday, and it appears to have stopped bleeding.  Our goal was to finish the round of IV antibiotics once we were confident that we had a therapeutic level of drugs on board.

However, his labs started inching in a head-scratching, nerve-raising direction this morning.  We were hoping it was maybe a fluke, but then my mom called this afternoon to say Audrey had spiked a fever.  So, once again, we ask for prayers of protection for Keegan and that Audrey finds comfort, being sick once again without her Mama and Daddy around.  We will repeat labs in the morning and a chest x-ray.  If things seem to be returning to normal, then we will aim for home tomorrow evening, while Audrey stays with my parents until the fever has passed.

Overall, we are very grateful for a rather quiet weekend for Keegan.  Thank you, as always, for supporting our family and praying with us through these trials.  It means everything to us.

Friday, August 10, 2012

New line in

Keegan made it through day 3 of anesthesia and has a new central line to show for it.  His surgeon said this was the smoothest line placement he has done on Keegan.  Unfortunately, the team felt very strongly that Keegan should not have another port put in due to his current wound-healing issues.  The surgeon and doctors were concerned that the incision above the port would not heal properly and that the port could literally come right out of the wound.  That would require another surgery to stitch him up and place yet another line, as well as pose a huge risk of infection.  We discussed it at length, and we agreed that a broviac was the safer choice right now.  This is an embedded central IV where the cannula tunnels directly out of his chest.  He has not had a broviac since he was a baby.  Unfortunately, this means the end of swimming for Keegan this summer, as the dressing cannot get wet.

Today was very difficult for Keegan emotionally, but we are hoping for better days to come as he gets a break from the anesthesia and peripheral IVs and draws.  His steroid pulse is over tomorrow.  If he stays stable and can reach our goal level of antibiotics, we will be able to go home early next week to finish the round of antibiotics there.

A smile to end this post?  After being covered repeatedly in surgical scrub the last several days, it was time to get cleaned up.

Much better!

Thursday, August 9, 2012

MRI done

Another long day and one coming up tomorrow too.  Not much energy or time to update tonight, as Keegan is very restless.

Keegan made it to the scanner for an MRV (magnetic resonance venogram) of his torso and an MRA (magnetic resonance angiogram) and images of his brain.  The venogram gave us some good images for sites for his new port placement tomorrow.  It will take a few days for the radiologist to compare the brain scans to last year's.

We had a long day with the peripheral IVs.  Both appear to work, but neither will draw blood.  They tried four times to get a blood draw for labs while he was under anesthesia.  What they got gave us some really wonky numbers, so we had to poke him yet again this afternoon for another set of labs and a culture this afternoon.

I cannot wait for tomorrow to be over.  Between the repeated rounds of anesthesia, the steroid pulse, and Keegan's underlying anxiety, he is miserable.  Not getting any real rest despite the drugs.  Tomorrow's surgery is scheduled for 11am, but his surgeon is on call for trauma, which means that he may get bumped.

We just can't say our thanks enough for your continued prayers this week.  We will update tomorrow with more information.

Wednesday, August 8, 2012

Line's out

A long day.  Keegan's old port is out, but it was a long, long day, indeed.

We woke up this morning to a line that would flush and work but not draw blood.  Hematology/oncology was consulted; they deemed the line safe to use, but we could not get the labs needed prior to surgery today.  So a peripheral IV was placed (thankfully) in his right hand.  Even though it didn't work properly, we kept it in after labs were sent in case the anesthesiologist needed it.  We were told he was on the OR schedule for 12:50pm.  

Between feeling like crud and his normal anxiety issues, the IV was a source of continuous problems all day for Keegan.  12:50pm came and went.  He had been on strict orders of nothing by mouth since midnight because we had no idea when surgery would be.  This was the first time in the last 4+ years that Keegan was able to (1) comprehend the feeling hunger and (2) express his desire for food to us.  Heartbreaking.  By 2pm, I told him to just close his eyes and take a nap, and the transport team arrived to take him to the pre-op area a little after 2:30pm.  It wasn't until after 4:30pm that he actually made it to the OR.  The procedure went well overall.  The surgeon had to take some extra time, as this line was much longer than a usual port.  It was actually in the femoral artery and tunneled across his chest.  There was very little evidence of infection in his chest cavity, so they were able to clean it out and close him up, rather than leave the site open for healing.  

This afternoon, the decision was made not to put an EJ line in while in the OR.  With his current wound-healing/bleeding issues, there was concern that the jugular would collapse or clot off and be unusable for caths and treatment in the future.  Crucial concerns for a heart transplant kid.  So now he has a peripheral IV in his left hand and right ankle.  This means he will not be able to walk until the next surgery.  His autistic preoccupation with bandages means that he is completely beside himself over the new IVs when he is awake. 

Luckily, most of tomorrow will be taken up by an MRI scan of his body and brain.  The body scan will assess sites for the next port placement, and the brain scan will be to see if he has suffered any further neurological damage from the MAS since last fall.  Because of the delays in the OR today, Keegan did not have the echo of his heart.  That should be done tomorrow.  As of right now, we do not have an OR time for Friday yet.

We have sedated him this evening to ease his anxiety, and we may have to do the same thing tomorrow.  Please pray with us for Keegan's acceptance and endurance over the next few days.  It is going to be rough for him.  And a few prayers of protection for his kidney would be appreciated.  It is showing signs of slowing down right now and is being challenged greatly by the antibiotics and anesthesia.  Thank you so much for your support and love today.  We are beyond grateful. 

Tuesday, August 7, 2012

Surgery tomorrow

As I mentioned briefly last night, the staph epi bacteria has resurfaced in Keegan's central line, which caused him to have another MAS flare.  We have successfully kept the flare to a low roar with yet another steroid pulse.  Unfortunately, the infection means that Keegan's line/port will have to be removed and replaced by a clean line.

This particular bacteria is one that usually only affects people with severely suppressed immune systems.  It is so common on our skin that it is often disregarded in blood cultures as a contaminate, rather than a true infection.  Staph epi has a particular habit of clinging to plastic in the body, and once it has, it can be particularly dangerous and difficult to treat.  Generally, line infections are treated to negative cultures, but the line doesn't have to be removed until the infection recurs three times.  This is only the second time with this line, but we will be removing the line anyway to give Keegan time to recover before we make the next round of treatment decisions.  

Between the steroids and maximum doses of anakinra, it is as if we put a lid on a boiling pot of water with this disease process.  This regimen is not sustainable for much longer, and we are not getting full control of his immune deficiency, rather just keeping it mildly in check.  Keegan's medical team is now fairly confident that what we are dealing with is an innate immune deficiency/defect that he was born with, not that he developed as a result of immune suppression from transplant.  It may sound counterintuitive that an immune deficiency could cause an overactive immune system (flare), but what is really happening is an overcompensation by one part of the immune system for another part that should be there but isn't.  The steroids and anakinra suppress the parts that we believe are overreacting. However, they are not strong enough and have too many drawbacks to work effectively in the long term.  Keegan is already showing symptoms of chronic steroid use (no vertical growth in over a year, bruising, wound-healing issues).  The anakinra has too short of a half-life to be used at this dose for more than short periods of time.

We are waiting on two different sets of labs to be finished in order to decide what step to take next.  One is a genetic test looking for known mutations of Severe Combined Immunodeficiency (SCID), or "bubble boy syndrome."  If he does have one of the forms of SCID, it will most likely put us back on a bone marrow transplant track.  If not, we are also waiting on some research-level labs being run by the rheumatology group that look at the inflammatory pathways of Keegan's immune system.  Those results may help us feel more confident in choosing the next, stronger drug to use.  In either situation (transplant or more immune suppression), moving forward with a risk of an infected central line is too risky.  We anticipate the results of these labs will be back by early September, so we have to act on the line now.  Additionally, we don't want to risk another flare like he had in Boston that we are unable to control because of a latent line infection.

So now we proceed with treating the infection while waiting on the rest of the pieces to fall into place.  That means Keegan will go into surgery tomorrow to have his current port removed.  They will then place a temporary IV in his external jugular vein (the side of his neck).  This is longer than your typical peripheral IV but not long enough to risk the staph migrating to it too.  Protocol requires that you wait 48 hours after removing an infected line before inserting another central line to be sure that any bacteria that is showered into the blood stream is treated and won't colonize on the new line.  We will use the EJ line for his antibiotics, a less concentrated version of his TPN, and hopefully his anakinra.  If the pharmacists decide that the anakinra is too strong for that line, we will have to inject it subcutaneously twice a day.  Anakinra is very acidic and very painful, so we want to avoid that at all costs.  Keegan's surgeon has the OR on Friday, and we are praying that he can fit Keegan into the schedule then for a new line.  Then, we could theoretically go home on IV antibiotics for two weeks to be sure that the line is and stays clean.

While the fever has broken and his labs have slightly improved, Keegan was a wreck today.  Extremely emotional, agitated, and very, very tired.  He all but fell asleep while eating his lunch, pickle in hand.  I was able to get him to go outside for about 20 minutes in the late afternoon (I know it's hot out there, but it's a frozen tundra in here!), but that made him so tired that he was begging for a second nap an hour after his first.  He made it to bedtime but just barely.

We do not have an OR time for tomorrow because he is an add-on.  Tomorrow he will also have an echo to make sure the staph did not migrate to his heart at the tip of the catheter, and in the next few days he may have to have an MRI scan to check for an open vein before the new line is placed, as he is running out of access sites.  We simply cannot thank everyone enough for your continued prayers as this long journey continues.  You give us hope and strength to continue on. 

Monday, August 6, 2012

Flare + infection

That's what it looks like at this point. See post below for reference.

We should know more tomorrow about a plan, so I will update with more detail then.

Until then, this about sums up how Keegan and the rest of us feel at this point. Not exactly how we pictured spending Gray's birthday. One more holiday/occasion checked off the list for which Keegan's been inpatient.

- Posted using BlogPress from my iPhone

Sunday, August 5, 2012

Round 32

Keegan spiked another fever last night and was admitted to the cardiac floor at Children's again this morning.  His 32nd hospital stay in four years.  This had been the longest Keegan was at home since October 2010.  This fever cycle actually started Friday, but his temperature soared past the cut-off for an admission around 3:30am last night.  He had a really rough morning between the fevers, the testing, a needle change, and transport downtown.  However, his fever has been down since late morning.

At this time, we are treating it as a flare with yet another steroid pulse, as his labs are showing early signs of this.  Cultures were drawn this morning and have to be watched for 48 hours before we can be sure it's not a recurrence of the staph epi line infection.  There are a lot of what-ifs that I don't want to discuss right now based on either of those situations (flare vs. flare+infection).  The fact that we found out his SCID genetics likely won't be back until September makes all of this even more frustrating.

Keegan has developed some wound-healing issues that are continuing to worsen.  He has been quick to bruise since the steroids started last summer, but now the slightest touch can cause a gaping wound.  Both shins and his bottom have ugly ones that have been there over two weeks, despite treating them as much as he will allow.  This morning, a simple touch of an otoscope in his ear canal to check for ear infections caused his ear to bleed so profusely that when it stopped, the entire opening was blocked by a plug of dried blood.  This appears to be extremely confusing to the team, and as of right now, they are telling us it is from chronic steroid use.

After losing weight and seeing his labs trend down for the last two months, we restarted TPN three times per week on Wednesday.  That capped a difficult week after the loss of our dear friend, Nate Arnold.  Nate was Keegan's transplant "twin", as he received his angel lungs on Keegan's second transplant anniversary.  I cannot even begin tonight to untangle the mass of emotions and grief that we have experienced over the last week.  To top it off with an inpatient admission for yet another fever after 11 weeks of the same kind of thinly veiled stability that Nate was experiencing is almost too much.

We hope to know more tomorrow with more time, the full team, and another set of labs in the morning. We are praying for a break for Keegan tonight, and we would greatly appreciate it if you would join us.