Emerson is still looking punky, but is much improved from over the weekend.  The current thinking is that this adrenal crisis was brought about by her infections from last week.  They don’t think there are any new or hidden infections to treat.  I’ll be feeling better when she’s back up and asking for “Lello”, though.  For now she’s still looking spacey, picking at her nails, and occasionally moaning.  She’s not talking or moving much.  She is, however, sitting upJ.  Her coloring is improved, but she still looks pale and weak.

Let me explain what happened this weekend…

Emerson has adrenal insufficiency (aka Addison’s Disease):

“Adrenal insufficiency is a condition in which the adrenal glands, located above the kidneys, do not produce adequate amounts of steroid hormones (chemicals produced by the body that regulate organ function), primarily cortisol.”  ~  Wikipedia

This weekend Emerson had an acute adrenal crisis (aka an Addisonian crisis): 

“An adrenal crisis often occurs (when a person who has adrenal insufficiency) is subjected to stress, such as an accident, injury, surgery, or severe infection; death may quickly follow”  ~  Wikipedia 

In a nutshell, Emerson’s body isn’t able to produce adequate amounts of a hormone called cortisol.  Our bodies naturally increase production of this hormone during times of extreme stress and illness.  Simply put, it is an increase in cortisol that keeps our organs functioning in stressful times.  Emerson’s body was thrown into an adrenal crisis last week & her body began to shut down.  Her organs needed cortisol & her body was unable to produce it.  Once identified, the team started high-dose IV cortisol which, in turn, started to reverse Eme’s overall deterioration.  I hope that makes sense.   

Thank you so much for all your prayers through this difficult weekend.  With the help of a gracious God, guiding a top-notch team of medical professionals, Emerson appears to be on the mend & again out of immediate danger.  I’m not sure how many ‘lives’ a young girl can have, but she clearly has more than her fair share!

Dr. B walked in this morning & announced, “She’s Addisonian, it hit me at 5:00 this morning”.  Sure enough, a blood cortisol test returned very low – the first markedly abnormal test we have yet.  This is good news, as it is treatable.  What caused this adrenal crisis, though, is the million dollar question.  They started IV cortisol replacement & Dr. B suspects she’ll be looking much better by this afternoon.  Still, though, one doesn’t become Addisonian for no reason.  It is a secondary finding, not a primary one.  She is on IV medication now to cover a wide spectrum of organisms and illnesses, including viral meningitis.  She required 2 additional fluid boluses over night to keep her blood pressure up and continues to be very lethargic.  She is awake and alert at the moment, though, & that is very encouraging!!  For me, I’m going to try and get a little something to eat… it’s been a tough couple days.

Thank you so much for all your words of encouragement & for all your prayers.

After a second fluid bolus Emerson’s blood pressure is up – that’s good!  She’s not looking any better otherwise, though.  The neurologist was here & said she’s “severely septic”.  He said he doesn’t care about what is (or more accurately isn’t) growing on the cultures.  He thinks it just hasn’t shown up yet.  She is sweating and clammy, her temps have been low, & her white blood count is not elevated.  He said that’s all very bad news.  It means she’s tired and not able to fight this.  He said if this was post seizure activity she’d be getting better, not worse, & if it was continuing seizure activity her blood pressures would be high.  Moreover, he said you would know.  There’d be no question…

He was rather upset (that’s putting it nicely) that she’s not on antibiotics & called infectious disease to express his opinion and concern.  ID is talking to Transplant.  In the interim, they’ve drawn more labs. I’m trying to sleep whenever possible because when I’m awake I feel like I’m going to vomit.  I’ll let you know what transpires this evening.  Until then I’m curling up in my little red recliner & praying these labs tell us something…

Emerson continues to deteriorate as the day progresses.  Her blood pressures are dropping & they’re no longer able to maintain her without a line.  They placed a femoral line at the bedside, along with a foley bladder catheter, and are now pushing fluids to try and bring her pressures back up.  Her coloring is worse (which I didn’t think was possible) & she continues to sleep.  Neurology has been consulted, but hasn’t shown yet.

I am sick to my stomach and have a splitting headache.  I really don’t feel good at all…  I sat in the ICU waiting room while they placed the line; until memories of 16 months ago came flooding back & I started to relive the code.  I then went and sat in an empty room next to hers - that way I could hear her cries and know she was still breathing.

There are no words, I know…  Your prayers, though, would be greatly appreciated at this time.

Thank You.

one I could never have anticipated & one, sadly, not worth celebrating.  Tomorrow marks the 2 year anniversary of this current hospital admission.  Two years ago tomorrow I put the boys on the bus with kisses & a promise for McDonald’s and hockey practice after school.  Two years ago tomorrow I returned home to find Emerson seizing & burning with fever.  Two years ago tomorrow I rushed her to the ER at Denver Children’s Hospital.  Two years ago tomorrow began a journey far exceeding my wildest imagination – filled with so many ups & so many downs, so much worry & so much hope.  Two years ago today was the last day I would spend at home.  It is amazing how suddenly & unexpectedly life can change…

Emerson had been improving all week & plans were made for discharge to our Omaha apartment yesterday.  Indeed Emerson left her room on the general pediatric floor yesterday… she did not go out-patient, though. Instead she transferred to the PICU.  She woke up looking “off” & deteriorated all through the day.  She’s been sleeping now since 11:30 yesterday morning – 23 hours straight.  She looks septic, but she doesn’t have a line.  Blood cultures for now are still negative.  Something is very wrong, but there are no good clues as to what’s going on… at least not yet.  She’s been making unusual, repetitive motions with her mouth.  Her blood pressure is low & she looks acidotic on labs this morning.  Barring any hidden infection, my bet is she’s been seizing again.  We’ll see what the team has to say this morning on rounds.

I’ve had a rough 24 hours.  I was mentally prepared for discharge yesterday… I was not mentally prepared for this.  Emerson does not look good at all.  Her coloring is awful.  In the past they’ve always had a good suspicion as to what’s going on & they’ve always started treatment immediately.  This time they are treating nothing.  They don’t know what to do.  What’s more, Emerson lost her peripheral IV yesterday & after 6 unsuccessful attempts is now left with no IV access.  The team won’t place a central line because of her historically high risk for sepsis, but she literally has NO peripheral access.  So, she sits here today with nothing.  That is very scary for me.  If she crashes there is no way to push meds… no way other than putting a line into her bone, that is.  The measures are getting extreme.  She’s just been too sick for too long.  Is there ever a point where her body just gets tired?  Is there ever a point where she just decides enough is enough?  I don’t know the answer to that, but I’m fearful…

I try so hard to stay positive & I try so hard to trust.  It’s not been easy though.  I’ve watched so many of our “friends” pass & I can’t help but wonder when it’s Eme’s turn.  I can’t help but wonder if today is the last day I’ll hear my sweet little girl’s voice.  I close my eyes and all I see is Jim and the boys surrounding her as she slips away.  I see Collin falling apart.  I want so badly to protect him.  I want so badly to spare him this, to give him the innocent childhood he so deserves.  I am powerless though.  I can’t get the images out of my mind.  I fall asleep & I dream about death.  I can’t shake it.  A great friend once told me it’s the devil trying to take over my thoughts.  I tell him to go away, but still the images will not.  I start to cry & I just can’t stop.  I feel so foolish, but nothing will stop the tears.  Conversely, even the smallest, most unexpected thing brings more…

Please don’t misunderstand me.  Emerson is not gravely ill today.  Something is wrong, but it could ‘simply’ be continuing &/or post seizure activity.  She could well be up and entertaining the team again tomorrow… I, actually, half expect it.  She’s a fighter with an incredible will to survive and to thrive.  I’m nearly certain I could not have endured all she has in her short life.  She is one remarkable little girl & I continue to have great hope for her future.       

So I started this post saying this anniversary is one not worth celebrating, but maybe I’m wrong.  Indeed Emerson is with us today – something we could only have dreamt for 18 months ago, something that seemed a near uncertainty just 16 months ago.  Without doubt this journey has been difficult.  Without doubt we’ve made sacrifices.  Without doubt we’ll be managing the consequences of those sacrifices for many, many years to come.  She’s here, though, & we have hope – still so much fear, but also so much hope.  Emerson began this journey, really, at birth.  She began this admission at a tender 16 months of age.  Today she’s almost 3 ½.  She’s grown up in a hospital.  It doesn’t make sense &, for me, it never will.  My greatest hope today, though, on this 2 year anniversary & through tears streaming down my cheeks, is that quieter days are ahead (or should I say ‘louder’ days - days with more energy and vitality).  She deserves that, she really does…   

Now Emerson has respiratory adenovirus too… ah, the life of an immune suppressed child.

She’s looking & feeling much better, thoughJ.  We’re hoping to escape this week & finish treatment at our “home” away from home (aka the apartment)!  

I’ve spent days now contemplating what to write… Ultimately I asked myself this question:  Years from now what do I want to remember from the weekend?  Answer:  Smiles, giggles, presents, & a yummy cake.  It was a birthday party in grand White Family styleJ!! 

Bradley turned 8 last Thursday & we decided to meet half way (North Platte, NE) Friday to celebrate the big day.  Collin was out of school for conferences & Birthday Boy got to miss a day.  After our two trips to Colorado ended abruptly, we decided not to chance it again.  Instead we opted to drive 4 hours and meet in the middle.

We got a hotel room for the night and Eme and I showed up early to decorate.  This year’s theme was Star Wars!  Bradley arrived very excited to find banners, balloons, & (most especially) presents!!  Even Bella cameJ.  All of Emerson’s favorites - “Dad-eye, Beet (aka brothers), & Lello”… a party for sure!!

We went out for dinner and embarrassed Bradley with a request for singing waiters.  He wasn’t too bothered by the giant brownie & 4 scoops of ice cream with a candle on top though!!  Afterwards we returned to the hotel to open & assemble ALL the birthday presents.  We had adjoining rooms & I told the boys they could stay up (in theirs) as late as they wanted – 2am & they were still building, playing, and battling.  Emerson loved all the activity & they loved being with her tooJ!!  It was Bradley’s birthday, indeed, but I got the greatest gift of all.  The smiles & giggles were priceless!

Unfortunately Emerson, in usual fashion, spiked a fever to 104◦ Friday night.  I brought her into bed with me (afraid she might seize), but she wouldn’t sleep.  Finally about 4am she passed out & Saturday morning she would not wake up.  All the activity of a crazy dog & 2 loud boys and nothing… not even a flinch.  I called the transplant clinic & they said, “Get her to the closest ER”.  The question at that point was whether or not it was safe to drive her to Omaha or if she needed (yet another) life flight back.  Honestly, I couldn’t make this stuff up if I tried.  24 hours – that’s all we attempted.  4 hours – that’s as far away as we went. 

Long story short, we made a quick trip to the local ER where she was whisked into a triage room & deemed stable for transport.  Luckily we avoided a helicopter flight back to Omaha.  I’m seriously sooooo tired of all the drama.  Eme is a princess all right, but private planes & helicopters we can do without!!  She was admitted through the treatment center & here we sit again – same room (#6475), same story, different day.

The current thought is that the Klebsiella infection is back.  Her symptoms emerged just one day after stopping antibiotics, & after we pulled her line and re-started antibiotics she looks good again.  I’m waiting for Infectious Disease to consult & am hoping we can manage this out-patient.  This is honestly getting harder & harder to endure.  Especially after a short visit with the boys – I just want to go home.  I’ve been fighting for Eme for so long now.  Maybe it’s time to start fighting for my family.  The team wants Emerson to string together 3+ months outpatient with no hospital admits.  Let’s just all agree that’s probably not going to happen & move on to a different plan.  I digress…

I honestly wish I knew what I do differently from every other mother.  How do others take their children out-patient and stay out?  How do others leave Omaha 4 months post-transplant and only return once or twice, or never at all?  How do they do it?  I want to know… I want to do it.  I really, really do. 

As usual, I’m tired, cranky, & overwhelmed.  I need help but don’t have the time or energy to reply to all the wonderful people who’ve offered their support.  Please bear with me.  I’ve had so many gracious offers for help with fundraising and medical/nursing assistance.  Thank You.   

There’s so much more… there always is – encounters to recount, emotions to share.  In time maybe I’ll get to them all.  For today, though, I’m stretched out on a little sofa, watching Barbie and the Twelve Dancing Princesses (again), & thanking God that I have one more day to share with my beautiful little girl, anywhere that may be. 

We’re “home”!  I’m exhausted but wouldn’t trade it for anythingJ.  Emerson is in a much better mood here & I’ve had my pj day!!  Eme’s white count is elevated & her kidney numbers are up (that’s bad), but you’d never know it – she’s as happy as ever!  She loves to “go” & is walking everywhere… all on her own!  She still has a week of IV antibiotics remaining, which means I still have a week of no sleep ahead.  But, as I said, lack of sleep is a small price to pay for the smiles, kisses, & belly laughs I’ve been enjoying with my sweet little angel.  All in all, we’re great! 

For those interested, I’ve got lots of info on how you can help with fundraising efforts to come soon. 

Thanks for checking in!

Small changes to last week’s plan…  Emerson’s kidneys didn’t like the switch to her antibiotic schedule and the team decided she couldn’t be discharged on Q6 (administered every 6 hours) IV meds.  Luckily, though, they changed around some other meds & her kidneys, at the moment, are cooperating again.  They opted to keep her a little longer, though, and watch them closely.  That, plus, the meds she needs to go out-patient couldn’t get to us until next week.  So, the new plan is to discharge Tuesday.

It’s been another 4 week admit & I’m going stir crazy!!!!!  I really, really hope Tuesday’s the day!  In big news (shhhhhh… we can’t tell Eme this time), I talked to Dr. B & he gave us the go ahead to re-attempt the Make-A-Wish trip for February!  It’ll be the last attempt.  If we cancel again she’ll have to opt for a non-trip wish.  Of course, I have no idea if she can do it but a Mommy certainly can hope!  As usual this journey is lived one day at a time. 

As for transitioning back to Colorado, the current agreed upon “plan” is to stay in Omaha through Emerson’s 1 year post-transplant #2 anniversary, which will be in April.  If she’s doing well at that time we’ll (hopefully) start packing our bags!!  There’s still much to work out between Denver & Omaha concerning Eme’s long term care.  It will not, unfortunately, be a seamless transition.  Emerson is complicated and Omaha is very protective.  I appreciate that & know we all have the same end goal in mind.  Of course, I also have the added goal of rejoining our family.  I’m pretty confident we can get it all worked though.

Not much else to report.  My main goal this weekend is to get caught up on promised replies to all my website friends & also to put more thought into fundraising ideas.  Hope your weekend is a productive one (or a totally nonproductive one, which sounds great tooJ)!

Quick update: 

We're going to try & switch Eme's IV antibiotic to every 8 hours & see if her kidneys will tolerate it (larger, less frequent doses).  If so, that should make things a little easier at "home"!  I'll let you know.

Emerson is going out-patient this week.  Pause…  Breathe…  No cheering, please.  There are no changes in her condition, she is not miraculously healed.  The cardiologist who is now involved said she has an infected thrombus (blood clot) & has extended treatment from 3 weeks then re-evaluate, to 4 weeks with a possible 2 more.  Today Eme is on day 12 of 28 for the initial treatment.  That means we have 16 – 28 more days, depending on the outcome of next week’s follow-up echo.  She’ll remain on the very strong IV antibiotic that threatens permanent damage to her kidneys.  She is also now being treated for c-diff.

Upon news of 2-4 more weeks here, I shut down.  I’ve said it before, I know, but I can’t do this anymore.  I can’t live in a hospital anymore.  Call it selfish, okay.  Maybe it is.  But when I say it this time I really mean it – I can’t do this anymore.  So, I asked the team if I could pleeeeease take her back to the apartment.  I think I know what I’m getting myself into & I KNOW it’s going to be difficult there too.  As a matter of fact, I fully anticipate a similar entry next week.  I’ll be drowning, I know I will.

Her daily care will involve 2 nebulizer treatments, 4 bladder catheterizations, a multitude of meds every 12 hours, several meds every 8 hours, and an IV infusion every 6 hours.  Unfortunately, every 6 hours does not mean 4 times during the day.  Indeed, it means every 6 hours.  Med schedules don’t allow for sleep.  Each infusion will take time to prepare & will run for 45 minutes.  If you do the math, you’ll find it leaves very little time for sleep.  Add to all that trips to the hospital 3-4 times a week for labs to monitor her kidneys.  I don’t know if I can do it.  I really don’t - she very well might be back next week.  I only know I have to try.  (And if you’re questioning, & as I’ve said before, Colorado Medicaid will not pay for any home assistance while we’re out of state – it’s simply not available.)

Emerson is feeling better & she doesn’t want to be here either.  She screams at me most of the day.  She needs to be reprimanded & I can’t do it here.  She’s starting to exhibit serious behavior problems & if I don’t address them soon they’re going to get out of control.  They’re quickly headed in that direction.  It’s easy to treat “medical children” with kid gloves.  It’s tempting to want to forgive all their bad behavior.  I can’t do that, though.  It does no service to her.  She needs to learn these hard lessons like every other child - especially if I ever want her to function “normally” in the outside world.   

I just set up her little kitchen.  I have tons of stuff here.  I can’t believe I’m taking it all home.  I just don’t know what to do anymore.  There are no easy solutions…

Thank you so much to all who’ve offered to help with our fundraising efforts.  Thank you so much!  Please be patient with me.  I promise to get back to you all this weekJ.