Tuesday, December 8, 2015

All We Want for Christmas...


Have I mentioned our girl is growing?  This time last year, Josephine was still in ICU and still weighing in at under 10 pounds.  As of her Immunology appointment last week, she has grown to nearly 23 pounds, and more impressively, just an inch or two shy of three feet tall!  Healthwise, she's doing well.  Confident with her progress, her doctors have reduced the frequency of her clinic visits.  Every week...to every two weeks...to every month...to every three months...and now once every SIX months!  Her next official (or more accurately, "scheduled") procedure will be a cardiac cath next August.  Dr. Ikemba and Dr. Nugent have even cautiously predicted that, due to her abnormally high oxygen saturation levels, Josephine may NOT have to undergo the Fontain surgery when she turns 3.  What an enormous blessing that would be!


Developmentally, Josephine has made big strides in the past several months.  When I last wrote, she was mock crawling.  Her chosen mode of transport these days - when not hitching a ride with Mommy or Daddy or coasting about in style on her elephant cruiser - is speed crawling.  (She has gotten quite good at it and will drop down and take off crawling whenever the urge hits...which is quite frequently!)  She's still using furniture for walking support, but has also taken to horizontally "scaling" walls, Spiderman-style.  Climbing stairs is also quickly becoming a favorite. (We call it "climbing Mount Doom"...and just like the greatest mountaineers, she always does it without supplemental oxygen. :))

Although she has made exceptional progress from a feeding standpoint, we're still about 8 oz. short per day from pulling her ng tube.  (On a normal day, Josephine needs about 28 oz. of puree and formula.  If she takes any less, she not only runs the risk of losing weight, she also could become dehydrated.  Very bad for a cardiac kid!)  Today, we'll be starting an inpatient stay at Our Children's House, a small, specialized hospital that serves kids with feeding challenges.  Over the next 4 weeks, Josephine will receive intensive PT, OT and speech/feeding therapies, with the ultimate goal of increasing her oral intake to a point that we can officially, once and for all BE TUBE-FREE!  While none of us are exactly thrilled at the prospect of spending another Christmas in the hospital, we have every confidence that this ninja-princess will continue to be a total rockstar when it comes to reaching her treatment goals.  Come New Year's, she'll be a walking, talking, eating machine. (And Mommy and Daddy will be in REAL trouble then!)


We'd appreciate prayers and positive thoughts specifically for:
1. An expeditious, productive stay at OCH...we wanna be tube-free!
2. Extra measures of sanity for Mommy and Daddy as we re-adjust to hospital life.
3. Understanding from our employers, who have been extremely supportive up to this point. 
4. Honesty, empathy and compassion from our medical insurance carriers.  (We have had quite a fight!)

Thursday, August 6, 2015

Pre-Cath Jitters

Difficult to believe another month has passed (and really difficult to believe it took me over a month to upload the prior post.  Where has the time gone?)

Tonight we’re preparing Josephine for her cardiac cath, which is set to take place tomorrow.  It would be a huge lie for me to say I’m not nervous.  (I read some research that said parents – in particular, mothers – who have children with heart defects that undergo surgery and have an extended hospital stay often develop symptoms of PTSD.  I believe the specific words were “at increased risk for intermediate and long-term psychological malfunctioning.”  Malfunctioning.  Is that what I’ve been doing over the past several months?  Why didn't someone warn me?) 

Despite our nerves, our sweet, precious girl, is playful and happy…and not at all wanting to go to sleep this evening. J

We’ll be at Children’s bright and early for our 6:00 a.m. appointment.  I’d imagine Josephine will go back at 7 or 7:30, and the procedure will probably take 3 hours or so.  We specifically requested Dr. Nugent to preside over the “festivities”.  He’s a great practitioner, explains everything thoroughly and has tons of experience with cath procedures.  (Plus, he has a ridiculously cool accent.  That’s a bonus. J)  Most importantly, he was attending physician at points during both of Josephine’s other post-surgical hospital stays, so he knows a bit of what she – and we – have been through thus far.  It’s nice to have people around who know her history.


We love this little girl so much!  Requesting tons of prayers and positive thoughts for tomorrow!


Oh, What a Difference a Day Makes!

Our little girl is growing!  

When we left the hospital back in January, Josephine was weighing in at roughly 12 pounds.  Thanks to some good nutrition – and in spite of a nasty tummy bug we weathered back in early May – she’s now up to almost 20 pounds!  According to Dr. Ikemba (her cardiologist), she’s steadily on track for her weight and above average in height (she gets that from Mommy) and head circumference (we’re gonna say she gets that from daddy. :P).  She has also made some remarkable medical strides over the past few months.  Although she’s stubbornly holding on to her ng tube (deep suction is a trauma that isn’t easily erased, apparently), we’ve entirely weaned her off of the pain and anxiety medications she required after surgery.  (No easy task.)  We’ve also managed to eliminate all 5 breathing treatments!  We still administer around 25 different doses of medications each day, but the regimen – at least in terms of time – is much improved. 

Josephine has also been working with physical and speech therapy to make up for developmental time lost while she was in the hospital. Michael and I are continually amazed at how very capable – but very willful – this girl is!  Everything (and I mean everything) is on Josephine time.  She’s constantly taking things in and assessing her options…”Hmmm…you want me to sit up, huh? Well, what’s in it for me?”  One day she’s not.  Then boom. She just decides things are going to happen.  Currently, our little princess is sitting, mock crawling and standing with a little assistance from the ottoman in our living room.  And for her next big trick?  Perhaps walking!   (We have no doubt that once this kid gets moving, there’ll be no stopping her. ☺)

Josephine’s next surgical procedure will be in early August. The doctors have scheduled another heart cath to coil some of her collaterals.  (Basically, this involves sealing off vessels using a coil-shaped device that is snaked through a vein in her leg via a catheter.  A serious deal, but minor in comparison to the other surgeries she has had.)  While the collaterals seem to be serving her well – her O2 levels are currently running around 92% - the cardiology team has explained that too much blood flow can increase the pressure in her lungs and potentially jeopardize the final surgery.  That could ultimately result in a transplant scenario…so, cath we will!


Between doctor’s appointments, therapy visits and general “life-with-a-baby moments”, we’ve been busy getting ready for Josephine’s first big adventure – to Alaska!  Michael’s grandparents will be celebrating 60 years of wedded bliss and have invited the entire family on a cruise to share in their joy.  The past few days have been a flurry of packing and preparation.  We even made a special trip to the pediatrician this week to get some ear-numbing drops (just in case), some allergy medication (just in case), and some advice on “what to do in case of…” (just in case ☺).  To make sure all of our bases are covered, I also purchased the economy-size package of ear plugs for the plane ride.  Maybe instead of being the family that got kicked off a plane for having a noisy infant, we’ll be that sweet, well-meaning family who did their best to minimize discomfort for all involved.  Hoping for the best!

(Sadly, I wrote this back in June...didn't post until August! Ack!)

Thursday, November 27, 2014

Thanksgiving and Giving Thanks

Having a child in the hospital is full of ups and downs, long-awaited triumphs and dismal disappointments.  On one hand, a successful plication surgery and subsequent reinflation of a lung that has struggled for weeks to heal.  On the other, a chylous effusion followed by a repeat lung collapse.  Up then down.  Up then down.  I won't lie.  Staying even somewhat positive is a constant challenge.

This past week, one of Josephine's hallmates died.  It was a little girl, I think.  And maybe a transplant patient.  We saw her family in the "consultation" room (the same one they took us to after Josephine's first surgery when she began hemorrhaging.)  Not a fun place to be.  Now, as awful as it feels, I'm grateful... because it could have been us.  It could have been our child whisked away to emergency surgery. It could have been our family members crying outside the consultation room.  It could have been us making funeral arrangements in the hallway outside the ICU.  But thankfully, it wasn't.  There's a duality to the situation that is fundamentally mind-numbing.  Simultaneously horrible and wonderful.  Nothing to do but keep moving and keep praying.  And so we do.

Although today is Thanksgiving and being in the hospital isn't exactly how we had originally planned to celebrate the day, we have much for which to be thankful:

A successful plication surgery.

A successful extubation.

Two working lungs and a plan to come off of ventilatory support.

The removal of all chest tubes, IVs, cardiac lines and pacing wires.

A few beautiful smiles.

Daily opportunities for holding and cuddling.

And six (SIX!) amazing months with our sweet Princess.

We're grateful.  To know her and love her, and to be called to comfort her, care for her and help her thrive.

Turkey dinner?  Who needs it.

Sunday, November 16, 2014

Like a G6

My oh my, how time does fly!  The last two weeks have held lots of ups and downs for our sweet girl.  A partially paralyzed diaphragm, alternating episodes of lung collapse and pneumonia have all been part of our very recent past.  And that brings us to where we are now: extubated and breathing comfortably with the support of Vapotherm ("non-invasive" oxygen flow delivered through a nasal canula).  Since Josephine's right diaphragm isn't working properly due to a damaged phrenic nerve, progress is painfully slow.  Each day her ventilatory support level is stepped down, and so far she has handled things well.  If she manages to adapt to the decreased settings through this evening and tomorrow, there's a chance they may decide plication surgery to "tack down" her diaphragm won't be necessary, and (best of all) they may remove her cardiac lines.  Once those are gone, we'll (finally!) be able to hold her again.  Two and a half weeks without being able to hold and comfort her is waaayyyy too long!

Over the past couple days, Josephine has had decent periods of wakefulness during which she has taken to swatting at her butterfly mobile.  Just last night (the first night that Michael and I had been home since starting this leg of Josephine's surgical adventures), Josephine smiled.  We were a bit jealous that Grandma--who had generously volunteered to spend the night at the hospital in our place--got the first smiles since surgery, but we're super excited that Josephine is emerging from under the sedation and her sweet personality is still intact.  She has spent much of the day today playing and smiling and looking happy.  For us, it just doesn't get much better than that.

If all goes well, Tuesday could be cardiac line removal day.  From there it will be a matter of weaning down the ventilatory support to a minimal level.  No telling what could happen in the next week, but we're hopeful that we may be home by Thanksgiving.  What a wonderful cause for thanks!


Sunday, November 2, 2014

The Glenn

The Glenn is the second in a series of three surgical procedures to mitigate the effects of hypoplastic right heart syndrome.  In the most basic of terms, it involves ligating the BT shunt (which was placed during the first procedure), then connecting the superior vena cava to the right pulmonary artery.  This allows blood to passively flow (by gravity) from the head and upper limbs into the pulmonary artery where it drains to the lungs for oxygenation.  Once oxygenated, the blood is returned to the heart through the pulmonary vein where it flows through the left atrium and ventricle and is pumped through the aorta to the rest of the body.  In Josephine's case, the operation also involved closing off the two large collaterals she had developed to insure that there would not be competing blood flow.  The period between the Norwood (1st procedure) and the Glenn (2nd procedure) is considered the time of highest risk for an HRHS baby.  Up to 1/3 of kids develop complications and die during "interstage".  Making it to the Glenn is a huge accomplishment.  Life after the Glenn, we were told, is considerably more stable.  Like night and day.  I wish this were true for us so far.

According to the surgeon, Josephine did very well during her surgery on Thursday morning.  We arrived at the hospital at 5:30 a.m., were checked in by 6:30 and off to surgery by 7:30.  At around 11:30, Michael and I were called to one of the consult rooms where Dr. Forbess soon arrived to let us know Josephine's status.  Josephine was taken from the OR directly back to her room in the ICU where the nursing staff got everything situated.  X-rays and ultrasound tests confirmed her lungs and heart were looking and functioning well.  She was intubated and had two cardiac lines, one set of pacemaker wires, a foley catheter, three IVs (placed in both hands and her right foot), and an internal jugular line in her neck.

As it was explained to us, extubation generally occurs very quickly after the Glenn.  This is due to the fact that the ventilator causes increased pressure in the lungs which makes it difficult for the Glenn circuit to operate fully.  (Too much pressure means the blood cannot flow as easily from the head and upper limbs to the lungs...which means there is less oyygen making it into the blood circulating to the body.  The overall result can be decreased oxygen saturation levels.)  Most kids are extubated within the first day, and many are released to "the floor" by the second or third day post-op.  By the end of the day on Thursday, Josephine had been extubated and was doing well, save a few areas of decreased function in her lungs.  Michael and I were thrilled that things - for once it seemed - were going according to plan.

Overnight Thursday to Friday morning, Josephine worked harder and harder to breathe.  Her tiny chest rose and fell rapidly, and tugged at her tummy and diaphragm which heaved in and out with every breath.  A look at her x-ray and labs revealed increasing areas of collapse in her lungs.  Talk of re-intubation began early in the day.  After attempting to provide support via a small nasal cannula and a larger RAM cannula, the decision was made to put Josephine on CPAP.  (In case you've never seen it, CPAP looks a bit like some sort of medieval torture device.  Air is delivered through a small mask which seals around the nose...not too bad.  But in order to keep it correctly positioned, a series of velcro straps must be positioned around and across the head, with another extending under the chin to keep the mouth closed.  CPAP looks like the ugly second cousin of old fashioned orthodontic headgear.  And Josephine hated it with a passion.)  While we weren't terribly happy about the discomfort CPAP seemed to be causing, we were happy to have averted reintubation.  By the end of the day, things were turning the corner.  Josephine's lungs were starting to reinflate and her blood gas numbers improved.

On Saturday morning, Michael left the hospital at 6:20 a.m. to meet a delivery person at the house.  Several weeks ago during a particularly brief but violent thunderstorm, a power surge caused the electrical striker on our gas stove to short out.  Rather than pay half the cost of a new unit to repair the 20+ year old model that came with the house, we opted to purchase a new stovetop.  We had hoped to have it delivered and installed before Josephine's surgery, but that didn't quite happen.  Josephine and I (along with our cadre of medical staff members) started the morning optimistically.  Although she was sleeping much of the time, I read her a couple stories and attempted my version of music time (which amounted to me singing along to music sputtering in on the hospital's much-too-slow internet connection.)  Aside from the occasional squirming fit (which I assumed was brought on by discomfort in some capacity), Josephine was doing well.  Her saturation levels were running in the high 80s and low 90s.  She seemed to be breathing almost effortlessly, at a moderate pace with no abdominal pulling. Everything was good.

Around 9:00 Josephine and I received Michael's text that he was driving back to the hospital.  "All's good" I texted back, noting that the nurse and I had discovered a small amount of blistering and skin breakdown behind Josephine's ear.  (Unfortunately, she has her momma's sensitive skin.)  A short while later, Josephine woke up.  It was clear to me that she was very uncomfortable.  In addition to her normal squirming, she was arching her back and jolting her head back and forth.  With each wiggle, she inched her CPAP mask further and further off of her nose, eventually moving it all the way to the side where it pressed into her face, hissing uselessly.  I quickly repositioned the mask and continued to pat her back gently, hoping that she would settle and drift back to sleep.  The absolute last thing I wanted was for her to be awake and in pain or scared.  Although I desperately wanted to see her open her eyes and smile at me for the first time since surgery, sleep - painless and stress-free - seemed like a much better option in this situation.  When she didn't respond to the normal repertoire of mommy-interventions (patting, shushing, sleepy sheep ocean sounds, placing her paci within distance), it occurred to me that she had not had any pain medication or anything for anxiety in several hours.  I stepped out into the hallway and signaled the nurse, who had just stepped away for a moment to grab some breakfast.  By the time the nurse arrived at Josephine's room 30 seconds later, Josephine's oxygen saturation levels had started to drop.  All morning, she had been hanging out in the high 80s oxygen-wise.  And now, she was at 75 and falling.  The nurse called for a respiratory therapist, who happened to be close by.  The respiratory therapist quickly pulled the ventilator flow tube attached to the mask and began to ventilate using the hand ventilation bag. Woosh...wooosh...woosh.  Josephine was getting more and more agitated.  Woosh..woosh...woosh.  At that point, the respiratory therapist decided to suction Josephine's mouth to see if there were secretions making airflow more difficult.  She stuck the suction tube into Josephine's mouth and I could hear what sounded like small amounts of saliva being pulled into the vacuum tube.  Josephine looked horrified.  Her eyes had snapped open and her face was slowly but steadily turning from a deep red color to purple.  "Get me the deep suction tube," the respiratory therapist called calmly, but firmly.  The energy level in the room began to rise.  Grabbing another plastic-wrapped package out of the in-room supply drawers, the nurse opened the window to the adjoining room and said, "Get Dr. Moreland, please."  Several nurses and an additional RT had entered the room and were moving around with purpose.  Dr. Moreland then rushed in and began barking orders.  By this time, Josephine's saturation level had dropped below 50.  As they worked trying to re-establish her airway, I watched helplessly as she flailed around, oxygen saturation level finally landing at 19 as they pushed a paralytic through her IV and placed a new endotracheal tube.  What had been our largest success in the past 36 hours had now entirely been undone in a matter of mere minutes.  Josephine had been reintubated and was no longer breathing on her own.  I could do little more than stand in the corner shaking and crying.

Over the past 24 hours, Josephine has been struggling a little with varying levels of lung collapse.  As happened during our first stay, they are able to reinflate one side seemingly only at the expense of the other.  They've sent off cultures of the secretions pulled from her mouth and throat, and so far the results have not indicated infection is present.  Early this morning a nasal swab was taken to determine if she may have a viral lung infection.  So far, nothing.

At this point - although intubated - she seems to be resting somewhat comfortably.  Her oxygen saturation levels are in the low 90s, which is fantastic.  The plan for today is to continue respiratory therapy in an attempt to get both lungs to reinflate.  They expect that may happen by tomorrow.  Best case scenario, the doctor says, is that if everything looks perfect we may be able to extubate by Tuesday or Wednesday.  I realize it's only a matter of days, but it feels like a lifetime.

Wednesday, October 29, 2014

Figuratively Speaking

The morning of Josephine's cath exam was one filled with activity.  Josephine wasn't allowed to have formula after midnight and while we were permitted to give her Pedialyte (which surprisingly, she loved), even that had to be cut off by 4:30 a.m.  Despite being what I would imagine was ravenously hungry, Josephine was in fairly good spirits.  We dressed her in her oh-so-stylish bright pink Dancing Queen onesie paired with her 80s throwback leg warmers adorned with pink prancing ponies, and after a quick run to Starbucks for some morning "fuel", headed to the hospital.


The check-in process was mostly painless.  We spent a few minutes in the Inpatient Admitting waiting area, giggling as Josephine marveled at the ceiling tiles and overhead lights.  (This kid is fascinated by the most mundane things.  :) )  After a short time, we were called to the desk where we signed a pile of releases and were freed to go upstairs.

The unit was just as we had remembered.  Same reception desk (minus the desk nazi who guarded the entrance to the CVICU,) Same twinkle lights illuminating the waiting room.  Same worn, but moderately comfy recliners.  Same stillness punctuated every so often by a passing staff member.  We had spent far too long here during Josephine's first stay, and it began to dawn on us that our second stay was edging closer by the day.

While we were waiting for the cath team to prepare, one of Josephine's favorite nurses happened to walk by.  From the darkness of the waiting area, I waved to her.  At first I don't believe she fully recognized us...man, woman, baby...wait a second, that's Josephine!  "No freakin' way!"  she exclaimed as she pulled open the waiting room doors and bounded over to see our much bigger, much healthier-looking little girl.  After a couple quick hugs and some reveling about how much Josephine had grown since going home, nurse Kim excused herself so she could get back to her duties.


A few minutes later, a member of the cath team came out to escort us back to the cath lab, which was deep within the maze of hallways behind the ICU.  Perhaps I was in a bit of denial about how this would all go.  After all, it seemed to me that a cath wasn't surgery exactly.  Just a procedure.  Take a little blood. Snap a few pictures.  And we're out.  The paperwork, however, made it pretty clear this was a serious medical procedure that came with a very specific set of risks.  Michael and I signed each consent, then met briefly with the anesthesiologist and cath doctor - Dr. Nugent, one of our favorites from Josephine's first stay.  We cuddled and kissed our sweet girl, then passed her off to the nurse to be taken back to the OR.

Back to the waiting room we went.  While I'd like to say I was able to get a few minutes rest, that simply wasn't meant to be.  After about an hour, we were advised that Josephine had been put to sleep and was doing well.  Dr. Nugent was just about to begin.  as we waited, other parents with other kids came and went.  A dad snoozed quietly in the corner recliner.  A mom nearby watched Let's Make a Deal.  We sat, glancing occasionally at the T.V., surfing the internet or catching up on Facebook posts we'd missed over the past several days.  Finally, Dr. Nugent appeared at the doors to the waiting room.  "Let's go to the consult room," he said, quickly reversing his suggestion when he noticed that the entire room, save Michael and I, had cleared.  I had confidence in Dr. Nugent...I trusted him.  But in the back of my mind I harbored some fear about what he was about to tell us.  "Well," he began, "I don't want to call it unique or bizarre...but it's bizarre."  (Words, I can assure you, are not necessarily the ones you want to hear your child's doctor use to describe her condition.)  He then pulled out pictures taken during the procedure which showed that Josephine's shunt had visibly narrowed on one end.  (We had learned during Josephine's first stay that full occlusion of the shunt was considered a catastrophic event.  Game over.)  Yet Josephine was fine. Her stats had been in the 80s.  Her color was a soft pink.  Her pulses were strong.  She was bright and alert and gaining weight.  We were even well on our way to removing the feeding tube.

As it turns out, Josephine's body had spontaneously developed two
collateral vessels leading off of her heart.  During the first surgery, the doctors had implanted an artificial shunt to keep blood flowing to her lungs for oxygenation.  Somehow, in the weeks since leaving the hospital, her body had managed to grow two more shunt-like structures.  Dr. Nugent punctuated the gravity of the situation by finally commenting, "Without these two collaterals, she would have been as blue as a squid a month ago."

*   *   *
Josephine has been very stable, very safe and very active over the past two weeks.  We're also pleased to say that she is now ng tube-free!  Dr. Ikemba, her cardiologist, gave us the O.K. to attempt exclusive bottle-feeding the next time she pulled out her tube.  True to form, Josephine had that task tackled in about a week's time.

Tomorrow, we're scheduled to take Josephine in for her second surgery, known as the Glenn.  Essentially, this will be the true game changer during which they remove the shunt and reroute her existing plumbing so that blood from her upper body passively flows down to her lungs where it will be oxygenated and sent back to the heart and pumped to her body by her one good ventricle.  Although it is still major open heart surgery, they tell us recovery time is typically significantly shorter than that of the first procedure.  We're hoping an praying that's true.