She Calls This Pose "Blue Steel"

 It's hard to believe we've been in IMU just over a week now. In addition to making major progress in her breathing, gaining a respectable amount of weight and managing to fight off any further infections, Josephine has been working on her model poses.  (Notice that she favors the sassy, Olan Mills, hand-below-the-chin look, which she has been perfecting since her sonogram days.) 

Besides spending a great deal of quality time snuggled up relaxing with either Mommy or Daddy, Josephine has continued with various therapies aimed at helping her recover the developmental time lost while she was intubated and unable to move.  Despite the fact that she has slept through every last session of her speech therapy (which isn't aimed at helping her speak, but rather at assisting her in mastering the ability to swallow effectively in spite of some ongoing vocal chord paralysis), she amazed the speech therapist today by taking a bottle and successfully drinking a little under an ounce of milk.  (She would have taken more, but the speech therapist wanted to wait and make sure she's not accidentally aspirating what she swallows.)  The long and short of it is that Josephine is a total champ when it comes to eating!  (Like mother, like daughter I suppose. :)  )

We're now waiting to get the go-ahead for Josephine to move to "the floor".  (It was pointed out to me that when taken literally, "the floor" seems awfully cruel.  In our situation, it refers to the 8th floor of the hospital where kids go just before they are sent home.)  Michael and I have been completing our education requirements - learning to give medications, place a feeding tube, work a feeding pump and administer CPR - so once we make it to the floor, we should only need to complete the room-in process before Josephine is discharged.  Rumor has it we may be moving upstairs within the next week and may be able to do our room-in a by next weekend.  We're getting so close!

 

Channeling the Jeffersons

Like George and Weezy Jefferson, we're moving on up...to the IMU! :)

Though I wasn't able to be there today to witness Josephine's triumphant transfer to the IMU (as I am feeling a bit ill and didn't want to risk passing anything to her), she has finally done it!  Over the week she made slow but steady progress toward her goals: gaining weight, fighting off infection and improving her respiratory status.  Early in the week we learned that the urine cultures they took over the weekend showed a small amount of Enterobacter.  (Apparently, this has been a problem among a few of the patients in the CICU.  Since the Infectious Diseases team at CMC is trying to determine the origin of the infections and wanted to prevent further spread, anyone who enters Josephine's room must now wear a yellow isolation gown.  While yellow isn't really my best color, I'll gladly do it to be able to spend the time with our sweet girl.)

Josephine's respiration rate has also steadily improved.  On Tuesday, she was transitioned from the RAM cannula to the extremely loud, but highly effective Vapotherm.  (Truly, at the highest level of support this thing sounded like a jet plane blowing up her nose.  Her reactions - blowing massive spit bubbles aided by the ventilator air and closing her mouth until the flow puffed out her cheeks hamster-style - were incredibly cute to see.)  She is currently on a support level of 5 which is one step above where she needs to be to transition off entirely.

Her most notable accomplishment however has been her weight gain, which was the final piece needed to get approval to move to the IMU.

On a fun note, Josephine transitioned to a big girl bed (she's very long and was outgrowing the baby bed) and got her first experiences in her bouncy seat this week.  Despite her yawn in the picture above, she enjoyed it immensely.  :)

By far, her favorite activity at this point is simply resting in our arms.  The Attending Physician this week told me I might be spoiling her by holding her so much, but I don't mind.  She and I have nearly six weeks worth of cuddling to catch up on...and I intend to reclaim every minute of it.

Shall We Dance?

 Despite the fact Josephine has been in CICU for almost 7 weeks now, Michael and I still haven't quite become accustomed to the intricacies of the daily dance, a precarious movement from illness to wellness that twists and turns in ways we never would have expected.

With Monday's extubation, Josephine took a huge step forward in her progress.  We spent Tuesday, Wednesday and Thursday in a state of near constant excitement.  News of Josephine's impending move to Step-down spread among the doctors, nurses, therapists, social workers and clergy at the hospital, many of whom stopped by to see Josephine and congratulate us on what finally seemed to be providence smiling in our direction.  Our course was becoming clearer:  Josephine needed only to show consistent weight gain and we would be the next family to take the triumphant walk down the hallway to IMU.  (As silly as it sounds, I've played this event over and over in my head since our first week at CMC: Josephine being wheeled down the hallway with Michael and I behind pulling a squeaky red hospital wagon filled with our belongings as we wave pageant-style to all of the nurses.  Obviously, moving to the IMU is not the same as being discharged all together, but the change in geographic location represents actual physical movement toward our ultimate goal.)

Once again, we've been assigned the more scenic route...

On Friday afternoon, Josephine's breathing became tachypnic, rising from her normal rate of 40 - 60 breaths per minute to 80...then 90...and finally, almost 120.  Her temperature, which had been hovering around 99 degrees for most of the day also began to climb.  By the time the Nurse Practitioner on duty was called, Josephine had entered fever territory, topping out at over 101 (not awful for a baby, but potentially disastrous for a heart baby.  Once an infant with a BT shunt becomes febrile, they run the risk of developing a dangerous tachycardia and dropping their oxygen saturation rates.  Definitely not something that would be good at this point.)  After looking at Josephine's labs from earlier in the day, the NP decided the best course of action would be to give Josephine some Tylenol and a dose of bicarbonate to remedy a bit of a metabolic acidosis.  With the administration of the bicarb, Josephine's respiration quickly returned to a more normal range and her temperature began to fall.  Michael and I went to sleep last night feeling as though things were more or less O.K.

Overnight, Josephine again developed a bit of a fever.  Her white blood cell count became elevated and her CRP (C-Reactive Protein, a marker of inflammation) also increased.  To be safe, the Attending Physician decided to run a series of blood tests to rule out infection.  (Since infection control is something taken very seriously in the critical care environment, Michael and I were made to wear masks and special gowns while Josephine's nasal swab was being processed.  Luckily, the RSV and flu tests were negative so we didn't have to wear the sterile gear for long.  During our time in CICU, we've seen a number of other families wearing these outfits...never for a minute thought it would be us.)

At this point, we are still waiting for the results of Josephine's blood cultures..and we're praying everything comes back negative for infection.  Through all of this, Josephine has looked remarkably well. (This, as evidenced by the rather animated photos below.  Note that yawning for Josephine isn't just a subtle facial movement, it's a full-body experience. :))  Her stats are all holding strong, and despite being very sleepy today after a dose of anti-anxiety medication early this morning, everything has seemed more or less normal in terms of her appearance and behavior.  (In short, she's not really acting like a sick baby...hoping that means she's not!)

We appreciate all of your prayers, but are requesting a few more toward the recovery - and specifically, forward movement without the burden of infection - for our sweet little girl.

 

 

A Breath of Fresh Air

Notice anything different?  :)

We're pleased to introduce you to the ET tube, cardiac line and pacer wire free Josephine Elora Sanford.  (Almost looks like a completely different baby, doesn't she?)

After receiving the go-ahead from the Nurse Practitioner on duty, Josephine and Mommy had a great time playing dress-up today.  Josephine can be seen below in a fashionable mint green romper adorned with a festive butterfly applique (notice too how very well the romper matches the glowy pulse oximeter on her wrist and the tubing for the new RAM cannula, positioned just under her nose.)  Although we couldn't fit headbands around the new tubes and wires, we made due by clipping her hair accessory to the Near wire.  The result is a look we like to call ICU chic.  Is it just me, or does this little diva know she's looking good?

Next order of business for Miss Josephine is to learn to tolerate feeds and gain weight and strength.  Having mastered the art of binky use in only one night, she's well on her way.  Step-down Unit, look out...Josephine is headed your way soon!

 

  

 

On Sundays We Wear Pink

Josephine had a good day today.  Following her bath (which daddy facilitated), Josephine began breathing exercises in preparation for her ERT (extubation readiness test) tomorrow.  Whether she was worn out from squirming during her bath or simply behaving in typical baby fashion, Josephine essentially slept right through the exercises and passed with flying colors.  During rounds, the Attending Physician decided that Josephine was ready for lower ventilatory support, and gave the O.K. to reduce her settings and begin a series of workouts at the same level as the ERT.  The two-hour workout done this afternoon was no challenge for our little fighter, and if she's able to pass two additional sessions this evening they may consider extubation tomorrow. (Hooray!)

True to form, Josephine also decided she was pretty much finished with her two remaining cardiac lines today.  (These are the lines placed in her heart during surgery that allow the nursing staff access to both administer medications and draw blood.)  Line number two stopped drawing yesterday and began leaking, so the nurse capped it off.  I think Josephine saw this as her opportunity to finally be free from this particular burden.  At some point this afternoon, the stitch holding cardiac line number one came loose and the tube slipped out entirely.  So, one line is out and the other (since it's not functional) will be removed tomorrow along with the pacer wires which are also loose and in danger of simply falling out. 

On a lighter note, today was pink day in CICU room 204.  Josephine was swaddled in her new bright pink giraffe blanket, and sported a pink and white bow and matching bright pink Mary Jane booties.  Dolly and Pony were also right in style, and even helped Josephine manage the bootie that she was unable to wear on her foot due to a new IV line.  Despite the fact that Panda wasn't wearing pink, the girls (Josephine, Dolly and Pony) were very accepting and allowed him to stick around and hold the ventilator tube from time to time.  No Mean Girls here. :p

 I've never been a big fan of pink...but my goodness, this little diva really makes it look good.  Looking forward to a wonderful (and maybe tube-free) day tomorrow.  :)

 

 

Independence Day

We had hoped that on the day celebrating the country's independence we would also be celebrating Josephine's release from the tangle of hoses and tubes that have been so persistently present over the past many weeks.  Unfortunately, there was once again a different path that we were intended to follow.

The week began in a mostly positive way.  Josephine's creatinin level (which provides an indication of kidney function) had fallen to a personal best.  Her lungs, while still a bit hazy on x-rays, continued to do well, and although her ET tube culture showed signs of bacterial colonization (Staphylococcus Aureus, not MRSA thank goodness), she showed no signs of infection.  All was good until mid-week.  On Wednesday - one day prior to our anticipated date of extubation - Josephine began showing a marked increase in agitation.  Our normally sweet little girl turned into a head whipping, body thrashing, out-of-control stranger.  From seemingly peaceful slumber, she would wake for no apparent reason, scrunch her face in agony and writhe in pain.  By afternoon, it was obvious her episodes were more than just typical baby fits.  After meeting with the doctors and discussing her changes in behavior as compared to changes in treatment, it was hypothesized that a new oil being added to her formula was causing gastro-intestinal distress.  In short, she had a massive case of extremely painful (and very noisy) gas.  Over the next day, they discontinued her feeds and transitioned back to the old formula, sans MCT oil.  Although it took a day for the oil to move out of her system, Josephine slowly but surely began to have fewer fits.  

Breathing trials started back up on Thursday evening, however, because Josephine had expended so much energy dealing with her tummy troubles, she was noticeably more tired and weak.  As a result, the trials didn't go as well as hoped and extubation was postponed...again.  

As I type, we're about 1 hour in on another breathing trial and Josephine is doing well.  She has two additional trials scheduled for this evening, and if all goes well will transition to a lower level of support for at least four additional trials tomorrow.  Depending on how she does, later this week we may be able to hear her cry for the first time since surgery - nearly 40 days ago. 

 Is it wrong for a mother to want so badly to hear her child cry?  Because I do.  I want to hear her cry and see her face without a gigantic mass of tape and finally be able to hold her in my arms to comfort her through this journey.  

Tomorrow is another day...hoping it brings us a step closer to Josephine's independence day.