It has been a month since I have blogged and I need to get you up to speed.
I've tried, in vain, to get more sleep this past month; which meant foregoing late night blogging. A whole lotta good that did me! I haven't blogged AND I have pretty under-eye circles. Glamorous.
The last time I checked in with you medically, Piper was undergoing a tummy procedure to correct a feeding problem. That procedure with Dr. Iqbal went smoothly, and Piper is now successfully being fed through her new GJ (gastric - jejunum) feeding tube. This tube goes the extra extra step in protecting her lungs from baby puke/aspirating, by taking her food straight into her small intestine, bypassing the stomach all together.
Lungs? Home? When? How much longer? - My word, these questions plague just about every conversation we have outside of the hospital, and I will do my best to clarify our home course shortly.
SCIENCE LESSON #1 - What is persistent pulmonary hypertension (PPHN) & what does this mean for Piper?
Infants with CDH also commonly have a very serious and potentially life-threatening problem called pulmonary hypertension. Pulmonary hypertension happens because the blood vessels in the lungs are not formed properly. In babies with CDH, the blood vessels often fail to relax normally. In addition, babies with CDH have an inadequate number of blood vessels to carry blood into the lungs. Therefore, the lungs receive inadequate blood flow which restricts the amount of oxygen delivered to the rest of the body. Further, because the heart has to work extra hard to pump blood through the tiny blood vessels in the lungs, it can eventually begin to fatigue.
PPHN can be treated in the NICU with multiple ways to help alleviate these pressures:
- Proper Ventilation (TRACH in Piper's case)
- Nitric Oxide gas
- TIME for lung disease to heal = PPHN alleviating itself
SCIENCE LESSON #2 - Pulmonary Hypertension can come into crisis when exacerbated!
On Piper's slow road to healing and home, we have ran into a couple big set backs that scared the living poo out of us! Piper getting sick in November, and again in January is enough to throw her tiny body back into a life and death fight.
ANY little stress stimuli can exacerbate PPHN and shut Piper's body down for good. Game over, baby too sick, body too tired, done. Scary situation after fighting so hard for 6 months, right?
Things like a tummy bug, respiratory virus, aspirating pneumonia, teething, & surgery can trigger PPHN crisis, and we are constantly on high alert for this stimuli.
We are so so so fortunate to have awesome nurses and docs that are QUICK to recognize Piper's signs of stress and treat her symptoms before her body becomes too tired. PPHN is always in the background on this journey towards home...and TIME is the only treatment that will eventually take this scary term out of Piper's equation.
WHEW! Did ya get all that? You now have your honorary doctorate from reading all that mumbo jumbo! Hopefully that answers the question of, if Piper seems completely healthy and doing well, why our team is extra careful and slow with changing things that could stimulate crisis.
That brings me to LIFE LESSONS.
Matt and I have been so proud of Piper's progress and, knowing the medical steps to get to home, can't help but get a bit antsy as we near the final stages of hospitalization. After months of improvement, and positive changes, Piper got a bit sick/out of sorts around mid-January with PPHN crisis. This THREW ME for a loop, and I was taken aback by my emotion.
I realized in one NICU visit, that no matter how great things are going, we are NOT out of the woods. I had let my mommy emotional guard down, I had buckled into trach care training and home going logistical talk, and then was slapped in the face by this (in the scope of things) minor setback. I lost it. God reminds me that we are on his time, Piper's time...BE STILL, woman!
I look back to July 2014, at my words of preparation for birthing and losing Piper. We were as emotionally prepared as we could be, and told ourselves, every minute - every hour - every day that Piper lived was a gift from God to cherish Piper before she died.
Along the way...Piper lived. Piper thrived. Piper became ours. The time we said we we'd feel blessed by, turned into months, and we began to believe she would live. She was ours. Ours to take for granted. To selfishly be frustrated at timing was taking Piper's astonishing fight for life for granted.
January's setback reminded me of my earlier preparations, and why impatience is something I cannot afford to grovel with. Frustration at our pace of progress should not be tolerated. I was prepared to lose this child....and I didn't. I have her. She will live. I will be frickin thankful for these woods! (Cue that Taylor Swift song!)
That being said, we have had a great February with Miss Piper. She has weaned on her trach vent settings and pain meds, resumed feeds after being sick, and her echos have showed great improvement in her pulmonary pressures! If you'd like to see some more medical speak, here's what we are thinking for Piper's coming home to-do list:
- Reach full feed amount
- Get OFF Nitric Oxide (NO) (for my nurse pals: she's on 0.5ppm! Come on, Pipes! Give it up!)
- Once NO is OFF, she will do about 1 month of home vent practice before discharge
- Wean narcotics to home manageable state
That's it. Seems like a small list, right? Only Piper can set her pace on completion of these tasks, and we will gladly BE STILL and wait. Silly goose like to keep us guessing!
Along the way, plastics might decide when they're willing to do her cleft lip repair...AND cardio may decide she's stable enough to patch her heart holes. Geeze - I'll save those for another blog.
We are happy to report that Piper is the happiest girl in the NICU, smiles at everyone, and is the D Pod Hall monitor as she sits in her swing and watches everyone walk by. She has a keen fashion sense, likes the one shoulder look, and is a whopping 20.2 lbs! She has been doing great sitting up with PT, grabbing and tracking with OT, and singing (let's face it she's an alto) with Music Therapy! Have I mentioned how awesome Children's Mercy is? Awesome!
PRAYER THIS WEEK - Get that darn Nitric Oxide weaned OFF!!!!! That would be a huge step, and is the only thing keeping Piper from switching to the vent she'll come home on.
We love you all, and are so glad to still have your support and prayers. Y'all are our proverbial "flashlights" for this long journey through the woods. What brightness each of you bring. We are so grateful.