Jett Matthew - Home

* Trigger Warning: This blog will contain horrible language (duh), and extremely raw and real discussion of death and dying. If you need to read Jett's original diagnosis blog to understand where we are coming from, CLICK HERE.



Upon Jett's entrance into this world, Matt and I knew we were waiting for ONE question to be answered moving forward. This answer would determine our plan, our course of action, our time.
Would Jett's tiny pulmonary arteries be as bad as assumed? Are they able to be helped surgically?

YES = surgery, lots of wait and see, a big huge MAYBE on survival of said surgery, and then months of hypoplastic left heart surgeries to come.

NO = NO. No help can be offered surgically. His arteries are too small, will always be too small, his body will live with current stability for a while, but his heart structures will cause his eventual demise. Hospice care.

We got our answer from the heart team 3 days into our NICU stay..... NO.
We were prepared for NO. Actually, we would have been shocked otherwise. We knew we would have limited time, we knew this was God and Jett's ballgame now. Love. Snuggle. What now?

If you follow our social media, you saw how great Jett was at breathing on his own in the NICU.  This is when we choose our miracle. Jett's little body created vessels most babies don't have. These helper vessels try their best at balancing blood flow to the body with the crappy plumbing of a heart he was given. These vessels are literally TIME...but they expire. We knew that going in. Helper vessels may make us stable enough for home...so we quietly geared up. What a miracle that a tiny baby body, in my tummy, tried his best to create its own vessels...for TIME with us.

Once we received word of our NO from cardio, Matt and I set out to accomplish "normal baby milestones" with Jett. Room air breathing with no oxygen help, full feeds via OG tube, parent training on said OG tube. These were the HOME parameters.  Quietly we cherished the snuggles, hoped for the best, and then snuck the mister home - June 14th - after just 10 days in the NICU.

So - HOME doesn't mean it's sunshine and rainbows. Home means hospice would follow Jett, we would check in with cardio to see how his ticker was progressing (or digressing), and eventually suffer the same loss we've done before with Piper - a baby who was too blue, not enough oxygen and blood flow to sustain life, we would have to say goodbye. Months? A year? Our literal deja vu nightmare....to have extended time, and still lose. We knew this. We had to come to terms with this. We choose to love. This fucking sucks; but let's love anyway. It's ALL we can do. I'm terrified to watch for the warning signs, I'm in a panic that we have to do this again, I'm trying SO SO hard to be present and grateful BUT I AM SO AFRAID of the pain I know is coming.

June 16th, 2019 - Jett gets his front door debut with Daddy on Father's Day! It is 3pm here...and life would fucking kick us in the face by 6pm. 

After brunch and afternoon snuggles with the family, and the house quieted from visitors, Mimi and I headed off to the Target to grab some baby supplies, then to grab take out dinner. 

Matt, Tallan and Jett stayed home to start a feed. 

I arrived home to lights, sirens, police cars, ambulance, fire truck, people. Everywhere. Panic. 

Running in the front door, I am only allowed to our stairway before I'm stopped by the chief medic. Matt is feet behind him at the kitchen island with firefighters, and is crying, looking at me and shaking his head. Jett is lying on the counter. We are done. 

Not able to get to my husband, all I can remember is screaming for Matty, and clawing at this poor man's shirt, as he explains Jett's heart has zero. Zero electrical activity. 

While we were gone on errands, actually in the last 10min of our drive home, Matt noticed something wrong, grabbed Jett to wake him with a diaper change, discovered no breathing and called 911. 

Matt Jarvis, alone with his limp newborn and 7yr old, called for help, began CPR and directed Tallan to open the door for first responders. He fucking had to do CPR. Alone. Terrified. 

My worst nightmare. Our worst nightmare. 

Our preparation for grief, quickly turned to processing trauma. 

Do you know what happens when a child dies at home? Police stay to take pictures like it's a crime scene. We were confined to our front office and couldn't go near the kitchen, where Jett's body lay. We had to vomit out every fathomable medical term we knew about Jett to explain his reasons for hospice care at home and for his medical condition causing this demise. Police stay until the Doc in charge of Jett contacts them to verify this condition. Matt and I are questioned about his diagnosis and the events of the day...separately. This is so fucked up. That's all I could say as I waited. It's all my brain would think. This. Is. So. Fucked. Up. 

The above scenario is protocol, and I am so thankful we had discussed this with with our palliative care team while we were still in the hospital. Once doctors are contacted, things ease up; but damn what a trip it is until then. These poor young cops are pacing, deer in headlights, tears in eyes, mortified. I realize "We're those people. We are their nightmare call. Baby. Dead. We are those people."

Surrounded by family and our palliative care team, who came running, we ended our night able to hold Jett Mathew one more time. He left the hospital with a crappy heart; and it was way more tired than we imagined. It stopped. Too tired to compensate for shitty plumbing. Stopped. 

Last night was a movie I felt I watched from above. I've never felt visceral pain like this. Matt was alone and had to do the unthinkable and I am sick about it. I am scared to death of what this does to him, to Tallan. I replay over and over myself screaming and clawing for him. I just needed him, I needed him to know it was ok. There was nothing we could do, nothing. I'm so so sorry for what he's experienced. I am sorry we have to do this again. We held our baby last night and said goodbye; and the fact that we've been here before is so fucked up. So unfair. So painful that there are NO words to describe it further. PAIN. 

After such trauma as last night, comes the silence. What now? 

We prepared for this, but not for the acuteness of how it happened. We are in shock. We've held one another up, that's all we know to do. We thanked God for the family surrounding us and for the small amount of time Jett spent with us. We thank God for the ability to remain upright at the moment. We will sit with this pain for a while, and let it be our reminder that there was love. We will remind each other we are enough.  We are enough. 

Jett Matthew Jarvis  - I'm so sorry this happened. That your heart never gave your body a chance. That we had to spend so little time with you. I may never know why our family has had to endure such loss; but I remain so grateful for you. I'm so grateful for our time spent loving you. We are in pain, but trust you are not, and for that I am thankful. You are HOME. 

We are not alone in our pain. We are in no need for divine answers for "why?" All we needed was love...and that was given. We are tired and numb and our hearts ache but we have LOVE that endures. Please, God, use this for good. We are so grateful for each other. That's all I know to say. 

Deja Vu

Ugh. Y'all.
I have no idea where I begin, but want you to know my heart has been set on writing this blog for a while now. I shall start from the beginning; and beg for your grace with my language, because frankly, I don't feel like censoring myself, my thoughts, my feelings, OR my F-words tonight.

The Timeline

Matt and I were quietly excited to be ready, trying, then pregnant this fall! After a couple years of grief counseling, a move, another move, we were overjoyed about adding one more to our family. About closure. About coming full circle and having life resume after we froze for a bit with Piper's death.

In November,
I answered a phone call from a familiar number, Children's Mercy Hospital here in KC.
* For back story - Matt, Piper and I had our entire genomes coded when Piper was born, to test, at the highest level CMH Genome Center, if we had any matching mutations that could explain Piper's condition. NO matches for any of us were found in 2014. Along the road, when narrating Piper's story to new friends, we do mention that any random day, on any random year, at any random age, we could get a call that the genome team had plugged in a new gene finding, and have a mutation explain it all. One day... far away.... we could get a call.
I was not prepared to receive that call so soon, this Fall, this November 2018.*

The person on the phone was our genetic counselor, who explained that a new gene mutation has been discovered, a double mutation of the HYAL2 gene, that they believe was the cause of Piper's birth defects. A homozygous mutation (meaning a gene copy from mom & a copy from dad) causes the pathological results of:
      * Cleft lip & palate
      * The VAST array of any heart defects
(Piper's Diaphragmatic Hernia defect is assumed to belong in these findings, and will be added when more and more cases are discovered.)

At the end of this call, I had to disclose that I was currently 10 weeks pregnant with a new baby, and that this call was changing everything, triggering PTSD, causing alarm, wreaking havoc on what I thought was about to be a rainbow pregnancy. This call frickin' sucked. What did we do? What will happen? What the hell do we do now?!!!!!!

A week later, I began the journey of the shittiest deja vu you can imagine.
With a new gene to target, I immediately went in for a CVS (Chorionic Villus Sampling) to gather genetic material from baby, and target test for this specific gene mutation on HYAL2. Matt and I, shocked into silence and despair in this last week, completed this procedure, then hopped on a plane to Disney. JOY.

In December, 
 While striving for a fun family week at the happiest place on Earth, Matt and I awaited the phone call from the genome team. Three scenarios were in front of us.
     * NO mutation of HYAL2 = all good, nothing wrong, breathe.
     * ONE mutation of HYAL2 = baby will be a carrier of this mutation but show no symptoms
     * DOUBLE mutation of HYAL2 = we will experience a pathological result of any defects I listed
        above: cleft lip, cleft palate, heart defects, diaphragmatic hernia?

Mid-vacation, we received our call. Good news? Baby had a Y chromosome and we now know it's Jett Matthew in my belly!
Bad? Double mutation of Jett's HYAL2 gene means I am no longer in ignorant pregnant bliss. I am now in hell. The same hell I've lived before. The hell that caused so much trauma that my body viscerally reacts when I think about going through it again. Double mutation. Fuck.
At this point we knew Jett would experience any number of birth defects; but we wouldn't know which until we got home, switched all care to Children's Mercy, and scanned for what we could find.

NOVEMBER. DECEMBER. - We have been in agony and fear and dismay since this Fall and haven't been able to share. It has been MONTHS of congratulations, phone calls, cute pregnancy questions, facebook bump comments, shower questions that we have been fielding in silent fucking misery. Statements like "ohhhh, your family is so deserving of this after what you went through!" or "Jett will come and heal your hearts!" have been met with a smile and a "thank you;" but received in such a dark place in my brain that it's been torture. Do I argue with you? Do I dare burst the bubble of what people HOPE AND PRAYED for our family, and tell them I'm actually living in terror? Do I tell the truth? Do I mention that all this has done is make me feel like the biggest fucking disappointment and failure? Do I ruin your day by sharing that THIS, this deja vu, is Matt and my daily nightmare?
This has been the struggle. Pushing through the darkness and despair of disappointment, while maintaining a happy face for our daughter, for our friends, while we gathered more information.

Since January, 
All my OB monthly scans have been at Children's Mercy.
Our first scan at 18 weeks immediately showed this little face, that was a spitting image of a gal I knew, Piper Grace. God and Piper must've known we'd smile, and not be phased by the discovery of the same little cleft lip & palate for Mr. Jett. What some parents find devastating about a facial defect, we chuckled, knew this was coming, and had decided we'd know who sent Jett, when he showed his gappy clefty grin on camera.
Cleft lip and palate... check. Pathological result #1 of gene mutation.

OUR JOY, albeit small and comical to our family, is that on this scan day, we saw a completely formed and functional diaphragm. 2 big functioning lungs. A belly where it was supposed to be.
This was my reminder. This isn't Piper's story all over again. It feels the same, some things may be the same, but to treat this journey as it's own. Deja vu with fear and pain and anger and baby defects, but damnnnnnnnn, boy has a diaphragm!

We would have to wait a few weeks to Echo Jett's heart to see what we would be dealing with. We knew every case found with this mutation WILL have a defect...it's just a crap shoot on which one(s).
Again, like with Piper's first blog, we started with fixable problems; and ran into some road blocks and deal breakers along the way.

Jett's fetal ECHO stomped on our hearts, and showed a multitude of problems. An explanation for my medical and nurse friends below:
     * Hypoplastic (too small) left ventricle
     * Mitrovalve blocked and not functioning
     * VSD (hole between lower chambers)
     * ASD (hole between upper chambers)
     * Tricuspid valve leaky (but helping with flow)
     * Aorta is good and helping with flow
     * Collaterals present (extra vessels created by body to help with bad heart plumbing)
     * Pulmonary Atresia. (Deal breaker. Teeny Tiny Pulmonary arteries not able to help with blood
        flow to the lungs.)

If you're still with me, basically Jett has hypoplastic left heart syndrome (HLHS), PLUS the additional Pulmonary Atresia.

HLHS is fixable. (If you've followed us since our time filming the Inside Pediatrics docu-series for Children's Mercy, you'll remember baby Winston's story with this heart condition.) Repair involves multiple surgeries from birth to toddlerdom, to revise plumbing of the heart. These repairs are serious, always life threatening, but this is fixable.

BUT. (Can I stop here and just say I'm sick of MY story always having a "BUT!")
Pulmonary Atresia is our deal breaker. Our game ender. Our ultimate trauma deja vu.
Jett has wayyyy too small pulmonary arteries. These arteries are what take deoxygenated blood to the lungs so he can breathe, so he can stabilize enough at birth and LIVE through any heart repair surgery. These pulmonary arteries MUST be normal, girthy and healthy to even begin discussing heart plumbing repairs.
Jett's are tiny. They can't be tiny in this life scenario, but dammit they are tiny and they are THE reason we are unsure about his life on the outside.

So. Here we are again. We have gathered all the information, and are left with the probability that life will not be long once Jett is born. There is just too much stacked against his little heart.
We have to do this again. We have to plan for this again. We have to prepare our family for this AGAIN. Except this time, we know how painful this is. We are not ignorant to infant demise. We are not ignorant to the strain of NICU time. We are not ignorant to the trauma and agony that comes with knowing WE TRIED SO HARD, ONLY TO FAIL. AGAIN.

What will be different - is that the nursing and clinician veil that once was apart of our journey with Piper, is no longer there. We know this hospital, we know these people, we have walked these halls.
From the moment we learned of the direness of Jett's condition, we literally have had CMH love from every possible angle. People who know, who knew Piper, who choose to not be afraid of our story and RUN to us in love and support, have been my saving grace during this silent journey. Girls who will choose to RUN to our birth, even though it may end in heartbreak, girls who will RUN to care for Jett if he's stable enough to spend any NICU time...These staffers have held my shattered heart these last few months, and I am so grateful.
When I ended that November phone call I was pissed. Please God, don't make this why we moved back! I don't want to be back so we can have our hospital, our people. Please don't make that the reason we're here.
NOW - I am so so so thankful. We are here, surrounded by Piper's people, who love on anyone Jarvis and will accept Jett Matthew with joy and hope for however long that may be. God placed us among these people, because they heal more than babies. Their love heals my heart. (MUSH ALERT!)


Whew. Now ya know.
Damn. That's a lot to spill, and I thank you for allowing me to spill it. Thank you for your grace.

Currently, 
Our family is in preparation mode as best as we can be. All grandparents, aunts and uncles have been informed and we will all await Jett's early June arrival. My layperson, although NICU-wisened brain, is preparing for the few scenarios we may meet when Jett is born. Here's what I know:
   
     * We plan to intubate and assess to give little man ample opportunity to tell us what he needs.
        We have not given up enough to just birth, swaddle, say bye. NO - he will have a chance to
        show us all what life on the outside may mean for his body.

     * Jett may not stabilize well. His pulmonary arteries may be so minuscule that life on the outside
        and remaining stable is not possible.

     * Jett may stabilize enough to be moved to the NICU. To live as long as those pulmonary arteries
        allow. A surgeon may asses his situation here, and determine the ability to enter any repair
        surgery, or if these arteries are just too small to sustain life.
        Only God and Jett know what his body can do and handle on the outside. We will just wait and
        see. If Piper Grace taught us anything, it was to prepare our hearts for the task at hand, knowing losing a baby may be in that preparation; BUT (ohhh, kind of a good "But") never assume we know the time frame of this little life. She showed us 10 months of love we didn't know we'd have. I will not just assume Jett will die immediately. We are prepared for that, absolutely, but I know Jett's time here is not in my hands in the slightest. Hope & Preparation for despair is a tricky thing to try to explain, but I can assure you your heart has room for both.

Emotionally, I have run the gamut during these last few months.
Full disclosure, there were some dark dark thoughts. Why? Why did I have to meet this Matt Jarvis if the end result of our love would only bring heartache to all involved? Why? Why did we need to continue our family? Why does NOTHING in regards to family and babies work out for us and bring pain? Why do we just bring everyone pain?
Then, in the midst of the fear and the dark, God tapped me on the shoulder.
He has reminded me to look back. Things went perfectly. We created all things good and perfect when we were ignorantly not paying attention! Tallan Hope Jarvis, in the midst of all this pain, is the light He gave to us. SHE was what worked out. SHE is why we had babies. If the worst of the worst happens I know, Matt knows, SHE - Tallan Hope, IS ENOUGH! Thank you, God, for this gift you gave before I knew how precious she'd be to our family. We three are enough.

Most days, as the lady carrying the baby, I deal with the brunt of the reminders of what will happen when this is all over. I think the thoughts, I feel the feelings, I say the things on my brain to the only dude (poor guy) who knows what it's really like to go through this hell twice. Matthew Jarvis didn't know this would be our journey 18 years ago. He is my quiet agreer to my thoughts, my literal pillar of support when my despair causes such panic that all he can do is physically hold me up while I cry. I am so sorry. I am so sorry he has to do this again. I want to grab his face and apologize every day, that he has to endure this again. It sucks, but Gahhhhhh I would be lost without his strength.

We are slowly coming into the time frame we are comfortable with telling Tallan. She is fully aware of cleft lip & palate; but has not been briefed about Jett's life prognosis. We are finding a window between her carrying a burden for too long, and also being prepared for what birthing him means to our family. We are determined to not allow her entire childhood be reminders of this frickin trauma. Our goal is essentially to explain that something keeps going wrong when we have babies, that we don't know why that keeps going wrong, BUT (good "but" again!) that SHE is what has gone right. We will not harp on what we are losing but constantly remind her of how perfectly and wonderfully she was made by God for our family. She has the perfect example in her sister, that lifespan is not determined by us, that years on Earth doesn't make life more meaningful. She will spend her childhood knowing SHE IS ENOUGH and that good has come from our losses as a family. We are worried about this grief for her, but know what resilience she has shown and will show moving forward. She will move mountains, my friends.

If you are left speechless, pissed, angry, confused about why the hell this would happen again - welcome to our world. We have experienced every emotion listed. We go through bouts of complete and utter anger of the unfairness; only to be followed by the "thank you's" that allow the light back in. Thank you, God, for Matt Jarvis. Thank you for allowing us to be near our hospital. Thank you for allowing me to share, even in the dark times, what my heart is feeling. Thank you for Tallan Hope. Thank you for Jett Matthew, and for the advancements, even in this year, our two babies have helped make in the genetic knowledge of the medical community. Thank you for my health. Thank you for allowing me to carry Jett to term. Thank you for giving me the knowledge that I can get through this, because I've been here before, and survived.

I read an article (desiringgod.org) detailing Psalm 91 recently that cut me to the core, and I will leave you with those thoughts. For the people who wonder "why?," or when I ask "why!?,"

"God doesn't pledge to keep us from all worldly suffering and trouble, but he does promise to be with us, to rescue us in his perfect timing, and graciously honor us for walking the path of pain with a heart of faith."

Matt and I have to become resolute in the fact that we may never know why we will have to endure these two experiences. It may hurt, it may be unbearable at times, but I know we will survive. I know we will strive to make good once this storm passes. I know we are grateful to have each other, and SO SO look forward to the days we are living life with this as our memory, not as our present. It will take time, but we have faith that endures, and love that knows no lifespan.

**P.S. - With the above million word blog of information, we have decided to press PAUSE on Grace Blanket Drive this summer, until things have calmed down emotionally. 

IF you are in KC and want to drop blankets by, we will be happy to see ya; but let's not plan on mailing us blankets from afar. We will post when we feel it's time to resume, and do all our fun deliveries late fall. Thank you so much for understanding.