Everett James was born on August 29 at 645pm, fast as lighting in our home, with undiagnosed congenital diaphragmatic hernia that was not picked up on ultrasound during his 20 week anatomy scan (only 10% of CDH cases are missed on ultrasound - we were one of the unfortunate ones). Congenital diaphragmatic hernia (CDH) is a rare condition that happens in a baby before birth (roughly 1600 babies are born in the US with CDH/annually). It occurs early in pregnancy when a baby's diaphragm — the muscle that separates the chest from the abdomen — fails to close as it should; therefore leaving a hole which allows organs to migrate into the chest cavity. 50% of babies diagnosed do not survive. The cause is unknown.
Immediately after birth we realized something was wrong - Everett was breathing very shallow and short and began to turn purple. I screamed for Elijah to call 911 and they were there within minutes (this all occurred within the first 15 minutes of his life). Everett wouldn't respond to me stimulating him, suctioning him or giving him breaths. Thankfully, EMS put him on oxygen immediately - this saved his life.
We were taken by ambulance to our local hospital. Initially we all thought, including the staff, that perhaps it was just birth trauma, he swallowed something, had fluid in his lungs, etc. They put him on cpap - did an x-ray and there it was confirmed they could see his intestines in his chest cavity. He was immediately taken off cpap (which was the wrong thing for him to be on), intubated and getting ready to be transported to CHOA.
We arrived at CHOA around 2am and he was placed in the critical (open) side of the NICU - that is when I finally started to process the severity of what was going on. We met with the on call doctor who confirmed Ev's CDH, and stated it looked relatively mild (we later found out it was much more).
The next day brought talks of repair surgery - the idea was to monitor him and keep him stable until the following Tuesday/Wednesday when he would have his repair surgery. We unfortunately were going into Labor Day weekend and since his surgery wasn't considered "emergent" and "critical" - we had to wait. Waiting almost cost us his life.
While everything seemed stable and fine those few days, Monday September 4, right before midnight we got a call that rocked our world. Everett had an episode, clogged his ET tube, de-satted and they couldn't get him back up. His heart was also having issues - he was diagnosed at the time with severe pulmonary hypertension which complicated things a bit more. Long story short, he was placed on ECMO. ECMO is full life support that pumps and oxygenates a patient's blood outside the body, allowing his lungs to rest. It is quite honestly the most terrifying thing to ever see your tiny baby hooked up to.
It was found out that the reason for his episode was MRSA... and MRSA caused severe pneumonia on Everett's under developed lungs (which is common in CDH babies). He contracted MRSA at some point at one of the hospitals or during transport. Those 20 days on ECMO were the darkest and scariest days of my life.
His ECMO run was difficult - there were times I was convinced he wouldn't pull through. Hearing a doctor tell you that your baby wouldn't be alive if there weren't on ECMO is gut wrenching. One Saturday during his ECMO run I went up to the hospital alone - shortly after getting there he started hemorrhaging from his lungs and bleeding from his mouth. Another nightmare, but he overcame it.
It was decided after the bleeding was controlled and his lung still whited out/full of secretions, we would do a bronchoscopy to help clear his lungs and hopefully get him off ECMO to do his repair surgery. Well, one bronchoscopy turned into FIVE days of them - the most the pulmonologist had ever done back to back on a kiddo, especially a baby. His lungs were so full of mucus secretions, old blood/clots - that it took five procedures to clear everything out - but it worked.
His last bronch was done on a Friday, September 22. He trialed to get off ECMO the next day - and when we walked in Sunday morning, my favorite doctor came up to us and told us he was coming off ECMO TODAY. The surgery was successful, and the same surgeon, my favorite, who took him off ECMO would also do his repair. We set a time for his repair surgery for September 27, first thing in the morning.
Ev had to stay stable for his surgery, but we also had to wait at least 48 hours to allow certain medications out of his system for surgery. The night before surgery was rocky for Ev, I think if we had waited one more day he wouldn't have been stable enough for surgery - but God willing... surgery finally happened.
Everett was able to get his repair surgery. However, during the surgery we found out it was much more intense than we initially thought. He was missing about 30-40% of his lower diaphragm. This allowed his colon, small intestines and part of his spleen to travel into the left side of his chest - which pushed over his heart and lung a bit. Our surgeon was able to move everything back into place, and close his hole with stiches and a mesh gor tex patch.
A day or so after surgery we walked in to Everett being on the oscillator again (he was on it for 2-3 days during ECMO when he was at his worst). I was SHOCKED seeing that thing and kept questioning why it was needed, he was breathing on his own and clearly wasn't tolerating it. The nurse agreed and during rounds we were going to ask to take him off. Everett had other plans before rounds and he had an episode where his heart went into arrhythmia and his sats were all over the place. Elijah and I were by his side when this started happening and I went numb and felt like I was about to pass out watching everything - thankfully, Elijah pulled me out of the NICU. Ev ended up getting bagged and put on iNO and, surprise, taken off the oscillator because he wasn't tolerating it. The next day he was stable again.
Four days after surgery, on Sunday October 1, we were able to finally hold our baby for the first time since he was born. We waited 34 days for this. Since then, we have been able to hold him every single day - which has been so healing for us all. We have also been able to FINALLY start him on breastmilk through a feeding tube (now that his surgery is done) and start to wean down the IV nutrition (what he has been on since birth). Getting on breastmilk is a big big win. His feeds have slowly increased (big win) - the more they increase, the more we can start running meds through this tube and remove some of his drips.
While he 'looks' so so good on the outside, our sweet boy is still having major heart/lung issues. After weaning off his iNO last week, his heart echo picked up bad news about his PH - it looked worse than it had every looked. He was put pack on iNO and his echo the next day improved by 50% - the iNO is working right now.
That brings me to the present.
Our journey is nowhere close to being done - at minimum we will be in the NICU until Christmas. Everett is still currently sedated, on many pain meds/narcotics, having heart issues due to his pulmonary hypertension, on the vent, amongst other things I will update daily on. However, the last few x-rays on his lungs have shown fully open and beautiful lungs - even his small lung looks bigger and beautiful.
He is a miracle, our miracle.
God has shown up for us in ways I never expected, this has pushed us deeper into our faith and I know God is with Everett James every single day.
It's been quite awhile since the last update - while I wanted this to be therapeutic to write in, it became too much when it seemed that every single update was steps back. November was a very dark month for me mentally but deeply due to Everett not being able to make any progress and constantly taking steps back, getting more infections,... the list was never ending. Conversations started to be had on the potential of Ev getting a trach and how medically complex our future would be; it was hard to process. Elijah and I had started the process of transferring his care to Boston - meaning our family would have been split up even more.
Then December 1 I got a phone call shortly before 7am from the hospital. My stomach dropped answering it - I knew something happened (otherwise, they would have just waited to tell me when I got there in a few hours). The doctor on the other end of the phone said Everett self extubated (meaning he pulled his breathing tube out)... and then she said "and he's doing great - we put him on bipap oxygen". I had a mix of emotions - how did this happen, but at the same time overjoyed that something I pleaded with God for every single night just.. happened. He wasn't intubated anymore and he was doing... great. He took it into his own hands and proved everyone that doubted him wrong. And he continues to.
Since then, Evy is now on cpap oxygen and we are actively weaning. He remains stable - the goal is to be on high flow oxygen a little after the new year. I still am in shock that I am writing that and it's been almost 3 weeks since it happened. I remember telling one of my favorite nurses a little before Thanksgiving that all I wanted for Christmas was for Ev to get extubated and to get off this really nasty drug he was on for his heart. Both happened, only a week later.
He has also weaned off 2 of his narcotics successfully (what he was on to keep him relaxed while intubated) and we started to wean the last yesterday. Once he is weaned off of this one, we will get to remove his PICC line! He does still have other medications through his feeding tube, and a few of those will need to be weaned as well. However, removing his picc line is HUGE.
He has been a completely different baby since he self extubated - happy, so alert, loves to play, takes his paci (I never thought that he would- this is also huge!), like to eat his hands, sit in his swing, play on his mat, and even wants to try to roll over... he is a normal baby underneath everything.
Some of the best news (I know, can I beat self extubating?) came earlier this week when we learned that, for the very first time, his echocardiogram (his heart ultrasound) had... IMPROVED. His heart is becoming stronger and working better with his lungs to pump oxygen.
We still have a long journey ahead - as in a few more months in the NICU (we have already been here 4months on the 29th), but the last month has illustrated even more how much of a Christmas miracle our little Evy is. God is good.
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