Notes from a CDH mom survivor

Over the last few months I have searched and pondered through many blogs in hope that I would find someone from our area that has a CDH survivor baby.  One that may have dealt with this in the last few years so we would know a  little more about what our experience at children's hospital may be like.  I have found one from the Seattle area so far.  Here is a piece of their blog that I thought may be more helpful for those of you at home wondering what we are really going to go through at the hospital and what you can do to help us along this tough journey ahead.  Some of this info you may have already heard but others may be new to you.  This was written to the Rondeau's family from a CDH mom survivor.  So here it goes:

 

 Mommy of a CDH Survivor

“It’s just a hernia…they’ll fix it and he’ll be fine, right?” 

“My baby was in the NICU too, it’ll be okay.” 

“Don’t worry, things will be JUST fine. He’s going to be okay.”

I heard these words one too many times, when our little boy was diagnosed with CDH. Often, the severity of this birth defect and it’s fifty/fifty chance of survival, is undermined by it’s name, so I wanted to share a little more about it from the medical perspective and share our story.

First, I want to share the basic facts (that were collected from several sources) that we shared with our family and friends when our little one was diagnosed (some of it may be a repeat for those of you blog followers that have done some research about CDH, so I bolded what I felt were the most important parts.) 

Congenital diaphragmatic hernia (commonly known as CDH) is an opening in the diaphragm (The dome-shaped muscle that separates the chest cavity from the abdomen.) It occurs at approximately 8-10 weeks gestation, when the diaphragm (for an unknown reason) fails to close completely. The hole allows the abdominal organs to push into the chest cavity. Organs that should be in the abdomen (such as the intestines, stomach, liver, and or spleen) slip through the hole in the diaphragm, into the chest, during the baby's development. Once in the chest, these organs take up space that should be available for the lungs to grow. CDH is a life-threatening birth defect because it limits the growth of the lungs, which is called pulmonary hypoplasia. 

Congenital = born with
Diaphragmatic = involving the diaphragm
Hernia = condition where organs/muscles protrude through a hole they aren't supposed to
 
CDH is as common as Cystic Fibrosis and Spina Bifida. Yet, until they have a friend, family member, or child diagnosed with it, most people have never heard of CDH.  

Healthy lungs have millions of small air sacs (alveoli), which resemble a balloon filled with air. With pulmonary hypoplasia, there are fewer air sacs than normal, the air sacs that are present are only able to partially fill with air, and the air sacs deflate easily due to a lack of a lubricating fluid called surfactant. When these conditions are present, the baby cannot to take in enough oxygen to stay healthy. 

After birth, babies are immediately intubated, allowing a ventilator to breathe for them. The very sick babies will require ECMO, which is a heart/lung bypass machine. It is a scary step to be put on ECMO, but sometimes it’s the only choice. The benefits need to far outweigh the risks for them to take this step. They will undergo repair surgery only when they are stable- sometimes this is between a few days to several weeks after birth. Most parents of CDH children will not hold their baby for the first time until several weeks old. 

One of the most severe issues with CDH babies is called pulmonary hypertension. The compression and impairment in the development of the lungs, leads to this dangerous condition involving high blood pressure in the arteries that supply the lungs. These blood vessels are underdeveloped, so they can't carry as much blood, as a normal baby. As in a kinked garden hose, pressure builds up and backs up. The heart works harder, trying to force the blood through. If the pressure is high enough, eventually the heart has trouble keeping up (and will start to fail unless ECMO is used), and the blood can’t circulate through the lungs to pick up oxygen. 

Now, with all the medical terminology out of the way, here’s a little from a personal perspective- the below is a short version of the some of the scariest, most intense, and most emotional two months of our lives: 

Our CDH survivor was intubated immediately and transferred after four hours to Seattle Children’s Hospital (SCH), after he was stable-ish. He ended up on ECMO on DAY 3 (which is often referred to as after the “honeymoon period” due to pulmonary hypertension that started as mild, then became severe and hit him like a ton of bricks on day three.) One side of his heart was failing, trying to work TOO HARD to pump into those underdeveloped lungs. ECMO would hopefully give his body a chance to rest, for the long fight ahead. He was taken off ECMO after several “trial offs”, when his body finally proved he was ready again for the ventilator alone, after almost two weeks of being on this lifesaving machine. On DAY 18, we had one of the best days of our lives-we GOT TO HOLD HIM FINALLY! He remained on the ventilator, getting the settings down to an acceptable level for surgery, until DAY 23, when he finally had his repair surgery. He had almost all of the left side of his diaphragm missing, a huge defect, with most of his abdominal organs in his chest. He spent over two weeks recovering from his surgery, before he was ready to come off the ventilator.  He was extubated on DAY 39- he breathed on his own, with a boost from a nasal cannula, for the first time ever! 

He spent over two months in the hospital total. After extubation, he battled reflux, worked on weaning down on oxygen levels that were administered through his nasal cannula, started to learn to eat, and weaned off the heavy doses of narcotics and other necessary medications that he had spent the first 40 plus days of his life. He was oxygen dependent until 9 months of age and was feeding tube dependent until 7 months of age. He is now a VERY healthy and happy two year old, but the CDH journey is never over. He has some remaining reflux and still has underdeveloped lungs (colds and germs hit him harder than most and we still fear hospital stays if he gets a yucky respiratory illness). We are also continually concerned with the risk of reherniation, since his patch won’t ever grow, but he will.  We are blessed he is a CDH survivor-there were days during his hospital stay that he was so sick, we weren’t sure if he would be a survivor. We never take anything for granted and we enjoy every moment of each day, knowing what the outcome could have been. 

I guess what I want to end with, is to tell those people who will love, support, and pray for the Rondeau's as they go on this journey, that it is a ROLLERCOASTER. There are SO many highs and lows with CDH.  They will need to have you celebrate the little successes (like diaper changes or weaning the ventilator settings “just a little”) and they will need tremendous support during their lows (and unfortunately, there will be those lows). They will need prayers, loving thoughts, offers of help, and listening ears. It is horrible feeling to see your child hooked up to SO many things and not being able to do anything to help them.  You lose a sense of time and place, just having to wait and see how they do, each hour of each day and not knowing when there is an end in sight. 

Sitting by a NICU bedside hour after hour is exhausting- watching those monitors will become their life, so give them all you have. Love on them, tell them you care, comment on their blog with your wishes of quick healing for Jacob.  Pray for no ECMO, stable days and nights, a repair surgery soon after he is born, lots of lung tissue under all those organs, and peace for Katy and Andy to help make decisions with the team of medical professionals that will be caring for Jacob. 

I can only tell you that Jacob is in the best of hands at Seattle Childrens Hospital. They are both progressive and conservative at the same time, if that makes sense. They are compassionate and realistic. The doctors, nurses, respiratory technicians, receptionists, cafeteria workers, social workers, playroom supervisors, and all who work there, exude compassion and understanding for parents of sick babies.

Jason and I got the chance to meet with a local family a few weeks ago that shared in our experience of a child with CDH.  I can't remember how many years ago it was, maybe 10-11 yrs ago, Dave and Vonda Vanderyacht (Dave was my softball and basketball coach in high school) had their second baby boy.  After he was 6 months old their baby suddenly became very sick.  They took him to the hospital and found out they needed to be rushed to Seattle and that their baby also had CDH.  This never showed up during Vonda's ultrasounds, but sure enough their boy had his stomach right next to the heart and it was pushing the heart to the the other side of his chest.  Lucky, he survived and is doing well today.  It is amazing that there son was doing so well at birth under these conditions and they had no clue anything was wrong.  He still has his heart on the opposite side of his chest and struggled with being sick for his first years of life.  You would never know he had such a thing at birth.  We felt blessed to have them pray for us and hear how their experience was at Children's hospital and what life is like today for them.  




I can still say its a day to day thing for me.  I get so scared when thinking of what Jason and I will have to go through.  The times I will be without Jason while he needs to work to help pay the bills and how hard that will be for him to be away from his family.  The hours I will be waking up to pump breast milk (every 2-3 hrs) and the times I will struggle with not being able to help her, cuddle her and breast feed her.  The times I wont get do things the things that mom's get to do when babies are first born.  I could go on and on with what scares me and makes me sad thinking about.  But most importantly, I am always reminded within these moments of sad thoughts, that no matter how hard this is going to be, it is God's story and he will use us and take care of us in this.  Also, that every second we get with Esther will be a gift and a miracle that she can even survive outside of my womb.  

We are overwhelmed and humbled by all your emails, posts, encouragement and offerings of help.  Just recently we talked to some friends who know people that want to help us and go even more above and beyond.  We have had someone offer to take professional pictures of Esther, Jason and I at birth.  Also, there is a hair salon in Fairhaven that does a fundraiser every year to help people in need.  On February 24th they want to have a fundraiser for us and all proceeds would go to helping with our expenses towards medical/living and sleeping arraignments/eating etc.  There will be more details to follow.  Also, a church that is making a quilt/blanket for Esther with her name on it.  The ladies of the church will pray over it while the blanket is being made and follow us along in our journey and continue to pray for us.  We have people praying all over the world!!! 


My next appointment in Seattle is February 15th.  We get to have a tour of Seattle Childrens NICU, get another ultra sound to see how she is doing and then meet with the doctors.  You can be praying for me as I head into the graveyard schedule at work this Wednesday.  I will be working this shift for the next two months before Esther is born.  This means that I get to work 6pm-6am 2 days on, 2 days off.  Lots of time to think and pray and hopefully not too much time to dwell. Jason and I can't say this enough- Thank you.  I wish there was some way we could repay you for everything you have been to us thus far. 


Rhonda (two more months to go!!!)





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