Let's Start at the Very Beginning...

On September 15th, Katy and Andy found out that Baby Jacob has a life-threatening condition called Congenital Diaphragmatic Hernia (CDH). CDH is a very serious condition in which a hole in the diaphragm allows abdominal organs to move into the chest restricting lung development. In Jacob's case, his liver is also squishing his heart and displacing other organs. CDH occurs in about 1 out of every 3,000 pregnancies and has a mortality rate of 50%. To make matters more complicated, Jacob has Right-Sided CDH which only occurs in about 10% of CDH cases and is typically more severe. They are so blessed to have found this out when they did, or Baby Jacob would have surely died at birth.

They will be delivering at UW Hospital in Seattle and later transferred to Seattle Children’s. Thank you to everyone who has already begun praying, and everyone who will now. Baby Jacob is blessed to have all of you thinking and praying for him.

Words from Our Friend, a 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. I hope this will help those that want to support the Rondeaus as they start on this journey on Friday, Katy’s induction date. 
I met Katy and Andy through a friend just three days after Jacob’s diagnosis. When my friend called and said her friend was just diagnosed with a CDH baby, the phone nearly fell out of my hand.  Given the incidence of CDH, numbers range between 1 in 2500 and 1 in 5000 babies, it was shocking that the Rondeaus lived a mere few blocks from us. I immediately contacted them and hoped by sharing our journey, they could get some much needed knowledge and some hope, from hearing the story about our CDH survivor. 

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 SCH. 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.

Katy and Andy are a tremendous gift to Jacob. The little I have gotten to know them in the past several weeks, make me SURE that Jacob is going to be surrounded by a caring, loving family. Even though it’s going to have a rough start, he is already incredibly lucky to have been blessed with parents like Katy and Andy. 

1 comment:

  1. Thank you for sharing your words with all of us. It puts everything into perspective. We will continue to pray and be supportive in anyway that we can.