Surgery

 

2/08/2023 

Today we set a date for a tentative induction date of 03/15/2023. I am hoping that I can go into labor naturally but it's looking more like an induction might just need to be part of the plan now. I will go into more detail about the birth plan but wanted to talk about what surgery will look like for JJ for those who want to know more and for any moms out there who are just embarking on this tough journey. As discussed in my previous posts I talked about how JJ has LCDH (left-sided congenital diaphragmatic hernia) which means she has a hole on the left side of her diaphragm with her stomach, bowel and liver in the chest, pressing her heart to the right side of her chest and compressing the lungs so that they are unable to fully develop. JJ's defect is considered severe and will require a lot of medical attention and time in the NICU. The pressure on the lungs causes hypoplasia which is the small lungs and hypertension is the overreactive blood vessels, making CDH babies really sick after birth. The hernia can be fixed but it doesn't resolve the problems with her lungs. This is something that will need a lot of time to improve. Since JJ's liver is up, this decreases her survivability from 97% (babies with liver down) to 70% (liver up), and has a 30-40% risk of being put on ECMO (heart/lung bypass machine or life support) These numbers include the most recent data for CDH babies at our specific children's hospital in our state. In 2016 the CDH specialists have changed the way they do things which have increased their survival rates and improved the outcomes. 70% is a scary number but it also gives us a lot of hope for JJ since it's above 50% we feel that this is not a death sentence for our baby. Juniper's o/e is 16.6%. At our hospital, most babies who have passed away were at or below 15%. 

When JJ is born she will be immediately taken to the NICU where they put in a breathing tube to breathe for her or help her breathe and start her on sedation to keep her comfortable and calm. This hospital also treats these babies a little differently than a lot of hospitals by making sure they don't overstimulate so that they don't worsen hypertension. If they do this, most babies will settle in within the next 24-72 hours and are usually ready for surgery. If JJ needs ECMO, once she comes off, they will do the repair. It's important to know they will operate while she is on ECMO if necessary. This is something I have found isn't available at all hospitals and at some hospitals they don't do ECMO at all. There are risks involved with ECMO but it can be life-saving if needed. Babies only qualify for ECMO if they are over 4 lbs. JJ is at 33 weeks gestation and is currently 3 lbs. 12oz but they think her actual weight is around or above 4 lbs., so she will have the option if it's needed. Most CDH babies do not come early but sometimes can due to other factors such as higher amniotic fluid or having a FETO procedure (which we decided not to do because of this risk) so we are hoping to make it to full term so she can develop as much as possible in utero. 

Surgery and repair

The surgery will be done bedside in the NICU. NICU rooms in the hospital are all individual rooms and the sickest kids are put in specific rooms and bring operating equipment to the baby so that they are not disturbed or stressed out. These rooms are kept just as sterile and clean as the operating rooms. JJ will have her surgery only when she is stable. Sometimes they will decide to do surgery the next day but then reschedule and delay surgery depending on how she is doing at that hour. When the repair is done that is when we can take the next steps in getting her home so we are hoping for JJ to be stable as soon as possible and have her repair done within 24-72 hrs. The way they will repair is with an incision in the belly between the ribcage and the belly button, bringing the organs to their proper location and closing the hole in the diaphragm. They are expecting JJ's hole in the diaphragm to be pretty large so they cannot just stitch it up like they would with a baby who has moderate CDH and leaves us with the other two options. One of the options is a Gortex patch and the other is a muscle flap. The surgeons at our hospital typically use the muscle flap for larger repairs. This is where they take the most internal (inside) oblique muscle and rotate it down to close the diaphragm. Using her own tissue means it'll grow with her, and we won't have to worry about an infection because it's her own tissue and not a patch and oftentimes starts to work like a diaphragm. The only thing that will be a little different is that you will be able to visually see it stick out more on one side but this typically goes away when they are 18-24 months old and using their core muscles. Using the muscle flap also drops the rate of re-herniation to about 2% which is great. In 10-20% of CDH babies, the space where they put the organs can sometimes cause a lot of pressure, if this is the case, they will place a dressing over the incision leaving the incision open so that the pressure doesn't make her sick until this pressure decreases enough to close it up. Once the repair is done, it's all about dialing down support getting her off machines, and getting her a feeding tube. Once she is off ventilation and breathing room air she will start eating by mouth but all of these milestones will take time. The average for babies with an e/o as low as hers averages 4-6 months in the hospital. it could be less or more but they want us to plan for 4-6 months.