33 week ultrasound - Feb 8th, 2023

 Juniper's current weight measurement is 3lb 12 oz. The way they measure this is by two measurements of the head, belly, and thigh. The most important measurement is the belly. Since Juniper doesn't have her organs in her belly, this is causing that particular measurement to be really small. Her measurements now put her at the 5th percentile and she is officially considered growth restricted. That being said, JJ weighs more than they think she does because they cannot account for the organs in the chest that are supposed to be in the belly. This means she is likely over 4 lbs. If you take out the belly measurement this will actually boost her back into the 10th or 12th percentile based just on the measurements of the head and thigh bone.  With all that information, although her belly is small putting her into the 5th percentile, we are still okay and on her growth curve. She is just a more complicated case due to her CDH diagnosis. Babies that are below the 10 percentile are recommended to have an umbilical doppler test where they test and listen to the heart. JJ's number was slightly elevated so when her heart contracts the heart should be stronger and push blood to the placenta, when the heart is relaxed it should still be able to push blood back into the placenta. With this measurement being a little high, this is a sign that the placenta isn't stronger than her heart but is getting closer to being stronger. With it being a little elevated she is still within the normal range. Abnormal would be when the heart is relaxed and they don't see any flow, or when the heart relaxes the blood flow goes back to the baby instead of the placenta (This is called reverse flow). JJ does not have this but is trending toward this. This means we need more tests and frequent watching so I will have two NSTs weekly and a doppler once a week until delivery. If we have the reversal on the doppler test, I will need to be admitted to the hospital and monitored daily until she would need to be delivered. Today we couldn't get an LHR (Lung-to-head ratio) because her stomach is bigger. The reason for that is when her stomach herniated into the chest, her stomach twisted so the fluid can't flow through like it normally would and this is causing more compression on the lungs so we can't see the lungs at this point. We are hoping the stomach will un-twist but it has been twisted since I had my MRI so we don't know if that will happen or not. If it does this could lessen the fluid build-up. 

They have scheduled me for an induction on the 15th of March but I am trying to figure out a way we can be close to the Hospital is SLC so that I can go into labor naturally. This is just how I prefer to proceed and believe this is the best option for both JJ and myself. We have a few options for this. One option would be to stay at the Ronald McDonald house which is probably the closest to the hospital or stay in a discounted hotel nearby. The only issue with this is that I would need someone with me 24/7 to drive me to the hospital when I do go into labor and Ryan's (my husband's) work doesn't permit him to work from home. The other option is for us to stay with Ryan's sister who lives about 35 minutes away. I am not sure if the doctors would be okay with being this far from the hospital or not because of all the risks involved such as pre-term labor and placental abruption (due to the fluid being high) but I will talk with them at our next ultrasound appointment on the 6th of March. They think I will likely go into labor around 38 weeks because the fluid is high enough and will keep building and even though I will be 38 weeks my uterus will think I am 40 weeks which would cause my water to break. having this high of fluid will also cause labor to occur much quicker than a normal birth. Sometimes, we can have the opposite problem where the uterus is so big that it can't contract or function like a muscle anymore, if this happens a c-section would be necessary. When I do go into labor, Ryan will go with JJ to the NICU and once I am recovered I will be able to follow behind. If I do get an epidural, they will wheel me in a wheelchair, if I end up needing a c-section they will bring me to the NICU in a bed. I am going to try to go without an epidural so that I am able to recover and walk as quickly as possible. Anything that happens at this point would be explained to us then since we will know a lot more about how JJ is doing, what her actual severity is, and how she is responding and stabilizing.  

Photo by Kenzie Neal 

www.kenzieneal.com 

Instagram: @kenziephotos2

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.