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. 

MRI results and update on Juniper Jane

 

January 18th, 2023

 Today we had our follow-up appointment to do an ultrasound and discuss the MRI findings. Juniper's observed-to-expected ratio from the MRI is showing 16.6% (lung volume) which is a little less than we had originally thought and not very much has changed. She is still considered in the high-risk category and with the amount of liver up into the chest she is considered a severe case of CDH. Juniper is in the 11th percentile so she is growing but still on the small side. (This tells us how Juniper's growth compares to the national survey) Part of her being on the smaller side is due to her organs not being in the right place but her other structures are developing normally. The concern with Juniper being on the smaller side is that she needs to be a certain weight to be considered an ECMO (Extracorporeal Membrane Oxygen) candidate if she ends up needing that type of life support. As long as she continues to grow like she is we should reach and pass the weight she will be required to achieve to be a candidate. So far she is doing well and as long as she doesn't dip below the 10th percentile we don't have to worry. While Juniper is still at higher risk for needing ECMO, they will still try to avoid this option if possible. However, we still want her to have that option if she needs it because it can and does save CDH babies' lives. In a few weeks, we will need to do none-stress tests (NST) every week to monitor how Juniper is doing to make sure she is doing well enough to continue to term. NST is just a heart rate tracing that takes about 20-30 minutes that lets the doctors know she is doing okay and no intervention is needed. We will have another ultrasound in three weeks to continue tracking her growth and have another fetal echocardiogram to check the structure they weren't able to see last time. At our next follow-up appointment in three weeks, we will discuss the birth plan, possibly having an induction, and what that will look like. I am still able to deliver her vaginally as long as there are no other complications and have her without medication. This is not a situation where they recommend c-sections as long as the baby is tolerating labor well. Juniper will be delivered by a high-risk OB at about 39 weeks with likely induction. They want Juniper to develop and grow as much as she can so that we don't have the complications of prematurity on top of CDH. During the ultrasound today they did measure the amniotic fluid which is a little on the higher side which can cause complications but isn't much of a concern at this point. When there is too much fluid, this is called polyhydramnios. Sometimes when there is too much fluid build-up in the uterus this can cause pre-term labor. Normal fluid should measure at 25, right now I am measuring at 35 so this will also be something that is monitored closely. If that number increases to the 40s it will become more of a concern. This will be monitored at the same time as the NST.  

We also met with the surgeon today who explained that part of the process to us and I will update the blog regarding that discussion at a later date. 

Juniper Jane's Story

What is CDH?

Congenital diaphragmatic hernia (CDH) occurs in an unborn baby around 6-8 weeks gestation when there is a hole in or incomplete formation of the diaphragm (the muscle under the lungs that assists breathing and separates the upper chest and lower abdominal organs). This hole allows the abdominal organs such as the stomach, intestines, liver, and sometimes the spleen to move into the chest and prevents the lungs from developing normally, and shifts the heart to the right side of the chest. This defect can occur on the left, right, or both sides but more commonly occurs on the left (LCDH). The defect is usually seen at the routine 20 weeks ultrasound and occurs in about every 1 in 3600 babies.

 Our baby Girl, Juniper Jane (JJ) is due March 30th, 2023. At 19 weeks and 6 days pregnant on November 8th, 2022, at our anatomy scan, we got the news that her stomach appeared to be in the incorrect position. Another ultrasound was scheduled to confirm. Before we could make our second ultrasound I ended up in the ER as I had gotten Covid-19 which put me into the ER due to severe chest pain, low potassium, and dehydration. Since they wanted to check on the baby via ultrasound, I requested they check the stomach position so that I didn't have to come back in a week to confirm it. 

They told me nothing has changed and that the stomach was still in the same position and referred us to a Maternal Fetal Medicine doctor. During this ultrasound, the tech checked and measured everything like they would at an anatomy scan and mentioned that the stomach was indeed up in the chest and above the diaphragm. My heart sank a little, but I remained calm and waited for the doctor to come in and share his opinion and thoughts. After waiting a few minutes for the doctor to review the images he stepped into the room to do his own imaging and talk to us about what he can see. At that time, he explained that the stomach is all the way up into the chest, he also mentioned he thinks there are small intestines up into the chest and went back and forth on whether the part of the liver is above the diaphragm or not. He then proceeded to look at the heart and let us know that Juniper's heart has been pushed to the right side but otherwise from what he can see seems healthy. He did mention that he is not a cardiologist, so he cannot confirm that, and we would need an echocardiogram to check her heart health and have a clearer answer regarding heart health. (An echocardiogram is just like an ultrasound but only focuses on the heart of the baby and is done by an expert cardiologist.)

 We are scheduled to have the echo done on 12/20/21 and an ultrasound on 12/21/22 at Primary Children's Hospital. It sounds like we will have to go back and forth through most of my 3rd trimester to have tests and monitoring and come up with predictions on the outcome of JJ's treatment, NICU stay time, and quality of life. 

12/20/22

The echocardiogram we had with the doctor went well. All of JJ’s structures look good and blood is flowing the way it should. Her heart is pushed over to the right side and is far to the right. This means that has very little space in the chest the only part they couldn’t see well enough was the aortic arch which is common to not get a great picture of since this structure is stretched and follows a different path than when a heart is in the correct position. JJ also kept her arms and hands close to her chest during this scan which made it difficult to see. Ultrasound that will give us a better picture of the severity of JJ’s defect is tomorrow.

What can be done to treat babies with CDH?

Babies with CDH will require surgery. During surgery, an incision will be made into the upper abdomen and the organs will be pulled back down into the correct position. The hole or the incomplete formations will be either stitched together or sealed using a mesh patch of some kind to prevent re-herniation. Babies with CDH will also need to have assistance to breathe with gentle ventilation. If necessary, they will be put on ECMO. ECMO is something that we want to avoid if possible as it’s a type of life support that keeps the heart and lungs functioning. When babies are put on ECMO they do become at higher risk for other challenges throughout their life. Babies with CDH will require long-term monitoring and are likely to stay in the NICU for an extended period.

What does JJ’s scan look like at this stage?

12/21/22

Today we had an ultrasound scan that confirmed a left congenital diaphragmatic hernia with stomach up, bowel up, and liver up. Since the liver is up this is putting more pressure on her heart and lungs. This will not allow JJ’s lungs to develop fully in utero. Right now, JJ is happy as can be since she doesn’t need her lungs until after birth. Once she is born and the umbilical cord is cut, this is when she will start to have problems breathing and will require some ventilation and medications to help her. 

 JJ’s condition as of right now is considered moderate to severe. When the liver is up in the chest this puts a lot more pressure on both the heart and lungs which will make her lungs even smaller at birth. When the liver is up it makes the condition worse and our outcomes worse and puts her at higher risk of being put on ECMO (life support). Everything else seen on the ultrasound looks normal. Brain anatomy, limbs, bladder, kidneys, and other organs look normal. 

JJ’s weight as of today is 1lb 11oz which puts her right at the 10^th percentile, meaning she is a little bit smaller which is common in babies with CDH. Less than the 10^th percentile is classified as fetal growth restriction and JJ is right at that cusp right now. JJ’s growth will be tracked over time but is doing well so far. I will have an ultrasound every four weeks until birth to track her growth, monitor any changes in what organs are up or down, and measure the fluid around JJ. At about 32 weeks we will start something called non-stress tests which is where JJ will be put on a monitor, and they will listen to her heart rate to give reassurance that she is doing well. 

The goal is to get JJ all the way to term so that she has time to develop as much as possible before coming into the world as this will increase her chances to survive this. Her survival will also depend on how much reserved functional lung there is. Primarily she will have to rely on the right lung since this one will be in better shape than the left lung. During the ultrasound, they did measure the lung-to-head (LHR) ratio where they measure the volume of the lung tissue and compare it to the measurement of the baby’s head. JJ’s measurement today is 0.46. When that number is less than 1 this also puts JJ at a higher risk of needing ECMO and puts her in the higher risk category of having cardiovascular compromise and increases her risk of passing away. 

This number can change throughout pregnancy, but they will still use the worst-case number for her prognosis so they can prepare and plan for that. There’s another measurement they use called O/E measurement and JJs is 18% today which tells us the same thing since it’s below 25%. This means they are anticipating JJ to be very sick after birth and will need a lot of support. We are anticipating a long-term NICU stay of 3-4 months but can potentially be longer. After JJ is born they will immediately assess her breathing and place her on a breathing tube and evaluate her to see if she needs ECMO right away or if she's stable enough to just be on the breathing tube. They will make sure she is first stable to do the surgery and this is usually within the first few days of being born. 

As you can imagine, we got a ton of information today so I will split these first updates into parts. In the next post, I will talk more about birth and the NICU and what has been explained to me as far as what to anticipate happening. 

(1) Tiny Hero (n.d.). What is CDH? Www.Tinyhero.org. https://www.tinyhero.org/what-is-cdh