The Beginning of the Journey

For those who did not know all the details before; Our little girl has Congenital Diaphragmatic Hernia (CDH). This means that her diaphragm did not form all the way and the contents of her abdomen have moved up into her chest cavity. The biggest concern with this serious birth defect is that the abdominal contents shift the heart up and prevent the lung on that side from developing completely. These babies biggest hurdle is not the surgery to repair the diaphragm but the fact that they often have immediate respiratory distress due to the lack of lung development. The reality is that the mortality rate is around 60% with many factors influencing those that survive. No two cases are alike and it is often hard to predict the outcome. We, however are 100% positive that she will be a survivor!

So, we had an appointment yesterday with a specialist in Kansas City. We were told here in Wichita that we would have to deliver her at Children’s Mercy in KC or somewhere else that is equipped to handle this serious issue. We met with the doctor from KU Med. She looked so perfect on ultrasound. She looks so much like Aria did when she was a newborn. It appears that only part of her stomach right now is in her chest cavity. They did not see that anything else was up. Her heart is shifted off to the right. Her lungs looked good to us although lungs are not easy to see on U/S. Her lung-head-ratio will be crucial to predicting her outcomes as we get closer. She did not appear to have any other soft markers for chromosomal abnormalities. There are a few that are sometimes associated with CDH including Trisomy 13, Trisomy 18 and Monosomy X. Trisomy 13 and 18 are fatal disorders. Some of the things they look for are growth retardation (She is measuring big in the 77th percentile), club foot, short nasal bone, arm and leg bone deformations and hand deformations. All of those things looked great. She also does not have a cardiac malformation (the shift off to the right does not count) which is associated with CDH and lowers mortality quite a bit. The doctor seemed to really want us to do an amniocentesis but we opted not to. The test would reveal any chromosomal abnormalities and the outcome would not change our decision making related to our pregnancy or doing all we can for our little girl.

The big issue was the hospital we would be using. We were surprised when he said KU Med, since we had been told by everyone it would be Children’s Mercy. He said that Children’s Mercy does not deliver and transferring these babies is a bad, bad option. One main issue though is that KU Med does not have an ECMO machine (I’ll let you guys look that one up on your own so you don’t have to read from me anymore). Every single thing I read about CDH babies says you MUST deliver somewhere with an ECMO machine. While I understand and partially agree with the doctor’s thought that they are sometimes used too soon when other things may have worked I am not comfortable delivering somewhere that does not have one, in case she would need one as a last resort.

When we left our appointment we were optimistic because of how great our daughter looked and the fact that so far only her stomach was up in her chest cavity. I however had a huge gut feeling that KU Med was not the right place for us to have our daughter. I asked the doctor how many CDH babies they had in the last year (a VERY important question, they should do at least 3 a year) and he said he did not know, he had only been there for 18-20 months (seems like more than a year to me). This doctor is the director of the KU Med Advanced Fetal Medicine Center. He would be the doctor to see the babies with CDH prior to birth. He knows how many they have had and he either avoided the question because it is too few or he avoided the question because the next one was, “what is your survival rate”.

When we got home Brad and I talked about KU Med and Kansas City not being the best option for our little girl. Children’s Mercy while having experience with CDH does not allow deliveries, leaving a baby seriously vulnerable during transfer. Many parents have reported that they could not even talk to their babies because it sent their heart rate into orbit and caused immediate d-stat. A transfer is dangerous.

We had previously discussed Omaha Children’s hospital which has extensive published information on CDH and doctors with many years experience on the issue. I also have met a few women online who had their CDH babies at Omaha Children’s and they sing the praises of the doctors and hospital. They do have an ECMO and they do have delivery on site (The Methodist Hospital is actually tied to the Children’s hospital to allow that). KU Med does not have any published information about CDH that I could find. What I did find was one blog from a mother who lost her baby to CDH in March at KU Med (she lost her during the transfer to Children’s Mercy). She did not have positive things to say about the doctor we met with either.

We have decided, given what is best for our daughter and given the fact that Brad’s company has an office they want him to transfer to in Omaha that it is the best place for our daughter. These babies average 3 months stay (often longer, although it can be shorter) in NICU and we will not leave her in a city with us hours away. We will be near her as a family and we will make sure that Aria and Miles do not feel displaced or in a temporary home without their comforts. Not to mention it would be very expensive to maintain residence in Wichita while paying for stay, food, gas, etc. in another city for that long.

We plan on moving to Omaha very soon, ideally by the end of November when my class is done being taught. They will want to induce me so they can insure the medical team is available when she is born and they will likely do that at or around 38 weeks. There are some risks in late pregnancy with CDH, namely Polyhydraminos, which is excess amniotic fluid. This is caused by the baby not being able to swallow due to compression of the esophagus from the abdominal contents in the chest cavity. I don’t have any problems so far but it often does not show up until later, if it does. It does put me at risk for premature labor if it does occur though and she is better off in the womb than outside of the womb. We will need to be near the place that can care for her immediately and not hours away in case something like that does happen.

I guess I have given everyone a quick cliff note lesson on CDH, which was not my intent. I just wanted everyone to know what was going on. This is a trying time emotionally for all of us. Our kids are our priority in everything we do and we not only want to make sure that our new little girl has the very best care she needs but Aria and Miles can also go through this with as little turmoil as possible. I never imagined the difficulty I would face carrying our little girl knowing the pain she would immediately face and the fight she will have to put up to live when she is born. We appreciate all of the good thoughts and prayers. Our family and our new daughter need all the positive thoughts we can get.

I will try to keep you posted on what is going on . We need to get our plan for our move outlined and figure out when it will all happen. We also need to head up to Omaha for an appointment and meeting with the doctors there. I hope to have that scheduled tomorrow through our doctor’s office.