Clara’s first full day of life was the roller coaster of health stability/instability that we had been told to expect, although I don’t think there was anything we could have done to adequately prepare for it. After the joy and elation of her birth yesterday and really strong numbers and signs out of the gate, today she faced her first of many serious trials. I asked Robert to update earlier this afternoon to get everyone focused and praying, but am now going to do my best to explain how she is doing and give you very specific things to pray for. Bear with me as my medical knowledge is new and limited.
Clara had a good night last night and this morning her blood gasses began to drop. This is to be expected and is a sign of the pulmonary hypertension that almost always accompanies CDH. However, it was causing her to struggle a bit and fight the ventilator and breathing was becoming more labored than they wanted so they started by putting her on some drugs for sedation. She was also put on dopamine to raise her systemic blood pressure which was effective.
The tests performed today were an echocardiogram (which looks closely at her heart), a chest x-ray, and an ultrasound of her brain to determine if there is any bleeding. Should she have to go on ECMO a brain bleed would be a concern because she would be on a lot of blood thinners. Her brain looks good. The echocardiogram confirmed the prenatal fetal echocardiogram - the structure of her heart looks normal and healthy, which is great. However, it also confirmed what her blood gas numbers were indicating today: Clara does have pulmonary hypertension. The chest x-ray let us know that her left lung is quite small and her right lung is probably smaller than normal as well. There is no way to really tell the exact size of the lungs until surgery. It also told us that in addition to her stomach and bowel being in her chest cavity that her spleen and part of a liver are also in the chest. The 19 week MRI indicated that both of these organs were below and we were hoping they still were. Because her abdomen is full and round (sometimes babies with CDH it appears concave) they suspect that those organs have probably moved up recently. Hopefully they will be able to be repair this as a part of surgery when we get to that point.
Other news we learned today is that Dr. Turner will be the pediatric surgeon who will do Clara’s surgery, when that time comes. All the pediatric surgeons in this department are wonderful, but we have heard many especially great things about Dr. Turner and are glad that he will be caring for our girl. Dr. Block is the neonatologist on rotation for the next two weeks. He was actually my soccer coach years and years ago and is also a fabulous physician and a good man. He met with us for about 45 minutes today and explained many things to us. He is very straightforward and honest, but also has a very compassionate heart. We are in great hands.
So, pulmonary hypertension is our big beast right now. I’m going to do my darndest to explain it. There are two different blood pressure readings in the body. One is the blood pressure that we all think about when we hear people mention “blood pressure.” That is called the systemic blood pressure and is just the blood pressure throughout the body. There is also blood pressure within the lungs. That is the pulmonary blood pressure. Clara’s systemic blood pressure is normal right now (in part due to the dopamine). The blood pressure in her lungs is high. We measure this by the percentage of oxygenation of that blood. There are two numbers we look at for her blood gasses (oxygen, carbon dioxide and pH). Ideally they would both be close to or at 100. That’s where they were last night. Today, the top number has mostly been in the 90s which is still okay, but the bottom number has been in the 60s and 70s. This discrepancy is what tells us she has pulmonary hypertension. We need to get the pulmonary hypertension under control before we can begin to think about surgery. The goal is for her pulmonary blood pressure to be ½ to 2/3 of her systemic pressure before surgery. Right now, her pulmonary blood pressure is higher than her systemic pressure. The tricky part is that this is just a waiting game. There is not much that we can DO to correct Clara’s pulmonary hypertension. We are trying to keep her stable, rested and sedated in the hopes that the pulmonary hypertension will slowly begin to correct itself. This happens in part as the blood vessels in her lungs begin to open up and allow for more oxygenation.
When Robert posted earlier this afternoon Clara had just hit a very rough patch. Both of those numbers mentioned above were dropping dramatically and despite some shortfall efforts to reverse them, she was unable to recover. The doctors eventually made the decision to start her on nitric oxide and fortunately that did correct the numbers for the short term. She was stable again and her top number was right at 100 while the bottom number was been around 85 or 90. Those are good readings, but we were hoping to get her off the nitric oxide at some point and still maintain those levels. If the nitric oxide had not been successful she would have faced ECMO tonight. While we are thankful for the technology available such as ECMO it is our fervent hope and prayer that her condition will not deteriorate to the point where she will require this treatment.
In addition to the nitric oxide Clara is currently on dopamine (keeping her systemic blood pressure up) and a combination of two sedatives.
11:19PM: We just got off the phone with the NICU again. The nitric oxide that was started about 4:00 worked well for several hours, keeping her numbers above 90. About 8:45 this evening those numbers dipped into the 80s. They stayed down for about 45 minutes and around 9:30 she recovered on her own. Then, about 10:30 her numbers dipped into the 60s and 70s again. They are giving her until about 11:30 to see if they will rebound on their own. If they do not, we will begin to think about ECMO tonight.
I’m going to stop writing now. It’s too difficult to update with things constantly changing. As soon as I type one sentence, the scenario seems to change again. For now, we ask that you pray specifically for Clara’s pulmonary hypertension and the blood oxygenation levels. Pray that she will stabilize and not require ECMO. Robert and I have been praying that she will reveal her fighting spirit that we know she has and that she will rest in the power of God’s hands tonight. Please pray that Robert and I are able to rest some too as we are both exhausted and overwhelmed and we know we still have a marathon ahead of us. I’ll try to post more tomorrow and I do apologize if I was unable to accurately explain some of this medical stuff. It’s all a little over my head.
Continue praying. We are praising our God, even through this unknown and scary times. He is the One who can work miracles in Clara.