It was a big day full of medical news and updates and another relatively slow and stable day for Clara. Here’s the scoop!
One of our big goals for this week has been to get her fluids down. Yesterday they started her on Lasix, a diuretic that pulls off some of that fluid. It is working well; yesterday she was negative 140ccs (she put out 140ccs more than she took in) and as of about 9:30 tonight she was negative 60 for the day so far and should be negative over 100 by morning. She continues to pee well! They gave her more Lasix today. I mentioned that they had lowered her sedation drug (Versed) a couple of days ago. We’d like her sedation to be as low as possible for several reasons. The more she is able to move, the more the fluids go down. We also want to reduce the likelihood of her building up a tolerance to the drug and the lower we can keep it the easier it will be for her to wean off of it and prevent withdrawal. It will also help her with the ventilator. With that said, we also want for her to be comfortable and she has been a lot more active and alert the past two days. They’ve had to give her several boluses of sedation the past few days, so there was some talk of increasing her sedation, but for now she seems comfortable and happy, so it looks like the Versed will stay where it is. That’s the news on her basic day-to-day stuff of today. Other than those small changes, she’s holding steady and remaining stable and restful.
We had a chance today to meet with some of the big key players in Clara’s care. We’re learning that there are lots and lots of different doctors involved in making decisions about her case. This is due in part to Brenner being a teaching hospital and there being many medical students, residents and fellows on board. Also, since her case is complicated there are doctors from lots of different teams who weigh in on her care (surgeons, PICU physicians, ECMO folks, nurses, etc.). That’s a good thing because it means that many people are looking at her numbers and test results and are able to all collaborate together to come up with what is hopefully the best decision. We know that nothing will “fall between the cracks” because there are so many people treating her each day. The challenge of that is that every medical professional has different opinions and different ways of interpreting things. So we don’t always hear the exact same story twice and it can be confusing for us as the patient/parents to not know exactly what is happening. With that said, most of what we heard today from several different people was consistent! That was refreshing and encouraging to us and made us feel like we’re starting to see some things as a little more clear cut. My type A personality likes that.
The first person I talked to today was Luke, the pediatric surgeon chief resident. I like Luke a lot. He is very personable (especially for a surgeon; please excuse the stereotype) and did a very thorough and clear job of explaining to things to me. Surgery has been scheduled for Thursday, March 31 – hopefully beginning about 8:00AM. Dr. Turner will do the surgery, although he will be accompanied by a big team of folks including Luke, medical students, a scrub tech, an anesthesiologist resident and attending, and various other people. Instead of moving Clara to the operating room while on ECMO, they will do the surgery in her room in the PICU. I’m relieved about that because although they certainly can move patients who are on ECM O, this machine is huge and complicated and it just seems like an unnecessary risk if the surgery can be done where she is now. The operating team will just come up to her room to operate. This is how the surgery will go: They will make a horizontal incision just above her belly button and take everything out (colon, spleen, liver, intestines, stomach, etc.) Then, they will begin repairing the diaphragm. Sometimes there is enough diaphragmatic tissue (Luke called it a “rim” of tissue) to just sew it together, muscle to muscle. If this is true in Clara’s case – great! However, they are expecting that the hole will be too big for that because of how many organs appear to be in the chest cavity. If there is not enough tissue to sew together directly, they will sew in a gortex patch. In about 40% of cases with a patch there is a recurrence and at some point, way down the road, the patch will have to be replaced. The next step will be to attempt to close the belly. They are hopeful that they will be able to fit all of her organs back in her belly and close her up since her stomach appears pretty round and full right now. Many CDH babies have a stomach that is somewhat concave and the organs don’t fit back in the belly. If this is the case, they will put in a silo, which Luke described as a cylindrical plastic bag sort of like a newspaper bag. They will put the organs into that bag and leave it on the outside while sewing the rest of her up. Then, day by day they will slowly push her organs back inside of her and then will have to do a second surgery to securely close everything. The surgery is expected to take somewhere around three hours.
Dr. Turner came by a few hours later and basically confirmed everything that Luke had told us. Although we know that all of the surgeons on this team are extremely talented, we are glad that Dr. Turner will be the one doing the surgery and have lots of confidence in his ability to operate (and Luke too!). Dr. Turner told us a little more about the echocardiogram that was performed yesterday. There does appear to be more left to right shunting which is good news. They were unable to determine if the pulmonary hypertension has changed any because there was no blood seeping back into the tricuspid valve. This is neither good nor bad; just inconclusive. There is still high pulmonary artery pressure on her left side… it does not seem worse, but also does not appear to be much better. There will be another echocardiogram tomorrow and we are hopeful we might be able to get more results from it.
He also gave us some results from the chest x-ray. Her right lung seems full sized. It’s a little hard to tell because her heart is on the right side (it’s displaced because all the other organs are on the left side where the heart belongs), but it appears to be a full sized right lung. Her left lung is what he described as a “nubbin.” It appears to be filling up one-fourth to one-third of her chest cavity. This lung is the main source of our concern. After surgery, we need for this lung to begin to inflate and to grow and to start doing its job in order for that pulmonary hypertension to decrease. A week ago, that nubbin of a lung was in the center of her chest. Now, it appears to have shifted back to the left side where it is supposed to be. It is good that it has moved because that does show some progress and improvement. The PICU attending doctor today showed us the chest x-rays and she said that she felt like it was likely that her left lung has also started to inflate some, in addition to shifting. Dr. Turner was not sure if we could tell that or not, but we are hopeful that it has because this is the kind of progress we will need to continue to see in order for Clara to survive.
Dr. Turner was very honest and realistic with us today as well. He reminded us that this surgery is “no walk in the park.” Performing surgery while on ECMO is always a risk because Clara is on a lot of blood thinners right now. And even if they’re able to perform surgery successfully, that is not at all the end of the road by any means. I think the last week or so we have been so focused on getting her surgery done that we may have forgotten this important fact: the surgery can fix what caused the problem, but it doesn’t fix the problem itself. They are able to get all of her organs in the right place and sew her up, but that won’t matter unless after surgery, Clara’s lungs begin to grow and fill with air and start working. And unfortunately, there is nothing medical that we can do to make that happen. There is no medicine to give her or procedure to perform that will cause that to happen. We just have to trust that she will be a fighter and that her spirit will want this! We truly need God to breathe His breath into her. You see, moving all her organs doesn’t change the size of that tiny left nubbin and it won’t make her lung instantly inflate and start working. All we can do is provide the support of ECMO and ventilators and give her the time to begin doing that on her own. And as I’ve mentioned before, ECMO is a race against complications. It is likely that after surgery Clara will get sicker before she gets better. We need her lungs to start working before other problems start arising so that we can wean her off of ECMO and off of ventilators and off of other support. In short, we still have a very long road ahead of us. I think we’re so accustomed to surgery being the final step that fixes things, but with CDH it is just one hurdle along the way. Today our eyes were opened to the many hurdles we will still have to jump over after surgery.
Speaking of opening eyes… today Clara really opened her eyes. This is a result of the sedation being lowered, but it sure was wonderful to see those two big beautiful blue eyes squint their way open and hold contact with mine. I whispered to her over and over again, “Hi, Clara! I’m your mommy and I love you!” I can’t tell you how it makes my heart soar.
We also read books and sang songs today and I treasure each moment of my hand held inside of her tiny little fingers. I’m so thankful that Clara is not a “touch-me-not” like some CDH babies and that I have the privilege and opportunity to spend these sacred moments with her each day. I am thankful for my precious baby girl.
Thank you all for your continued prayers. I know this was a dense update, full of medical jargon and explanations. I felt a little heavy today after talking with Dr. Turner. I’m so grateful that surgery is scheduled and remain optimistic for Clara. But it was certainly a reality check for me that we do have a very sick little baby here. The stability of the last few days has been an answered prayer and we are so grateful for the time of rest on ECMO. However, after surgery we will need more than rest and stability; we will need to see our girl start to really fight for her life.
Things for you to pray for: Praise God for her continued success with small goals along the way like the reduced fluids and the shifting (maybe inflating?) left lung nubbin. Please pray for her surgery on Thursday. Pray for the team who will be operating on her and for no complications from the surgery. Pray that it is as simple as possible – that perhaps no patch will be needed and that her organs will fit back inside her belly during this surgery so that a silo and a second surgery will not be necessary. Pray that she will remain strong after surgery and that there will be no backsliding or if there is that it would be minimal before she starts improving again. Pray that she will be a fighter and that she will have a spirit of tenacity and a strong desire to fight for her life. Pray that her left lung will gradually grow and inflate and that her pulmonary hypertension will resolve. Pray for our family as we continue to try to listen and understand the doctors and as we pace ourselves for this long road ahead.
We truly believe that the thousands of focused prayers on the day of her birth were behind her successful entrance into this world and that your continued prayers in the days since have been what has kept her safe and stable while on ECMO. Don’t stop now! We’re looking into setting up another intercessory prayer webpage to allow you an opportunity to sign up again for a slot of time to specifically pray for Clara on Thursday. Stay tuned for that.
Also, I want to mention that Thursday (March 31) is National CDH Awareness Day. We think it is no coincidence that the day Clara will have her operation is a day that CDH families across the country are trying to help raise awareness about this birth defect that affects so many children (1 in 2500), has such low survival rates (nationally only about 50%), is as common as spina bifida and cystic fibrosis, but that very few have heard of. We hope that Clara’s story has helped to spread the word about CDH. In her support (and in support of all CDH babies), national CDH organizations are asking people to wear turquoise on Thursday and help spread the word about CDH.
"But they that wait upon the Lord shall renew their strength; they shall mount up with wings as eagles; they shall run, and not be weary, and they shall walk and not faint." (Isaiah 40:31)
"For I will restore health unto you, and I will heal you of your wounds, says the Lord."
"Behold, I will bring you health and cure, and I will cure you, and will reveal unto you the abundance of peace and truth." (Jeremiah 33:6)