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Post by Amanda Walker

The CF Waiting Game

Our waiting started July 3rd, 2016. I was 33 weeks pregnant with our son, Emmett, and was sent to the ER for some mild spotting. Once admitted, they saw I was having contractions which they were able to stop. This was my third pregnancy and my first two, with our beautiful daughters Presley and Audrey, had gone smoothly. This time however, I had gestational diabetes. After doing an ultrasound, they noticed Emmett’s heart wall may have been thickening. I was ready to leave the hospital the next morning and the nurse came in and asked if the doctor had been in to talk to me. She hadn’t, so the nurse had to break the news that I would be staying put for the remainder of my pregnancy, so they could keep close tabs on Emmett. This meant 3 weeks (I was high risk and they had already decided to take him at 36 weeks via c-section) more of waiting. The days dragged on at the hospital. I enjoyed every visit from friends and family, and I would try to chat with the nurses to pass time quicker.

Tuesday, July 26th, 2016 was FINALLY here! Emmett’s Birthday! The wait was over! Or so I thought. Emmett was born at 7:11 am. We enjoyed holding him and introducing him to our family and his big sisters. We celebrated and loved on him. We began noticing he wasn’t having an easy time feeding and would gag and spit up quite a bit more than usual. It had been 24 hours since birth and he had not passed any meconium. We waited. 48 hours passed. The nurse decided it was time to get an X-ray. It showed his bowels were distended. They took him to the NICU for an enema, hoping to move things along. We waited all night. At 6am the doctor called us and told us to come downstairs to the NICU, so we could talk. Our minds raced as we went downstairs. The doctor told us they thought Emmett had a bowel blockage or Hirschsprung’s Disease. We were told that he needed to be transferred to another hospital two blocks away, so he could have surgery. At this point I was distraught, thinking ‘here I was still recovering from surgery and my precious boy was being taken down the street to another hospital to have surgery’. We waited with him in the NICU for about an hour until the ambulance came to transport him. Once he was retrieved, we called my OB and she released me early so we could go be with Emmett.

They decided to do Emmett’s surgery the next day. We held him and waited for him to be taken to surgery. Friends and family came to sit, pray, and be with us while he was in surgery. We waited. The surgeon walked in and took my husband and I into a small room. He was straight forward. Emmett did not have a bowel blockage, he had what was known as Meconium Ileus and “could mean he has Cystic Fibrosis”. He sketched us a quick picture of how he repaired Emmett’s intestines and walked out. And with that single “off the cuff” statement, he blew up our whole world.

My mind raced! What could this mean? Could he really have that? Our girls were healthy! We walked out and told everyone what the surgeon had said. We waited for Emmett to come down the hallway after surgery, so we could see him pass by in his isolette. As the time passed, I googled Cystic Fibrosis….HUGE mistake!

In the following days, Emmett was recovering nicely from surgery. The hospital had sent a DNA test off to confirm CF. There we were, again, waiting for the results, anxiously. Days passed, Emmett got stronger and stronger and was able to tolerate bigger feeds. We received a letter in the mail concerning one of his newborn screens. It came back with an elevated IRT level. Another sign pointing to Cystic Fibrosis. The DNA test had not come back, so there was still hope they were wrong. I secretly wished, while praying to God, that Emmett did not have CF. And we waited some more. It took well over two weeks for us to confirm his diagnosis. Emmett had been transferred back to the NICU at the original hospital. Every day that passed, I thought about the lab person who may know Emmett had CF before us as we torturously waited.

August 13th, 2016 the DNA test results finally came in. Emmett, indeed, did have Cystic Fibrosis. He had two copies of the Delta F508 gene. We were crushed, but I was (still am!) determined to give Emmett the best life possible and do everything within my power to ensure that he is as healthy as he can be. We learned about enzymes, vitamins and had a whole list of drugs he would need to start when we were discharged. I was so overwhelmed but one of my best friends suggested a chart for keeping track of his feeds/meds. It was perfect and so helpful those first few weeks. More days passed, and we waited to bring home our sweet boy.

August 19th, 2016 Emmett was discharged! We brought him home and waited for our first Pediatric CF clinic visit at Vanderbilt Children’s Hospital. For that appointment, we even made a list of all of our questions to ask. It was such a great feeling after that initial visit. Questions were answered, and we felt like we were ready to tackle CF head on and help Emmett stay healthy!

In September of 2016, Emmett’s blood tests showed concerning liver numbers. We waited a few more weeks to see if they liver numbers came up on their own. They were still too high. It was determined he needed a liver biopsy done. I remember the dread leading up to that biopsy. The night before, I remember looking at our precious boy sleeping and thinking how grey in color he looked; something that I hadn’t noticed it before. The biopsy went great. We waited on the test results while Emmett was in post-operation observation. I remember the intern coming in and giving us the results in a roundabout way. Emmett’s hepatologist was out that day, so an intern had to tell us the results. I could tell it was not good and directly asked if he was going to need a liver surgery. Yes, he was.

A week passed, and we waited for surgery day. We had pre-op visits with his CF team, hepatologist, surgeon and anesthesiologist. The amount of worry and waiting during this time was immeasurable. The surgeon played out three different scenarios on how this surgery was going to go. He wouldn’t know which route for sure until he had Emmett opened. The worst option was a “Kasai” procedure, the easiest being a medicine injected into his bile ducts to clear them out. The latter option was most likely not going to work according to his surgeon and hepatologist who mentioned that on multiple occasions. The day of his surgery arrived. I was so anxious. I felt sick. Emmett was prepped and ready for surgery. I remember the feeling of my heart being on the gurney when Emmett was rolled down that hallway. It was one of most helpless feelings of my life. We went to the waiting area and sat with precious family and friends. The nurse called to check in and tell us things were going well but that the easy option was not successful, and they would have to move to the next option. Tears flowed instantly as we sat in the waiting room. Ten minutes passed, and the nurse came on the phone for us again. She told us surgery was over and the surgeon would be out to talk with us. We were shocked and scared! How could surgery have been over so soon? I felt nauseous as we walked down the hallway to the meeting room. We went to the private room and the surgeon walked in grinning. So, it must not be terrible news. He told us the first procedure had worked!! We were ecstatic and confused because the OR (operation room) nurse had told us wrong on the phone! It was almost as if a weight had been lifted off our shoulders. We cried tears of joys with friends and family, celebrated and prayed bountiful thanks.

Thankfully, Emmett’s 3 years have been mostly uneventful. Recently, we went back for his yearly ultrasound and hepatologist appointment. It has been routine and, truthfully, I was thinking we would be in and out and all would be fine. CF catches you off guard time and time again. His doctor told me the ultrasound had shown his liver had stiffened, due to CF. I was stunned even though I knew he had some liver damage from the very beginning. His labs during the year had not shown further damage. His doctor then mentioned eventually his liver will become cirrhotic. Again, I immediately thought HOW LONG? We are just going to sit back and wait for his liver to fail?! I’m sure I asked her in about a million different ways how long until that happened. She didn’t know. She told me she thought it would be a long and slow process, but nothing was certain. Another situation where CF consumes your thoughts and torturously makes you wait. So again, we wait, and pray and hope that it is a long and slow process.

It is agonizing to have the constant worry in the back of your mind over every small detail. Did I give him enough enzymes? Did he touch something germy? Did someone cough too close to him? Will his liver hold up (until hopefully he is an old man needing a liver transplant)? These questions and a million others are what can drive you mad. It literally feels like waiting for something to go wrong which is one way you could look at it, but, you can choose to have a positive outlook. I choose to be thankful for every single second we have with Emmett. Cystic Fibrosis makes you learn to live more intentionally – something for which I am extremely grateful.

Emmett’s first few months were something like the worst rollercoaster you’ve ever ridden in your life. There were so many emotions, highs and lows. But one thing that seemed evident was cystic fibrosis is a big waiting game. During the highs of good health, it seems like you are constantly waiting on the fallout. When everything seems to be rainbows and sunshine, you worry about what lab work or illness is lurking around the corner. What the waiting has taught me to do is: be even more grateful for the good health when Emmett has it and to cherish the time we have when things are going well! The waiting is an excellent reminder that there is no time like the present. Live intentionally! Don’t be so pre-consumed with the worry of CF that you forget to savor every day! Live boldly, because the clock will always be there regardless.

Other posts by Amanda Walker

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Our Community Column is a home for thoughts and opinions of members across the cystic fibrosis community. Sometimes serious, every so often lighthearted, but always engaging. It is our duty and privilege to listen to these stories and to share them with others.