Elise was born on Tuesday, October 18, 2011. She was five weeks premature and weighed 5 lb 9 oz. During her 14-day stay in the NICU (neonatal intensive care unit) she battled low oxygen saturation, high sodium levels, pneumonia, trouble feeding, and jaundice. She was briefly placed on a ventilator, then supplemental oxygen, and was also put under the phototherapy lights for a brief time. When we took her home, we were told that she was healthy and just needed to "plump up." We were thrilled to be bringing our baby girl home and had no reason to suspect an underlying threat to her life when her beautiful life was just beginning.
Elise was not sick at that time, so her diagnosis caught us very much off guard. I remember bringing her to her pediatrician for her "well baby" check and 2 month immunizations. Her doctor was literally leaving the exam room, and then almost like an after-thought, she came back into the room and said, "Do you think her eyes still look a little bit jaundiced?" I responded, "No," but the doctor was already writing an order for blood work. The blood work yielded results of a significantly elevated conjugated bilirubin level, and so we were sent to TX Children's Hospital for an immediate admission and work-up for "Biliary Atresia."
After about one week of testing, including blood work, numerous abdominal ultrasounds, X-Rays, HIDA scan, upper GI series, and finally a liver biopsy, the diagnosis of Biliary Atresia was confirmed, and she went to surgery for the "Kasai procedure," which is an operation that should "buy time" until a transplant is needed by removing the gallbladder and connecting the liver directly to the small intestines, allowing bile to flow and to be excreted rather than backing up into the liver. The surgery took 6 1/2 hours, and it was without a doubt the longest hours of my entire life. She was extubated successfully shortly after surgery, and was returned to the neonatal intensive care unit, where we were reunited. Oh, I couldn't wait any longer to hold her. I wanted to feed her too- immediately- but it would be three more days before that was allowed.
While in the hospital, we met with numerous GI doctors and Hepatologists (liver specialists), a dietician, and a social worker, and we were educated on the BA diagnosis and related issues. The entire team emphasized the importance of nutrition and weight gain. Elise was prescribed a special high-calorie formula- Pregestimil- designed for babies with fat malabsorption issues. We learned that malabsorption of nutrients is a common problem with Biliary Atresia. Elise was also placed on a oral multivitamin, AquADEKS, and an additional Vitamin D supplement. We were also warned about a significant risk of the Kasai operation- Cholangitis- bacterial infection of the liver. We were advised that any high fever should be reported immediately to the GI/liver team at TX Childrens Hospital and would most likely warrant a trip to the hospital in order to rule out Cholangitis.
Elise was discharged five days after her Kasai, but was readmitted to TX Childrens Hospital about one week later due to high fever. She was diagnosed with Cholangitis and was found to be septic, meaning there was bacteria in her blood causing her vitals to be elevated. After three days of positive blood cultures, the infectious disease team was consulted, and Elise was started on a different IV antibiotic, Meropenem. Elise had a PICC line (peripherally inserted central catheter) inserted and was discharged to continue the 21 days IV Meropenem at home.
Now that Elise is home, we are enjoying her and trying to treat her like any new baby. Of course, we have to give her meds throughout the day, and she is on a very strict feeding schedule in an effort to have her gain weight. We have liver clinic appointments at TX Children's Hospital every couple of weeks, and weight checks in between. I have become obsessed with Elise's labs, specifically her liver panels. I pray for her liver enzymes to continue on a downward trend towards normal. More than anything, I pray that our little Elise can enjoy a long, healthy life.
Yes, our life and daily routine has changed dramatically, but hopefully it means that Elise's life will be as normal as possible. It is my personal mission to ensure that BA does not hold Elise back in any way. And so I embrace my new role as a "Liver Mom," and the purpose of this blog is to document our family's experience in Liverland.
ADDENDUM:
When Elise began experiencing complications related to her liver disease including ascites, portal hypertension, and nutrient malabsorption, the doctors at Texas Children’s Hospital in Houston, Texas, recommended a life-saving liver transplant. She was officially listed for transplant in June 2012. As time went on, Elise encountered more serious and life-threatening issues related to liver disease such as esophogeal varices and upper GI bleeds. On March 23, 2013, Elise received "the call," and she received a liver transplant, her "gift of life."
