Evan's Web site

In April, 2002, our third son, Evan Matthew was born. Ironically, this date is also my older brother's birthday. What was supposed to be a happy, joyous moment in our lives turned out to be a whirlwind of medical fright. Just twelve hours after first holding Evan in our arms, he was whisked away to our state’s children’s hospital via their ambulatory transport team.

The team arrived for him around 5:45 pm; I kissed my newborn good-bye, squeezed my husband’s hand and watched helplessly as they wheeled Evan away in an isolette. Although Evan was quite stable at that moment, the cardiologist felt the heart defect he heard needed immediate repair. The wires attached to all the medical equipment seemed to entangle his entire tiny frame. Weighing in at just five pounds and fifteen ounces and only measuring eighteen inches long, he seemed very fragile. In the months to come, our local cardiologist and the children’s hospital cardiologists had an on-going debate whether Evan’s congenital defect was Tetralogy of Fallot or pulmonary stenosis. We didn’t care what they called it---we just wanted it fixed. Five minutes after the transport team pulled out of the Women’s Hospital parking lot, my husband and I were on our way home to say good-bye to Trevor and Trent. I dreaded explaining the situation to his very proud big brothers.

The three and one half hour drive from our home to the children’s hospital was grueling. It didn’t help that I myself was just fourteen hours post partum. We arrived around 11:45 pm. I remember thinking, “Let’s just go straight up to his room so I can hold him!” As we entered the NICU for the first time, alarms were beeping, machines were humming and nurses were tending to the smallest babies that I’d ever seen. Evan’s isolette was situated just inside the door of the unit. The cardiologist on call had just finished reading his echocardiogram. She explained that Evan would need a shunt the next day so that his lungs would receive adequate oxygen. The shunt was a temporary fix to a major heart problem that would need to be evaluated as he grew. I heard everything she was saying, but it took awhile for it all to sink in.

So at just over one day old, Evan had his first major surgery. Our whirlwind of medical fright seemed to be calming down. Just seven days after his heart surgery, we brought Evan home for the first time. The nurse who walked us out told us she’d never had a baby with Evan’s type of defect leave the hospital this early. We gave God all the glory. The eight days we were at the hospital our faith in God’s power to heal helped sustain us. We knew that our God is a healer and that Evan would be okay. Each day that he progressed, I was reminded of the meaning of his name. Evan means “strong warrior” and Matthew means “gift of the Lord.” At the time we chose his name, little did we know that he would need to be a strong warrior fighting for his life.

I wish I could say that Evan’s troubles ended here, but unfortunately his tumultuous medical storm didn’t end with the heart surgery. Just a little under six weeks later, his cardiologist asked our pediatrician to test Evan’s liver function. Evan had been quite jaundiced for almost three weeks by then. “Not again…hasn’t his little body been through enough?” I thought to myself. The blood test was performed on a Thursday and the results came back on Friday showing an elevated bilirubin count. During a phone call, Evan’s pediatrician explained that there are two types of bilirubin, direct and indirect. Many children who have elevated indirect bilirubin counts can be placed under a “bili light” and all is well. However, Evan’s bilirubin count was elevated due to the direct bilirubin and this is an indication of greater significance. Evan was going to need immediate diagnosis to rule out liver problems. “Okay, we can handle this; God always keeps his promises.” The diagnostic test to rule out liver problems is a HIDA scan. Evan’s scan showed that his liver was not emptying bile into his small intestines. This was what our pediatrician had feared. He sent us back to the children’s hospital for the second time. (Later, we found out he should have been given phenobarbital for 5-7 days to aid in bile production. Babies this small can have false results from the HIDA scan if their livers aren’t “primed” with the drug prior to the scan.)

I’ll never forget the first and second week of June 2002 as long as I live. For the second time in his short life, Evan had another major surgery. In order to accurately diagnose congenital liver defects, an infant’s stomach is opened up so the liver can be biopsied. Long story short, the surgeon informed us that our little baby would need a liver transplant because he had biliary atresia (BA). With BA the body’s biliary system eventually becomes so damaged that the injury to the liver is irreversible. Even with this horrific news, Dirk and I knew Evan was going to be all right. We weren’t going to let the devil waiver our faith and steal our peace. God keeps his promises. He is the Alpha (beginning) and the Omega (end). I can’t say that I didn’t grieve. My heart ached that Evan had to be in so much pain. Dirk and I prayed over him daily.

After five days of recovery, we were sent home to await the big transplant team consultation. Later we discovered that we had to wait two weeks to see these doctors because they were on vacation. Ironically, we never made it to that appointment because God intervened. A couple days after returning home, something inside me said to call our insurance company. This one little phone call saved our son from going through a liver transplant. As it turned out, our state’s children’s hospital was not on the list for acceptable transplant sites. We had to choose either Chicago Children’s Memorial or Cincinnati Children’s Hospital.

For the first time since Evan’s birth, I remember feeling totally overwhelmed. I began crying uncontrollably. I hated the thought of Evan having to change hospitals and change doctors. God is so good though. We soon found out that this horrific news was actually a blessing. The case manager nurse at my insurance company encouraged us to take Evan to Cincinnati so I called immediately. The nurse from the GI department in Cincinnati was great. After gathering all of Evan’s information she called back within an hour. Several bits of information had jumped out at her during our initial conversation. Upon her return phone call, she said Dr. B wanted to re-evaluate Evan. Many of his medical symptoms seemed to relate to another liver disorder, not biliary atresia.

Many of the medical issues I had shared with the Cincinnati nurse were also expressed to the doctors from our state. My husband and I both wondered why were these same details jumping out at Dr. B but had been disregarded by the doctors back in June? Evan had spinal cord anomalies, a swollen left kidney, liver dysfunction and a heart defect. Dirk and I had read on the Internet that these problems tend to follow a syndrome called Alagille. As we had suspected, Dr. B wanted to rule this syndrome out before placing Evan on the transplant list there. Only 3-4% of Alagille patients ever require a liver transplant. So, on June 19, 2002 Evan had his second liver biopsy. This time it was in Cincinnati and this time he was not opened up. It was simply a needle biopsy.

The day before he had the procedure, we met with Dr. B. God put him in our path. Since he is the head of the GI department, Dr. B happens to only take new patients every now & again. The best part is that he is also one of the top three doctors in the United States for dealing with Alagille syndrome. Even before the liver biopsy, he was 99% sure Evan did not have biliary atresia. A biopsy that showed 30-50% of the biliary ductules indicates Alagille syndrome. As he had suspected, Evan’s biopsy the next day showed 30%. Just weeks earlier, the other pathologist saw zero ductules. We know this was God’s work. We thanked God for this good news, but knew that God had even greater things in store for Evan. Another finding that is one of the indicators for having Alagille syndrome is a thickened Schwalbe’s line. Dr. B had us see an ophthalmologist right away, and Evan’s line was indeed thicker. Medically, this eye finding causes no problems; it is just another piece of the puzzle to help diagnosis Alagilles. We wondered why this simple eye exam wasn’t performed before Evan was put through a surgery back in June.

Evan was discharged from Cincinnati on June 20th weighing 9 lbs. 4 oz. & measuring 21 ½ inches long. We continued our daily regimen of prayer and medicine for him. He had to take vitamins A, D, E, & K, Poly-Vi-Sol with iron and Actigall to help with the liver issue. For his heart, he was placed on a blood pressure medicine, Atenolol. To prevent clotting around the shunt in his heart, he had to take baby aspirin. Along our journeys, Evan had also been diagnosed with bladder reflux. He is supposed to be re-evaluated at one year to see if it is gone. So, finally to prevent an upper urinary tract infection he was prescribed amoxicillin. Just three weeks later (he was 3 months old) at his check-up appointment he had grown 1 ¼ inch and gained 1 lb. 4 oz. We thanked God again. The doctor said most Alagille patients do not grow this well. The rest of this check up was exceptional as well. The doctors were so pleased that we didn’t have to return for three months.

Seven months later…

I wrote the beginning of Evan’s story back in August of 2002. I wanted to have everything fresh in my mind and believe it or not, I have typed the shortened version for you! Evan is now going on eleven months old and is progressing as God has promised us. In my prayers, God often reminds me…he is the Alpha and the Omega, the beginning and the end. We believe the wonderful gift of life from conception is from God (the beginning). Satan might have interrupted Evan’s development in my womb, but God is the Omega and he will finish and complete everything that has been stolen from Evan. We know Evan will have his inheritance of complete health restored to him. Evan saw the liver doctor in August and was doing so well they said, “See ya in February.” He now weighs nearly 16 pounds and is 27 1/4 inches long. He registers on a “normal” baby chart for his height. I mention this because we were told that Alagille patients do not normally register on a “normal” growth chart. Recently, he hasn’t been putting on the weight as quickly as we would like for him to so he is now taking Peptamen Jr. along with his Pregestimil. The Peptamen Jr. has more MCT oil, which his body digests more easily. He is a very happy baby. He is crawling everywhere, loves to pull up to things, and loves table food! We praise God every day that Evan does not itch from the cholestasis like many liver patients do. He still looks yellow, but we know that it will soon fade.

On February 4th Evan had a heart catheterization to check the pressures in all the chambers of his heart. After the heart cath., they decided to schedule his heart repair for April 14th. Having a child go through this medical fright is horrific, but with God on Evan’s side, we know all things are possible. God has a mighty plan for his gift to us, Evan Matthew…Strong Warrior.