Tuesday, January 20, 2015

Joshua's Story

(I apologize for the redundancy. Earlier posts have presented very similar information, but this composition was put together recently for a publication. We are very pleased with how it turned out and would like to share with our friends and family to read once more.) 

In the fall of 2005, my husband and I were delighted to find out I was pregnant with our second child. Like everyone does, I had scheduled an appointment with the obstetrician at 20 weeks to discover whether we were having a boy or a girl. This is typically the most exciting prenatal doctor's visit of them all. Lying on the sonogram table, I could see on the technician's face that there was something wrong, terribly wrong. Her words, "You’re having a boy, but there's a problem." echoed over and over in my ears as she left the room to get the doctor. Excitement quickly faded into sheer terror. My husband and I were left alone in the room for what felt like an eternity. The doctor entered the room with an apologetic look on his face. "Your baby has gastroschisis, and I'm referring you to a specialist." Gastroschisis? I had never heard the word before. I thought my world had ended. Gastroschisis (gas-tro-skee-sis) is a rare birth defect in which some or all of the baby's intestines protrude through a hole in the abdominal wall. At that time, the experts reported that 1 in 5,000 babies born in the United States were diagnosed with this birth defect. We happened to be the lucky one. Leaving the doctor’s office, I remember calling my mom and hysterically trying to explain what was wrong. The rest of the day was a blur. The word "problem" kept circling in my head. Just what did all this mean? What was in store for me and my child? No one knows why it happens, there is no known cause or prevention for it. I knew I needed more information, after all, knowledge is power and at that point I was feeling extremely powerless. Unfortunately, as all newly diagnosed parents are told not to do, we jumped online and found statistics that only fueled my fears. The perinatologist I was referred to actually ended up referring me to get a second doctor because he did not feel comfortable caring for me and my unborn child. This ended up being a blessing in disguise because the doctors that began following me would be crucially instrumental in saving our son's life. Even though I knew I was not carrying a "normal" baby, my pregnancy progressed fairly smoothly. I was seeing a high-risk doctor twice a week and it became so routine that I was attending these appointments solo. Sonograms showed the small intestine that was left inside his body was becoming extremely dilated meaning the bowels were blocked off at some point. Blood flow was restricted and there was little to no peristalsis (muscle movement) in the bowel. The doctor wanted me to deliver early, so at week 33 two rounds of steroid shots were given to reduce the baby's chances of respiratory problems. We were planning for a 34 week delivery and an amniocentesis was scheduled for the following Monday. Friday afternoon, April 28, 2006 I was in for yet another "routine" appointment when once again during a sonogram the room got eerily quiet. The perinatologist explained to me that he was not happy with the baby's heart rate and wanted me to go to the hospital's maternal observation. He instructed a nurse to walk me over right away. When we arrive on the floor, a very stern old nurse scowled at me and asked why I was on my feet. A team of nurses quickly sat me in a wheelchair and whisked me off into a delivery room. My head was spinning. In no time nurses were bustling about asking me questions and preparing me for surgery. Before I knew it, I was lying in a bed with an IV started the whole time thinking, "Wait, you are all making a huge mistake. You've got the wrong person!" Finally, one of the nurses asked me if I was there by myself. When I replied yes, she handed me a phone to notify loved ones. It is a miracle I was able to dial anyone’s number. I was only able to reach my mother-in-law, my mom, and my preacher. I had never been so scared in my entire life. Only twenty minutes had passed from when I was getting my "routine" sonogram to that point. We were quickly leaving the delivery room, and I was wheeled into an operating room. I will never forget how cold and white it was. So sterile. Looking up at the ceiling while nurses counted utensils, I couldn't even cry. It was so unreal. I remember thinking, "I'm so alone, where is everyone?" No one came and panic was setting in. Almost as if I heard a voice, something told me, "Get it together, you're not alone." A peace settled over me that I cannot explain with words. I have never been closer to God than that very moment. The surgery itself was a blur. So much bustling about. At times I struggled to breathe. I remember the tugging and pulling and a heaviness on my chest. In no time at all, Joshua was born. It felt like an eternity before a nurse told me to turn my head to the right so that I could see him as they wheeled him out the door. Oh, our baby. He was wrapped in sterile gauze and was being rushed out of the room in an isolate. Just a glimpse, is all I caught. Joshua was born with nearly his entire small intestine on the outside of his body. That section of the bowel had died. His small bowel that remained in his abdomen (about 12 cm) was very dilated due to lack of adequate blood flow. As I was recovering from the emergency caesarean, the NICU team was preparing Joshua for surgery. His surgeon came to me just before they took him across the street to our local children's hospital. With the most solemn of looks on his face he told me, "This is bad, this is very bad." The herniated section of Joshua's intestine would need to be removed, and the part remaining (minus the oh so important ileocecal valve) was so grossly dilated that it was too large to connect to his large bowel. The surgeon was able to taper down the remaining small intestine in the first of three Serial Transverse Enteroplasty (STEP) procedures. On top of that, his bowel was extremely large and would have to shrink. It would have been like trying to connect tubing the size of a quarter to tubing the size of a pencil eraser. He was in for a long stay in the NICU and an even longer journey for survival. A portion of Joshua’s small bowel was brought out through a stoma (an opening) in his abdomen. His ostomy was in use until he was about one and a half months old. At that point it had shrunk in size and was ready for reanastomosis (reuniting of divided vessels.) Two months later he was suffering from violent episodes of vomiting and retching. An upper GI, small bowel follow-through with contrast was performed and showed a very dilated small intestine. Doctors decided to once again perform a STEP. The procedure successfully gained Joshua another 25 cm. His bowel was roughly 75 cm long and the bacterial overgrowth that is so common with these kind of gut issues, was now a little more manageable. After 168 days in the NICU, Joshua came home diagnosed with Short Bowel Syndrome, chronic diarrhea, malabsorption and failure to thrive. He was home on IV nutrition, g-tube feeds, and medications around the clock. Caring for my new baby meant becoming a nurse. It meant becoming an expert on my child, and so I did. He did wonderfully for a while. About three and a half months after leaving the NICU, vomiting was once again a very serious problem. So little was going in, but SO MUCH was coming out. Joshua was getting very dehydrated and had to be seen immediately. While in the hospital, tests were done to check for severe reflux. He had a ph probe inserted through his nose down his esophagus. Each time he vomited, retched, coughed, cried, pooped, moved...pretty much did anything, we were to enter the information into a device attached to the probe. The results were conclusive. Yes, Joshua did have severe reflux. So, at that time it was decided to do a fundoplication (a procedure where the upper part of the stomach is wrapped, or plicated, around the lower end of the esophagus.) At the same time, Joshua's surgeon repaired a minor hernia and did his third, and last, STEP. Joshua then had over 100 cm of small bowel. Unfortunately, the length did not necessarily cause the bowel to work better. Our underlying and persistent issue remained the same, malabsorption. The STEP procedures have helped, but time and constant encouragement of the bowel's activity seem to have made the most difference. There has not been any other major surgeries to date, and we pray it remains that way. I have asked about the possibility of a fourth STEP. Many of the experts we have seen feel like it would only be a temporary fix. I have to agree with them. God has created the body in such a way that it will adapt to high stress situations and injuries. His bowel seems to "know" it does not have adequate surface area to absorb enough nutrients to survive so it stretches creating a wide, dilated bowel. Unfortunately this bowel is sluggish and a breeding ground for bacteria which in turn causes lots of gas/bloating. This bacteria often finds its way into the bloodstream and can strike up a nice case of sepsis quite easily. The STEP is a major surgery and anytime the bowel is touched, it undergoes a state of paralysis. The thought of that possibly becoming a long term reality is not worth trying once more to gain more length just to most likely end up dilated. Through the years, we have attempted to treat Joshua's malabsorption of nutrients with medications. Some of those medications have tragically caused pancreatitis. Pancreatitis is a very painful condition of an irritated and inflamed pancreas. Two episodes of this condition proved to be major setbacks in Joshua's advancement. At the age of 5, we were concerned that he would not ever be able to grow and thrive without the supplements of medicinal nutrients. His electrolyte imbalance due to his chronic diarrhea was the hardest to control, especially in the warmer months. On top of that, he just was not growing. Doctors agreed with me that it was time to pursue growth hormone therapy. I remember having read somewhere that studies had shown growth hormones actually aided in electrolyte absorption, and I was anxious to see if this was a possibility for Joshua. We started hormones in May 2012, and to this date Joshua has not darkened the ER doors for dehydration. This is a miracle for him. As a bonus, he quadrupled his growth from the previous year. Today, my husband and I celebrate every moment; we take nothing for granted. The road we've traveled was so long and strenuous, but we've all pushed on. Joshua no longer depends on IV or tube fed nutrition. His biggest concern in life is to be just like other boys his age. He and his two brothers are competitive in every way. The strategic balance of proper nutrition, medications and parental instincts along with the aid of growth hormones has given him a chance. A chance to be more "normal,” a chance to survive, a chance to thrive. Joshua's journey continues as he lives every day to its fullest.

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