Lyra's Intestinal Malrotation Story
Lyra Faith was born via c-section after a very long labor on August 22. She had a fever and stayed in the hospital on IV antibiotics for the next four days. The hospitalist was never sure what caused the fever but her labs cleared, she passed her meconium stool, and we took our beautiful girl home.
At one week old, Lyra was diagnosed with severe reflux, or GERD. At one month, she had only gained a few ounces and her pediatrician decided she needed formula to add more calories to her diet. Like many babies, Lyra had tummy aches and spit up quite a bit, but constipation was forever our biggest struggle.
At Lyra’s two year well child check-up, I mentioned how frequently she urinated. She had always had to pee at least every 30 minutes. I had been around children my whole life and knew that seemed abnormal. I didn't think to ask about anything GI related but wondered if there was any concern with her kidney function. The pediatrician felt Lyra’s stomach, without doing any scans he told me how impacted she was and said it was likely putting pressure on her bladder. He suggested we start daily laxatives and probiotics with Lyra. Aside from her issues with constipation, our little girl continued to be rambunctious, smart and healthy.
On the day before Lyra’s fifth birthday she woke up later than usual and asked for a bowl of cereal. In the middle of eating, she got down from the table and immediately started screaming in pain. She yelled that her belly hurt and she felt sick. My first reaction was that she needed to use the bathroom. Impaction due to Lyra’s issues with constipation was not foreign to us and severe stomach pain accompanied it most of the time.
Lyra used the bathroom and was able to have a bowel movement, but the moment she stood up from the toilet she began to vomit. Not long after, she started sweating, vomited again and began writhing in pain. Lyra’s symptoms scared me, but my husband was at work so I called my mom to come over and give her opinion. By the time my mom arrived 20 minutes later Lyra’s skin was almost ashy gray, her hair and clothes were soaked with sweat, she had vomited seven times, and she kept tossing and turning, crying that she couldn’t get comfortable.
I made the remark to my mom, “If I didn’t know better, I would think she had a bowel obstruction, but I’m sure she doesn’t because she hasn’t thrown up anything bile colored.” I had worked in hospital administration for the last nine years and completed five semesters of nursing school so I had some basic medical knowledge. However, the knowledge I had was limited, I believed an intestinal obstruction always presented with green bilious vomit.
Lyra had vomited so much, I was certain she was dehydrated. I decided to take her to the local emergency department for fluids. When we arrived at the hospital I told them I thought she had a severe stomach bug and needed IV fluids. Due to Lyra's level of pain, lack of bowel sounds and a distended abdomen, the emergency physician decided to do further testing. After a CT with contrast they came in to tell us that an ambulance had been called and Lyra would need immediate transfer to the children’s hospital for surgery. I was floored when they said she had a bowel obstruction. I was convinced up until that point that Lyra couldn’t have anything other than a stomach flu.
The doctor put in an additional IV site, placed Lyra’s very first NG tube, and within 25 minutes we were in emergency traffic. My husband and son, parents, in-laws, sisters, and grandparents all followed the ambulance. When we arrived the lead pediatric surgeon literally ran into Lyra’s room and gave us a diagnosis we could not wrap our heads around. He said our sweet girl had been born with a congenital defect of the intestinal tract known as intestinal malrotation. This defect had caused her intestine to twist creating a very dangerous intestinal obstruction known as a volvulus. We had 10 minutes to digest all the information, which included a surgical plan, risk factors, and the fact that the surgeon had no idea what he would find when he opened her up.
We were wheeled up to the holding area at 11:57 pm. I rode on the gurney and held my beautiful girl, praying harder than I ever had before. At 12:00 am, Lyra officially turned five years old, and at 12:02 she was gone with the surgical team.
Four and a half hours later we were given the rundown on the extent of the damage to Lyra’s intestines. She had a closed-loop volvulus, a 12.5 inch intussusception, her mesenteric arteries had to be fanned out, and multiple areas had to be patched because of tears. The surgeon was able to do a Ladd’s procedure and had removed 13 inches of necrotic bowel. It was during this conversation that we learned the Ladd’s procedure did not fix intestinal malrotation, there was still a small chance Lyra could have a recurring bowel obstruction in her life.
For nine long days, Lyra wasn’t allowed anything to eat or drink and seemed to be deteriorating. She begged for ice chips or anything wet, we were heartbroken to have to deny her. Finally, after two weeks, she was discharged home with an order for daily Miralax.
Over the next six months it became obvious to me that Lyra was not recovering. Since her Ladd’s procedure it had became nearly impossible for Lyra to have a bowel movement. We finally found a pediatric GI who agreed to see her as a patient but nothing he did seemed to help. In addition to GI issues she had become tachycardic and was found to have hydronephrosis, a condition where one kidney doesn’t empty completely. We began seeing a cardiologist, a nephrologist, and a child psychologist as well as continuing to see Lyra’s surgeon, GI, and primary care doctor.
Lyra had numerous diagnostic tests which couldn’t find the cause of her symptoms, she had NG tubes put in four times, and she was hospitalized three times for partial obstructions due to both adhesions and constipation. She was taking three times the adult recommended dose of Miralax daily, probiotics, we had cut out dairy and gluten, she used a Squatty Potty to help better position her body when using the bathroom, she had taken Flagyl for months due to an H Pylori infection that would not go away, Prevacid and Zantac for reflux, enemas and magnesium citrate to help her use the bathroom. Lyra drank two PediaSure nutritional drinks each day just to try and maintain her already low weight. She developed PICA due to iron deficiency anemia and began eating chalk, then tissues. She was always exhausted, always.
It got to the point that Lyra would often vomit or pass out from trying so hard to use the bathroom. Our last straw came when she collapsed twice in one week while attempting to force the stool out. I started to fear Lyra would spend the rest of her life in pain, fighting for some sense of normalcy. My Jesus loving, kindhearted, sweet as sugar, five-year-old little girl would spend the rest of her life in and out of hospitals, taking medication to mask her symptoms. Everyone told me I should accept that life as we knew it was gone and this was Lyra’s new normal. I absolutely refused to believe or accept that.
I had done research on gut rehabilitation programs around the country and had joined several online support groups. There was very minimal information on intestinal malrotation. No one could tell me why Lyra was having the issues she was having after her Ladd’s procedure. I had made multiple physicians in multiple specialties upset because I questioned them. One doctor even insinuated that I wanted something to be wrong with Lyra when nothing actually was. I drove my husband crazy staying up each night grasping at straws, trying to make sense of what Lyra was dealing with. Finally I read about a transplant surgeon who had developed a radical new procedure which corrected the anatomy of patients with intestinal malrotation. Reading about this new surgery sparked a fire I would not let die out.
At the time, this surgical procedure had only been performed on adults but I still felt drawn to it. I contacted their office at the Cleveland Clinic and basically pleaded for someone to look at my daughter’s records. I cried as I explained how terrible my little girl’s quality of life was since her first surgery, and I begged for some insight. One week later, I received a phone call that Dr Kareem Abu-Elmagd reviewed Lyra’s records and scans and was certain he could help her.
I cannot relay how emotional I was after receiving Dr Kareem’s call. I did not know how, but I knew I had to find a way to get Lyra from Tennessee to Cleveland to see Dr Kareem. At this point in time I realized advocating for my daughter needed to be kicked in to a higher gear. I fought for months with Lyra’s health insurance to cover the surgery, eventually taking her case all the way to the governor’s office until it was finally approved. Our church and community helped by holding benefits for our family. On June 17th, almost 10 months after Lyra’s original surgery, we made the long drive to the Cleveland Clinic for what we thought was going to be a pre-op visit and consultation.
Once we met Dr Kareem and his pediatric coordinator, Sandy, our lives were forever changed. Dr Kareem told us something no one had told us before, “I can fix her.” This was hard to hear and very hard to believe given everything Lyra had been through up to that point. We were told Lyra would be the youngest patient to ever have Dr Kareem’s procedure, with the next youngest being a 19 year old.
When we had spoken by phone, Dr Kareem had planned to schedule Lyra’s surgery for two months after her pre-op visit. After evaluating Lyra in person Dr Kareem wasn’t comfortable waiting and suggested we move Lyra’s surgery up. After that office visit we had only three days to prepare ourselves. Anxiety hit me like a ton of bricks and never let up. Dr Kareem was very thorough in explaining how the procedure and recovery would go. His team was amazing at answering our questions and Sandy was with us every step of the way. As parents, knowing Lyra would be the first child still made us nervous.
The night before surgery, I questioned everything. “Should I go through with it?” This was not a “required” surgery. There were so many risks involved and I was putting her life in the team’s hands. I prayed and prayed for some sort of guidance. In the end I knew there was no other choice, if we didn’t go through with the surgery, Lyra would never have a happy childhood.
The morning of her surgery it was very hard to let Lyra go. Dr Kareem came in and hugged me, he told Lyra he loved her and would take care of her. During surgery a nurse kept me updated every hour, sometimes more. Dr Kareem removed adhesions and some necrotic bowel. As he operated he found what he thought was a benign tumor in Lyra’s small intestine which he sent to pathology.
Lyra’s surgery lasted almost nine hours. When they finally took my husband and I back to see Lyra in the PICU I was overwhelmed. There is no way to describe the feeling of seeing your small child sedated with tubes and IVs everywhere. There were feelings of guilt because we chose to put her through that surgery, feelings of relief to even see her chest rise and fall, and feelings of terror with every beep of the monitor that something could still go wrong.
After pathology came back several days later, we were all surprised to learn that rather than a benign tumor Lyra actually had a small, growing, second stomach inside her intestine. Dr Kareem said the likely explanation for how it developed was that when Lyra’s intestines were forming in utero, some of the stomach cells deposited where her intestine were and began to grow. He joked with Lyra that she must be part animal with multiple stomachs. It blew all of our minds and infuriated us to know it hadn’t been found during Lyra’s first surgery or noticed on any of the diagnostic tests her other doctors had done.
Lyra’s recovery was absolutely not a piece of cake. She had a vertical incision from her sternum to her pelvic area. When she came out of surgery, she had 4 IV sites, a central line in her neck, an NG tube, an epidural pain pump in her back, a Foley catheter, and two wound drains. Lyra had many setbacks and bumps in the road to recovery. She went into atrial fibrillation two times. She showed symptoms of a blood clot in her leg and screamed in pain for seven hours, a venous ultrasound was done but couldn’t find a clot. Her IV infiltrated her arm and hardened her veins, causing her to become dehydrated quickly, she then had to be stuck 13 times in one night to replace the IV. I continued to have intense anxiety that something could still go wrong, each setback felt like a blow to the chest.
Lyra was discharged after 15 days in the hospital. We spent another month recovering at Cleveland Clinic’s Ronald McDonald house before Dr Kareem felt Lyra was ready to make the 10 hour drive back to Tennessee. It has now been over a year since her surgery and Lyra is enjoying life more than ever. Her growth chart has gone from the 3rd percentile for weight to the 75th percentile. She is no longer taking any medication. She uses the bathroom regularly without any issues. She eats whatever she wants, whenever she wants. She plays actively just like other kids her age. She has had a few small bouts of constipation since surgery, but nothing major and once was because she ate way too many bread sticks!
Intestinal malrotation made my little girl’s first few years misery but she has taken her life back. As a parent who watched my child go through so much trauma and come so close to death, I am taking my life back too. Like many parents of children with intestinal malrotation I’ve made it a goal to educate and raise awareness. I want parents to know they should trust their instincts. Never be afraid to ask questions or advocate for yourself or child. No patient should ever have to accept a life of suffering, repeat surgeries or band aid fixes. My girl has her childhood back, I wish that and more for every person born with this condition.
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