Minnesota

by Deb Bachman

Was I imagining this? My skin felt extremely tight and when I laid on my side, I could feel my baby float downward. I had not experienced this sensation during my first pregnancy, but somehow, I was not alarmed.  Extra-large maternity clothes were overly snug by 29 weeks. I felt huge - a common feeling among pregnant women. The weight of my belly strained my back - another common pregnancy thing. Only this time, it was not normal.

Full blown labor began at 32 weeks. Unbeknownst to me, I had too much amniotic fluid. For some reason, my baby would not, or could not, swallow the amniotic fluid. My baby’s stomach was not visible on an ultrasound. The high-risk OB/GYN doctor explained that too much amniotic fluid is typically caused by an abnormal connection somewhere between the trachea and esophagus. She shared that these connections, called tracheoesophageal fistulas (TEF), are fixable, but I would need to stay in the hospital until delivery. Until then, IV medications and the removal of excess amniotic fluid gave my baby extra time to grow. 

My son looked perfect when he was born. Hearing his first cry, I knew something was not right. His voice was quiet and hoarse. Brown saliva bubbles came out of his mouth. He was quickly taken to the NICU for evaluation. A bedside bronchoscopy explained the atypical cry, the extra amniotic fluid, his increasing struggles to breathe. My baby had a type 4 laryngeal cleft. His airway and food tube had never separated; they were one tube. 

“You can do nothing, and he will surely die,” the surgeon told us. “Or you can allow us to try a surgery we have never done before and still your baby will probably die. If he does survive, he will need a trach to breathe, a feeding tube to eat, and a long-term IV for medications. He may never walk, talk, or eat.” 

Our hearts shattered. Before he was eight hours old, our son Markus was in the operating room, undergoing procedures that lasted 12 hours. His trachea and esophagus were separated and stitched top to bottom. He was given a trach, feeding tube, and a long-term IV in his chest. Back in 1999, a type 4 laryngeal cleft had a 97 percent mortality rate. My son miraculously survived surgery, spending the next five months and six days in the NICU.

Bringing Markus home was both wonderful and overwhelming. His nursery became a mini-hospital room, complete with homecare nurses. The school district sent early childhood teachers and therapists to our home. Markus continued to surprise us with an amazing resilience in the face of numerous health issues. He coughed everyday, his breathing sounded wet and gurgly. Vomiting was a constant threat. Formula ran into his intestine while his stomach contents drained into a diaper. We quickly learned that what caused a runny nose in most children made Markus sick enough to require hospitalization. Everything in our lives had changed.

Markus eventually weaned off bipap during the day. He took his first step at 18 months. Many milestones were delayed due to long hospitalizations, illness, and other health problems. Each milestone was truly celebrated, because we could never take anything for granted. 

We tried to let Markus eat some soft foods by mouth. Sometimes food would trickle out the stoma (hole) in his throat. He would cough. Looking back, those were not signs to be ignored.

Markus loved going to school. Unfortunately, it was not unusual for him to miss long stretches due to illness. We worked with the school district to ensure that he kept up with the same lessons as his classmates. In second grade, he had his trach taken out and his stoma repaired. It was incredible to see my son without a trach tube in his throat.

In middle school, Markus started coughing out blood. His right lung was severely damaged. Two lobes of his right lung were removed in the ninth grade. The following year he was evaluated for a lung transplant at two other hospital systems. Both hospitals said he was not a candidate for a lung transplant. The remaining right lobe was removed his senior year of high school. 

There seemed nothing left to do to help Markus. During his high school and college years, he wrote lists of goals and dreams. We were intentional about those experiences. Test driving sports cars, staying at my sister’s lake home, anything that interested Markus, we did our best to make it happen. 

Then in the summer of 2021, we received a phone call from Markus’ lung doctor. A Minnesota ENT that had trained at Cincinnati Children’s Hospital heard about Markus. He was shocked to learn there was a 21-year-old who had been born with a type 4 laryngeal cleft. 

Minutes later, Dr. Michael Rutter from CCH called my son. The doctors in Cincinnati wanted to meet him and see if there was any way they could help. We did not expect them to find anything, but they discovered a huge hole hiding between his trachea and esophagus. Markus had been aspirating through this hole, causing damage to his one remaining lung. Repairing the fistula involved another trach tube for 40 days,  a seven-week stay in Cincinnati, and a long course of antifungal medication.  

Over the next two years, Markus’ lung gradually improved. His coughing significantly decreased, as did respiratory distress. This positive change was unexpected, something we did not think was possible. Follow-up trips to Cincinnati changed from every three months to twice a year. The focus of appointments has now shifted from dire, to hope for a future.

At 24, Markus is doing well, really well. He works full-time as an accountant, still maintains a list of hopes and dreams, and continues to be a patient at Cincinnati Children’s.

Fall 2024 Update

Markus returned to Cincinnati Children’s last winter to address a fungal ball in his lung. After a long hospitalization, Markus is now doing well. He even has some big things happening in his life. After two years of employment as an associate accountant, Markus recently received his first promotion, which includes the official title of accountant! 

Besides working full-time, Markus is enjoying time with his new dog, Buddy. Buddy is being trained to alert Markus to his feeding pump alarm. Soon the duo hope to move into their own apartment or condo.

Congratulations, Markus!