Erin and Matt wanted to be parents for many years and are now the proud parents of two young boys, Benji (age 6) and Charlie (age 3). Benji was adopted when he was a newborn and thus very little is known about his medical or family history. The beginning of parenthood started fairly typical with the new family at home, sleepless nights, and a tiny baby to care for. When Benji was 10 days old, Erin herself was having a sleepless night and so she opted to hold Benji while he slept on her. During that night’s typical feedings, Benji refused to eat, which was abnormal. Later, he began crying uncontrollably and as many parents can relate to, intuition kicked in and Erin called the pediatrician who acted quickly and had her bring Benji in to be seen since he was so little and wasn’t getting fluids.
At the pediatrician’s office, as soon as the nurse took his vitals they found that Benji’s heart rate was racing and his blood pressure was dangerously high for a newborn. The nurse immediately called an ambulance to take him to the emergency center. First, the emergency team thought that Benji was sick with an infection and they tried antibiotics, which didn’t work. Thereafter, a heart ultrasound revealed abnormal function. That led to Benji being flown by helicopter to a larger hospital with cardiology expertise, where they got his blood pressure under control with medications. But, because there was no medical history and no explanation for the high blood pressure, Benji stayed in the Pediatric Intensive Care Unit (PICU) for about a month for a seemingly endless series of tests.
“It was quite a whirlwind living in the hospital with him through that as my husband would work long hours during the week and then drive an hour and a half to be with us for a couple days,” Erin remembers. “Not only was it incredibly emotional as I watched Benji suffer many things in that month, but there were so many unknowns still. All the tests came back great and looking fine, yet he still needed many meds to control his blood pressure. We were released after a month in the PICU with five different medicines that were maxed out for his age.” The family had yet to receive a diagnosis.
Those medications controlled Benji’s blood pressure well for about six months—despite the family having to reassure the pharmacist that yes, those were the correct dosages for a newborn baby. The family managed the medications at home and took Benji’s blood pressure several times a day.
At about six months old, his blood pressure spiked again. After more medical efforts to control it failed, Benji was admitted back into the PICU. It was then, after several more tests, a diagnosis of bilateral renal artery stenosis was finally made. Benji’s arteries to his kidneys were narrowed, causing the high blood pressure. Erin noted that his blood pressure was not taken at birth and wonders if his initial episode at 10 days old could have meant that he was born with the narrowed arteries.
The diagnosis of bilateral renal artery stenosis prompted Erin and Matt to search for hospitals that specialize in this rare disease. The next step they were guided toward was a procedure in which a balloon would be inserted into Benji’s artery and inflated to widen the narrowed artery. Benji underwent this procedure (angioplasty) at a high-volume referral hospital center that specialized in this rare disease, after making several trips north to discuss Benji’s case and meet the medical team.
“We were so excited to hear about the ballooning procedure as we always thought he’d need surgery. There was a 90% success rate quoted. Unfortunately, we were in that 10%,” Erin recounts. “Benji came home after the ballooning procedure and we were able to decrease some meds over the next year, but not a lot. After more scans they found more areas of narrowing, which is extremely rare. This led to his needing surgery at the age of 4.”
Throughout it all, Benji was a cheerful, energetic and engaging boy. (“He would light up anytime a nurse came in and would smile away. The nurses would swoon over him every trip!”) They would try to make the hospital stays an adventure with new bedding and special toys. But the experience wasn’t without a toll; the years-long cycle of procedures, hospitalizations, and medications had an impact on Benji.
All the medications and their side effects also made it difficult for Benji to keep the schedule of a normal child when he wasn’t in the hospital. One of the pills, clonidine, made him very tired, so he’d have to take naps several times a day. The family’s goal was to wean Benji off of clonidine prior to school starting so that he could fully participate in the school schedule.
The surgery Benji required at 4 years of age for diffuse (widespread) arterial narrowing involved the revascularization (reimplantation/repair) of a segmental branch artery and resection of half of his kidney that was deemed unreconstructable. In these cases, the remaining kidney will over-grow (hypertrophy) to compensate with the goal of normal kidney function. Things have been steadily improving since surgery. After a month of recovery, Benji has no long-term effects and surprisingly and thankfully, Benji was able to wean off of all of his medications right before school started.
Erin describes herself as someone who did not have a lot of experience with the medical world before their journey with Benji. Together, she and Matt learned to ask good questions, the importance of teamwork, and gratitude for the healthcare team. “This journey has affected us in more ways than I can describe. We have grown in faith, compassion, tenderness, and genuine empathy for those who endure hospital trials. We have learned to take one day, one moment at a time. We grew in our marriage as we endured and persevered together through very hard days, but also rejoiced together in those days of victory!” Erin said.