From Heartbreak to Hope: A Baby's Journey Through Kidney Failure
And the Power of Organ Donation
Many times, while practicing in the NICU, I was graced by being able to participate in the care of exceptional cases - terribly ill babies and their amazing parents. Back in 1988, I was called STAT to attend the delivery of Emily, a baby born with infantile polycystic kidney disease, or IPKD. Her pediatrician needed help with a newborn in severe respiratory distress. Emily was born to her lawyer mother and theoretical chemist father; she was a much wanted and planned pregnancy. Her older sister was seven years old.
When I arrived, Emily was blue and struggling to breathe despite receiving some blow-by oxygen. After quickly examining her, I noted her bell-shaped thorax, her deep chest retractions, and her protuberant abdomen which contained easily palpable, large cystic kidneys. They felt like two huge, hard bunches of grapes.
I told the pediatrician that she probably had Potter’s syndrome. This beautiful little girl did prove to have infantile polycystic kidney disease, which presents with Potter’s syndrome—the physical features described above. IPKD is a rare genetic disorder affecting one in 20,000 children.
After Emily was intubated and supported on mechanical ventilation, we transported her to the NICU and inserted bilateral chest tubes for her pneumothoraxes. Pneumothorax occurs when perforation in the diseased lung allows air to trap between the lung and chest wall, causing compression of the lung.
Thankfully, her pulmonary hypoplasia—lung underdevelopment—was not as life-threatening as typical for most infants born with Potter’s syndrome. Her kidney ultrasound confirmed bilateral polycystic kidneys. Her kidneys’ production of urine, which makes up most of the amniotic fluid, had been adequate throughout her mother’s pregnancy, so her lungs were only partially underdeveloped. The chest tubes were removed within the first week, she was easily weaned off the ventilator during her second week, and her lungs healed completely.
Although Emily continued to produce large volumes of urine during her first few weeks, her kidneys did not function effectively and soon she was in renal (kidney) failure. My husband was her pediatric nephrologist, and we experienced many emotions while working together on Emily’s case. It was a challenging and thought-provoking situation.
Early on, her parents were, understandably, still reeling from her diagnosis. IPKD causes severe kidney failure and leads to dialysis and ultimately the need for kidney transplantation. Liver scarring is present at birth, but liver dysfunction does not occur until later in childhood. Her parents slowly adjusted to her diagnosis and care needs. Within a few weeks Emily began peritoneal dialysis, through a catheter entering her abdominal cavity. The dialysis machine cycled fluid into and out of her abdominal cavity to remove chemicals and toxins that built up in the absence of proper kidney function.
Her father, a brilliant chemist and university professor, would sit by her side and study the dialysis machine, its settings and functioning. Paul was a tall, handsome man who was typically quiet, although he seemed to enjoy talking with the pediatric residents about these settings and how dialysis works.
Emily’s mother, Joanie, a practicing lawyer, was an attractive, thin woman who looked despondent and seemed always to be on the verge of tears. She expressed breastmilk for her nutritional support and proceeded to cope with having a chronically hospitalized baby, another child at home, and a career. Most days when Joanie came in and out of the NICU to see Emily, she looked haggard. However, we always enjoyed talking about breastfeeding and any struggles we encountered while pumping breastmilk together. Answering her questions and commenting on how cute Emily was always made her smile, though.
Unlike a lot of other chronically ill babies, her daughter had a sweet temperament, and I enjoyed pointing this out to her mother. Years later, Joanie recollected that I “was the one who got them thinking of Emily as a person.” During that period in my life, with two small children of my own, I didn’t have enough free time to get to know Joanie very well, or to support her more fully, but my husband did. Emily’s parents were agreeable, intelligent, and levelheaded, and I found it painful to watch them handle everything that happened to their daughter so courageously. Perhaps my husband and I identified with them too much. I often wondered if, in the same situation, I would be as calm as Emily’s mother was.
Emily went home on dialysis when she was a few months old. My husband continued to oversee and direct her dialysis and care. In the interim, she required several readmissions to the hospital for various problems. Blood type and various other histocompatibility markers were studied among her family members, and it was determined that Emily and her grandmother were a perfect match. When she was only two years old, Emily received a kidney transplant, a gift from her sixty-year-old maternal grandmother. Theirs became a tremendous success story for the hospital renal transplant program.
Joanie and I wrote to each other so that we could maintain contact after we moved away from Houston. My husband saw Emily in the summer when she, like the other kidney kids, attended Camp Okawehna in Tennessee each year. The renal dialysis patients always attended camp as a group. It was an overnight camp in the piney woods outside of Nashville. Each June, my husband and the dialysis nurses shepherded a large contingency of patients, nurses, dieticians, social workers, and physicians to attend the camp for one week.
All the children at Camp O were either on peritoneal or hemodialysis or had already been transplanted. Emily attended camp each summer for several years, from about age eight onward. Like all the other kids there, she played with other children who were just like her and felt normal. These “kidney kids,” as they were lovingly called, all had lines and tubes sticking out of them, and they all had scars. Most were very short, having had poor growth for many years. But they still loved normal child-like activities—running, playing, kicking, throwing, competing, swimming, making craft items, and performing in talent shows.
Years later, after moving to Austin, I continued to correspond with Emily’s mom. We exchanged Christmas cards, family pictures, and personal letters. It was inspiring to watch her parents become champions and activists for organ transplantation. Emily’s life was not easy by any estimation. Throughout her childhood, she demonstrated enormous courage in overcoming many complications, infections, and hospitalizations, but Emily never rejected her grandmother’s kidney. When she grew to be a happy teenager, however, her liver function began to deteriorate.
Everyone viewed Emily’s family as strong and loving. From the time Emily was eight years old, her parents and big sister lived on campus and served as College Masters at the university. As a result, Emily grew up keen on learning, constantly surrounded by students. She was a good student herself and, despite all that she had gone through, she was praised by teachers for her mind, her wit, and her sense of humor.
Despite her chronic illness, Emily loved life and grew to be fiercely independent. She performed in musical theater, played tennis, and hiked in the Colorado Mountains with her family. At home she enjoyed playing the guitar and composing songs. Emily continued to do well in school and ultimately attended a small college in Texas. She was there for two years before her untimely and tragic death at age twenty.
To this day, Emily’s mother raises money for Polycystic Kidney Disease research and serves on the board of the International Polycystic Kidney Disease Foundation. Both Joanie and Paul continue to serve as advocates for organ donation in Texas.
All throughout my career, my husband and I both received profound gifts from special people like Paul and Joanie, extraordinary parents who settled close by our sickest patients and slowly revealed their loving and caring character.
This story is an excerpt from my memoir, “So Many Babies” which you can access here.
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Thank you for writing about this important topic, Dr. Landers.