Tumor
Think about the feelings you have when you are on a rollercoaster. Fear, excitement, nervousness – how your heart is in your throat and how your stomach turns. Then imagine being two years old and feeling that way all the time. That is how Children’s of Alabama nephrologist Frank Tenney describes what Joseph Pickett of Montgomery was experiencing when doctors at Children’s first diagnosed his extremely high blood pressure in August of 2013. Joseph was at Children’s for an unrelated outpatient procedure when his blood pressure problem was discovered. When medications resulted in little improvement, doctors in the hospital’s Imaging department were consulted. “An adrenal mass was detected,” explains Jennifer D. Hamm, MD, the first year fellow in the UAB Division of Pediatric Hematology-Oncology who oversees Joseph’s care through the Alabama Center for Childhood Cancer and Blood Related Disorders at Children’s. “We would later determine that the mass was a ganglioneuroblastoma – and that chemical secretions from it were causing both blood pressure problems and diarrhea.” A ganglioneuroblastoma is a type of intermediate tumor (one that has both malignant and benign cells) that develops out of a bundled mass of nerve cells. These tumors are rare, occurring in fewer than five out of one million children each year. Joseph’s renal tumor would have to be removed, but his “unbelievably high” (150 over 120 at times) blood pressure was the more immediate concern, according to Dr. Tenney. “It took quite a while to get Joseph’s pressure regulated with various medications,” he explains. “His case is probably among the three most difficult to manage that I have seen in more than 35 years of practice. “But we had address his hypertension or Joseph would have died,” he adds. “He most likely would have experienced brain swelling, seizures or some sort of cardiovascular event – probably a stroke. I am sure he would not have survived much longer.” Because Joseph’s mother, Kevinetta, is also ill, his paternal great-grandmother, Vera J. Booker of Selma, is his primary caregiver. “She is a former pediatric nurse practitioner,” Dr. Tenney explains. “She is also an amputee who gets around in a wheelchair – but she is calm and cool and manages her family’s health challenges with aplomb.” When Vera brought Joseph to Children’s on August 8, she was expecting to take him back home the same day; but he would not leave the hospital until September 17. Joseph spent much of this time in the intensive care unit with his mother, “Grandmomma VJ” and other family members at his bedside. “I was shocked by his diagnosis,” Vera says. “I am a retired nurse, and we just don’t normally think about checking blood pressure in a child who is less than three years old. Fortunately, Dr. Tenney was able to get Joseph’s hypertension under control so that Dr. Elizabeth Beierle could safely do his surgery on September 6. And she just did a wonderful job – she was able to get the entire tumor!” Even after removal of the tumor, Joseph remained hypertensive and very sick, according to Dr. Tenney. “We still needed to manage his blood pressure, and it was a really tricky thing to wean him off the medications,” he says. “When he was released from Children’s, he went home on four blood pressure medicines. But because Mrs. Booker is a very good nurse, we were able to manage the weaning process over the phone and he was off all the medications in about a month. Now, we just continue to monitor his blood pressure.” Today, Joseph’s prognosis is very good. “There has been no evidence of any recurrence of the ganglioneuroblastoma, and his elevated blood pressure and diarrhea have also resolved,” says Dr. Hamm. “He is a very bright, social child who always seems to have a way of making our day a little better when he comes to clinic. Our clinic staff adores both Joseph and his family. It has been an honor and privilege to take part in his care.” “He is a real survivor,” adds Dr. Tenney. “Caring for him was one of the more remarkable events in my career.” On January 19, 2014, Joseph celebrated his third birthday with a party and a trip to church with Grandmomma VJ. “He is so smart – he knows his alphabet, he can count to 100 and he can even read his own blood pressure,” says Vera. “He is just anointed! At church, everyone was pinning money on him and he came home with $54. We’re going to use that money to buy him an Easter suit. “He’s just a little joy,” she adds. “I want him have a well-rounded life -- to get a good education, to play sports if possible and to look forward to a good future. And I know God is going to make it all right!”
