By Maureen Gilmer, IU Health senior journalist, email@example.com
From the sparkles on her butterfly shirt to the sparkle in her eyes, Pharah Paul brings light and life to everyone she meets.
The 3-year-old displays an innate fashion sense, with her floppy rainbow hat, her jazzy eyeglasses and her heart-shaped earrings.
She has left her rainbow shoes at home on this day at Riley Hospital for Children, but her mom will tell you she is a clothes horse, a tiny fashionista whose favorite color is purple.
“She has different shoes and hats for every day,” Natalie Paul said, laughing as her daughter twirled around the lobby of Riley. “She has more clothes and shoes than an adult female. She’s a little diva, but honestly it has helped her get through this.”
“This” is cancer. Wilms tumor to be exact. It is a solid cancerous tumor of the kidney that forms from immature kidney cells. It has already taken one of Pharah’s kidneys and a portion of the other.
The fight is on.
It’s a fast-growing cancer, so when emergency room physicians at Deaconess Gateway in Evansville – where Natalie Paul works as a patient care assistant – saw the bulge in Pharah’s abdomen and looked closer, they called for immediate transport to Riley.
THEIR WORST FEARS
“I asked one of the doctors, ‘Can you please just look at this and tell me I’m crazy. Tell me it’s a hematoma from falling, or poop, or appendicitis,” Natalie said. “I can deal with a one-time surgery and be done.”
But the doctor found what she and husband Craig feared most.
“He told me there was something in there – a foreign mass.”
Pharah was transported via ambulance from Evansville to Riley in Indianapolis that same night.
A lump they thought was about the size of a golf ball was actually as big as a grapefruit. And it had been growing inside Pharah since she was a baby.
The tumor had metastasized, so it was also in her lung and on her other kidney. Riley surgeon Dr. Troy Markel operated on Pharah to remove her right kidney, where the cancer had first taken hold, along with a portion of her left kidney.
That was followed by a heavy dose of chemo and 13 days of radiation to clean up the lungs.
And then the long slog back to normal. About 42 weeks of treatment.
Unlike some Wilms tumors that affect just one kidney and can be managed often through surgery alone, Pharah’s is aggressive, spreading to the second kidney, along with nearby lymph nodes and into the lungs.
“After removing one kidney and taking out as much as we could from the other kidney safely, we radiated what was left behind, and now we’re giving chemotherapy to treat any remnants of disease and help totally clear it from her system,” Dr. Severance said.
What makes that a challenge is that the kidneys are responsible for filtering and processing a lot of chemotherapy, he explained. So her remaining kidney has to work overtime to process and clear a lot of her chemo.
“We really want to treat this as aggressively as we can … but her remaining kidney is very fragile,” the oncologist said. “It is a tough balance.”
But don’t tell any of that to Pharah, who returns to Riley for in-patient treatment July 19, the day after her 4th birthday. Dr. Severance said that while chemo can make kids pretty sick, his patient seems unfazed.
“When it comes to Pharah, her overall joy, demeanor and personality are totally immune to chemo. Even on her sickest day, she is still Pharah and she is still wonderful,” he said. “The whole team, from physicians to nurses to trainees to pharmacists, social workers, child life specialists, everybody is absolutely in love with her.”
Count oncology nurses Carol Hayden and Ashley Smith among Pharah’s fans.
“She is always a ray of sunshine and full of energy,” Hayden said, recalling how Pharah conducted a dance party with a glow stick in her room one day. “It was just the cutest thing.”
And then there was the time Pharah called Hayden into her room on a day when the nurse was particularly busy.
“Can we take a walk,” the little girl asked.
Of course, Hayden couldn’t say no, regardless of how busy she was. But it wasn’t just a quick walk.
“The walk turned into a sprint, and by the time we got done it was probably like two miles around the unit,” Hayden laughed. “It left her mom and me out of breath, but we just kept going, hopping and skipping, and she held my hand really tight.”
“FULL OF SPIRIT AND SPUNK”
Smith first met Pharah when the preschooler had just been diagnosed. A nurse on the hem-onc unit for just about 10 months now, she was working nights, and picked up Pharah as her first primary patient. When she would listen to Pharah’s heart with a stethoscope, Pharah would pull out her own stethoscope from the child’s doctor kit she received at the hospital and listen to the nurse’s heart too.
“She’s just a good kid, full of spirit and spunk,” Smith said, recalling how Pharah would poke her head out of her room when her mom would leave for a bit. She would greet everyone with the same bright smile and the words, “Hi, I’m Pharah Paul,” followed by a compliment like: “I like your shoes” or “I like your earrings.”
“She’s so funny and a joy to take care of,” Smith said. “And her parents are great, too.”
Hayden, a nurse for 2½ years and a PCA on the hem-onc unit for several years before that, considers herself lucky to have met Pharah.
“I love all my patients, but she’s definitely made a big impact on me.”
The nurses’ compassion and care have meant a lot to both Natalie and Craig, who have also been lifted up by friends and family in Evansville and in Indianapolis, where they stay with close friends when Pharah needs treatment at Riley.
“Both Ashley and Carol adore her,” Natalie said. “They were there in the beginning … it’s a comfort.”
“All the nurses fight over who gets to take care of her,” Craig said.
“A JOY TO BE AROUND”
Dr. Severance has seen his young patient power through her illness, still leading with her joyful spirit.
“She can befriend anybody at any time,” he said. “It doesn’t matter how much chemo we throw at her, how much surgery, how much radiation, she’s still just happy and loves to be around folks. She’s the kind of person you volunteer to take care of because she’s just a joy to be around.”
Pharah’s care team extends well beyond oncology, he added, explaining that Wilms tumor requires a multidisciplinary response.
“We really have collaboration among the oncology team, the surgery team, the radiation-oncology team, pathology, radiology, nephrology … so many groups come together to help navigate this,” Dr. Severance said.
“That’s one of the benefits of working at Riley. We have wonderful collaborating specialty services that really help us care for patients like Pharah.”
The oncologist, who just completed his fellowship at Riley, returns as full-time faculty next week and will continue seeing Pharah and her parents through this difficult journey.
“Mom and Dad are just wonderful,” he said. “They approach chemotherapy like veterans, even though she’s still pretty early in treatment, and that’s with a baby at home. I can see where Pharah gets her resilience.”
AND BABY MAKES FOUR
Pharah is big sister to 6½-month-old Charley, who was born just a few weeks before Pharah’s diagnosis. The young family recently took their first trip to the beach, where Pharah had the time of her life.
Being together as a family is something they’ve not been able to enjoy as much as they’d like, so the beach was a special retreat.
“We’ve spent so much time away from Charley,” Natalie said, while in the same breath expressing gratitude for the way all of the grandparents have stepped up to care for their baby girl when Pharah has to be at Riley.
“It’s not just their granddaughter who is sick; it’s all of us going through this,” she said.
Natalie and Craig say they haven’t tried to explain too much to Pharah about her illness, keeping it on a level she can understand.
When it’s time to go to Riley, “we say she is going to go see her friends in red,” Natalie said.
“You just never think it’s going to be your kid. You see other families going through cancer, and you think, ‘I hope I never have to go through that.’ Now that we are, we’ve had to change our entire family dynamic,” Natalie said.
“It has affected everyone. It’s really been a domino effect – even our relationship as a couple had to change because we’re constantly in a state of pretense,” she said, explaining how they try not to let Pharah see how her illness terrifies them.
But when they come to Riley to see their “friends in red,” they let their guard down a little, knowing Pharah is in good hands.
“They are all so amazing,” Craig said. “It takes a special type of person to do what they do.”
“Oh my God, everyone on the hem-onc unit has been so wonderful to her. We just could not have asked for a better place to have the treatment. She has soared through it.”
Photos submitted and by Mike Dickbernd, IU Health visual journalist, firstname.lastname@example.org