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Suffering in silence

Vivienne Weil was an unusually quiet baby.

“She never cried loudly enough to bother us,” recalled Natalia Weil of her daughter, who was born in 2011.

Vivienne Weil suffers from recurrent respiratory papillomatosis, a rare disease caused by two strains of the human papilloma virus, or HPV. (Courtesy of Natalia Weil)

Although Vivienne babbled energetically in her early months, her vocalizing diminished around the time of her first birthday. So did the quality of her voice, which dwindled from normal to raspy to little more than a whisper. Vivienne also was a late talker: She didn’t begin speaking until she was 2.

Her suburban Maryland pediatrician initially suspected a respiratory infection and counseled patience. But after the problem persisted, the doctor diagnosed acid reflux and prescribed a drug.

But Vivienne’s problem turned out to be far more serious – and unusual – than excess stomach acid. The day her mother learned what was wrong ranks among the worst of Weil’s life.

“I had never heard of it,” Weil, now 33, said of her daughter’s diagnosis.

At first, Natalia, a statistician, and her husband, Jason, a photographer, were reassured by the pediatrician who blamed a respiratory infection for their daughter’s voice problem.

Weil said the couple assumed Vivienne’s voice would return to normal – and didn’t want to overreact.

“We were first-time parents and we were worried,” Weil said, “but thought maybe we worried too much and should wait. … We didn’t know how much children were supposed to talk at the age of 1 or 2. … We just did what the doctors told us.”

But Vivienne’s paternal grandmother grew increasingly concerned. Because Vivienne was slow to talk, her grandmother wondered whether she might have a developmental delay or a speech problem and suggested a speech pathologist.

During a visit in September 2013, the pediatrician prescribed a liquid antacid for the 2½-year-old. The doctor also approved a referral to an ear, nose and throat specialist.

A specialist who saw Vivienne soon afterward diagnosed her with dysphonia – an impaired voice that can result from a problem with the vocal cords. He sent her to a pediatric otolaryngologist for a more complete evaluation.

The pediatric specialist scheduled a laryngoscopy, involving a visual inspection of the back of the throat. In some cases, doctors use a thin, flexible tube attached to a tiny fiber-optic camera that is threaded up the nose and down the throat to permit inspection of the upper airway.

The procedure, Weil recalled, was traumatic for Vivienne and her parents. The little girl had to be held down by several nurses so the doctor could perform the test.

Its results explained the reason for Vivienne’s voicelessness. She was suffering from a rare disease called recurrent respiratory papillomatosis, or RRP, caused by two strains of the human papilloma virus, or HPV, a sexually transmitted infection that can be acquired at or before birth. The disease is incurable; it can be treated by surgery to remove the tumors, which temporarily restores the voice. The goal of treatment is to lengthen the interval between surgeries while preventing permanent damage to the vocal cords.

HPV is ubiquitous; nearly all sexually active adults have been exposed to it. Most people clear the infection from their bodies without ever knowing they had it. But in some cases, two strains, HPV 6 and 11, can cause genital warts known as papillomas. These warts can occur months or years after exposure.

In some cases, mothers with genital warts can pass the virus during childbirth, resulting in the development of papillomas in the child’s respiratory tract, particularly the larynx. (Two other strains considered high-risk – HPV 16 and 18 – can cause cervical cancer. HPV can also cause oral, anal and penile cancer.)

The Centers for Disease Control and Prevention estimates that 2 in every 100,000 children have RRP, which can be prevented by a vaccine called Gardasil. Federal health officials recommend giving the vaccine, first licensed in 2006, to children at 11 or 12 before they are sexually active.

The pediatric ENT told the Weils it was a good thing they hadn’t waited longer to seek treatment. Vivienne’s tumors were so large they were threatening her airway.

“I was speechless,” recalled Weil, at the time pregnant with her second daughter. “I thought, ‘I gave this to my baby, she’ll have to deal with this forever.’ ”

Weil said that she had no idea she ever had been exposed to HPV. In the car on the way back to their Maryland home, she said, she wept as she frantically tried to learn about the disease, terrified her second child might contract it, too.

Shortly before Vivienne’s first surgery, in November 2013, Weil sought answers from her obstetrician. How, she asked, could HPV have been missed?

The doctor replied that Pap smears performed in 2009 and 2011 were normal. Health officials do not recommend that women under 30 – Weil was 25 when Vivienne was born – routinely receive an HPV test because the virus is so common.

Nor is it clear that a Caesarean delivery would have prevented the disease. Experts say some cases appear to have been contracted in utero.

Because of her age, Weil was not included in the initial targeting for the vaccine, which focused on preteen girls. (Federal health officials recently approved the latest version of the vaccine, Gardasil 9, which protects against nine strains of HPV, for adults up to age 45.)

Vivienne’s first surgery, under general anesthesia, involved debridement, a procedure that essentially shaves off the tumors.

Weil said she remembers walking into the recovery room with her husband to hear Vivienne “crying loudly …,” she recalled. “For us, it was the best sound in the world.”

But as is almost always the case, Vivienne’s voice faded to a whisper after a few months as the tumors grew back. For the next few years, she underwent simultaneous debridement of both vocal cords every 4-6 months.

In March 2018, after her 11th operation, her voice did not return. No physical explanation could be found and doctors suggested the cause might stem from vocal cord weakness or psychological factors. For the next six months, Vivienne underwent hypnosis and saw speech therapists, to no avail.

In desperation, Weil posted a video of her daughter on Instagram. She hoped someone might have some advice.

Within days, a California woman whose daughter has RRP suggested a different treatment. Instead of debridement, she recommended finding a doctor who uses a potassium titanyl phosphate, or KTP, laser. Some specialists believe that the laser is superior because it removes more tumor while minimizing damage to the vocal cords.

“I did a lot of research,” Weil said. She found Simon Best, an otolaryngologist and researcher at Johns Hopkins University who studies the disease and is an expert in laser treatment.

Weil said she tried to make an appointment with Best, but was told he is not a pediatric otolaryngologist and doesn’t treat children.

Undeterred, she unearthed his email address and sent him a message describing her daughter’s case.

Best agreed to see Vivienne, and Weil’s insurer approved the out-of-network visit.

Best estimates that in his 13-year career, he has treated about 100 people, mostly adults, with RRP. Some developed the disease as children. In others, it arose in their 30s and 40s.

“It has a horrible propensity to just keep coming back,” Best said. One of his patients had endured 300 operations by the time he was 20.

Best treats only one vocal cord at a time to prevent webbing, which occurs when the vocal cords grow together and can harm the voice.

“I was pleasantly surprised that there wasn’t a huge amount of scarring,” Best said of Vivienne, whom he first saw in September 2018.

The first laser surgery, on the second-grader’s right vocal cord, was in November 2018; her voice returned, but remained raspy. A second operation on the left vocal cord in January 2019 has had excellent results. A few days ago, Vivienne underwent a successful repeat procedure on her right side.

“This is the best she has ever sounded,” said her mother, adding that even the noise of her daughters’ squabbling delights her.

In the past few months, she said, Vivienne has blossomed, becoming a “happy, babbly little girl.”

“She says that having a voice is even better than she thought it would be,” Weil said.

Best recommends that a voice problem that lasts longer than a month should prompt an examination by “someone who can visualize the larynx.”