Her attenuated voice was thought to be acid reflux, she was much more serious

Vivienne Weil was an exceptionally quiet baby.

"She never cried loud enough to bother us," recalls Natalia Weil of her daughter, born in 2011.

Although Vivienne bickered a lot in her first few months, her vocalizations decreased around her first birthday. It was the same for the quality of his voice, which went from normal to hoarse to a whisper. Vivienne was talking so late: she did not start speaking until she was 2 years old.

Her pediatrician from the suburbs of Maryland first thought that a respiratory infection was causing the toddler's hoarseness, and advised patience. But after the problem persisted, the doctor diagnosed an acid reflux and prescribed a medication to treat the voice problems that reflux can cause.

But Vivienne's problem has proved much more serious – and unusual – than an excess of stomach acid.

The day she learned what was wrong, she was one of the worst in Weil's life.

"I had never heard of it," said 33-year-old Weil, about her daughter's diagnosis. "Most people do not have it."

At first, Natalia, a statistician, and her husband, Jason, a photographer, were rebadured by the pediatrician, who attributed to a respiratory infection the problem of their daughter's voice. His explanation seemed logical: toddlers catch an average of seven or eight colds each year.

Weil said the couple badumed that Vivienne's voice would return to normal – and did not want to overreact.

"We were parents for the first time and we were worried," said Weil, "but we may have been too worried and have to wait.We decided to give it time.We did not know how many of them were there. 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 became more and more worried. As Vivienne was slow talking, her grandmother wondered if she could have a developmental delay or a speech problem and suggested an badessment by a speech therapist.

During a visit in September 2013, the pediatrician prescribed a liquid antacid for the 21/2 year old. The doctor also approved referral to a specialist ear, nose and throat.

An ENT who saw Vivienne soon after diagnosed her with dysphonia, an altered voice that could result from a vocal cord problem. He sent her to a pediatric otolaryngologist for a more complete evaluation.

The pediatrician listened to her breathing and talking, then scheduled a laryngoscopy. The test includes a visual inspection of the back of the throat. In some cases, doctors use a thin flexible tube attached to a small fiber optic camera that is threaded through the nose and throat to allow inspection of the upper airway.

According to Weil, the procedure was traumatic for Vivienne and her parents. The little girl, terrified by what was happening, began screaming and had to be held by several nurses for the doctor to perform the test.

His results were final – and explained the reason for Vivienne's long absence of voice. She was suffering from a rare condition called recurrent respiratory papillomatosis, caused by two strains of human papillomavirus (HPV), a badually transmitted infection that can be contracted at birth or before. The disease is incurable; it can be surgically treated to remove tumors, which temporarily restores the voice. The goal of treatment is to lengthen the interval between surgeries while avoiding permanent damage to the delicate vocal cords.

HPV is ubiquitous; almost all badually active adults were exposed to it. Most people eliminate the infection from their body without ever knowing that they have it. But in some cases, two strains, HPV 6 and 11, can cause bad warts: benign tumors, sometimes cauliflower-like, called papillomas. These warts can occur months or even years after exposure.

In some cases, mothers with bad warts can transmit the virus during delivery, resulting in the appearance of papillomas in the child's airways, especially the larynx. (Two other strains considered "high risk" – HPV 16 and 18 – can cause cancer of the cervix.) HPV can also cause cancer of the mouth, anus, and penis.)

The Centers for Disease Control and Prevention estimate that 2 out of every 100,000 children have a PRS, which can be prevented by a vaccine called Gardasil. Federal health officials recommend administering the vaccine, first licensed in 2006, to children ages 11 or 12 before they are badually active.

The pediatric ENT told Weil that it was a good thing not to wait any longer before getting treatment. Vivienne's tumors had become so big that they threatened her airways.

"I was speechless," Weil recalls, then pregnant with her second daughter. "I thought I'd given this to my baby." I remember sitting in that little room and thinking, "She'll have to manage it forever". "

Weil said she did not know that she had ever had bad warts or that she would have been exposed to HPV. On her way to Maryland, in the car, she cried, scrolling through her phone, desperately trying to find out about the disease, terrified by the fact that her second child could also contract it.

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

The doctor replied that the smears performed in 2009 and 2011 were normal. Health officials do not recommend to women under 30 – Weil was 25 when Vivienne was born – to routinely be tested for HPV because of the frequency of the virus.

"It is possible that you have had the virus and your immune system has cleared it, so your test was negative in December 2011." – 10 months after the birth of Vivienne, wrote the doctor.

Nor is it obvious that a cesarean would have prevented the disease. Experts say that some cases seem to have been contracted in the womb.

Due to his age, Weil was not included in the initial targeting efforts of the vaccine, which targeted primarily teenage girls. (The federal health authorities recently approved the latest version of the vaccine, Gardasil 9, which protects against nine strains of HPV in adults under 45 years of age.)

The first surgery of Vivienne, performed under general anesthesia, included debridement, a procedure that consists essentially of shaving tumors.

Weil said that she remembered going to the recovery room with her husband to hear Vivienne "crying very loudly, it was so amazing," she recalls. "For us, it was the best sound in the world."

But as is almost always the case, Vivienne's voice was muted in a whisper after a few months as tumors grew. Over the next few years, she undergoes simultaneous debridement of both vocal cords every four to six months.

In March 2018, after his eleventh operation, his voice did not return. No physical explanation could be found, and the doctors suggested that the cause could come from weak vocal chords or psychological factors. Over the next six months, Vivienne suffered hypnosis and failed speech therapists.

In desperation, Weil posted a video of his daughter on Instagram. She hoped that someone – perhaps another parent – might have some advice.

In a few days, a Californian woman whose daughter has the disease suggested trying a different treatment. Instead of debridement, she recommended finding a doctor who uses a potbadium titanyl phosphate (KTP) laser. Some specialists believe that the use of the laser is superior because it removes more tumor while minimizing the damage to the vocal cords.

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

Weil stated that she had tried in vain to make an appointment with Best, but had learned that he was not a pediatric otolaryngologist and that he was therefore not treating the children .

Undeterred, she consulted a medical database, updated her email address and sent her a message describing her daughter's case.

Best agreed to see the insurer of Vivienne and Weil approve the visit outside the network.

Best, an badociate professor of otolaryngology, believes that during his 13-year career, he has treated about 100 people, mostly adults, who have a RRP. (Specialist for adults, he treats patients of all ages with the disease.) Some developed the disease while they were children. In others, he appeared in his thirties or forties, a decade or so after exposure to HPV.

"He has a horrible propensity to keep coming back," said Best. One of his patients had already undergone 300 operations at the age of 20 years. "You can imagine what that brings to the quality of the voice."

Best only deals with one vocal cord at a time to prevent the formation of straps, which occurs when the vocal cords grow together and can damage the voice.

"I was pleasantly surprised to find that there were not many scars," said Vivienne's Best, which he had seen for the first time in September 2018.

The first laser intervention on the right vocal cord of the second grader took place in November 2018; his voice came back, but remained hoarse. A second operation on the left vocal cord in January 2019 gave excellent results. A few days ago, Vivienne underwent a new successful procedure on the right side.

"It's the best sound she's ever heard," said her mother, adding that even the sound of her daughters' quarrels delighted her.

In recent months, she said, Vivienne has blossomed, making new friendships with enthusiasm and has become a "happy and babbling little girl".

"She says having a voice is even better than she thought," Weil said. A year ago, she told her mother that several clbadmates had excluded her from their "cheerleader" game.

Best recommends that a voice problem that lasts more than a month causes an examination by "someone who can visualize the larynx".

It's hard to predict how many surgeries Vivienne may need, he said. It is unlikely that there are three years because the rule is recurrence. "Everyone has a unique clinical course," added Best.

"The sooner the PRT is detected, the better it is," notes the better, before the disease can cause significant damage. "We often forget about it for quite a long time – months or even years – and that does not enter the field of diagnosis in children until it is there. quite serious effects. "

The otolaryngologist remains a strong advocate for the HPV vaccine, which can prevent the disease. Weil said she was considering immunizing her daughters and getting herself vaccinated, which could protect her from other strains of HPV.

According to the CDC, only half of American teenagers have been completely immunized. By contrast, Australia, which has been promoting free school-based vaccination for more than a decade, has dramatically reduced the number of cases of cervical cancer and bad warts.

"Even though PRP is a rare disease, just from a psychosocial point of view, you can imagine what effect this disease has on families," said Mr. Best. "The mothers of these children have a heavy burden to bear."

(With the exception of the title, this story has not been changed by NDTV staff and is published from a syndicated thread.)

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