"She never cried loud enough to disturb us," Natalia Weil recalls of her daughter, born in 2011.
Although Vivienne blabbered energetically in her first few months, her voice dropped around her first birthday. Likewise the quality of her voice, which went from normal to raspy and barely more than a whisper. Vivienne also spoke very late: she did not speak until she was two.
Her suburban pediatrician in Maryland initially suspected that a respiratory infection was responsible for the hoarseness of the toddler, and advised patience. However, after the problem persisted, the doctor diagnosed acid reflux and prescribed a drug to treat the voice problems caused by reflux.
However, Vivienne's problem proved to be much more serious and unusual than excessive stomach acidity. The day she learned what was wrong is one of the worst in Weil's life.
"I had never heard of it," said Weil, now 33, about her daughter's diagnosis. "Most people do not have it."
First, Natalia, a statistician, and her husband, Jason, a photographer, were reassured by the pediatrician who blamed a respiratory infection on their daughter's voice problem. Their explanation sounded logical: infants get an average of seven or eight colds per year.
Because the couple suspected, Vivienne's voice would normalize again – and she did not want to overreact.
"We were parents for the first time" We were worried, "Weil said," but we thought maybe we were too worried and should wait. We decided to take our time. We did not know how much children should talk at the age of 1 or 2. "We just did what the doctors told us."
Vivienen's paternal grandmother, however, became increasingly worried. As Vivienne spoke slowly, her grandmother wondered if she could have a developmental delay or speech problem, and suggested that she be assessed by a speech pathologist.
During a visit in September 2013, the pediatrician prescribed a liquid antacid for the patients. 2 years old The doctor also approved a referral to an ear, nose and throat doctor.
An ENT who had diagnosed Vivienne shortly thereafter was diagnosed with dysphonia – an impaired voice that can be caused by a vocal cords problem. He sent her to a pediatric ENT doctor for a more comprehensive assessment.
The pediatric specialist listened to her breathing and conversations and then planned a laryngoscopy. The test involves a visual inspection of the neck. In some cases, physicians use a thin, flexible tube attached to a small fiber optic camera, which is screwed up the nose and neck to allow for upper respiratory tract inspection.
The procedure, Weil remembers, was a dream for Vivienne and her parents. The little girl, appalled by what was happening, began screaming and had to be held down by several nurses so that the doctor could do the test.
His results were final – and explained the reason for Vivien's long-lasting vocalism. She suffered from a rare disease, recurrent respiratory papillomatosis, which is caused by two strains of human papillomavirus (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, temporarily restoring the voice. The aim of treatment is to extend the interval between surgeries and to avoid permanent damage to the sensitive vocal cords.
HPV is omnipresent; Almost all sexually active adults were exposed to it. Most people remove the infection from their bodies without ever knowing that they had it. In some cases, however, two strains, HPV 6 and 11, can cause genital warts: benign, sometimes cauliflower tumors called papillomas. These warts can occur months or sometimes years after exposure.
In some cases, mothers with genital warts can pass the virus during birth, leading to the development of papillomas in the child's airways, especially the larynx. (Two other strains known as "high-risk" – HPV 16 and 18 – can cause cervical cancer.) HPV can also cause oral, anal and penile cancers.)
The Centers for Disease Control and Prevention estimated that That 2 out of every 100,000 children have this RRP, which can be prevented by a vaccine called Gardasil. Federal health officials recommend that the vaccine, first approved in 2006, be given to children 11 or 12 years of age before they become sexually active.
The ENT doctor told The Weils that it was a good thing they had stopped waiting for treatment. Vivienne's tumors had become so big that they threatened her airways.
"I was speechless," recalls Weil, who was pregnant with her second daughter at the time. "I thought I gave this to my baby." I remember sitting in that little room and thinking, 'She'll have to deal with it forever.' Weil said she had no idea that she had ever had genital warts or was exposed to HPV, and on her way back to her home in Maryland, crying through her phone and trying desperately, she cried for the disease She was afraid that her second child might hurt her, too.
Just before Vivienne's first After an operation in November 2013, Weil was looking for answers from her obstetrician How, she asked, could HPV have been overlooked
The doctor replied that Pap smears performed in 2009 and 2011 were normal and health officials do not recommend women under 30. – Because she was 25 years old when Vivienne was born, she is routinely undergoing HPV testing. since the virus is so common.
"Maybe you had the virus and then your immune system your test was negative in December 2011." – Ten months after the birth of Vivienne, the doctor wrote.
E It is also not clear that a caesarean section would have prevented the disease. Experts say that some cases appear to have occurred in utero.
Because of her age, Weil was not involved in the initial targets for the vaccine, which focused on adolescent girls. (Federal health officials recently approved the newest version of the vaccine, Gardasil 9, which protects against nine strains of HPV, for adults up to age 45.)
First surgery of Vivienne performed under general anesthesia. involved debridement, a procedure that essentially cuts off the tumors.
Weil said she remembers going to the recovery room with her husband to hear Vivienne "cry aloud," she recalls. "For us it was the best sound in the world."
But, as is almost always, Vivienne's voice fades to a whisper after a few months as the tumors regrow. In the next few years, a simultaneous debridement of both vocal cords was performed every four to six months.
In March 2018, after her 11th operation, her voice did not come back. No physical explanation could be found and the physicians suggested that the cause could be due to vocal cord weakness or psychological factors. For the next six months, Vivienne underwent hypnosis and saw unsuccessful speech therapists.
In despair Weil posted a video of her daughter on Instagram. She hoped someone, possibly another parent, would have some advice.
Within days, a Californian woman whose daughter is suffering from the disease suggested a different treatment. Instead of debridement, she recommended looking for a doctor who uses a potassium titanyl phosphate (KTP) laser. Some experts believe that using the laser is superior as it removes more tumor while minimizing vocal cord damage.
"I did a lot of research," Weil said. She found Simon Best, an ENT doctor and researcher with Johns Hopkins, who deals with the disease and is an expert in laser treatment. Because she said she had unsuccessfully tried to make an appointment with Best, but was cautioned that he was not a pediatric otolaryngologist and therefore no pediatric ear doctor was not treating children.
Undaunted, she searched a medical database, dug up his e-mail address, and sent him a message describing her daughter's case.
Best to agree that Vivienne and Weil's insurer approved the visit outside the network
Best, an associate professor of Otorhinolaryngology, estimates that in his 13- year-old career has treated about 100 people, mostly adults who have RRP. (As an adult specialist, he treats patients of all ages with the disease.) Some have already developed the disease as children. In other cases, it originated in the 1930s and 1940s, about a decade after HPV exposure.
"There is a terrible tendency to just keep coming back," said Best. One of his patients had undergone 300 operations until the age of 20. "You can imagine what the quality of the voice means."
It is best to treat only one vocal cords at a time to prevent the strap that occurs when the vocal cords grow together and can damage the voice.
"I was pleasantly surprised that there were not many scars," said Best of Vivienne, whom he first saw in September 2018.
The first laser operation on the second right vocal cord of the plaintiff occurred in November 2018; Her voice returned but remained scratchy. A second operation on the left vocal cord in January 2019 had excellent results. A few days ago Vivienne had undergone a successful retry on her right side.
"This is the best she has ever sounded," her mother said, adding that even the sound of her daughters' quarrels pleases her.
In the last few months, she said, Vivienne is flourishing, making new friends and becoming a "happy, chubby little girl".
"She says a voice is even better than she thought," Weil said. A year ago she had told her mother that several classmates had excluded her from her "cheerleader".
Best recommends that a voice problem that lasts more than a month should be checked by "someone who can visualize the larynx." , "
It's difficult to predict how many operations Vivienne might need," he said, "It's unlikely that there are only three, because recurrence is the rule." "Everyone has a unique clinical course," Best added.  "The sooner the RRP is detected, the better", best said, before the disease can cause significant harm. "It is often overlooked over a long period of time – months or years. And it's only being diagnosed in children when it has serious consequences. "
The Otolaryngologist is still a strong proponent of the HPV vaccine, which can prevent the disease because it said it Intended to immunize their daughters and obtain the vaccine itself that could protect them against other strains of HPV.
According to the CDC, only half of American adolescents have been fully immunized a decade that promotes free immunization in schools that drastically reduces cervical cancer and genital wart cases.
"Even though RRP is a rare disease, only from a psychosocial perspective can imagine the impact this disease has on families," Best said. "The mothers of these children have a great burden to bear."
This article was written by Sandra G. Boodman, ei a Washington Post reporter.