"She never cried loudly enough to bother us," reminded Natalia Weil of her daughter who was born in 2011.
Although Vivienne bubbled energetically in her early months, her vocalization fell around her first birthday. So did the quality of her voice, which dropped from normal to raspy to slightly more than a whisper. Vivienne was also a late talker: She did not start talking until she was 2.
Her suburban Maryland pediatrician originally believed that a respiratory infection was the cause of the infant's hoarseness and patient patience. But after the problem persisted, the doctor diagnosed acid reflux and prescribed a drug to treat the voice problems that reflux may cause.
But Vivien's problem turned out to be much more serious ̵
"I had never heard of it," said Weil, now 33, of his daughter's diagnosis. "Most people don't."
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Initially, Natalia, a statistician and her husband, Jason, a photographer, was reassured by the pediatrician, who owed a respiratory infection to their daughter's voice problem. Her explanation sounds logical: Small children get on average seven or eight colds annually.
Weil said the couple assumed that Vivienne's voice would return to normal – and did not want to overreact.
"We were parents' first parents and We were worried," said Weil, "but we thought we were worried too much and had to wait. We decided to give it time. We did not know how much children should speak at the age of 1 or 2. … We did just what the doctors told us. "
But Vivienne's grandfather was growing worried. Because Vivienne was slow to talk, Grandma asked her to have a development delay or a speech problem and suggested an evaluation of a speech pathologist.
During a visit in September 2013 The doctor also approved a referral to an ear, nose and throat specialist
An ENT who saw Vivienne shortly after diagnosing her with dysphonia – a diminished voice that may be due to a He sent her to a pediatric otolaryngologist for a more complete evaluation.
The pediatric specialist listened to her inhalation and spoke and then planned a laryngoscopy, the sample involving a visual inspection of the back of the neck. Doctors use a thin, flexible tube attached to a small fiber optic camera screwed up into the nose and throat to allow inspection of the island. our airway.
The procedure Weil recalled was traumatic for Vivienne and her parents. The little girl, scared of what happened, started screaming and had to be put down by several nurses so the doctor could perform the test.
Its results were final – and explained the reason for Vivienne's prolonged voicelessness. She suffered from a rare disease called recurrent respiratory papillomatosis caused by two strains of the 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 tumors that temporarily restore the voice. The goal of the treatment is to prolong the interval between operations while preventing permanent damage to the sensitive voice cables.
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HPV is ubiquitous; Almost all sexually active adults have been exposed to it. Most people clear the infection from their bodies without knowing they were. However, in some cases, two strains, HPV 6 and 11, can cause genital warts: benign, sometimes cauliflower-shaped tumors known as papillomas. These warts may occur months or sometimes years after exposure.
In some cases, mothers with genital warts can pass the virus during birth, resulting in the development of papillomas in the baby's respiratory tract, especially the larynx. (Two other strains are 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 estimate that 2 out of every 100,000 children have RRP which can be prevented by a vaccine called Gardasil. Federal health officials recommend administering the vaccine, first licensed in 2006, to children at 11 or 12 before being sexually active.
The pediatric ENT told Weils, that was a good thing they hadn't been waiting for anymore to seek treatment. Vivienne's tumors had become so large that they threatened her airway.
"I was speechless," Weil reminded, who at the time was pregnant with his second daughter. "I thought," I gave it to my baby. "I remember sitting in the little room and thinking," She'll have to deal with this forever. ""
Weil said she had no idea she ever had genital warts or had been exposed to HPV . In the car heading back to their home in Maryland, she said she was crying as she rolled through the phone and tried to learn about the disease, scared that her other child might also be able to do it.
Shortly before Vivienne's first operation in November 2013, Weil sought answers from her obstetrician. How did she ask, could HPV be missed?
The doctor replied that Pap smears performed in 2009 and 2011 were normal. Health officials do not recommend that women under the age of 30 – Weil were 25 when Vivienne was born – routinely received an HPV test because the virus is so common.
"It is possible that you had the virus and then your immune system removed what your test was negative in Dec 2011" – 10 months after Vivienne's birth, the doctor wrote.
Nor is it clear that a caesarean section will have prevented the disease. Experts say some cases appear to have been contracted in utero.
Due to his age, Weil was not included in the first targeting effort for the vaccine that focused on preteen girls. (The Food Safety Authority has recently approved the latest version of the Gardasil 9 vaccine, which protects against 9 strains of HPV for adults up to 45 years.)
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The first operation of the Vivienne, performed under general anesthesia, involved debridement, a procedure that essentially shaves the tumors.
Weil said she remembers going into the recovery room with her husband to hear Vivienne "cry aloud." It was so amazing, "she reminded." For us it was the best sound in the world. "
But as is almost always the case, Vivienne's voice turned to a whisper after a few months when the tumors grew back. The next couple of years, she underwent debridement of both vocal tapes every four to six months.
In March 2018 after her 11th operation, the voice did not return, no physical explanation could be found, and the doctors suggested that the cause may stem from vocal cords. or psychological factors: For the next six months, Vivienne has undergone hypnosis and saw no speech therapists.
In desperation, Weil sent a video of her daughter on Instagram, hoping that someone – possibly another parent – could get some advice
Within days, a California woman whose daughter has the disease has suggested trying another treatment, instead of debridement, she recommended finding a doctor using a potassium titanyl phosphate (KTP) laser. Some specialists believe the use of the laser is superior because it removes more tumor and minimizes damage to the vocal cords.
"I did a lot of research," Weil said. She found Simon Best, an otolaryngologist and researcher at Johns Hopkins who is studying the disease and is an expert in laser treatment.
Weil said she tried unsuccessfully with Best, but was told that he is not a pediatric otolaryngologist and therefore does not treat children.
Undeterred she searched for a medical database, revealed her email address and sent him a message describing the daughter's case.
Best agreed to see Vivienne and Weil's insurance company approved visits outside the Best network, an associate professor of otolaryngology, assessing that in his 13-year career he has treated about. 100 people, mostly adults who have RRP. (An adult specialist, he treats patients of all ages with the disease.) Some developed the disease as children. In others, it occurred in their 30's and 40's, a decade or so after HPV exposure.
"It has a terrible propensity to just come back," Best said. One of his patients had shown 300 operations when he was 20. "You can imagine what makes for voice quality."
Best treats only one vocal cord at a time to prevent bands that occur when the vocal bands grow together and can damage the voice.
"I was pleasantly surprised that there was not a large amount of scarring," said Best of Vivienne, as he first saw in September 2018.
The first laser surgery on the second-degree right vocal lead occurred in November 2018 ; her voice came back, but remained ferocious. Another operation on the left voice cable in January 2019 has had excellent results. A few days ago, Vivienne underwent a successful repetition on her right side.
"This is the best thing she ever sounded," said her mother, adding that even the quakes of the door's doors are leaking her.
In the last couple of months she has said that Vivienne has flourished, eagerly made new friends and becomes a "happy, ridiculous little girl."
"She says having a voice is even better than she thought it would be," Weil said. A year ago she had told her mother that several classmates had excluded her from their "cheerleader" games.
Best recommends that a voice problem lasting longer than one month should request a study of "someone who can visualize the larynx."
It is difficult to predict how many operations Vivienne might need, said he. It is unlikely that it is only three because repetition is the rule. "Everyone has a unique clinical course," Best added.
"The former RRP is found, the better," Best noted before the disease can do extensive damage. "It is often overlooked for quite long periods – months or years. And it doesn't go into the diagnosis area for children until there are quite serious effects."
The otolaryngologist remains a strong advocate of the HPV vaccine, which can prevent the disease. Weil said she plans to immunize her daughters and get the vaccine herself, which could protect her from other strains of HPV.
According to the CDC, only half of the American youth have been fully immunized. In contrast, Australia, which has promoted free immunization in schools for more than a decade, has dramatically reduced cases of cervical cancer and genital warts.
"Although RRP is a rare disease, just from a psychosocial point of view, I can imagine the effect this disease has on families," best said. "These mothers' mothers have a great burden to bear."
This article was written by Sandra G. Boodman, a reporter for The Washington Post.