‘Kids ask why I have a wobbly face’: Life with Justin Bieber’s Ramsay Hunt Syndrome

Matt Carney, a stage manager who lives in Bedford, was at work in 2017 when he experienced the first of a series of mysterious symptoms. “We had just started a performance of Hairspray, and almost with the first beat of the opening, an intense earache started – like no earache I’ve ever had before.” The painkillers had no effect and when he returned home later, his face was strange in a way he couldn’t put his finger on. Within two days, he said, “he was completely paralyzed on his right side.”

Anyone who’s seen the news lately might correctly guess that Carney’s condition was Ramsay Hunt Syndrome – a complication of the varicella virus – thanks to Justin Bieber’s Instagram post announcing he had it. In a short video, Bieber demonstrated that he can only smile, twitch his nostrils, and blink to one side of his face. But before Bieber’s post, the syndrome was little known – it’s not even mentioned on the NHS website. It is often misdiagnosed as Bell’s palsy, which also manifests as facial paralysis on one side, meaning patients miss prompt treatment with antiviral drugs, which if given within 72 hours, could save them from life-altering chronic symptoms. .

For Carney, now 33, the worst part was not being able to blink. “I had to put drops in my eye every 20 minutes to keep it from drying out, and manually blink by pulling my eyelid down with my fingers. I had to tape it shut at night so I could sleep Just walking outside, even on a calm summer day, there was enough breeze to dry my eyes in seconds.

After an initial diagnosis of Bell’s palsy, it was not until the fourth day that he had an attack of what doctors later suspected was vertigo, and went to hospital, where he was finally diagnosed with Ramsay Hunt Syndrome – too late for antiviral drugs.

Charles Nduka, consultant plastic surgeon at Queen Victoria Hospital in East Grinstead, West Sussex, and Guy’s and St Thomas Hospital in London, founded the charity Facial Palsy UK in 2012 to raise awareness of the condition the long-term effects of which his team sees daily. , but in vain. Although precise data are difficult to obtain because misdiagnosis is so common – the reported incidence of Bell’s palsy is 20-30 per 100,000, for Ramsay Hunt syndrome it is around five cases per 100,000 – he estimates that in the UK there are around 25,000 new cases. of sudden-onset facial paralysis per year, and perhaps a third of those affected will end up with chronic facial symptoms. Ironically, he says, he even developed the condition himself during lockdown.

Matt Carney: ‘If I cough my right eye cries which was absolute pain when I had Covid.’ Photography: Facial paralysis UK

It is caused by the varicella-zoster virus which, after an episode of chickenpox, becomes dormant in the body – in the case of Ramsay Hunt syndrome, in the nerve cells. It’s basically a shingles infection affecting the facial nerve after the dormant virus has activated, “when your immune system is down,” says Nduka. “Patients we see will have previous physical or emotional stress, such as cancer, chemotherapy, immunotherapy, moving house, or workplace stress.”

Nduka had worked hard during the pandemic, he said. “I was giving a lecture on facial paralysis when my face started shaking, and I got this awful feeling on the side of my tongue and an awful taste, which is one of the first symptoms – altered taste because the facial nerve also supplies the forehead part of the tongue I knew what it was and was able to get treatment early on and managed to abort the onset He had another minor flare up a year later late, but again, he says, “I was able to hit it early.”

Unlike the sudden onset of a stroke, facial paralysis with paralysis develops gradually. The main symptoms that distinguish Ramsay Hunt syndrome from Bell’s palsy are, according to Nduka, pain in the ear, face or head, rash or blisters – often painful – in or around the ear, scalp, hairline or inside the mouth, altered taste on half of the tongue, hearing loss or tinnitus on the affected side and dizziness or vertigo. Sometimes, however, the rash can be hidden inside and the facial paralysis appears first, which means that even if the doctor knows about the syndrome, he can ignore this diagnosis. That’s why Nduka encourages treating any new facial paralysis with antivirals – which he says are cheap and have few side effects – to be on the safe side.

The two forms of sudden onset facial paralysis – Bell’s and Ramsay Hunt – are also treated with steroids. “The facial nerve runs through a bony tunnel in the skull, so if it becomes inflamed, there’s no room for it to swell, so it will cut off its own blood supply and stop working,” says Nduka. “Steroids are there to reduce inflammation and therefore reduce swelling and allow the nerve to recover sooner.” Early treatment with antivirals and steroids, he says, increases recovery rates in cases of Ramsay Hunt syndrome by about 50% to about 70%. “If patients don’t get prompt treatment with a combination of steroids and antivirals,” he says, “it’s a coin as to whether they make a full recovery.” Nduka says he really wants doctors to know about the disease.

It’s been nine years since Deborah Lack’s Ramsay Hunt Syndrome was misdiagnosed as Bell’s Palsy. Now 42 and based in Hertfordshire, she still has tinnitus and in her latest checkup – the first in four years, thanks to Covid – found no improvement in her facial movements. Other than giving him Botox to ease the stiffness in his facial muscles, she says, “They said to me, ‘There’s nothing else we can do for you.’ They want to do the Botox again, to see where we are in six months, and the next step would be to have resuscitation surgery: they take nerves from an area of ​​your body and then put them in your face to try to find the look and the smile. Obviously, there are no guarantees.

Deborah Lack
Deborah Lack: “I’m one of the most expressive people you’ll ever meet, and I can’t even pucker my lips.” Photography: Facial paralysis UK

Lack ran a dance school, but she gave it up after developing the disease. “I work with children, and children are so honest,” she says. “What’s wrong with your face?” Why do you have a wobbly face? Why is one eye smaller than the other? There’s not much you can take.

These days, she says, “At rest, if I wasn’t tired or stressed, you probably wouldn’t notice. I even learned how to pluck my eyebrows in a different way to make sure my eyebrows are level. I can’t even pucker my lips.

This makes everyday things like spitting up after brushing your teeth or drinking from sports bottles impossible. “You can also dribble, and if I go out to eat, I often put my hand in front of my mouth because I know one side can be open,” she says. For family photos, she always stands at an angle to hide her affected side.

Nduka says the disease can lead to blindness in the affected eye, and the long-term symptoms can have serious mental health consequences. “Basically, your face is what makes you human,” he says. “It allows you to interact with strangers, which is unique to humans.”

Facial rehabilitation can include a combination of approaches, he says, “such as neuromuscular rehabilitation, biofeedback training – learning to feel the face. It’s a long process that takes a lot of time and patience. Patients with weakness on one side may experience exaggerated movement on the other as they attempt to compensate for the lack of movement.

If therapy has not been effective enough, surgery is an option – for example, weighting the eyelid with platinum to help it close properly, “freeing” overactive muscles, or, “if the smile muscles don’t quite work,” Nduka says, “we can apply a nerve graft to those muscles..”

Carney considers himself lucky to have regained all of his movements. After two or three months, he recalls, “it started to come back on its own, but then I realized I had strange movements and stiff muscles.” He was referred to a specialist physio who helped him, but, he says, “if I close my left eye, my right eyebrow goes up, and if I smile – a really big smile – then my right eye goes up. closes a bit. And the weirdest thing is if I cough my right eye cries which was absolute pain when I had Covid.

He still has tinnitus, which comes and goes, but you probably won’t notice it now. “It took a few years to get to this point,” he says. “There is a chance that if I had had the antivirals from the start, I could have avoided paralysis altogether.” It’s just a shame we had to wait for Justin Bieber, he says, “to be able to bring it to people’s attention.”

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