HNPP · Profiles

Profile: Barney Lee journeys around Britain’s coastline for HNPP awareness

Featured on Radio Derby on August 10.

“Life is really what you make of it and going on a big adventure is something I really want to do and do something great with it.”

Barney Lee could potentially become the youngest person to walk around the coastline of mainland Britain. What makes this plucky 19-year-old even more unique, is that he walks with purpose, raising awareness about a “life-changing” condition called hereditary neuropathy with liability to pressure palsies (HNPP) – something he suffers from himself.

The Derby teenager commenced his 4,500 mile walk on August 10 from Mablethorpe in Lincolnshire, in a bid to try to fund further research and possible treatment for this little-known degenerative neuromuscular disorder through donations and sponsorship. 

Ahead of the walk, the Littleover adventurer consulted with the Wayne State University Neurology Department Chair in Michigan, Dr Jun Li. He says: “He seemed positive about the ambition of it but he had concerns about the potential side effects and things to do with HNPP with my body.”

The three factors he has been told to watch out for are doing repetitive movements for a prolonged period of time, stretching excessively, and limit staying in the same position for too long. Unfortunately for Lee, walking clearly consists of the former. He says: “I can alter [my walking] so I can do less in the day and have rest days, I can also walk in a different style – just think of [Monty Python’s] Ministry of Silly Walks with John Cleese going down the coast!”

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While Lee is no stranger to the great outdoors, having climbed five of the highest peaks across the UK and Ireland, some of the symptoms he has already experienced are hand and arm numbness, loss of grip, as well as some strain on his legs. However, it has not stopped him from undertaking this huge feat.

He says: “[Realistically] some kind of damage will happen which is irreparable, but that’s kind of part of the sacrifice of it, to get it out there and potentially make a difference”.

The rare genetic illness has however, stopped him from going to the gym with his friends, as well as choosing a career path of his own.

“I have noticed that’s it gotten progressively worse if I compare myself to a year ago, things aren’t working as good.”

The plumber, who had also trained to become a handyman and landscape gardener, says that the condition has inhibited him in considerable ways.

“It felt like I had ten pairs of gloves on my hand and I just couldn’t move them,” he says about  how he felt the day after digging a garden during landscaping job, adding that career-wise, it would be a “bad idea” in the long and short-term, in regards to the impact on his body. 

Officially diagnosed only several months ago, but knowing about the condition through his mother’s own debilitating experience with it, the hiker says he avoided getting tested because he “did not want the label”.

He adds: “Once you have a label on you it’s not something you shake off, you have it and it’s just something you live with. And I thought why get a label on something that is currently incurable and untreatable?”

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Hence the avid traveller is embarking on this journey to “generally put [the condition] in people’s minds”, and hopes that by alerting individuals to the disorder, more will notice their own symptoms, and therefore push medical companies to conduct clinical trials. With currently only 2 to 5 per 100,000 individuals diagnosed with it, Lee is certain that there are far more affected than currently recognised.

“The doctors and experts think that it’s more like 1 in 2500 people because of misdiagnosis. And the only way we can change this is by doctors more aware of this so they are able to diagnose correctly and know about the condition,” he says.

And Lee hopes that during his trip, he will be able to “hit all the nails on the head” after Dr Li, a leading researcher of HNPP, helped him find a way of channelling his initiative by recording his symptoms and movements in a medical journal, as part of a study to share with his team.

“The only way I can change my fate and other people’s fates is doing something positive like doing this walk and raising awareness.”

“My hope is that in my lifetime I’ll have played a part or a role in bringing about a change which will help potentially me, and definitely the next generation, my kids and things like that will help them out.”

And walking around the coastline appears to mean much more to Lee than a fun (but gruelling) way to raise funds, stating that he sees the campaign as a “parallel” to how people experience the condition.

“It can be a long hard struggle and it’s something that goes on forever until some kind of cure. So I’m trying to mimic that in some way to try represent what the condition is about […] you have good days and bad days.”

The rambler is also hoping to have friends, family and other walkers join him throughout the 14 month trip, as well as offers of basic amenities such as accommodation and showers. Fellow HNPP suffers have also lent a hand by providing beds throughout the adventure.

Lee, who sees the possibility of writing a book about his tour, says: “It’s just really amazing how people have got behind it, I’m really looking forward to it and what it brings and how I change and develop as a person from it, and potential amazing new friends and amazing stories to tell.”

There are lots of ways to get involved – find up to date information on Lee’s Facebook page or follow him on Instagram. And check out his journey progress at Barney Lee: My Long Walk Around Britain. To donate towards Lee’s trip or to HNPP research directly visit his page here

HNPP · Profiles

Profile: Branden Jacob-Jenkins on the importance of being ‘self-aware’ with HNPP

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“You’ve just got to make it work for you”, says award-winning American playwright Branden Jacob-Jenkins about living with the condition hereditary neuropathy with liability to pressure palsies.

Jacob-Jenkins, who hails from New York City, is no stranger to the public eye. The 35-year-old playwright is already a two-time finalist for the Pulitzer Prize, and a 2016 MacArthur fellow, having toured the world with his renowned adaptations. But less known is the fact that while achieving huge success in the theatre world, winning both the Windham–Campbell Literature Prize and PEN/Laura Pels Award in 2016, Jacob-Jenkins battles with migraines associated with HNPP.

“I think [HNPP] has influenced a lot about the way I think about my relationships with my body, which has only enhanced or runs alongside the way that being a black male already does that.”

“I live with visual auras pretty frequently, and that is a really significant issue”, says the writer, who has recently joined the faculty of the University of Texas’ MFA playwriting program.

The rare genetic nerve condition can present an array of symptoms ranging from chronic pain to fatigue, varying from one person to another.

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Speaking to him in London last year, during his sellout run of An Octoroon at the National Theatre, Jacob-Jenkins mentioned that his frequent migraines began soon after being diagnosed with the condition at the age of 19.

He says: “It was crazy, there’s just a period of not-knowing that is so frightening. The worst part was that people thought it was a brain tumour, I had so many scans done.”

Jacob-Jenkins has subsequently had two surgeries on both his ulnar nerves to rectify some of the more extreme symptoms that he initially experienced.

“The first major symptom was I woke up one morning and I could not lift my left arm, which meant there was some sort of major palsy happening […] And in that original diagnosis, I realised that the numbness I had been feeling in my fingers [when younger] was due to this. I remember having no feeling in my pinky finger. And it wasn’t until that doctor said that this is probably related that I realised.”

The playwright, who was born in Washington DC, speaks about the uncertainty that came with his original diagnosis, and the fact that it took months to pinpoint. He says he was baffled by what this implied: “What do you mean I have HNPP, what does that mean? I couldn’t Google it. This did not tell me anything. All I know is that my arm can’t move and they would give me carpal tunnel exercises that would make it worse and it was just really difficult.”

“Here was a moment where a total lack of information about the disease was having an actual repercussion on me socially.”

While he describes his primary reaction as “traumatising”, Jacob-Jenkins, who has a major in Anthropology from Princeton University, says it may have inadvertently helped him develop his theatrical style. “I think it has influenced a lot about the way I think about my relationships with my body which has only enhanced or runs alongside the way that being a black male already does that […] Weirdly, almost every play of mine is obsessed with asking actors to use their bodies in different and stranger ways. They are either changing character over the course of the piece or they are negotiating it as an obstacle in terms of telling a story. I think that’s totally, weirdly related to my experience with this disease.”

This is certainly highlighted in An Octoroon, a radical adaptation of Dion Boucicault’s 1860’s play The Octoroon, in which the actors adopt blackface and whiteface in this version, while Jacob-Jenkins emphasises the politics of actual bodies onstage as conduits for meaning or emotional value that have changed over time.

“I’m always observing my body removed and I’m always thinking about the limitations in a way that I don’t think a lot of people do, so I do think that oddly it has some deep level that has affected my aesthetic impulses in a funny way.”

The writer first spoke about his condition publicly in a New York Times article, in which journalist Alex Witchel describes the “shocking news of his health”. However, Jacob-Jenkins appears to take it on the chin: “They think you’re dying of a degenerative disease […]  So me in this article, appears as if I’m stoic in the face of impending doom. It was also the first time I realised that I manage it fine but suddenly everyone knew I had this thing.”

“It’s about self-education and the fact that my nerve health is going to degenerate, that’s the definition of the disease, but you can take preventative steps to try and slow the process down.”

He adds: “And here was a moment where a total lack of information about the disease was having an actual repercussion on me socially.”

Despite the impact of revealing his condition, he says he has the support of his partner who he married last year. “I have my partner. But it would be nice to know more people who have this. I like my neurologists a lot and I have my friends. Right now, I’m fortunate enough to be able to walk around by myself. In a way I know the hardest is yet to come so I’m trying to enjoy what I have today.”

Regularly seeing neurologist Bridget Carey at the Weill Cornell Medicine in New York has aided Jacob-Jenkins with managing the condition. “Having someone being able to monitor this thing and coming to a conclusion about that just feels so good. You just have to own it, and stay vigilant about it.”

“Right now, I’m fortunate enough to be able to walk around by myself. In a way I know the hardest is yet to come so I’m trying to enjoy what I have today.”

The playwright says he maintains a balanced lifestyle with his “low-impact job”: “Supplements are a huge part of my diet, so Vitamins D and K , Magnesium – these things are really important and I do think they help […]  You don’t have to go to a gym and pull weights but actually you can work at flexibility and strength in small ways and it still makes in the long term a big difference. Just modify! Physically I don’t think of myself as ill. I have an illness, but I’m not an ill person […]  But in terms of work, I’m a very lucky person.”

“Physically I don’t think of myself as ill. I have an illness, but I’m not an ill person.”

And fortune favours the bold and unstoppable, as Jacob-Jenkins’ outstanding play Gloria, receives its regional premiere at Curious Theatre in Denver, collecting glittering reviews.

“I’m never going to stop working in my life. And it could have been much worse. I still have to live my life, and the body is quite resilient in spite of itself so you’re going to bounce back.”

His final word on the matter? “It’s about self-education and the fact that my nerve health is going to degenerate, that’s the definition of the disease, but you can take preventative steps to try and slow the process down.”

Gloria, presented by Curious Theatre Company through February 16, 1080 Acoma Street, 303-623-0524, curioustheatre.org.

HNPP · Profiles

Profile: Jessica Kellgren-Fozard on living with an ‘invisible disability’

“I received a lot of online hate based on how I am not visibly disabled,” says TV presenter Jessica Kellgren-Fozard. Jessica, 28, from Brighton, UK, is one of the only known people with Hereditary Neuropathy with Liability to Pressure Palsies (HNPP) to be in the public eye.

As the presenter of Latest TV, a Brighton-based TV station and a former contestant on BBC’s Britain’s Top Missing Model, keeping up appearances can be tough, especially with a hidden illness.

“When I was working for a TV station in Brighton I received messages on how I wasn’t disabled enough. And that was it, I decided to put myself out more and talk more about life with invisible disabilities in order to help people like me,” says the model and writer.

“I received a lot of online hate based on how I am not visibly disabled, and supposedly not disabled enough.”

At a young age, Jessica began showing symptoms of HNPP, including extreme palsy in her arms as well as patches of skin being numb, difficulty gripping things, weakness in limbs, as well as fatigue – common symptoms of this inherited condition. “As a child I thought it was normal to not feel the chins in my knees,” she says, after being eventually diagnosed at the age of 17 with the disorder.

She adds: “My reaction was one of relief because I finally had a name for what had been happening to me, and also for why it was happening though my childhood.”

View this post on Instagram

This is me eight years ago, this is me this year and here are some things you might not know about me… I missed most of my teenage milestones because I was either in hospital or lying in the dark. I was too ill to open my eyes, to sit up or to eat. I missed exams, parties and the friendships I lost. I was made to feel like a burden, like a problem, as if my presence was a hindrance to other people's lives: "how good you are to look after her"… I'm still in the room. But there were some amazing people who stepped in to my lonely space, took my hand and helped me through those rough years. They didn't mind helping out physically or spending time making me laugh and always let me know it was no big deal- I was never a burden to them. That was the best gift they could have given me. That was love. All of this is why I'm so angry when media like #MeBeforeYou comes along! It's a dangerous thing to tell people that the only way you can thank those who have helped you is by removing yourself from their lives! I'm so much better than I was but still need help and I know the best way to repay that help is to live the best life I can; to be brave, to be bright, to be happy! I'm proud of everything I have achieved and where I am now. You can watch my latest vlog for more: https://youtu.be/nE8CESB0NeI Love to everyone still in that place ❤️ #MeBeforeAbleism #LiveBoldly #DisabledandProud #disability #ableism #chronicallyill #beforeandafter #mylifeisworthy #spoonie #disabilityawareness #wheelchair #bestfriend #nowandthen #instagood #instadaily #bichon #deaf #ootdsocialclub #happiness #love #vintage #retro #pinupgirl #glamour #iger #youtuber #celebratedontdiscriminate #chronicallyfabulous

A post shared by Jessica Kellgren-Fozard (@jessicaoutofthecloset) on

The condition affects one or more of the peripheral nerves, which connect the brain and spinal cord to the muscles and to the sensory cells that detect touch, pain and temperature. One copy of the gene PMP22 is lost, causing the protective outer layer of each nerve (myelin) to become fragile and more susceptible to injury.

On top of having HNPP, Jessica battles with Mixed Connective Tissue Disorder (MCTD), an autoimmune disease in which the body’s defence system attacks itself. MCTD causes chronic joint pain, muscle inflammation, hypermobility, pulmonary hypertension alongside a huge list of other symptoms.

And owing to weak connective tissue and damage to the nerves, Jessica is also deaf. Hence, fighting these conditions while attempting to live a relatively steady life poses its own issues.

“I avoid sitting, standing or laying on the same position for too long,” she says.

After recovering from a long bout of illness, the then 19-year-old Jessica applied to an advert looking for people to take part in a BBC TV show about people with disabilities, specifically focusing on young women who were interested in fashion. “It ended up being a six part series about disabled girls becoming models.”

The show, Britain’s Top Missing Model, featured an array of young hopefuls with various disabilities from across the globe. Sadly, Jessica received a torrent of online abuse for her invisible illness. She says: “Afterwards I received a lot of online hate based on how I am not visibly disabled, and supposedly not disabled enough.”

Watch more here.

The stigma surrounding hidden illnesses spurred Jessica on to continue keeping up a public profile so that others can speak out about their own experiences about invisible disabilities.

“I did struggle in school and university, because I felt they weren’t prepared even if they were willing to help. There was just not enough funding or education on how to properly help students with a disability.”

Thankfully, Jessica is backed by her loving wife, Claudia, her two adorable dogs, as well as her parents who have been an important part of her support network. “My wife lovely rolls me through the night,” says Jessica, who married her partner last year.

Managing the condition is critical to how Jessica functions, as she recommends listening to your body for the correct answers. She asserts: “[Your body will] tell you when you rest and when to go for it and do all those things you want to do. Don’t overwork yourself, that’s very important. I also take a lot of prescribed medication, so talk to your doctor if you are doing to pain daily.”

So what next for the feisty fashionista? Jessica says she plans to continue spreading the word: “I am going to carry on with my YouTube channel and my writing. Just generally be there and support and inspiration for people going through something and need someone to look up to.”

Find out more about Jessica’s journey at Jessica Out Of The Closet.

Follow Jessica’s YouTube channel here: Jessica Kellgren-Fozard.