Drawing strength from growing up with LHON

With a successful music career already in the bag, 24-year-old Fredrik Guzmán Lindemark is now making his mark as a start-up entrepreneur and investor. But his long list of accomplishments did not seem possible when he was diagnosed with the life-changing and rare sight loss condition, Leber’s hereditary optic neuropathy (LHON), at just 15.

As part of our Living with LHON series, Fredrik tells us what it’s like to grow up in the shadow of sight loss, and how he has drawn strength from learning to live with his condition.

Living with the fear of death

Fredrik first experienced the symptoms of LHON in his left eye when he was just 14 years old and was diagnosed at the age of 15.  Asked about his first symptoms, he said he remembered them vividly.

“I stepped into the kitchen where my Mum was preparing breakfast. I told her there was something bothering me, and that I might have to get glasses. I couldn’t see the oven timer that was between four and six yards away from me,” he said, adding that his Mum chalked up the issue to a migraine – something Fredrik often experienced.

However, the problem persisted for a couple of weeks and after some “nagging” his Mum took him to see a GP. Once there, they were met with an “unknowing silence” and Fredrik was referred to a specialist hospital.

“I met with the doctor on the 23rd November 2009, which was my 15th birthday. He did a whole bunch of different tests, after which he just looked at me with a blank face.”

With hindsight, Fredrik said the doctor’s facial expression was probably one of confusion. Because LHON is a rare disease, affecting around one in 50,000 people in Europe, not all healthcare professionals are aware of it, he explained.

“Then he grabs a book and starts skimming through the pages. As I hear him mumble some words to himself, such as ‘nerve pressure’ and ‘brain tumour’, my Mum looks at me and demands he request I leave the room for a moment so that they can talk in private,” said Fredrik.

“I remember coming back into the room, and he had called for a couple more doctors to add their thoughts and opinions, but they still didn’t know or understand what could possibly be causing the sudden drastic loss of vision.”

The doctors said it could have been a stroke or simply pressure on the optical nerve, but they also said that the “absolute worst-case scenario” was a brain tumour. This, Fredrik said, scared him “out of my mind”.

“I want to allow people to access healthcare so they will be able to ask questions when they have them and receive treatment when they need it, not when they are told they can have it”

It took three to six months and countless hospital visits for the doctors to arrive at the diagnosis of LHON. The genetic disease is passed down the maternal line and leads to central vision sight loss, usually within weeks or months of onset.

“I can’t remember much in detail of how I handled that time. Some moments just feel like a big blur. Most days I couldn’t get myself to school or do anything else for that matter. It was just hell.

“In the months before they suspected LHON, they thought they had to treat it as though it was a brain tumour. It was really, really tough.”

When he received the diagnosis, he tried to “look on the bright side of things” – after all at least he wasn’t going to die.

“I asked, ‘do you know what it is?’ When they said they did, without thinking I quickly asked, ‘am I going to die?’  They said no and I shouted ‘awesome’. At the time my right eye was still functional, so I thought I was all right.

“But living with LHON is like a rollercoaster. You don’t know if you are going to get better, worse or stay the same. I could go home, sleep, wake up the next day and not be able to see out of my right eye either.”

Growing up different

Being a teenager is difficult at the best of times but adding a complicated long-term condition into the mix can make growing up even tougher.

Fredrik explained: “LHON had affected my personality in many ways. My perspective on life had changed but luckily it was for the better. I felt more tolerant, open and understanding. I had adopted a more serious outlook on life, which sometimes made it complicated to connect with my old friends.

“I had a girlfriend, my first love, and she was there for me but it’s hard to go through such a thing at that age. She could see how I had changed completely.”

Unfortunately, things got worse when his right eye also became affected by the condition within weeks of starting secondary school.

“I got really depressed again. It was a huge shock, and I was distraught. I already knew that it might happen, and somehow I felt like I was weak for not just getting through the struggle without all the depression,” he said.

Splitting up with his girlfriend around the same time only served to add to the despair, but Fredrik’s love for music become his saviour.

He had played the piano since he was five, and then he started making house music on his Apple computer by utilising special zoom functions and speech functionality.

“I wanted to be the best I could be and to see how far I could go on a professional level. I thought it would be pretty cool if I could make music even though I could barely see. The fact that it shouldn’t be possible triggered me to want to do it even more,” he said.

This passion and dedication led to the formation of his first company, a production label, and before long Fredrik was ditching school to collaborate with some of the biggest names in house music.

LHON as a benefactor

Fredrik believes the diagnosis of LHON changed his life in many ways, but mostly for the better.

By his own admission, before the condition developed, he was “getting into a lot of trouble” and his family were afraid he could even end up in prison. But when he was diagnosed, things started to change.

“I felt I had been given a new perspective on life. I wanted to achieve something and do the best I could for myself and the people around me. I simply wanted to live life to the fullest,” Fredrik explained.

He has been true to his ambition. After winding up his professional career as a music producer, a new opportunity quickly arose.

A chance meeting with a small group of start-up entrepreneurs in his mum’s kitchen led to him becoming a serial entrepreneur and investor. His interests include clean energy and water, healthcare and medical technology.

One of his more recent investments is a med tech company that allows people with chronic diseases to be monitored remotely. It’s a cause close to Fredrik’s heart.

Outlining his vision of the future, he said: “I believe that we are going to see a more proactive healthcare environment in which it’s cheaper to keep people healthy than to wait until they are ill.

“If you get a chronic condition like LHON or diabetes your life completely changes. It interrupts your everyday life. That’s one of the issues I want to change. I want to allow people to live normal lives and access healthcare so they will be able to ask questions when they have them and receive treatment when they need it, not when they are told they can have it.”

All his investments are driven by his desire to make the world a better place, and this desire has been fed by his own experiences.

“When you have LHON, your whole perspective on life changes dramatically. I’ve heard the same from several other people who were diagnosed around the same age: that their whole life changed, and they feel more mature because of it.

“LHON is a huge obstacle, but it can also be a huge benefactor,” he said.

Re-evaluate and reassess

Being diagnosed with LHON at just 15 was a devastating blow for Fredrik. It forced him to re-evaluate his life and adapt to a new reality at a time when his friends had nothing more to worry about than keeping up with the latest trends.

But through sheer determination, he turned his love of a music into a career before he’d even left school. And now he is using the resources and strength he gathered along the way to make the world a better place.

To read further articles in this series please visit our Disease Spotlight: LHON channel produced by pharmaphorum in conjunction with Santhera Pharmaceuticals.

About the interviewee

24-year-old Fredrik Guzmán Lindemark was diagnosed with LHON at the age of 15. After winding up his professional career as a music producer, a new opportunity quickly arose. A chance meeting with a small group of start-up entrepreneurs led to him becoming a serial entrepreneur and investor. His interests include clean energy and water, healthcare and medical technology. One of his more recent investments is a med tech company that allows people with chronic diseases to be monitored remotely.