If you read some articles on what causes cystic fibrosis, it can all seem rather confusing and esoteric – with technical jargon flying left and right.
To the rescue: this piece here, which explains – in simple English – the biology of cystic fibrosis. We’ll answer the question of what it is, exactly, that happens on a biological level to trigger the onset of cystic fibrosis.
Here’s what you need to know:
In people without cystic fibrosis, their airways – from the respiratory tract down to the lungs – are lined with a sticky, slippery substance known popularly as “mucus.”
This mucus lining, as it turns out, is actually incredibly important to the body.
Because the air we breathe is not pure. It’s filled with microscopic dust particles and bacteria. But whenever we take a breath, all of this dust and bacteria gets caught by the sticky mucus lining – preventing these particles from flooding into our lungs. What’s more, this mucus lining is actually in a constant state of motion – propelled by tiny molecular motors known as cilia, which transport the mucus (along with all the particles it has caught) into your gastrointestinal tract (which includes the stomach, among other organs).
It’s easy for the body to clear out old mucus – and replace it with new lining – because your airway surfaces are constantly hydrated with a water-based coat of liquid. This liquid makes it easy for mucus to move from the airways to the gastrointestinal tract.
But things don’t work like that in a person with cystic fibrosis – not at all, in fact.
Essentially, in someone who has cystic fibrosis – their airway surfaces aren’t hydrated with very much water at all. This is because of a mutation which destroys the function of a gene known as CFTR – or, in technical parlance, the cystic fibrosis transmembrane conductance regulator.
This gene is responsible for regulating the flow of chloride and bicarbonate ions throughout the body’s cells. Proper flow of these ions is absolutely necessary for cells to be well-hydrated (think of these ions as tiny “magnets” which help control the movement of water throughout the body’s tissues).
Without this adequate hydration, airway surfaces are unable to rid themselves of old mucus lining. So the mucus stays put, all throughout the airways, collecting more and more bacteria (and other microbes), dust, and debris.
The result? Microbes begin multiplying rapidly in the body’s air passages, and after years of microbes colonising the body in this way, they begin to produce inflammation and infection – severely damaging lung tissues which are only supposed to come into contact with pure air (and not an army of microscopic invaders). This, ultimately, makes breathing increasingly difficult.
That’s the gist of it – the underlying reason why cystic fibrosis occurs. Treatment strategies have evolved over time, but a cure is urgently needed. That’s why every JWLS watch purchase supports the Cystic Fibrosis Foundation – providing funds for the research and development needed for a cure.
“What – exactly – is cystic fibrosis, and why is it so deadly?”
This is a question that people often ask those who are deeply engaged in the fight against this genetic disease. Despite the devastating lethality of cystic fibrosis, not much is known about it by the general public. It is our view that spreading awareness about the exact nature of CF – its main features, its progression, treatment options, and so on – is an effective means of combatting the disease over the long-term.
And recently, a report was published by the American Thoracic Society entitled “Twenty Facts About Cystic Fibrosis.” Using this resource, and others, we have created the following, brief Q&A to address a few questions you may have about cystic fibrosis.
Q: What causes cystic fibrosis?
A: All humans are born with a protein known as CFTR (proteins are molecules found in all of your cells, and they perform a vast range of essential cellular functions). CFTR regulates the movement of salt and water within cells.
Some individuals, however, have a defective version of CFTR – purely the result of a genetic accident, or mutation. This less-than-optimal version of the CFTR protein is unable to effectively regulate cellular movement of salt and water molecules. Consequently, individuals with this form of CFTR experience dehydrated airways and an often deadly build-up of mucus – making breathing extremely difficult.
Q: What is the life expectancy for individuals with cystic fibrosis?
A: The average life expectancy for those with cystic fibrosis varies based on the nation under consideration. In Canada, for instance, the average life expectancy is slightly above 50 years of age. On the other hand, in the United States, the average life expectancy is around 40 years of age.
What these numbers do not reveal, however, is this troubling fact: half of those who pass away as a result of cystic fibrosis are 30 years of age or younger.
Q: How is cystic fibrosis detected?
A: One of the most common ways to detect cystic fibrosis is through the sweat test – which measures sodium levels in sweat (people with cystic fibrosis have an elevated concentration of salt in their sweat).
Q: What potential cures for cystic fibrosis show the most promise?
A: Currently, gene therapy appears to be a promising avenue for curing cystic fibrosis. The basis of this technology is the ability to replace defective genes and proteins with better functioning ones. However, there is still much more research and funding required to make this cure a reality – which is just one of many good reasons for getting a “timepiece with a purpose.”
“Having CF, on a good day,” writes Jo-Anne Giles, who’s lived with cystic fibrosis for 48 years, “Is knowing your body is highly unusual, a mutant body because of a mutant gene. CF isn’t like cancer or influenza; it isn’t the result of a foreign pathogen invading your body; it’s your code; it is a fundamental condition of your existence.”
Jo-Anne is not alone in this fundamental condition of her existence; approximately 1 out of every 3,600 children born in Canada are born with this incurable, and deadly, genetic disease. And worldwide, tens upon tens of thousands of people are afflicted with cystic fibrosis.
The lived realities of those with cystic fibrosis is not well-understood by many people; children diagnosed with CF often begin their mornings at earlier hours than most – as demanded by a regimen of breathing treatments and medications. And, on top of the physical stresses imposed by CF, the psychological ravages of the disease – heightened anxiety, for example – take a toll of their own.
These are realities which Emily Lyons – CEO and founder of JWLS – is all-too familiar with, having witnessed her sister Julia endure through these and other hardships of cystic fibrosis.
Now, more than 6 years after Julia passed away, Emily is determined to make an impact by channeling financial resources towards organizations involved in the search for a cure for cystic fibrosis.
One such organization is Cystic Fibrosis Canada, a not-for-profit charity which – for years – has accumulated a vast storehouse of information and data on cystic fibrosis. Known as “one of the world’s top three charitable organizations committed to finding a cure for cystic fibrosis,” Cystic Fibrosis Canada – which has existed for more than 50 years – was recently been selected by Emily Lyons as the recipient of a $10,000 donation.
With these funds – and other funds the organization receives from donors – Cystic Fibrosis Canada will be in a strong position to continue its quest for an effective cure for cystic fibrosis.
(Indeed, largely thanks to the efforts of Cystic Fibrosis Canada, the prospects of long-term survival for those with cystic fibrosis is steadily increasing.)
Emily isn’t stopping at this donation, either. Her company JWLS, which was launched earlier this year, is expressly devoted to providing funds to organizations engaged in the fight against cystic fibrosis. This, in fact, is the meaning behind the JWLS slogan, “Timepieces with a Purpose.” Every time a JWLS watch is purchased, a percentage of the company’s profits are donated directly to relevant organizations. Emily sees this approach – harnessing the power of capital created through business activities – as a powerful way to eliminate cystic fibrosis once and for all. It’s her hope – and the hope of thousands of people across the world – that cystic fibrosis will soon no longer be a fundamental condition of anyone’s reality.
Now that the holiday season is upon us, it’s time to start thinking about gifts for your loved ones. But let’s face it: some of us aren’t the best at gift-giving. It’s the thought that counts, of course, but one of the best ways to show someone your appreciation for them is through a well-thought-out gift.
So suppose you want to improve your gift-giving skills – and learn the art of finding gifts for people in your life that they really connect with on an emotional level.
How do you do that?
Here, straight from our gift-giving experts at JWLS, are some tips for you (of course, if you or a loved one would like a watch for the holidays, don’t forget to check out the JWLS collection here):
- One of the best ways to hone your gift-giving skills is by developing the ability to pick up on hints that people drop (consciously or subconsciously) – things they say that clue you in on what sort of gifts they might love. For example, if you’re at the mall with them – or perhaps browsing the web together – and they see a watch and make the comment that they love watches, then simply make a mental note of this. People will unintentionally drop these sort of hints throughout the year, too, so it may be a good idea to keep track of such comments on a notepad app on your smartphone. Then, when the gift-giving season rolls around, you’ll have a good idea of what they like – and it’ll make you look unbelievably thoughtful, too.
- Not everyone has this – but if they have a wishlist somewhere (like Amazon Wishlist), then don’t forget to have a look at that.
- It’s also a good idea to record the gift ideas that spontaneously occur to you. Sometimes, you might get struck by an epiphany of a possible gift to get them – and when that happens, it’s useful to collect these ideas somewhere. To that end, picture boards like Pinterest (and other boards) are ideal – simply find a picture of the gift that occurred to you, and add it to your board.
- You may also want to try a brainstorming exercise to come up with good gift ideas for your loved one. Start by making a list of things you know about them. Begin with something simple – for example, list their favourite colour, preferred kind of music, TV shows they enjoy, and so on. Then, continue listing even more things you know about them – the first items you jot down will help jog your memory so your brainstorming can move forward. Use this brainstorming list to find a gift that is personalized to their tastes; for example, knowing what their favourite colours are can allow you to choose a watch with those colours. Your gift, then, will appeal specifically to their tastes, sense of fashion, and so on.
Nestled along the Euphrates River in Mesopotamia – some 3,500 years ago – lay a thriving, bustling city known as Babylon. Here, some of the first, primitive sundials would be invented and used for early efforts in astronomy and timekeeping.
With the creation of the sundial, human civilizations had found a more reliable, consistent way to keep track of time. But efforts to track time even more precisely continued – relentlessly. Thus, from pendulums to atomic clocks, and from water clocks to wristwatches, innumerable timekeeping methods have emerged.
Now, as horological aficionados, we think watches are one of the most beautiful examples of timekeeping, because the watch combines fashion with function (the function of tracking time, to be exact). Fellow horological enthusiasts, then, will enjoy reading the following surprising, intriguing facts about watches.
- Before the invention of the wristwatch, pocket watches were “in.” Pocket watches were seen as a symbol of good taste and fashion sense. In fact, during the very early years of the 1900s, wristwatches were at first mocked as a passing fad – and were derisively referred to as “wristlets.” It would take a few decades before wristwatches gained prominence in culture and society – and turned the pocket watch into an obsolete piece of history.
- How did the word “watch” come to mean a personal instrument for timekeeping? No one knows sure, but one hypothesis goes back to the word’s Old English roots. In England, in bygone days, town watchmen – charged with public safety tasks – would use crude watches to keep their shifts on schedule. And a watchman was called a “woecce” – hence the origin of the word “watch” in the timekeeping sense (according to this hypothesis, anyway).
- Modern watches use a variety of power sources, including battery cells and solar power. One of the most unique (but rate) ways to power a watch? Through the body temperature of the watch wearer. Such watches are basically heat-powered.
- Early watches had to be opened up regularly for winding and maintenance by the watch wearer. Thus, watch designers paid just as much attention to the design and aesthetic of the watch’s inner workings as they did to the watch’s outer appearance. Some examples of this kind of “internal decoration” included engravings of stars, roses, and tulips – which could be seen only when the watch was opened and its interior revealed.
- One of the first wristwatches was invented by Louis Cartier for early 20th century airship pilots. Because these pilots had to keep their hands on the airship’s steering equipment at all times, it was impractical (and even dangerous) for them to dig into their pocket for a pocket-watch. Yet keeping track of time was essential to approximating how much fuel was left in the airship. Louis Cartier’s solution? The wristwatch. Pilots could then steer the airship while taking quick glances at their wristwatch – no pocket-watch needed.
When Instagramming and Watch-Wearing Collide: How to Take the Most Social Media-Worthy Photos of Your Watch
This goes out to all of you who are both avid watch enthusiasts and inveterate Instagrammers (with an instinct for posting captivating pictures and selfies).
If you’ve ever tried to take a good photo of your watch, and post it to Instagram (or Facebook and other social media sites) – then you likely know it can be a bit difficult.
For example, unintended glare – from the light reflecting off the face of the watch – can spoil an otherwise good shot.
So what to do? How do you take the perfect photo of your watch?
To begin, your phone needs a high-quality camera. This is kind of a no-brainer for most Instagram aficionados, but is still worth mentioning. You definitely don’t want photos of your watch that are either too grainy or blurry – these are the sorts of pics that get 0 likes, after all.
So – say you have a high-quality camera. What then?
Well, you want to get the lighting right. Proper lighting is always important in the photographic arts – especially when you’re taking shots of a fashionable timepiece. Ideally, don’t use your camera’s flash function – this can mess up the shot due to light reflecting off your watch.
Instead, take the photo of your watch in an area that’s flooded with natural light.
That way, you don’t have to use the flash feature – and you’re still taking a photo where your watch is very visible, surrounded by ambient light. The key here is to eliminate all possible sources of reflection when snapping a pic of your watch.
Alternatively, you may wish to purchase a lightbox – or create your own (DIY instructions can be found here and here). Lightboxes are awesome tools for showcasing your taste in fashion – and completely eliminate undesired sources of reflection.
Another word of advice: use your phone camera’s timer function when you take the shot, because this allows you to take a picture of your watch without having to press down on any buttons to capture the actual photo.
Then, have a look at the photo – experience a satisfied smile on your face – and upload it to Instagram.
“Life is about circumstance and choice.” – Michael J. Fox
I thought I knew what loss was.
But it wasn’t until 2011 when I lost my sister that I truly experienced indescribable grief.
My beautiful, strong sister succumbed to the disease (cystic fibrosis) she fought her entire life. I always knew she was sick since I was a little kid, but for some reason I never thought I’d actually lose her.
I took it for granted that every day I would wake up – and she would, too.
It wasn’t until that moment in the hospital when she took her last breath, that I took one of my own last breaths. One where my sister was in the world. The former me also died that day – and a completely new Emily was born.
It’s hard to describe the things that happen when you lose someone you love so much.
The inexplicable pain.
My sister was my biggest supporter, my toughest critic, and my oldest friend. She was the one who told me how things really were, who knew every memory I could call to mind, and who healed my oldest, deepest wounds.
She bought me the book that inspired my career into entrepreneurship – as well as my first laptop to get started. My earliest memories are of the two of us playing and laughing – hiding in our old attic to make prank phone calls, sneaking fudgesicles at our dear Auntie Carol’s. I remember looking up to her in everything that she did.
I was 25 years old when Julia passed. Grief was the catalyst I needed to become the person I was meant to be. For many, death is a wake up call – and it was like that for me. Julia’s death gave me a constant reminder of how short life is – and what is really important in life.
To always be kind to others.
That superficial things don’t matter.
And to dive in head first towards chasing your dreams. Take risks, because none of us know when our time is up.
After losing my sister my life became full of total purpose. The grief I suffered carried me to places I never knew I could go – and forged me into a person I never knew I could be. It sparked within me the drive to succeed so I could help others. Each business became combined with my personal purpose. My new-found values became entrenched into all business activities I undertook. Everything I had learned before – about keeping the personal separate from the business – washed away. And the theory that you have to be ruthless to succeed proved to be entirely untrue for me. I can help others, be kind, and still be successful.
Creating JWLS was my next step in continuing this journey. Inspired by Julia – with endless possibilities. She lives on with each timepiece worn – so her journey and inspiring, unstoppable spirit continues on, too.
4 Reasons to Get a “Timepiece with a Purpose”
Why do some people prefer to wear fashion pieces that have a deep meaning or a purpose behind them? (For example, a JWLS watch isn’t just a watch – it’s a watch designed specifically as a symbol of the fight against cystic fibrosis.)
If you’ve ever asked yourself that question, then here are just some of the reasons why fashion-conscious individuals often choose to wear fashion-with-a-purpose items.
(1) It makes a statement. And who doesn’t like a fashion item that makes a statement? It can be a bold thing to wear – but if bold is in your blood, then getting a “timepiece with purpose” might be an appealing thing to do.
(2) It starts conversations. People in social situations love finding “excuses” to talk to someone, and wearing a fashion piece with a purpose is the perfect reason for someone to start a conversation with you.
(3) It makes a great gift. If you’re looking for the perfect gift for someone who’s important to you, then a fashion piece with meaning behind it makes an amazing gift. You are gifting the person more than just a product; you are giving them something with real meaning.
(4) Last, but definitely not least – in fact, this is the most important reason – wearing a fashion piece with a purpose can help a cause in a very real way. For example, every time a JWLS watch is purchased, a portion of profits are contributed to research organizations on the hunt for a cure for cystic fibrosis.
To get yourself a great-looking watch – and help advance the mission of defeating cystic fibrosis for good – then start here.
There’s an upbeat Canadian rock song by Trooper that shares imparted wisdom to the songwriter.
“A very good friend of mine,” the song’s singer soothes, “told me something the other day …”
Of course, the singer continues with their anecdote, fully aware that with every sunny day, there’s got to be a little rain that falls.
“We’re here for a good time, not a long time … so have a good time, the sun can’t shine everyday.”
The 1977 ditty, although a positive groove, reminds us of our own mortality, and the importance of enjoying that time we have.
What better way than to give back to those who are experiencing their own rainy days with cystic fibrosis.
Our CEO, Emily Lyons lost her sister Julia to the genetic disorder, and in a recent interview with her hometown paper, the Stratford Beacon Herald, shared the goal to help families dealing with the repeated trips to the hospital.
During her childhood, the family had to stay at the Ronald McDonald house. “We had tough times,” Lyons said. “It’s hard enough when you have a dearly-loved family member in the hospital, but when you also have to endure through financial stresses – it can get pretty hard.”
WATCH AND LEARN
Ergo the launch of JWLS, a watch line that harkens back to Trooper’s plea to enjoy life. In tandem with the watches, Lyons is also introducing the Julia Lyons Foundation.
The goal is to help families struggling to live while having a sick child. Forty percent of sales from the JWLS watches will go to help alleviate this strain.
A friend of Lyons’ had a brother receive a double-lung transplant. Their mother had to stay home from work to help take him to doctors’ appointments and deal with side effects of anti-rejection therapy.
“If we could facilitate those things and make it a lot easier, it would relieve a little stress for those families,” Lyons told the Herald.
It’s all about the importance of giving back. And like the minute hand, we’ve come full circle. Simply put, the watch is a reminder of how precious time is.
Shop the JWLS collection online, or visit their Toronto watch store.