[00:00]
[Music] My name is Michael Vinda, I'm 28 years old, and I'm a severe Hemophilia A patient. I was diagnosed when I was 7 months old, and it was a spontaneous mutation — so I was the first in my family, and I hope to be the last, of course. Hemophilia is a rare disease, so if you've never been in touch with it, you might have just heard of it as "the bleeder disease" or something like that. Often, if people don't have any association with hemophilia, they might say, "So if you cut yourself, you bleed to death?" — that’s a reaction you hear a lot.
[00:52]
It had a very, very big impact on our lives. We had never heard about this disease before, so everything was new, and we were very shocked. Every time he was a little child and he fell, we had to go to the hospital. Between his first birthday and about his sixth birthday, we had to go to the hospital daily. It had a huge impact, because the hospital was 30 km away from our home, and it took almost 4 hours to inject him. So it was like a job for me — I couldn't work at that time because it took too much time to manage Michael’s treatment.
[01:34]
Hemophilia to me is like brushing my teeth — and I almost mean that literally. When I get up, I take a shower, do my hair, brush my teeth, go downstairs, take my breakfast, and then I infuse. I infuse prophylactically, which means I do it every day. I go through life like any normal person would, but I have hemophilia — so I always have to keep it in the back of my mind. Sometimes it comes up, and sometimes I have to take extra precautions or make certain decisions differently. I can do basically everything — but not everything is necessarily the smart thing to do when you have hemophilia.
[02:18]
When you treat your hemophilia, you can develop what we call an inhibitor. That means your body creates a reaction against the treatment, breaking it down really fast — which is kind of like the treatment not working at all. When I was younger, I had an inhibitor, and having an inhibitor had a big impact on how I managed and treated my hemophilia. I have some friends who still have inhibitors, and it's like having no treatment available — it just doesn’t work. I actually still have joint issues today that trace back to having that inhibitor.
[03:39]
Now, together with my doctor, we create a management plan to make sure I don’t experience any major problems. We’re fortunate that hemophilia is a very treatable disease — as long as you make a good treatment plan and stick to it, you usually don’t experience serious bleeds. If you have hemophilia, you will experience bleeds — but there’s a big difference in the types of bleeds that require action. For example, if you have a muscle bleed, you’re going to have a big bleed inside the muscle, which is a serious problem. You have to avoid using that muscle and treat it properly, and it can take time to recover — the impact on daily life is much bigger.
[04:46]
The same goes for joint bleeds. That means blood enters the joint, and as you might imagine, a joint is a very tight, small space. When it fills with blood, you're no longer able to move it. The impact of a joint bleed is also significant — blood contains iron, and iron actually damages the joint. Your body has to clear that out, which takes time, and it can have long-term effects as well.
[05:22]
I try not to worry too much about what might happen in the long term with my hemophilia, because you really don’t know. I know I have some issues with my ankle and my elbow — they might get worse over time, or they might not. So I try to go about life as it is. I focus on what I can do, and try not to dwell too much on what I can’t. And that’s kind of my motto in life.
[Music]

[00:01]
How does our blood clot blood clotting is an important process that stops a person from bleeding in order for a blood clot to form a specific sequence of events with many different components must take place think of these components as a team of sports people known as team clotting who are each trained in a different sport but all taking part in an overall team competition the team members all depend on each other to complete the competition if one team member's event is disrupted it affects the competition
[00:36]
As the team cannot move forward without each other this is the same for blood clotting where different components work together to form a blood clot if one component is affected then the blood does not clot how does bleeding stop the first to arrive are the platelets attracted to damaged blood vessels a bit like spectator is attracted to the sports arena where the action takes place the competition Begins the first event is a relay race involving a team of clotting proteins called factors each relay Runner
[01:13]
activates the next one in sequence by passing on a baton factor 8 is the key Runner that passes the Baton between clotting factors 9A and 10 this activates Factor 10 who finishes the race only then can the next event begin the next event in the competition is a test of strength with a protein called fibrin who is a resilient team member like a weightlifter the weightlifter rallies the crowd to act together fibrin acts like a mesh to keep platelets together so that they can form a blood clot to stop bleeding with this
[01:52]
the competition is over team clotting wins what happens in the blood of a person with hemophilia a people with hemophilia a either lack factor 8 or have factor 8 that does not function properly without factor 8 the team's Star Runner the race cannot be finished and the team competition is stopped as there is no fibrin to bring the platelets together a clot cannot form properly and so bleeding continues people with hemophilia a can be treated with replacement Factor h replacement factor 8 is like asking a
[02:33]
different athlete say a high jumper to substitute for the runner so that the team competition can continue meaning the successful formation of a clot the high jumper shares skills with our relay Runner but behaves in a different manner running in short bursts they do the job then leave the stadium as each high jumper finishes another comes on to keep the activity going this is similar to factor 8 replacement therapy that is given at frequent intervals to stop bleeding in some cases Inhibitors may
[03:07]
stop replacement factor 8 from working keeping our high jumpers from competing stay tuned to find out what this means for our team event episode 2 coming soon [Music]