The Sickle Cell Chronicles Part 2

Trying to keep fit with sickle cell is like rowing a one man boat that’s full of lots of small holes that you can’t fix.

People see your boat moving smoothly through the water, but what they can’t see is all the extra work you’re doing to constantly empty water out of the boat to stop yourself from sinking.

Eventually when you do sink people are surprised as you appeared to be rowing quite well with no apparent problems, you might even have been further ahead than the average boat.

Exercise is one of the most important factors in determining the quality of your life. But when you have sickle cell anaemia, exercise is one of the commonest reasons for hospitalization due to sickle cell crisis.

Therefore we have a bit of a dilemma, either:

1. Give up exercise and sink into cardiovascular disease, muscle atrophy and depression

2. Risk triggering the pain of the sickle cell crisis.

Before I discuss what the science makes of this dilemma, I want to share more of my story with you.

Learning the hard way

I’ve always enjoyed playing sport and being physically active since I was a kid. Basketball became my main sport when I was about 9. I learnt that by practicing regularly I could get better and more skilful.

My intuition told me that if I continued practicing and improving I would eventually be playing at the highest level in the world.

As I progressed to national levels of competition at around 14-15 years old I just couldn’t keep up with the exercise intensity level required.

All I wanted was to be able to play basketball without feeling exhausted. I knew I had the same level of skill as anyone else but I didn’t have the exercise capacity to show it.

The Oxygen Debt

You know when you see Olympic sprinters giving an interview at the end of a race and they're out of breath and can barely speak.

Thats what I felt like constantly when I played basketball. People used to ask me where my inhaler was assuming I had asthma. If only it was that simple.

No, when your muscles don't receive enough oxygen to generate energy during exercise (aerobic respiration), they have to temporarily function without oxygen (anaerobic respiration).

The result of this is that when you stop exercising if you haven’t already collapsed, your body is in an oxygen debt. You have to breath hard to recover the debt. My problem is that my oxygen debt takes forever to recover from compared to normal people hence the concern about locating an inhaler.

I worked so hard to maintain my fitness, I remember training with my Dad three times a week in the park. Rain or shine I did my routine and gradually built myself up over time.

I remember the first time I experienced the sensation of running unencumbered by fatigue and being able to breath freely. Playing my best and being able to keep up with my team mates was an incredible feeling for me. I felt free, there was no fear or dread after the water break was over.

I felt like I was on the path to success, I kept pushing myself in gradual increments and it was working until I got injured and had to pull out of attending England trials.

Osgood Schlatters Disease prevented me from playing for almost a year. If you know you know. Its basically a knee problem that very active growing teenagers get.

When I returned to basketball after my year out I knew I had a mountain to climb. This is when sickle cell really powered up and hurled me back to the bottom of the mountain each time I ascended.

Trying to get fit quick resulted in sickle cell crisis time after time. Between crises it would take me 2-3 weeks to recover and start over. If it wasn’t a crisis it was another injury that kept me off for a few weeks and during those weeks all the gains I made evaporated.

This was when I realised that I would never be able to remain competitive. Any sense of enjoyment was long gone. I made the logical decision that it wasn’t worth continuing because I knew I was never going to get to a professional level.

I still played for fun and I was able to enjoy playing again at university for a few years until I started to have the same problems. Once I started my clinical medical years I just didn’t have the time or will to keep grinding fitness.

I’d often think about whether I made the right choice when I was 15-16 years old.

There came a moment of realisation during a conversation with my specialist Sickle Cell nurse. She was looking through my blood results and expressed concern that my Haemoglobin (Hb) was getting abit high. I’d always thought having a high Hb was a sign of fitness and prided myself on it being in the lower range of normal. However in sickle cell a higher Hb level increases the likelihood of crisis due to the increased concentration of cells causing more traffic.

It was then I knew that I’d made the right decision. It made sense as my crises frequently occurred when I was exercising at the peak of my fitness.

The fitter I got, the more likely I was to have a crisis if I pushed too far.

What does the Science say?

Well not that much. There really isn’t that much research on cardiovascular exercise in sickle cell.

The primary concern is that the increased demand for oxygen by the muscles in exercise results in red blood cells containing sickle haemoglobin releasing so much oxygen to the muscles that they become sickled and cause a crisis.

Historically there seems to have been a general consensus that people with sickle cell anaemia should just not exercise at all to avoid crisis.

Understandable and explains the lack of research, but what about all the benefits of exercise?

Its only quite recently in 2018 that a review by Robert I. Liem started to examine evidence for the risks versus the benefits of exercise in sickle cell.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245992/

They concluded more research was needed but the main recomendations for exercising were to:

• Avoid dehydration

• Avoid extreme temperatures during exercise

• Avoid prolonged physical exertion at moderate to high intensity without adequate rest periods.

Then in 2019 the American Society for Haematology published a study which showed improvements in blood vessels and physical ability after sickle cell patients completed an 8 week exercise program.

Lead study author Laurent Messonnier, Ph.D., of the Université Savoie Mont Blanc in France said:

So now we can start to conclude that with high intensity exercise the muscles greedily consume all the oxygen that the blood is able to supply too quickly which lowers the oxygen in the blood and results in sickling.

However with light/moderate exercise the muscles use oxygen at a slower rate so its generally safer from the point of view of avoiding a crisis.

So light/moderate exercise is recommended to increase blood supply and vessels in the muscles. The key seems to be to build up slowly and never overdo it.

Hindsight is 20 20

If only I’d known the exercise physiology of sickle cell aneamia when I was 14. I like to think I would have been better off, but at the end of the day that story arc is part of my character. Its how I learned these important life lessons:

1. Life is not fair and you can’t always get what you want. But that doesn’t mean that you can’t achieve happiness. It just means that you have to look deeper to find the resources within yourself that will help you achieve happiness in a different way.

2. Know when to let go of something that is doing you more harm than good.

How I stay in shape now

I’ve tried multiple exercise programs, groups, classes and all the get guaranteed results in x amount of days, weeks, months you can think of.

The most important thing I’ve learnt…

When designed by someone who doesn't understand sickle cell anaemia it will not work for me or I’ll just end up having a crisis.

Therefore I’ve designed my own routine that keeps me fit and healthy without any crises and is sustainable long term.

1. Yoga

2. Calisthenics

3. Walking

As soon as I wake up every morning:

• I drink a pint of water

• 25 mins of yin yoga

• 1x set of max rep push ups

• 1x set of body weight squats (Rep max capped at double the amount of push ups I can do)

• 1x set of max rep pull ups

Daily 40-60 minute walk in the park

3x weekly Yoga Vinyasa flow practice 60 minutes

Recently I’ve started working in a once weekly Bouldering session for 60 minutes to substitute for one of the Yoga sessions.

I’m not recommending anyone with sickle cell should do this exact routine. This is just an example and is currently what my body is able to handle in terms of light/moderate intensity exercise that I’ve built up over the past 4 years.

By far the most important aspect of what I do is the consistent daily morning workout. It has to be daily otherwise it won’t work. I try and get as many consecutive days in as I can because on any given day if I’m aware of a potential crisis trigger I will have to skip.

Crisis Triggers:

• Dehydration

• Sleep deprivation

• Infection, simple cold, tummy bug etc

The key is mindset and low expectations. In fact the lower initial expectations the better. This is why it helps to workout alone as you’re not influenced by anyone else’s ability or gains. I focus on my own exercise capacity and only step up very cautiously once I’m comfortable.

For me personally running and cardio have never been kind to me, its too easy for me to keep going at moderate to high intensity. I’m just too used to the burning sensation in my lungs and muscles to know when I’ve reached my limit.

Resistance training with weights is actually much better. You develop muscles and increase blood flow and also get some cardiovascular benefit. Its much harder to push too far with weights as once your at your limit your muscles can’t lift the weight and you have to stop and fully recover before you can continue.

This is why HIIT (High Intensity Interval Training) is probably the worst type of exercise you can do. If you are already very fit then you might get away with it, but doing this as an unconditioned beginner is very risky. Playing basketball falls in the same risk zone as HIIT for me.

Every now and again I’m invited to play basketball and I’m confronted with the decision of whether or not its worth playing a simple game. Even with everything I know now I still feel like I’m letting people down by saying no.

I feel like people tend to remember me based on when I was grinding fitness regularly enough to play. I also remember and feel encouraged by the nostalgia to play. But then there’s this cognitive dissonance because I know the risk of a crisis is too high.

In the past I would play and just stop when I got tired, but that sucked worse than not playing at all. Even if I didn’t have a crisis afterwards the thought of it would stress me out to no end.

My plan now is to keep my daily and weekly work outs going consistently so that one day I will feel fit enough to play a game of basketball again.

I’m not putting any pressure on myself and not being influenced by anyone else as to when that day will be.

Conclusion

You only have one life and you’ve got to find the balance that allows you to make the most out of it.

Exercise has many physical and mental health benefits which are just as important if not more so in sickle cell.

There needs to be more awareness about sickle cell and exercise. People need to be aware:

1. The importance of actually exercising with sickle cell

2. The safe way to exercise with sickle cell.

Its not a one size fits all when it comes to exercise and keeping fit. Special consideration needs to be taken when creating personal sickle cell exercise programs.

More research is needed on the optimal conditions for exercise and more specific evidence based guidance on maximum limits/parameters that people with sickle cell should avoid going over.

Right now people like me are having to figure it out themselves in an unsupervised way through trial and error which can result in unnecessary pain and suffering.

And finally, I’ll never be and never would have been an elite athlete.

I’m cool with that.

Now I have the mental space to focus on what I can be.

That’s all for now

Lewis

PS. The Sickle Cell Chronicles will be continued…