Making choices is part of life – we all make choices every day, sometimes they are good ones, sometimes they are bad ones. Very few of us are free to make unrestricted choices, to some degree or other, all the choices and decisions we make in life are limited in some way, whether by money, time, imagination or relationships. If you have sickle cell disease then that is just another factor which has to be taken into account. This blog is about how sickle cell can affect the choices you make in life and how to work with that.
Sickle cell is an unpredictable disease; it is a matter of common experience that even if you do everything right and follow all the rules, you can still end up in hospital with a painful crisis. That is no ones fault – it is just the way it is. It is not possible to avoid all the problems from sickle cell all the time, even if you make all the right lifestyle choices.
That being said, there are certain factors and circumstances which do make the condition worse and are likely to trigger more problems and more crises. It makes sense to try and avoid these factors as much as possible, to limit the problems that the condition causes. It is important therefore to understand what these factors are and to know how they affect you as an individual, so that you are able to make informed, sensible choices.
On occasion you may decide that you are going to ignore the standard advice and go ahead and do something that you know may put you at increased risk. The important issue here is that you make a decision, understanding the consequences and risks, because this is something that you really want to do. This is all about you controlling and managing the disease and not letting sickle cell always dictate to you what you can and cannot do. But it is important to be aware of the risks and to make intelligent and informed decisions.
For example, maybe you want to go to a festival, it’s an outside performance and it is going to be very hot, meaning that you will probably get dehydrated. It goes on for hours and is bound to be physically exhausting; but its a band you really like and all your friends are going. You go and, sure enough, the next day have a bad crisis in your arm and have to go to the Emergency Department. You understood the risks, went ahead and had a good time, but paid a price. No one should blame you for that.
So, what are these lifestyle choices and how do you go about making sensible decisions one way or another? Well some of them you will have heard about repeatedly when you have gone to the clinic but there some others which may be new to you and perhaps will give you pause for thought. Just in case you don’t want to read through the whole thing here is a list of the topics I will discuss in order:
1. Prevention of Infection 2. Fluid Intake 3. Keeping Warm 4. Education & Employment 5. Healthy Living 6. Holidays 7. Red Flags 8. Stress 9. Psychology 10. Hospitals
1. Prevention of Infection: Infections, whether they are serious bacterial infections or minor viral illnesses, can cause a multitude of problems and it makes sense to maximise your protection against them. This means taking penicillin twice a day and keeping up to date with your vaccinations, including having the ‘flu vaccine every year.
Many people choose not to follow this advice, although it really is no big deal and does not limit your life or what you can do in any way. “I can’t remember to take the tablets every day” is not really an excuse – we all remember to brush our teeth every day after all. Another often stated concern is “if I take penicillin every day the bacteria will get resistant to it”, but there is no hard evidence to support this at all. Occasionally, patients do have a genuine problem with penicillin – they may be allergic for example – but in that case there are alternatives and your doctor will advise you.
The vaccines we use are safe and effective and none of them, including the ‘flu vaccine, make you ill or risk giving you the infection themselves, contrary to some popular misconceptions. Make sure that you ask your doctor when you are next due to have your booster doses so that you can remind clinic staff to make sure they are given. Be informed and in control of the process.
More controversially, it also makes sense to avoid close contact with friends or family when they have an infection. This may have a significant, although short term, impact on your social life and certainly won’t mean that you never pick up random viral infections, but it will reduce the risks.
2. Fluid Intake: If you have sickle cell disease you are very prone to dehydration and, if you become dehydrated, this will almost certainly provoke a crisis. Why is this? Sickle cell damages the kidneys early in life and, as a result, they are unable to vary the concentration of the urine, instead they produce a fixed, large volume of dilute urine every day. This means that if your intake of fluid is low, for whatever reason, or you are losing fluids in other ways – if you are vomiting or have diarrhoea for example – you will become dehydrated very quickly, because your kidneys are unable to conserve fluid by reducing the volume of urine they produce. This is the reason why everyone will tell you to make sure that you drink a large volume of fluid every day – for an adult this amounts to 3.0 litres of water or other similar fluids. Best to avoid sweetened or fizzy drinks – they will only lead to other problems in time like tooth decay.
Consistently drinking 3.0 litres a day is a very difficult goal to achieve. It will involve carrying water with you at all times and drinking regularly throughout the day. It will also mean frequent trips to the toilet. Unlike the steps you can take to prevent infection, this does have the potential to be quite disruptive, but most patients seem to adapt very well to this regime, probably because the penalty of not drinking enough tends to have a bad effect almost straight away.
Another point is that you may need to increase your fluid intake even further if you are losing more fluid that usual. I have already mentioned vomiting and diarrhoea, but this also applies if the weather is very hot or you have a temperature, both of which will result in increased fluid loss through sweat. Alcohol is also notorious for causing dehydration and is best avoided but, if you are going to drink, you must also increase your non-alcoholic fluid intake as well to counteract these dehydrating effects.
3. Keeping Warm: The importance of keeping warm at all times is again something you will be reminded of frequently. If you are cold, and particularly if there is skin cooling – say due to a cool breeze or air conditioning – then you are much more likely to get a crisis. Exactly how this works is not really understood, but probably involves a re-distribution of blood flow in the body. Less blood goes to the bones and bone marrow and, as a result of the slower blood flow, more oxygen is lost from the blood in the tissues and there is an increased chance of sickling.
What does this mean in practice? Well, you have to watch the weather forecast and be prepared to carry extra layers of clothing with you when you go out, so that you can add, or remove, layers if the weather changes. Most patients will keep windows closed and the home well heated at all times. Skiing holidays, mountain climbing or swimming are probably not good ideas, whereas hot baths, saunas and holidays in the sun might be – provided you take extra fluids.
So, making a choice to keep warm all the time is also fairly restrictive and can certainly interfere with your ability to dress the way you want to. However, it is still possible to be fashionable whilst wearing layers of clothing and avoiding unnecessary skin exposure. Keeping warm also has the potential to be very expensive when it comes to heating your home. Sometimes financial help is available in these circumstances and you can ask in the clinic if this is a problem.
Prevention of infection, a high fluid intake and keeping warm at all times are the three lifestyle choices which are most often talked about in terms of minimising the frequency of painful crises and I expect that you will all have heard a lot about them before. Most patients are aware of them and try, more or less, to follow these three basic rules. I will discuss next some less obvious issues which can sometimes have a major effect on people’s health.
4. Education & Employment: For many people with sickle cell getting a decent education and the right qualifications to stand a chance of accessing the job market is very difficult. Time off school when you are ill, missed exams and low expectations on the part of your teachers all play a part in this. Yet, in terms of long term benefits, leaving school or higher education with the right qualifications, is probably one of the most important things of all to try and achieve.
In the current economic climate job opportunities are limited anyway, but if you have sickle cell they are further restricted. It is difficult to work outside – remember keeping warm – or hold down a physically demanding or stressful job and not all employers are sympathetic to young people who have unpredictable bouts of illness and frequent time off work. All of this means that being ahead of the game and getting the right qualifications for the career that you want is vital if you are to succeed. The benefits of employment in later life are significant, including financial independence, a daily focus and a feeling that you are playing a part in society. It is therefore very important to pursue your education and not to be put off by illness or failing exams. Keep your ultimate goal in mind and play the long game.
5. Healthy Living: By which I mean diet and exercise, both of which can be problematic if you have sickle cell disease.
To start with exercise – regular exercise has all sorts of benefits, including making you feel good about yourself, as well as improving cardiovascular fitness and reducing the risks of all sorts of diseases like cancer and dementia. It is sometimes very difficult to exercise if you have sickle cell, and you may be put off by well meaning health professionals, but it is very important that you find a way to exercise that suits you. Choosing to build a regular exercise regime into your daily life will bring many benefits.
Aerobic exercise, such as running, cross training, dancing, or anything that gets you out of breath, can be difficult because of the chronic anaemia, which is a consistent feature of sickle cell disease, and which limits exercise capacity. Exercise may also provoke a crisis either directly, secondary to the physical exertion itself, or indirectly due to dehydration or getting cold afterwards. But it is possible to exercise regularly, within the limits set by your sickle cell, and everyone should explore what is the best form of aerobic exercise most suited to them. All that is required, to derive maximum health benefits, is about 45 minutes of moderate exercise, three to four times a week, together with walking as much as possible and using the stairs at every opportunity. Clearly, you need to listen to your body; only build up exercise intensity slowly, stop if you get very short of breath or develop any pains, particularly chest pains, and take some other sensible precautions – don’t get cold, wear enough layers of clothing, especially after exercise, and replace all the fluid lost by increasing your fluid intake.
Unfortunately, fitness levels are very quickly affected by periods of ill health and it is sometimes a struggle to recover fitness after a bad crisis or hospital admission. Hospitals should really have physiotherapy programmes to put right the physical de-conditioning induced by a hospital admission, but most don’t and its ends up down to you. When you are better get back to your regular exercise programme as soon as possible, but remember that you will have to start low and gradually increase the intensity of the exercise as your body allows. Always be guided by what your body is telling you.
What about other forms of exercise are available? Training with weights is often easier than aerobic exercise and can have a very positive effect on body image. Swimming is problematic though, very few people with sickle cell can swim without getting a crisis – the water, even if heated, tends to be cold and it is difficult when you get out of the pool not to get chilled. So, for most patients swimming is not an option. Yoga, pilates and stretching are excellent ways of keeping flexible and managing stiff and painful joints and should be used more often. You should feel free to explore these, and other options, to find the right combination for you.
Body image issues are common in all young people and are sometimes made worse by the presence of a chronic illness like sickle cell. Many patients complain that they are underweight and have enormous difficulty in gaining any weight at all. This is partly because weight loss, due to poor food intake, is inevitable during a period of illness. But also because large amounts your daily calorie intake goes straight to maintaining your blood and haemoglobin levels if you have sickle cell, meaning that there are fewer calories available to restore or increase body weight. So, how can you manage your diet to try and improve this?
This is a difficult problem but there are three cardinal rules if you want to maximise your body weight. Firstly, you must eat three regular meals every day to stand any chance of taking in enough calories to achieve weight gain. Skipping breakfast or lunch is not an option. Secondly, each meal must be high in calorie value and should always include cereals, rice, pasta, potatoes or bread, as well as protein and fat. And finally, keep the diet healthy, avoid junk food and sweetened fizzy drinks and make sure that you have at least five portions of fruit and vegetables every day. Even if you do all this weight gain may be difficult and many of you will be prescribed high calorie fluid supplements by your doctor. It is important to remember that these supplements are in addition to a high calorie diet and will achieve nothing on their own without following the three rules.
6. Holidays: Holidays are always good for us – something different and a break away from the normal routine is always restorative. But if you have sickle cell you need choose your holiday wisely and plan carefully, holidays are often complicated by more crises and worse health. Why is this?
There are many possible reasons. It may be due to the stress associated with going away, worries about catching the plane or finding the hotel. We all go a bit crazy when we escape everyday life and attempt to do things we would never dream of doing at home, or maybe we just try and cram in as many exciting events as possible. If you have sickle cell your body will react badly to this change in the normal tempo of life. Maybe it’s hot and sunny and you do not replace increased fluid losses or maybe you pick up some local infection which makes you ill. Whatever the reason, there are many things you can choose to do which will limit the risks and allow you to enjoy your holiday.
Firstly, there are certain holidays which carry much more risk – skiing, mountain walking or climbing or travelling to see The Northern Lights are probably not sensible – too cold. Choose your holiday carefully. Secondly, always take out holiday health insurance. This may be difficult to locate and the premiums maybe expensive but it means that if you do get ill you will not be left with an enormous bill at the end of the day. Always have a pre-travel health check, either at your clinic or with your GP. Discuss where you are going and what you plan to do. Your doctor will give you all the necessary vaccinations if you are travelling abroad, and you must make sure that you get from him or her enough of all your normal tablets and drugs to keep you going whilst you are away, including all your normal pain killers, folic acid and penicillin. Ask your doctor for a covering letter detailing your diagnosis, and any particular health issues, together with a list of all the medications you use. This will avoid issues at security, if your luggage is searched, and will be helpful information for any doctor that you have to see whilst you are away.
Don’t forget malaria! If you are holidaying in a malarial part of the world you will need to avoid mosquito bites (insect repellant sprays, long trousers and long shirt sleeves in the evening and mosquito nets over the doors, windows and bed) and take anti-malarial tablets. Your doctor or a travel clinic will advise which are the best tablets depending on where you are travelling. Anti-malaria precautions are exactly the same for anyone travelling to a malarial region but they are even more important if you have sickle cell. Everyone knows that if you are a carrier for sickle cell you have a minor degree of protection against malaria, although even individuals with sickle cell trait can still get very ill and die from malaria. But if you have sickle cell disease you are very vulnerable; an attack of malaria will almost certainly cause life threatening anaemia and provoke a major crisis. Living in a malarial region does give some immunity, but this immunity is lost very quickly after you leave and is no protection if you return as a visitor. Never take risks with malaria – get protected.
Taking all these precautions before you travel will go a long way to keep you well when you are on holiday. Here are some other tips for the journey and your time away.
(i) If you are flying, the air conditioning on planes can be powerful and the atmosphere can be dry and dehydrating. Take extra layers of clothing to keep warm on the plane and drink extra fluid on the journey. You will need to purchase additional fluid in the Departure Lounge. Airplane cabins are pressurised with a lower oxygen concentration than is normally present at ground level. This is not usually an issue and you will not require additional oxygen unless you have a sickle cell related lung condition, such as pulmonary hypertension. If your doctor is concerned at the pre-travel health check you can have a flight simulation test of your lung function to make sure that it is adequate to cope with the lower oxygen levels on the flight. Finally, don’t forget to take some painkilling tablets in your hand luggage just in case a crisis develops during the flight.
(ii) Once you arrive take time to acclimatise – take things easy for a few days, relax and enjoy the new surroundings. A crisis often develops in the early stages of a holiday because people try and do too much too soon and get physically exhausted.
(iii) Keep up your fluid intake and increase how much you drink each day if it is hot.
(iv) Gastroenteritis – diarrhoea and vomiting – is very common in travellers abroad, but must be avoided if you have sickle cell as it will inevitably dehydrate you and provoke a major attack. Maintain personnel hygiene by washing your hands frequently and, if there are any concerns with the water supply, only drink bottled water, wash all fruit and salad vegetables in bottled water and also use the bottled water to brush your teeth. These precautions will go a long way to protect you against catching gastroenteritis.
7. Red Flags: This is all about knowing when you need to seek help. The most successful patients with sickle cell understand their disease and know when to ask for help. Most of their problems they have learnt to manage themselves, but they recognise warning signs, or “red flags” when things are not going well and which prompt them to present to their doctor early. It is very important that you learn to recognise your own “red flags” and act quickly if they are present. What are these “red flags”?
For many people the most important one is “this crisis does not feel like the usual crisis”. In other words you feel different or the crisis feels different in some way. To recognise this “red flag” means that you have to be fairly introspective and be able to compare how you feel at the moment with other times when you have been ill. For example, many people manage attacks of pain in their arms or legs without too much difficulty, but find pain in the chest or lower back much more difficult to manage with standard pain killers. Or it may be, that for you, if the pain increases in intensity very quickly that is a sign of a crisis which is not going to respond to the medication you have at home. So, the site of pain or the speed with which the pain increases maybe the key feature identifying this crisis as one where you will need help. Try asking yourself “what features of a crisis are particularly worrying for me?” and make a mental note of them for the future.
But there are also “red flags” which are not connected to pain. Being unusually short of breath at rest or on exercise for example, feeling weak or dizzy, having an unusual headache or a fever should all prompt you to seek help quickly.
Most patients with sickle cell disease want to be able to stand on their own two feet and be as independent as possible and that is to be commended and supported at all times. But, the nature of the illness, means that ideal is not always possible and the trick is to learn when it is necessary to seek help. The ideas above may help you decide, but this is something which comes mainly from experience, thinking about what decisions you have made when you have been ill in the past and being willing to learn from previous mistakes or misjudgements.
8. Stress: Stress, or getting upset, is a fact of life. Whether the stress is physical – running for a bus, coping with the underground or looking after small children, or psychological – relationship difficulties, exams or worries about money – it is universal and impossible to avoid. Nevertheless, every patient with sickle cell will tell you that any sort of stress will increase the risks of a crisis, it is as if the stress zooms in unerringly on your own personnel vulnerability. Many patients use avoidance strategies, going out of their way to limit their exposure to stressful situations, others use alcohol or drugs, prescribed or recreational, as a coping mechanism, but, these are restrictive and self-destructive choices and ultimately are not successful. There has to be a better way.
Well there is another way to look at this. It is not so much the stressful situation which is damaging, but rather the individual’s response to it. We all know cool, laid back types, who remain completely unruffled, despite being in the middle of some stressful event. The trick is to develop your own internal strategies, which allow you to stay calm and focused, whatever the situation, keeping events in context and preventing them from threatening to overwhelm you. This all sounds a bit “new age” but this mind over matter approach can be very helpful in sickle cell as a way of managing the everyday stresses of life. Different people have different ways of approaching this. For some, religion offers a framework to keep life’s upsets and traumas in perspective but there are other, simpler techniques such as deep breathing, yoga or meditation. The important thing is to avoid the urge to act self-destructively. Why not discuss these issues with a psychologist?
9. Psychology: That brings us to psychology. Much has been written about the psychological aspects of sickle cell and pain management. I do not want to repeat all of that, but would like instead to approach the issue in a slightly different way, by describing for you two opposite ways of “coping” and suggesting that the best way forward is to find your own middle way between these two extremes. Of course, these two extremes, I am about to describe, are exaggerated caricatures and are certainly not only confined to those with sickle cell disease, but can be found in all walks of life. All of us will recognise aspects of ourselves in these descriptions. Nevertheless, they do illustrate the dangerous ways of thinking and behaving which it is very easy for any of us to succumb to.
Firstly, there is the accepting patient. They feel powerless in the face of their disease and placidly accept that it will dominate them and all they ever try and do. They lead restricted and limited lives, fearful of provoking their disease, always deciding not to do something, just in case it triggers a crisis. Each crisis when it occurs, as it inevitably does, is seen as “another nail in the coffin”. They tend to be depressed and socially isolated with few friends and limited support networks. They have poorly developed coping strategies and may come to depend excessively on the support of their local hospital, not feeling able to cope with any aspect of their disease on their own. Everything that happens to them they see through the prism of their illness, which pervades every aspect of their life. They are their illness and their illness is them.
In contrast, is the rebellious patient. They ignore their illness and, in fact, a part of them has never really accepted that they are ill at all. They spurn all preventative measures and take no responsibility for making sure they have adequate supplies of medicines. They make no compromises with their illness and will, seemingly deliberately, indulge in all sorts of risk taking behaviour, which inevitably results in crisis after crisis. When they are ill they delay seeking help until they are very unwell, and then often leave hospital before they are properly better. They tend to be angry, turning their fury with their disease and their situation outwards against the world. Relationships with hospital staff are fraught and difficult. They exhaust themselves in a constant battle against sickle cell, which is perceived as an external enemy to be ignored, belittled or fought against.
Clearly both of these are pretty poor ways of coping with an illness like sickle cell, although many patients, from time to time, will experience their disease in one or other of these ways. Both extremes result in poorly controlled disease, with multiple hospital admissions, and both put the individual’s life and well being at risk. Is it possible to chart a middle way through this minefield?
Well, I think it is possible, and the most important place to start is with an acceptance that sickle cell is part of you, it is imprinted in your genes, part of your DNA. Not the whole of you for sure, and no one should define themselves by their sickle cell disease alone – that is why the term “sickler” is so offensive – you are person with sickle cell, who has many other interesting and varied aspects to their character. Nevertheless, sickle cell is an important part of you and has to be part of the equation whenever you are making choices between different courses of action in life.
Being seen as responsible for your own health is a sure sign that you have managed that acceptance step. Taking your medication regularly, making sure you don’t run out of tablets, using pain killers sensibly, coming to your clinic appointments and seeking help when necessary are all key indicators. They all reveal someone constructively engaged in their own health care.
The next most important thing is knowledge and understanding. It is critical to know what sickle cell is and how it affects you. This can come partly from reading material like this blog and partly from talking to friends, family and clinic staff but also, and just as importantly, from listening to and thinking about what your own body is telling you. No two people are the same and sickle cell is a famously variable condition. You need to understand your own particular reactions to and interactions with your illness.
Once you have come to accept sickle cell as an integral part of yourself and learnt how it affects you, then you can begin to actively manage the situation, making informed and intelligent choices to enable you to do the things you want to do in your life. Of course, there will be things which are “out of bounds”, there is no doubt that sickle cell does impose limits on what is possible and what is just too difficult or too dangerous, but those limits will not be the same for everyone and they are up for you to negotiate. Inevitably, you will not always make the “right” choices, but then it is important to learn from the mistakes, taking the lessons on board so that they can inform future decisions.
10. Hospitals: Contact with hospitals and health professionals – appointments, admissions, blood tests, scans and x-rays – is an inevitable part of having sickle cell disease, but is often fraught and difficult and characterised by mis-understandings on all sides. The reasons for this are complex and probably best left for discussion on another occasion. But again, I think there is a choice to be made, a middle way between the quite, passive “accepting patient” and the angry, aggressive “rebellious patient”. Charting the right course is vital to getting the right hospital care for you.
The place to start is that you are the expert in the management of your sickle cell and you should develop coping strategies of your own for dealing with most of the issues the disease throws at you. But, it is equally important that you know when to seek help and, in those circumstances, are a willing and active partner in the “getting better” process.
You need to develop constructive ways of working with the doctors, nurses and other health professionals that you will come across throughout your life. Many of them will know little about sickle cell, or may think about it in negative and unhelpful ways. For your sake and for the sake of others, you should be prepared to tackle this and teach them about the condition, what it means to you and what you need from them to get better. The most successful relationships are made when the interaction between patient and health professional is a two way process and where each party learns from the other. Unfortunately, this is usually an ideal to strive for rather than a present day reality.
Always beware however, that hospitals are dangerous places and should be the fall back option and not the main source of care and support. Institutionalisation – meaning an exaggerated and unnecessary dependency on an institution, like a hospital – is an ever present danger. It is an easy option for anyone with a chronic illness to fall into, but it strips away your independence and makes any possibility of choice a non-starter. Learning to manage the relationship with your hospital successfully is one of the main ways of maximising your independence and your ability to make your own lifestyle choices.