A long life with sickle cell disease

Sickle cell disease is often described as a “life limiting disorder”. Not so long ago children and teenagers were sometimes told that they would be “lucky to see their 21st birthday”. In many parts of the world it remains the case that children die young as a result of sickle cell, but the situation in Europe and America has improved enormously over the last few decades. As an example, in a series of 252 children, followed from birth in East London, childhood mortality was virtually eliminated with 99% of children surviving to reach their teenage years. With good medical care survival into middle age is now the norm, although the cumulative damage caused by sickle cell can become more and more of a burden as the years pass.

Although this improvement in mortality in the developed world is the result of better medical care, a recent publication from the USA makes it clear that there active steps patients themselves can take which will improve their overall life expectancy. The report, in the journal Blood, describes four remarkable patients with sickle cell disease who survived into their 80’s; two with sickle cell anaemia (Hb SS) and two with Hb SC disease. It wasn’t as if these four patients had escaped undamaged by their sickle cell; all four had significant complications including, retinopathy, the acute chest syndrome, avascular necrosis, leg ulcers, gallstones, an enlarged spleen, severe anaemia, heart failure and iron overload. A daunting disease burden for anyone to carry and yet they survived into old age – how was this possible?

Well some of the reasons were just related to the luck of the draw. Two had Hb SC disease and this is known to be a milder condition than Hb SS disease; in addition to which, the two patients with Hb SS disease both had a naturally high Hb F level, which we know would have helped to protect them. As a result, all four individuals reported only infrequent painful crises, less than 3 times a year. Finally, all four were women; from the teenage years onwards men with sickle cell tend to have higher mortality rates than women. This maybe because women with sickle cell generally have a lower haemoglobin level than men and this will tend to reduce the frequency of painful crises. But, we also know, that men in general indulge in more high risk behaviours than women and this may be just as relevant to their chances of survival as their haemoglobin levels.

Type of sickle cell disease; male or female; Hb F level – you cannot really change any of these factors, although you can choose to artificially raise your Hb F level by taking hydroxycarbamide and, if you are a man, you can minimise risk taking behaviour. But the authors did identify other factors in their four patients, which are very much under individual control, and which may be just as important in determining longevity.

Firstly, all four patients maintained a “healthy lifestyle” – they did not smoke or drink alcohol and they were not overweight. Secondly, they were all “good patients” – meaning they took their drugs and other medications regularly and always came to outpatient and clinic appointments. Thirdly, they all had “supportive family networks” – either their husbands, brothers and sisters or children, who were all very active and involved in looking after them. Finally, although not commented on by the authors, all four patients had excellent medical care throughout their lives and this is also likely to be relevant to their longevity.

So, you could be an octogenarian as well, and it’s not high tech rocket science! Think seriously about taking hydroxycarbamide, live a healthy lifestyle, try to do what your doctor or nurse says and cherish your family, you need them. Finally, don’t settle for second best in your medical and nursing care.

Case series of octogenarians with sickle cell disease. Samir K. Ballas, E. Dianne Pulte, Clarisse Lobo and Gaye Riddick-Burden. 

Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Telfer P, Coen P, Chakravorty S, Wilkey O, Evans J, Newell H, Smalling B, Amos R, Stephens A, Rogers D and Kirkham F. Haematologica (2007), volume 92, pages 905-12.

Other relevant blogs

Lifestyle choices and sickle cell – 10/06/14

More on the benefits of exercise – 03/05/15

How the world around you affects the severity of sickle cell disease – 13/10/15

Reassurance about the effects of exercise – 18/12/15

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About rogerjamos

I am a consultant haematologist who has worked in Hackney, London, UK with patients who have sickle cell disease for many years. Knowledge is power; the hope is that this blog will empower patients by putting them in touch with contemporary research into sickle cell disease and facilitating informed discussion on the issues raised. Dr Roger Amos MA, MD, FRCPath
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