Helpful websites on sickle cell disease

A pick of the best websites on sickle cell disease by Dr Keith Barnard

Website of the week

It is strange to recommend a web page on an essentially medical topic that originates from a surgical site, but there you go.

The pathology section is a marvel of economy, listing the background to the disease in nine bullet points, telling you that the amino acid valine is substituted for glutamic acid at position six of the beta chain of haemoglobin and that this results in deoxygenated haemoglobin undergoing polymerisation and the resultant typical sickle cells.

Sickle cell crises and their treatment are covered in a similar way. The problems of anaemia and the acute chest syndrome are also mentioned. It only takes a few minutes to look at this site but it is well worth the effort.

Why go there: An excellent refresher.

Downside: None.

Information from: Surgical-Tutor.

Pulmonary manifestations of sickle cell disease

This paper appeared in the Postgraduate Medical Journal in 2003 and is a comprehensive account of the two main complications that affect the lung in sickle cell disease. These are the acute chest syndrome and chronic sickle cell lung disease.

These conditions account for a large proportion of deaths among adult patients. If you have a patient with the condition, it will probably be useful  to have a look at this article.

Although this is mostly relating to hospital practice, it never does any harm to know about the latest developments in management, especially as a patient with this condition is likely to be knowledgeable about their disease.

The article runs to eight pages of a PDF, is well presented and has all the important information in five of them.

Why go there: Useful if you have a patient with the disease.

Downside: Pretty meaty stuff.

Information from: Albert Einstein College of Medicine, New York.

Preventing infection

Preventing infection is an important issue, especially in children with sickle cell disease where infection can rapidly prove to be fatal. Any child with a high fever and a suspicion of bacterial (usually pneumococcal) infection should have intravenous antibiotics.

This summary of a Cochrane review makes interesting reading, as it tells us that pneumococcal vaccination, although recommended, tends to be of limited value in the young (and most susceptible) age group.

Why go there: Because it is a useful reminder.

Downside: None.

Information from: The Cochrane Library.

See the cells    

The only reason I am suggesting you visit this site is because it contains one of the best images of a sickled cell pictured alongside a normal red blood cell.

There is other useful information about sickle cell disease on this website, but it is worth noting that it is done better elsewhere.

Why go there: For the sickle cell image.

Downside: None.

Information from: The Carnegie Institution.

Dr Barnard is a former GP in Fareham, Hampshire

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