Bloods

A UK All-Party Parliamentary Group (APPG) on the blood disorders Sickle Cell and Thalassaemia, found evidence of substandard NHS care for Sickle Cell patients, admitted to General hospital wards or Accident and Emergency departments (https://www.theguardian.com/society/2021/nov/15/sickle-cell-disease-inquiry-finds-serious-care-failings-in-nhs). This initially seems remarkable, as Sickle Cell is one of the best known (and taught) examples of an inherited blood disease. The Sickle Cell gene persists in people from an African or Afro-Caribbean background. It involves substitution of a single base in the DNA coding for haemoglobin. Haemoglobin is the respiratory pigment, found in red blood cells, that carries oxygen and carbon dioxide around the body. Heterozygous individuals have one copy of the gene, and it causes them no particular problems. Such people are, however, more resistant to infection by the mosquito-carried malarial parasite than people with 'normal' haemoglobin genes. Malaria kills many people (especially young children) in Africa. This is the reason why the gene has persisted in people with an African background. Heterozygous carriers of the Sickle Cell gene, can pass it on to their offspring. Homozygous individuals, with 2 copies of the Sickle Cell genes (one from each parent) develop, in contrast, the full-blown disease. Such individuals have distorted (sickle-shaped) red blood cells. The shape of these erythrocytes, means they do not efficiently carry oxygen around the body (hence the anaemia). The shape also means the cells do not pass easily through blood vessels. This results in painful episodes, with varying degrees of intensity. People with Sickle Cell disease need regular blood tranfusions, as well as effective pain management. The APPG found both low awareness of and poor training for dealing with Sickle Cell disease in NHS hospitals. People have died. It seems likely that there is a racial aspect to this flaw in treatment.