‘They Can Replace You at Any Time!’: (In)Visible Hyper-Ableism, Employment and Sickle Cell Disorders in England

Splenic function in sickle hemoglobinopathy syndromes was assessed to determine the developmental pattern of splenic dysfunction. Nonvisualization of the spleen using technetium-99 metastable (99mTc) spleen scans correlated strongly with pocked (vesiculated) RBCs ≥3.5%. Cross-sectional analysis of pocked RBC data from 2,086 patients showed differences in the developmental pattern of splenic dysfunction between several disorders. In hemoglobin SS disease (sickle cell anemia) and hemoglobin Sβ° thalassemia, splenic dysfunction (≥3.5% pocked RBCs) often occurred in the first 6 to 12 months of life. In hemoglobin Sβ+ thalassemia, splenic dysfunction occurred less frequently and later. Splenic dysfunction in hemoglobin SC disease (sickle cell-hemoglobin C) was intermediate. The level of pocked RBCs was inversely associated with fetal hemoglobin (P < .007) and directly associated with age (P ≤ .001). These patterns of splenic dysfunction reflect the known severity of hemolysis and intravascular sickling and are consistent with the epidemiology of severe bacterial meningitis and sepsis in these diseases. Serial measurement of pocked RBCs permits determination of the onset of splenic dysfunction and the time of increased susceptibility to severe bacterial infections.

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Comparison of Actual with Estimated Weight and Height in Children with Sickle Cell Anaemia in Lagos, Nigeria

Journal of BioMedical Research and Clinical Practice ◽

10.46912/2i4.2019126 ◽

2019 ◽

Vol 2 (4) ◽

pp. 185-190

Author(s):

SO Akodu ◽

AF Adekanmbi ◽

AT Ogunlesi

Keyword(s):

Sickle Cell ◽

Sickle Cell Anaemia ◽

Genetic Disorders ◽

Actual Length ◽

Mean Values ◽

Standard Methods ◽

Positive Linear Relationship ◽

Measured Weight ◽

Sickle Cell Disorders ◽

Paediatric Practice

Sickle cell disorders are the most common genetic disorders worldwide as well as in Nigeria. The weight and height are the most used anthropometric measurements in paediatric practice for the calculation of many parameters. The objective is to determine the reliability of weight and height estimates among children with sickle cell anaemia compared with measured weight and height. A random sample of SCA children aged eight months to 12 years was studied. Height and weight were measured using standard methods, while BMI was estimated using kg/m2. These were compared with estimated weight, height and BMI. Five subjects were adjudged obese using estimated BMI: three of these five subjects were identified as obese using actual BMI. Regression analysis showed that there appears to be more positive linear relationship for weight and length/height among children aged 1– 6 years. The measured mean weight and BMI were significantly lower than the estimated mean values. The overall mean of estimated length/height did not differ significantly from overall mean of the actual length/height. In comparison with the actual BMI, the estimated BMI did not identify any of the subjects with either thinness or overweight while children with obesity were over-estimated. The formula methods of estimating weights and heights for the various ages in children with sickle cell anaemia are over-estimating their actual values; this is more obvious for weight.

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6 Rheology of the sickle cell disorders

Baillière s Clinical Haematology ◽

10.1016/s0950-3536(87)80023-9 ◽

1987 ◽

Vol 1 (3) ◽

pp. 747-776 ◽

Cited By ~ 34

Author(s):

J. Stuart ◽

C.S. Johnson

Keyword(s):

Sickle Cell ◽

Sickle Cell Disorders

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Erythrocyte-endothelial cell adherence in sickle cell disorders

Blood ◽

10.1182/blood.v68.5.1050.1050 ◽

1986 ◽

Vol 68 (5) ◽

pp. 1050-1054 ◽

Cited By ~ 30

Author(s):

BD Smith ◽

PL La Celle

Keyword(s):

Endothelial Cell ◽

Sickle Cell ◽

Cell Interaction ◽

Cell Contact ◽

Shear Stresses ◽

Cell Monolayers ◽

Sickle Erythrocyte ◽

Low Shear Stress ◽

Sickle Cell Disorders

Abstract Detachment of individual sickle erythrocytes from cultured endothelial cell monolayers has been evaluated by a fluid-shearing technique in an effort to quantitate adherence at shear forces that would be anticipated in the in vivo circulation. Nonirreversibly sickled cells (non-ISC) were more adherent at normal oxygen tensions than control cells. More than 1% non-ISC remained attached to the monolayer at forces greater than physiologic shear stresses in capillary and venous circulations, and many of the most avidly attached cells, once separated, immediately reattached to adjacent endothelial cells. These data suggest that hemoglobin S-containing erythrocytes may have a higher frequency of adherence in vivo in regions of low shear stress where prolonged erythrocyte-endothelial cell contact could occur. Some of these cells detached by shear force would subsequently reattach in in vivo conditions. Plasma-enhanced attachment frequency and plasma from blood in a case of sickle crisis caused further increase. These observations further support the concept that sickle erythrocyte- endothelial cell interaction may be a significant factor in initiation of vascular occlusive events in sickle cell disease.

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Improving Screening Programmes for Sickle Cell Disorders and Other Haemoglobinopathies in Europe: The Role of Patient Organisations

International Journal of Neonatal Screening ◽

10.3390/ijns5010012 ◽

2019 ◽

Vol 5 (1) ◽

pp. 12

Author(s):

John James ◽

Elizabeth Dormandy

Keyword(s):

National Health Service ◽

Sickle Cell ◽

Screening Programme ◽

Added Value ◽

Unique Contribution ◽

Discussion Paper ◽

Patient Organisations ◽

Sickle Cell Disorders ◽

Cell Disorder

This discussion paper has been written to show the unique contribution and added value that Patient Organisations can give to the development and improvement of newborn screening programmes for sickle cell disorder (SCD) and other haemoglobinopathies in Europe. As an example, the action of the Sickle Cell Society (SCS) in partnership with statutory organisations in the U.K., such as the National Health Service (NHS) Sickle Cell and Thalassaemia Screening Programme (NHS SCT SP), will be described.

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