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      Pathological basis of symptoms and crises in sickle cell disorder: implications for counseling and psychotherapy


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          Sickle Cell Disorder (SCD) is a congenital hemoglobinopathy. There is little in literature regarding the psychological variables affecting individuals living with SCD and all of the significant people around them. There are also limited numbers of trained clinical psychologists and genetic counselors to cater for the psychotherapeutic needs of individuals living with SCD. Even among those who have been trained, only a few might have fully grasped the complexities of the disease pathology.

          Early understanding of its pathological nature, sources, types, complications, pathophysiological basis, and clinical severity of symptoms among clinical psychologists, genetic counselors and psychotherapists, as well as general medical practitioners, could guide them in providing holistic care for dealing with and reducing pain among individuals living with SCD. It could allow risk-based counseling for families and individuals. It could also justify the early use of disease-modifying or curative interventions, such as hydroxyurea (HU), chronic transfusions (CTs), or stem-cell transplantation (SCT) by general medical practitioners. Hence, the need for this paper on the pathophysiology of SCD.

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          Most cited references85

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          Pulmonary hypertension as a risk factor for death in patients with sickle cell disease.

          The prevalence of pulmonary hypertension in adults with sickle cell disease, the mechanism of its development, and its prospective prognostic significance are unknown. We performed Doppler echocardiographic assessments of pulmonary-artery systolic pressure in 195 consecutive patients (82 men and 113 women; mean [+/-SD] age, 36+/-12 years). Pulmonary hypertension was prospectively defined as a tricuspid regurgitant jet velocity of at least 2.5 m per second. Patients were followed for a mean of 18 months, and data were censored at the time of death or loss to follow-up. Doppler-defined pulmonary hypertension occurred in 32 percent of patients. Multiple logistic-regression analysis, with the use of the dichotomous variable of a tricuspid regurgitant jet velocity of less than 2.5 m per second or 2.5 m per second or more, identified a self-reported history of cardiovascular or renal complications, increased systolic blood pressure, high lactate dehydrogenase levels (a marker of hemolysis), high levels of alkaline phosphatase, and low transferrin levels as significant independent correlates of pulmonary hypertension. The fetal hemoglobin level, white-cell count, and platelet count and the use of hydroxyurea therapy were unrelated to pulmonary hypertension. A tricuspid regurgitant jet velocity of at least 2.5 m per second, as compared with a velocity of less than 2.5 m per second, was strongly associated with an increased risk of death (rate ratio, 10.1; 95 percent confidence interval, 2.2 to 47.0; P<0.001) and remained so after adjustment for other possible risk factors in a proportional-hazards regression model. Pulmonary hypertension, diagnosed by Doppler echocardiography, is common in adults with sickle cell disease. It appears to be a complication of chronic hemolysis, is resistant to hydroxyurea therapy, and confers a high risk of death. Therapeutic trials targeting this population of patients are indicated. Copyright 2004 Massachusetts Medical Society
            • Record: found
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            Gene mutations in human haemoglobin: the chemical difference between normal and sickle cell haemoglobin.

            V. Ingram (1957)
              • Record: found
              • Abstract: not found
              • Article: not found

              Sickle cell anemia a molecular disease.


                Author and article information

                Hematol Rep
                Hematology Reports
                PAGEPress Publications (Pavia, Italy )
                13 April 2010
                26 January 2010
                : 2
                : 1
                Behavioural Studies Department, College of Management Sciences, Redeemer's University, Mowe, Ogun State, Nigeria
                Author notes
                Correspondence: Oluwatoyin Olatundun Ilesanmi, Behavioural Studies Department, College of Management Sciences, Redeemer's University, Mowe, Ogun State, Nigeria E-mail: toytundun@ 123456yahoo.com

                Dr Ilesanmi Oluwatoyin Olatundun, Clinical Psychologist, PN & M, Trained Genetic Counselor, is concerned with taking care of people with the sickle cell disorder, to create awareness about the prevention and control of sickle cell disorder, blood group Rhesus factor, malaria control in sickle cell disease, research and raising awareness on HIV/AIDS in Nigeria and beyond, through information, psychoeducation and communication, screening and genetic counseling.

                ©Copyright O.O. Ilesanmi., 2010

                This work is licensed under a Creative Commons Attribution 3.0 License (by-nc 3.0).

                Licensee PAGEPress, Italy


                counseling and psychotherapy.,implications,sickle cell disorder,pathology
                counseling and psychotherapy., implications, sickle cell disorder, pathology


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