Prevalence, Geographical Distribution, Social Problems and Treatment of Thalassemia Patients.

A total of 700 beta thalassemia patients were gathered at the outdoor of the Hematology department of Medical College, Kolkata, attending during the period from January 2010 to December 2010. The data is generated by the interaction between them and their family members in presence of the physicians. Actual ethnic identities, place of residence, parity and sibship together with social stigma, if any, faced by the patient or by the member of the family and details of their treatment including transfusion history were noted down and reconfirmed from past records. The prevalence of thalassemia was found 70% among Hindus of which the 62.26% was contributed by castes like Maishya, Namasudra, Barga Kshatriya and Panda Kshatriya. The relative prevalence of subtypes of b-thalassemia was found to be 65% E-b thalassemia, 30% Homozygous b-thalassemia and 5% S-b thalassemia. The aboard of the 85.28% patients were found to be localised along banks river Hooghly and Ganga delta region and we can thus consider tentatively that this belt is a thalassemia zone in West Bengal. The maximum number of cases showed parity to be 1 and there was considerable amount of reproductive wastage. Regarding social stigma, 82.71% responded with no stigma. Early diagnosis and early onset of transfusion with chelation therapy was found to provide benefit for the patient reducing the total amount of transfusion needed per year and also restoring the quality of their life.

Psychological problems and quality of life in children with thalassemia.

OBJECTIVE: The study is aimed to assess psychological problems and quality of life (QOL) in children with thalassemia. METHODS: Thirty-nine children (8-16 yr) with transfusion dependent thalassemia attending day care services for blood transfusion were assessed for psychological problems using the Childhood Psychopathology Measurement Schedule and QOL was assessed using the EQ-5D. RESULTS: Forty-four percent of the children had psychological problems and 74% had a poor QOL. Anxiety-related symptoms (67%), emotional problems, particularly depression (62%) and conduct problems (49%) were the main findings. The children were most likely to report impaired QOL due to severe difficulties in pain/discomfort (64%) dimension, followed by depression and mobility problems of equal severity (33%). The side effects of chelation were an independent predictor of psychological problems and impaired QOL. Also psychological problems were a significant predictor of impaired QOL. CONCLUSION: The recognition and management of the psychological problems that accompany chronic physical illnesses including thalassemia would optimize treatment outcomes and QOL.

Psychopathology and coping in parents of chronically ill children.

OBJECTIVE: To evaluate psychopathology and coping mechanisms in parents of children with a chronic illness. METHODS: 30 parents whose children had Thalassaemia were randomly selected from the Thalassaemia Day Care Centre of a teaching general hospital. The parents were interviewed on a semi structured proforma and also rated on SCL-90-R and Mechanisms of Coping scales. The data was analyzed using Pearson's correlation coefficient. RESULTS: The parents were seen to have high psychopathology on SCL-90-R. Depression was the subscale with the maximum elevation. Somatisation was also significantly elevated The GSI (General Symptomatic Index), a measure of general distress was extremely high in about 86.66% of the parents. Most parents were fatalistic in their approach towards the illness. Fatalism positively correlated with somatisation and depression. Avoidant coping strategies were associated with higher scores on various dimensions of the SCL-90-R. CONCLUSION: Chronic illness in children affects the psychological health of the parents. Active coping strategies are associated with fewer distress indices and thus if inculcated may improve the ability to bear the burden of the illness without becoming themselves affected by psychiatric illnesses.

Psychopathology and self-esteem in chronic illness.

OBJECTIVE: To evaluate psychopathology and self-esteem in chronic illness. METHODS: 60 children and their parents were selected to participate in an open study. 30 children had epilepsy and the other 30 had thalassemia. Both the groups consisted of children randomly selected from the Epilepsy Clinic and Thalassemia Centre respectively, of a teaching general hospital. The children and their parents were interviewed and also rated on Childhood Psychopathology Measurement Schedule (CPMS) and Rosenberg's self esteem scale. The data was analysed using Pearson's chi square test and Pearson's correlation coefficient. RESULTS: The children were seen to have high psychopathology on CPMS (average score: thalassemia group = 28.56, epilepsy group = 26.06). Depression was the subscale with the maximum elevation in both groups. Behavior problems were high in epilepsy. In addition, sadness and disinterest in life were common symptoms in thalassemia while irritability and panic were high in epilepsy. Children with epilepsy perceived a change in lifestyle after diagnosis. Self-esteem was moderately affected in both groups and this affected compliance with treatment in thalassemia. CONCLUSION: Chronic illness affects psychological health and self esteem in children. Hence, in addition to the physical aspects it is necessary also, to focus on the psychological health of the child in order to ensure compliance and thus treat the child comprehensively.

Psycho-social aspects of HIV infection and AIDS in multiple transfused thalassemic children.

Two hundred and three multi-transfused children with thalassemia attending the thalassemia clinic of the Charak Palika Hospital, New Delhi were screened for human immuno-deficiency virus (HIV) antibodies by ELISA test. All positive cases were confirmed by the Western blot test. Of the 203 children screened, 17 (8.37%) were HIV seropositive and the other 3 children referred to the AIIMS were from neighbouring states. These children were compared with 20 age matched HIV negative thalassemics as controls. Of the 20 HIV positive children, four were diagnosed to have clinical AIDS according to the WHO criteria. The clinical features were similar to those described in pediatric AIDS from other parts of the world. This paper describes the clinical features, behavioural problems, cognitive functions and developmental milestones of the children with AIDS.