Jayanti Basu, Monami Roy Chowdhury, Apurba Kumar Mitra
Hemophilia is a sex chromosome-gene-related bleeding disorder specifically for males in which an essential clotting factor in blood is either partly or completely missing. In case of any internalor external bleeding, injection of blood derivatives or the missing factor is the major treatment. In rural and even some urban areas, the diagnosis of Hemophilia is made quite late, even in late adolescence, either owing to negligence and ignorance of the parents, or worse still, occasionally due to wrong or uninformed medical practice. Even when diagnosis has been made correctly, owing to thehigh price of the processed blood factor, many Hemophilic patients In India are left untreated and suffer damage of joints and muscles. In more extreme cases of CNS bleeding or intracranial hemorrhage (ICH), even death may happen. At the same time, unprocessed whole blood obtained through standard blood donation procedure or other processed blood derivatives like plasma concentrate may be injectedand are supplied at some centers free of cost; but repeated injection of these derivatives exposes the patient to the risk of blood borne infections including HBV, HCV and HIV, or development of inhibitors.
These constraints are likely to give rise to a number of psychosocial problems for Hemophilic children. There is paucity of studies relating to the psychosocial condition of persons withHemophilia, and studies in India are practically negligible. A few studies abroad have theoretically emphasized the role of psychosocial factors and intervention (Casey & Brown, 2003; Jones, 2002), but only a few have actually conducted empirical studies. Two studies in the mid-nineties conducted in Kolkata highlighted the importance of such empirical work (Bhattacharyya et al., 1995; Basu &Bhattacharyya, 1995) by demonstrating differences between the persons with Hemophilia and normal controls in terms of some strategic psychological functions.
Children with Hemophilia are often deprived of normal experiences of childhood owing to their recurrent bleeding episodes, fear of viral infection contraction and related psychological problems. The ICH, though infrequent, might alsoaffect neuropsychological functioning. A number of studies have revealed neuropsychological impairment in persons with Hemophilia (Kulkarni & Lusher, 1999; de Tezanos et al., 1992; Bale et al., 1992; Revel-vilk et al., 2004).
While there is no clear reason for the Hemophilic children being less intelligent than their normal counterparts, some studies reveal that Hemophilics are lower on themeasure of intelligence in comparison to normal (Hooper et al., 1997). This may be accounted for by the risk of ICH or due to the factor that Hemophilics have a tendency to deploy much of their intellectual faculty to attain relative independence from environmental stressors. Other studies do not support the intellectual decline in Hemophilics (Handford et al., 1986; Mayes et al., 1988; Riabov et al.,1989).
In terms of personality, Hemophilic children suffer the possibility of psychopathological development due to repeated hemorrhages. Certain studies have been conducted which support this viewpoint (Gutton, 1976; Clingman et al., 1979). However other studies conducted on Hemophilic persons have shown that they have normal scores on per-sonality, mood and behavior assessment. (Handford etal., 1986; Mayes et al., 1988; Canclini et al., 2003). Literature reveals that in case of chronic diseases social sensitivity and defensive social behavior increase, and the patient may tend toward avoidance of negative self presentation by adhering strictly to normative social behaviour and thus limiting one’s free socialization (Carnrike Jr. et al., 2005). A significant study using a measure...