From the Department of Pediatrics, Mahatma Gandhi Institute of Medical Sciences, Sewagram, Wardha 442 102, Maharashtra, India.
Reprint requests: Dr. Pushpa Chaturvedi, Professor and Head, Department of Pediatrics, M.G.I.M.S. Sewagram, Wardha 442 102, M.S, India.

Manuscript received: August 3, 1998; Initial review completed: October 6, 1998; Revision accepted: October 21, 1999.

Objective: To study the epidemiology of sickle cell disease in pediatric age group in a rural hospital of Central India. Design: Prospective descriptive hospital based study. Subjects: 99 admitted patients of sickle cell disease were studied for a period of 1 year. Results: Prevalance of sickle cell disease was 5.7% (99/1753) hospitalizations of which 61.6% (n=61) had homozygous sickle cell disease (HbSS) whereas 38.4% (n=38) had heterozygous state (HbAS). Of these, 62 (63%) were below five years of age. Male : Female ratio was 1.65:1 in HbSS cases and 1.71:1 in HbAS cases. History of consanguinity was present in 7 (7%) of which 5 (8.2%) had HbSS and 2 (5.2%) had HbAS. Incidence was maximum in the Mahar community (70%) followed by Kunbi (8 %) and Teli (6%). Vascular occlusive crisis (23.3%) was the commonest crisis encountered followed by hyperhemolytic crisis (16.3%). There was no correlation between hemoglobin levels and the occurrence of vascular occlusive crisis. Maximum cases required their first blood transfusion between second and third year of age. Requirement of blod transfusion was more in HbSS cases. Four patients died of which three had HbSS and were below five years of age. Splenic sequestration crisis was the commonest cause of death. Conclusion: Sickle cell disease is prevalent in this area and most cases present before 5 year of age. VOC is the commonest crisis seen, but death often occurs due to sequestration crisis and usually below 5 years of age.

Key words: Anemia, Blood transfusion, Sequestration crisis, Sickle cell disease, Vaso-occlusive crisis.

Sickle cell disease (SCD) is an autosomal recessive genetically transmitted hemo-globinopathy responsible for considerable morbidity and mortality. It is prevalent in many parts of India including Central India, where the prevalence in different communities has ranged from 9.4-22.2%(1). We conducted a hospital based investigation to evaluate the epidemio-logy and clinical presentation of this disease in children.

All patients admitted to the pediatric ward of Kasturba Hospital, Mahatma Gandhi Institute of Medical Sciences, Sevagram from August 1995 to July 1996 were screened for SCD by sickling test(2) and hemoglobin (Hb) electrophoresis. Details of patients in whom a diagnosis of SCD was confirmed by Hb electrophoresis were entered in a specially designed proforma and they were subsequently followed up for Hb levels and clinical picture for a period of one year. Hb electrophoresis was done on cellulose acetate membrane(3), to confirm the diagnosis and classify HbSS and HbAS patients. Diagnosis of sickle cell crisis was made on admission and follow-up as per earlier criteria(4).

A total of 1753 admissions were screened of which 99 (5.7%) were diagnosed to have SCD. Of these, 61 (61.6%) had homozygous state (HbSS) whereas 38 (38.4%) had hetero-zygous state (HbAS). SCD was more common in males, the male : female ratio being 1.65 : 1 in HbSS and 1.71: 1 in HbAS (Table I). History of consanguinity was present in 7 (7%) of which 5 (8.2%) had HbSS and 2 (5.2%) had HbAS. The consanguinity was traced to the first generation only.

Table I - Age and Distribution at the Time of Diagnosis

(Cf : Cumulative frequency).

On analyzing the religion, it was seen that 66% were Boudhs and 34% were Hindus. Caste wise the incidence was maximum in Mahar (70%) followed by Kunbi (8%), Teli (6%), Gowari (4%), Gond (3%), Navi (2%), Dhangar (1%) and Pradhan (1%). Some new castes like Sonar (1%) and Kshatriya (1%), were also identified.

Table II shows the frequency of crisis in SCD. Sixty one cases of HbSS were examined 101 times and 38 cases of HbAS were examined 70 times. A total 171 examinations of 99 patients showed that the vascular occlusive crisis (VOC) was the most common (23.3%) followed by hyperhemolytic crisis (16.3%). Table III shows correlation of hemoglobin to VOC in SCD. Cases of HbAS who were designated as VOC had symptoms suggestive of VOC and hence were classified in this group. The mean hemoglobin levels in cases of HbSS having VOC was lower as compared to no VOC. But in HbAS, cases designated as VOC had higher mean hemoglobin as compared to those with no VOC.

Table II - Crisis in Sickle Cell Disease

Table III - Correlation of Hemoglobin and VOC in SCD

* Symptoms were suggestive of VOC

The age of receiving first blood transfusion and frequency of blood transfusions in SCD is shown in Table IV. Thirty six per cent of HbSS and 10.5% of HbAS required first blood transfusion before going to school (<5 years). Amongst them, maximum children received it between 13-36 months. Forty six per cent with HbSS and 84.2% with HbAS did not require blood transfusion during the study period. Out of a total of 50 blood transfusions, 43 were required by HbSS patients.

Table IV - Age of First Blood Transfusion and Frequency of Blood Transfusion

Figures in parentheses indicate percentages.

Four patients died; 3 had HbSS and they were all below 5 years of age. The cause of death was sequestration crises in 2 and severe anemia in one. The fourth death was in a case having HbAS who died at the age of 12 years due to severe anemia. The peripheral smear picture of this patient showed dimorphic anemia with signs of hemolysis.

The prevalence of SCD was 5.7% of which HbSS was 3.51% (61/1753) and that of HbAS was 2.1% (38/1753). Studies from Orissa report the incidence of SCD in hospitalized pediatric patients to be 6.42%(5) (results based on positive sickling test) and 11.1%(6) (results based on hemoglobin electrophoresis). Central Maharashtra is reported to be in the sickle cell belt(7). In Central India, prevalence of sickle cell trait (SCT) has been reported to be 11.1%(1). A community survey of the rural population in this area, based on sickling test, showed a prevalence of 5.5%, of which on electrophoresis, 5.86% had HbSS and 94.1% had HbAS(8). The prevalence of sickling in some states of India is depicted in Table V(9).

Table V -  Prevalence of Sickle Cell in Some States of India

Amongst cases having HbSS, 68.8% were diagnosed before five years of age and were symptomatic. An earlier study from Orissa found 50% patients to be symptomatic before five years of age(7), whereas in a study from Jamaica, 90% were symptomatic by the age of 5 years(10). In the present study 52.6% cases with HbAS presented to the hospital before the age of 5 years.

Males are more prone to be exposed to known precipitating factors as compared to females(4,11). There is also a gender bias in the community as males are given better care and females are neglected. Both these reasons may have contributed to the preponderance of males over females in the present study. SCD being an autosomal recessive genetic disorder, one of the important preventive measures is to avoid inbreeding between patients of SCT as with positive history of consanguinity the risk of homozygous disease increases. However, in our study only 7% of hospitalized cases of SCD had a positive history of consanguinity traced to first generation only.

The maximum prevalence was noted in the Mahar caste followed by Kunbi and Teli. Similar findings have been reported by other workers from Central India(1,12). Most Mahars are Boudhs by religion, this explains our findings of increased cases of SCD among the Boudhs as compared to the Hindus.

Vascular occlusive crisis was the common-est crisis seen by us and others(4,5,6,11). Though we found low mean Hb levels in case of HbSS having VOC, but overall there seemed to be no correlation between hemoglobin and VOC. This is in conformity to observations by earlier workers(11,13).

In our study 26.2% (26/99) required their first blood transfusion before the age of five years. Amongst these 84.6 (22/26) had HbSS and 15.3% (4/26) had HbAS. Maxium cases needed their first blood transfusion between one to three years of age reflecting a high morbidity in the preschool age. At this age, cross infection is more common, which could precipitate a sickle cell crisis. With growth the body func-tions probably get adjusted to low hemoglobin levels. There were 50 blood transfusions given to patients of SCD during the study period. Blood transfusions were required in 54% of cases having HbSS and 17% of those with HbAS. Only one patient having HbAS required multiple transfusions as compared to 8 (13.1%) of HbSS. No patient of HbAS required more than 2 blood transfusions whereas 2 cases of HbSS required 3 transfusions during the study period. Thus anemia in HbAS is mild and infrequent and one must look for other causes of anemia in them. Of the 6 HbAS patients who received blood transfusion, one had iron deficiency anemia, and 3 had dimorphic anemia.

Three of the four deaths in the present study occurred below 5 year of age. A Jamaican study(14) reported greatest number of deaths during the first five years and maximum in the first year. They also reported 9.5% deaths due to sequestration crisis. However, there was no death during the first year in this study but two deaths were seen during the second year. Amongst the six patients who had sequestration crisis, 2 patients expired, thus confirming more deaths during first episode of acute splenic sequestration. Incidence of deaths during the first episode of sequestration crisis have been reported earlier to be 6%(14) and 12%(15,16).

To conclude, SCD is prevalent in this area and out of every 100 admission roughly 5 are likely to have SCD, most presenting before 5 years of age. The most common crisis en-countered is VOC, followed by hyperhemolytic and mixed crisis. The first blood transfusion is more often required between the second and the third years of life. Mortality is highest below the age of 5 years, sequestration crisis being the commonest cause of death.

Contributors: PC coordinated the study particularly its design and interpretation. MK participated in data collection and helped PC in drafting the paper.

Funding: None.
Competing interests: None stated.

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