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Indian Journal of Community Medicine

Sickle Cell Anameia in Tribal Children of Gajpati District in South Orissa

Author(s): T. Sahu, N.C, Sahani, S. Das, S.K. Sahu

Vol. 28, No. 4 (2003-10 - 2003-12)

Deptt. of Community Medicine,
M.K.C.G. Medical College, Berhampur, Orissa

Abstract:

Research questions: What is the extent of the health problems due to sickle cell anaemia in the children of South Orissa? Objectives: 1. To know the frequency and distribution of Sickle Cell Anaemia in 0-15 years children of tribal area. 2. To know the association of different morbidities with Sickle Cell disorders. 3. To suggest measures to promote healthy living in tribal children. Study design: Cross-sectional study.

Setting: Tribal area of Gajapati district in South Orissa.

Participants: Children below 15 years of age.

Study variables: Age, sex, sickling test, differential count, haemoglobin level, splenomegaly, morbidities.

Statistical analysis: Simple proportions and chi-square test.

Results: A cross sectional study on 1704 children in three tribal blocks revealed that 16.55% of children were sickling positive on screening with highest frequency in 5-9 years age group (60.64%) and found in various sub-castes of tribal communities. Anaemia (96.5%) and splenomegaly (30.1%) were constant features in these children. ARI (20.8%), fever (7.3%), pain in abdomen (4.7%) and musculoskeletal pain (0.6%) were the predominant features besides anaemia and splenomegaly. Screening for sickling in this area is suggested for early diagnosis and to promote preventive measures so as to decrease childhood morbidity and mortality.

Key Words: Sickling, Anaemia, Splenomegaly, Haemoglobinopathies, Childhood morbidities

Introduction:

Sickle Cell disease is an inherited disorder of haemoglobin synthesis. This is due to replacement of Valine for Glutamic acid in position 6 of the beta-globin chain of haemoglobin. This genetic alteration yields an unstable RBC with a shortened survival of 10-20 days instead of the normal of 120 days. In situations of stress, the red cells become sickle shaped and get lysed.

India caters to nearly 20 million people with sickle cell disease 1 . The sickle cell gene in India was first described among tribal groups in South India 2 , which spread the wrong message that the disease is confined to tribal people. But the recent data unfolds that the disease is not restricted only to tribal belt but is widely prevalent and has penetrated different castes and communities in our country 3 . It is widely recognized especially in the central parts of India4. The highest frequency of sickle cell gene in India is reported in Orissa followed by Assam, MP, UP, Tamilnadu and Gujarat5 . The average frequency of sickle cell disease in India is 4.3% and that of Orissa is as high as 9.1%6 . Children comprised 52% of sickle cell patients and the three predominant forms of the disease (SS, SB and SD) are clinically and haematologically indistinguishable 7 .

The general impression in Orissa is that, this disease is one of the major health problems only in western parts of Orissa. But cases from different parts of south Orissa reporting to the department of Paediatrics, MKCG Medical College & Hospital, were found to be positive for sickling on investigation. Therefore a community based screening was taken up to assess the problem in this regard within its limited scope and resources.

Material and Methods:

During 2001-2002, health check up camps for children were organized by a voluntary organization in coordination with the Community Medicine department of MKCG Medical College, Berhampur, in three blocks namely Mohana, R. Udaygiri and Nuagada of the Gajapati district in South Orissa. The outreach tribal villages of the mentioned blocks were selected for the purpose of study. Children under the age of 15 years were assessed, who attended the health camps on their own. Children were screened for sickling (by both chemical and direct method). Other laboratory examinations such as haemoglobin estimation (by Sahli's acid haematin method) and Differential Counts were done along with the clinical examination.

Results:

Table I: Age and sex wise distribution of sickling positive children.

Age Group Sex Total Affected
(in yrs) Male Female Children
0-4 48 41 89 (691)
5-9 94 77 171 (873)
10-15 16 06 22 (140)
Total 158 (914) 124 (790) 282 (1704)

The figures in parentheses show the frequency of total children as per age and sex.

A total of 1,704 children under 15 years of age had voluntarily attended the health camp. Among these children 914(53.64%) were males and 790(46.36%) were females. 282(16.55%) children were found sickling positive on screening.

Among the male children 158(17.29%) and among the female children 124( 15.7%) were found to be sickling positive. The difference in sex wise prevalence was not found statistically significant ( Χ 2 = 0.776; df =1; p>0.05). 89(12.88%) children in the age group of 0-4 years, 171(19.59%) children in the age group 5-9 years and 22(15.71%) children in the age group of 10-14 years were sickling positive. The difference in age wise prevalence of sickling positives was found significant ( Χ 2 = 12.64; df = 2; p<0.001).

Table II: Distribution of sickling positive children according to their castes.

Caste No. %
Raita 42 14.89
Sabar 27 9.57
Beera 24 8.51
Mandal 25 8.87
Lima 21 7.45
Majhi 18 6.38
Karad 18 6.38
Gomango 13 4.61
Dalbehera 11 3.90
Raika 9 3.19
Others (Karjee, Jani. Mali, Misal, Ganta, Paspureddy, Kaunri, Desnaidu, etc.) 74 26.24

The sickling positive children were belonging mostly to scheduled castes and scheduled tribes. They included Raita (14.89%), Sabar (9.57%), Beera (8.51%), Mandal (8.87%), Lima (7.45%), Majhi (6.38%), Karad (6.38%), Gomango (4.6%), Dalbehera (3.9%), Raika (3.19%) and others like Karjee, Jani, Mali, Misal, Ganta, Paspureddy, Kaunri and Desnaidu etc. (26.24%).

Table HI: Prevalence of anaemia in 0-15 years children.

[A] Sickling positive children with anaemia
Age Group Anaemia Total Anaemia
Mild Moderate Severe No. %
< 5 years (n=89) 58 23 04 85 95.5
> 5yrs (n=193) 135 43 09 187 96.89
Subtotal (n=282) 193 66 13 272 96.5
[B] Sickling negative children with anaemia (n=1,422) 1,283 90.2
Total children with Anaemia (n=(1,704)       1,555 91.26

91.26% of the total children were anaemic. The prevalence of anaemia in sickling positive children was 96.5% and the same in sickling negative children was 90.2%. The difference in the prevalence of anaemia in sickling positives as compared to that of normal children was found statistically significant ( Χ 2 = 11.44; df = l; p<0.001). Among the sickling positive children, 85(95.5%) of the under 5 years and 187 (96.89%) in the age group of 5-15 years were found to be anaemic. There was no significant difference in the prevalence of anaemia in different age groups (Χ 2 = 0.34; df=1; p>0.05). Among sickling positive children 193(71%) of the anaemics had mild anaemia, 66(24%) moderate anaemia and 13(5%) had severe anaemia.

Table IV: Distribution of splenomegaly in 0-15 years children.

[A] Sickling children with anaemia

Age Group Splenomagaly
No. %
0-4 years (n=89) 22 24.72
5-9 years (n=171) 60 35.1
10-15 yrs (n=22) 03 13.64
Subtotal (n=282) 85 30.1
[B]Sickling negative children (n=1,422) 149 10.48
Total (n=1,704) 234 13.73

[A] Sickling positive children with anaemia: 13.73% of total children had palpabale spleen below left costal margin. Splenomegaly was found in 30.1% of the sickling positive children which was significantly high ( Χ 2 = 76.8; df =1; p<0.001). Among sickling positive children, 24.72% of underfives, 35.1% of 5-9 years and 13.64% of 10-15 years were found to have splenomegaly. The difference in prevalence of splenomegaly in different age groups of sickling positive children was found to be significant ( Χ 2 = 6.04; df = 2; p<0.05).

In addition to anaemia and splenomegaly, ARI (20.8%), malnutrition (16.7%), fever (7.3%), avitaminosis (6%), pain abdomen (4.7%), musculoskeletal pain (0.6%) and other morbidities like pyoderma, ear discharge and caries teeth were encountered in sickling positive children during the clinical examination.

0.75% of sickling positive children with palpable spleen were branded on their abdomen over the area of splenic enlargement.

Neutrophilia was found in 14% of the sickling positive children and malaria parasite was not detected in any of them.

Discussion:

The childhood diseases in tribal area pose difficulty for diagnosis and management, as the signs and symptoms are modified by the haemoglobinopathies like sickle cell disorders. The manifestations of crisis overlap and aggravate the course of the disease. Moreover, it is beyond the scope and competency by the peripheral health institutions to identity such genetic disorders. Only high suspicion and screening can guide the community physicians for better management of cases and prevent childhood morbidity and mortality. The present study measures and highlights the epidemiological picture of sickle cell disorders in the tribal children. The prevalence of sickle cell disorders was found to be 16.55% in under fifteen years children. It was significantly high (19.59%) in 5-9 years age group children. Out of 282 sickling positive children, 89(31.56%) were under fives, 171 (60.64%) in 5-9 years age group and 22 (7.8%) were above 10 years of age. No significant difference was observed in sex wise prevalence of sickle cell disorder.

In a hospital based clinical study from western Orissa, the investigators found 52% of the SS disease patients within 15 years of age 7 . Maximum frequency of cases (24.4%) was found in 5-9 years age group 8 .

According to B.C. Kar, 50% of the children had their 1st manifestation within 5th year of life and another 25% manifested by 10th year of age. There was no difference in disease frequency between male and female 9 .

In the present study, majority of the children with positive sickling test were belonging to scheduled castes and scheduled tribes as these castes formed major part of the society. But in western Orissa, the patients were found to belong to almost all sub castes of the community, 19.6% were scheduled castes and 1.4% were scheduled tribes 7 .

Anaemia was the most common association and was found in 96.5% of sickling positive children. Majority of the cases had mild to moderate anaemia. Although anaemia is significantly found in sickling positive children, the hemoglobin status may not be steady throughout, but affected by crisis state and other intercurrent infections, aggravating the morbidity in children.

In a hospital based study, 77% of the sickle cell disease patients had total haemoglobin between 4-10 gm/dl 7 . Mean hemoglobin level in Indian SS disease patients was found to be 8.73±1.69 gm/dl (range 3.9-13.5) and the raised foetal hemoglobin level was attributed for mild to moderate anaemia in these patients 8 .

Although it is difficult to define hypersplenism in community without reticulocyte count, clinical splenomegaly was found in 30.1% of sickling positive children.

Splenomegaly was significantly associated with this disorder, with high frequency (35%) in 5-9 years age group. Still higher prevalence of splenomegaly (67.4%) was reported in Indian patients 9 which increased with age from 61 % in under fives to 72% in those aged 5-9 years and 100% in 10- 14 years age children. This could be due to disease state in children and small sample size in the hospital study 8 .

The point prevalence of different morbidities was confounded by the childhood disorders like ARI (20.8%), malnutrition (16.7%), avitaminosis (6%), febrile illness (7.3%) and other manifestations like epistaxis, generalized body pain, weakness, caries teeth, ear discharge and disorders of skin (34.9%). However, pain abdomen (4.7%), mucoculoskeletal pain (0.6%) and branded marks over the area of splenic enlargement suggested the manifestations of sickle cell disorder in childhood.

Sickle cell disorders in children are indistinguishable both clinically and haematologically. They run in families and seen in siblings. Therefore, the children with recurrent episodes of painful attacks in abdomen, muculoskeletal pain, ARI, fever, splenomegaly, anaemia and epistaxis should be suspected of sickle cell disorders. They should be screened by simple sickling test to identify the genetic disorder. Parental counselling and preventive measures like penicillin prophylaxis, regular folate supplementation, early treatment of ARI with simple antibiotics and management of pain with simple analgesics will be helpful in decreasing morbidity and mortality in childhood with sickle cell disease, hence will be promoting normal growth and development in these under privileged children.

References:

  1. O.P. Ghai, Essential Paediatrics, New Delhi, Interprint, 5 th Edn., pp. 100.
  2. Lehmann H, Cutbush M. Sickle cell trait in Southern India, British Medical Journal, 1952, Vol. I, 404-5.
  3. Kar BC, Devi S, Dash KG, Das M. The sickle cell gene is widespread in India, Trans Royal Soc. Trop. Med. Hyg., 1987; Vol. 81:275-8.
  4. Negi RS. Sickle cell trait in India. A review of known distribution, Bull. Anthropol Survey India, 1972; Vol. 17: 439-49.
  5. Balgir RS. Genetic epidemiology of the three predominant abnormal haemoglobins in India, JAPI, 1996; Vol. 44: 25-8.
  6. Ambedkar SS, Phadke MA, Mokashi GD et al. Pattern of Haemoglobinopathies in western Maharashtra, J. of the Indian Academy of Pediatrics, Vol. 38, May 17, 2001, 530-3.
  7. Kar BC, Devi S. Clinical profile of sickle cell disease in Orissa, Indian J. Paediatrics, 1997; Vol. 64: 73-7.
  8. Kar BC, Satapathy RK, Kulozik AE et al. Sickle cell disease in Orissa State, India, The LANCET, Nov. 22, 1986,1198-1201.
  9. Kar BC. Sickle cell disease in India, JAPI, 1991; Vol.39 No. 12: 954-60. 183

Sickle cell anaemia in tribal children Sahu T et al

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