|Year : 2016 | Volume
| Issue : 2 | Page : 31-36
Sickle cell disorders in females: Screening of sickle hemoglobinopathy be part of Antenatal and Intensive care?
Jitendra D Lakhani, Varsha Shah, Dulari Gandhi, Niraj Pandit, Deep Mehta, Bakul Leuva, Jasmin Jasani, Pawan Toshiniwal, Sucheta Lakhani, A Muley, Hemal Dave, Paresh Golwala, Dimple Thakore
Smt. B. K. Shah Medical Institute and Research Center & Dhiraj Hospital, Sumandeep Vidyapeeth, Piparia, Waghodia, Vadodara, Gujarat, India
|Date of Web Publication||30-Aug-2018|
Jitendra D Lakhani
Smt. B. K. Shah Medical Institute and Research Center & Dhiraj Hospital, Sumandeep Vidyapeeth, Piparia, Waghodia, Vadodara, Gujarat
Source of Support: None, Conflict of Interest: None
Introduction: Women with sickle cell anemia may have complications especially during pregnancy. It may contribute to anemia in pregnancy.
Methodology: As a part of Sumandeep Vidyapeeth’s Sickle Project, this observational study of profile of 125 female patients was done.
Observations: 125 female patients with sickle hemoglobinopathy who came either for antenatal checkup or for other health services in last 2 months were studied. 119 had sickle cell trait (SCT) while 6 (4.8%) had sickle cell disease (SCD). Majority of them were young, average age being 22.26 years. 14 (11+3, SCT and SCD) patients required indoor admissions. 3(SCT) patients were admitted in Critical Care Units, 7(6+1) in Obstetrics, 2(1+ 1) in medical and 2 (1+1) were admitted in pediatrics ward. Mean Hb of these 125 patients was 10.79 gm/dL, TC= 10702±3922/cu.mm, PCV of 32.70±7.98%, RBC count of 4.62±1.04 mil/uL, MCV=76.16±66.08fl, MCH=21.74±5.18pg, MCHC=28.35±8.74%, RDW-CV was 16.53±4.09. HPLC in percentage of A1a, A1b, A1c, LA1c and P3 were 0.96±0.72, 0.76±0.56, 4.90±0.81, 0.56±0.17 and 3.70±1.05 respectively. HBF=1.55±2.56, A0=57.34±11.69, A2=3.67±0.49 and S WINDOW was of 28.12±10.82.3. Out of 6 SCD patients,3 were admitted, 2 for severe Anemia and 1 for Crises. Mean Hb of SCD was 8.3±1.99 gm/dL, TC 15438±10562/cumm, PCV 27±5.75%, MCV 76.32±7.95fl, MCH=23.15±2.23pg, MCHC=30.37±1.75% RBC count of 3.60±1.04 Mil/uL, RDW-CV=21.73±6.99, HbS and HbF was 68.11±22.21% and 9.95 ± 6.3%.
Conclusions: Female patients coming from tribal area may need screening for sickling during antenatal checkup. SCT patients may also have morbidity and may require hospital admission.
Keywords: Sickle cell trait (SCT), Sickle cell disease (SCD), HPLC. (High Performance liquid chromatography). Tribal population
|How to cite this article:|
Lakhani JD, Shah V, Gandhi D, Pandit N, Mehta D, Leuva B, Jasani J, Toshiniwal P, Lakhani S, Muley A, Dave H, Golwala P, Thakore D. Sickle cell disorders in females: Screening of sickle hemoglobinopathy be part of Antenatal and Intensive care?. J Integr Health Sci 2016;4:31-6
|How to cite this URL:|
Lakhani JD, Shah V, Gandhi D, Pandit N, Mehta D, Leuva B, Jasani J, Toshiniwal P, Lakhani S, Muley A, Dave H, Golwala P, Thakore D. Sickle cell disorders in females: Screening of sickle hemoglobinopathy be part of Antenatal and Intensive care?. J Integr Health Sci [serial online] 2016 [cited 2022 Dec 4];4:31-6. Available from: https://www.jihs.in/text.asp?2016/4/2/31/240199
| Introduction|| |
Women with sickle cell anemia needs special attention as their profile of presentation and complications may be different. They may need different type of care in comparison with males. They pose special complications especially during pregnancy. It may result into multisystem problems which may cause misery. About one third of pregnant women with sickle cell disorder have complications in form of neonatal death, abortion or stillbirth. these complications may be related to Sickle cell Disease (SCD) (homozygous) patients. It is believed that Sickle cell trait (SCT) (heterozygous) pregnant women do not have complications during pregnancy however reports suggests that fetal loss may occur in trait patients also. Urinary tract infections are also common in pregnant women having SCT.
Neonatal screening is considered as a part of governmental health program in many countries, , however it has limitations. To know natural history of local Sickle cell disorder, neonatal screening may prove beneficial. It is also imperative for timely intervention with hydroxyurea therapy, vaccines and blood transfusions. Prevention as well as treatment of crises and multi organ failure is vital and they may need services for the same in form of indoor admission. Many of such patients need admission in critical care wards for infections, crises and multiorgan dysfunction.
To know what problem this sickle cell disorders female patients have and how useful screening in tribal population of our health seeking patients needs, this study was undertaken.
| Methodology|| |
As a part of Sumandeep Vidyapeeth’s Sickle Project, this observational study aimed to find out profile of 125 female patients having sickle cell disorder (Sickle cell Disease or Sickle cell Trait) was done. These were the patients who came for getting health services from our institute. We also had objective to offer them need based and adequate health services including counseling and support services. We also had intention to follow them for pregnancy outcome and to go for neonatal screening. All female patients who came to seek health services in previous 2 months either for antenatal checkup or for other health services at Dhiraj Hospital (attached to SBKS Medical Institute and Research Centre) and who were either found to have sickle cell trait (SCT) or disease (SCD) were included. Screening was done by sickling solubility test and was confirmed by HPLC (High Performance liquid chromatography).
All hematological investigations like CBC, Blood Indices, RDW-CV, RBC count, Mentzer index and Srivastava Index, was taken. Discriminate Indices like Mentzer Index was calculated by MCV/RBC count and Srivastava Index by MCH/RBC count. Relevant biochemical, microbiological, imaging and pathological ancillary investigations needed by clinicians to treat/offer the services were also done.
| Results|| |
Of 125 female patients, 119 had sickle cell trait (SCT) while 6 (4.8%) had sickle cell disease (SCD). Average age was 22.26±4.77 years. Majority (101 of 125) were in age group of 18 to 25, 18 were of 26-35 years while 6 were below 18 years. Of total 125 female patients, 14(11.2%) patients required indoor admission. Of this14 patients, 11 had SCT (9.24% of all trait) and 3 had SCD (50% of total 6). 7(6 SCT,1SCD) patients were admitted in Obstetrics ,3(all SCT) in CCUs, , 2(1 SCD,1 SCT) in medical and 2 (1 SCD,1 SCT) were admitted in pediatrics ward. [Figure 1] 3 out of 6 SCD were admitted, 2 for severe Anemia and 1 for Crises, while 3 patients of Sickle cell disease could be treated on outdoor basis.
Mean Hb of these 125 patients was 10.79 gm/dL, TC= 10702±3922, PCV of 32.70±7.98%, RBC count of 4.62±1.04Mil/uL, MCV=76.16±66.08fl, MCH=21.74±5.18pg, MCHC=28.35±8.74%, RDW- CV was 16.53±4.09. HPLC in percentage of A1a, A1b, A1c, LA1c and P3 were 0.96±0.72, 0.76±0.56, 4.90±0.81, 0.56±0.17 and 3.70±1.05 respectively. HBF=1.55±2.56, A0=57.34±11.69, A2=3.67±0.49 and S WINDOW was of 28.12±10.82.3 out of 6 SCD were admitted, 2 for severe Anemia and 1 for Crises. Mean Hb of SCD was 8.3±1.99 gm/dL, TC, 15438±10562, PCV 27±5.75%, MCV 76.32±7.95fl, MCH=23.15±2.23pg, MCHC=30.37±1.75% RBC count of 3.60±1.04 Mil/uL, RDW-CV=21.73±6.99, HbS and HbF was 68.11±22.21% and 9.95 ± 6.3%.
Hematological profile of females having sickle hemoglobinopathy is shown in [Table 1]. The second column in the described table shows parameters are of both disease as well as trait patients, while third column of only SCD patients. Data of 6 patients of Sickle cell disease in form of hematological and HPLC profile is shown separately as mentioned earlier, as only small number had homozygous sickle cell disorder.
|Table 1: Hematological and HPLC profile of all female (N=125 having SCD & SCT) and of only Sickle cell Disease patients (n=06)|
Click here to view
| Discussion|| |
It is believed that Sickle cell trait (SCT) (Heterozygous) pregnant women do not have complications during pregnancy. However, Taylor M.Y. et al. after their retrospective analysis of two years data, concluded that “sickle cell trait may not be as benign for the fetus as was previously thought”. They studied 131 pregnant women who had sickle cell trait and found complications like fetal loss and IUGR (intrauterine growth retardation). They also found premature rupture of the membranes which was at preterm. One neonatal and 10 IUFDs (Intrauterine fetal death) was present in trait patients. 50% had amniotic fluid infection. other complication described with SCT is Urinary tract infections which is common during pregnancy.
Though we intended to find outcome of pregnancy in both Sickle cell disease and trait patients, our primary aim was to know the profile of these patients. We observed that mean hemoglobin of female patients study patients was 10.79 ±6.66 gm/dl, which was low both for pregnant as well as non pregnant females. WHO has defined anemia in pregnant female as hemoglobin below 11 gm/dl and non-pregnant pregnant female of 12 gm/dl.10 if we do not screen such patients, pregnant female maybe considered having anemia of pregnancy and maybe treated with iron therapy. We observed low MCV, low MCH and increased RDW with Mentzer and Srivastava Index results suggesting iron deficiency and excluding associated thalessemia in our group of patients, further evidence may be needed to establish role of iron therapy in sickling disorder patients. Nevertheless iron overload has to be avoided.
Mean Mentzer index in our patients was 18.07 ± 19.99, Srivastava Index was 5.09 ±2.58and mean RDW-CV was 16.52 ±4.09 which was suggestive of Iron Deficiency. Mentzer Index more than 13, Srivatava index was more than 3.8 and RDW-CV more than 15 % is suggestive of Iron deficiency Anemia. The Mentzer index gives the highest reliabilities for differentiating βeta-thalessemia trait from Iron deficiency anemia, sensitivity being (98.7%) and specificity (82.3%).11 Srivastava and Bevington Index has sensitivity for Iron deficiency anemia of 79% and specificity of 74%, and for differentiating from βeta thalessemia trait, sensitivity is 74% and specificity 79%.
Our study showed that female patients coming from tribal area like Devgedh Baria, Nasvadi, Chhota Udaipur, Rajpipla, Kwant of Gujarat state and bordering States like MP and Maharastra having surnames of Vasava, Bariya, Rathwa, Bhil, Tadvi, Bhilala, Nayak etc and clinically appearing anemic or admitted with infections and/or features suggestive of crises may need screening for sickling during antenatal checkup and in ICUs.
Though SCD patients may need indoor admission more, trait patients also require indoor admissions. SCT patients may also have morbidity which was evident in this study. Of 119 sickle cell trait patients, 11 (9.24%) required indoor admissions. In fact all three patients who required ICU admission were trait patients. It was also observed in these three patients whose admission was because of severe infections and/or multiorgan dysfunction. Seven patients were admitted in Obstetrics ward, only one had disease while 6 had trait. Of 2 admitted patients of Pediatrics ward, one had trait while other had disease. In Medicine ward, 2 patients were admitted, one had disease and another one had trait. In SCD patients, obvious cause like crises or anemia was present which made management easy. In contrast to this, in trait patients, multiorgan and complex symtomatology made discernible difference in further management.
Majority of time trait patients are considered asymptomatic and there screening is disputed. our study suggest that in certain group of tribal population, knowing trait status is helpful. Though it has effect on counseling aspect, it also important to clinicians. Trait patient may mimic other ailments and unnecessary other investigations may be carried out. Though in quantitative terms, sickle hemoglobin is trait patient is less and normal hemoglobin does exist, still abnormal hemoglobin is present in such patients. This small quantity abnormal hemoglobin may cause problems in altered physiological state like pregnancy. SCT may also pose problems when demanding situation comes up like infections, dehydration, electrolyte disturbance and others.
| Conclusion|| |
Sickle cell trait screening during antenatal period may be beneficial in management as it may be the cause of anemia during pregnancy. Sickle cell trait status cannot be consider benign and may require hospital admission. Screening patients coming from tribal population who are admitted in ICU may be useful for proper management.
| References|| |
Dauphin-McKenzie N, Gilles JM, Jacques E, Harrington T : Sickle cell anemia in the female patient. Obstet Gynecol Surv. 2006 May;61(5):343-52.
Taylor MY, Wyatt-Ashmead J, Gray J, Bofill JA, Martin R, Morrison JC. Pregnancy loss after first-trimester viability in women with sickle cell trait: time for a reappraisal? Am J Obstet Gynecol. 2006; Jun;194(6):1604-8.
Bencaiova G. , Krafft A, Breymann Ch. Sickle cell trait and urinary tract infection in pregnancy, Int J Gynaecol Obstet. 2006 Feb;92(2):128-9. Epub 2005 Dec 15.
De Jesús VR, Mei JV, Cordovado SK, Cuthbert CD. The Newborn Screening Quality Assurance Program at the Centers for Disease Control and Prevention: Thirty-five Year Experience Assuring Newborn Screening Laboratory Quality. International journal of neonatal screening. 2015;1(1):13-26.
Naik RP, Haywood C. Sickle cell trait diagnosis: clinical and social implications. Hematology / the Education Program of the American Society of Hematology American Society of Hematology Education Program. 2015;2015(1):160-167.
Serjeant GR. Evolving locally appropriate models of care for Indian sickle cell disease. Indian J Med Res. 2016 Apr; 143(4): 405-413
] [Full text]
Mentzer WC. Differentiation of iron deficiency from thalassaemia trait”. Lancet. April 1973. 1 (7808): 882.
Srivastava PC. Differentiation of thalassemia minor from iron deficiency. Lancet 1973; 2:154-155.
WHO/UNICEF/UNO; IDA. Prevention, assessment and control. Report of a WHO/UNICEF/UNO Consultation. Geneva: WHO; 1998. page 33.
Vehapoglu A, Ozgurhan G, Demir AD, Uzuner S, Nursoy MA, Turkmen S, Kacan A. Hematological indices for differential
diagnosis of beta thalassemia trait and iron deficiency anemia. Anemia. 2014 Apr 10;2014.
Okan V, Cigiloglu A, Cifci S, Yilmaz M, Pehlivan M. Red cell indices and functions differentiating patients with the β- thalassaemia trait from those with iron deficiency anaemia. Journal of International Medical Research. 2009 Feb 1;37(1):25-30.