PRO75 PRESCRIPTION MEDICATION UTILIZATION FOR THE MANAGEMENT OF SICKLE CELL DISEASE BY AGE GROUP

Introduction: Sickle cell disease (SCD) is associated with recurrent complications and healthcare burden. Although SCD management guidelines differ based on age groups, little is known regarding actual utilization of preventative (hydroxyurea) and palliative therapies (opioid and nonopioid analgesics) to manage complications. This study assessed whether there were age-related differences in SCD index therapy type and SCD-related medication utilization.Design and patients: Texas Medicaid prescription claims from September 1, 2011 to August 31, 2016 were retrospectively analyzed for SCD patients aged 2-63 years who received one or more SCD-related medications (hydroxyurea, opioid, or nonopioid analgesics).Outcome measures: The primary outcomes were SCD index drug type and medication utilization: hydroxyurea adherence, and days’ supply of opioid, and nonopioid analgesics. Chi-square, analysis of variance, and Kruskal–Wallis tests were used.Results: Index therapy percentages for included patients (N = 2,339) were the following: opioids (45.7 percent), nonopioids (36.6 percent), dual therapy-opioids and nonopioids (11.2 percent), and hydroxyurea (6.5 percent), and they differed by age-groups (χ2 = 243.0, p 0.0001). Hydroxyurea as index therapy was higher among children (2-12:9.1 percent) compared to adults (26-40:3.7 percent; 41-63:2.9 percent). Opioids as index therapy were higher among adults (18-25:48.0 percent; 26-40:54.9 percent; 41-63:65.2 percent) compared to children (2-12:36.6 percent). Mean hydroxyurea adherence was higher (p 0.0001) for younger ages, and opioid days’ supply was higher for older ages.Conclusions: Texas Medicaid SCD patients had low hydroxyurea utilization and adherence across all age groups. Interventions to increase the use of hydroxyurea and newer preventative therapies could result in better management of SCDrelated complications and reduce the frequency of pain crises, which may reduce the need for opioid use.

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Emergency Department Encounters, Hospitalizations and ED Reliance Among Medicaid Eligible Patients with Sickle Cell Disease in North Carolina

Blood ◽

10.1182/blood-2019-127196 ◽

2019 ◽

Vol 134 (Supplement_1) ◽

pp. 2113-2113

Author(s):

Paula Tanabe ◽

Nancy Crego ◽

Christian Douglas ◽

Emily Bonnabeau ◽

Marian Earls ◽

...

Keyword(s):

North Carolina ◽

Sickle Cell Disease ◽

Old Age ◽

Sickle Cell ◽

Research Funding ◽

Claims Data ◽

Age Group ◽

Cell Disease ◽

Old Patients ◽

The Mean

Introduction: Sickle cell disease (SCD) is a complex disease for which pain is the hallmark. Pain from vaso-occlusive episodes is the number one reason for ED visits and hospital admissions. This paper reports Medicaid claims data from NC for individuals with SCD, including: 1) ED encounters and re-encounters within 7, 14 and 30 days; 2) hospitalization and re-hospitalization within 7, 14 and 30 days; and 3) ED reliance (EDR) score. Methods: We examined Medicaid claims data from for patients with a diagnosis of SCD (ICD 9 CM codes: 282.6x, ICD 10 CM codes: D57.0x, D57.1, D57.2x, D57.4x, D57.8x) in North Carolina. Data is reported for a cohort of 2,790 patients with a diagnosis of SCD, age 1 to 65+ and enrolled at least 11 months in NC Medicaid between March 1, 2016 and February 28, 2017. ED re-encounters and re-hospitalizations within 7, 14 and 30 days were identified using the time between the date of service listed on the ED or hospital claim and the next date of service in the subsequent claim. Individual ED Reliance (EDR) score was calculated as the total number of ED encounters divided by the total ambulatory visits (outpatient + ED encounters) per enrollee, (ambulatory visits reported elsewhere). Similar to Kroner et al, an EDR of >0.33 was considered a high score. Inpatient claims were identified using a category of service code indicating hospitalization. Results: The participants in the sample (n=2790) were majority female (57.92%), lived in metropolitan areas (77.63%) and had a mean age of 23.05 years old (SD=16.06). Of the 9,075 total ED encounters, 69.86% of the total sample had an ED encounter during the 12-month study period. There was a mean of 3.25 (SD=7.38) and median of 1 (IQR = 0 - 3) ED encounters per patient for the sample. Those who were 18-30 years old had the highest mean and median ED encounters per patient (4.98, SD= 9.34 and 2, IQR 1 to 5). The 31-45 year old group had the second most, with 4.82 (SD= 11.03) total ED encounters. The percentage of the sample with an ED re-encounter within 7, 14, and 30 days was also highest among the 18-30 year old group (29.17%, 33.98% and 40.89%) followed by those 31-45 years old (23.71%, 28.49%, and 34.80%), respectively. The 31-45 age group had the second most hospitalizations/patient and re-hospitalizations. The mean EDR was highest among 18-30 year old patients (0.35) and 46.48% of this age group had an EDR of 0.33 or greater. In the 31-45 year-old age group, the mean EDR was 0.28 and 35.18% had an EDR of 0.33 or greater. The overall sample had a mean of 1.30 (SD= 2.75) hospitalizations/patient. The 18-30 year old age group also had the highest mean total hospitalizations (2.08, SD= 3.72) and mean re-hospitalizations within 7 (0.16; SD=0.77), 14 (0.41; SD=1.68), and 30 (0.82; SD=2.79) days. The 31-45 age group had the second most hospitalizations/patient and re-hospitalizations (Table 1). Conclusions: Overall, increasing age coincided with increased ED and inpatient utilization, as well as with the period of transition from pediatric to adult SCD care. Furthermore, high EDR was most prevalent in the 18-30 age group. Our study further supports the need for increased focus on acute care utilization in the 18-45 year-old age group and considerations for improved care transition interventions. Disclosures Tanabe: NIH: Research Funding; AHRQ: Research Funding. Shah:Novartis: Consultancy, Research Funding, Speakers Bureau; Alexion: Speakers Bureau; GBT: Research Funding.

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Assessing Disease Knowledge and Self-Management in Youth With Sickle Cell Disease Prior to Transition

Journal of Pediatric Oncology Nursing ◽

10.1177/1043454218819447 ◽

2018 ◽

Vol 36 (2) ◽

pp. 143-149 ◽

Cited By ~ 1

Author(s):

Barbara Speller-Brown ◽

Maureen Varty ◽

Lisa Thaniel ◽

Marni B. Jacobs

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Basic Knowledge ◽

Self Management ◽

Medical Attention ◽

Age Group ◽

Cell Disease ◽

Management Skills ◽

Disease Knowledge ◽

Baseline Hemoglobin

Introduction: Transition of medical care from pediatrics to adult can be challenging and difficult. Until the 1970s, only half of patients diagnosed with sickle cell disease (SCD) reached adulthood. As a result of patients living longer, there is a growing need to understand factors that influence readiness to transition. This descriptive study examined age-specific SCD knowledge, self-management skills of patients, and education goals in a convenience sample of patients and their parents. Method: One hundred eighty-three transition surveys were distributed during scheduled hematology clinic visits. Surveys were analyzed with descriptive statistics to determine differences of knowledge between age groups, self-care skills, vocational supports, and educational goals. The parent group consists of children aged 0 to 4 years (32), 5 to 8 years (52), 9 to 11 years (12); the child group consists of children aged 9 to 11 years (24) and 12 to 15 years (31); and adolescent and young adult (AYA) group consists of children aged 16 to 21 years (32). Results: Indeed, 50% of parents of the 0 to 4 years age group and 33% of 5 to 8 years age group knew their child’s baseline hemoglobin. Only 38% of patients aged 16 to 21 years knew their baseline hemoglobin. However, 79% of patients aged 9 to 11 years, 74% of patient aged 12 to 15 years, and 78% of AYAs could name their hematology provider. Only 66% of patients aged 16 to 21 years knew what symptoms required medical attention. Discussion: Most patients and parents had adequate basic knowledge regarding SCD. AYAs lack the disease knowledge necessary to transition care away from parents to become more independent. An assessment for transition readiness should be ongoing to include disease-specific knowledge and self-management skills.

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Transfusion and alloimmunization in sickle cell disease. The Cooperative Study of Sickle Cell Disease

Blood ◽

10.1182/blood.v76.7.1431.bloodjournal7671431 ◽

1990 ◽

Vol 76 (7) ◽

pp. 1431-1437 ◽

Cited By ~ 3

Author(s):

WF Rosse ◽

D Gallagher ◽

TR Kinney ◽

O Castro ◽

H Dosik ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Age Groups ◽

Beta Thalassemia ◽

Lewis Antigens ◽

Age Group ◽

Cooperative Study ◽

Cell Disease ◽

Explicit Function ◽

Hemoglobin Sc Disease

In 1,814 patients with sickle cell disease who had been transfused, the overall rate of alloimmunization to erythrocyte antigens was 18.6%. The rate of alloimmunization in this group appears to be an explicit function of the number of transfusions received because it increases exponentially with increasing numbers of transfusions. Alloimmunization usually occurred with less than 15 transfusions, although the rate of alloimmunization continued to increase when more transfusions were given. The rate of alloimmunization was less in patients with hemoglobin SC disease and sickle-beta+ thalassemia because these patients had received fewer transfusions. Children less than 10 years old had a slightly lower rate of alloimmunization than patients in other age groups even after correction for the number of transfusions given. Women were more frequently alloimmunized than men; this was largely due to the fact that women received more transfusions than men, but in the age group 16 to 20 years the increase may have been due in part to alloimmunization owing to pregnancy. Forty-five percent of those alloimmunized made antibodies of only one specificity; 17% made four or more antibodies reacting with different antigens. Antibodies to the C and E antigens of the Rh group, the Kell antigen, and the Lewis antigens were most commonly made. These findings may be important in formulating a rational transfusion policy in sickle cell disease.

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Establishing Sickle Cell Diagnostics and Characterizing a Pediatric Sickle Cell Disease Cohort in Malawi

Blood ◽

10.1182/blood.v126.23.2070.2070 ◽

2015 ◽

Vol 126 (23) ◽

pp. 2070-2070

Author(s):

Jonathan Brett Heimlich ◽

Godwin Chipoka ◽

Portia Kamthunzi ◽

Yuri D. Fedoriw ◽

Nigel S. Key ◽

...

Keyword(s):

Blood Pressure ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Joint Pain ◽

Clinical Care ◽

Diagnostic Methods ◽

Sub Saharan Africa ◽

Age Group ◽

Cell Disease ◽

Sub Saharan

Abstract Sickle cell disease (SCD) is highly prevalent in sub-Saharan Africa; however, there are relatively few studies describing the clinical profile for children with laboratory-confirmed SCD. Prior to December 2014, neither neonatal screening nor standardized methods for SCD diagnosis were routinely available in Malawi, as hemoglobin electrophoresis and alternative diagnostic methods were absent. We describe implementation of hemoglobin electrophoresis for children with clinically suspected SCD at Kamuzu Central Hospital, one of two national teaching hospitals in Malawi. Children with clinically suspected SCD were recruited January - May 2015 and underwent comprehensive clinical and laboratory characterization. 137 total patients were recruited and 117 were confirmed to have HbSS disease. Among children who were being cared for as SCD prior to enrollment, 86% had HbSS suggesting generally accurate clinical diagnosis by local providers. Baseline clinical parameters and self-reported SCD complications for the study population are displayed in Table 1. Of those with confirmed SCD, median age was 7.3 years (IQR 2.7-10.4) with 53% males. Prior malaria was reported by 39% of patients, and was higher in the 0-5 age group compared with the over 5 age group (46% vs. 31%, p=0.03). The most commonly reported SCD complications were anemia (72%), joint pain (56%), jaundice (52%), and acute pain episodes (50%). Children with confirmed SCD had median hemoglobin of 7.3 g/dL (IQR 6.9-7.9), total bilirubin of 1.7 mg/dL (IQR 1.1-2.6) and lactate dehydrogenase of 658 IU/L (IQR 527-773). Urinalysis demonstrated 26% of patients with blood and 7% with proteinuria by dipstick. As of May 2015, more than 250 samples for enrolled children as well as routine clinical care had been batch-processed weekly with an average turn-around time of 36 hours for results. Three Malawian laboratory technicians were trained to perform hemoglobin electrophoresis, all of whom have been performing the test independently since April 2015. Our findings highlight a need for wider implementation of resource-appropriate diagnostics as an essential foundation for care and research. Children had substantial clinical and laboratory evidence of SCD-related morbidity. Earlier diagnosis can improve care for this population by facilitating earlier therapeutic interventions, as well as providing a basis for research to better understand SCD-related morbidity in sub-Saharan Africa. These efforts can ultimately inform management strategies to improve outcomes and increase life expectancy among children with SCD in Malawi. Table 1. All (n=117) Male (n= 62) Female (n=55) p value Age years, median (IQR) 7.3 (2.7-10.4) 5.3 (2.3-9.4) 8.9 (4.2-11.9) 0.004 Height cm, median (IQR, n) 115 (88-131, 60) 111 (89-128, 36) 119.5 (93-140, 24) 0.21 Weight kg, median (IQR, n) 19 (13-27, 108) 16.5 (12-23.6, 58) 21 (14-30, 50) 0.01 Blood Pressure Systolic mmHg, median (IQR, n) 103 (98-110, 83) 101 (94-108, 43) 103 (99-110, 40) 0.37 Blood Pressure Diastolic mmHg, median (IQR, n) 60 (55-65, 83) 58 (53-65, 43) 61 (56-68, 40) 0.13 Heart Rate BPM, median (IQR, n) 104 (91-118, 114) 105 (94-123, 61) 104 (88-112, 53) 0.15 O2 Saturation %, median (IQR, n) 93 (88-97, 108) 91 (85-96, 59) 95 (91-98, 49) 0.004 % Hypoxemic (SPO2 < 90%), n (%) 36 (30.7) 26 (41.9) 10 (18.2) 0.005 Body Temperature Celsius, median (IQR, n) 37 (36.7-37.4, 91) 37 (36.7-37, 46) 37 (36.4-37.2, 45) 0.22 Positive History of: Malaria, n (%) 45 (38.5) 22 23 0.34 0-5 years, n (%) 25 (46.3) - - 0.03 6-18 years, n (%) 20 (31.7) - - Pneumonia, n (%) 29 (24.8) 10 (16.1) 19 (34.5) 0.02 HIV, n (%) 0 0 0 - Anemia, n (%) 84 (71.8) 49 (79.0) 35 (63.6) 0.06 Pallor, n (%) 16 (13.7) 7 (11.3) 9 (16.4) 0.43 Jaundice, n (%) 61 (52.1) 33 (53.2) 28 (50.9) 0.82 Received Blood Transfusion, n (%) 87 (74.4) 47 (75.8) 40 (72.7) 0.47 Days since last transfusion, median (IQR) 316 (133-1144) 240 (111-410) 577 (180-1784) 0.03 Pain episodes, n (%) 58 (49.6) 27 (43.5) 31 (56.4) 0.16 Joint pain, n (%) 66 (56.4) 33 (53.2) 33 (60.0) 0.34 Dactylitis, n (%) 41 (35.0) 19 (30.6) 22 (40.0) 0.29 Leg ulcers, n (%) 5 (4.3) 5 (8.1) 0 0.03 Stroke, n (%) 10 (8.5) 5 (8.1) 5 (9.1) 0.84 Nocturnal Enuresis, n (%) 24 (20.5) 12 (19.4) 12 (21.8) 0.74 Disclosures No relevant conflicts of interest to declare.

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Renal manifestations of sickle cell disease in children

International Journal of Contemporary Pediatrics ◽

10.18203/2349-3291.ijcp20202609 ◽

2020 ◽

Vol 7 (7) ◽

pp. 1524

Author(s):

Milind B. Kamble ◽

Vidhya S. Totewad

Keyword(s):

Risk Factors ◽

Steady State ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Renal Involvement ◽

Screening Tests ◽

Age Group ◽

Coagulation Test ◽

Cell Disease ◽

Homozygous State

Background: Sickle cell disease prevalence is more in central part of India. This study tries to find extent of renal involvement, risk factors and screening tests in sickle cell disease.Methods: Study was Cross sectional observational study. Demographic and clinical findings were recorded. Renal function tests like serum creatinine, blood urea nitrogen, eGFR were studied. Presence of microalbuminuria was checkeed. Ultrasonography abdomen was done to see the texture and corticomedullary differentiation of kidney.Results: Total 143 patients were studied. Out of which, 117 homozygous (SS type), 26 heterozygous (AS type). Majority belonged to school going age group (i.e. 5 to 10 year age group) with male preponderance in homozygous state. Microalbuminuria was more common in crisis state (61.84% in SS pattern and 83.33% in AS pattern) as compared to steady state in both SS and AS pattern patients. Dipstic method detected more proteinuria than heat coagulation suggesting dipstick method superior to heat coagulation test. Low eGFR was common in patients with SS pattern as compared to patients with AS pattern and was significantly associated with microalbuminuria in both steady state and crisis state. Abnormal USG was seen 3.44% in patients with SS pattern. Renal involvement started below 5 years of age and then increases with age, homozygous childrens in crisis state are affected more.Conclusions: Renal involvement is common in sickle cell anemia i.e it is seen in 50% of the patients. Increasing age, male sex and homozygous state were risk factors. Simple test like dipstic method and heat coagulation test can be used as screening test to detect microalbumiburia, with dipstic method being superior. Presence of microalbuminuria can detect early renal involvement in sickle cell disease. This will help in early diagnosis and management of such patients which avoids further renal complications and thus prevents mortality and morbidity.

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Mean Platelet Volume Predicts Mortality in Sickle Cell Disease

Blood ◽

10.1182/blood-2018-99-117648 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 2383-2383

Author(s):

Aravind Thavamani ◽

Regi Ramanathan ◽

Mammen Puliyel

Keyword(s):

Sickle Cell Disease ◽

Disease Severity ◽

Sickle Cell ◽

Mean Platelet Volume ◽

Age Group ◽

Pediatric Age ◽

Cell Disease ◽

Platelet Volume ◽

Pediatric Age Group ◽

Probability Of Death

Abstract Background: Sickle cell disease (SCD) is a life-threatening disease with varied clinical spectrum and severity leading to premature death. There is a lack of validated prognostic markers in SCD. Recent evidence suggests that inflammation and platelet adhesion plays a critical role in the pathophysiology of vaso-occlusion in SCD. Elevated Mean Platelet Volume (MPV) values are associated with a higher degree of inflammation in many disease states but its effect on sickle cell disease or its severity is unknown. Objective: To analyze the role of MPV in predicting disease severity/mortality in patients with SCD. Methodology: This is a retrospective single center study and included patients with sickle cell disease between 6 months and 60 years of age during a 10-year period (2006-2016). Demographic information, lab data and clinical information including acute chest syndrome (ACS), priapism, transfusions, sepsis, pain crisis, avascular necrosis (AVN) were collected. All laboratory data were collected in steady state with no crisis in the recent past 3 months. The disease severity score/probability of death was calculated using a validated model to predict risk of death in sickle cell disease (Sebastiani et al. Blood 2007). Spearman's correlation test was used to analyze correlation coefficient between MPV and probability of death. Results: Total no. of patients =230; Male 112 (49%); Female 118 (51%). All patients were of African-American origin. Disease severity, Hb SS - 156 (67.5%); Hb SC - 64 (27.8%) and Sickle-Beta thalassemia 11 (4.7%). MPV has a significant positive correlation with the probability of death, p < 0.001 and correlation coefficient, r=0.222. Of the total population, 78 had acute chest syndrome, 30 had AVN, 47 had recurrent pain crisis, 16 had stroke (2 -moyamoya cases), 18 had culture proven sepsis. Our population was further divided into 3 subgroups based on their age (Group 1 - 0-18 years; Group 2 - 19-40 years; group 3- above 40 years) and we found no statistical difference in the mean platelet volume across different age groups. However in pediatric population (Group 1), hydroxyurea was associated with significantly lower MPV, p=0.023 and this is independent of Hb F levels. Using linear regression model, with probability of death as a dependent variable and hydroxyurea, MPV as independent variables, MPV maintains a significant association with probability of death (p=0.003). We also found strong positive correlation of MPV with the probability of death in pediatric age group, p=0.004, r =0.405. Conclusion: MPV is an independent biomarker predicting disease severity and probability of death in patients with sickle cell disease with a strong correlation especially in pediatric age group. Although there was no statistical significance across age groups, the variation of MPV for each patient with age needs to be studied for better understanding. Hydroxyurea a known disease-ameliorating agent is associated with lower MPV values in pediatric age group. This effect is independent of the levels of fetal hemoglobin and may be due to anti-inflammatory effect of hydroxyurea or decreased platelet consumption. Figure. Figure. Disclosures No relevant conflicts of interest to declare.

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Mortality Trends in People with Sickle Cell Disease in North Carolina.

Blood ◽

10.1182/blood.v106.11.3794.3794 ◽

2005 ◽

Vol 106 (11) ◽

pp. 3794-3794

Author(s):

Rupa Redding-Lallinger

Keyword(s):

North Carolina ◽

Sickle Cell Disease ◽

Cause Of Death ◽

Sickle Cell ◽

Childhood Mortality ◽

Age Group ◽

Death Certificates ◽

Cell Disease ◽

Mortality Trends ◽

Underlying Cause Of Death

Abstract Background. Using data from the Cooperative Study of Sickle Cell Disease which ran from 1978 -19**, published figures for median life expectancy for men with sickle cell disease is 42 years and for women 46 years. Recent data from Texas show a declining childhood mortality rate, compared to the CSSCD data, in a cohort who have been followed from infancy. However, despite the strides made in reducing childhood mortality and improving length and quality of life in some adults, there is still very significant morbidity and mortality from cumulative damage to organs, especially the lungs and the kidneys. Aims. The purpose of this investigation is to analyze the numbers and causes of death in individuals with sickle cell disease over the past several years in North Carolina. The information gathered may suggest areas in which health services to people with sickle cell disease may be improved. Methods. The North Carolina State Center for Health Statistics provided the following data for the years 1997–2003. Total number of SCD deaths for each of those years, and the age, county of residence, place of death, the ICD codes for the underlying and contributing cause of death as listed on the death certificates of the decedents. Results. There were 277 deaths over the 7 year period. Fifty-three percent of these deaths occurred during the earlier half of this period. Overall, 49% of the deaths occurred in people from age 30 to age 49, and this proportion remained quite stable during the 7 years. Deaths of people 20 years and younger made up 14% of total deaths; there were fewer in the second half of the time period than in the first half—40% of the total deaths in this age group occurred from late 2000 through 2003 vs 60% from 1997 through early 2000. Deaths in the youngest age group (0–5) were only 4.3% of the total deaths for the period. For the individuals who died between birth and 20 years of age, the underlying cause of death from the death certificates were grouped as “sickle cell disease with or without complications” in 50%, congenital heart disease in 11%, bacterial infection in 17%, gastroenteritis in 9.5%, UTI in 6% and other in 4%. For those who died between 30 and 40 years of age the underlying cause of death as listed on the death certificates were grouped as: sickle cell anemia, with and without crisis—58%, pulmonary complications—12%, cardiovascular problems—10%, CNS problems—5%, hepatic disorders and infections—each <5%, and other—10%. Conclusions. Mortality in the youngest children appears to have lessened in comparison with historical data, and this age group no longer leads in mortality rates. Deaths in the young adult age range of 30–49 years have showed no downward trend over 7 years. The cause of death from death certificates do not match well with the clinical problems typically seen in people in this age group. The lack of precise diagnoses on death certificates may reflect a lack of understanding of sickle cell complications among doctors in the community.

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The study of the incidence of pregnant women with sickle cell disease

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20194360 ◽

2019 ◽

Vol 8 (10) ◽

pp. 3950

Author(s):

Jyoti Lagoo ◽

Arpita Lagoo

Keyword(s):

Sickle Cell Disease ◽

Pregnant Women ◽

Sickle Cell ◽

Globin Gene ◽

Public Health Problem ◽

High Morbidity ◽

Major Public Health Problem ◽

Age Group ◽

Cell Disease ◽

Inherited Disorders

Background: The sickle cell disease is major public health problem which causes high morbidity and mortality in India. It is observed that SCD is scourge in Chhattisgarh since long past. Sickle cell disease is a term for a group of genetically inherited disorders characterized by production of abnormal hemoglobin. “Hemoglobin-S” results from a point mutation in the beta globin gene. The main objective is to study the incidence of pregnant women with sickle cell disease.Methods: It is a hospital based prospective study. It was conducted at Obstetrics and Gynecology department of LTBRKM Govt. Medical College, Jagdalpur, Chhattisgarh. The study was carried out from August 2014 to October 2015. The study included screening of all patients attending antenatal clinic and in labour ward during emergency. 75 cases were found to be sickling positive. Permission from Institutional Ethics Committee was obtained.Results: The incidence of SCD in India is 44%, in Chhattisgarh is 17%.At our institute in pregnant women is 1.75%. The incidence of HbAs group was 70.66% and HbSS was 26.66%. In Hb AS group maximum 47% patients were in age group of 26-30 years. and also in same age group the incidence of HbSS group was 60%. In age group of 31-35 years. 22% of patients were of HbAS group, but only 10% of patients were of HbSS group. HbAS group and HbSS group the percentage of primi gravida were 49% and 60% respectively. It is noted that in HbAS group only 3% of patients had parity >4, but in HbSS group it was 10%.Conclusions: In conclusion, it has been shown that the clinical statuses of the most sickle cell diseases patience were not seriously affected by pregnancy if they are given appropriate prenatal care. All pregnant women should be screened for sickle sell hemoglobinopathy in endemic region, like in our state Chhattisgarh.

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Quantifying the Levels of Knowledge, Attitude, and Practice Associated with Sickle Cell Disease and Premarital Genetic Counseling in 350 Saudi Adults

Advances in Hematology ◽

10.1155/2019/3961201 ◽

2019 ◽

Vol 2019 ◽

pp. 1-7 ◽

Cited By ~ 1

Author(s):

Heba M. Al-Qattan ◽

Dana F. Amlih ◽

Fatima S. Sirajuddin ◽

Dalal I. Alhuzaimi ◽

Mai S. Alageel ◽

...

Keyword(s):

Genetic Counseling ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Public Healthcare ◽

Sociodemographic Characteristics ◽

Age Group ◽

Cell Disease ◽

Care Clinic ◽

Attitude And Practice ◽

Knowledge Attitude And Practice

Our study aims to observe the levels of knowledge, attitude, and practice (KAP) associated with sickle cell disease (SCD) and premarital genetic counseling (PMGC) in 351 Saudi adults. The relationships between KAP levels and sociodemographic characteristics (age, gender, marital status, and educational level) were observed. The study was conducted in King Khalid University Hospital between February 21, 2017, and March 7, 2018. A total of 351 Saudi participants attending the primary care clinic were selected using convenience sampling and were given a self-administered questionnaire. Overall, the 351 participants had the best attitude (41% scoring “good”), followed by knowledge (28.8%), and, lastly, practice (19.1%). Out of the sociodemographic characteristics, age group was the most statistically significant in all the three categories (knowledge, attitude, and practice). The > 50-year age group performed the worst in all the three categories. Despite the advancements in public healthcare measures in Saudi Arabia, our study revealed that there are still many gaps to be filled regarding the knowledge, attitude, and practice associated with SCD and PMGC.

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