| Literature DB >> 35198289 |
Lemchukwu Amaeshi1, Olufunto O Kalejaiye2,3, Chibuzor F Ogamba4, Folasade Adelekan Popoola4, Yusuf A Adelabu2, Chibuikem A Ikwuegbuenyi4, Ijeoma B Nwankwo2, Oluwademilade Adeniran2, Michael Imeh4, Michael O Kehinde2,3.
Abstract
Background Sickle cell disease (SCD) is a genetic disease of public health concern. Improved quality healthcare has increased the life expectancy of these patients; however, they also face an increased frequency of vaso-occlusive crises and other SCD complications. These complications affect their quality of life, an area of care, which healthcare providers often overlook. We sought to determine the health-related quality of life among patients living with sickle cell disease in Lagos, Nigeria. Materials and methods We conducted a cross-sectional study of 198 patients with sickle cell disease who attended the adult sickle cell clinic at a tertiary hospital in Lagos, Nigeria, during the period from October 1, 2018, to February 28, 2019. A self-administered questionnaire was used to obtain the clinical and socio-demographic characteristics of the patients and the 35-item Short-Form Health Survey (SF-36) questionnaire was used to determine their health-related quality of life (HRQoL). Determinants of HRQoL were established using bivariate and multivariate regression analysis. Results The mean age of the 198 patients who participated in the study was 28.4±9.1 years, mean steady-state hemoglobin was 8.2 ± 1.3 g/dl, and 85 (42.9%) patients had a monthly income of 150 USD or less. In the previous year, 65 (32.1 %) and 33 (16.6%) patients, respectively, suffered one to two episodes (s) of acute bone pain crises and acute chest syndrome, and 43 (24.7%) had blood transfusion. Using the scoring system for SF-36 provided by RAND Health, role limitation due to physical health had the lowest median score of 50 (interquartile range {IQR}: 0-100). On bivariate analysis, bone pain crisis was associated with statistically significant low scores across all the 8 HRQoL domains of the SF36 questionnaire. Other variables, including having received blood transfusion, recent hospitalization, acute chest syndrome, lower level of income, and younger age, were also associated with significantly low scores. On regression analysis, bone pain crisis, level of income, and acute chest syndrome were found to be independent determinants of quality of life in the patients. Conclusion Sickle cell disease has a negative impact on the health-related quality of life of those affected. The presence of bone pain crisis is an important predictor of health-related quality of life in sickle cell disease patients. To improve patient outcomes, healthcare providers should take a holistic approach in evaluating and managing this disease, taking into cognizance how the complications and the financial burden of this disease impact the quality of life of affected patients.Entities:
Keywords: bone-pain crises; health-related quality of life; lagos; sf-36 questionnaire; sickle cell disease
Year: 2022 PMID: 35198289 PMCID: PMC8854203 DOI: 10.7759/cureus.21377
Source DB: PubMed Journal: Cureus ISSN: 2168-8184
Socio-demographic and clinical characteristics of patients with sickle cell disease
Hb SS: hemoglobin SS; Hb SC: hemoglobin SC; Hb SB: hemoglobin S beta-thalassemia; AVN: avascular necrosis; SCD: sickle cell disease; n: number of participants
| Characteristic | n (%) | |
| Age | 18-29 | 124 (62.6) |
| 30-39 | 47 (23.7) | |
| 40-49 | 10 (5.1) | |
| 50-59 | 10 (5.1) | |
| >60 | 7 (3.5) | |
| Mean ± SD (years) | 28.4 ± 9.1 | |
| Sex | Male | 90 (45.5) |
| Female | 108 (54.5) | |
| Marital status | Single | 155(78.3) |
| Married | 41 (20.7) | |
| Widowed | 2 (105) | |
| Education level | Primary | 5(2.5) |
| Secondary | 41(20.7) | |
| Tertiary | 107(54.0) | |
| Post-graduate | 45(22.7) | |
| Occupation | Employed | 110 (55.5) |
| Student | 79(39.8) | |
| Unemployed | 9 (4.5) | |
| Monthly income | ≤$150 | 85 (42.9) |
| >$150-$300 | 33(16.7) | |
| >$300-$750 | 21 (10.6) | |
| >250,000 | 7(3.5) | |
| No response | 52 (26.2) | |
| Genotype | Hb SS | 178 (89.9) |
| Hb SC | 19 (9.5) | |
| Hb SB thalassemia | 1 (0.5) | |
| Co-morbidities and SCD associated complication | Chronic leg ulcer | 25 (12.6) |
| AVN of the hip | 21 (10.8) | |
| Hypertension | 9 (4.5) | |
| Stroke | 4 (2) | |
| End-stage renal disease | 2 (1.0) | |
| Diabetes | 1 (0.5) | |
| Seizure disorder | 1 (0.5) | |
| Asthma | 2 (1.0) | |
| No co-morbidities | 133 (67.1) | |
| Hemoglobin (mean ± SD) | 8.2 ± 1.3 g/dl | |
Sickle cell-related events over the previous year
n: number of participants
| Sickle cell-related events | n (%) | ||||
| None | 1-2 | 3-5 | 6-10 | >10 | |
| Moderate-to-severe bone pain crisis | 78 (39.4) | 65 (32.8) | 29 (14.6) | 13 (6.57) | 13 (6.56) |
| Priapism | 161 (81.3) | 16 (8.1) | 12 (6.1) | 3 (1.5) | 6 (3.0) |
| Acute chest syndrome | 153 (77.2) | 33(16.6) | 8 (4.0) | 4 (2.0) | 0 (0.0) |
| Blood transfusion | 127 (64.1) | 49 (24.7) | 18 (9.1) | 1 (0.5%) | 3 (1.5%) |
| Hospitalization | 54 (27.2) | 82 (41.4) | 36 (18.2) | 9 (4.5) | 17 (8.6) |
Figure 1Sickle cell-related events over the previous year (n=198)
n: number of participants
Health-related quality of life of sickle cell disease using the SF-36 questionnaire
SF-36: 35-item Short-Form Health Survey
| SF-36 domain | Median (interquartile range) |
| Role limitation due to emotional problems | 100.0 (33.3-100.0) |
| Emotional well being | 76.0 (64.0-88.0) |
| Social functioning | 75.0 (50.0-875.) |
| General health | 70.0 (55.0-80.0) |
| Physical functioning | 70.0 (50.0-85.0) |
| Pain | 67.5 (42.5-90.0) |
| Energy/fatigue | 60.0 (50.0-74.0) |
| Role limitation due to physical health | 50.0 (0.0-100.0) |
Bivariate analysis of HRQoL domains and socio-demographics of patients
RL: role limitation; HRQoL: health-related quality of life
| Median score of HRQoL domains | |||||||||
| Variable | General health | Pain | Social functioning | Emotional well being | Energy/fatigue | Physical functioning | RL physical health | RL emotional problem | |
| Age (years) | <30 | 70 | 57.5 | 75.0 | 76.0 | 60.0 | 75.0 | 50.0 | 100.0 |
| >30 | 70 | 77.5 | 75.0 | 80.0 | 60.0 | 70.0 | 75.0 | 100.0 | |
| P-value | 0.784 | (<0.0001) | (0.215) | (0.168) | (0.219) | (0.219) | (0.794) | (0.571) | |
| Gender | Male | 65.0 | 70.0 | 75.0 | 76.0 | 60.0 | 75.0 | 75.0 | 100.0 |
| Female | 70.0 | 65.0 | 72.0 | 76.0 | 60.0 | 70.0 | 50.0 | 100.0 | |
| P-value | (0.98) | (0.23) | (0.78) | (0.77) | (0.95) | (0.18) | (0.05) | (0.49) | |
| Marital status | Single | 70.0 | 67.5 | 75.0 | 76.0 | 60.0 | 75.0 | 50.0 | 100.0 |
| Married | 65.0 | 72.5 | 75.0 | 80.0 | 60.0 | 65.0 | 62.5 | 100.0 | |
| P-value | (0.39) | (0.15) | (0.35) | (0.23) | (0.95) | (0.18) | (0.80) | (0.86) | |
| Level of education | Tertiary | 70.0 | 67.5 | 62.5 | 76.0 | 60.0 | 70.0 | 50.0 | 100.0 |
| Non-tertiary | 70.0 | 67.5 | 75.0 | 76.0 | 60.0 | 70.0 | 50.0 | 100.0 | |
| P-value | (0.55) | (0.89) | (0.326) | (0.576) | (0.55) | (0.55) | (0.682) | (0.959) | |
| Employment status | Employed | 70.0 | 67.5 | 75.0 | 80.0 | 60.0 | 75.0 | 50.0 | 100.0 |
| Unemployed | 70.0 | 67.5 | 75.0 | 76.0 | 60.0 | 70.0 | 50.0 | 100.0 | |
| P-value | 0.26 | 0.39 | 0.59 | 0.65 | 0.62 | 0.67 | 0.85 | 0.96 | |
| Level of income | More than $150 | 66.0 | 60.0 | 62.5 | 76.0 | 60.0 | 65.0 | 33.3 | 100.0 |
| $150 or less | 70.0 | 77.5 | 75.0 | 84.0 | 65.0 | 76.0 | 75.0 | 100.0 | |
| P-value | 0.43 | 0.05 | 0.06 | 0.03 | 0.08 | 0.02 | 0.01 | 0.35 | |
Bivariate analysis of HRQoL domains and clinical characteristics of patients
RL: role limitation; HRQoL: health-related quality of life
| Median score of HRQoL domains | |||||||||
| Variable | General health | Pain | Social functioning | Emotional well being | Energy/fatigue | Physical functioning | RL physical health | RL emotional problem | |
| Major bone pain crisis | No | 70 | 88.0 | 87.5 | 80.0 | 65.0 | 80.0 | 75.0 | 80.0 |
| Yes | 65.0 | 55.0 | 62.5 | 72.0 | 55.0 | 65.0 | 25.0 | 72.0 | |
| P-value | 0.03 | <0.001 | <0.001 | 0.02 | 0.007 | 0.001 | <0.001 | 0.003 | |
| Priapism | No | 70.0 | 67.5 | 75.0 | 76.0 | 65.0 | 85.0 | 75.0 | 100.0 |
| Yes | 70.0 | 67.5 | 75.0 | 76.0 | 60.0 | 70.0 | 50.0 | 100.0 | |
| P-value | 0.45 | 0.853 | 0.298 | 0.74 | 0.34 | 0.003 | 0.33 | 0.25 | |
| Blood transfusion | Yes | 65.0 | 62.5 | 50.0 | 72.0 | 55.0 | 65.0 | 29.2 | 100.0 |
| No | 70.0 | 70.0 | 75.0 | 80.0 | 60.0 | 75.0 | 75.0 | 100.0 | |
| P-value | 0.152 | 0.04 | 0.008 | 0.02 | 0.462 | 0.01 | 0.06 | 0.125 | |
| Hospital admission | Yes | 65.0 | 57.5 | 62.5 | 72.0 | 55.0 | 60.0 | 25.0 | 66.0 |
| No | 70.0 | 77.5 | 62.5 | 76.0 | 60.0 | 70.0 | 50.0 | 100.0 | |
| P-value | 0.64 | 0.03 | 0.09 | 0.09 | 0.44 | 0.32 | 0.04 | 0.67 | |
| Acute chest syndrome | Yes | 65.0 | 57.5 | 62.5 | 72.0 | 60.0 | 70.0 | 25.0 | 66.7 |
| No | 75.0 | 67.5 | 75.0 | 80.0 | 60.0 | 70.0 | 75.0 | 100.0 | |
| P-value | 0.153 | 0.02 | 0.04 | 0.06 | 0.77 | 0.48 | 0.07 | 0.25 | |
| Co-morbidities/chronic complications | Yes | 70.0 | 68.7 | 68.7 | 80.0 | 65.0 | 75.0 | 75.0 | 100.0 |
| No | 70.0 | 67.5 | 75.0 | 76.0 | 60.0 | 70.0 | 50.0 | 100.0 | |
| P-value | 0.77 | 0.83 | 0.80 | 0.25 | 0.67 | 0.70 | 0.85 | 0.30 | |
Multivariate regression analysis of socio-demographic and clinical variables across the HRQoL domains
Data represent standardized beta coefficients and p-value in parenthesis.
RL: role limitation; HRQoL: health-related quality of life
| HRQoL domains | ||||||||
| Independent variables | Physical functioning | RL due to physical health | RL due to emotional problem | Energy | Emotional well being | Social functioning | Pain | General health |
| Age | -0.179 (0.120) | -0.143 (0.233) | -0.146 (0.276) | 0.06 (0.650) | 0.151 (0.241) | -0.001 (0.994) | 0.147 (0.237) | -0.166 (0.203) |
| Gender | 0.097 (0.390) | -0.071 (0.545) | 0.069 (0.597) | -0.081 (0.524) | 0.087 (0.493) | 0.059 (0.638) | -0.195 (0.113) | -0.349 (0.728) |
| Level of education | 0.070 (0.540) | -0.864 (0.391) | 0.088 (0.463) | -0.053 (0.643) | -0.015 (0.898) | 0.045 (0.694) | 0.069 (0.536) | 0.021 (0.854) |
| Level of income | 0.255 (0.025) | 0.298 (0.013) | 0.064 (0.622) | 0.140 (0.267) | 0.195 (0.122) | 0.054 (0.668) | 0.086 (0.480) | 0.021 (0.854) |
| Bone pain crisis | 0.066 (0.698) | -0.412 (0.022) | -0.291 (0.143) | -0.099 (0.606) | 0.021 (0.911) | -0.057 (0.765) | -0.228 (0.219) | -0.203 (0.296) |
| Acute chest syndrome | -0.077 (0.491) | 0.265 (0.026) | 0.003 (0.980) | 0.104 (0.407) | 0.006 (0.960) | 0.013 (0.915) | 0.136 (0.261) | 0.080 (0.532) |
| Priapism | 0.195 (0.108) | 0.132 (0.290) | 0.259 (0.07) | 0.149 (0.271) | 0.104 (0.438) | 0.221 (0.103) | 0.029 (0.821) | 0.085 (0.536) |
| Number of blood transfusion | -0.144 (0.274) | 0..139 (0.311) | 0.111 (0.465) | 0.117 (0.428) | 0.006 (0.967) | 0.069 (0.638) | 0.712 (0.479) | 0.187 (0.213) |
| Number of hospital admissions | -0.203 (0.237) | -0.168 (0.344) | 0.168 (0.396) | -0.256 (0.183) | -0.199 (0.297) | -0.298 (0.121) | -0.201 (0.278) | -0.268 (0.167) |
| R-square | 0.389 | 0.308 | 0.106 | 0.145 | 0.153 | 0.150 | 0.204 | 0.122 |
| F-test | 3.551 | 2.413 | 0.914 | 1.342 | 1.422 | 1.395 | 2.024 | 1.099 |
Questionnaire - 1
| 1. In general, would you say your health is: | |
| Excellent | 1 |
| Very good | 2 |
| Good | 3 |
| Fair | 4 |
| Poor | 5 |
| 2. Compared to one year ago | |
| Much better now than one year ago | 1 |
| Somewhat better now than one year ago | 2 |
| About the same | 3 |
| Somewhat worse now than one year ago | 4 |
| Much worse now than one year ago | 5 |
Questionnaire - 2
| 3. The following items are about activities you might do during a typical day. Does your health now limit you in these activities? If so, how much? | |||
| Yes, limited a lot (1) | Yes, limited a little (2) | No, not limited at all (3) | |
| a. Vigorous activities, such as running, lifting heavy objects, participating in strenuous sports | 1 | 2 | 3 |
| b. Moderate activities, such as moving a table, pushing a vacuum cleaner, bowling, or playing golf | 1 | 2 | 3 |
| c. Lifting or carrying groceries | 1 | 2 | 3 |
| d. Climbing several flights of stairs | 1 | 2 | 3 |
| e. Climbing one flight of stairs | 1 | 2 | 3 |
| f. Bending, kneeling, or stooping | 1 | 2 | 3 |
| g. Walking more than a mile | 1 | 2 | 3 |
| h. Walking several blocks | 1 | 2 | 3 |
| i. Walking one block | 1 | 2 | 3 |
| j. Bathing or dressing yourself | 1 | 2 | 3 |
Questionnaire - 3
| 4. During the past 4 weeks, have you had any of the following problems with your work or other regular daily activities as a result of your physical health? | ||
| Yes, limited a lot (1) | Yes, limited a little (2) | |
| a. Cut down the amount of time you spent on work or other activities | 1 | 2 |
| b. Accomplished less than you would like | 1 | 2 |
| c. Were limited in the kind of work or other activities | 1 | 2 |
| d. Had difficulty performing the work or other activities (for example, it took extra effort) | 1 | 2 |
Questionnaire - 4
| 5. During the past 4 weeks, have you had any of the following problems with your work or other regular daily activities as a result of any emotional problems (such as feeling depressed or anxious)? | ||
| Yes, limited a lot (1) | Yes, limited a little (2) | |
| a. Cut down the amount of time you spent on work or other activities | 1 | 2 |
| b. Accomplished less than you would like | 1 | 2 |
| c. Didn't do work or other activities as carefully as usual | 1 | 2 |
Questionnaire - 5
| 6. During the past 4 weeks, to what extent have your physical health or emotional problems interfered with your normal social activities with family, friends, neighbors, or groups? | |
| Not at all | 1 |
| Slightly | 2 |
| Moderately | 3 |
| Quite a bit | 4 |
| Extremely | 5 |
Questionnaire - 6
| 7. How much bodily pain have you had during the past 4 weeks? | |
| None | 1 |
| Very mild | 2 |
| Mild | 3 |
| Moderate | 4 |
| Severe | 5 |
| Very severe | |
| 8. During the past 4 weeks, how much did pain interfere with your normal work (including both work outside the home and housework)? | |
| Not at all | 1 |
| A little bit | 2 |
| Moderately | 3 |
| Quite a bit | 4 |
| Extremely | 5 |
Questionnaire - 7
| 9. How much of the time during the past 4 weeks … | ||||||
| All of the time | Most of the time | A good bit of the time | Some of the time | A little of the time | None of the time | |
| Did you feel full of prep? | 1 | 2 | 3 | 4 | 5 | 6 |
| Have been a very nervous person | 1 | 2 | 3 | 4 | 5 | 6 |
| Have you felt calm and peaceful? | 1 | 2 | 3 | 4 | 5 | 6 |
| Have you felt calm and peaceful moderate? | 1 | 2 | 3 | 4 | 5 | 6 |
| Did you have a lot of energy? | 1 | 2 | 3 | 4 | 5 | 6 |
| Have you felt downhearted and blue? | 1 | 2 | 3 | 4 | 5 | 6 |
| Did you feel worn out? | 1 | 2 | 3 | 4 | 5 | 6 |
| Have you been a happy person? | 1 | 2 | 3 | 4 | 5 | 6 |
| Did you feel tired | 1 | 2 | 3 | 4 | 5 | 6 |
Questionnaire - 8
| 10. During the past 4 weeks, how much of the time has your physical health or emotional problems interfered with your social activities (like visiting with friends, relatives, etc.)? | |
| All of the time | 1 |
| Most of the time | 2 |
| Some of the time | 3 |
| A little of the time | 4 |
| None of the time | 5 |
Questionnaire - 9
| 11. How TRUE or FALSE is each of the following statements for you? | |||||
| Definitely true | Mostly true | Don’t know | Mostly false | Definitely false | |
| I seem to get sick a little easier than other people | 1 | 2 | 3 | 4 | 5 |
| I am as healthy as anybody I know | 1 | 2 | 3 | 4 | 5 |
| I expect my health to get worse | 1 | 2 | 3 | 4 | 5 |
| My health is excellent | 1 | 2 | 3 | 4 | 5 |