Submitted:
15 August 2025
Posted:
18 August 2025
You are already at the latest version
Abstract
Keywords:
1. Background
2. Methodological Approach
Study Design and Scope
Study Setting and Data Collection
Questionnaire Design and Measures
Quantitative Items
Qualitative Items
Data Analysis
3. Results
Internal Consistency
Group Characteristics
Awareness

Quantitative Results
Knowledge
Attitudes/Beliefs

Practices
Qualitative Results
- Theme 1: Knowledge Gaps in SCA Management
- Theme 2: Expectations of Family Responsibility and Implicit Bias
- Theme 3: Systemic Barriers to Good Practice
4. Discussion
Strengths, Weaknesses and Future Directions
5. Conclusion
Funding
Authors’ contributions
Ethics approval and consent to participate
Availability of data and materials
Disclaimer
Acknowledgements
Competing interests
References
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| Characteristics | Number of Participants n (%) | ±Knowledge Score (x̄±s) | p Value |
|---|---|---|---|
| Total | 293 (100) | 14.79±4.95 | |
| Gender | 0.8704 | ||
| Male | 52 (17.7) | 14.75±6.06 | |
| Female | 241(82.3) | 14.80±4.70 | |
| Age group (years) | 0.571 | ||
| 18-25 | 69 (23.5) | 14.30±4.53 | |
| 26-35 | 63 (21.5) | 14.49±5.55 | |
| 36-45 | 91 (31.1) | 15.09±4.74 | |
| ≥46 | 70 (23.9) | 15.16±5.11 | |
| Marital status | 0.034* | ||
| Marrieda | 47 (16.0) | 16.45±4.50 | |
| Singleb | 232(79.2) | 14.61±4.94 | |
| Other | 14(4.8) | 12.37±5.29 | |
| Education | 0.030* | ||
| High school | 139 (49.4) | 13.96±5.02 | |
| University degree | 154 (52.6) | 15.54±4.78 | |
| Profession | 0.013* | ||
| Clinical psychologist | 20 (6.8) | 14.77±4.74 | |
| Doctora | 27 (9.2) | 17.87 ±5.05 | |
| Healthcare student | 16 (5.5) | 15.72±.5.39 | |
| Nurseab | 175 (59.7) | 14.68±4.87 | |
| Other healthcare professional | 12 (4.1) | 13.08±4.59 | |
| Technician therapistac | 43 (14.7) | 13.42±4.58 | |
| Position | 0.606 | ||
| No | 265 (90.4) | 14.81±4.92 | |
| Yes | 28 (9.6) | 14.58±5.29 | |
| Facility | 0.598 | ||
| Healthcare centre | 169 (57.8) | 14.63±5.04 | |
| Hospital unit | 124 (42.3) | 15.01±4.84 | |
| Sector | 0.268 | ||
| Public | 285 (97.1) | 14.85±4.96 | |
| Still in training | 8 (2.9) | 12.77±3.34 | |
| Experience | 0.647 | ||
| <5 | 90 (30.7) | 14.95±5.18 | |
| >10 | 119 (40.6) | 15.02±4.94 | |
| 5-10. | 54 (18.4) | 14.14±5.57 | |
| Still in training | 31 (10.6) | 14.64±4.90 | |
| Residence | 0.554 | ||
| Belize | 19 (6.5) | 14.05±5.04 | |
| Buco-Zau | 19 (6.5) | 13.10±7.16 | |
| Cabinda | 202 (68.9) | 15.15±4.61 | |
| Cacongo | 53 (18.1) | 14.30±5.17 | |
| Language | 0.793 | ||
| Portuguese | 278 (94.9) | 14.83±4.90 | |
| Other | 15 (5.1) | 14.00±5.92 | |
| Regional language | 0.843 | ||
| Iwoyo | 102 (34.8) | 14.97±4.89 | |
| Kikongo | 55 (18.8) | 14.95±5.27 | |
| Kilindgi | 52 (17.7) | 14.46±4.80 | |
| Kiyombe | 84 (28.7) | 14.72±4.87 | |
| Religion | <0.001* | ||
| Catholica | 127 (43.3) | 13.61±5.06 | |
| Protestantb | 153 (52.2) | 15.66±4.68 | |
| Other | 14 (4.8) | 16.24±4.82 |
| Response N (%) | ||||
|---|---|---|---|---|
| Questions |
Yes |
No |
Don’t know |
Missing |
| Awareness | ||||
| Have you ever heard about SCA? | 286 (97.6) | 2 (2.4) | 0 | 5 (1.7) |
| General Knowledge | ||||
| *Sickle cell trait (SCT) carriers can experience painful crises. (FALSE) |
203 (69.3) |
43 (14.7) |
18 (6.1) |
29 (9.9) |
| SCA is an important health problem in Angola (TRUE) | 195 (66.6) | 44 (15.0) | 38 (13.0) | 16 (5.5) |
| SCA can be diagnosed in an unborn baby by testing the baby while in the womb (TRUE) |
95 (32.4) |
137 (46.8) |
44 (15.0) |
17 (5.8) |
| Children who carry the sickle cell trait (SCT) are less likely to die of malaria compared to those who do not have the trait (TRUE) |
85 (29.0) |
120 (41.0) |
57 (19.5) |
31 (10.6) |
| SCA only occurs in African individuals or descendants (FALSE) |
50 (17.1) |
168 (57.3) |
49 (16.7) |
26 (8.9) |
| Manifestations and complications | ||||
| Glaucoma is a manifestation and complication of SCA(FALSE) | 271 (92.5) | 7 (2.4) | 0 | 15 (5.1) |
| *Low blood level is a manifestation and complication (TRUE) | 270 (92.2) | 6 (2.0) | 0 | 17 (5.8) |
| Bone pains is a manifestation and complication of SCA(TRUE) |
240 (81.9) |
7 (2.4) |
12 (4.1) |
34 (11.6) |
| Swollen hand and foot are a manifestation and complication of SCA (TRUE) |
235 (80.2) |
16 (5.5) |
8 (2.7) |
34 (11.6) |
| Chronic diarrhoea is a manifestation and complication of SCA (FALSE) |
221 (75.4) |
19 (6.5) |
9 (3.1) |
44 (15.0) |
| Arthritis is a manifestation and complication of SCA (TRUE) | 147 (50.2) | 43 (14.7) | 35(11.9) | 68 (23.2) |
| Chronic leg ulcer is a manifestation and complication of SCA (TRUE) |
95 (32.4) |
76 (25.9) |
51 (17.4) |
71 (24.2) |
| Stroke is a manifestation and complication of SCA (TRUE) | 81 (27.6) | 98 (33.4) | 35(11.9) | 79 (27.0) |
| Heart disease is a manifestation and complication of SCA (TRUE) |
78 (26.6) |
70 (23.9) |
53 (18.1) |
92 (31.4) |
| Kidney failure is a manifestation and complication of SCA (TRUE) |
77 (26.3) |
73 (24.9) |
56(19.1) |
87 (29.7) |
| Gallstones are a manifestation and complication of SCA (TRUE) |
54 (18.4) |
72 (24.6) |
87 (29.7) |
80 (27.3) |
| Delayed growth and development are a manifestation and complication of SCA (TRUE) |
42 (14.3) |
75 (25.6) |
67(22.9) |
109 (37.2) |
| Lung cancer is a manifestation and complication of SCA (FALSE) |
40 (13.7) |
91 (31.1) |
78 (26.6) |
84 (28.7) |
| Blindness is a manifestation and complication of SCA (TRUE) |
38 (13.0) |
112 (38.2) |
50 (17.1) |
93 (31.7) |
| Control and prevention of complications | ||||
| Genetic counselling is a method for control and prevention of SCA (TRUE) |
247 (84.3) |
7 (2.4) |
0 |
39 (13.3) |
| Premarital genotype testing is a method for control and prevention of SCA (TRUE) |
229 (78.2) |
7 (2.4) |
12 (4.1) |
45 (15.4) |
| Pneumococcal vaccination is a method for control and prevention of SCA (TRUE) |
195 (66.6) |
20 (6.8) |
18 (6.1) |
60 (20.5) |
| Newborn screening is a method for control and prevention of SCA (TRUE) |
166 (56.7) |
34 (11.6) |
14 (4.8) |
79 (27.0) |
| Frequent blood transfusion is a method for control and prevention of SCA (TRUE) |
160 (54.6) |
67 (22.9) |
7 (2.4) |
59 (20.1) |
| Early diagnosis and treatment are a method for control and prevention of SCA (TRUE) |
115 (39.2) |
111 (37.9) |
8 (2.7) |
59 (20.1) |
| Use of mosquito net is a method for control and prevention of SCA (FALSE) |
79 (27.0) |
71 (24.2) |
58 (19.8) |
85 (29.0) |
| Hydroxyl therapy is a method for control and prevention of SCA. (TRUE) |
115 (39.2) |
32 (10.9) |
78 (26.6) |
68 (23.2) |
| Management | ||||
| Children with SCA should always remain hydrated. (TRUE) | 168 (57.3) | 31 (10.6) | 48 (16.4) | 46 (15.7) |
| A child with a clinical suspicion of stroke should undergo immediate blood transfusion. (TRUE) |
158 (53.9) |
26 (8.9) |
52 (17.7) |
57 (19.5) |
|
Children with SCA cannot have an iron-rich diet. (FALSE) |
152 (51.9) |
72 (24.6) |
12 (4.1) |
57 (19.5) |
| Management of acute chest syndrome includes hydration, bronchodilators, antibiotics, and oxygen therapy. (TRUE) |
140 (47.8) |
22 (7.5) |
56 (19.1) |
75 (25.6) |
| Fever is not an emergency in children with SCA. (FALSE) | 111 (37.9) | 112 (38.2) | 10 (3.4) | 60 (20.5) |
| Children with sudden increase in abdominal volume do not need to be evaluated or treated urgently. (FALSE) |
105 (35.8) |
100 (34.1) |
21 (7.2) |
67 (22.9) |
| Hydroxyurea (HU) is an oral medication proven to improve the quality of life of SCA patients. (TRUE) |
95 (32.4) |
8 (2.7) |
7 (2.4) |
183 (62.5) |
| Moderate anaemia or pain episode without any other complication are requisites for an emergency blood therapy in SCA. (FALSE) |
77 (26.3) |
131 (44.7) |
25 (8.5) |
60 (20.5) |
|
Attitudinal statements |
Response N (%) |
|||
|---|---|---|---|---|
|
Yes |
No |
Don’t know |
Missing |
|
| Have you ever checked your haemoglobin genotype status? |
79 (27.0) |
184 (62.8) |
0 |
30 (10.2) |
| Couples at risk of having a child with SCA should be dissuaded from marriage by law |
143 (48.8) |
70 (23.9) |
43 (14.7) |
37 (12.6) |
| Pregnancy termination should be encouraged if a baby is found to have SCA during pregnancy? |
76 (25.9) |
136 (46.4) |
42 (14.3) |
39 (13.3) |
| Having a child with SCA is a punishment from God for those who do not believe in Him |
6 (2.0) |
244 (83.3) |
17 (5.8) |
26 (8.9) |
| Subjecting oneself to genetic counselling before marriage shows lack of faith in God |
17 (5.8) |
230 (78.5) |
19 (6.5) |
27 (9.2) |
| Practice Statement | Response N (%) | ||||||
|---|---|---|---|---|---|---|---|
|
Strongly agree |
Agree |
Somewhat agree |
Disagree |
Strongly disagree |
Missing |
||
| Leaders and/or senior managers of healthcare facilities support and openly promote SCA patient care |
39 (13.3) |
69 (23.5) |
68 (23.2) |
35 (11.9) |
22 (7.5) |
60 (20.5) |
|
| SCA patient care flyers or posters are displayed at each point of care in all healthcare facilities, as reminders. | 19 (6.5) | 70 (23.9) | 63 (21.5) |
57 (19.5) |
29 (9.9) |
55 (18.8) |
|
| The health-care facilities make SCA patient care always possible at each point of care | 42 (14.3) | 89 (30.4) | 50 (17.1) | 31 (10.6) | 23 (7.8) | 58 (19.8) | |
| Clear and simple instructions for SCA patient care are made visible for every healthcare provider |
15 (5.1) |
56 (19.1) |
49 (16.7) |
66 (22.5) |
50 (17.1) |
57 (19.5) |
|
| Would you support the establishment of a standalone clinic for SCA patients in Cabinda? * |
224 (76.5) |
0 | 0 | 0 | 0 |
69 (23.5) |
|
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