586051
research-article2015
CPJXXX10.1177/0009922815586051Clinical PediatricsMalheiros et al
Article
Hip Dysfunction and Quality of Life in Patients With Sickle Cell Disease
Clinical Pediatrics 2015, Vol. 54(14) 1354–1358 © The Author(s) 2015 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922815586051 cpj.sagepub.com
Cristiane Dias Malheiros, PT1, Luanne Lisle, MD1, Marilda Castelar, PS1, PhD1, Katia Nunes Sá, PT, PhD1, and Marcos Almeida Matos, MD, PhD1
Abstract Objective. To evaluate the influence of hip dysfunction on quality of life of pediatric patients with sickle cell disease (SCD). Methods. Clinical demographic, Charnley hip score, Barthel scale and Pediatric Quality of Life Inventory (PedsQL 4.0) questionnaires were used. Results. Seventeen patients were placed into the group “with hip dysfunction,” and 54 into the group “without hip dysfunction.” The group “with hip dysfunction” had a higher average age (P = .026), weight (P = .029), and height (P = .019) than the group “without hip dysfunction.” There was also a higher prevalence of hip trauma (P = .05) and pain (P = .00). The study demonstrated that hip dysfunction negatively influenced quality of life in the physical activity (P = .11) and psychosocial (P = .003) domains. Conclusion. Hip dysfunction and previous hip trauma negatively influenced quality of life, especially in the physical activity and psychosocial domains. Keywords hip, quality of life, sickle cell anemia
Introduction Hip dysfunction can be defined as partial or total loss of the function of this joint associated with decreased range of motion or inability to walk.1-3 Hip dysfunction is a common condition of disability in patients suffering from sickle cell disease (SCD).4,5 Hip dysfunction does not have any specific treatment and therefore produces continuous and progressive dysfunction of the hip joint throughout the patient’s life. Besides, it can be an important cause of an individual’s limitation and loss in quality of life.2,5-8 Hip dysfunction may be caused by several musculoskeletal manifestations of SCD, including stroke, pain crisis, bone infarction, vasculo-oclusive episodes, hip inflammation, joint stiffness, and infection.9 However, osteonecrosis of the femoral head (ONFH) is the most frequent disabling complication affecting the hip of those patients2,4,5. The prevalence of ONFH ranges from 10% to 30%4 in adults and 8.7% in the pediatric population.2,4,5 It is also estimated that incapacitating osteoarthritis overlaps up to 70% of adult cases.4 It is well known that all chronic and acute complications of SCD could be expected to have a negative impact on quality of life of the patients, especially for children and adolescent.7,8,10 All the studies focusing on this subject revealed the consistently poor quality of life in children with SCD. Therefore, the overlaps of hip dysfunction
surely represents a devastating complication leading to physical, social, and emotional problems adding even more suffering to those children. Birrell et al1 demonstrate that hip pain has the greatest influence on physical aspects of quality of life, but they studied patients with osteoarthritis. The functional limitations of the hip, mainly resulting from ONFH, are constantly cited in cases of patients with SCD as well without any reference to subjective evaluatio.n2,4,5 Despite the fact that functional deficits resulting from hip dysfunction seem to be very relevant to quality of life in patients with SCD, we were unable to find studies approaching specifically this topic. Taking into account the lack of available information, the objective of this study is to evaluate the influence of hip dysfunction on quality of life of pediatric patients with SCD.
Methods An analytical cross-sectional study was performed to evaluate the influence of hip dysfunction on quality of 1
Bahiana School of Medicine and Public Health, Salvador, Brazil
Corresponding Author: Marcos Almeida Matos, Bahiana School of Medicine and Public Health, Av. D. João VI, 274, Brotas, Salvador, Bahia, 40290-000 Brazil. Email: [email protected]
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Malheiros et al life of patients with SCD younger than 21 years. Subjects were recruited from among those regularly seen at the Center for Hemotherapy and Hematology of the State of Bahia, Brazil (HEMOBA) between February and November of 2011. This study was approved by the Ethics Committee of the Bahian School of Medicine and Public Health (protocol number 111/2010), and all selected subjects and their guardians were duly informed of the risks and benefits of the research. Those who participated in the study signed an informed consent form. The sample size was calculated to detect a difference of 10%, assuming a prevalence of hip dysfunction in 20%2.4.5 of the target population and an alpha error of 0.05. The sample size was estimated at 64 subjects, plus 10% to compensate for possible losses, resulting in a total of 71 patients. All patients with SCD between the ages of 8 and 21 years were included in the study. The following patients were excluded: those who had already undergone hip surgery; with motor or cognitive changes arising from neurological system complications; and with a history of fractures of the proximal femur; with associated diseases of the musculoskeletal system (rheumatic fever, lupus, and systemic juvenile rheumatoid arthritis) and osteoarticular infections in other joints. After the subjects were selected to participate, clinical and demographic data were collected from each of them, as well as a hip joint evaluation based on the Charnley hip score.3 The Brazilian-Portuguese version of the Barthel scale, which assesses overall functional capacity,11,12 and the Pediatric Quality of Life Inventory (PedsQL 4.0) were also used.13,14 The clinical-demographic questionnaire consisted of personal and clinical data, including patient history regarding number of hospitalizations, infections, and the presence of ulcers, reported smoke exposure, family history (history of vascular disease), and laboratory data. The Charnley hip score3 evaluates 3 important aspects related to hip function. They are pain, gait, and range of motion. Each aspect is scored on a scale of 1 to 6, in which 6 represents the best score. The patients with hips that were considered normal, or without dysfunction, received the maximum score of 18. Patients were divided into 2 groups according to the Charnley hip score. Patients with scores less than 18 were considered to have hip dysfunction and were placed in the group “with dysfunction” while patients with scores of 18 were placed in the group “without dysfunction.” Although this score was designed for adults, it has also been efficiently tested in the pediatric population.2 The 2 groups (with and without dysfunction) were also evaluated based on Barthel’s scale,11,12 which
assesses overall functional capacity of the individual, as well as the PedsQL 4.0.13,14 Barthel’s scale is widely used in various specialties of health care, such as geriatrics, neurology, and orthopedics. It evaluates 10 general aspects of activities of daily living and its score ranges from 0 to 100.13 The PedsQL 4.0 questionnaire13,14 was developed specifically for children in defined age ranges, and it was used for children in age intervals of 8 to 12 and 13 to 18 years. Patient data were tabulated into distribution tables by frequency in the case of categorical variables or into mean and standard deviation in the case of continuous variables. For comparison between the groups with and without hip dysfunction, the chi-square test was used for discrete variables and the Student’s t test for continuous variables. To evaluate confounding variables, multivariate analysis was used when appropriate.
Results The study consisted of a total of 71 patients, 17 of whom (23.9%) had hip dysfunction and made up the “with dysfunction” group and 54 (76.1%) of whom did not have hip dysfunction and made up “without dysfunction” group. The characteristics of the sample are presented in Table 1. In the analysis of quality of life (from the perspective of the patients) and values of the Barthel scale, we can observe that patients with hip dysfunction have poorer quality of life and there are also significant association between hip dysfunction and the physical activity, social, and scholar domains (2 of the psychosocial domains) referred to by the affected individuals themselves (Table 2). The multivariate analysis of factors that could be clinically associated to a loss in quality of life in this population revealed that hip dysfunction (activity domain; P = .01), lower limb ulcer (emotional domain; P = .03), previous trauma (activity domain; P = .02), and hip pain (scholar domain; P = .04) are the only parameters consistently associated with quality of life decrease from the perspective of the affected individuals.
Discussion The current study showed that 23.9% of pediatric patients with SCD have hip dysfunction. The dysfunction was more prevalent among older patients and was associated with hip pain and history of previous trauma. Patients with hip dysfunction were show to have a lower quality of life in all the evaluated major domains (psychosocial and activities). The mean score was 56.57 in the group with dysfunction and 70.70 in the group
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Clinical Pediatrics 54(14)
Table 1. General Characteristics of the Patient Sample and General Analysis Between the Groups. Total n (%) or mean (±SD) Sample Gender Male Female Age, years Weight, kg Height, m Jaundicea Pallora Hepatitis Lower limb ulcersa Hospitalized in past year No. of hospitalizations in past year Showed infections in past year No. Infections in past year Exposure to secondhand smoke Vascular abnormalities Previous trauma Hip pain Chronic use of medication Blood transfusion
71 (100.0) 33 (46.5) 38 (53.5) 12.19 (±3.20) 42.77 (±9.78) 1.54 (±0.12) 19 (35.2) 23 (43.4) 3 (4.2) 4 (5.9) 34 (47.9) 0.88 (±1.32) 22 (31.0) 0.29 (±0.59) 14 (20.0) 8 (11.3) 7 (9.9) 10 (14.1) 71 (100.0) 21 (30.0)
With Dysfunction 17 (23.9) 5 (29.4) 12 (70.6) 13.71 (±3.21) 42.77 (±9.77) 1.53 (±0.12) 5 (35.7) 7 (53.8) 0 (0.0) 2 (12.5) 9 (52.9) 0.80 (±1.27) 4 (23.5) 0.33 (±0.62) 6 (35.3) 3 (17.6) 4 (23.5) 10 (58.8) 17 (100.0) 7 (41.2)
Without Dysfunction
P Value
54 (76.1)
28 (51.9) 26 (48.1) 11.72 (±3.12) 35.27(±12.26) 1.43 (±0.16) 14 (35.0) 16 (40.0) 3 (5.6) 2 (3.8) 25 (46.3) 0.76 (±1.13) 18 (33.3) 0.43 (±0.68) 8 (15.1) 5 (9.3) 3 (5.6) 0 (0.0) 54 (100.0) 14 (26.4)
.106 .026 .029 .019 .962 .382 1.000 .233 .632 .715 .556 .479 .070 .387 .052 .000 — .248
a Jaundice n = 54, with dysfunction = 14, without dysfunction = 40; pallor n = 53, with dysfunction = 13, without dysfunction = 40; ulcers n = 68, with dysfunction = 16, without dysfunction = 52.
Table 2. Analysis of Quality of Life Between the Groups. Total Score, Mean (±SD) Mean PedsQL child (activity and psychosocial) PedsQL child: activities PedsQL child: psychosocial PedsQL child emotional PedsQL child social PedsQL child scholar Barthel scale
With Dysfunction, Mean (±SD)
Without Dysfunction, Mean (±SD)
P Value
67.40 (±17.07)
56.57 (±13.47)
70.70 (±16.92)
.003
68.60 (±18.73) 66.21 (±18.72) 62.90 (±21.74) 72.90 (±23.69) 60.97 (±21.88) 98.24 (±3.51)
58.78 (±14.36) 54.37 (±15.58) 54.70 (±19.56) 58.82 (±21.25) 47.94 (±23.91) 98.24 (±3.51)
71.68 (±18.72) 69.71 (±18.32) 65.25 (±22.08) 77.01 (±22.99) 64.90 (±19.91) 98.15 (±3.26)
.011 .003 .082 .005 .005 .925
Abbreviation: PedsQL, Pediatric Quality of Life Inventory. a Peds child n = 71, with dysfunction = 17, without dysfunction = 54.
without dysfunction, which represents a decrease of about 20% in the quality of life. Hip dysfunction was demonstrated to be a relevant factor and in the multivariate analysis it was independently associated with poorer quality of life within the activity domain. Previous trauma, hip pain, and lower limb ulcers were also independent predictors of quality of life in the activities, scholar, and emotional domains, respectively. Conversely, the Barthel scale did not showed any difference in the quality of life of children in each of the 2 groups (with and without dysfunction).
Our findings suggest that hip dysfunction interferes with almost all domains of the patient’s life with the exception of the emotional domain. Therefore, hip dysfunction could worsen even more the quality of life of patients suffering from a chronic and severe disease such as SCD. Hip dysfunction in this population can be caused mainly by ONFH, which has an overall prevalence of 11.1% in pediatric patients with SCD.2 ONFH is closely related to age, affecting 3% of individuals younger than 15 years and 50% of those older than 35 years.2,4 Hip
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Malheiros et al dysfunction accompanies the distribution of ONFH, though the prevalence of hip dysfunction in the current study was surprisingly high when compared with similar reports. This finding may be explained by the fact that hip dysfunction is a complication of SCD that includes not only ONFH but also other factors, such as painful crisis, sequel of infection, joint stiffness, or decreased range of motion, without a necessary diagnosis of osteonecrosis. Sickle cell disease is a severe chronic diseases and the need for continuous treatment over an extended period of time is a factor which directly influence quality of life.6,8 Roberti et al15 emphasize that SCD impairs the quality of life of affected patients. In addition to this, significant correlation was confirmed with social discrimination, the so-called “SCD stigma,” due to the illness itself, educational level, and taking into account that SCD mainly affects poor and black population. The results found in implementing the Barthel scale11,12 demonstrate that hip dysfunction did not alter the general functional capacity of the subjects (P = .93). The items related to independence that showed results below the norm were urinary and/or fecal continence (occasional accidence), consistent with the young age or immaturity of some of the subjects. The patients with hip dysfunction scored values close to 18 on the Charnley hip score.3 The majority of patients with dysfunction reached values around 15 and 16 on the Charnley scale; the lowest score was 10 (only 1 patient). These data show that there was only a discrete change in hip function in the studied patients. As a general function evaluation, this change could not be detected by the Barthel scale, but was significantly perceived by the patients, especially in the physical function as demonstrated by the decrease in the quality of life showed as an independent association between hip dysfunction and the activities domain of PedsQL 4.0. In the multivariate analysis, the worse quality of life reported by the patients was also consistently associated with hip dysfunction, hip pain, and previous trauma to the hip. The worse quality of life perceived by the patients with hip dysfunction is related to the presence of pain as well, which occurred in 58.8% of the cases. Some studies reaffirm this fact by proving that pain treatment leads to an improvement of quality of life.16,17 It can thus be inferred that individuals who suffer from chronic pain who are able to independently perform activities of daily living cannot show others (including parents and family) the functional and psychosocial impact that a single, isolated factor can have on their quality of life despite being a consistent and relevant aspect of their suffering. Note that because of the young age of the population in this study, the subjects have milder symptoms when
compared with the adult population, in which there are reports of more severe pain, restricted range of motion, and hence more pronounced changes in gait.18 The Barthel scale comparison between 2 groups in the adult population might have a more significant difference and the deterioration of quality of life might be more noticeable by the accompanying evaluators (doctors and parents). It was expected that major complications such as infections, number of hospitalizations, the presence of ulcers, and frequent transfusions would be determining factors in quality of life, as cited in the literature.6-8 However, the lone factor that contributed most to the deterioration in quality of life of the patients was hip dysfunction. To reinforce the extent to which hip dysfunction interferes with quality of life,19 even mentions that the self-evaluation of quality of life in patients with SCD awaiting hip or knee arthroplasty was worse in the group awaiting hip arthroplasty. The result of this research is understandable, since the importance of the hip is understood in evaluating the biomechanics of the lower limbs. Because of its location and important role in the majority of activities of daily living performed by the lower limbs (such as sedestation, walking, and ascending and descending stairways), the hip may have a greater influence on quality of life than the knee. Sickle cell disease is a chronic disease that causes deterioration in the health of patients with it, and that requires frequent interventions and medical treatments. These treatments may or may not be invasive, and the patients are accustomed to them. Hip dysfunction causes functional loss and psychosocial differentiation which are added on to the routine suffering experienced by the patients,6-8 raising even more awareness to the perception of a worse quality of life by these patients in comparison with sickle cell patients without hip dysfunction. The current study has made a significant contribution to the literature by demonstrating for the first time that hip dysfunction is common in patients with SCD (reaching rates of 23.9%) and that it is associated to a decrease of 20% in the overall quality of life. It was also the variable that most negatively influenced the quality of life according to the patients’ own perception, in both the psychosocial and physical activity domains. Author Contributions CDM, MAM - made substantial contribution to the concept and design, acquisition of data, analysis and interpretation of data; drafted the article and revised it critically for important intellectual content; approved the version to be published. LL, MC, KNS - drafted the article and revised it critically for important intellectual content; approved the version to be published.
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Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Birrell F, Croft P, Cooper C, Hosie G, Macfarlane G, Silman A. Health impact of pain in the hip region with and without radiographic evidence of osteoarthritis: a study of new attenders to primary care. Ann Rheum Dis. 2000;59:857-863. 2. Matos MA, dos Santos Silva LL, Brito Fernandes R, Dias Malheiros C, Pinto da Silva BV. Avascular necrosis of the femoral head in sickle cell disease patients. Ortop Traumatol Rehabil. 2012;14:155-160. 3. Charnley J. The Long-term results of low-friction arthroplasty of the hip performed as a primary intervention. J Bone Joint Surg Br. 1972;54:61-76. 4. Milner PF, Kraus AP, Sebes JI, et al. Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991;325:1476-1481. 5. Henrigou P, Bachir D, Galacteros F. The natural history of symptomatic osteonecrosis in adults with sickle cell disease. J Bone Joint Surg Am. 2003;85:500-504. 6. Ohara DG, Ruas G, Castro SS, Martins PR, Walsh IA. Musculoskeletal pain, profile and quality of life of individuals with sickle cell disease. Rev Bras Fisioter. 2012;16:431-438. 7. Dale JC, Cochran CJ, Roy L, Jernigan E, Buchanan GR. Health-related quality of life in children and adolescents with sickle cell disease. J Pediatr Health Care. 2011;25:208-215. 8. Menezes AS, Len CA, Hilário MO, Terreri MT, Braga JA. Quality of life in patients with sickle cell disease. Rev Paul Pediatr. 2013;31:24-29.
9. Onuba O. Bone disorders in sickle-cell disease. Int Orthop. 1993;17:397-399. 10. Pereira SAS, Cardoso CS, Brener S, Proietti ABFC. Sickle cell disease and quality of life: a study on the subjective perception of patients from the Fundação Hemominas, Minas Gerais, Brazil. Rev Bras Hematol Hemoter. 2008;30:411-416. 11. Guimarães RB, Guimarães RB. Validação e adaptação cultural para a língua portuguesa de escalas de avaliação funcional em doenças cerebrovasculares: uma tentativa de padronização e melhora da qualidade de vida. Rev Bras Neurol. 2004;40(3):5-13. 12. Mahoney FI, Barthel DW. Functional evaluation: the Barthel Index. Md State Med J. 1965;14:56-61. 13. Klatchoian DA, Len CA, Terreri MT, et al. Quality of life of children and adolescents from São Paulo: reliability and validity of the Brazilian version of the Pediatric Quality of Life Inventory® version 4.0 generic core scales. J Pediatr (Rio J). 2008;84:308-315. 14. Varni JW, Seid M, Rode CA. The PedsQL: measurement model for the pediatric quality of life inventory. Med Care. 1999;37:126-139. 15. Roberti MRF, Moreira CLNSO, Tavares RS, Filho HMB, Silva AG, Maia CHG. Evaluation of quality of life of patients with sickle cell disease in a General Hospital of Goiás, Brazil. Rev Bras Hematol Hemoter. 2010;32:449-454. 16. Al-Taki MM, Masri BA, Duncan CP, Garbuz DS. Quality of life following proximal femoral replacement using a modular system in revision THA. Clin Orthop Relat Res. 2012;469:470-475. 17. Tinti G, Somera JR, Valente FM, Domingod CRB. Benefits of kinesiotherapy and aquatic rehabilitation on sickle cell anemia. A case report. Gen Mol Res. 2010;9:360-364. 18. Dampier C, LeBeau P, Rhee S, et al. Health-related quality of life in adults with sickle cell disease (SCD): a report from the comprehensive sickle cell centers clinical trial consortium. Am J Hematol. 2011;86:203-205. 19. Ackerman IN, Bennell KL, Osbone RH. Decline in health-related quality of life reported by more than half of those waiting for joint replacement surgery: a prospective cohort study. BMC Musculoskelet Disord. 2011;12:108.
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research-article2015
CPJXXX10.1177/0009922815586051Clinical PediatricsMalheiros et al
Article
Hip Dysfunction and Quality of Life in Patients With Sickle Cell Disease
Clinical Pediatrics 2015, Vol. 54(14) 1354–1358 © The Author(s) 2015 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922815586051 cpj.sagepub.com
Cristiane Dias Malheiros, PT1, Luanne Lisle, MD1, Marilda Castelar, PS1, PhD1, Katia Nunes Sá, PT, PhD1, and Marcos Almeida Matos, MD, PhD1
Abstract Objective. To evaluate the influence of hip dysfunction on quality of life of pediatric patients with sickle cell disease (SCD). Methods. Clinical demographic, Charnley hip score, Barthel scale and Pediatric Quality of Life Inventory (PedsQL 4.0) questionnaires were used. Results. Seventeen patients were placed into the group “with hip dysfunction,” and 54 into the group “without hip dysfunction.” The group “with hip dysfunction” had a higher average age (P = .026), weight (P = .029), and height (P = .019) than the group “without hip dysfunction.” There was also a higher prevalence of hip trauma (P = .05) and pain (P = .00). The study demonstrated that hip dysfunction negatively influenced quality of life in the physical activity (P = .11) and psychosocial (P = .003) domains. Conclusion. Hip dysfunction and previous hip trauma negatively influenced quality of life, especially in the physical activity and psychosocial domains. Keywords hip, quality of life, sickle cell anemia
Introduction Hip dysfunction can be defined as partial or total loss of the function of this joint associated with decreased range of motion or inability to walk.1-3 Hip dysfunction is a common condition of disability in patients suffering from sickle cell disease (SCD).4,5 Hip dysfunction does not have any specific treatment and therefore produces continuous and progressive dysfunction of the hip joint throughout the patient’s life. Besides, it can be an important cause of an individual’s limitation and loss in quality of life.2,5-8 Hip dysfunction may be caused by several musculoskeletal manifestations of SCD, including stroke, pain crisis, bone infarction, vasculo-oclusive episodes, hip inflammation, joint stiffness, and infection.9 However, osteonecrosis of the femoral head (ONFH) is the most frequent disabling complication affecting the hip of those patients2,4,5. The prevalence of ONFH ranges from 10% to 30%4 in adults and 8.7% in the pediatric population.2,4,5 It is also estimated that incapacitating osteoarthritis overlaps up to 70% of adult cases.4 It is well known that all chronic and acute complications of SCD could be expected to have a negative impact on quality of life of the patients, especially for children and adolescent.7,8,10 All the studies focusing on this subject revealed the consistently poor quality of life in children with SCD. Therefore, the overlaps of hip dysfunction
surely represents a devastating complication leading to physical, social, and emotional problems adding even more suffering to those children. Birrell et al1 demonstrate that hip pain has the greatest influence on physical aspects of quality of life, but they studied patients with osteoarthritis. The functional limitations of the hip, mainly resulting from ONFH, are constantly cited in cases of patients with SCD as well without any reference to subjective evaluatio.n2,4,5 Despite the fact that functional deficits resulting from hip dysfunction seem to be very relevant to quality of life in patients with SCD, we were unable to find studies approaching specifically this topic. Taking into account the lack of available information, the objective of this study is to evaluate the influence of hip dysfunction on quality of life of pediatric patients with SCD.
Methods An analytical cross-sectional study was performed to evaluate the influence of hip dysfunction on quality of 1
Bahiana School of Medicine and Public Health, Salvador, Brazil
Corresponding Author: Marcos Almeida Matos, Bahiana School of Medicine and Public Health, Av. D. João VI, 274, Brotas, Salvador, Bahia, 40290-000 Brazil. Email: [email protected]
Downloaded from cpj.sagepub.com at Kungl Tekniska Hogskolan / Royal Institute of Technology on November 15, 2015
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Malheiros et al life of patients with SCD younger than 21 years. Subjects were recruited from among those regularly seen at the Center for Hemotherapy and Hematology of the State of Bahia, Brazil (HEMOBA) between February and November of 2011. This study was approved by the Ethics Committee of the Bahian School of Medicine and Public Health (protocol number 111/2010), and all selected subjects and their guardians were duly informed of the risks and benefits of the research. Those who participated in the study signed an informed consent form. The sample size was calculated to detect a difference of 10%, assuming a prevalence of hip dysfunction in 20%2.4.5 of the target population and an alpha error of 0.05. The sample size was estimated at 64 subjects, plus 10% to compensate for possible losses, resulting in a total of 71 patients. All patients with SCD between the ages of 8 and 21 years were included in the study. The following patients were excluded: those who had already undergone hip surgery; with motor or cognitive changes arising from neurological system complications; and with a history of fractures of the proximal femur; with associated diseases of the musculoskeletal system (rheumatic fever, lupus, and systemic juvenile rheumatoid arthritis) and osteoarticular infections in other joints. After the subjects were selected to participate, clinical and demographic data were collected from each of them, as well as a hip joint evaluation based on the Charnley hip score.3 The Brazilian-Portuguese version of the Barthel scale, which assesses overall functional capacity,11,12 and the Pediatric Quality of Life Inventory (PedsQL 4.0) were also used.13,14 The clinical-demographic questionnaire consisted of personal and clinical data, including patient history regarding number of hospitalizations, infections, and the presence of ulcers, reported smoke exposure, family history (history of vascular disease), and laboratory data. The Charnley hip score3 evaluates 3 important aspects related to hip function. They are pain, gait, and range of motion. Each aspect is scored on a scale of 1 to 6, in which 6 represents the best score. The patients with hips that were considered normal, or without dysfunction, received the maximum score of 18. Patients were divided into 2 groups according to the Charnley hip score. Patients with scores less than 18 were considered to have hip dysfunction and were placed in the group “with dysfunction” while patients with scores of 18 were placed in the group “without dysfunction.” Although this score was designed for adults, it has also been efficiently tested in the pediatric population.2 The 2 groups (with and without dysfunction) were also evaluated based on Barthel’s scale,11,12 which
assesses overall functional capacity of the individual, as well as the PedsQL 4.0.13,14 Barthel’s scale is widely used in various specialties of health care, such as geriatrics, neurology, and orthopedics. It evaluates 10 general aspects of activities of daily living and its score ranges from 0 to 100.13 The PedsQL 4.0 questionnaire13,14 was developed specifically for children in defined age ranges, and it was used for children in age intervals of 8 to 12 and 13 to 18 years. Patient data were tabulated into distribution tables by frequency in the case of categorical variables or into mean and standard deviation in the case of continuous variables. For comparison between the groups with and without hip dysfunction, the chi-square test was used for discrete variables and the Student’s t test for continuous variables. To evaluate confounding variables, multivariate analysis was used when appropriate.
Results The study consisted of a total of 71 patients, 17 of whom (23.9%) had hip dysfunction and made up the “with dysfunction” group and 54 (76.1%) of whom did not have hip dysfunction and made up “without dysfunction” group. The characteristics of the sample are presented in Table 1. In the analysis of quality of life (from the perspective of the patients) and values of the Barthel scale, we can observe that patients with hip dysfunction have poorer quality of life and there are also significant association between hip dysfunction and the physical activity, social, and scholar domains (2 of the psychosocial domains) referred to by the affected individuals themselves (Table 2). The multivariate analysis of factors that could be clinically associated to a loss in quality of life in this population revealed that hip dysfunction (activity domain; P = .01), lower limb ulcer (emotional domain; P = .03), previous trauma (activity domain; P = .02), and hip pain (scholar domain; P = .04) are the only parameters consistently associated with quality of life decrease from the perspective of the affected individuals.
Discussion The current study showed that 23.9% of pediatric patients with SCD have hip dysfunction. The dysfunction was more prevalent among older patients and was associated with hip pain and history of previous trauma. Patients with hip dysfunction were show to have a lower quality of life in all the evaluated major domains (psychosocial and activities). The mean score was 56.57 in the group with dysfunction and 70.70 in the group
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Table 1. General Characteristics of the Patient Sample and General Analysis Between the Groups. Total n (%) or mean (±SD) Sample Gender Male Female Age, years Weight, kg Height, m Jaundicea Pallora Hepatitis Lower limb ulcersa Hospitalized in past year No. of hospitalizations in past year Showed infections in past year No. Infections in past year Exposure to secondhand smoke Vascular abnormalities Previous trauma Hip pain Chronic use of medication Blood transfusion
71 (100.0) 33 (46.5) 38 (53.5) 12.19 (±3.20) 42.77 (±9.78) 1.54 (±0.12) 19 (35.2) 23 (43.4) 3 (4.2) 4 (5.9) 34 (47.9) 0.88 (±1.32) 22 (31.0) 0.29 (±0.59) 14 (20.0) 8 (11.3) 7 (9.9) 10 (14.1) 71 (100.0) 21 (30.0)
With Dysfunction 17 (23.9) 5 (29.4) 12 (70.6) 13.71 (±3.21) 42.77 (±9.77) 1.53 (±0.12) 5 (35.7) 7 (53.8) 0 (0.0) 2 (12.5) 9 (52.9) 0.80 (±1.27) 4 (23.5) 0.33 (±0.62) 6 (35.3) 3 (17.6) 4 (23.5) 10 (58.8) 17 (100.0) 7 (41.2)
Without Dysfunction
P Value
54 (76.1)
28 (51.9) 26 (48.1) 11.72 (±3.12) 35.27(±12.26) 1.43 (±0.16) 14 (35.0) 16 (40.0) 3 (5.6) 2 (3.8) 25 (46.3) 0.76 (±1.13) 18 (33.3) 0.43 (±0.68) 8 (15.1) 5 (9.3) 3 (5.6) 0 (0.0) 54 (100.0) 14 (26.4)
.106 .026 .029 .019 .962 .382 1.000 .233 .632 .715 .556 .479 .070 .387 .052 .000 — .248
a Jaundice n = 54, with dysfunction = 14, without dysfunction = 40; pallor n = 53, with dysfunction = 13, without dysfunction = 40; ulcers n = 68, with dysfunction = 16, without dysfunction = 52.
Table 2. Analysis of Quality of Life Between the Groups. Total Score, Mean (±SD) Mean PedsQL child (activity and psychosocial) PedsQL child: activities PedsQL child: psychosocial PedsQL child emotional PedsQL child social PedsQL child scholar Barthel scale
With Dysfunction, Mean (±SD)
Without Dysfunction, Mean (±SD)
P Value
67.40 (±17.07)
56.57 (±13.47)
70.70 (±16.92)
.003
68.60 (±18.73) 66.21 (±18.72) 62.90 (±21.74) 72.90 (±23.69) 60.97 (±21.88) 98.24 (±3.51)
58.78 (±14.36) 54.37 (±15.58) 54.70 (±19.56) 58.82 (±21.25) 47.94 (±23.91) 98.24 (±3.51)
71.68 (±18.72) 69.71 (±18.32) 65.25 (±22.08) 77.01 (±22.99) 64.90 (±19.91) 98.15 (±3.26)
.011 .003 .082 .005 .005 .925
Abbreviation: PedsQL, Pediatric Quality of Life Inventory. a Peds child n = 71, with dysfunction = 17, without dysfunction = 54.
without dysfunction, which represents a decrease of about 20% in the quality of life. Hip dysfunction was demonstrated to be a relevant factor and in the multivariate analysis it was independently associated with poorer quality of life within the activity domain. Previous trauma, hip pain, and lower limb ulcers were also independent predictors of quality of life in the activities, scholar, and emotional domains, respectively. Conversely, the Barthel scale did not showed any difference in the quality of life of children in each of the 2 groups (with and without dysfunction).
Our findings suggest that hip dysfunction interferes with almost all domains of the patient’s life with the exception of the emotional domain. Therefore, hip dysfunction could worsen even more the quality of life of patients suffering from a chronic and severe disease such as SCD. Hip dysfunction in this population can be caused mainly by ONFH, which has an overall prevalence of 11.1% in pediatric patients with SCD.2 ONFH is closely related to age, affecting 3% of individuals younger than 15 years and 50% of those older than 35 years.2,4 Hip
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Malheiros et al dysfunction accompanies the distribution of ONFH, though the prevalence of hip dysfunction in the current study was surprisingly high when compared with similar reports. This finding may be explained by the fact that hip dysfunction is a complication of SCD that includes not only ONFH but also other factors, such as painful crisis, sequel of infection, joint stiffness, or decreased range of motion, without a necessary diagnosis of osteonecrosis. Sickle cell disease is a severe chronic diseases and the need for continuous treatment over an extended period of time is a factor which directly influence quality of life.6,8 Roberti et al15 emphasize that SCD impairs the quality of life of affected patients. In addition to this, significant correlation was confirmed with social discrimination, the so-called “SCD stigma,” due to the illness itself, educational level, and taking into account that SCD mainly affects poor and black population. The results found in implementing the Barthel scale11,12 demonstrate that hip dysfunction did not alter the general functional capacity of the subjects (P = .93). The items related to independence that showed results below the norm were urinary and/or fecal continence (occasional accidence), consistent with the young age or immaturity of some of the subjects. The patients with hip dysfunction scored values close to 18 on the Charnley hip score.3 The majority of patients with dysfunction reached values around 15 and 16 on the Charnley scale; the lowest score was 10 (only 1 patient). These data show that there was only a discrete change in hip function in the studied patients. As a general function evaluation, this change could not be detected by the Barthel scale, but was significantly perceived by the patients, especially in the physical function as demonstrated by the decrease in the quality of life showed as an independent association between hip dysfunction and the activities domain of PedsQL 4.0. In the multivariate analysis, the worse quality of life reported by the patients was also consistently associated with hip dysfunction, hip pain, and previous trauma to the hip. The worse quality of life perceived by the patients with hip dysfunction is related to the presence of pain as well, which occurred in 58.8% of the cases. Some studies reaffirm this fact by proving that pain treatment leads to an improvement of quality of life.16,17 It can thus be inferred that individuals who suffer from chronic pain who are able to independently perform activities of daily living cannot show others (including parents and family) the functional and psychosocial impact that a single, isolated factor can have on their quality of life despite being a consistent and relevant aspect of their suffering. Note that because of the young age of the population in this study, the subjects have milder symptoms when
compared with the adult population, in which there are reports of more severe pain, restricted range of motion, and hence more pronounced changes in gait.18 The Barthel scale comparison between 2 groups in the adult population might have a more significant difference and the deterioration of quality of life might be more noticeable by the accompanying evaluators (doctors and parents). It was expected that major complications such as infections, number of hospitalizations, the presence of ulcers, and frequent transfusions would be determining factors in quality of life, as cited in the literature.6-8 However, the lone factor that contributed most to the deterioration in quality of life of the patients was hip dysfunction. To reinforce the extent to which hip dysfunction interferes with quality of life,19 even mentions that the self-evaluation of quality of life in patients with SCD awaiting hip or knee arthroplasty was worse in the group awaiting hip arthroplasty. The result of this research is understandable, since the importance of the hip is understood in evaluating the biomechanics of the lower limbs. Because of its location and important role in the majority of activities of daily living performed by the lower limbs (such as sedestation, walking, and ascending and descending stairways), the hip may have a greater influence on quality of life than the knee. Sickle cell disease is a chronic disease that causes deterioration in the health of patients with it, and that requires frequent interventions and medical treatments. These treatments may or may not be invasive, and the patients are accustomed to them. Hip dysfunction causes functional loss and psychosocial differentiation which are added on to the routine suffering experienced by the patients,6-8 raising even more awareness to the perception of a worse quality of life by these patients in comparison with sickle cell patients without hip dysfunction. The current study has made a significant contribution to the literature by demonstrating for the first time that hip dysfunction is common in patients with SCD (reaching rates of 23.9%) and that it is associated to a decrease of 20% in the overall quality of life. It was also the variable that most negatively influenced the quality of life according to the patients’ own perception, in both the psychosocial and physical activity domains. Author Contributions CDM, MAM - made substantial contribution to the concept and design, acquisition of data, analysis and interpretation of data; drafted the article and revised it critically for important intellectual content; approved the version to be published. LL, MC, KNS - drafted the article and revised it critically for important intellectual content; approved the version to be published.
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Clinical Pediatrics 54(14)
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Birrell F, Croft P, Cooper C, Hosie G, Macfarlane G, Silman A. Health impact of pain in the hip region with and without radiographic evidence of osteoarthritis: a study of new attenders to primary care. Ann Rheum Dis. 2000;59:857-863. 2. Matos MA, dos Santos Silva LL, Brito Fernandes R, Dias Malheiros C, Pinto da Silva BV. Avascular necrosis of the femoral head in sickle cell disease patients. Ortop Traumatol Rehabil. 2012;14:155-160. 3. Charnley J. The Long-term results of low-friction arthroplasty of the hip performed as a primary intervention. J Bone Joint Surg Br. 1972;54:61-76. 4. Milner PF, Kraus AP, Sebes JI, et al. Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991;325:1476-1481. 5. Henrigou P, Bachir D, Galacteros F. The natural history of symptomatic osteonecrosis in adults with sickle cell disease. J Bone Joint Surg Am. 2003;85:500-504. 6. Ohara DG, Ruas G, Castro SS, Martins PR, Walsh IA. Musculoskeletal pain, profile and quality of life of individuals with sickle cell disease. Rev Bras Fisioter. 2012;16:431-438. 7. Dale JC, Cochran CJ, Roy L, Jernigan E, Buchanan GR. Health-related quality of life in children and adolescents with sickle cell disease. J Pediatr Health Care. 2011;25:208-215. 8. Menezes AS, Len CA, Hilário MO, Terreri MT, Braga JA. Quality of life in patients with sickle cell disease. Rev Paul Pediatr. 2013;31:24-29.
9. Onuba O. Bone disorders in sickle-cell disease. Int Orthop. 1993;17:397-399. 10. Pereira SAS, Cardoso CS, Brener S, Proietti ABFC. Sickle cell disease and quality of life: a study on the subjective perception of patients from the Fundação Hemominas, Minas Gerais, Brazil. Rev Bras Hematol Hemoter. 2008;30:411-416. 11. Guimarães RB, Guimarães RB. Validação e adaptação cultural para a língua portuguesa de escalas de avaliação funcional em doenças cerebrovasculares: uma tentativa de padronização e melhora da qualidade de vida. Rev Bras Neurol. 2004;40(3):5-13. 12. Mahoney FI, Barthel DW. Functional evaluation: the Barthel Index. Md State Med J. 1965;14:56-61. 13. Klatchoian DA, Len CA, Terreri MT, et al. Quality of life of children and adolescents from São Paulo: reliability and validity of the Brazilian version of the Pediatric Quality of Life Inventory® version 4.0 generic core scales. J Pediatr (Rio J). 2008;84:308-315. 14. Varni JW, Seid M, Rode CA. The PedsQL: measurement model for the pediatric quality of life inventory. Med Care. 1999;37:126-139. 15. Roberti MRF, Moreira CLNSO, Tavares RS, Filho HMB, Silva AG, Maia CHG. Evaluation of quality of life of patients with sickle cell disease in a General Hospital of Goiás, Brazil. Rev Bras Hematol Hemoter. 2010;32:449-454. 16. Al-Taki MM, Masri BA, Duncan CP, Garbuz DS. Quality of life following proximal femoral replacement using a modular system in revision THA. Clin Orthop Relat Res. 2012;469:470-475. 17. Tinti G, Somera JR, Valente FM, Domingod CRB. Benefits of kinesiotherapy and aquatic rehabilitation on sickle cell anemia. A case report. Gen Mol Res. 2010;9:360-364. 18. Dampier C, LeBeau P, Rhee S, et al. Health-related quality of life in adults with sickle cell disease (SCD): a report from the comprehensive sickle cell centers clinical trial consortium. Am J Hematol. 2011;86:203-205. 19. Ackerman IN, Bennell KL, Osbone RH. Decline in health-related quality of life reported by more than half of those waiting for joint replacement surgery: a prospective cohort study. BMC Musculoskelet Disord. 2011;12:108.
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