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. Author manuscript; available in PMC: 2023 Jun 1.
Published in final edited form as: Arch Phys Med Rehabil. 2021 Sep 27;103(6):1144–1167.e2. doi: 10.1016/j.apmr.2021.08.022

Table 6.

Health-related Quality of Life: Physical Function

Study N Age range/mean Diagnoses Non-SCD Control Relevant Outcome Measures Findings Related to Physical Impairments and Function
Adeyemo et al. 201579 80 15–18 years/16 ± 1.5 years HbSS
HbSC		 Yes

  • SF-36

  • Participants with SCD report lower physical function and physical role compared to controls

  • Physical function (SCD: 69.0 ± 23.2; controls: 83.0 ± 20.7); p = 0.002

  • Role physical (SCD: 61.4 ± 28.5; controls: 74.2 ± 23.9); p = 0.02
Barakat et al. 200880 42 12–18 years/15 ± 1.82 years HbSS No

  • CHQ

  • Participants with SCD scored within the normative range for physical functioning

  • Participants with SCD reported higher physical function compared to caregivers (p = 0.009)
Dampier et al. 201013 1,772 2–18 years/9.6 ± 4.7 years HbSS
HbSC
HbSβ+
HbSβ0 		No

  • PedsQL
  • Physical functioning
    • Child (72.28 ± 16.02)

  • Parent (71.13 ± 22.97)
Dampier et al. 201612 121 8–17
years/12.5 ± 3.1 years		 HbSS
HbSC		 Yes

  • PROMIS

  • Participants with SCD had similar scores compared to the mean of a reference sample (children with and without health conditions)

  • Pain interference (56.1 ± 1.2)

  • Mobility (48.2 ± 0.9)

  • Upper dexterity (50.0 ± 0.9)
Dampier et al. 201676 235 8–17 years/12.5 ± 2.8 years HbSS
HbSC
HbSβ+
HbSβ0 		Yes

  • PROMIS

  • Participants with SCD had similar scores compared to the mean of a reference sample (children with and without health conditions)

  • Pain interference (48.7 ± 13.6)

  • Mobility (50.6 ± 8.3)

  • Upper dexterity (50.6 ± 7.5)
Dougherty et al. 202039 21 5–20 years/11 ± 4 years HbSS Yes

  • PROMIS

  • Participants with SCD reported similar physical function (upper and mobility), and pain impact scores compared to controls

  • Physical function upper (SCD: 45.9 ± 10.9; controls: 50.6 ± 8.8); NS

  • Physical function mobility (SCD: 53.1 ± 6.2; controls: 57. 8 ± 3.3); NS

  • Pain impact (SCD: 54.4 ± 13.3; controls: 48.6 ± 8.7); NS
Hoff et al. 200681 56 8–17 years/12.14 ± 2.46 years HbSS
HbSC
HbSβ+ 		No

  • FDI

  • FDI (10.99 ± 10.67)
Hussein et al. 201961 100 6–12 years/8.90 ± 2.06 SCA No

  • Self-made questionnaire
  • Activities and motor (transferring):
    • Poor (13.0%),
    • Moderate (55.0%)
    • Good (32.0%)
  • Play activities:
    • Poor (11.0%)
    • Moderate (53.0%)
    • Good (23.0%)
Kambasu et al. 201962 140 8–17 years/14.25 years HbSS NO

  • PedsQL
  • Physical function
    • Child (57.5 ± 20.3)
    • Caregiver (52.8 ± 22.1)

  • Participants with SCD reported higher physical function compared to caregivers (p < 0.001)
Karlson et al. 202075 206 8–17 years/11.72 ± 4.42 years HbSS
HbSC		 No

  • Child Physical Activity Questionnaire
  • Reported days of physical activity (30 minutes) per week:
    • Child:
      • 0 days (10.88%)
      • 1–2 days (23.13%)
      • 3–5 days (43.54%)
      • 6–7 days (22.45%)
    • Caregiver:
      • 0 days (3.66%)
      • 1–2 days (21.95%)
      • 3–5 days (40.24%)
      • 6–7 days (34.15%)

  • No significant difference between child and caregiver report
Malheiros et al. 201563 71 8–21 years/12.19 ± 3.2 years SCA with and without hip dysfunction No

  • PedsQL

  • Barthel Index
  • PedsQL activities score
    • All (68.60 ± 18.73)
    • With hip dysfunction (58.78 ± 14.3)
    • Without dysfunction (71.68 ± 18.72)
    • A significant difference between groups with and without hip dysfunction (p = 0.011)
  • Barthel Index
    • All (98.24 ± 3.51)
    • With hip dysfunction (98.24 ± 3.51)
    • Without hip dysfunction 98.15 ± 3.51)
    • No significant difference between groups with and without hip dysfunction (p = 0.925)
Matos et al. 201864 24 8–18 years/11.1 ± 3.9
years		 SCA Yes

  • PedsQL

  • Participants with SCA and avascular necrosis presented with decreased PedsQL physical function compared to the group without necrosis (SCD: 65.1 ± 19.3; controls: 87.5 ± 6.9); p = 0.005

  • No significant difference between SCD and Perthes disease groups
Melo et al. 20179 57 6–18 years/11.9 ± 3.5 years HbSS Yes
  • PAQ-C
    • Brazilian version

  • Participants with SCD presented with lower PAQ-C scores compared to controls

  • Total score (SCD: 1.64 ± 0.39; controls: 3.2 ± 0.38); p < 0.001

  • Spare time activity (SCD: 0.77 ± 0.37; controls: 2.37 ± 0.55); p < 0.001

  • Activity during physical education classes (SCD: 1.77 ± 0.68; controls: 3.2 ± 0.55); p < 0.001

  • Lunch-time activity (SCD: 1.4 ± 0.53; controls: 3.1 ± 0.72); p < 0.001

  • After school activity (SCD: 1.77 ± 0.92; controls: 3.0 ± 0.61); p < 0.001

  • Evening activity (SCD: 1.63 ± 0.77; controls: 3.22 ± 0.77); p < 0.001

  • Weekend activity (SCD: 2 ± 0.75; controls: 3.44 ± 0.77); p < 0.001

  • Activity frequency in the last 7 days (SCD: 2.26 ± 0.76; controls: 3.43 ± 0.62); p < 0.001

  • Activity during each day last week (SCD: 1.63 ± 0.53; controls: 3.21 ± 0.50); p < 0.001
Melo et al. 201859 50 6–18 years/12.02 ± 3.63 years HbSS Yes
  • PAQ-C
    • Brazilian version

  • Participants with SCD presented with lower PAQ-C scores compared to controls

  • Total score (SCD: 1.65 ± 0.41; controls: 3.39 ± 0.38); p < 0.01

  • Spare time activity (SCD: 0.75 ± 0.31; controls: 2.48 ± 0.61); p < 0.01

  • Activity during physical education classes (SCD: 1.74 ± 0.62; controls: 3.36 ± 0.65); p < 0.01

  • Break-time activity (SCD: 1.61 ± 0.69; controls: 3.36 ± 0.65); p < 0.01

  • Lunch-time activity (SCD: 1.42 ± 0.53; controls: 3.32 ± 0.71); p < 0.01

  • After school activity (SCD: 1.85 ± 0.98; controls: 3.38 ± 0.56); p < 0.01

  • Evening activity (SCD: 1.77 ± 0.84; SCD: 3.58 ± 0.73); p < 0.01

  • Weekend activity (SCD: 2.08 ± 0.75; controls: 3.63 ± 0.78); p < 0 01

  • Activity frequency in the last 7 days (SCD: 2.26 ± 0.76; controls: 3.43 ± 0.62); p < 0.001

  • Activity during each day last week (SCD: 1.62 ± 0.53; controls: 3.37 ± 0.58); p < 0.01
Menezes et al. 200865 100 5–18 years HbSS
HbSC
HbSβ+ 		Yes

  • PedsQL
  • Participants with SCD presented with lower PedsQL physical function and compared to controls
    • 5–7 years (SCD: 56.16 ± 12.33; controls: 91.81 ± 9.36); p < 0.0001
    • 8–12 years (SCD: 48.37 ± 13.77; controls: 94.10 ± 6.64); p < 0.0001
    • 13–18 years (SCD: 42.86 ± 13.77; controls: 94.55 ± 6.31); p < 0.0001
Möckesch et al. 20177 46 10–16 years/15 ± 2.4 (HbSS) 15.1 ± 2.6
(HbSC)		 HbSS
HbSC		 Yes
  • IPAQ
    • adapted for adolescents

  • Participants with SCD reported decreased daily expenditure related to moderate and intense physical activity compared to controls (p = 0.02)
Omwanghe et al. 201777 100 6–12th graders SCD Yes
  • NHANES
    • Physical Activity Questionnaire
  • A lower proportion of participants with SCD reported:
    • Less time spent in sustained physical activity (60 minutes or more) on any given day of the week
      • Vigorous physical activity (SCD: 24%; controls: 43%); p = 0.01
      • Moderate-to-vigorous physical activity (SCD: 17%; controls: 23%); p < 0.01
  • A greater proportion of participants with SCD reported:
    • At least 10 minutes of moderate-to-vigorous activity (SCD: 67%; controls: 42%); p < 0.01
    • The number of days spent in moderate-to-vigorous physical activity was higher in SCD (2.3 vs. 1.4 days per week; p < 0.01
  • Similar portions of participants with SCD reported:
    • At least 10 continuous minutes of vigorous physical activity in a typical week (SCD: 66%; controls: 65%); p = 0.91
    • The number of days per week in vigorous physical activity (SCD: 2.8 days; controls: 2.5 days); p = 0.44
Oliver-Carpenter et al. 201166 47 6–18 years/11.93 ± 3.8 years HbSS
HbSC
HbSβ+
HbSβ0 		Yes

  • FDI

  • Participants with SCD reported higher functional disability compared to caregiver-report (child: 19.2 ± 15.2; parent: 10.6 ± 14.5); p < 0.05
Palermo et al. 200267 58 5–18 years/10.97 ± 3.41 HbSS
HbSC
HbSβ+ 		Yes
  • CHQ
    • Parent-form (PF50)

  • Caregivers of children and adolescents with SCD reported lower scores on CHQ-PF50 physical functioning, role impact-physical, and physical health scales compared to controls

  • Physical functioning (SCD: 78.7 ± 11.4; controls: 94.5 ± 8.6); p = 0.0001

  • Role impact - physical (SCD: 80.2 ± 13.8; controls: 98.6 ± 4.6); p = 0.0001

  • Physical health summary score (SCD: 39.4 ± 6.4; controls: 54.9 ± 3.2); p < 0.0001
Palermo et al. 200578 42 9–17 years/13.36 ± 1.34 years HbSS
HbSC
HbSβ+ 		No

  • FDI

  • A significant difference in functional disability between migraine and tension and migraine and no headache groups

  • FDI score
    • Migraine ± aura (15.81 ± 4.69)
    • Tension headache (7.14 ± 4.89)
    • No headache (4.71 ± 3.58)
Panepinto et al. 200568 99 5–8 years/10.67 ± 3.71 years HbSS
HbSC
HbSβ+
HbSβ0
HbS
tacoma		 No
  • CHQ
    • Child form (CF87)
    • Parent form (PF28)

  • Participants with SCD reported higher physical function compared to caregiver-report (child: 75.22; caregiver: 58.82); p < 0.005
Patel & Pathan 200569 25 8–18 years/11.2 ± 1.7 years SCA Yes

  • EUROQOL
  • Mobility:
    • No problem (SCD: 76%; controls: 100%)
    • Problem on some days (SCD: 24%; controls: 0%)
    • Unable to do on most days (SCD: 0%; controls 0%)
Sil et al. 201670 100 8–18 years/13.54 ± 2.7 years HbSS
HbSC
HbSβ+
HbSβ0 		No

  • FDI

  • A significant difference in reported functional disability between no pain and episodic pain, and no pain and chronic pain.

  • FDI
    • No pain (6.70 ± 0.67)
    • Episodic pain (14.33 ± 11.02)
    • Chronic pain (20.58 ± 11.09)
Sil et al. 202071 42 8–18 years/14.95 years HbSS
HbSC
HbSβ+
HbSβ0 		No

  • FDI
  • FDI
    • Asymptomatic and episodic pain (10.15 ± 12.69)
    • Chronic pain (24.53 ± 12.87)
Singh et al. 202072 67 8–18 years/10.7 ± 3.6 years HbSS
HbSC
HbSβ+
HbSβ0
Other		 No

  • PROMIS from Pediatric-25 profile
  • Physical activity
    • ED visit: 48.1 ± 6.8
    • 7–10 days: 44.3 ± 10.2
    • 1–3 months: 44.2 ± 6.6
  • Physical strength impact
    • ED visit: 34.8 ± 4.1
    • 7–10 days: 38.9 ± 9.4
    • 1–3 months: 40.3 ± 9.0
  • Physical function mobility
    • ED visit: 41.0 ± 9.1
    • 7–10 days: 43.7 ± 10.2
    • 1–3 months: 49.6 ± 9.4
Singh et al. 201973 164 5–17 years/10 ± 4 years HbSS
HbSC
HbSβ0
Other		 No

  • PROMIS
  • Physical activity
    • All (49 ± 7.6)
    • No pain medication in the prior week (48.6 ± 7.8)
    • Pain medication in the prior week (50.2 ± 6.8)
    • Pain intensity 0–3 (49.4 ± 8.4)
    • Pain intensity 4–6 (47.9 ± 7.6)
    • Pain intensity 7–10 (49 ± 5.6)
    • No significant difference between pain medication in the prior week or pain intensity groups
  • Strength impact
    • All (39.7 ± 8.1)
    • No pain medication in the prior week (40.4 ± 8.6)
    • Pain medication in the prior week (36.9 ± 5.0)
    • Pain intensity 0–3 (41.6 ± 8.6)
    • Pain intensity 4–6 (39.9 ± 8.1)
    • Pain intensity 7–10 (36.2 ± 5.7)
    • Significant difference between medication in prior week (p = 0.02) and pain intensity (p = 0.01) groups
Thornburg et al. 201174 191 2–18 years/10.4 ± 4.7 years HbSS
HbSC
HbSβ+
HbSβ0
HbSOA
rab
HbSGP
hil-adelphia		 No

  • PedsQL
  • Physical functioning – child-report
    • Hydroxyurea (79.7† [IQR 62.5–90.6])
    • No Hydroxyurea (71.4† [IQR 58.6–81.2]
    • p = 0.01
  • Physical Function – caregiver-report
    • Hydroxyurea (75† [IQR 53.9–8.75])
    • No Hydroxyurea (71.9† [IQR 53.2–90.6])
    • p = 0.05
Zempsky et al. 201455 159 7.2621.82 years/15.73 ± 3.63 years HbSS
HbSC
HbSβ+
HbSβ0 		No

  • YAPFAQ

  • CALI

  • FIM

  • YAPFAQ (17.56 ± 11.95)

  • CALI (23.96 ± 17.56)

  • FIM (56.65 ± 15.23)

Abbreviations: N = number of participants with sickle cell disease; NS = not significant; SCA = Sickle Cell Anemia; SCD = Sickle Cell Disease; Barthel Index = The Barthel Index for Activities of Daily Living; CALI = Child Activities Limitations Interview; CHQ = Child Health Questionnaire; FDI = Functional Disability Index; FIM = Functional Independence Measure; IPAQ = International physical activity questionnaire; NHANES = National Health and Nutrition Examination Survey; PAQ-C = Physical Activity Questionnaire for Older Children; PedsQL = Pediatric Quality of Life Inventory; PROMIS = Patient-Reported Outcomes Measurement Information System; SF-36 = 36-Item Short Form Health Survey; YAPFAQ = Youth Acute Pain Functional Ability Questionnaire. Results are reported as mean ± standard deviation, except where noted otherwise.

*

Standard error.

†

Median (Interquartile range [IQR]).