Haemophilia item bank based on International Classification of Functioning, Disability and Health: a systematic review

Figures & data

Table 1. Source of ICF categories.

Steps	Sources of ICF Categories	b	d	e	s	Total	Citation
1. ICF core Sets	Osteoporosis Comprehensive	13	19	17	6	55	[23]
	Generic-7	3	4	0	0	7	[24]
	Rehabilitation	9	21	0	0	30	[25]
	Minimal Set of Enviromental Factors	0	0	12	0	12	[25]
	Rheumatoid Arthritis Comprehensive	25	32	21	18	96	[26]
	Muscloskeletal Post-acute Comprehensive	23	22	18	7	70	[27]
2. Studies for Haemophilia-specific ICF Categories	Riva et al., 2010.	18	30	10	4	62	[5]
	Krasuska et al., 2012.	2	2	1	0	5	[28]
	De la Corte-Rodriguez, et al. 2016.	2	2	2	1	7	[20]
3. ICF Categories Linking to Scales Recommended by WHF	Cieza_2005	5	23	0	0	28	[19]
	Bernardelli_2021	5	18	0	0	23	[16]

b: body function, d: activity and participation, e: environment factors, s: body structure

Figure 1. Flowchart of screening the eligible articles.

Table 2. ICF-based Haemophilia Outcome Measures.

	Definition	Assessment tools	Characteristics
Body Function and Body Structure	Body structure refers to anatomical structures and body parts such as organs, limbs, and their components. Body function refers to the physiological functions of these systems, such as range of motion, strength, and joint stability. It specifically refers to the status of joints and specific muscle groups as assessed clinically and radiographically in haemophilia.	The Haemophilia Joint Health Score (HJHS)	HJHS is a reliable tool and has been widely used in children and adult [29]
		X-ray	Although Pettersson scores are widely used, they are insensitive to early lesions [30]
		Ultrasound (US)	US is helpful to detect joint exudation, early lesions, subclinical joint disease, and promote drug adhesion
		MRI	MRI is the most sensitive approach to measuring joint structure. However, its shortcomings include cost and time. Children cannot cooperate well with MRI
Activity	Activity refers to a person able to perform a task or action. In the context of haemophilia, activity generally refers to instrumental activities of daily living (e.g. walking, climbing steps, brushing teeth, and toileting).	The Functional Independence Score in Haemophilia (FISH)	FISH is the most recommended measure of performance in haemophilia [22,31]
		HAL	HAL is a disease-specific outcome measure that is mainly used in adults with haemophilia [22]
		PedHAL	PedHAL is an age-appropriate and self-reported outcome measure for children
Participation	Participation indicates engagement in activity in the context of social interaction. It is often difficult to categorize items and outcome assessment tools unambiguously as falling into only one of these two domains. Thus, the two domains are often combined in outcome assessment.	HAL/PedHAL	It might not be suitable for different cultural backgrounds [11,32]
		COPM (The Canadian Occupational Performance Measure)	It is used for daily assessment of a person's perception of change in areas of activity and participation
		SF-36	SF-36 is a widely used generic tool for assessing quality of life in patients with haemophilia
		The Patient-Reported Outcomes, Burdens and Experiences (PROBE)	PROBE assesses the impact of activities and participation, such as school/education, employment, family and daily life [33]
		HAEMO-QoL-A	Quality of life evaluation for adults with haemophilia [34]

Table 3. Mapping of outcome measure themes to ICF category codes.

No.	Category	OC	G.7	R.30	MSEF	RAC	MPAC	Ri.2010	Kr.2012	Co.2016	Ci.2005	Be.2021
1	b1263											+
2	b1265							+				+
3	b1266							+
4	b130	+	+	+		+	+
5	b1300							+			+	+
6	b134	+		+		+	+
7	b140							+
8	b1400							+
9	b1401							+
10	b152	+	+	+		+	+	+	+		+	+
11	b1529										+
12	b180					+
13	b1801					+		+
14	b260						+
15	b270						+
16	b280	+	+	+		+	+	+	+		+	+
17	b2800					+
18	b2801					+		+
19	b28010					+
20	b28013					+
21	b28014					+
22	b28015					+
23	b28016					+		+
24	b289										+
25	b415						+
26	b430					+		+		+
27	b4303							+
28	b435						+
29	b440						+
30	b455			+		+	+	+
31	b4551							+
32	b510					+
33	b525						+
34	b530						+
35	b620			+			+
36	b640			+		+
37	b710	+		+		+	+	+		+
38	b7101							+
39	b7102					+
40	b715	+				+	+
41	b720	+
42	b730	+		+		+	+	+
43	b735	+					+
44	b740	+				+	+
45	b755						+
46	b760	+					+
47	b770	+				+	+
48	b780	+				+	+
49	b7800					+
50	b810						+
51	d155						+
52	d161							+
53	d170					+
54	d177						+
55	d230		+	+		+	+	+			+	+
56	d240			+			+
57	d310						+
58	d360					+
59	d410	+		+		+	+
60	d4101											+
61	d4102										+	+
62	d4105										+	+
63	d415	+		+		+	+
64	d420			+			+
65	d430	+				+	+	+			+	+
66	d4300							+				+
67	d4302							+
68	d4309										+
69	d440	+				+	+
70	d445	+				+	+
71	d4459										+
72	d449					+
73	d450	+	+	+		+	+	+			+
74	d4500										+	+
75	d4501							+			+	+
76	d4502							+
77	d455	+	+	+		+
78	d4551							+			+	+
79	d4552											+
80	d4559										+
81	d460					+	+			+
82	d465			+		+	+
83	d470	+		+		+
84	d475	+				+
85	d498										+
86	d510	+		+		+	+				+	+
87	d5101										+
88	d520			+		+	+
89	d530	+		+		+	+
90	d540	+		+		+	+				+	+
91	d550			+		+	+
92	d560					+	+
93	d570			+		+	+	+
94	d5702							+
95	d620	+				+
96	d630					+
97	d640	+		+		+
98	d6402											+
99	d6403											+
100	d6409										+
101	d660	+		+		+
102	d710			+				+
103	d740							+
104	d750							+	+			+
105	d7500							+
106	d7504							+
107	d760					+	+	+
108	d7600							+
109	d7609										+
110	d770	+		+		+		+
111	d820							+
112	d830							+
113	d835							+
114	d839										+
115	d8450							+
116	d8451							+
117	d850	+	+	+		+		+			+	+
118	d8509										+
119	d859					+					+
120	d880							+
121	d910	+				+
122	d920	+		+		+		+	+	+
123	d9201							+				+
124	d9202							+
125	d9205							+			+	+
126	d9209										+
127	e110	+			+	+	+
128	e1101							+
129	e115	+			+	+	+
130	e120	+			+	+	+
131	e1201							+
132	e125					+	+
133	e135	+			+	+
134	e150	+			+	+	+
135	e155	+			+	+
136	e225	+			+	+	+
137	e310	+			+	+	+
138	e320	+			+	+	+	+		+
139	e340	+				+	+
140	e355	+			+	+	+	+
141	e360					+
142	e410	+				+	+	+	+
143	e420					+	+
144	e425					+		+
145	e430						+	+
146	e440						+
147	e450	+			+	+	+	+
148	e460	+				+
149	e515									+
150	e540	+				+
151	e555						+
152	e570					+
153	e575	+					+
154	e580	+			+	+	+	+
155	e5800							+
156	s299					+
157	s710					+	+
158	s720	+				+	+
159	s730	+				+	+	+
160	s73001					+
161	s73011					+
162	s7302					+
163	s73021					+
164	s73022					+
165	s740	+					+
166	s750	+				+	+	+
167	s75001					+
168	s75011					+
169	s7502					+
170	s760					+	+
171	s7600					+
172	s76000					+
173	s770	+				+		+
174	s7701							+
175	s799	+
176	s810					+	+

‘+’ indicates that these ICF categories were linked to the item of the corresponding outcome measures

OC: Osteoporosis Comprehensive Core Set

G.7: Generic Set with 7 Categories

R.30: Rehabilitation Set with 30 Categories

MSEF: Minimal Set of Environmental Factors

RAC: Rheumatoid Arthritis Comprehensive Set

MPAC: Muscloskeletal Post-acute Comprehensive

Ri.2010: Report of Riva et al., 2010

Kr.2012: Report of Krasuska et al., 2012

Co.2016: Report of De la Corte-Rodriguez & Rodriguez-Merchan, 2016

Ci.2005: Report of Cieza & Stucki, 2005

Be.2021: Report of Bernardelli et al., 2021

Table 4. Haemophilia-related ICF Comprehensive Core Set.

Code	ICF Categories
b1263	Psychic stability
b1265	Optimism
b1266	Confidence
b130	Energy and drive functions (G)
b134	Sleep functions
b140	Attention functions
b152	Emotional functions (G)
b180	Experience of self and time functions
b260	Proprioceptive function
b270	Sensory functions related to temperature and other stimuli
b280	Sensation of pain (G)
b415	Blood vessel functions
b430	Haematological system functions
b435	Immunological system functions
b440	Respiration functions
b455	Exercise tolerance functions
b510	Ingestion functions
b525	Defecation functions
b530	Weight maintenance functions
b620	Urination functions
b640	Sexual functions
b710	Mobility of joint functions
b715	Stability of joint functions
b720	Mobility of bone functions
b730	Muscle power functions
b735	Muscle tone functions
b740	Muscle endurance functions
b755	Involuntary movement reaction functions
b760	Control of voluntary movement functions
b770	Gait pattern functions
b780	Sensations related to muscles and movement functions
b810	Protective functions of the skin
d155	Acquiring skills
d170	Writing
d177	Making decisions
d230	Carrying out daily routine
d240	Handling stress and other psychological demands
d310	Communicating with - receiving - spoken messages
d360	Using communication devices and techniques
d410	Changing basic body position
d415	Maintaining a body position
d420	Transferring oneself
d4300	Lifting
d4302	Carrying in the arms
d440	Fine hand use
d445	Hand and arm use
d4500	Walking short distances
d4501	Walking long distances
d4502	Walking on different surfaces
d455	Moving around
d460	Moving around in different locations
d465	Moving around using equipment
d470	Using transportation
d475	Driving
d510	Washing oneself
d520	Caring for body parts
d530	Toileting
d540	Dressing
d550	Eating
d560	Drinking
d570	Looking after one's health
d620	Acquisition of goods and services
d630	Preparing meals
d640	Doing housework
d660	Assisting others
d710	Basic interpersonal interactions
d740	Formal relationships
d750	Informal social relationships
d760	Family relationships
d770	Intimate relationships
d820	School education
d830	Higher education
d8450	Seeking employment
d8451	Maintaining a job
d850	Remunerative employment
d910	Community life
d920	Recreation and leisure
e110	Products or substances for personal consumption
e1101	Drugs
e115	Products and technology for personal use in daily living
e120	Products and technology for personal indoor and outdoor mobility and transportation
e125	Products and technology for communication
e135	Products and technology for employment
e150	Design, construction and building products and technology of buildings for public use
e155	Design, construction and building products and technology of buildings for private use
e225	Climate
e310	Immediate family
e320	Friends
e340	Personal care providers and personal assistants
e355	Health professionals
e360	Other professionals
e410	Individual attitudes of immediate family members
e420	Individual attitudes of friends
e425	Individual attitudes of acquaintances, peers, colleagues, neighbors and community members
e430	Individual attitudes of people in positions of authority
e440	Individual attitudes of personal care providers and personal assistants
e450	Individual attitudes of health professionals
e460	Societal attitudes
e515	Architecture and construction services, systems and policies
e540	Transportation services, systems and policies
e555	Associations and organizational services, systems and policies
e570	Social security services, systems and policies
e575	General social support services, systems and policies
e580	Health services, systems and policies
s710	Structure of head and neck region
s720	Structure of shoulder region
s730	Structure of upper extremity
s740	Structure of pelvic region
s750	Structure of lower extremity
s760	Structure of trunk
s770	Additional musculoskeletal structures related to movement
s810	Structure of areas of skin

Kurtaiş Y, Őztuna D, Küçükdeveci AA, et al. Reliability, construct validity and measurement potential of the ICF comprehensive core set for osteoarthritis. BMC Musculoskelet Disord. 2011;12(1):255.

 PubMedGoogle Scholar

Ehrmann C, Prodinger B, Stucki G, et al. Icf generic Set as New standard for the system wide assessment of functioning in China: a multicentre prospective study on metric properties and responsiveness applying item response theory. BMJ Open. 2018;8(12):e021696.

 PubMed Web of Science ®Google Scholar

Prodinger B, Cieza A, Oberhauser C, et al. Toward the international classification of functioning, disability and health (ICF) rehabilitation set: a minimal generic set of domains for rehabilitation as a health strategy. Arch Phys Med Rehabil. 2016;97(6):875–884.

 PubMed Web of Science ®Google Scholar

Stucki G, Cieza A, Geyh S, et al. ICF Core Sets for rheumatoid arthritis. J Rehabil Med. 2004;36(0):87–93.

 Google Scholar

Scheuringer M, Stucki G, Huber EO, et al. ICF Core Set for patients with musculoskeletal conditions in early post-acute rehabilitation facilities. Disabil Rehabil. 2005;27(7–8):405–410.

 PubMed Web of Science ®Google Scholar

Riva S, Bullinger M, Amann E, et al. Content comparison of haemophilia specific patient-rated outcome measures with the international classification of functioning, disability and health (ICF, ICF-CY). Health Qual Life Outcomes. 2010;8(1):139.

 PubMedGoogle Scholar

Krasuska M, Riva S, Fava L, et al. Linking quality-of-life measures using the International Classification of Functioning, Disability and Health and the International Classification of Functioning, Disability and Health-Children and Youth Version in chronic health conditions: the example of young people with hemophilia. Am J Phys Med Rehabil. 2012;91(13 Suppl 1):S74–S83.

 PubMedGoogle Scholar

De la Corte-Rodriguez H, Rodriguez-Merchan EC. The ICF (International Classification of Functioning, Disability and Health) developed by the WHO for measuring function in hemophilia. Expert Rev Hematol. 2016;9(7):661–668.

 PubMed Web of Science ®Google Scholar

Cieza A, Stucki G. Content comparison of health-related quality of life (HRQOL) instruments based on the international classification of functioning, disability and health (ICF). Qual Life Res. 2005;14(5):1225–1237.

 PubMed Web of Science ®Google Scholar

Bernardelli RS, Santos BC, Scharan KO, et al. Application of the refinements of ICF linking rules to the Visual Analogue Scale, Roland Morris questionnaire and SF-36. Cienc Saude Coletiva. 2021;26(3):1137–1152.

 PubMed Web of Science ®Google Scholar

Gouw SC, Timmer MA, Srivastava A, et al. Measurement of joint health in persons with haemophilia: a systematic review of the measurement properties of haemophilia-specific instruments. Haemophilia. 2019;25(1):e1–e10.

 PubMed Web of Science ®Google Scholar

Pettersson H, Ahlberg A, Nilsson IM. A radiologic classification of hemophilic arthropathy. Clin Orthop. 1980;149:153–159.

 PubMed Web of Science ®Google Scholar

Timmer MA, Gouw SC, Feldman BM, et al. Measuring activities and participation in persons with haemophilia: a systematic review of commonly used instruments. Haemoph Off J World Fed Hemoph. 2018;24(2):e33–e49.

 PubMed Web of Science ®Google Scholar

Poonnoose PM, Thomas R, Keshava SN, et al. Psychometric analysis of the functional independence score in haemophilia (FISH). Haemoph Off J World Fed Hemoph. 2007;13(5):620–626.

 PubMed Web of Science ®Google Scholar

Rentz A, Flood E, Altisent C, et al. Cross-cultural development and psychometric evaluation of a patient-reported health-related quality of life questionnaire for adults with haemophilia. Haemoph Off J World Fed Hemoph. 2008;14(5):1023–1034.

 PubMed Web of Science ®Google Scholar

Wharfe G, Buchner-Daley L, Gibson T, et al. The Jamaican haemophilia registry: describing the burden of disease. Haemoph Off J World Fed Hemoph. 2018;24(4):e179–e186.

 PubMed Web of Science ®Google Scholar

Skinner MW, Chai-Adisaksopha C, Curtis R, et al. The Patient Reported Outcomes, Burdens and Experiences (PROBE) Project: development and evaluation of a questionnaire assessing patient reported outcomes in people with haemophilia. Pilot Feasibility Stud. 2018;4:58.

 PubMedGoogle Scholar

Remor E. Development and psychometric testing of the Hemophilia Well-being Index. Int J Behav Med. 2013;20(4):609–617.

 PubMed Web of Science ®Google Scholar