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Analysis of the Italian cohort of adults with mild or moderate haemophilia A in the CHESS II dataset shows a meaningful clinical and psychosocial burden of disease, impacted by bleeding events, chronic pain, anxiety, depression, and limitations on activities of daily life
Analysis of the Italian cohort of adults with mild or moderate haemophilia A in the CHESS II dataset shows a meaningful clinical and psychosocial burden of disease, impacted by bleeding events, chronic pain, anxiety, depression, and limitations on activities of daily life

Figure 1:

Psychosocial impact of haemophilia A among participants completing the patient self-complete questionnaire
Note: the figure above is relative to the subsample who also provided a patient-questionnaire composed by n=19 with mild HA and n=25 with moderate HA
HA: haemophilia A
Psychosocial impact of haemophilia A among participants completing the patient self-complete questionnaire Note: the figure above is relative to the subsample who also provided a patient-questionnaire composed by n=19 with mild HA and n=25 with moderate HA HA: haemophilia A

Demographic characteristics and clinical outcomes of people with mild or moderate haemophilia A

MILD HA (N=34) MODERATE HA (N=79)
Age
    Mean (SD) 36.6 (12.0) 41.4 (14.1)
    Median (IQR) 37.0 (27.0, 42.0) 41.0 (29.0, 54.0)
Age, n (%)
    18-35 15 (44) 31 (39)
    36-59 16 (47) 39 (49)
    >60 3 (9) 9 (11)
Body mass index, n (%)
    Underweight 0 (0) 1 (1)
    Normal weight 18 (53) 48 (61)
    Overweight 16 (47) 28 (35)
    Obese 0 2 (3)
Current treatment regimen, n (%)
    On-demand 10 (29) 24 (30)
    No recent FVIII treatmenta 24 (71) 55 (70)
    Prophylaxis 0 0
ABR (all cause)
    Bleeding reported, n (%) 24 (71) 67 (85)
    Mean (SD) 1.1 (0.9) 2.9 (2.7)
    Median (Q1, Q3) 1.0 (0.0, 2.0) 2.0 (1.0, 4.0)
ABR (spontaneous)b
    Spontaneous bleeding reported, n (%) - 57 (72)
    Mean (SD) - 0.88 (0.93)
    Median (Q1, Q3) - 0.6 (0, 1.2)
ABR, n (%)
    0 10 (29) 12 (15)
    1 14 (41) 14 (18)
    >2 10 (29) 53 (67)
Baseline factor level (overall)
    Mean (SD) 21.0 (10.3) 3.5 (1.2)
    Median (Q1, Q3, range) 20 (11, 30, 5.01-40) 4 (3, 5, 1-5)
Baseline factor level in those reporting a bleeding event
    Mean (SD) 21.3 (9.8) 3.4 (1.3)
    Median (Q1, Q3, range) 20 (15, 27, 5.1-37) 3.3 (2.3, 5, 1-5)
Target joints
    Mean (SD) 0.1 (0.3) 0.3 (0.7)
    Median (Q1, Q3) 0 0
Target joints, n (%)
    0 30 (88) 62 (79)
    1 4 (12) 11 (14)
    >2 0 6 (8)
Problem joints
    Mean (SD) 0.1 (0.4) 0.7 (0.8)
    Median (Q1, Q3) 0 1.0 (0.0, 1.0)
Problem joints, n (%)
    0 30 (88) 39 (49)
    1 3 (9) 26 (33)
    >2 1 (3) 14 (18)
Chronic pain level, n (%)
    None 22 (65) 21 (27)
    Mild pain 11 (32) 41 (52)
    Moderate pain 1 (3) 17 (22)
    Severe pain 0 0
    Anxiety, n (%) 4 (12) 16 (20)
    Depression, n (%) 1 (3) 12 (15)

Clinical outcomes of participants who completed the patient self-complete questionnaire, by haemophilia A severity

MILD HA (N=19) MODERATE HA (N=25)
Age
    Mean (SD) 37.7 (13.1) 39.6 (14.6)
    Median (Q1, Q3) 37.0 (27.0, 46.0) 31.0 (28.0, 47.0)
Age, n (%)
    18-35 8 (42) 14 (56)
    36-59 9 (47) 7 (28)
    >60 2 (11) 4 (16)
Body mass index, n (%)
    Underweight 0 (0) 0 (0)
    Normal weight 11 (58) 13 (52)
    Overweight 8 (42) 11 (44)
    Obese 0 1 (4)
Current treatment regimen, n (%)
    On-demand 8 (42) 9 (36)
    No recent FVIII treatmenta 11 (58) 16 (64)
    Prophylaxis 0 0
ABR (all cause)
    Mean (SD) 1.3 (0.9) 3.0 (2.7)
    Median (IQR) 1.0 (1.0, 2.0) 2.0 (1.0, 4.0)
Target joints
    Mean (SD) 0.1 (0.3) 0.3 (0.6)
    Median (IQR) 0 0
Target joints, n (%)
    0 17 (90) 20 (80)
    1 2 (11) 3 (12)
    >2 0 2 (8)
Problem joints
    Mean (SD) 0.1 (0.5) 0.6 (0.7)
    Median (IQR) 0 0
Problem joints, n (%)
    0 18 (95) 13 (52)
    1 0 9 (36)
    >2 1 (5) 3 (12)
Chronic pain level, n (%)
    None 11 (58) 5 (20)
    Mild pain 8 (42) 14 (56)
    Moderate pain 0 6 (24)
    Severe pain 0 0
    Anxiety, n (%) 2 (11) 3 (12)
    Depression, n (%) 0 2 (8)
eISSN:
2055-3390
Sprache:
Englisch
Zeitrahmen der Veröffentlichung:
Volume Open
Fachgebiete der Zeitschrift:
Medizin, Vorklinische Medizin, Grundlagenmedizin, andere, Klinische Medizin, Pharmazie, Pharmakologie