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New challenges for an expanding generation of older persons with haemophilia

Jamie O’Hara
Jamie O’Hara
, 
Declan Noone
Declan Noone
, 
Persefoni Kritikou
Persefoni Kritikou
, 
Karl-Johan Myren
Karl-Johan Myren
, 
Steve Chaplin
Steve Chaplin
 y 
Daniel P Hart
Daniel P Hart
   | 09 mar 2022
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Article Category: Community Focus
Publicado en línea: 09 mar 2022
Páginas: 1 - 13
DOI: https://doi.org/10.2478/jhp-2022-0001
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© 2022 Jamie O’Hara et al., published by Sciendo
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

The advent of prophylaxis has raised expectations that people with haemophilia will develop less joint damage and experience a normal life expectancy compared with earlier generations – but considerations around quality of life and age-related disorders must also be taken into account© Shutterstock
The advent of prophylaxis has raised expectations that people with haemophilia will develop less joint damage and experience a normal life expectancy compared with earlier generations – but considerations around quality of life and age-related disorders must also be taken into account© Shutterstock

Figure 1

Median number of affected joints by age category in the CHESS populationNote: Figures presents the median and inter-quartile range (IQR) of the number of affected joints for each age category. The Kruskal-Wallis test compares difference between the overall groups (global p-value). Individual, sequential pairwise comparison are made between each age category pairs based on Wilcoxon test. All variables presented are from the CRF.
Median number of affected joints by age category in the CHESS populationNote: Figures presents the median and inter-quartile range (IQR) of the number of affected joints for each age category. The Kruskal-Wallis test compares difference between the overall groups (global p-value). Individual, sequential pairwise comparison are made between each age category pairs based on Wilcoxon test. All variables presented are from the CRF.

Figure 2

Median number of comorbidities by age group in the CHESS populationNote: Figures presents the median and inter-quartile range (IQR) of the number of affected joints for each age category. The Kruskal-Wallis test compares difference between the overall groups (global p-value). Individual, sequential pairwise comparison are made between each age category pairs based on Wilcoxon test. All variables presented are from the CRF.
Median number of comorbidities by age group in the CHESS populationNote: Figures presents the median and inter-quartile range (IQR) of the number of affected joints for each age category. The Kruskal-Wallis test compares difference between the overall groups (global p-value). Individual, sequential pairwise comparison are made between each age category pairs based on Wilcoxon test. All variables presented are from the CRF.

Figure 3

EQ-Visual analogue scale (EQ-VAS) and EQ-5D index score by age category (standard errors)Note: All variables presented in Figure 3 are from the PSCAbbreviations: CHESS: The Cost of Haemophilia across Europe: a Socioeconomic Survey; HSE: Health Survey for England
EQ-Visual analogue scale (EQ-VAS) and EQ-5D index score by age category (standard errors)Note: All variables presented in Figure 3 are from the PSCAbbreviations: CHESS: The Cost of Haemophilia across Europe: a Socioeconomic Survey; HSE: Health Survey for England

Prevalence of each comorbid disease and mean number of reported comorbidities

COMORBIDITIES N % WITH COMORBIDITY MEAN COMORBIDITY ± SE MEAN COMORBIDITY ± SD
None 624 - - -
Alcohol dependency 41 3% 1.93 ± 0.36 1.93 ± 2.33
Anaemia 73 6% 2.03 ± 0.24 2.03 ± 2.05
Anxiety 177 14% 1.29 ± 0.12 1.29 ± 1.53
Depression 167 14% 1.5 ± 0.13 1.5 ± 1.71
Diabetes mellitus 64 5% 1.84 ± 0.29 1.84 ± 2.33
Fibromyalgia 41 3% 1.85 ± 0.34 1.85 ± 2.19
Hepatitis B Virus 22 2% 2.55 ± 0.61 2.55 ± 2.84
Hepatitis C Virus 65 5% 1.92 ± 0.26 1.92 ± 2.11
HIV 35 3% 2.06 ± 0.38 2.06 ± 2.24
Hypertension 150 12% 1.61 ± 0.15 1.61 ± 1.89
Hypercholesterolemia 86 7% 2.48 ± 0.23 2.48 ± 2.13
Ischaemic heart disease 20 2% 3.8 ± 0.57 3.8 ± 2.57
Obesity 69 6% 2.09 ± 0.27 2.09 ± 2.24
Osteoarthritis 78 6% 2.35 ± 0.25 2.35 ± 2.25
Osteoporosis 16 1% 3.63 ± 0.82 3.63 ± 3.28
Rheumatoid arthritis 12 1% 3.67 ± 1.05 3.67 ± 3.65
Other 19 2% 2.11 ± 0.49 2.11 ± 2.13

Mean (standard deviation) and median of minor, major and total bleeds in the previous 12 months by treatment strategy

18–30 31–40 41–50 51–60 61+
N MEDIAN MEAN SD N MEDIAN MEAN SD N MEDIAN MEAN SD N MEDIAN MEAN SD N MEDIAN MEAN SD
Minor Bleeds
PX 217 1 2.37 7.02 - - - - - - - - - - - - - - - -
PXOD 131 2 3.33 3.60 164 2 2.89 3.75 102 3 4.07 4.77 62 3 3.19 3.05 32 2 3.38 4.14
AOD 149 3 4.74 4.88 59 1 2.64 4.00 50 1 3.04 4.59 32 2 3.34 4.88 47 1 1.91 3.02
PPOD 72 2 3.94 4.20 38 2 2.97 3.04 34 2 4.91 10.33 16 2 3.69 4.25 22 2 3.41 2.61
Major Bleeds
PX 217 0 0.24 0.58 - - - - - - - - - - - - - - - -
PXOD 131 0 0.72 1.17 164 0 0.83 1.69 102 0 1.14 2.13 62 1 1.55 2.01 32 0.5 1.22 1.70
AOD 149 0 0.88 1.40 59 0 0.63 1.08 50 0 0.92 1.38 32 1 0.72 0.77 47 1 0.94 1.19
PPOD 72 0 0.83 1.45 38 0 0.82 1.64 34 0 2.00 6.83 16 0.5 0.69 0.79 22 1 1.41 1.87
ABR
PX 217 1 2.61 7.10 - - - - - - - - - - - - - - - -
PXOD 131 3 4.05 4.12 164 2 3.72 5.10 102 3.5 5.21 6.12 62 4 4.74 4.62 32 3 4.59 5.41
AOD 149 4 5.62 5.51 59 2 3.27 4.66 50 2 3.96 5.64 32 2 4.06 5.11 47 2 2.85 4.02
PPOD 72 3 4.78 5.02 38 3 3.79 3.88 34 3 6.91 16.96 16 2 4.38 4.81 22 3 4.82 3.71

Percentage of populations reporting problems for each EQ-5D domain and mean (standard error) of EQ-5D index and EQ-VAS for both the CHESS and HSE sample populations

AGE GROUP POPULATION 18–30 31–40 41–50 51–60 61+
CHESS HSE CHESS HSE CHESS HSE CHESS HSE CHESS HSE
Mobility (n) 210 488 120 477 89 568 51 571 52 1090
  No Problems 78.1% 95.5% 60.8% 94.1% 48.3% 90.0% 41.2% 80.9% 32.7% 68.6%
  Some Problems 21.9% 4.3% 39.2% 5.9% 50.6% 10.0% 54.9% 18.2% 63.5% 31.3%
  Unable/Extreme Problems - 0.2% - - 1.1% - 3.9% 0.9% 3.8% 0.1%
Self-care (n) 206 488 120 477 89 569 50 570 52 1076
  No Problems 92.7% 99.4% 77.5% 98.7% 75.3% 96.1% 56.0% 93.5% 48.1% 89.9%
  Some Problems 7.3% 0.6% 20.8% 1.3% 24.7% 3.5% 40.0% 6.1% 44.2% 9.8%
  Unable/Extreme Problems - - 1.7% - - 0.4% 4.0% 0.4% 7.7% 0.4%
Usual Activities (n) 209 487 120 476 89 569 51 570 52 1085
  No Problems 80.4% 93.6% 68.3% 94.7% 52.8% 88.6% 52.9% 84.7% 48.1% 76.6%
  Some Problems 19.1% 6.2% 30.8% 5.0% 46.1% 10.7% 39.2% 13.5% 44.2% 20.9%
  Unable/Extreme Problems 0.5% 0.2% 0.8% 0.2% 1.1% 0.7% 7.8% 1.8% 7.7% 2.5%
Pain (n) 210 488 120 477 89 569 51 571 52 1090
  No Problems 51.9% 87.3% 31.7% 83.6% 31.5% 71.9% 33.3% 65.5% 34.6% 53.2%
  Some Problems 45.7% 12.1% 65.8% 15.9% 65.2% 25.1% 58.8% 29.2% 57.7% 41.3%
  Unable/Extreme Problems 2.4% 0.6% 2.5% 0.4% 3.4% 3.0% 7.8% 5.3% 7.7% 5.5%
Anxiety/depression (n) 210 487 119 477 89 565 51 570 52 1084
  No Problems 70.0% 86.0% 59.7% 86.8% 55.1% 82.3% 51.0% 79.3% 38.5% 83.0%
  Some Problems 28.1% 12.3% 34.5% 12.6% 37.1% 14.3% 47.1% 17.4% 50.0% 15.1%
  Unable/Extreme Problems 1.9% 1.6% 5.9% 0.6% 7.9% 3.4% 2.0% 3.3% 11.5% 1.8%
EQ-5D Index Score (n) 205 483 119 476 89 564 50 565 52 1065
  Mean (se) 0.82 (0.01) 0.94 (0.01) 0.72 (0.02) 0.94 (0.01) 0.69 (0.03) 0.88 (0.01) 0.63 (0.05) 0.84 (0.01) 0.57 (0.05) 0.81 (0.01)
EQ-VAS (n) 209 427 119 428 89 505 50 510 51 965
  Mean (se) 75.21 (0.99) 81.98 (0.71) 69.29 (1.37) 81.76 (0.67) 64.96 (1.72) 79.84 (0.73) 65.50 (2.49) 76.73 (1.73) 60.92 (2.49) 74.30 (0.62)

j.jhp-2022-0001.tab.002a

AGE GROUP MINOR BLEEDS MAJOR BLEEDS ABR
PX PXOD AOD PPOD PX PXOD AOD PPOD PX PXOD AOD PPOD
18–30
n 217 131 149 72 217 131 149 72 217 131 149 72
Median 1 2 3 2 0 0 0 0 1 3 4 3
Mean 2.37 3.33 4.74 3.94 0.24 0.72 0.88 0.83 2.61 4.05 5.62 4.78
SD 7.02 3.60 4.88 4.20 0.58 1.17 1.40 1.45 7.10 4.12 5.51 5.02
Min 0 0 0 0 0 0 0 0 0 0 0 0
Max 100 30 30 18 4 8 8 8 100 30 38 22
31–40
n - 164 59 38 - 164 59 38 - 164 59 38
Median - 2 1 2 - 0 0 0 - 2 2 3
Mean - 2.89 2.64 2.97 - 0.83 0.63 0.82 - 3.72 3.27 3.79
SD - 3.75 4.00 3.04 - 1.69 1.08 1.64 - 5.10 4.66 3.88
Min - 0 0 0 - 0 0 0 - 0 0 0
Max - 35 20 12 - 12 6 6 - 47 26 15
41–50
n - 102 50 34 - 102 50 34 - 102 50 34
Median - 3 1 2 - 0 0 0 - 3.5 2 3
Mean - 4.07 3.04 4.91 - 1.14 0.92 2.00 - 5.21 3.96 6.91
SD - 4.77 4.59 10.33 - 2.13 1.38 6.83 - 6.12 5.64 16.96
Min - 0 0 0 - 0 0 0 - 0 0 0
Max - 30 20 60 - 15 6 40 - 33 26 100
51–60
n - 62 32 16 - 62 32 16 - 62 32 16
Median - 3 2 2 - 1 1 0.5 - 4 2 2
Mean - 3.19 3.34 3.69 - 1.55 0.72 0.69 - 4.74 4.06 4.38
SD - 3.05 4.88 4.25 - 2.01 0.77 0.79 - 4.62 5.11 4.81
Min - 0 0 0 - 0 0 0 - 0 0 0
Max - 16 24 15 - 10 3 2 - 26 26 16
61+
n - 32 47 22 - 32 47 22 - 32 47 22
Median - 2 1 2 - 0.5 1 1 - 3 2 3
Mean - 3.38 1.91 3.41 - 1.22 0.94 1.41 - 4.59 2.85 4.82
SD - 4.14 3.02 2.61 - 1.70 1.19 1.87 - 5.41 4.02 3.71
Min - 0 0 1 - 0 0 0 - 0 0 1
Max - 16 20 12 - 6 7 8 - 22 27 14

CHESS sample demographics

18–29 30–39 40–49 50–59 60+ P-VALUE
CRF Complete (n) 569 261 186 110 101
Age (mean (SD)) 23.4 (3.77) 35.3 (2.85) 44.9 (2.87) 55.2 (2.76) 67.7 (5.30)
PSC Complete 37% (n=211) 46% (n=120) 48% (n=89) 46% (n=51) 51% (n=52) 0.0064*
Haemophilia subtype
A 77% (n=436) 79% (n=207) 82% (n=153) 71% (n=78) 74% (n=75) 0.1676†
B 23% (n=133) 21% (n=54) 18% (n=33) 29% (n=32) 26% (n=26)
Education Level
None 2% (n=4) 1% (n=1) 3% (n=3) 2% (n=1) 6% (n=3) 0.1493†
Primary 17% (n=35) 17% (n=20) 17% (n=15) 20% (n=10) 19% (n=10)
Secondary 40% (n=85) 37% (n=44) 48% (n=43) 41% (n=21) 42% (n=22)
Undergraduate 25% (n=53) 32% (n=38) 24% (n=21) 24% (n=12) 17% (n=9)
Postgraduate 14% (n=30) 11% (n=13) 6% (n=5) 14% (n=7) 6% (n=3)
Other 2% (n=4) 3% (n=4) 2% (n=2) – 10% (n=5)
Employment
Full-time employed 26% (n=147) 46% (n=121) 44% (n=82) 31% (n=34) 8% (n=8) <0.000†
Part-time employed 13% (n=75) 23% (n=61) 23% (n=43) 16% (n=18) 8% (n=8)
Self-employed 6% (n=32) 12% (n=31) 11% (n=21) 14% (n=15) 12% (n=12)
Student 45% (n=254) 2% (n=5) 1% (n=1) – –
Retired – 0% (n=1) 1% (n=1) 14% (n=15) 63% (n=64)
Unemployed 9% (n=53) 10% (n=25) 10% (n=18) 14% (n=15) 4% (n=4)
Homemaker – 1% (n=2) 2% (n=3) 4% (n=4) 2% (n=2)
Physically Unable Haem Related 1% (n=4) 5% (n=13) 9% (n=16) 6% (n=7) 3% (n=3)
Physically Unable Other Reason 0% (n=2) – 1% (n=1) 2% (n=2) –
Other 0% (n=2) 1% (n=2) – – –
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