New challenges for an expanding generation of older persons with haemophilia

O’Hara, Jamie; Noone, Declan; Kritikou, Persefoni; Myren, Karl-Johan; Chaplin, Steve; Hart, Daniel P

doi:10.2478/jhp-2022-0001

Figures & Tables

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

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 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.

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+
AGE GROUP POPULATION	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
AGE GROUP	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)	–	–	–

New challenges for an expanding generation of older persons with haemophilia

Figures & Tables

Figure 1

Figure 2

Figure 3

Prevalence of each comorbid disease and mean number of reported comorbidities

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

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

j_jhp-2022-0001_tab_002a

CHESS sample demographics

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