The Impact of Inherited Retinal Diseases in the Republic of Ireland (ROI) and the United Kingdom (UK) from a Cost-of-Illness Perspective

Figures & data

Table 1 Estimated Prevalent Cases (Number of People) with IRDs in ROI and UK

Region	Republic of Ireland		United Kingdom
IRD	Base Cases	Upper Band	Base Cases	Upper Band
Retinitis Pigmentosa	755	1274	10,324	17,422
Usher Syndrome	189	320	2590	4371
Stargardt Disease	154	260	2109	3559
LCA/EOSRD	116	196	1587	2679
Best Disease	73	124	1003	1693
Cone Dystrophy	64	108	876	1478
Cone Rod Dystrophy	59	100	812	1370
Achromatopsia	54	90	733	1237
X-linked Retinoschisis	35	60	483	814
Choroideremia	22	37	298	504
Total	1,521	2,569	20,815	35,126

Table 2 Survey Responses in Relation to Genetic Testing and Genetic Counselling

Survey Responses in Relation to Genetic Testing and Genetic Counselling	ROI	UK
Received a genetic test in their lifetime*	59.2%	56.6%
Never received genetic counselling	69.7%	50.9%
Received genetic test in last 12 months*	22.2%	26.7%
Received genetic counselling in last 12 months	10.9%	8.3%
Family member received a genetic test*	66.0%	24.5%
Family member received genetic counselling	33.0%	14.8%

Note: *Undergoing a genetic test does not indicate receipt of a genetic test result.

Table 3 Cost Associated with Individual IRDs Based on Base Case Prevalence Estimates

Region	ROI	UK
IRD	£ Million	£ Million
RP	21.4	262.3
Usher syndrome	5.3	64.9
Stargardt disease	4.2	52.0
LCA/EOSRD	3.1	38.5
Best disease	2.0	24.8
Cone dystrophy	1.8	22.1
Cone-rod dystrophy	1.6	20.5
Achromatopsia	1.5	18.1
XLRS	1.0	12.6
Choroideremia	0.6	7.5
Total	42.6	523.3

Table 4 Breakdown of Cost Type Based on Base Case Prevalence Estimates

Region	ROI		UK
Cost Type	% Total	£ Million	% Total	£ Million
WellbeingCosts	32.3	13.8	37.5	196.1
Productivity Costs	18.9	8.1	21.8	114.1
Deadweight losses	16.6	7.1	5.5	28.7
Informal Carer Costs	13.6	5.8	16.1	84.5
Other Costs	14.2	6.0	14.3	74.9
Health System Costs	4.5	1.9	4.8	25.0
Total	100	42.6	100	523.3

Table 5 Cost of Wellbeing Associated with IRDs in the ROI and the UK

Region	ROI	UK
Per Person Cost	£9,023	£9,421
Total Cost	£13.8 Million	£196.1 Million

Table 6 IRD Impact on Mental Wellbeing of Persons Living with an IRD

Region	ROI	UK
Wellbeing state occurring as a direct result of IRD	Percentage (%) of respondents	Percentage (%) of respondents
Anxiety	85.5	86.8
Depression	63.2	67.9
Social Isolation	44.7	56.6
Financial Stress	43.4	45.3
Other (fear, stress, fatigue, hopelessness, loneliness, panic attacks, suicidal thoughts and the development of tics)	7.9	13.2

Table 7 IRD Impact on Mental Wellbeing of Parents/Guardian of Children Under 18 Living with an IRD

Region	ROI	UK
Wellbeing State Occurring as a Direct Result of IRD	Percentage (%) of Respondents	Percentage (%) of Respondents
Anxiety	75	77.8
Depression	50	77.8
Other (stress, loneliness and concerns for the safety of their child)	8.3	22.2

Table 8 Working or Student Status of Survey Respondents in the ROI and the UK

Working or Student Status	ROI	UK
Full-Time	30.5%	4.5%
Part-Time	9.5%	29.5%
Casual Work	5.8%	2.3%
Underemployed	1.9%	15.9%
Not Employed and not looking for work	19.2%	–
Retired	23.1%	38.6%
Students	9.6%	9.1%

Table 9 Health System Costs by Type in ROI and UK

Health System Type	ROI (Million)	UK (Million)
Primary Health Care	£0.6	£3.4
Secondary Health Care	£0.7	£9.0
Diagnostic Tests	£0.3	£4.2
Pharmaceuticals	£0.2	£4.6
Vitamins and Supplements	£0.1	£0.7
Medical Research	–	£3.1
Total	£1.9	£25.0

Figure 1 (A) Payers in the Republic of Ireland. (B) Payers in the United Kingdom.