The Perspectives of Adolescents and Young Adults on Adherence to Prophylaxis in Hemophilia: A Qualitative Study

Figures & data

Table 1 Pre-specified definitions used for sampling variation

Characteristic	Definition
Adherence level^Citation18	Adherent	Missed infusions	0–15%
		Changes in dosing	0–10%
		Changes in timing	0–30%
	Sub optimally adherent	Missed infusions	15–25%
		Changes in dosing	10–25%
		Changes in timing	25–100%
	Non adherent	Missed infusions	25–100%
		Changes in dosing	25–100%
Bleeding history	Occurrence of a bleeding in the last year (diagnosed by physician)
Education	High school (HS), vocational education (VE), higher vocational education (HVE), university (U)

Box 1 Topic list

Experiences with prophylaxis Integration of prophylaxis in daily life Hemophilia related skills Perception and expectations about hemophilia, prophylaxis and adherence Barriers, motivators, and facilitators of prophylaxis Decision making Self-monitoring Social environment/context Parental influence Patient characteristics

Table 2 Demographic and background characteristics (N=18 patients with severe hemophilia)

Age	Interview	Prescribed frequency (times a week)	Prescribed dose (IU)	Adherence level^a	Bleeding last yr.	Education	Daily activity
14	Focus	3.5	1000	+	Yes	High school	School
14	Focus	3	750	+	Yes	High school	School
14	Focus	3.0	1500	+	No	High school	School and work
14	Individual	3.0	1000	+	No	High school	School
15	Focus	3	1000	±	No	High school	School
15	Focus	3.5	1000	±	Yes	High school	School and work
16	Focus	3.5	1500	+	No	High school	School and work
17	Individual	3.5	1500	+	Yes	Voacational	School and work
19	Focus	2	1000	+	No	Voacational	School and work
19	Individual	3.5	1000	+	Yes	Advanced vocational	School
19	Individual	2.0	2000	+	Yes	Advanced vocational	School and work
19	Individual	3.0	1000	+	No	Advanced vocational	School and work
19	Individual	2.0	1000	–	Yes	Voacational	School and work
21	Focus	2.5	1000	±	Yes	Advanced vocational	School and work
21	Individual	2.0	1000	±	No	Voacational	School and work
21	Individual	3.0	2000	–	Yes	Advanced vocational	Work
22	Individual	3.5	1250	+	No	University	School and work
24	Focus	3.0	1000	–	No	Voacational	Work

Note: ^a+ = adherent, ± = sub-optimal adherent, - = non-adherent.

Figure 1 Decision making concerning prophylaxis adherence among AYAs: Treatment responsibility and estimating bleeding risk.

Notes: Phase 1: parents took responsibility for their prophylactic treatment and performed bleeding management phase 2 and 3 increased self-management causing considerations concerning adherence

Table 3 Quotes explaining adherence to prophylactic treatment in adolescents with hemophilia

Treatment responsibility
	Phase 1	Phase 2	Phase 3
Varying treatment responsibilities	I am able to perform self-infusion, yet my parents help me most of the time. My parents take care of any bleeding. I feel that my parents are still responsible for prophylactic treatment. I rely on my parents.	I prepare and infuse myself, yet sometimes my parents remind me or prepare the prophylaxis. I recognize bleeding, yet decision making varies for each bleed. I am gradually taking over the prophylactic treatment or my parentsare gradually transferring the prophylactic treatment to me.	I prepare and infuse myself; my parents are no longer involved. I perform bleeding management myself. I ask advice from my parents only in case of an emergency. I feel fully responsible for prophylactic treatment.
Discussion about adherence with parents	I prefer that my parents support me in adhering to prophylaxis; we sometimes discuss the necessity for prophylaxis, and mostly I agree with my parents.	I have regular discussions with my parents about prophylaxis. My parents are too “careful” with me.	I have no discussions with my parents because they do not know what I am doing or see it as my responsibility.
Adherence level	The AYA is high/fully adherent because of parental supervision.	Adherence levels vary among AYAs: AYA could be adherent or non-adherent.	Adherence levels vary between AYAs: AYA could be adherent or non-adherent.
Estimating risks
Doing what I prefer and feeling safe at the same time	When no bleeding occurred after skipping, it does not motivate me to regularly use prophylaxis. Before engaging in an activity, I judge its intensity and bleeding risk and decide if I require (extra) prophylaxis for that activity. If an activity does not feel safe, even with (extra) prophylaxis, I skip that specific activity.
Bleeding	When bleeding limits me in activities, it motivates me to become more adherent.

Schrijvers LH, Cnossen MH, Beijlevelt-van der Zande M, Peters M, Schuurmans MJ, Fischer K. Defining adherence to propylaxis in haemophilia. Haemophilia. 2016;22:e301–e348. doi:10.1111/hae.12935

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