Advance Care Planning In Adults with Sickle Cell Disease (SCD)
Authors: Bartholomew Frederick , De Castro Laura M. 
Published: October 2019

Abstract
Abstract 391
Advance Care Planning (ACP) is the process by which patients, families and health care practitioners consider their values and goals and express preferences for future care. Written ACP includes living wills, designation of a health care proxy, and do not resuscitate (DNR) orders. ACP provides a legal framework for end-of-life decisions and aligns patient's care with his/her end-of-life decisions if their decision-making ability becomes compromised. The high mortality and potential for unexpected deadly complications among sickle cell disease (SCD) patients, suggests that end-of-life care conversations between patients, family members and providers should be a necessary and helpful aspect of routine health care. Despite the early mortality of SCD patients, there is a lack of information regarding: 1) use of ACP in this population; 2) need for ACP as perceived by patients; and 3) psychosocial impact of ACP information. We therefore performed a study with a three-fold purpose: 1) to determine patient's perceived need for ACP information and their experiences with ACP: thoughts, wishes, and comfort discussing ACP; 2) to explore how an informational intervention designed to teach patients how to understand and complete ACP changes patients' perception of need and comfort discussing ACP and 3) to examine the impact of ACP information on patient perceptions of disease controllability. We hypothesized that when asked about ACP planning, most SCD adults would indicate thinking about ACP issues, but a significant portion of this population would not have completed advance directives and/or indicated specific end-of-life wishes. All 70 subjects -47 (67%) females and 23 males- completed a Pre-Informational Questionnaire (Pre-Q), 60 requested and received the ACP education session, and 62 completed the Post-Informational Questionnaire (Post-Q). Mean age for the patients studied was 38.07 years (females 40.3, males 33.6). Sickle genotypes were as follows: HgbSS / Sβ0thal genotype: 45 (64%), HgbSC 16 (23%), and HgbSβ+thal 9 (13%). Sixty one (87% of the subjects), reported not having written decisions for end-of-life medical treatment. All 9 patients with written decision were females. The mean age was significantly different (p=.0128) between those with written decisions (52 yrs, 32 – 77 yrs) and those without (35 yrs, 18–60 yrs). Fifty-four of those who completed the Post-Q reported not having written ACPs when enrolled in the study. Mean time between completion of Pre-Q and Post-Q was 11.6 weeks (range 3 – 41).Sixty one patients requested ACP information. The mean age of those who did not want ACP information was 34.7, while the mean age of those who did was 38.6. All patients who had written ACPs requested more information.
Variables Pre – Q N=61* Post – Q N=54** Thoughts on whom you would like to make decisions   Very Much 17 (28) 25 (47)   Somehow 29 (47) 23 (43)   Not much 15 (25) 5 (9) Specific wishes about medical treatments   Yes 28 (46) 30 (55)   No 5 (8) 8 (15)   Not sure 28 (46) 16 (30) Comfort level   Taking with Family     A 39 (64) 33 (61)     B 11 (18) 15 (27)     C 3 (5) 4 (8)     D 3 (5) 1 (2)     E 5 (8) 1 (2)   Taking with SCD provider     A 33 (54) 32 (60)     B 19 (30) 16 (30)     C 3 (5) 3 (6)     D 3 (5) 2 (4)     E 5 (8) 1 (2)   Appointing someone     A 28 (46) 26 (48)     B 21 (35) 18 (33)     C 2 (3) 6 (11)     D 5 (8) 2 (4)     E 5 (8) 2 (4) Control   Pain Episodes     A 9 (15) 8 (15)     B 32 (52) 28 (52)     C 15 (24) 14 (25)     D 5 (8) 3 (6)     E 0 1 (2)   Hospitalizations     A 13 (21) 12 (22)     B 33 (54) 29 (54)     C 12 (20) 10 (19)     D 3 (5) 2 (4)     E 0 1 (2)   Need/Use Medications     A 20 (33) 18 (33)     B 28 (46) 26 (48)     C 9 (15) 7 (13)     D 2 (3) 2 (4)     E 2 (3) 1 (2)   Disease Outcome     A 7 (11) 6 (11)     B 34 (55) 34 (62)     C 10 (16) 9 (17)     D 9 (16) 4 (8)     E 1 (2) 1 (2) A=Very B=Somewhat C=Not Very D=Not at All E= Not sure. * Patients without. ACP ** Patients without ACP that completed visit 2.
We conclude that the majority of adult SCD patients studied do not have written advance directives (87%). This represents a large proportion of patients with chronic illness and high health services utilization but with unknown ACP wishes. About half (46%) of patients report having specific wishes about medical treatment if becoming very ill while an equal number are unsure, which shows a dichotomy regarding self-identified ACP decision making. There is a need for increasing SCD patient awareness and use of ACP. Further studies will help define how to address this need in SCD and similar chronically ill populations

Disclosures:
No relevant conflicts of interest to declare.