Unmet Needs of Immune Thrombotic Thrombocytopenic Purpura (iTTP)-Specific Pregnancy Counseling, Care and Outcomes in Women with Ittp

Unmet needs of iTTP-specific pregnancy counseling, care and outcomes in women with iTTP

INTRODUCTION: Pregnancy is a known trigger for an initial episode or relapse of immune thrombotic thrombocytopenic purpura (iTTP) and outcomes of pregnancy in women with iTTP have historically been suboptimal with high rates of recurrent iTTP, fetal loss, preeclampsia and HELLP syndrome. More recent data suggest that prophylactic immunosuppression to normalize ADAMTS13 levels before conception, and ADAMTS13 monitoring and prophylactic steroids or plasma exchange during pregnancy can improve maternal and fetal outcomes. However, it is not clear whether women with iTTP are routinely counseled regarding these options. We conducted this study to understand the experiences of women with iTTP with reproductive healthcare, how iTTP has impacted their reproductive decisions, and outcomes of pregnancy.

METHODS: We developed a web-based (RedCap) survey focused on the reproductive healthcare experiences of women with iTTP with in three major sections: (1) counseling and education around pregnancy that was provided to them, (2) the impact of iTTP on their reproductive decisions, and (3) outcomes of any pregnancies they had experienced. The survey was distributed through social media and email mailing lists of a patient advocacy organization and was kept open for one month (January 2023). Survey data were summarized using counts and percentages. The focus of the analysis was to identify gaps in patient education and care where efforts could be focused on educating clinicians or providing information to patients though patient advocacy groups.

RESULTS: Seventy-five females with iTTP responded to the survey. The median age of the respondents was 44 years (range 23 – 65). Sixty-five (74.7%) identified as White, 13 (14.9%) as Black, 6 (6.9%) as Asian and 3 (3.4%) identified as other/prefer not to say. Nearly all (94.6%) were followed by a hematologist or hematologist-oncologist and 82.9% reported being monitored regularly for iTTP. Only 26.7% (20 of 75) reported that their doctor talked to them about measures that may help achieve a successful pregnancy. These included ADAMTS13 monitoring (n=15), plasma exchange treatments (n=10), rituximab (n=10), and aspirin (n=3) (options are not mutually exclusive so total is > 20). Moreover, 30.0% (21 of 70) reported that they were advised by their doctor to not get pregnant due to their iTTP. Some patients (36.0%) avoided pregnancy out of fear of the consequences of iTTP and 28.4% (21 of 74) responded that their iTTP diagnosis has affected their ability to get into a relationship, which impacts their quality of life (Figure 1).

Fifty-three patients reported that they had had at least one pregnancy. Of these, 35 had pregnancies before iTTP diagnosis, 10 with the initial presentation of iTTP, and 8 women had a total of 11 pregnancies following the diagnosis of iTTP. Consistent with prior reports, there was a high rate of maternal and fetal complications in pregnancies that occurred in the setting of a first iTTP episode complicating pregnancy, or pregnancies after an iTTP diagnosis (Table 1). iTTP relapse occurred in 27.3% (3 of 11) pregnancies in women with prior iTTP. Fetal loss occurred in 30% of pregnancies with first TTP diagnosis and 9% of pregnancies after iTTP. In both groups, approximately 20% were small for gestational age. Preeclampsia or HELLP syndrome occurred in 50% and 18.2% of pregnancies complicated by initial TTP diagnosis or after iTTP diagnosis, respectively.

CONCLUSION: Though pregnancy in women with iTTP is high risk, close monitoring and prophylactic interventions can improve outcomes. While many women with iTTP are counseled regarding the risks of pregnancy, few receive counseling regarding their options to potentially improve pregnancy outcomes. This limits their reproductive choices and impacts their relationships. Educating clinicians and providing information to patients though patient advocacy groups may help address these healthcare gaps.