Life quality with iTTP remains lower, even in remission: Survey
Scientists note need for psychological support beyond disease's acute phase
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Adults with immune-mediated thrombotic thrombocytopenic purpura (iTTP) who are in remission experience worse health-related quality of life (HRQoL), anxiety, and depression than the general population, according to a French survey study.
“Our results highlight the importance of more systematic psychological care and support for patients with iTTP after the acute phase of the disease,” the scientists wrote.
Older iTTP patients experienced better HRQoL and lower anxiety and severe anxiety than their younger counterparts, the data showed. Treatment with Cablivi (caplacizumab-yhdp) during the acute phase of disease did not affect HRQoL or mood disorders while in remission, though it was linked to lower severe anxiety.
The survey study, “Health-related quality of life and mental health in autoimmune thrombotic thrombocytopenic purpura patients in the caplacizumab era,” was published in Research and Practice in Thrombosis and Haemostasis.
In iTTP, also called acquired TTP, self-directed antibodies attack ADAMTS13, an enzyme that normally prevents platelets from forming clots when they aren’t needed. Without sufficient ADAMTS13 activity, platelets start to form small clots within blood vessels, which can lead to organ damage, red blood cell destruction, and low platelet counts.
Cablivi benefits include shorter hospital stays
Current iTTP treatments include therapeutic plasma exchange, immunosuppressive glucocorticoids, and rituximab, which depletes antibody-producing B cells.
Cablivi is an approved iTTP therapy sold by Sanofi that’s designed to block platelet aggregation. Cablivi treatment has led to shorter hospitalizations, suggesting a beneficial effect on health-related quality of life (HRQoL).
Despite these advances, many iTTP patients may struggle with mental health problems and lower HRQoL.
The research team surveyed 101 iTTP patients, ages 38-62, who were in remission achieved by daily therapeutic plasma exchange. Some patients also received glucocorticoids and rituximab. About half (44.5%) had received Cablivi for active disease. A group of 76 healthy people served as controls.
Participants completed the Short Form-36 (SF-36) to assess HRQoL, the Hospital Anxiety and Depression Scale (HADS) for anxiety and depression, and the Posttraumatic Stress Disorder Checklist (PCL-5) for posttraumatic stress disorder (PTSD).
The researchers found that people with iTTP had significantly reduced HRQoL, as indicated by lower scores across all eight SF-36 domains, than healthy controls. Domains affected were physical functioning, role limitations due to physical problems, pain, general health perception, vitality, social functioning, role limitations due to emotional issues, and mental health.
A physical composite score combining physical functioning, role limitations, pain, and general health was also significantly lower among iTTP patients. Similar results were observed for a mental composite score (MCS), which combined vitality, social functioning, emotional role limitations, and mental health.
Although prior Cablivi treatment did not affect any SF-36 survey results, older age was associated with improved general health perceptions, vitality, mental health, and MCS scores.
“Patients with more advanced age reported more favorable outcomes, which could result from more resilience in older patients,” the researchers said.
Based on the HADS questionnaire, iTTP patients had worse anxiety and depression than healthy controls, as indicated by higher median scores. More than half (60%) of patients with iTTP had mild anxiety or worse, while less than half (40%) had at least mild depression. One-sixth (17%) experienced severe anxiety, while one in 25 (4%) had severe depression.
The researchers noted that the estimated incidence of mood disorders in the general French population is about three times lower for anxiety (21.6%) and depression (14%) than in iTTP patients surveyed.
Anxiety and depression scores were comparable between those who had been treated with Cablivi and those who hadn’t. Yet severe anxiety was significantly less prevalent in patients who had received Cablivi.
“The use of [Cablivi] during the acute phase could be associated with less severe anxiety in iTTP survivors, possibly because of the decrease in the burden of care provided by [Cablivi],” the team noted.
When the team assessed possible risk factors, advanced age was associated with reduced anxiety. Previous ADAMTS-13 relapses, or a drop in enzyme activity, were also linked with less anxiety, “possibly following a growing familiarization of patients with their disease,” the team noted. And the time at which ADAMTS-13 activity was at least 20% before the study was not associated with anxiety.
Severe anxiety, however, was tied to younger age and no previous ADAMTS-13 relapses, and worse depression was related to a shorter time since diagnosis.
About half of the patients had a PCL-5 score of at least 43, which was suggestive of posttraumatic stress. Cablivi treatment did not affect PTSD scores, and no related risk factors could be identified.
“We confirm from a large cohort of well-characterized population the high incidence of anxiety, depression, PTSD, and decreased HRQoL in iTTP survivors,” the authors concluded. “Furthermore, we showed that [Cablivi] is associated with decreased risk of severe anxiety.”
