Evidence indicates that caregivers of children with primary immunodeficiency disorders (PIDs) encounter profound psychological anxiety as their children undergo the hematopoietic cell transplantation (HCT) process. Volatile medical symptoms in the child, uncertainty about the child’s outcome, and feeling ill prepared as a caregiver often lead to caregiver psychosocial distress. In fact, many caregivers ultimately suffer from posttraumatic stress symptoms and depression. Prior studies suggest that immediately before HCT and during hospital discharge are particularly high-distress periods, thereby allowing for targeted interventions. “There is little known about how healthcare delivery systems could better address these needs,” says Dr. Christina Mangurian, MD, MAS. This often results in “missed opportunities to provide psychosocial support to these caregivers.” 

Taking a Deeper Look

For a study published in the Journal of Allergy and Clinical Immunology, Dr. Mangurian, Jennie Yoo, MD, and colleagues analyzed the well-being of caregivers for children with PIDs, determining what common support systems and organizational-level factors might ease caregiver distress. From August through November 2017, they contacted caregivers via Immune Deficiency Foundation email lists, online Facebook groups for PID families, and PID research groups. Participating caregivers were at least 18 years old, had a child with a PID, had a child who received HCT when younger than 5 years old, resided with the child during HCT, had HCT performed in the US or Canada, and came from an English-speaking family.

“Caregivers were invited to participate in a confidential online survey composed of questions assessing demographic and medical variables, use of psychosocial support systems, well-being, and open-ended questions,” says Yoo. This provides an understanding of “caregiver perspectives on potential organizational solutions to enhance well-being.”

Among respondents, caregivers had a median age of 34 years (range, 23-62 years). They were mostly female (98%), Caucasian (87%), married/living together (88%), and had additional children (65%). Of affected children, 79% were male. They had a median age of 5 months (range, 1-44 months), and 74% were diagnosed with severe combined immune deficiency (SCID).

Examining Key Findings

The study showed that caregivers largely depend on the medical care team (70%) and extended family (63%) for support. However, they also greatly rely on online support groups (45%), close friends (40%), and the religious community (29%).

“Caregivers of PID children experience a great deal of emotional stress during the HCT hospitalization and need support,” says Dr. Mangurian. “This hospitalization is a strain to personal relationships of caregivers, including caregivers’ other healthy children (lack of sibling visitation) and partners (geographic distance, sharing of responsibilities). Often, caregivers have difficulty managing their own self-care, including disruption of sleep.”

An adjusted multivariate linear regression model showing post-HCT influences on caregiver well-being is included in the Table. Results demonstrated that five predictors (age, income, insurance, hospitalization length, and complications) accounted for 33% of the variance in well-being. “We found that caregivers who were low income, had children with complications after HCT, or both experienced significantly lower well-being after hospitalization,” says Ms. Yoo, signifying that particular high-risk subpopulations of caregivers might require more targeted psychosocial support. “Further research is needed to assess the challenges and needs of populations with limited English proficiency and/or minority populations, in addition to families who experienced fatal outcomes.”

Assessing Implications

Caregivers offered up a number of organizational-level solutions to assist psychosocial support, including respite services, online PID-caregiver groups, and bedside mental health services. Hospital-based respite services allow “time for caregivers to manage their self-care and/or attend to other duties,” says Yoo. “Caregivers also requested more frequent check-ins from their healthcare team and more time with providers to discuss medical decisions.” Many opportunities exist to address low-hanging fruit at the organizational level, according to Dr. Mangurian. “A few key suggestions include screening caregivers for depression and targeting increased support for caregivers with additional social stressors and more complicated medical courses,” she notes. Additionally, revising hospital policies to allow for more than one caregiver to stay overnight would significantly improve caregiver well-being by strengthening relationships both with healthy siblings and with a spouse/partner.