Purpose Systemic mastocytosis (SM) is a rare group of hematological malignancies with heterogeneous symptoms from various organs, and overall survival ranges from normal for indolent SM (ISM) to 2–4 years for advanced SM subtypes (Adv SM). There is limited knowledge about the experience of living with SM. Thus, the purpose of this study was to describe the experiences of everyday life among persons diagnosed with ISM or Adv SM. 

Methods In this descriptive qualitative study, data were collected through purposive sampling and semi-structured interviews with 16 participants diagnosed with ISM (n = 9) or Adv SM (n = 7). Data were subsequently analysed with content analysis. 

Results Three main categories were identified. The persistent presence of the disease includes findings of how the symptoms affected the participants, how they handled its limitations, and the adaptions and medication management required. Struggling against ignorance illustrates contact with both healthcare professionals and strangers. The participants described being forced to be experts on SM due to a general lack of knowledge. An illness or wellness perspective encompasses the participants’ feelings of vulnerability and alienation, but also how various strategies were used to gain emotional control.

Conclusions 

The disease has a considerable impact on everyday life, with constant efforts required to manage symptoms and medication side effects. The complexity and rarity of the disease complicated relationships with healthcare professionals and contributed to feelings of marginalisation and alienation. The participants described feeling vulnerable but used a multitude of strategies in their striving for resilience. Trusting relationships with healthcare professionals, family members and friends promoted a wellness perspective.