The experience of parents of children with rare diseases when communicating with healthcare professionals: towards an integrative theory of trust.

BACKGROUND: Given the inherent complexity of rare paediatric diseases and the sensitive emotional context of the situations they create (due to the patients' age and the tense uncertainty surrounding the progression of the disease), communication between the adults involved is a key tool in the efforts to provide these children and youths a better quality of life. We conducted ten interviews with families of children with rare diseases, in the aim of exploring how communication between doctors and patients affect their daily lives. All participants, members of FEDER (a Spanish federation of associations of patients with rare diseases) were invited by phone or email to participate in a semi-structured interview including questions on clinical information, communication experiences with healthcare professionals, and the impact these had on the interviewees' relationships with them. To analyse these interviews, we used the 'grounded theory' methodology and open and axial text coding techniques, in addition to those identifying the properties and dimensions of the categories formulated. RESULTS: The core category we have proposed is 'adjustment of mutual trust', with said category describing the attitude and behaviour of doctors who inspire trust in the parents of paediatric patients diagnosed with a rare disease. More specifically, said behaviours or sources of trust are: appearing human, sensitive and empathetic; showing transparency and communicative openness; being supportive of parental proactivity; and being available to families at all times. CONCLUSIONS: Trust is the cornerstone of parent-doctor communication in the field of children with rare diseases. If the sources of trust are present, they create a degree of trust that bolsters both parties in the search for a common goal: providing the child with the best possible care.

Degree of dyspnoea at admission and discharge in patients with heart failure and respiratory diseases.

BACKGROUND: Dyspnoea is a disabling symptom in patients admitted with heart failure (HF) and respiratory diseases (RD). The main aim of this study is to evaluate its intensity at admission and discharge and the relation with quality of life. We also describe its management, intensity, and evolution in HF and RD. METHODS: In this descriptive, cross-sectional study, we included prospectively all patients admitted with decompensated HF and chronic obstructive pulmonary disease (COPD)/pulmonary fibrosis during 4 months. Surveys quantifying dyspnoea (Numerical Rating Scale 1-10) and quality of life (EuroQoL 5d) were administered at discharge. RESULTS: A total of 258 patients were included: 190 (73.6%) with HF and 68 (26.4%) with RD (62 COPD and 6 pulmonary fibrosis). Mean age was 74.0±1.2 years, and 157 (60.6%) were men. Dyspnoea before admission was 7.5±0.1. Patients with RD showed greater dyspnoea than those with HF both before admission (8.1±0.2 vs. 7.3±0.2, p=0.01) and at discharge (3.2±0.3 vs. 2.0±0.2, p=0.0001). They also presented a higher rate of severe dyspnoea (≥5) at discharge (23 [34.3%] vs. 36 [19.1%], p=0.02). Opioids were used in 41 (15.9%), mean dose 8.7±0.8 mg Morphine Equivalent Daily Dose. HF patients had worse EuroQoL 5d scores than those with RD, due to mobility problems (118 [62.1%] vs. 28 [41.8%], p=0.004), and lower punctuation in Visual Analogue Scale (57.9±1.6 vs. 65.6±1.0, p=0.006). CONCLUSIONS: About a quarter of patients admitted with HF or RD persist with severe dyspnoea at discharge. Opioids are probably underused. HF patients have less dyspnoea than patients with RD but present worse quality of life.