A total of 62 scientific publications were initially considered; ultimately, 20 studies were selected that included information about the guide of treatment for patients with PAH, patient quality of life in rare diseases, or aspects related to patient experiences.

A total of 16 female participants diagnosed with PAH participated in the study, with ages ranging from 40 to 68 years. Two participants had been enrolled in the support program before 2020, and all had been diagnosed with the condition for at least 10 years. Each association provided three participants. In total, two focus groups with nine participants each were organized; however, owing to absences, only eight participants attended the session.

The 'Patient Journey' of the participants in the support program was analyzed, identifying three key phases: pre-enrollment experiences, the process of joining the program, and the subsequent follow-up.

Before their incorporation into the PAH support program, the patients reported being diagnosed after experiencing critical situations and urgent hospitalization. Direct instructions by program professionals in the hospital on the use of the infusion pump and medication were mentioned. However, there were cases where the primary care physician had limited knowledge of the disease and the program and situations where patients or their families had to manage complications on their own. Information about the program was often received at the hospital, and some expressed the usefulness of the after-hour support provided by program professionals.

Once enrolled, participants emphasized the importance of receiving detailed information and educational materials on infusion pump use, pain management, and infection prevention. They mentioned variability in the pain experience and pain management strategies, such as the use of paracetamol and anti-inflammatory drugs. The duration between the point changes and psychological management of pain anticipation was also noted. Some expressed the need for additional support in managing pediatric patients, especially at point changes, and the usefulness of direct contact with program professionals to facilitate adaptation.

During follow-up within the program, several areas for improvement were identified: the need for more training of healthcare personnel on the disease and treatment, the lack of nurses specializing in the management of PAH, and the need for specific training in schools to support pediatric patients. In addition, the importance of providing psychological support proactively was emphasized, and the need to empower patients to take a more active role in their care and reduce dependence on specific advice from professionals was highlighted.

A total of 36 patients with PAH participated in this study (Table 1).

Regarding patients' experiences with primary care professionals (P1), 26.5% of the respondents stated that they always received informed and appropriate care, while another 26.5% indicated that this only happened occasionally. Regarding coordination between the different levels of healthcare (P2), 45.7% of the patients reported that there was always good communication between professionals, suggesting effective collaboration in their care.

With respect to the knowledge and assistance provided by emergency departments (Q3), 25% of the respondents always had knowledgeable staff and adequate assistance, compared with 18.7% who rarely or never had this experience.

Regarding the information provided by hospital professionals about the disease and its treatment (Q4), 65.7% of the patients reported that they were always given all the necessary information, compared with 2.86% who never received it. With respect to the information provided by health center professionals (Q5), 9.09% of the respondents stated that they always received all the necessary information, whereas 39.39% never received it.

Regarding information on the patient support program at the hospital where treatment was indicated (Q6), 63.6% of patients stated that they were always informed about the program, compared with 24.2% who reported never receiving such information.

A total of 75.8% of patients reported that the hospital or service where they were instructed on treatment and infusion pump use (P7) connected them to the referral person for the PAH support program. However, 18.2% reported not receiving this referral. Furthermore, 52.9% had a preliminary meeting with their physician and nurse before joining the support program (Q8), whereas 44.1% did not. On the other hand, 33.3% of respondents felt insecure and had difficulty accessing information before entering the support program (Q9), while 22.2% did not experience such difficulties.

Table 2 shows the patients’ views regarding the process of information and contact with the support program for patients with PAH and the information provided for pump management.

Most survey participants rated the support received through the patient support program positively. In response to question Q13, 80% stated that the staff provided them with useful information to prevent and address complications and side effects of the treatment. Regarding question Q14, 50% of the respondents indicated that thanks to the program, they avoided emergency room visits or long waits for medical consultations. In question Q15, 68.6% expressed feelings of being in control and were able to face problems related to their disease owing to the help provided by the program.

In the context of the support provided by professionals in the support program for patients with PAH (Q16), an overwhelming 91.7% of the patients stated that they always received the necessary help in the event of any doubt or complications. In emergencies related to their disease (Q17), more than half (58.3%) preferred to go to support program professionals rather than to the emergency department. In addition, a significant majority (63.9%) of respondents felt that without the support program, they would feel helpless and would be forced to resort more often to emergency services to resolve problems or complications (Q18).

Regarding the emotional assistance provided by the psychologist in the support program (Q19), a significant majority (51.6%) of the patients stated that this help enabled them to overcome the emotional impact of the disease. Regarding communication and coordination between professionals in the support program and those in the hospital (Q20), 58.3% perceived effective collaboration between the two. Finally, in relation to the care received in the hospital and the availability of contact in case of problems (Q21), 63.9% of the respondents felt that healthcare professionals were attentive to their needs and accessible for consultations about the disease or use of the pump.

Patients in the support program suggested some key areas for improvement: they highlighted the importance of psychological care, even if they did not use it frequently; proposed the inclusion of group psychotherapy to improve psychological care; requested more information about possible side effects before starting treatment; and expressed the need for more knowledge about all services offered by the program.