Pulmonary arterial hypertension: Nursing support optimizes care

Ms. Aidila Ismail
National Heart Centre Singapore
Ms. Tara Hannon
Respiratory West
Ms. Jane Mitchell
Royal Hobart Hospital
Ms. Kerri-Lee Driver
The Prince Charles Hospital
03 Jan 2022
Pulmonary arterial hypertension: Nursing support optimizes care

Pulmonary arterial hypertension (PAH) is a progressive disease with systemic manifestations and complications, requiring a multidisciplinary team (MDT) management approach. Specialist nurses play a pivotal role in the support and care of PAH patients. At a recent webinar, pulmonary nurse specialists discussed the importance of holistic care, shared their experience in supporting PAH patients on oral selexipag therapy, and provided insights into PAH care during the coronavirus disease 2019 (COVID-19) pandemic.


International guidelines on PAH management

The 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines for PAH recommends the use of orally available selective prostacyclin prostaglandin I2 (IP) receptor agonist (eg, selexipag) in patients with WHO functional class (WHO-FC) II–III, either as part of double or triple sequential combination regimens, or as monotherapy in those who are not candidates for combination therapies. [Rev Esp Cardiol (Engl Ed) 2016;69:177; Selexipag Hong Kong Prescribing Information, February 2021]

“Selexipag [200 µg BID, 12 hours apart] is the first oral prostacyclin pathway agonist that received US FDA approval for long-term treatment of PAH,” noted Ms Aidila Ismail of National Heart Centre Singapore (NHCS), Singapore. [https://www.acc.org/latest-in-cardiology/articles/2015/12/22/11/39/fda-approves-sel-exipag-for-pah-patients]

Selexipag therapy requires uptitration to the individual patient’s highest tolerated dose, with an individualized dose ranging from 200 µg BID to 1,600 µg BID. A stepwise and flexible approach is taken to optimize the titration process and manage side effects. (Figure 1) [Eur Respir Rev 2016;25:418-430]


Selexipag titration with nursing support: Singapore’s experience

PAH specialist nurses play a crucial role in addressing the complexities of titrating selexipag therapy. As such, selexipag treatment should be initiated under the guidance of an experienced PAH MDT.

“The NHCS is the national and regional referral centre for cardiovascular diseases in Singapore, with a MDT experienced in initiating selexipag therapy for a diverse range of PAH populations, including those with idiopathic PAH as well as connective tissue disease and congenital heart disease–associated PAH,” said Ismail. “At our centre, specialist nurses work in partnership with patients to manage their expectations by setting realistic, measurable and achievable treatment goals in relation to their symptoms, functional capacity and clinical response, which improves motivation in continuing with the titration process.”

“A gradual approach in uptitration is the key to minimizing selexipag-related side effects [eg, headache, diarrhoea, nausea and vomiting], which are mostly self-limiting and require minimal intervention. Lowering the dose of selexipag or switching to another prostacyclin pathway agonist could also be considered,” she pointed out.

“Comprehensive patient education is required to ensure patients are well-informed about the purpose and function of selexipag therapy and what to do if a dose is delayed or missed. Patients are also advised to ingest the tablet as a whole at the same time every day with meals,” added Ismail.

“Reassurance and support should be provided to patients through regular follow-up during the titration phase and during long-term treatment,” she emphasized. (Figure 2) [Selexipag Hong Kong Prescribing Information, February 2021]


Holistic approach in PAH care

Advances in PAH knowledge and treatments have resulted in a positive impact on patient care. Currently, challenges in PAH management extend beyond treating physical symptoms. The burden of PAH and its impact on health-related quality of life of both patients and caregivers have become increasingly recognized, as the condition is associated with a significant impact on psychological, social (including financial), emotional and spiritual functioning of patients and their families. [Rev Esp Cardiol (Engl Ed) 2016;69:177]

“Effective management of PAH therefore requires a MDT approach involving various specialists as well as allied healthcare professionals [such as physiotherapists, clinical psychologists, and welfare and social workers] who have the skills and expertise to manage different issues and to meet the diverse needs of patients,” said Ms Tara Hannon of Respiratory West, Perth Western Australia, Australia. (Table) [Nurs Stand 2010;24:40-47] “Furthermore, global and regional PAH patient support groups are available, and patients should be encouraged to join such groups.”


“[In line with the current ESC/ERS guidelines,] effective communication among MDT members is important for optimal PAH care, and all members should be aware of each individual patient’s issues. Close links between MDT members and patient support groups would ensure a good level of understanding of support and services available, enabling appropriate referrals,” pointed out Hannon. [Rev Esp Cardiol (Engl Ed) 2016;69:177; Nurs Stand 2010;24:40-47; https://www. health.org.uk/publications/how-engaged-are-people-in-their-healthcare]

“PAH is a disease that may be severely life-limiting. In addition to psychological and social support, proactive advanced care planning with referral to specialist palliative care services should be made available when appropriate,” she added. [Rev Esp Cardiol (Engl Ed) 2016;69:177]

Moving forward: PAH nursing care in the post–COVID-19 era

“The COVID-19 pandemic has presented many unique challenges towards the care of PAH patients. In Tasmania, the pandemic has altered routine practice and the acute management of PAH patients who are on long-term treatment,” said Ms Jane Mitchell of Royal Hobart Hospital, Tasmania, Australia. [Pulm Circ 2020;10:2045894020920153] “One of the most challenging aspects of PAH care has been to balance the potential risk of being exposed to severe acute respiratory syndrome coronavirus 2 [SARS-CoV-2] in PAH clinics during follow-up visits and new appointments and the need to ensure ongoing care of PAH patients, such as timely medication adjustments.”

“Telemedicine has gained importance in streamlining outpatient PAH care during the pandemic lockdown in Tasmania. Virtual visits have improved touch points with patients and prevented missing signs of worsening clinical and risk status,” Mitchell noted. [Pulm Circ 2020;10:2045894020920153; J Multidiscip Healthc 2020;13:1905-1926]

“Moving forward and beyond the pandemic, adjusting how PAH care is provided with adoption of telemedicine and virtual visits will address continued patient needs despite the everchanging PAH care landscape,” summarized Ms Kerri-Lee Driver of The Prince Charles Hospital, Brisbane, Australia.


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