Actelion Pharmaceuticals UK has announced that NHS patients in Wales, who have a rare and incurable disease, are now able to access selexipag, a new twice daily tablet for patients with pulmonary arterial hypertension (PAH).
PAH is a devastating disease that causes an increase in blood pressure in the blood vessels between the right side of the heart and lungs. In its final appraisal, the All Wales Medicines Strategy Group (AWMSG) recommended the use of selexipag as an option for restricted use within NHS Wales as a triple combination therapy for the treatment of PAH. Specifically, selexipag will be made available for adult patients with one of the more severe forms of the disease (World Health Organisation [WHO] functional class [FC] III) who are insufficiently controlled on oral treatment with two other classes of PAH medicines. The decision now brings access in Wales in line with those patients already benefiting in Scotland.
It is essential that patients receive the best quality and evidence-based care for this debilitating, life-shortening disease
Current therapies that target an integral pathway of the disease, known as the prostacyclin pathway, whilst effective, can be difficult for patients to administer. For example, inhaled iloprost requires the use of a nebuliser up to nine times a day which can be difficult for patients to prepare and take. Due to the nature of these treatments, over 90% of FC III PAH patients do not currently receive prostanoid treatment before they die.
The recommendation from the AWMSG is based on an assessment of evidence from
Actelion which is reviewed by the New Medicines Group, alongside advice from patients and
independent clinicians. The clinical effectiveness and price of the medicine, which group of
patients would benefit, and how it compares to currently available treatments is considered.
The GRIPHON trial found that selexipag was significantly better than placebo as measured by a composite primary outcome of complications related to PAH or death. A 40% reduction in the risk of a primary outcome event was seen in patients treated with selexipag. This was driven by a reduction in hospitalisations for PAH and events related to progression of the disease.
Dr. Luke Howard, Consultant Pulmonologist, London Imperial College Healthcare NHS Trust said, “The announcement that selexipag will be funded on the NHS in Wales is another step-change in the treatment of PAH. This devastating condition can affect patients in the prime of their life.”
Dr Iain Armstrong, Chair, Pulmonary Hypertension Association UK commented, “The symptom burden of pulmonary hypertension is substantial, so access to any drug that can improve outcomes for these patients is vitally important for their health and wellbeing. It is essential that PH patients receive the best quality and evidence-based care for this debilitating, life-shortening disease.”