PULMONARY ARTERIAL HYPERTENSION (PAH) FACT SHEET
WHAT IS PULMONARY ARTERIAL HYPERTENSION (PAH)?
· Pulmonary arterial hypertension (PAH) is a rare but very complex and serious lung disease, which is progressive and potentially fatal.
· In PAH the arteries of the lungs become closed off or scarred, impeding blood flow resulting in continuous high blood pressure in the lungs.[i]
· It is estimated that more than 5,000 Canadians have been diagnosed with Pulmonary hypertension (PH) but as many as 10,000 may be affected by the condition.[ii]
· PAH is the most severe form of PH and can lead to heart failure and death.
· PAH is a chronic condition for which there is currently no known cure.[iii]
· Canadians diagnosed with PAH live an average of two to three years if left untreated.[iv]
SYMPTOMS & DIAGNOSIS
· PAH can strike at any time and has no regard for race or age. Physical symptoms can include:[v]
o Breathlessness or shortness of breath, especially with activity;
o Fainting and/or feeling tired all the time;
o Lightheadedness, especially when climbing stairs or standing up;
o Swollen ankles, legs, or abdomen; and
o Chest pain, especially during physical activity.
· PAH shares many symptoms with other diseases. This often leads to lengthy delays in diagnosis and misdiagnosis.
· To establish a diagnosis of PAH and to assess its severity, a series of tests are performed that show how well a person’s heart and lungs are working. These tests may include:[vi]
o Echocardiogram (ultrasound of the heart);
o Right heart catheterization (direct measurement of pressure in the pulmonary arteries and determination of how well the heart is pumping blood to the body); and
o Six-minute walk test to assess exercise capacity.
PAH & SCLERODERMA[vii]
· PAH is a common complication of scleroderma, a chronic hardening and contraction of the skin and connective tissue.
· Virtually all persons with scleroderma have some loss of lung function in the forms of pulmonary fibrosis and/or pulmonary hypertension.
· Unfortunately, those living with scleroderma have an increased risk of developing PAH because of the significant fibrosis of their lungs, which can in turn reduce the blood oxygen levels and cause a reflex of increased blood pressure in the pulmonary arteries.
TREATMENT OPTIONS
· Since 1997, nine PH-specific treatments have been approved in Canada and, thanks to these advancements, many patients are living longer and healthier lives.[viii]
· Despite available treatments, rates of hospitalization for pulmonary hypertension have increased by 44 per cent during the past decade.[ix]
· Innovative new therapies that are now available in Canada or are under investigation in clinical trials are bringing hope to PAH patients for better outcomes and improved quality of life.
· PAH is a complex and progressive disease, which requires specialized PAH physicians the ability to prescribe the treatments they believe will result in optimal short-term and long-term outcomes for their patients.
· If all else fails, transplantation of the lungs, or both the heart and lungs may be considered.
· In a recent survey conducted by PHA Canada, one-third of patients reported they already felt there were insufficient medication options to manage their PH.[x]
· In November 2013, Health Canada’s approval of Opsumit (macitentan) offered new hope for adult PAH patients and their physicians.[xi]
o Opsumit is the only oral treatment proven in a randomized controlled trial to reduce long-term morbidity in patients, including reductions in PAH worsening and hospitalizations.[xii]
ACCESS TO TREATMENT
· In January 2015, the Common Drug Review (CDR) recommended that Opsumit be publicly funded (with criteria and a condition) for “the long-term treatment of PAH to reduce morbidity in patients with World Health Organization (WHO) Functional Class II or III.”[xiii]
· Since the end of March 2015, Opsumit has been the subject of provincial funding negotiations through the Pan-Canadian Pharmaceutical Alliance (pCPA), further prolonging patients’ wait for access to treatment.[xiv]
· The Institut national d'excellence en santé et services sociaux (INESSS) in Quebec recommended that Opsumit be added to the list of drugs covered by the prescription drug insurance plan administered by the Régie de l'assurance maladie du Québec (RAMQ) and since October 1, 2014, the drug has been reimbursed in Quebec.[xv]
· In the rest of Canada, only PAH patients with private health insurance are able to access Opsumit, while those who rely on public drug plans cannot.
References
For more information visit www.PHACanada.ca or www.SclerodermaOntario.ca
[i] Pulmonary Hypertension Association of Canada. About PH; Learn More. Retrieved from http://www.phacanada.ca/en/about-ph/learn-more/.
[ii] Pulmonary Hypertension Association of Canada. About PH. Retrieved from http://www.phacanada.ca/en/about-ph/.
[iii] Living with PH. The Disease; 16. Will I die of PH? Retrieved from http://livingwithph.ca/disease_16.htm.
[iv] Pulmonary Hypertension Association of Canada, Toronto Chapter. About PH. Retrieved from http://phatoronto.ca/aboutph.html
[v] Living with PH. The Disease; 13. What are the symptoms of PH? Retrieved from http://www.livingwithph.ca/disease_13.htm.
[vi] Pulmonary Hypertension Association of Canada. (2009). Helpful Information for Patients and Families [Brochure]. Vancouver, BC: PHA Canada.
[vii] Scleroderma Society of Canada. Scleroderma and Pulmonary Arterial Hypertension. Retrieved from http://www.scleroderma.ca/pdf%20pages/SSO_Pulmonary_Arterial_Hypertension.pdf
[viii] Pulmonary Hypertension Association of Canada. About PH; Learn More. Retrieved from http://www.phacanada.ca/en/about-ph/learn-more/.
[ix] George M, Schieb L, Ayala C, Talwalkar A and Levant S (2014). United States, 2001–2010. Pulmonary Hypertension Surveillance. 146(2). pp. 476-495. Retrieved from http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4122278/pdf/chest_146_2_476.pdf.
[x] Information obtained through the Burden of Illness Survey undertaken by the Pulmonary Hypertension Association of Canada in October 2013.
[xi] Health Canada. Drugs and Health Products: OPSUMIT. Retrieved from http://www.hc-sc.gc.ca/dhp-mps/prodpharma/sbd-smd/drug-med/sbd_smd_2013_opsumit_161372-eng.php
[xii] Pulido T, Adzerikho I, Channick RN, Delcroix M, Galiè N, Ghofrani H-A, et al (2013). Macitentan and Morbidity and Mortality in Pulmonary Arterial Hypertension. New England Journal of Medicine. 369(9). pp.809-818. Retrieved from http://www.nejm.org/doi/pdf/10.1056/NEJMoa1213917.
[xiii] Canadian Agency for Drugs and Technologies in Health. Common Drug Review. CDEC FINAL RECCOMENDATION–Macitentan (Opsumit–Actelion Pharmaceuticals Canada Inc.) Indication: Pulmonary Arterial Hypertension. January 28, 2015. Retrieved from https://www.cadth.ca/sites/default/files/cdr/complete/cdr_complete_SR0364_Opsumit_Jan-30-15.pdf.
[xiv] Pan-Canadian Pharmaceutical Alliance: Active Negotiations. Retrieved from http://www.pmprovincesterritoires.ca/phocadownload/pcpa/pcpa_active_negotiations_march31_2015.pdf.
[xv] Régie de l'assurance maladie du Québec (RAMQ). List of Medications. Retrieved from https://www.prod.ramq.gouv.qc.ca/DPI/PO/Commun/PDF/Liste_Med/Liste_Med/liste_med_cor1_2015_10_01_en.pdf.