PROVIDING SPECIALIST CARE FOR PATIENTS WITH PULMONARY HYPERTENSION

Pulmonary hypertension is a progressive, life-shortening condition which, untreated, has a survival worse than some common cancers.1–3

David Kiely is a respiratory physician and professor of pulmonary vascular medicine in Sheffield. He has worked with patients with pulmonary hypertension (PH) for over 20 years.

For many patients with underlying cardiac and respiratory disease, treatment is currently aimed at the underlying condition but therapies now exist for specific forms of PH.For patients with pulmonary arterial hypertension (PAH), drug therapies have significantly improved outcomes,4 and patients with chronic thromboembolic pulmonary hypertension (CTEPH) can potentially be cured by pulmonary endarterectomy.5

 

Recognising the challenge of providing care for patients with a rare illness, a UK network of Pulmonary Hypertension Centres was commissioned in 2001 to provide diagnostic services for patients with suspected severe PH and specialist care for patients with PAH and CTEPH.

 

There are seven adult centres in the UK; Glasgow, Newcastle, Sheffield, Cambridge and three centres based in London. There is also one centre for children in London at Great Ormond Street Hospital.6,7 These centres work closely with other hospitals and specialist services throughout the UK to provide patient-centred care.

Location of the UK Specialist PH Centres – Click here for contact details

These specialist centres provide a range of diagnostic services, including right heart catheterisation to confirm PH, as well as therapies and support for patients by working closely with local healthcare professionals and the UK patient association, Pulmonary Hypertension Association UK (PHA UK).

 

In addition to managing complex drug therapies, centres provide on-going support for patients using a multi-professional approach and adhering to national standards of care.6 Importantly, the national standards of care are based on priorities identified by patients and people affected by PH. These focus on timelines of referral and initiation of treatment, ensuring a clear diagnosis is established and collecting data reflecting the impact of the disease on people’s lives, such as quality of life measures, and assessments reflecting disease severity.8 By developing ambitious national standards, the UK centres are all working towards improving the quality of care they provide for patients and people affected by PH. Performance against national standards is published annually and centres are subject to annual peer review.6

 

Given that patients spend the majority of their time at home, it is crucial that specialist centres work closely with local healthcare professionals and are responsive to the needs of patients. The multi-professional team includes nurses, doctors, pharmacists, physiotherapists, social workers and many others. Each hospital has a 24-hour on-call service and patients are managed in specialist units in each hospital.6

 

Concentrating patients with a rare disease in specialist centres has facilitated research into this illness. The UK centres have played an important role in participating in international multicentre studies that have seen the development of a number of new therapies for patients over the last 20 years.9–13 The UK PH Clinical Trials Network has also initiated investigator-led clinical trials evaluating novel therapies. Working collaboratively, and with major funding from the Medical Research Council, British Heart Foundation and National Institute for Health Research, the UK has established a PH biobank with samples taken from over 400 patients.14 This has led to an improved understanding of the mechanisms underlying PH.

 

One of the potential downsides of centralising the care of patients with a rare illness is that patients may have to travel long distances to receive care. Working in partnership with specialist services, a number of shared-care centres exist.6 PHA UK have conducted a number of surveys over the years to ensure that the UK PH service is meeting the needs of patients and their kinship. A recent survey identified that over 90% of patients are happy to travel to receive specialist care and there are high levels of satisfaction with the service provided, with around 90% of patients rating the care they receive as excellent or good.15

Patient satisfaction with specialist PH centres in the UK15

 

The success of the national network of specialist centres is reflected in the number of patients accessing the network and receiving treatment. Over the last 17 years, since the establishment of a specialist network, there has been a year on year increase in patients seen.

Now, more than 4,000 patients receive therapies targeting the pulmonary vasculature;8 a 6-fold increase since the first national consensus in 2004.16 Large numbers of patients are evaluated every year and over 9,000 different patients were seen in 2016/2017.8 In 2018 the 9th annual report of the national audit of PH was published. Click here to access the report.

 

Number of active patients in the UK PH service, 2004–20178

The UK network of PH centres represents a unique approach to the management of patients with PAH. The service promotes high standards of treatment for patients and has advanced international understanding of the disease. It is anticipated that as our knowledge and the detection of the condition grows, so will the number of patients accessing the service.

References

  1. D’Alonzo G, et al. Ann Intern Med 1991;115:343–49
  2. Chung C and Carlson R. Oncologist 2003;8;514–20
  3. Stathopoulos, GP. Oncol Lett 2011;2:731–33
  4. Galiè N, et al. Eur Heart J 2016;37:67–119
  5. Jenkins D. Eur Resp Rev 2015;24:263–71
  6. NHS Commissioning Board (2013). 2013/14 NHS Standard Contract for Pulmonary Hypertension Centres (Adult). A11/S/a
  7. Corris, P. Glob Cardiol Sci Pract 2015(3):37
  8. NHS Digital (2017). National Audit of Pulmonary Hypertension Great Britain, 2016-17
  9. Suntharalingam J, et al. Clin Med (Lond) 2016;16:135–41
  10. Sitbon O, et al. N Engl J Med 2015;373:2522–33
  11. Pulido T, et al. N Engl J Med 2013;369:809–18
  12. Ghofrani H, et al. N Engl J Med 2013;369:330–40
  13. Galiè N, et al. N Engl J Med 2015;373:834–44
  14. The University of Sheffield, 2015. What is the Pulmonary Hypertension Biobank? Available from: https://www.sheffield.ac.uk/iicd/news/what_is_the_pulmonary_hypertension_biobank-1.471112. Accessed March 2018
  15. Pulmonary Hypertension Association UK (2017). What it means to live with PH today. PHA UK. Available from: www.phauk.org. Accessed March 2018
  16. The NHS Information Centre (2011). National Audit of Pulmonary Hypertension, 2011

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