There is a growing population of people with CF in Europe. Latest forecasts indicate an increase of approximately 50% by 2025 in Western Europe (20% in the child population; 75% in the adult population), and an increase in CF in Eastern Europe, predominantly in the child population.1
The increasing number of patients can be attributed to improved survival, and suggests the need for additional centres and, indeed, healthcare professionals with expertise in the management of CF.2 Key to the effectiveness of the specialist CF centre is the multidisciplinary team (MDT), which should include a pharmacist experienced in the care of people with CF.
The European Cystic Fibrosis Society (ECFS) is an international community of professionals committed to improving survival and quality of life for people with CF. One of the outputs of this organisation is the ‘Standards of Care: Framework for the Cystic Fibrosis Centre,’ which describes the CF Clinical Pharmacist as pivotal to the medicines management programme to support the optimisation of medicines for patients, and describes the role of the CF clinical pharmacist (Box 1).3
Despite the recommendation that the CF MDT includes a specialist clinical pharmacist, a recent survey has found that less than half of the European CF centres surveyed have access to one.4 This is most likely explained by decentralised clinical pharmacy services being uncommon in Europe, with only the UK and Ireland having developed these services to a significant extent.5 There are, however, clear benefits to patients and services from the appointment of specialist CF clinical pharmacists, and pharmacists with an interest in CF are encouraged to join the European CF pharmacy group (Box 2).
The benefits of a dedicated CF pharmacist have previously been described, including benefits in terms of financial savings, improvements in patient care and efficient use of resources.6 This article highlights some of the key practical aspects of the role from two of the largest CF centres in the UK.
With the advent of newborn screening for CF, pathways have been established in many CF centres to deal with not only the disclosure of the CF diagnosis to families, but also swift and timely education, to prevent the delay and uncertainty that may exacerbate the impact of a diagnosis to families.7 The CF pharmacist is integral to this education process, not least because the introduction of new medicines can bring home the reality of lifelong medication needs.
The CF pharmacist meets with the family primarily to explain the purpose of the new medicines prescribed for the baby, and how to administer them. It also provides an opportunity for the family to ask questions about medications for CF that may have arisen since the diagnosis. There is a discussion around how to arrange further supplies of these medicines, and what to do should there be any problems.
The information covered during the session is detailed in Box 3. The session is also an opportunity for the CF pharmacist to introduce the pharmacy service that is offered and begin to build up what is likely to be a longstanding professional relationship.
Medicines optimisation is about ensuring the right patient gets the right medicine at the right time. The goal of medicines optimisation is to improve patient outcomes, improve adherence and ensure patients take their medicines correctly, reduce wastage of medicines, unnecessary medication use, and improve medicines safely.8
The pharmacist’s role in undertaking an accurate medicines reconciliation in CF ensures that medicines are taken correctly and potential adverse effects are identified. There are various opportunities for this to occur for examples, inpatient admissions, clinic attendance, and annual review.
People with CF are encouraged to attend a CF annual review. At this review, each patient is seen by the CF pharmacist and a comprehensive medication review is undertaken. It has been shown that at annual review, a pharmacist makes an average of 4.75 interventions per patient, most of which were deemed significant or very significant.9 Prior to the review, the patient’s most recent clinic or discharge letter and general practitioner’s medication records are reviewed. Discrepancies, inappropriate prescribing, and potential adherence issues can therefore be identified in advance, so that they can be discussed during the review.
The review provides an excellent opportunity for the pharmacist to identify ways to manage and support patients, for example with issues around adherence, adverse effects, or any other problems. Examples of potential changes to therapy which may be made include changes to inhaled antibiotic formulations, changing the timing of treatments, or recommending alternative treatments.
Patients with CF may also be on long term medicines that require plasma drug levels to be monitored, such as oral antifungals or aminophylline. During the review, the pharmacist checks the plasma drug levels and advises on any necessary dose changes, or requests a level to be taken where this is not available. Problems with obtaining supplies of medicines are also addressed – the pharmacist can help to resolve these to prevent interruption to the patient’s treatment.
Having a pharmacist at annual review allows patients to openly discuss their medicines, any issues they are experiencing, and to ask questions. The pharmacist ensures the patients medicines are optimised, and where necessary, patients are re-educated about the importance of their treatment and the benefits of taking them, to support adherence.
Adherence to treatment is a complex, multifactorial issue and the subject of much discussion in the management of CF, where appropriate poly-pharmacy is usual. Alongside the patient centred aim to reduce or halt disease progression, with the increasing cost of CF therapies, optimising adherence is essential to thereby ensure judicious use of valuable resources by avoiding inappropriate escalation of treatment.
The pharmacist has a significant role to play in supporting adherence, particularly in CF, where FEV1 and BMI may decline due to inadequate treatment. Recent work has identified that self-reported adherence is the most common method of measuring adherence in practice and it has been suggested that greater specialist pharmacist involvement in CF patient care could facilitate improvements in the monitoring of adherence.10
Elsewhere, the use of technology has been used to assess adherence; whilst these may have a role in identifying non-adherence, the CF pharmacist will then be able to support health-behaviour change by providing information, motivation, and helping to design strategies to achieve and maintain any changes.
The role of the pharmacist is particularly important during the transition of adolescents from children’s to adult services, where traditionally the responsibility of managing medication moves from parent to patient. The use of self administration schemes to improve education and autonomy, and encourage responsibility of taking medication whilst in hospital can be particularly useful to this process.11
During the clinical care of people with CF, there is often a need to use medicines outside of their product licence. A new medicine, for example, may not have been subject to the rigorous trials necessary to gain a paediatric product licence; or it may be necessary to manipulate a formulation so that it can be administered via an unlicensed route, such as nebulisation of an intravenous drug formulation.
Although off-label use of medicines is not routinely recommended, it is often necessary in the CF population due to the complexities of the disease, and heterogeneity of the complications encountered.
The CF pharmacist will be involved in ensuring these medicines are used in the most safe and effective way and can apply pharmacokinetic and pharmacodynamic principles to determine appropriate dosing.
An example of work led by CF clinical pharmacists is the use of posaconazole in children for the management of Aspergillus fumigatus or Allergic Bronchopulmonary Aspergillosis (ABPA).
Posaconazole is licensed in adults aged 18 years or above, and there is no literature on its use in children with CF.12 In one intervention, the pharmacist recommended dosing in children, and subsequent use of therapeutic drug monitoring to assess whether appropriate therapeutic levels were attained. From this work, the incidence of adverse effects in children, and the dose required to attain appropriate therapeutic levels was established.13
The use of unlicensed medicines introduces further complexity in supply arrangements as primary care physicians may have restrictions to continued prescribing. The CF pharmacist can navigate these issues by identifying the most appropriate supply model for the individual patient, which may include a combination of supply routes, such as prescribing by specialist centre or primary care physician, or use of homecare services. Where available, the CF pharmacist – as an independent prescriber – can ensure seamless and timely ongoing supply of medicines to patients.
Antimicrobial stewardship describes systems and processes for effective antimicrobial medicine use.14 Pharmacists have been shown to have a key role in this process especially in secondary care.15 As antibiotics are the mainstay of treatment for respiratory exacerbations in CF, and inhaled antibiotic formulations help reduce these exacerbations, the CF pharmacist plays a crucial role in antimicrobial stewardship within the CF team.
The prompt use of effective antibiotics has been one of the advances in therapy that has lead to increased longevity of the CF patient.16 However, the use of antibiotics is not without issues – for example, adverse effects such as progressive renal toxicity following repeated courses of aminoglycosides is one of the many challenges faced by the CF physician.17
The CF pharmacist is well placed to help manage and minimise these adverse effects by ensuring the correct dose using patient individualised parameters such as renal and hepatic function and body weight.
The CF pharmacist will also ensure the correct monitoring is undertaken throughout the treatment course, such as therapeutic drug monitoring with aminoglycoside therapy and the continual monitoring of renal, hepatic, electrolyte and blood indices.
Hypersensitivity reactions, particularly to antibiotics, are commonly seen in the CF patient.18 This can lead to suboptimal treatment to be prescribed in the hypersensitive patient. The CF pharmacist can assist with the identification and documentation of allergies and, in the case of IgE-mediated reactions, the CF pharmacist can suggest an alternative antibiotic regimen, or a desensitisation regime.19 This will ensure the CF patient receives optimal treatment and therefore reduce the risk of further loss of lung function.
Communication across different interfaces
Patients with CF are prescribed many different types of medicines, some of which are prescribed by their GP and others which are prescribed by the CF centre, and as discussed, some of the medicines prescribed are used outside of their product license, or are not routinely available.
It is therefore important that regular and detailed information is communicated from the CF centre to the GP and community pharmacists, particularly when medicines are newly started, stopped or changed, along with information about prescribing responsibility. The CF pharmacist will liaise with GPs, clinical commissioning pharmacists and community pharmacists to prevent delays to patients receiving their medicines, maximising patient safety and convenience.
New drugs and budgets
The advent of targeted gene-specific therapies have heralded a new era of ever more complex and expensive treatments. The introduction of new therapies into the clinic needs to be carefully managed to ensure equitable access. The CF clinical pharmacist will be able to identify which patients are eligible for treatment within the product license and local funding arrangements; review and implement patient access schemes; write guidelines where necessary; educate patients and healthcare professionals on new medicines; and monitor adherence to novel treatments along with outcomes and adverse events.
The CF pharmacist brings together knowledge of funding arrangements, clinical knowledge, service specifications and patient registry data. They are therefore well placed to make recommendations on budget setting, monitor drug expenditure and implement cost efficiencies within a CF centre.
The specialist CF clinical pharmacist, as an integral member of the CF MDT, has a varied and important role in the clinical care of people with CF, from diagnosis, throughout childhood and adolescence, and adulthood.
The benefits of a CF clinical pharmacist are clear, and whilst the role is well described throughout the UK and Ireland, there remains a large proportion of CF centres throughout Europe who could enjoy the benefits to patient care of a CF clinical pharmacist.
With a CF population increasing in both numbers and age (which will bring further complexities in terms of co-morbidities associated with older age), and the advent of increasingly expensive personalised medicine, CF centres should work to ensure that patients have access to a clinical CF pharmacist, and the international clinical pharmacy community should continue to seek opportunities to support people with CF.
- Burgel P-R et al. Future trends in cystic fibrosis demography in 34 European countries. Eur Resp J 2015;46(1):133–41.
- Elborn JS et al. Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis. Eur Resp J; Oct 2015 [Epub ahead of print].
- Conway S et al. European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre. J Cystic Fibrosis 2014;13:S3–S22.
- Personal Communication. Dr S Madge. Department of Cystic Fibrosis, Royal Brompton Hospital, London UK.
- Frontini R, Miharija-Gala T, Sykora J. EAHP survey 2010 on hospital pharmacy in Europe: parts 4 and 5. Clinical services and patient safety. Eur J Hosp Pharm 2013;20:69–73.
- Redfern J, Webb A. Benefits of a dedicated cystic fibrosis pharmacist. J R Soc Med 2004;97(Suppl. 44):2–7.
- Duff A, Brownlee A. Psychosocial aspects of newborn screening programs for cystic fibrosis. Child Health Care 2008;37:21–37.
- Royal Pharmaceutical Society. Medicines optimisation: Helping patients to make the most of medicines. May 2013. www.rpharms.com/promoting-pharmacy-pdfs/helping-patients-make-the-most-o… (accessed 7 January 2016).
- Philpott LM et al. Introduction and evaluation of a pharmacist-run medication review for cystic fibrosis patients. J Cystic Fibrosis 2007;6(Suppl 1):S93.
- Mooney K, Ryan C, Downey DG. Pharmacists’ perspectives on monitoring adherence to treatment in cystic fibrosis. Int J Clin Pharm 2015; Dec 29.[Epub ahead of print].
- Khan K et al. Self-administration of inpatient medicines (SAM): A pilot study in children with cystic fibrosis [Abstract]. North American Cystic Fibrosis Conference, 2015.
- Merck Sharp & Dohme Ltd. Summary of Product Characteristics. Noxafil 40mg/ml oral suspension. Updated 9 June 2015.
- Bentley S et al. Comparison of the tolerability of posaconazole versus voriconazole in CF children with Aspergillus or ABPA [Abstract]. North American Cystic Fibrosis Conference, 2015.
- National Institute for Health and Care Excellence. Antimicrobial stewardship: systems and processes for effective antimicrobial medicine use. NICE Guideline NG15. August 2015. www.nice.org.uk/guidance/ng15 (accessed 7 January 2015).
- Wickens HJ et al. The increasing role of pharmacists in antimicrobial stewardship in English hospitals. J Antimicr Chemother 2013;68(11):2675–81.
- Antibiotic treatment for Cystic Fibrosis, 3rd Edition. Report of UK Cystic Fibrosis Trust Antibiotic Working Group. May 2009.
- Al-Aloul M et al. Renal impairment in cystic fibrosis due to repeated intravenous antibiotic use. Pediatr Pulmonol 2005:39:15–20.
- Parmar JS, Nasser S. Antibiotic allergy in cystic fibrosis. Thorax 2005;60:517–20.
- National Institute for Health and Care Excellence. Drug allergy: Diagnosis and management. NICE Guideline CG183. September 2014. www.nice.org.uk/guidance/cg183 (accessed 7 January 2016).