Interstitial lung disease (ILD) is an umbrella term describing a diverse group of lung diseases that result in impairment or fibrosis of the alveolar interstitium. Idiopathic interstitial pneumonias are the most common ILDs; and idiopathic pulmonary fibrosis (IPF) is most common among this cohort of patients. Symptoms of ILD include breathlessness and a persistent, dry and irritating cough. Patients with ILD may experience acute exacerbations, which can increase the burden of disease, reduce quality of life and contribute to progression of disease and mortality.
The accurate diagnosis and treatment of ILD often requires specialist input from a multidisciplinary team. The National Institute for Health and Care Excellence recommends including a consultant respiratory physician and clinical nurse specialist in the multidisciplinary diagnosis of patients with IPF, but the role of the pharmacist in this team has not been fully explored. Immunomodulatory and antifibrotic medicines prescribed for the management of ILD and IPF, respectively, are specialist, high risk and/or high cost. Inappropriate use can result in failed treatment and avoidable adverse effects, which could lead to serious harm or fatality; specialist input at diagnosis and ongoing review is desirable.
A review was carried out in an outpatient clinic setting to determine the impact of a specialist pharmacist in the management of patients with ILD. We hypothesised that a pharmacist can have a significant positive impact on improving patient choice, understanding and adherence to therapy. A retrospective review was conducted in patients with a diagnosis of ILD who had been prescribed immunomodulatory or antifibrotic therapy over an 18-month period. Data collected from the electronic database and laboratory results were analysed. Data included medicine prescribed, dosage, drug interactions identified, information and support provided to patients and any interventions made by the specialist pharmacist. Patients were reviewed by a consultant respiratory physician (and rheumatologist, where appropriate) in a tertiary referral centre. Where a multidisciplinary diagnosis of ILD was confirmed, the consultant(s) discussed management options with the patient. If the patient agreed to pharmacological therapy, they were referred to the specialist pharmacist.
On initiation of therapy, the specialist pharmacist counselled each patient on their diagnosis, management of symptoms, monitoring and supply of medicines. The pharmacist undertook a personalised medication review of a full medicine history, to optimise prescribed therapies and de-prescribe inappropriate or harmful therapies. Comorbidities that contribute to breathlessness may alter patient perception on the effectiveness of therapy; therapeutic management of co-existing airway disease was therefore optimised to improve quality of life and clinical outcomes. It was equally important that patients misdiagnosed with conditions, such as chronic obstructive pulmonary disease or congestive heart failure, had their diagnosis reviewed and potentially harmful therapies deprescribed.
A third of ILDs have identifiable causes, such as environmental or occupational factors, infection or drug toxicity. Factors that could cause or exacerbate ILD were identified and reviewed. The pharmacist was well placed to inform the patient about the pharmacological management of ILD and to aid drug selection. All cautions and contraindications were carefully considered and risks of adverse events such as hypersensitivity, hepatotoxicity, renal toxicity and cardiovascular events were assessed, minimised and monitored. This included rationalisation of therapy and checking for drug interactions. Patients were educated on avoiding interactions where possible and recommendations were made to the GP where alternatives should be considered. Alcohol consumption was documented and patients were reminded of national recommendations. Smoking status and history were also documented; current smokers were encouraged to quit smoking. Patients were referred for oxygen assessment, smoking cessation, vaccination, pulmonary rehabilitation, supportive care and/or palliative care services, where necessary. All patients were provided with verbal and written information on the disease and medicine, a contact card with the ILD helpline and email address for the ILD team. Patients were also signposted to the British Lung Foundation website and local support groups.
Depending on the medicine prescribed, patients were initially reviewed fortnightly or monthly for monitoring and dose titration; the frequency was reduced when patients were deemed stable. At ongoing review, the specialist pharmacist assessed tolerability and adherence to therapy, escalated or reduced dose where appropriate, undertook blood monitoring, and supplied medicines. Drug interactions for any new therapies initiated by primary or secondary care were assessed at every visit. The review also considered lung function, oxygen therapy, smoking cessation, vaccination, pulmonary rehabilitation, psychosocial needs, identification of exacerbations and any respiratory hospital admissions. The pharmacist continued to alleviate patient concerns about their condition and/or medicines.
Over an 18-month period, an average of 125 patients per month were reviewed by the specialist pharmacist. All patients were counselled on disease, management options, potential adverse effects, monitoring and supply. All drug interactions were checked and managed appropriately. The specialist pharmacist undertook 116 significant interventions, which may contribute to adherence to therapy, and cost savings when inappropriately prescribed medicines are stopped.
Specialist ILD pharmacist involvement has a positive impact on improving patient choice, understanding and therapy adherence. Specialist pharmacists are well placed to educate patients on their diagnosis, treatment strategies and prognosis from onset of disease. Pharmacists can also counsel patients on medicine, dosage and potential adverse effects, and how to manage or avoid them. As medicines specialists, pharmacists can optimise the management of co-existing conditions, as well as identify and refer patients with comorbidities for management by specialists and/or GPs. Specialist ILD pharmacists should work within multidisciplinary teams to offer integrated care and the best outcomes for patients.
Marium Naqvi, specialist ILD pharmacist at Guy’s and St Thomas’ NHS Trust, London