Respiratory conditions - quality prescribing strategy: improvement guide 2024 to 2027

8. Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis (IPF) is a progressive and often fatal condition. In a 2016 publication it was estimated that over 5,000 new IPF cases were diagnosed each year in the UK and over 30,000 people were living with the disease.^[64]

Patients with this condition often have disabling symptoms of breathlessness and cough. Anti-fibrotic (AF) therapy has been shown to reduce loss of lung function and preserve life when used effectively in IPF.^{[66],[67],[70]} These medicines (pirfenidone and nintedanib) have a high side effect profile, however, and do not improve the symptoms of IPF.^[66],[67] Nintedanib has also shown benefit for patients with other Progressive Fibrosing Interstitial Lung Diseases.^[65] Patient awareness of these issues and the risks and benefits of taking treatment is important to ensure appropriate adherence with therapy. Anti-fibrotic therapy should only be prescribed in secondary care.

For GP practices these medicines should be added to the patient record to highlight prescribing in secondary care (as an ‘outside issue’). This allows interactions to be checked when prescribing for another condition.

Good patient care of people with IPF will require effective communication between secondary and primary care clinicians alongside the use of respiratory support services such as Pulmonary Rehabilitation and secondary care Respiratory Nurse Specialists where available.

End of life care can often be managed in primary care. GP practice teams should ensure that anticipatory care plans and medicines are in place when approaching end of life, to allow timely access to these when required.

Summary of recommendations in Idiopathic Pulmonary fibrosis

• anti-fibrotics prescribed only by a clinician with experience of treating IPF
• only prescribe anti-fibrotics when there is confirmed fibrotic lung disease with evidence of physiological progression

Principles of prescribing for Idiopathic Pulmonary Fibrosis in Secondary Care

There are various principles to be mindful of when prescribing nintedanib or pirfenidone for patients with IPF. These include the following:

• nintedanib and pirfenidone should only be prescribed by a clinician with experience of treating IPF and they should monitor the benefit of AF medicines
• nintedanib and pirfenidone are approved for restricted use in Scotland for patients with a predicted forced vital capacity (FVC) less than or equal to 80%^{[66], [67]}
• the two available drugs have similar efficacy and have different side effect profiles - most patients who cannot tolerate one therapy will tolerate the other
• ensure appropriate treatment choice including drug interactions and potential side effect profile
  • nintedanib is associated with liver injury and requires blood monitoring monthly for the first three months then six monthly thereafter^[68] - common side effects include diarrhoea, nausea, abdominal pain, weight loss and decreased appetite
  • pirfenidone is associated with hepatic injury and requires blood monitoring monthly for the first six months then three monthly thereafter^[69] - common side effects include nausea, indigestion, photosensitivity and rash
• primary care clinicians should refer the patient presenting with these side effects to the consultant.
• shared care agreements are variable across Scotland, so it is important to establish who is responsible for blood monitoring

Prescribing areas to address for quality prescribing in Idiopathic Pulmonary Fibrosis

Prescribing appropriate anti-fibrotic treatment for patients with progressive Idiopathic Pulmonary Fibrosis

Anti-fibrotic therapy is difficult to tolerate with a high side effect profile. Patients should only be prescribed this treatment when there is confirmed fibrotic lung disease with evidence of physiological progression. Ideally this should have been reviewed at an IPF multi-disciplinary team meeting before initiation of therapy.

There is no conclusive evidence to support use of any medicines to increase the survival of people with IPF ^[70] however NICE technology appraisals for pirfenidone or nintedanib should be consulted prior to prescribing.^[71],[72]

IPF therapy is currently included in the NHS Scotland Patient Access Scheme which improves the cost effectiveness of nintedanib and pirfenidone.^[73]

Adopting the ‘what matters to you?’ principles is recommended and knowledge of comorbidities and co-prescribing will allow an approach to incorporating the Polypharmacy 7-Steps approach to the review. Table 5 outlines the main principle for treating patients with IPF.

Table 5: Principles of treating patients with IPF

Polypharmacy review 7-Steps / IPF

1 What matters to the patient?

• Ask the patient what matters to them?
• Ask patient to complete PROMs (questions to prepare for my review) before their review
• How does the condition affect patient’s day to day life / activities
  • cough interfering with ability to work
• Patient awareness of the reason for taking medications- to preserve lung function with anti-fibrotic treatment
• Patient awareness of side effect profiles of medicines versus benefit
• Pulmonary Rehabilitation (PR)
• Discuss monitoring oxygenation when mobilising

2 Identify essential drug therapy

• Assess adherence
• Confirm ongoing need for and effectiveness of medication and screen for side effects
• Ensure therapy is optimised with no drug interaction

3 Does the patient take unnecessary drug therapy?

• Is there evidence of benefit from taking the treatment, e.g. reassuring physiology, maintaining exercise tolerance

4 Are therapeutic objectives being achieved?

• Halted rate of decline of lung function
• Ensure regular monitoring of physiology
• Review oxygenation at rest and on mobilizing
• Medication should be titrated to a dose which balances maximum clinical efficacy with minimal risk and stopped if found to be ineffective or if adverse effects outweigh benefits
• Once the dose is stable and effectiveness has been established, ongoing recorded review should occur as clinically appropriate for the individual patient, bearing in mind that side effects can develop after established on therapy
• Vaccinations should be offered if not up to date (influenza, pneumococcal, DTaP (if not vaccinated in adolescence) and Covid-19)
• Can the patient use their inhaler properly?
• Patients should be encouraged to engage in appropriate physical activity. Social prescribing such as exercise dependent on ability, singing classes
• Smoking cessation should be advised and the adverse effects of smoking on children highlighted. Offer appropriate support. Signpost patients to the NHS inform Quit Your Way Scotland website (which includes community pharmacy services).
• Weight reduction is recommended in obese patients (BMI >30)
• Nutritional advice and support will be necessary in those with a BMI less than 20

5 Does the patient have ADR/ side effect or is at risk of side effects?

• Discuss side effect profile with perceived benefit of treatment - often patients may not be aware that side effects are related to the drug treatment
• Ensure regular drug monitoring as per local protocol
• Consider additional therapy to control side effects e.g. loperamide for GI upset, morphine for cough
• Review potential drug interactions which can potentiate side effects
• Yellow card reporting of ADRs

6 Sustainability

• Discuss dispensing options with patient e.g. potential home delivery schemes
• Ensure that drug is either within current guidelines or has been discussed at a regional IPF multidisciplinary team

7 Is the patient willing and able to take drug therapy as intended?

• A personalised action plan is key to this approach, with focus on maintain lung function, preserving quality of life
• Agree with the patient arrangements for repeat prescribing. Signpost to Home care delivery arrangements / Medicines Care and Review (MCR) service in community pharmacy
• Make patient aware of support information
• Non-attenders should be followed up. Alternative strategies to encourage engagement may be required e.g., through community pharmacy / Near Me / telehealth acknowledging limitations
• Ask patient to complete the post-review PROMs questions after their review

Idiopathic Pulmonary Fibrosis Case Study

Background Details - (Age, Sex, Occupation, baseline function)

• Male
• age 78
• MRC grade 4 Shortness of Breath

History of presentation/ reason for review

• Presented to clinic with persistent cough which impacted on his ability to work
• Slow progression of condition

Current Medical History and Relevant Comorbidities

• No previous medical history, previously fit and well

Current Medication and drug allergies (include OTC preparation and Herbal remedies)

• Struggled to tolerate therapy, with multiple side effects but treatment eventually established- had to switch from pirfenidone to nintedanib
• Opiates (morphine sulfate 10mg/5ml oral solution, 2.5ml) to control cough / breathlessness due to progression
• Oxygen (maintain saturation / promote mobility)

Lifestyle and Current Function (inc. Frailty score for >65yrs) alcohol/ smoking/ diet/ exercise

• Condition slowly progressed – required opiates to control breathlessness

Results e.g., biochemistry, other relevant investigations or monitoring

• Investigations confirmed IPF
• Monitored at clinic - when lung function showed evidence of progression antifibrotic treatment started

Most recent consultations

• Despite progressive condition and high symptom burden he was able to maintain a reasonable quality of life, socialising, spending time with family, travelling and painting