ILD Patient Handover to Local Follow-up for Pirfenidone Monitoring

Patient initials: NHS number: DOB:

Diagnosis Date established on treatment:

Tests/Referrals
Pulmonary Function Tests:
Ref meas %ref
Date: FEV1
FVC
Date next due: DLCO

Oxygen: If not on oxygen Sp02…….

Shuttle performed Yes/No
LTOT ….L ….% NC/mask If yes results……………………………
Ambulatory …L ...% Refer locally for O2 Ax □
NC/mask

Pulmonary Rehabilitation:

Yes/No Date:

If no please refer to local PR service □

If patient tolerates treatment refer to Manchester ILD team to be transferred to
801mg formulation: Yes/No

Bloods
Pre treatment Post treatment
Date:
LFT NAD: yes/no NAD: yes/no
Abnormalities: Abnormalities:

U&E NAD: yes/no NAD: yes/no

Abnormalities: Abnormalities:

FBC NAD: yes/no NAD: yes/no

Abnormalities: Abnormalities:

Page | 1
Monitoring Schedule:
1. 6 x monthly monitoring then reduce to 3 monthly
2. Pulmonary Function tests 6 monthly send to Manchester ILD team
3. Annual consultant review by Manchester ILD team.

During every appointment please:

1. Check weight.
2. Changes in breathlessness/cough.
3. Any infections/ exacerbations/hospital admissions/ associated GP visits. Any
changes in medication?
4. Bloods
5. Side effects
6. Changes in dosage.

Month Bloods Side effects Dose Altered dose.

Change Inform
MANCHESTER.
Month 1 NAD/variance NAD/variance yes/no …..
Month 2 NAD/variance NAD/variance Yes/no …..
Month 3 NAD/variance NAD/variance Yes/no …..
Month 4 NAD/variance NAD/variance Yes/no …..
Month 5 NAD/variance NAD/variance Yes/no …..
Month 6 NAD/variance NAD/variance Yes/no …..
Month 9 NAD/variance NAD/variance Yes/no …..
Month 12 (Manchester …..
ILD team)

Pirfenidone Side Effect Management

Side effects Pirfenidone Variance

Nausea Sickness Variance 5
Photosensitive Rash Variance 4
Tiredness Variance 1 or 3 (If unacceptable)
Headaches Variance 1 or 3 (If unacceptable)
Abnormal Liver Function Tests Variance 7 then Variance 2
Reduced Appetite Variance 5+6 then Variance 9
Weight loss/ Anorexia Variance 5+6 then Variance 9
Infection Variance 8
Indigestion Variance 6

Page | 2
 Variances

Variance 1 Reduce the dose of Pirfenidone to 1 tablet per day, then increase by 1 tablet
every week until reaches tolerable dose.
Inform Manchester ILD team
Variance 2 Stop Pirfenidone.
Inform Manchester ILD team for further guidance
Variance 3 Reduce Pirfenidone to 1+1+1 then re titrate slowly by 1 tablet 1x per week.
Inform Manchester ILD team
Variance 4 Reduce to 1+1+1 until the rash has gone/significantly better then re-titrate to
full dose.
 If rash remains the same after dose reduction and does not worsen on
re-titration, continue Pirfenidone at the full dose.
 If the rash comes back or worsens stop Pirfenidone and stay off until
the rash has completely gone then reintroduce Pirfenidone as per
guideline (see appendix 2).
 Ensure that the patients are vigilant applying sun protection factor 50
and that they cover up and stay out of the daylight as much as
possible. Avoid other medications that induce photosensitivity e.g.
Doxycycline.
Inform Manchester ILD team
Variance 5 Ensure that the patient is taking their medication in the middle of a
meal/substantial snack with a glass of water, if not ask them to try this and see
if that solves the problem. Consider indigestion or post nasal drip, If so treat.
If not, for short term management consider Metoclopramide up to 5 days only
(MHRA Guidance).
For long term nausea management consider Cyclizine or Ondansetron (see
appendix 3 for further information)
Variance 6 Ensure the patient is taking their tablets separately within their meal with a
large glass of water, if not ask them to try this and see if that solves the
problem.
Consider adding Pantoprazole as a PPI. If already on Pantoprazole, increase
to BD or consider changing to Lansoprazole or adding Ranitidine at night time.
Gaviscon advance can be used PRN.
If indigestion continues refer to GP for further investigation. Please note
Omeprazole may decrease the efficacy of Pirfenidone by 5-10%
Variance 7 In the event of significant elevation of liver transaminases >3x upper limit of
normal, either reduce dose or interrupt treatment, if >5xupper limit of normal
permanently discontinue.
If the increase of liver transaminases was <5x upper limit of normal and/or no
bilirubin increase was observed re titrate to the full dose checking LFTs every 2
weeks.
If ALT/AST >5 discontinue treatment and don’t restart.
Inform MANCHESTER ILD team
Variance 8 Infection, if clinical signs of infection consider prescribing antibiotics such as
Amoxicillin. Please note Doxycycline is contraindicated and Ciprofloxacin
should be used in caution.
Inform MANCHESTER ILD team
Variance 9 Encourage dietary input, advise 5 small meals per day with added snacks if
weight loss and refer to GP for dietician input/drink supplements if significant.
If continues consider dose reduction to Variance 1.
Inform MANCHESTER ILD team.

Page | 3
 Appendix

Appendix 1 If the patient has a new diagnosis of skin cancer please Inform Manchester ILD
team for further instruction. Ensure that the patients are vigilant applying sun
protection factor 50 and that they cover up, wear a hat and minimise direct sun
exposure.
Appendix 2 If the patient has been off Pirfenidone for less than 2 weeks they can restart on
the full of their tolerable dose. Ensure that the patients are vigilant applying sun
protection factor 50 and that they cover up and stay out of the daylight as much
as possible.
Appendix 3 1st line and 2ND line antiemetic choice depending upon local guidance. As we are
unable to use Metoclopramide long term (MHRA) Cyclizine and ondansetron
have few side effects for long term use. Caution in Cyclizine includes
misuse/abuse and high alcohol usage. Caution in ondansetron includes avoid in
patient with a cardiac history due to prolonged QT interval and patients with
constipation. There is no evidence for long term use of an antiemetic; this
guidance has been made when considering risk verses benefit.
Appendix 4 If they have been off Pirfenidone for more than 2 weeks retitrate 1+1+1 for 1
week 2+2+2 for 1 week then full dose of 3+3+3

Contact Details
 Interstitial Lung Disease Specialist Nurses: Helen Morris, Tracey Marshall and Katie Zakis tel
0161 291 4936 email ILDNurseManc@mft.nhs.uk mft.ildnursemanc@nhs.net
 Consultants: contact c/o Secretaries Ann Solomon and Liz Kendra tel 0161 291 5054 email
ildsecmanc@mft.nhs.uk
 MDT coordinator Collette Hollinworth email collette.hollinworth@nhs.net
 Polarspeed- Abbie Fisk 01525 216771 email Abbie.Fisk@polarspeed.com.

Fibrosis Support Groups

 Please refer to www.actionpulmonaryfibrosis.org for a list of local support groups.

Charities
 The British Lung Foundation: www.blf.org.uk Helpline 03000 030 555
 Action for Pulmonary Fibrosis: www.actionpulmonaryfibrosis.org Helpline 01223 785725

Website and social media

 Website: https://northwestipfmanchester.weebly.com/
 Facebook: North west and Greater Manchester Interstitial Lung Disease Unit
 Twitter: @NorthWestILDIPF Nurses: @ipf_manchester

References:
1. Costabel, U., Bendstrup, E. and Cottin, V. et al (2014). Pirfenidone in Idiopathic Pulmonary
Fibrosis: Expert panel discussion on the management of drug-related adverse events. Adv
Ther 31 p. 375-391.
2. MHRA: Metoclopramide: risk of neurological adverse effects. Available
at:https://www.gov.uk/drug-safety-update/metoclopramide-risk-of-neurological-adverse-
effects#advice-for-healthcare-professionals (accessed 20/7/18).
3. National Institute for Health and Care Excellence (2013). Idiopathic Pulmonary Fibrosis the
diagnosis and management of suspected idiopathic pulmonary fibrosis available at:
https://www.nice.org.uk/guidance/cg163/chapter/introduction (Accessed 20/07/18)
4. National Institute for Health and Care Excellence (2013b). Pirfenidone for treating Idiopathic
Pulmonary Fibrosis. Available at: https://www.nice.org.uk/guidance/TA282/chapter/1-
guidance (Accessed on 31st October 2017)
5. Pirfenidone (Esbriet) – Summary of Product Characteristics. Available at:
https://www.medicines.org.uk/emc/medicine/29932 (Accessed 31st October 2017)

Page | 4