Falling through the net; why clinicians agreeing familial PF is an ILD from a known cause is a ‘game changer’ for patients

Falling through the net? Familial PF
August 2022

ILD clinicians from across the UK have agreed with immediate effect:

Patients with Familial Pulmonary Fibrosis (defined as having at least one first degree relative with pulmonary fibrosis regardless of cause) can be considered as having pulmonary fibrosis of known cause and follow NICE treatment algorithms for PPF.

Until February 2021, the only way to access the limited treatment options to slow the progress of the lung scarring (fibrosis) was if people had an unknown cause for their pulmonary fibrosis.  This meant a diagnosis of idiopathic pulmonary fibrosis (IPF). This treatment comes with restrictions – people having to wait until their force vital capacity (FVC) drops to 80% or less.  

A criminal and immoral act, and one that APF is fighting, but that’s another story!

We all welcomed the news in November 2021 when Nintedanib would be available to patients with a known cause for their pulmonary fibrosis, without an FVC restriction.  This has meant a further c. 15,000 patients have access to a treatment that can increase longevity and quality of life.

So where does this leave the 5,000 – 7,000 people with Familial PF across the UK?

Frances is living with Familial PF, but her FVC is above 80%

Having watched what happened to my brother, and now watching my Dad going through the same thing, it`s terrifying to see what your own path may be. Knowing that medication is available to help slow the progression down, but that you can`t access it because you aren`t yet ill enough is incredibly hard. Hearing this fantastic news, that people with familial PF can be prescribed this medication upon diagnosis, will be a huge relief to all those families affected. With APF fighting our corner, good sense has prevailed and this decision will bring hope to many.

By mutual agreement, ILD clinicians agreed that Familial PF fell within guidance of treating progressive lung fibrosing from a known cause.

We thank all ILD clinicians who have taken this life enhancing decision, as APF takes steps to share this decision with the patient and HCP community.