Coronavirus (COVID-19): Advice and guidance for patients, plus the latest visiting restrictions
We have 351 patients under the care of our cystic fibrosis team at Royal Papworth Hospital, with referrals from across the East of England and parts of north London.
Cystic fibrosis (CF) is an inherited condition that causes sticky mucus to build up in the lungs and digestive system. This is due to a reduced number of or inneffective proteins called ‘cystic fibrosis transmembrane conductance regulator’ (CFTR) and causes lung infections and problems with digesting food.
In the UK, most cases of cystic fibrosis are picked up at birth using the newborn screening heel prick test.
Symptoms usually start in early childhood and vary from child to child, but the condition gets slowly worse over time, with the lungs and digestive system becoming increasingly damaged.
CF is incurable and in severe cases a lung transplant may be recommended. However, new drugs Kaftrio, Sykmevi, Orkambi and Kalydeco have been shown to improve lung function and quality of life for some CF patients (see below).
Our cystic fibrosis service was established in 1994 and now looks after more than 350 adults with CF from the Eastern Region and beyond.
The service is part of the Cambridge Centre for Lung Infection (CCLI), and is supported by a multidisciplinary team including: six CF specialist consultants; three CF specialist nurses; a dedicated ward nursing team; research nurses; specialist physiotherapists, dieticians, pharmacists, social workers; a psychologist and psychiatrist; secretaries; and a management team.
Patients are seen in specialist CF clinics according to their sputum microbiology: no regular infection, non-epidemic Pseudomonas aeruginosa, epidemic Pseudomonas aeruginosa, Burkholderia cepacia complex, Mycobacterium abscessus and MRSA clinics occur weekly. The centre also provides a 24/7 emergency service for people with CF who are acutely unwell.
The Royal Papworth Adult CF service also provides specialist CF psychological medicine clinics, CF diabetes clinics with the Cambridge University Hospitals diabetes team, and specialist CF liver disease clinics with CUH hepatologists.
Annual CF transition clinics are performed jointly with the paediatric teams to meet children and families prior to their care moving to the adult service. We currently run transition clinics at Great Ormond Street Hospital in London, CUH in Cambridge, Ipswich Hospital, Luton & Dunstable Hospital, Kettering Hospital, Peterborough Hospital and Chelmsford Hospital. A link to a website containing useful information about transitioning from paediatric care to adult care at Royal Papworth Hospital NHS Foundation Trust is found below.
The inpatient facilities at Royal Papworth Hospital include 12 ensuite rooms on the CF ward on Floor 4 with a further six inpatient rooms on an adjacent ward, for patients with CF with more antibiotic resistant organisms. Each room contains air conditioning, high-speed WIFI, a flat screen TV and games console.
The outpatient facilities include 10 rooms in a dedicated CCLI area in the outpatient department, four of which are en-suite.
The hospital has some state-of-the-art features to make it as safe as possible for people with CF from a cross-infection prevention perspective:
In February 2019, we began a new home monitoring trial for our cystic fibrosis patients. This is a first-of-its-kind project being led by Royal Papworth Hospital and funded by the US CF Foundation. Project Breathe has a wide range of benefits for patients, allowing them to monitor their health at home far more regularly than relying on hospital visits. This gives patients more control over the health and a better understanding of when they are showing early signs of deterioration. Read more about Project Breathe.
In July 2020 came the landmark news that Kaftrio (known as Trikafta in the USA) would be made available to NHS patients following a deal between NHS England and Vertex Pharmaceuticals.
The licence was granted by the European Medicines Agency (EMA) on Friday 21 August, when it decided that Kaftrio’s benefits are greater than its risks and it can be authorised for use in the EU.
It will be available to patients:
Find out more about how Kaftrio works by improving the number and activity of CFTR proteins (EMA website)
Sam Henman, Lead Nurse for CF, and Dr Uta Hill, Clinical Lead for CF, react to the Kaftrio news:
Just eight months before the Kaftrio news, NHS England had announced that Symkevi, Orkambi and Kalydeco would be made available to all eligible patients after striking a deal with the manufacturer, Vertex.
Dr Helen Barker, Consultant in Respiratory Medicine, and Chi Palas, CF Specialist Nurse, react to the breakthrough announcement:
Some people who have rarer mutations will not be eligible for these drugs. However, there is ongoing research and clinical trials which will ensure that these patients also will benefit from therapies in the future.
Project Breathe - Royal Papworth Hospital's cystic fibrosis team is leading the world with a new, advanced home-monitoring trial for our CF patients.
Kaftrio (July 2020)
Orkambi, Symkevi and Kalydeco (November 2019)
Cystic Fibrosis Trust (Link to external site)