Cystic fibrosis (adults)

The Royal Brompton Hospital Adult Cystic Fibrosis Centre is one of the largest centres in Europe and treats approximately 600 adults from the age of 17 years. We aim to provide high quality, evidence-based healthcare in a supportive and caring environment.

Outpatients

To prevent cross-infection (or stop the transmission of certain 'bugs'), we keep people with CF separate and group each clinic by bacteria type because they may be vulnerable. CF clinics are held on Monday, Tuesday and Friday afternoons.

Other clinics include:

  • CF-related diabetes (monthly)
  • Gastrointestinal (monthly)
  • CF-related liver disease (monthly)
  • Difficult cystic fibrosis diagnosis (alternate months)
  • Sweat testing (three times per month)

Clinic staffing

There are at least two consultants in every clinic, plus junior doctors, research fellows and respiratory registrars.

At the beginning of each clinic, before the first appointment of the day, staff have a 30-minute team meeting to discuss clinic attendees for the day. 

All patients returning for an annual review follow-up, in person or via the internet, will see a consultant. On a routine visit, individuals will see the nurse specialist, dietitian and physiotherapist. We also try to schedule appointments to coincide with clinic visits from the welfare rights advisor, the psychologist, or any extra tests - like CT scans  - which may be necessary.

The clinic also provides a good opportunity to talk to about current research studies.

Inpatients

Because of cross-infection issues, adults with CF are only admitted to single rooms. Each room has an en-suite bath or shower, a fridge, television and telephone (incoming calls only). All rooms and bed spaces have network access and laptop computers are available.

For younger people with CF, links with the  Chelsea Community Hospital School  mean that teachers are available to support studies during an admission. The ward is also an accredited examination centre. Reflexology is available several days a week and there is a booking service on the ward available for this.

The ward nursing staff are skilled in specific CF care including transition, pregnancy, complex disease, managing NIV, and palliative/end of life care. The ward nurses work closely with the CF MDT and patients to agree a package of care at each admission.

Stress incontinence and CF

When urine leaks, during sudden extra pressure on the bladder (like coughing or laughing, for example), this is called stress incontinence. It happens because your pelvic floor muscles and urethra can't withstand the extra pressure. 

Stress incontinence is the most common form of urinary incontinence, and happens to many people. People with respiratory disease are particularly prone to it because of the increased stress of chronic coughing on the pelvic floor muscles. 

Dealing with stress incontinence

One of the simplest ways to treat leakage is by strengthening your pelvic floor muscles, which can be done through exercise. Once you learn the technique, you can do the exercises anywhere and no one will know.

Finding your pelvic floor muscles

Tighten the muscles around your back passage, vagina and front passage and lift up inside as if trying to stop passing wind and urine at the same time. It's quite easy to use other, irrelevant muscles while doing this, but if you focus on the following, you'll be able to isolate your pelvic floor more easily and make the exercise more effective. Try not to:

  • pull your tummy in
  • squeeze your legs together
  • tighten your buttocks
  • hold your breath

Practise your exercises in a comfortable, relaxed position, such as crook lying, while you're learning them and then you can progress to practising them while sitting or standing.

Pelvic floor exercises
Crook lying position

Pelvic floor exercises

Exercise 1

Contract your pelvic floor slowly and hold for up to 10 seconds. Rest for a count of four, and then aim to repeat the exercise up to 10 times. 

Exercise 2

Once you're able to isolate your pelvic floor, and contract your muscles slowly, it's important to be able to work these muscles quickly to help them react to sudden stresses, such as coughing.

Practise quick contractions, drawing in the pelvic floor and holding for one second before relaxing. Aim for strong muscle tightening with each contraction, repeating up to a maximum of 10 times.

Aim to do one set of slow contractions (exercise 1) followed by one set of quick contractions (exercise 2) six times each day.

Annual review

Each year, adults with CF receive an annual review which is organised by the CF administration hub team. They book dates, investigations and appointments and post them out. 

The first part of annual review is based in the day case ward and involves a half day of tests and investigations, followed by clinic a clinic visit (in person or via the internet) with a consultant. This is followed up, 4-6 weeks later (in person or via the internet), with at least 30 minutes with the CNS, dietitian, pharmacist and physiotherapist. 

Pyschologists will send out a psychological measures questionnaire which they can discuss with patients at a later date, if requested. After this appointment, a final report is written by the consultant and sent on to both the patient and their GP. 

Contact the cystic fibrosis administration hub team for further information. 

Transition

Across the year there are approximately four transition clinics where older children from the Royal Brompton and Harefield Paediatric CF service, and one or two (depending on numbers) from the Great Ormond Street Hospital CF service, move to the CF adult clinic. Young adults from paediatric CF services in the London area may also transition, either through choice or because of location. 

Approximately 20-30 young adults with cystic fibrosis transition to adult care at Royal Brompton Hospital each year. The paediatric teams from both centres work closely with the RBH adult team to ensure a smooth transition process. 

 

Research

In partnership with the Royal Brompton Hospital Paediatric Cystic Fibrosis Centre, the Royal Brompton Hospital Adult Cystic Fibrosis Centre pursues improvement in quality as well as care delivery, and is active in research.

As a partnership, they are a designated European Cystic Fibrosis Society Clinical Trials Network Site (ECFS CTN), which provides opportunities for us to lead and participate in trials of new treatments across Europe. The Partnership is also part of the Cystic Fibrosis Trust Clinical Trials Accelerator Platform (CTAP) – London Network.

Find out more about the CTN and the CTAP

National Cystic Fibrosis Registry

We enter annual data onto the UK CF Registry following individual consent for anonymous data to be recorded. Find out more

New patients

If you would like to be referred to this centre, we will need a referral letter from either your GP or, if you currently attend a CF centre, your consultant. Public transport and distance mean that most of our referrals come from people living in North West London.

Useful contacts

Get in touch with us to:

  • change an appointment
  • contact Lind ward: 0207 352 8121
  • contact Foulis ward: 0207 351 8069
  • let Outpatients (west) know that you cannot attend an appointment on the day, or will be late: 0207 352 8121 ext 4021

For all other queries, please contact Patricia Granahan: p.granahan@rbht.nhs.uk

Service development

We are developing virtual clinical consultations and self-monitoring for adults with CF attending this service. We have recently appointed Medopad partnering with Nuvoair to build a digital platform that will allow safe and confidential data sharing. This development has been financially supported through a Joint Working Contract with Vertex Pharmaceuticals. Find out more

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