Cystic fibrosis is an inherited condition where mucus builds up in the lungs and digestive system. This then causes lung infections and issues with digesting food properly.  

We are the largest centre for cystic fibrosis (CF) in Europe. We care for nearly 700 adult patients at Royal Brompton Hospital, and we also care for children with CF too.

We want our patients to have high-quality, evidence-based healthcare. We also want them to be in a supportive and caring atmosphere.

We also take part in national and international research studies into the condition. This is we can keep improving how we help patients manage their CF.

CF clinics

At Royal Brompton's adult cystic fibrosis centre we run clinics for:

  • CF outpatients
  • CF-related diabetes
  • CF gastro-enterology

Read more about our CF clinics.

Multidisciplinary team

Our central CF team is made up of many different members, including consultant, nurses, physiotherapists, dietitians and administrators. They are all focused on providing you and your family with the best CF care possible. 

The team review their patients daily and also meet with the wider multidisciplinary team. 

A multidisciplinary team is very important in caring for adults with CF. Our team has close links with many teams across the Trust including: 

  • Transplant team
  • Psychology/psychiatry
  • Obstetricians/gynaecologists
  • Ear nose and throat specialists
  • Rheumatologists
  • Palliative care team
  • Endocrinologists
  • Pain management team
  • Cardiothoracic surgeons
  • Radiologists
  • Gastroenterologists
  • School team

Clinics

We run three cystic fibrosis (CF) outpatient clinics a week. These happen on Mondays, Tuesdays and Friday afternoons. We also run a monthly CF-related diabetes clinic on the last Friday of every month. These are all held in outpatients west at Royal Brompton Hospital.

We also have a CF gastroenterology clinic at Chelsea and Westminster hospital every fourth Friday of the month.

We also run a day-case unit where we can review patients if we need to. This is only by appointment. 

All clinics are closed during UK bank holidays (England and Wales).

CF Trust guidelines

We follow the CF Trust's guidelines on cross-infection for both inpatients and outpatients. We separate clinics and when patients arrive they go into separate consulting rooms. So as to prevent cross-infection, patients growing different bugs in their sputum are seen on different days.

Appointments

Clinics operate on an appointment system. To minimise waiting times patients will be seen in appointment order rather than arrival time. 

Please arrive at the clinic 10 minutes before your appointment time. We realise travel to the hospital can sometimes be difficult. If you are unavoidably delayed, please call to let us know.

  • Outpatients west clinic reception: 020 7351 8121, extension 4515.
  • Outpatients east clinic reception: 020 7351 8121, extension 4513.

When you arrive at the clinic, please book in with reception. You will then be allocated a clinic room where you will be weighed. Other members of the CF team, including the dietitian, the physiotherapist, the CF nurse specialist and the doctor, will visit to assess and talk to you. You will have your lung function completed (blowing or spirometry tests), your oxygen levels checked, a sputum sample or cough swab taken, and you may be asked to go for an X-ray or blood tests, if these are required. 

When you have finished in the clinic, please hand your clinic slip to the reception desk where they will be able to book a follow-up appointment for you. 

If you are unable to attend a clinic, please call the appointments line on 020 7351 8011 in advance so you can rebook the appointment. 

If you are feeling unwell, and feel you need to be seen by the team earlier than your next appointment, please call the CF nurse specialists on 020 7351 8065. They will be able to advise you on how to proceed. If you happen to feel unwell overnight or at a weekend and feel unable to wait until the next working day, you should phone the main hospital switchboard on 020 7352 8121 and ask to talk to the on-call respiratory registrar for adults.
 

Domiciliary ventilation

At Royal Brompton Hospital, we pioneered the use of non-invasive ventilation in adults and children with nocturnal hypoventilation, and have over 1,600 adults and children using small por...

Physiotherapy

We offer a comprehensive physiotherapy service, covering outpatient, inpatient and community services for children and adults. 

Transition clinics

We have transition clinics for patients who want to transfer their care to the adult clinic. The clinic helps to make the transition from paediatric to adult care easier for you and your family. We run these clinics alongside the adult and paediatric teams from Royal Brompton Hospital and Great Ormond Street Hospital. But we do also have other patients who transfer to us from other CF clinics in the London area.

Read about our paediatric CF clinics.

Help with treatment 

If home IVs are an appropriate treatment choice for you, we will assess you on Lind ward day unit, or on the ward if you are an inpatient. 

If you do not have a portacath, a qualified nurse will assess your veins and discuss whether a long line or venflon is more suitable. They will teach you how to draw up drugs and safely administer IV antibiotics. Care for your IV access is carried out by a suitable nurse. 

The learning process will be tailored to you. There is no time limit and we encourage you to pace yourself and ask as many questions as you need. We will assess you on each visit, even if you have done home IVs before. This is to refresh your memory and give you the opportunity to ask questions, as procedures may have changed.

After your assessment 

We will check on you for an hour after your first dose of treatment. We need to do this each time as adverse reactions and side effects can occur.  This can happen even if you have had the medication many times before.
If you do not stay for this required hour, it could affect you being able to do home IVs in the future. 

Our pharmacy department will provide you with the first three doses of your treatment.  The rest will be delivered to your home, usually the next day by a company called Willow. They will phone you, usually on the day you start treatment and agree a suitable delivery time. You will also get a sharps bin and fridge to store your treatments if you need one.

Monitoring your response to treatment

If you start on aminoglycosides (eg:Tobramycin) we will need to check your blood levels. This will help us track your response to these treatments. It is extremely important to have checks as these treatments can be toxic. We have to take these levels and you must not miss taking them. If you do miss these, it may affect if you are able to continue home TVs in the future.

Depending on the day you start treatment you may be able to do your own blood levels at home. For Tobramycin, take your levels on Day three and seven before your dose. You can do this by pricking your finger with a fine needle, putting some blood into a special tube and posting it back to us in a prepaid box. you can also come into hospital to have them done if they are due on a Saturday or Sunday. Your GP can also take your levels, but this only under special circumstances.  

One of our nurse specialists will check your level results and only get in touch if any is abnormal. 

If you start your home IV on a Monday, Tuesday or Wednesday you can post your levels back to us. If you start your IV on a Thursday or Friday you will need to come to Foulis ward, as the due date for your levels with be either Saturday or Sunday. One of our nurse specialists will see you before you leave to explain this in more detail.  

Support

There is on-going support from the team whilst doing your treatment at home. The CF medical team and nurse specialists are available Monday to Friday from 9am-5pm. There is an on-call service at weekends.

Monday to Friday – 9am-5pm

Hospital switchboard: 020 7352 8121
CF nursing team tel: 020 7351 8065 
Email: cfhomecare@rbht.nhs.uk

Monday to Friday – 5pm-9am

Call the hospital switichboard and ask for: 
CF registrar Bleep 1011 
On-call registrar Bleep 1097 

One of our nurse specialists will call you on a Tuesday afternoon to see how you are doing.

We will see you in clinic or on Lind ward at the end of your course to see how you are doing.

We will give you a request slip to take downstairs to the administrators to book a follow up appointment.

You should make sure you have a date to return before you leave to go home.

You must attend your follow up appointment. If you do not, this could affect your opportunity to do any future IVs at home. 

You should be able to carry on with working or educations whilst having home IVs, but you can talk about this with the team when you start treatment. If you are an inpatient, we also have a hospital school team who can help you with any education needs you have. 

To get the best outcome from your treatment, it is important to eat well, get plenty of rest and have thorough physiotherapy. 

Your annual review

What is an annual review?

Your CF annual review is a bit like a car's MOT - everything gets checked!

Our clinics get busy, and even if you attend on a regular basis, an aspect of your care might get overlooked. This is why an annual assessment is helpful. It is a comprehensive multidisciplinary overview that includes tests, investigations and assessments. The results give us an update on your health and help in establishing your management plan for the following year. 

You will need to visit Royal Brompton Hospital twice for your annual review. This is so that the results of tests taken during the first visit are available for your second visit.

First visit

Your first visit is a “fact-finding mission” where we do some investigations including: 

  • lung function
  • blood tests (including vitamin A, D and E levels)
  • chest X-ray, oral glucose tolerance test (for people who do not have CF-related diabetes) 
  • a sputum specimen and a bone density scan (if required).

You will also have some assessments done by the dietitian, physiotherapist and clinical nurse specialist.

  • Dietitian assessment - you will look at pancreatic enzymes, management of CF-related diabetes, vitamin supplementation, calcium intake, dietary advice and bowel function
  • Physiotherapist assessment - this will look at your airway clearance technique, exercise and posture. It will also look at any leakage of wind/urine when coughing or laughing, and sometimes a six-minute walk test.
  • CF nurse specialist assessment - this will look at your health during the previous year. You will review your medication, allergies, medication sensitivities and hospital admission history. We will talk about your life, including:
    • employment
    • education/studies
    • family support and living arrangements
    • contraception and fertility status

This is so that we can provide you with any information you need or refer you to the right person. We will also explain what the welfare rights adviser and psychology service, and make you an appointment if you need it.

Before you see the pharmacist, they will review your medication history from your most recent clinic or discharge letter, and your GP medication record. They will then review and discuss each of your medicines with you. You can also talk about any issues or problems you are having with your medications. The pharmacist will also ask about any allergies you have, and answer any questions you have.

Second visit

The second visit is an important appointment with a CF consultant. This appointment is your opportunity to discuss your management plan with them. It will look to the coming year, based on the results of your investigations and assessments, so it is important that you have your say in this.   

If you have any questions about your annual review, please email cfannualreview@rbht.nhs.uk

Contact details: 

CF Nurses office:

Email: CFHomecare@rbht.nhs.uk
Tel: 020 7351 8065

Physiotherapists:

Email: cfadultphysio@rbht.nhs.uk

Dietitians:

Email: cfdiet@rbht.nhs.uk
Tel: 020 7351 8071

Pharmacists:

Email: cfadultpharmacy@rbht.nhs.uk
Tel: 020 7351 8059

Travelling to the centre

You can find the CF centre on Level 3 of Fulham Wing, on Fulham Road. Report to the ward administrator when you arrive. If you are attending the clinic, turn left at main reception on the ground floor as though you heading towards outpatients east. Turn right for outpatients west and follow the signs. If you feel unsure where to go, ask for directions at reception.

Read more information on how to get to Fulham Road using public or private transport.

Going on holiday

Nowadays there are more and more places to travel and activities to do. So it is natural that young people with CF are more keen than ever to make the most of any opportunity to be adventurous. But you should talk about any travel plans with your CF team.

Optimising health before travelling

You may need to have a course of IV antibiotics before you travel to optimise your health. Let your CF team know in advance about your travels dates so they can plan your admission in advance. But admission priority will always be for patients who are unwell and need treatment. We will make every effort to accommodate pre-holiday treatment but admission dates have to be flexible. You may also need to consider taking back-up oral antibiotics with you.

There may be circumstances where your CF team will recommend you not to travel. This could be because of recent haemoptysis, recent pneumothorax, gastrointestinal obstruction or acute chest infections. You can discuss this with your consultant at the time. But you may have to delay your travel plans or possibly cancel them.

Preparation

  • Fit-to-fly tests – if you have an FEV1 of <50% or oxygen saturations of <95% we will recommend you have a fitness-to-fly test. This will identify if you need oxygen on a flight. You will need to have a repeat test for each trip as you oxygen requirements may change.

  • Oxygen, nebulisers and compressors – each airline will have its own policy on oxygen transport and in-flight usage. Contact the airline before you book to find out their policy. The airline will need to know if you will need oxygen throughout the entire flight, or just at certain points. Some airlines will provide oxygen free of charge or for a small fee. The airline will request a MEDIF form, which one of the doctors from your CF team will need to complete. This will state what your oxygen requirements are and if you fit to travel. Some low-cost airlines do not supply oxygen but they will allow you to bring your own approved oxygen supply on board, such as portable oxygen concentrators. Portable oxygen concentrators are fairly new but are becoming more accepted on flights. These are lightweight and can run on electricity or battery. You can buy one but they are expensive pieces of equipment. If you need oxygen at your destination or during a stop-over, contact your UK oxygen supplier who will be able to arrange this for you.

  • Medication – you should pack a separate set of medication in your hand luggage as well as your suitcase in case your luggage goes missing. If your medicines need to be refrigerated, you should carry a cool bag or thermos flask on board. Any equipment that you will need during the flight should be battery powered. Most airlines will not allow the use of nebulisers during take-off or landing. If you need to carry out treatment/medication during the flight, let the airline know in advance.

  • Power, pumps and plugs – power supply varies from country to country. UK electrical appliances such as nebulisers may not work in certain countries. You may be able to get a travel compressor from the physiotherapy department. You should also buy plug adapters for different plug sockets.

  • Paperwork – when you are travelling abroad, keep with you a letter from your CF team listing all the medicines and equipment you need. This should include needles, syringes and compressors. Travel insurance is essential and even though though it may be more expensive, it must cover “pre-existing” conditions. Otherwise you may not be covered for CF-related treatment. When travelling in the EU you can apply for a European Health Insurance Card (EHIC). This entitles you to either free treatment or treatment at a reduced cost if you fall ill when travelling in Europe. It also covers treatment for chronic or pre-existing conditions. But this should not be a substitute for travel insurance – it only covers emergency care and does not include some medication costs or repatriation.

  • Vaccinations and immunisations – you should visit your GP as soon as possible to check if you need any vaccinations or other preventive measures (such as malaria tablets). Remember, these treatments are not usually available as NHS prescriptions.

Documentation required for patients to travel

  • Fitness-to-fly letter from doctor

  • A letter detailing current medical condition, medication and equipment

  • Travel insurance certificate

  • EHIC (if travelling in EU)

Places to avoid

Over recent years, there have been several cases of melioidosis. This is an infection caused by Burkholderia pseudomallei and can be life threatening. Burkholderia pseudomallei is found in fresh water and damp soil in some areas of Asia and northern Australia.

You should also be aware of the potential risks of going to countries where hygiene standards and medical care are poor. Australia's Northern Territory government has a helpful fact sheet. The CF Trust website for also has information on travelling with CF.

Risk of salt depletion

Heat can cause excessive sweating, which can lead to dehydration. We recommend taking Slo-Sodium (4-6 daily), if you are travelling to a hot country or if you will be doing strenuous activity such as cycling and skiing. Make sure you have enough for the entire holiday and stay well hydrated (especially if you are drinking alcohol).

General travel health advice

Sometimes you can forget general health issues when planning travel. So make sure to use a high-factor sunscreen and avoid excessive sunbathing, especially if you are on certain oral medication such as voriconazole, ciprofloxacin. You should also remember to practice safe sex and take condoms with you, as the quality of them can vary in other countries.

Web resources for transition patients

Transition information

From CF Trust:

Building independence

From CF Trust:

From the government:

CF Care

From CF Trust:

Personal relationships and adult issues

From CF Trust:

From Cystic Fibrosis Victoria (Australia):

From Talk to Frank:

From Drink Aware:

Careers advice


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