Standards of care for people with CF

If you have CF, you should be looked after by a team of specialist healthcare professionals who are trained and experienced in CF. This team should include:

• Specialist doctors, who work with you to make decisions about your treatment and oversee your care.
• Clinical nurse specialists, who provide clinical care, education, advocacy and support for many different aspects of CF care. 
• Physiotherapists, who assess and treat issues with joints, bones and muscles, urinary incontinence, airway clearance, inhaled therapies, and exercise plans.
• Dietitians, who assess your nutritional health and will advise on ways to manage and improve this.
• Clinical psychologists, who use non-medical ways to help you with emotional or physical health challenges, usually through ‘talking therapy’. Read more about CF clinical psychology services.
• Social workers, who assess and address social issues to help you to manage your daily living, health, and welfare. 
• Pharmacists, who review and optimise your medication. 

There should be good communication between your CF team and your general practitioner (GP). Your GP is responsible for prescribing the routine medication that your CF team recommend, and making sure that you have at least one month’s worth of medication at a time. Your GP should also help you with routine vaccinations, population screening programmes, and health problems that are not related to CF.

You will be reviewed regularly by your CF team – exactly how often will depend upon your individual needs and preferences. At a minimum, you should see your CF team once a year for a face-to-face review. When you go into clinic, your CF team might ask to:

• Measure your growth (in children) or weight
• Monitor your lung function through spirometry (by blowing into a tube) or pulse oximetry (using a monitor clipped onto your finger)
• Take a sample of your airways (such as coughed up mucus or a cough swab) to check for lung infections
• Measure your blood pressure.

You might prefer to have virtual consultations (via video or telephone) with your CF team, supported by remote monitoring (ways to monitor your health from home). If you are monitoring your health from home, your CF team should provide you with the appropriate equipment and training to do this.

Once a year, you will have an annual review, which involves a detailed assessment of every aspect of your CF and treatments. This is to monitor changes, see if treatments can be improved, and plan for the year ahead. An annual review is a process, and does not necessarily need to happen all in one day, but will always include some in-person contact. As well as the usual measurements, your CF team take when you go into clinic, for annual review they may also ask to:

• Take a chest x-ray
• Take an ultrasound of your liver and spleen
• Check your bone mineral density with a type of X-ray scan (known as a DEXA scan)
• Perform exercise testing
• Check your blood glucose levels
• Collect data for the UK CF Registry
• Take a blood test.

Your CF team might not ask you for everything in that list, some tests are only needed once you reach a certain age or if there is a clinical reason for it. Once all your results from annual review are available, you should be sent a report to discuss with your CF team.

You do not need to wait until your next appointment or annual review to get in touch with your CF team. You should have clear contact details for your CF team, including out of hours, to be able to reach them whenever you have questions or concerns.

Screening can help to identify problems before there are symptoms so that treatment can be started early. Screening as part of CF care should include:

• Annual screening for liver disease through a blood test, and a liver and spleen ultrasound from three years of age and then when needed
• Annual screening for CF diabetes (such as through an oral glucose tolerance test or continuous glucose monitoring) from 10 years of age
• Psychosocial screening by a psychologist and social worker as part of annual review
• Screening for reduced bone mineral density with a DEXA scan, which is offered depending on your risk factors, baseline scans, and fracture history
• Screening for an increased risk of hearing loss caused by taking certain antibiotics. This involves a blood test that looks at your DNA to check if you have a variant (mutation) in a gene that means you are at an increased risk of experiencing hearing loss with aminoglycoside antibiotics. This test is only done once, ideally immediately following a diagnosis of CF. If you’ve not been tested, but think you should have this test done, discuss it with your CF team at your next appointment or at your annual review. Find out more in our factsheet.
• Due to an increased risk of colorectal cancer within an ageing population of people with CF, bowel screening should be offered. However, there are currently no UK bowel screening guidelines for people with CF. If your CF team are able to offer bowel screening, they should follow the US guidance from the Cystic Fibrosis Foundation, which recommends that screening should start from the age of 40 years (or 30 years for those with a solid organ transplant), repeated every three to five years.

Your GP should invite you to take part in other NHS population screening programmes that are not related to CF.

If you need to be admitted to hospital as an inpatient, you should have your own room with an ensuite bathroom. This is to help prevent you from catching an infection. If you are a parent or carer of a child with CF who is admitted to hospital, there should be facilities for you to stay with them whilst they are in hospital. Children with CF who cannot attend school because they are in hospital should also have access to learning, such as through hospital school or schooling at their bedside. Having an individual healthcare plan in place and planning for known admissions in advance can help schools to support a child with CF and ensure school work is available to do whilst in hospital. As well as learning, children should also have access to play and entertainment whilst they’re in hospital.

Whilst you are in hospital, you should:

• Have an assessment from your physiotherapist upon admission, who should then be available to deliver, or supervise, your physiotherapy treatment twice a day (including weekends) whilst you’re in hospital, or more often if necessary.
• Have a choice of high-quality food and drink during your admission to meet your needs, and your dietitian should be available to input on this. 
• See a consultant with expertise in CF at least twice a week, and they’ll be updated daily by the rest of the team.
• Have a pharmacist review and prescribe your medication when you’re admitted and as needed during your stay, as well as when you leave hospital for your discharge medications. 
• Have access to a clinical nurse specialist, a clinical psychologist, and a social worker. 

If you don’t have a totally implantable venous access device (such as a portacath), your healthcare team will need to make sure they can easily access your veins to give you medicines, fluids, or take blood. This is usually done by inserting a peripherally inserted central catheter (a thin tube that is put into a vein in your upper arm).

When you are ready to leave hospital, you should be a given a follow-up appointment. A summary of your discharge plan should be sent to you, your GP, and your shared care consultant (if you have one).

Infection can be spread from one person with CF to another, so hospitals must make sure that people with CF are isolated from each other. If you are admitted as an inpatient, you should have your own room with an ensuite bathroom. Hospitals should have processes in place to avoid people with CF being in contact with each other in waiting areas, such as in CF clinics, pharmacy, and radiology. Other ways to stop the spread of infection include keeping facilities clean, good hygiene practised by staff (particularly hand washing) and cleaning equipment between patients. 

Most CF centres in the UK offer a home-based care service, which is usually provided by the clinical nurse specialist. Home-based care might include:

• Clinical assessments at home e.g. weight, monitoring blood sugar, and lung function.
• Psychological and social support and advocacy
• Home intravenous (IV) antibiotic courses – this is suitable for some people with CF and requires training to administer IV antibiotics and close monitoring to assess progress
• Flushing totally implantable venous access devices
• Optimising airway clearance techniques and encouraging exercise programs
• Collecting airway samples
• Discussing personal issues, such as urinary incontinence
• Pregnancy and postnatal support
• Supporting home enteral feeding (tube feeding)
• Support when starting a new treatment 
• Health education
• Liaising with local authorities, nurseries and schools, workplaces, and GPs and other healthcare providers
• Support when waiting for a transplant
• End-of-life and bereavement support for family members.

CF teams should support people with CF to reach their goals throughout life, for example in education, career development, travel, and/or parenthood. The new edition of the standards of care gives more detail to CF teams on how they should adapt their service to meet the needs of people with CF at different life stages. As people grow older with CF, this might involve CF teams working more with other clinical specialties, as well as primary care, to help deliver co-ordinated care for health issues that can arise with age. 

You should be involved in decisions about your care and how you receive this. These decisions should be made jointly between you and your CF team, taking into account your opinions, preferences, and other aspects of your life. Your preferences matter, and you should be encouraged and empowered to play an active role in your care, sharing your views and preferences with your CF team. The standards outline best practice in CF care and could be a resource to help you advocate for your care. 

“I found the standards of care helpful when I was attending a meeting regarding the care I received in hospital. It was easy to find the information I needed and was worded in a clear and accessible manner.” Tracey who has CF.

The standards outline best practice in CF care, but they are not a strict set of rules that all healthcare professionals must legally follow. In other words, they are not designed to replace clinical judgement. Knowing what is recommended best practice can help healthcare professionals to make decisions about your care and treatment, but there are other factors that will influence these decisions, such as your personal preferences and circumstances, your CF team’s expertise, and what the NHS can provide. Some aspects of your care may be different from what is recommended in the standards, and there may be important reasons for this related to your health and wellbeing. Therefore, if you feel that the care you receive does not align with recommended standards, you should speak to your clinical team in the first instance to find out if this is the case for you.

In some cases, your clinical team may not be able to provide what is recommended in the standards for other reasons, such as limited resources. You might be able to work with your clinical team to try to overcome these barriers, for example, by writing a letter, making a formal complaint, or seeking help from Cystic Fibrosis Trust. 

If you do not feel like you are getting the level of care outlined in the standards and a discussion with your clinical team has not helped, you have various options including asking for a second opinion, asking to be transferred to a different unit, or even making an official complaint. 

The standards address all aspects of CF care in hospital and out of hospital, building in the latest advances in what we know about CF and how it is managed. For example, the third edition covers topics such as virtual consultations and remote monitoring; CFTR modulators; person-centred CF care; sustainability in CF care; sexual health, fertility, pregnancy and parenthood; ageing with CF; and more.