Role of the Cystic Fibrosis Specialist Nurse

• Part of the multi-disciplinary team but his/her role acts as a link between the team members and the patient as often she/he is first port of call.
• Good communication between the multidisciplinary team, patients and families as problems are often resolved more easily if pathways of communication are well established for all parties.
• There are great demands on the patient and team therefore the patient and family advocacy is the most important role for the nurse specialist.
• Involved in the decision making ensuring each patient receives optimum care and acting as a link between patient and family, community services and hospital multidisciplinary team.
• Part of the genetic screening process providing support and education.
• Providing education for parents and patients as there are many treatment regimes that have to be learnt throughout the patient’s life. Successful teaching of the patient and all concerned (parent, carer, school and work colleagues) and their understanding of the disease process will ensure that treatment is carried out safely and effectively and any issues surrounding adherence to treatment can be dealt with.
• Facilitating home iv service.
• Linking in with other speciality’s as needed diabetes, liver disease, neurology, gastroenterology, Cancer, rheumatology, pain team ,Palliative care.
• Education and research is an  important part of the CF nurse’s  role in developing own professional practice and keeping up to date with new advances in treatment and new development in the world of CF.
• The wards also have link nurses to act as information gatherers for both the CF Team and the wards.

Role of the Cystic Fibrosis Dietician

We work with patients, families and carers to help ensure:

• Food and nutritional intake is optimised for children to grow normally and adults to have a healthy weight.
• The correct pancreatic enzymes (e.g. Creon, Nutrizym) are taken and how many capsules are needed. This will help the patients get the most from their food.
• The necessary extra fat soluble vitamins (A,D and E) and salt supplementation are given at the right doses.
  How do dietitians work?
• Patients are reviewed every 2 months including an annual review.
• The  dietitian will review patients on the ward during each hospital admission and will liaise with the catering department if extra meals, snacks or supplements are needed.
• The Dietitian will work closely with the Diabetologist and Diabetes Specialist Nurses to ensure optimum glycaemic control. We provide education as needed regarding diet and diabetes in Cystic Fibrosis.
• We will provide written information, education resources and individual dietary plans to help support the patient’s  nutrition and enzyme plan.
• Dietitians liaise with schools, colleges and G.P practices as needed and can arrange visits to home, school, university, workplaces as required to provide additional education.

Our Aims

To provide patient’s families and carers with adequate support and information to help them to maintain a healthy BMI. We aim to support patient to achieve optimum digestion and absorption with appropriate pancreatic enzyme replacement therapy and adequate vitamin and salt supplementation. To work with the Diabetes team to optimise glycaemic control. This will help to maximise good lung function and live a long and healthy life.

Role of the Cystic Fibrosis Physiotherapist

Physiotherapy plays a key role in the respiratory management of Cystic Fibrosis; providing an individualised treatment programme in order to improve quality of life and promote independent Cystic Fibrosis management.

The role of the physiotherapy team at the NWMCFC is to provide continuous assessment, advice and education on:

• Airway Clearance Techniques (e.g. active cycle of breathing, postural drainage, percussion, cornet, acapella, flutter etc) 
• Management of breathlessness 
• Nebuliser therapies (e.g. Ineb, e-flow, portaneb, PARI) 
• Nebulised or inhaled medication trials 
• Exercise and exercise testing 
• Musculoskeletal and postural problems 
• Urinary Incontinence 

If you would like further information regarding any of the above topics please contact the Cystic Fibrosis Office on 01782 675105 or discuss with your physiotherapist at your next clinic appointment.