CF Care

Annual Assessment is a yearly review to assess in detail your health status.
In preparation for your annual assessment you should:

  • - fast from 12 midnight for blood tests (whether you're diabetic or not)
  • - wear or bring with you appropriate clothing and footwear for your walking test
  • - bring your medications and inhalers with you also

Annual assessment includes:

  • - A full set of fasting blood tests, including an oral glucose tolerance test (if not diabetic)
  • - Lung function(in the pulmonary function lab or spirometry in the CF unit)
  • - bring your medications and inhalers with you also
  • - Chest xray
  • - Six minute walking test/ Modified shuttle walk test
  • - Specimen sample collection
  • - Discussions with the CF nurse, CF physiotherapist, CF dietician, and CF pharmacist around potential changes in your treatment that may benefit you.

Typically at annual assessment you don't meet a member of the medical team unless you are unwell in some way on the day. After annual assessment we will arrange a date with you to return to a clinic appointment (approx. 6-8 weeks after your annual assessment) for review of your results where you will meet the team again and also the CF registrar or the CF consultant.

All outpatient reviews including scheduled CF clinic , annual assessments or reviews outside of these appointments take place in the Adult CF day centre. The day centre which is located in the ambulatory care facility, has 5 ensuite review rooms and a fully equipped gym. There is an air filtration system in each room that sees 12 air exchanges occurring per hour along with a UV lighting disinfection system that is turned on for a period overnight to disinfect all solid surfaces in the room.

Our scheduled CF clinics run every Wednesday afternoon.

We will make contact by calling or texting you 2-3 days prior to your clinic appointment to confirm your attendance and to allocate you a time to come to clinic. Your time for clinic may differ from the time you received on your posted clinic letter as we rearrange times more appropriately nearer the day of the clinic so as to reduce cross infection risks to all patients attending. We will try to facilitate your time needs as much as possible. We ask that you text or call us when you have arrived on the hospital grounds/carpark so we can allocate a room for you to go to directly. We do not encourage you to wait in the waiting room in the ambulatory care facility unless it is advised by one of your team.

At your clinic appointment you will be reviewed by the the CF nurse specialist, CF physiotherapist, CF dietitian, CF pharmacist, and the CF registrar or consultant.

Outside of scheduled reviews, if you need a review by a member of the CF team, please don't hesitate to contact the CF nurses on 0872241824.

Should you need to access our CF team for a more urgent review contact the CF nurses by phone on 0872241824. Reviews of this nature also take place in the Adult CF day centre during day centre working hours. We will facilitate you as soon as possible based on the urgency of your issue. If the matter is an emergency you may be advised to call emergency services where you will then be brought directly to the emergency department in CUH where we will meet you. If you do not live locally, you will be brought to your nearest Emergency department. Outside of the working hours of the Adult CF day centre, all urgent reviews will be through the emergency department if the issue is too urgent to wait for review the following day in the day centre. From there if you need admission, this will be worked on as speedily as possible to ensure that you are in the emergency department for as little time as possible.

The Cystic Fibrosis Physiotherapy team are here to advise and assist patients with airway clearance techniques and exercise.
Chest physiotherapy plays a very important role in the overall respiratory management of CF. As physiotherapists we aim to provide each patient with an individual physiotherapy programme. This programme consists of airway clearance and exercise.
We can advise you on your inhaled and nebulised treatments and will carry out any test doses of new inhaled and nebulised medications along with the Clinical Nurse Specialists in Cystic Fibrosis. We also offer exercise rehabilitation programmes to both pre and post lung transplanted patients. We can help with musculoskeletal problems by direct exercise prescription or by referring you to your local Musculoskeletal Physiotherapist for further assessment and treatment. You will meet a member of the adult Physiotherapy Cystic Fibrosis team at your annual assessment, regular clinic visit, on review of new modulator therapy and as an inpatient. If you have any issues and would like to be reviewed by your physiotherapist you can contact us directly on 086 0081806.

Psychology is considered a key part of standard CF care in Ireland and the UK. We are delighted to now have two Senior Clinical Psychologists working as part of our CF team. This means that alongside your regular medical check-ups, we also want to make sure we're looking after your mental well-being. The main job of Psychology reviews is to check how you're doing emotionally and if there are any concerns related to living with CF. We will also let you know about the support available and help you understand more about Psychology.

We will try to meet you when you attend clinic for your Annual Review. While we aim for face-to-face chats, we understand it's not always possible, so we may use a telephone call or schedule a video call instead. We will chat to you, and we will also give you some questionnaires. We'll make sure it's easy for you, especially if you find questionnaires tricky. You can also choose not to complete the questionnaires; it is your choice.

Importantly, it's not just about the Annual Review. If you ever need support or want to talk to a Psychologist about difficulties related to living with CF, the door is always open. The team can refer you, and we're here to support you.

Nutritional management is an essential part of MDT care for adults with Cystic Fibrosis (CF). The main goal is to achieve good nutritional status and to maintain this throughout life. All people with CF require regular evaluation of nutritional status and body composition. Reliable and sequential measurements of growth and nutritional status are an essential part of the clinical assessment of health status in CF. Poor growth and malnutrition contribute to increased mortality and morbidity in CF and have a long term impact on lung function. A high energy, high protein diet has been a standard part of the nutritional management of CF. This approach has resulted in improved nutritional status and survival. However, recently with modern management of CF, overweight and obesity have become a problem for some. This group will need advice to adopt healthier eating principles in order to lose weight or prevent further weight gain. Our patients attend for annual assessment, 3 monthly clinics and can be reviewed on a drop in basis as required. Some patients are also admitted to CUH and are seen throughout their inpatient stay. The following are nutritional issues which are addressed

  • Track weight, height and body composition
  • Using International recommended goals: Aim for BMI of > 22 kg/m2 for females and > 23 kg/m2 for males
  • When we calculate energy requirement we take into account
    • severity of lung disease
    • degree of malabsorption
    • medications
    • activity
    • other factors
  • 85-90% of all individuals with CF are pancreatic insufficient (PI). The goal of pancreatic enzyme replacement therapy (PERT) is to minimise the symptoms of malabsorption, promote adequate weight gain and growth, and prevent nutritional deficiencies.
  • Increased need for fat soluble vitamins A, D, E, K. There are several options for CF specific vitamins and we aim to meet individual requirements.
  • Constipation is common in CF and can lead to decreased quality of life. We liaise with the Multidisciplinary Team (MDT) to try and manage this.
  • Cystic Fibrosis Related Diabetes (CFRD) is the most common co-morbidity in CF affecting about 30% of adults with CF (CF Registry of Ireland, annual report 2017). The diagnosis of CFRD results in additional treatment burden and individuals need additional support to manage this aspect of their care. The goal is to achieve and maintain optimal nutritional status and normalize blood sugars. Insulin is the treatment of choice for any patient with CFRD. A combination of long-acting and/or rapid-acting insulin is often the treatment to cover basal metabolic needs and carbohydrates consumed at meals and snacks.
  • Reduced bone mineral density is more common in CF. We aim to ensure adequate calcium intake, including calcium from food and oral supplements. We assess nutritional adequacy for risk factors for bone loss which include poor nutritional status, Vit D, Vit K and calcium deficiencies.
  • Use appetite stimulants when other causes of poor appetite and inadequate weight gain/BMI have been ruled out.
  • Enteral tube feeding can be used when it becomes difficult to meet nutritional requirements orally. Consideration to start enteral nutrition via tube feeding should be a multidisciplinary approach, including and most importantly the patient and the family in the discussion and decision.
  • Pre transplant nutrition: Patients are often malnourished as a result of severe lung disease with high metabolic demand. We can check with the lung transplant centre to determine minimum BMI requirement at that center to be listed for lung transplant. During acute illness, weight maintenance may be a practical goal.
  • Cystic Fibrosis transmembrane conductance regulator (CFTR) modulators are a new class of medications. To date in Ireland there are four different medications approved. Modulators bring changes in nutritional advice which will be tailored to the individual patient e.g weight, enzyme doses, GI Symptoms (such as tummy pain, bloating, constipation, and diarrhoea), blood glucose control and adherence issues.

You can contact the CF Dietitians on: 0860463459

With the explosion of new therapies in the area of CF over the last 10 years, and in particular the roll out of CFTR Modulators, the importance of a designated full time CF pharmacist cannot be overstated in a modern day CF MDT. Our CF pharmacist is available to talk to if you have any problems with your medicines, or you would like advice about how your medicines work, side effects, drug interactions or any other medicine-related queries.

The CF pharmacist is involved in:

  • Medication reviews at outpatient clinics
  • Optimisation of medication for best patient outcomes
  • Discussing any adherence issues
  • Identifying patient and medication risk factors
  • Preventing, detecting and reporting adverse drug reactions
  • In-patient clinical pharmacy and medicines management service
  • Ensuring the supply of medications both in and out of the hospital
  • Individualising drug and dosage requirements
  • Educating and counselling patients and carers
You can contact our CF pharmacist on 087 3566016