Clinics and wards used by the Cystic Fibrosis Unit

The Cystic Fibrosis Unit at Great Ormond Street Hospital (GOSH) treats patients on Leopard Ward and runs several clinics. Leopard Ward is our respiratory ward, treating a wide range of children from newborn to 17 years. It is located on Level 2 of the Premier Inn Clinical Building. Within the ward there is a dedicated CF area with five beds, all of which have ensuite facilities and enable one parent to stay with their child overnight. Each room has its own small fridge so that families can bring in extra snacks/food for the admission.

Children with cystic fibrosis may be admitted to the ward for a variety of reasons including:

• an acute chest infection
• planned course of intravenous antibiotics
• minor surgical procedures
• other complications of cystic fibrosis

The cystic fibrosis outpatient clinic is held on a weekly basis and takes place in the Outpatient department on the fourth floor of the Royal Homeopathic Hospital on Great Ormond Street on Tuesday afternoons.

Each clinic is attended by at least one consultant and the full Multidisciplinary team.

For cross infection purposes, all patients are segregated and therefore receive timed appointments. On arrival, children and families are allocated a clinic room and then (apart from height and weight measurements) all other consultations including lung function take place within that room.

Each room is cleaned before use by another patient.

If the clinic visit is an annual review follow up (see below for more information), the family should always be seen by a consultant.

Height and weight are measured. If there has been a significant weight loss a urine sample may be requested (to check for sugar).

• Lung function and oxygen saturation measured on all children over approximately three to four years of age (oxygen saturation only in those under three).
• A clinical nurse specialist is always available to discuss any issues.
• A physiotherapy assessment including review of airway clearance technique, exercise and sputum sample or cough swab will be undertaken at each visit.
• A nutritional review will usually be undertaken at all routine clinic visits.
• A medical review (by a consultant or Fellow).

The Cystic Fibrosis Psychology and Social Work team can be available during the clinic to see children and their families.

Each week the Cystic Fibrosis Multidisciplinary team meet to review and discuss patients due in CF clinic, patients after their clinic visit and any other patients who require a team discussion about their management. Any relevant results or changes in treatment are discussed and plans for future management are decided.

A letter is then sent to the GP with copies, where appropriate, to the local paediatrician and other relevant health care professionals. Parents are also sent copies of clinic letters, where this has been requested. If parents have signed up to MyGOSH then all correspondence will be available for them to see on this platform.

We endeavour to provide holistic, family-centred care believing that children thrive when both they and their families are empowered and supported by staff.

All children with cystic fibrosis are seen once a year for a detailed clinical assessment – rather like an MOT where we assess the current status and progress over the previous year. Treatment plans are then put together for the forthcoming year. The process is split into two parts, the assessment day and the actual review in CF clinic approx. 6weeks later with a consultant once all the results are to hand.

The annual assessment day takes place on Wednesdays and Thursdays each week

Once the appointment is made, families receive a full timetable for the day along with a food diary, which should be completed the week before the annual review day. It is important also to bring along your airway clearance device if you have one (eg PEP mask, Acapella, Flutter).

This is the day that you attend hospital to see the various members of the team and have all the necessary tests. The appointment times with the team are longer than in a usual cystic fibrosis clinic to give the opportunity for a thorough review of progress. No results of tests/investigations are discussed on this day, unless of course a specific problem is identified.

Review by team members:

• medical review (usually undertaken by the cystic fibrosis fellow) includes:
  • Detailed review of past year
  • Clinical examination
  • Medication reviewcystic fibrosis nurse specialist assessment
• cystic fibrosis nurse specialist assessment:
  • Review of the last year in terms of adherence, positives & challenges
  • School and nursery advice and support
  • Education
  • Transition
• dietitian review includes:
  • review of intake, vitamins and enzymes
  • nutritional advice
• psychology/social work review
• physiotherapy assessment includes:
  • review of airway clearance method and technique
  • postural assessment
  • exercise review (including an exercise test so please wear suitable clothing)
  • cough swab/sputum collection

Investigations undertaken on the day includes:

• height and weight
• lung function (in all children over five and may also be pre-arranged on an individual basis in younger children
• chest x-ray
• blood tests
• sputum/cough swab (done during physiotherapy assessment)
• bone scan (every two years from age 12)
• other investigations such as ultrasound of the abdomen are completed from 3yrs of age

The appointment for the review will take place at GOSH six to eight weeks following the assessment day during a Tuesday afternoon cystic fibrosis clinic. This will be a longer consultation than a routine clinic appointment.

At this appointment all the findings from the annual review are discussed with the family and child (if old enough) and a clear plan of management for the following year is made. This is the most useful part of the review for both parents/carers to attend if possible. Children and families will of course have the opportunity to ask questions and raise any concerns.

The review results, discussions and plan of care will be summarised in a letter and sent to you soon after the appointment.

If there are any more urgent issues highlighted by the review the family will be contacted immediately and appropriate follow up made.

The Cystic Fibrosis Unit runs an outreach service.

Home visits, when indicated, are undertaken by one of the cystic fibrosis clinical nurse specialists or cystic fibrosis physiotherapist. These appointments cannot replace routine hospital appointments and are undertaken only when there is a specific indication (eg during a course of home IV antibiotics)

The cystic fibrosis social worker may also undertake occasional community visits. These visits are arranged on an individual basis as necessary but families can also request a visit (although this cannot be offered in place of routine hospital appointments).

The aims of the service are to provide assessment, support, guidance and education for children and families from diagnosis through to transition to adult care on all issues of cystic fibrosis care.

Outreach clinics at our formal network hospitals are undertaken at regular intervals, usually twice yearly, and are attended by the GOSH specialist Cystic Fibrosis team (consultant, clinical nurse specialist, physiotherapist and dietitian). The clinics are held jointly with the local network team.

In certain circumstances, it may not be appropriate to wait until a Tuesday clinic to review a child who is unwell. In these cases, children may be given one-off appointments to see the cystic fibrosis fellow and/or clinical nurse specialist. These appointments usually take place in our outpatient department.