Annual review for tamariki (children) and rangatahi (young people) with Cystic Fibrosis (CF)

Summary of main changes to guideline

• DEXA scans recommended at 10 and 15 years (previously every 2nd year from 12 years of age)

• OGTT or CGT recommended at 10 and 14 years (previously every year from 12 years of age)

Key points

• All tamariki and rangatahi with CF in Aotearoa New Zealand should have a full clinical review of their progress over the past year. This is generally undertaken around their birthday month, unless they are in a smaller centre when timing may coincide with a visiting CF specialist clinic.

• It is recommended that all testing should be undertaken a few weeks before the annual review appointment. The results can then be discussed with the relevant multi-disciplinary team (MDT) members at the annual review appointment. 

• The tamariki/rangatahi should be reviewed by all members of the CF team (e.g. doctor, nurse, dietitian, and physiotherapist) and testing undertaken as outlined below.

• Members of other healthcare teams may be required and should be referred to where necessary e.g. clinical psychologist/psychiatrist, social worker, play specialist, endocrine, gastroenterology and ENT teams.

• An annual review letter should be made available with comments from all members of the CF team plus all results of annual testing. Copies should be sent to whānau (family/families) or kaitiaki (guardian), GP and shared care consultants or regional CF team, and copy to rangatahi from 14 years of age. It is the responsibility of the SMO to collate the MDT results and provide the annual review letter. This letter provides the basis for subsequent reviews and any changes made should be highlighted.

  • The annual letter should include list of present and past problems, known allergies, list of medications currently taken, hospital admission days over past 12 months (including inpatient and home IV days), surgeries over past 12 months, IV access with any problems, growth - current and 12 months ago, lung function (with changes to previous), heart rate, respiratory rate, oxygen saturations, blood pressure, annual blood results with glucose monitoring results (abnormal highlighted), summary of sputum cultures over past 12 months, urine / stool results, radiology findings (include CXR, abdominal ultrasound, bone density), annual reports from physiotherapist, dietitian and nurse, summary of care for past 12 months and plan for future.

• The nurse specialist, clinic nurse or clinic co-ordinator will organise and co-ordinate all annual tests.

• All data to be entered into the NZ PortCF database by an identified member of the CF team.

• Infection Control recommendations must be followed for all children with CF. 

Testing required prior to annual review clinic

• Lung Function

  > 6 years spirometry (and plethysmography if available)

• Blood testing
  

  0 - 4 yrs of age

  5 - 18 yrs of age

  Vitamin A,D & E HbA1C FBC ESR CRP Na/K/Cl, Urea, Creatinine, Glucose Calcium, Phosphate Magnesium LFT - Total Bilirubin, ALP, GGT, AST, ALT Total Protein, Albumin

  Vitamin A, D & E HbA1C FBC ESR CRP Na/K/Cl, Urea, Creatinine, Glucose Calcium, Phosphate Magnesium Zinc if malabsorption/faltering growth concerns Total IgE Aspergillus precipitins Aspergillus RAST APTT, PR, Fibrinogen Iron/Ferritin LFT - Total Bilirubin, ALP, GGT, AST, ALT Total Protein and Albumin OGTT or CGM at 10 years and 14 years of age (Annually for those with frequent steroid usage, poor nutrition/growth, deteriorating chest)

• Sputum microbiology

  Obtained by expectoration or cough suction/cough swab
  MC & S, fungus, non-tuberculous mycobacteria (NTM only on sputum not cough specimens)

• Urine: electrolytes and creatinine, albumin/creatinine ratio

• Radiology:

  • CXR - AP & Lateral including a Brasfield score.

  • Abdominal ultrasound

    Abdominal ultrasound to be done at ages: 3 years, 6 years, 9 years, 12 years and annual thereafter. If there are concerns with previous ultrasound abnormality, hepato-splenomegaly or abnormal liver enzymes, repeat abdominal ultrasound annually. If elastography measurements are available: should start at 6 years of age with abdominal ultrasound.

• Bone Densitometry (DEXA):
  DEXA scan at 10 years and 15 years of age, OR annually on those:

  • on frequent or long term steroids

  • with poor growth +/- poor nutrition

  • any vertebral abnormality on standard CXR

  • fracture over past year

Additional annual diagnostic tests to be considered

• If pancreatic sufficient: consider faecal elastase and faecal chymotrypsin to check for developing pancreatic insufficiency if clinically indicated e.g. poor growth, gut/malabsorption symptoms.

• For those on PERT: Faecal steatocrit, faecal chymotrypsin, faecal calprotectin where indications / evidence of malabsorption, constipation, gut inflammation, bacterial overgrowth, poor / faltering growth, suboptimal nutritional status or persistent under nutrition re CF weight status criteria.

• For those on modulator therapy: faecal elastase and faecal chymotrypsin to check for changes / improvements in pancreatic function (annually or more frequently as appropriate).

• If there has been long term NSAID use or frequent aminoglycoside courses: consider screening for nephropathy with Cystatin C.

• If Cystic Fibrosis related diabetes (CFRD) is present: screening for complications is not required until minimum of 5 years after diagnosis of CFRD.

• Blood pressure from 6 years of age, and on all those on modulator drugs

• Audiometry should be performed annually if tamariki / rangatahi has had IV aminoglycoside over past year PLUS:

  • Baseline before the start of treatment for NTM and repeated after 1 year

  • Annually in all children starting on regular 3 monthly IV aminoglycosides OR

  • If high aminoglycoside trough level over past year

• ECG before starting long term Azithromycin

• Oxygen saturation.

Clinic roles

Clinic doctor (lead consultant if possible)

• Discussion and clinical examination by one of the clinic doctors. Discuss overall progress over the past year, including all annual tests performed e.g. (lung function, chest radiology, blood testing, ultrasound of abdomen, bone density, and sputum microbiology results etc.)

• Identify and document allergies.

• Review will also include usual symptoms and number of oral and IV antibiotic courses over past year (hospitalisation and home IV courses)

• There should also be an emphasis on growth and puberty. Review gut symptoms including bowel motion frequency, constipation, staining, abdominal pain, excessive flatulence, bacterial overgrowth indicators.

• Rangatahi should be seen in the clinic room by themselves from age 14 years for the first part of the consultation. The doctor will also ensure that the issue of transition to adult services and fertility has been discussed with rangatahi at the appropriate age. 

Cystic Fibrosis Nurse

CF Nurse to assess the following areas:

• Home situation

• Social supports

• School review and issues identified

• Discuss if school/preschool visits needed and arrange when appropriate

• Emotional wellbeing. YouthCHAT for rangatahi from 13 years of age (if available at centre) https://youthchat.co.nz/

• HEADSS assessment undertaken for rangatahi https://starship.org.nz/guidelines/adolescent-consultation

• Psychological screening using:

  • PHQ-A Patient Health Questionnaire - Adolescent version (PHQ-A) to screen for depression in children aged 12 years and above

  • GAD-7 screening tools. Generalised Anxiety Disorder - 7 item scale (GAD-7)

• Fertility issues

• Transition and transfer to adult services discussed. All rangatahi will be transferred to adult care by 18 years of age, but can occur between 16-18 years of age or as per hospital policy. Transition to be discussed with rangatahi and whānau from 13 years of age

• Smokefree screening and cessation support offered as required. Risks of vaping also discussed

• Vaccination history including annual influenza vaccination. Print out / review Aotearoa Immunisation Register every year, check for overdue vaccines and reminder about annual influenza / COVID vaccinations

• Dental history, enrol with local dental clinic from 1 year of age, to be seen annually.

• Equipment maintenance

  • Review home nebulisation equipment (compressor and nebulisers)

  • Compressors require an annual service, electrical and output checked, filter changed

  • Nebulisers, antibiotic filter valve sets, masks, physiotherapy devices and spacers are to be replaced annually.

• Review inhaled medications

• Special Authority numbers reviewed

CF Nurse to provide education for the following (as appropriate):

• Review of CF education with CF knowledge, adherence and impact of CF.

• Portacath / PICC / gastrostomy

• Discuss and arrange admissions for inpatient care when required.

Dietitian

The nutrition review should be done in conjunction with the 2017 Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand and local Nutrition Standards of Care for infants and children with CF and the NCPT process (Nutrition Care Process Terminology). https://thoracic.org.au/clinical-documents/cystic-fibrosis/

Areas covered in the Annual Review include: 

• Anthropometry: weight, length, wgt/lgt centile (WHO) under 2 yrs of age, BMI centiles 2-18yrs, growth trends. CF weight status from BMI centile. Sub-optimal weight status and persistent under-nutrition weight status require detailed review with the MDT. Skin fold measurements if appropriate, REE (resting energy expenditure) with Delta traq measurements if appropriate.

• Biochemistry, urinary electrolytes

• Dietary assessment: food and fluid intake, salt intake, quantitative food record assessment, infant feeding and complementary foods, feeding behaviours.

• Pancreatic enzyme replacement therapy (PERT)

• Nutritional supplements, oral nutritional supplements and enteral feeds, vitamins, probiotics, laxatives

• Bowel motions: signs of malabsorption, constipation, straining to pass stool, excessive flatulence, bacterial overgrowth

• Changes in nutritional and clinical status

• Previous nutritional assessment and goals

• Exercise and activity

• School and preschool care

• Cystic Fibrosis Related Diabetes

Physiotherapist

The physiotherapist review should follow CF Physiotherapy Australia and NZ Guidelines: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4840479/#resp12764-supitem-0001

Areas covered in the annual physiotherapy review with education and advice of airway clearance technique including review of equipment and inhaled medication treatments.

• Exercise regimen reviewed

• Urinary incontinence discussed from 8 years of age

• Exercise testing if required but not routinely done