Bronchiectasis acute exacerbation
Bronchiectasis is a chronic lung disease that can occur at any age. Without treatment the disease can be progressive, leading to breathlessness and deteriorating lung function
Note: there are also guidelines on the diagnosis and aetiology of bronchiectasis, as well as general bronchiectasis management
Definition
An increase in at least 3 of the following symptoms for at least 48 hours.
frequency of cough
severity of cough
sputum volume and / or consistency
sputum purulence
breathlessness and / or exercise tolerance
fatigue and / or malaise
haemoptysis
Fever is not typically seen in bronchiectasis exacerbation, and presence suggests either intercurrent viral infection, or a possible pneumonia.
History
Identification of an exacerbation requires knowledge of clinical features present when the child is at their best and this should be documented in clinic letters. This will allow assessment of acute changes.
Examination
Features that often indicate an exacerbation include:
Increased sputum productivity, or wet quality to audible cough if child cannot expectorate
Increased respiratory rate
Increased work of breathing
Increased hyperinflation
Coarse crackles - may be widespread or localised to the areas involved
Wheeze - may not be bronchospasm as bronchial secretions can fill the airways, causing obstruction to airflow.
Management of acute exacerbation
Treatment of an exacerbation can usually be achieved with oral /enteral antibiotics and an increase in daily physiotherapy frequency and duration. Children who are moderate to severely unwell, hypoxic or who have pneumonia should be admitted acutely for intravenous therapy (see below)
Outpatient management
Prescribe a minimum of 2 weeks of a broad-spectrum oral antibiotic.
Chest physiotherapy should be done at least twice every day.
If the whānau/family are already doing physiotherapy twice daily then increase to three times daily.
Even if you suspect a viral aetiology for the respiratory exacerbation, antibiotics are normally prescribed to reduce the microbial load.
Antibiotic choice is guided by sputum bacterial culture and sensitivity. For initial treatment, empirical treatment based on likely organisms, or previous species present in the sputum samples can be clinically effective.
Prescribe doses recommended in the NZ Formulary for Children.
Haemophlus influenzae is frequently found and is usually sensitive to amoxicillin/clavulanic acid or cotrimoxazole. Use amoxycillin if Haemophlus influenzae is known to be sensitive. If the child is allergic to penicillin, oral cefaclor, cotrimoxazole or erythromycin should be considered.
Ongoing symptoms: if the child has clinically improved, but is still not back to baseline clinical status, a further 2 weeks of oral antibiotics should be given. Decision to repeat sputum/ cough suction/ NPA should be made on individual clinical circumstances.
Pseudomonas aeruginosa
When Pseudomonas aeruginosa is first detected, repeat the specimen, and clinically determine if the child is well or not.
Discuss with a Starship respiratory specialist whether eradication therapy is appropriate. Patients with bronchiectasis and Pseudomonas aeruginosa have worse health outcomes and higher symptom burdens than those without.
Treatment
One course only - check NZ Formulary for dosing
Outpatient oral therapy for mild illness: Oral ciprofloxacin for 2 weeks. Tablets: 250 mg, 500 mg and 750 mg
Named Patient Pharmaceutical Assessment (NPPA). Liquid formulation of ciprofloxacin is a Section 29 product and not subsidised on the PHARMAC Community Schedule. If required for use in the community a NPPA application is needed. In some situations, it may be supplied by the hospital inpatient or retail pharmacy according to Rule 3.5 of the Pharmaceutical Schedule.
Making liquid from tablets: Ciprofloxacin tablets can be halved then crushed and dispersed in a small volume of water or food to administer to young children. The tablets are not soluble in water so part doses cannot accurately be given. For this reason, it is preferable to round doses to the nearest half tablet size where possible.
Inpatient treatment for Pseudomonas aeruginosa causing moderate to severe clinical illness: IV ceftazidime and tobramycin for 10-14 days. This will usually be for “acute” clinical decline
Admission for intravenous therapy
Children who are moderate to severely unwell, hypoxic or who have pneumonia should be admitted acutely for intravenous therapy (see below).
Children who have had several weeks of oral therapy (>4 weeks continuously, or > 3 x 2 week course in last 3 months) may also be considered in need for admission. Whilst this may not be perceived as an “acute admission,” it should not be unnecessarily deferred because the child seems “reasonably well in themselves.”
If the child’s condition deteriorates despite being on oral treatment.
If acute admission is contemplated:
For SCH patients - please consult with the on call respiratory consultant to determine immediate management, and preferred location of admission.
For non- SCH patients, the local paediatric SMO on call is welcome to call the on call SCH respiratory paediatrician to discuss any aspects of care of such children.
If admitted, chest physiotherapy should be done a minimum of twice daily and ideally with paediatric physiotherapy oversight. A third session of physiotherapy may be indicated, but this often is done by the parents/carers.
Usual initial IV treatment in bronchiectasis is amoxycillin/clavulanate or cefuroxime for 10 - 14 days.
Duration of IV treatment may be shorter if the child recovers enough to reach predetermined clinical discharge criteria.
If admitted a PICC or long line is preferable to a peripheral venous cannula, due to better durability than a peripheral venous cannula.
Haemoptysis
This is rare in bronchiectasis. It may range from blood-stained sputum to large volumes of fresh blood (and/or clots) and be life threatening.
Small volume haemoptysis (volume < 5 mLs):
Stop physiotherapy for minimum 48 hours, or until ongoing blood loss stops
Start oral antibiotics
Consider tranexamic acid
Discuss with on call paediatric respiratory consultant at SCH
Moderate volume haemoptysis (volume up to 240 mLs – may be fresh or old blood, child hemodynamically stable):
Stop physiotherapy for minimum 48 hours, or until ongoing blood loss stops
Start oral (or intravenous) antibiotics
Start tranexamic acid
Discuss with on call paediatric respiratory consultant at Starship
Large volume haemoptysis (>240 mls/24h, or child haemodynamically unstable, or needing supplemental O2 or other respiratory support):
Ensure child is hemodynamically stable
Discuss with on call paediatric respiratory consultant at Starship
Arrange transfer to Starship or to another centre where child can receive interventional radiology, and/or thoracotomy and surgery if bleeding doesn’t settle.
Start IV antibiotics (chose generic acute IV antibiotics for bronchiectasis)
Start tranexamic acid
Cross match blood
Ventilate locally via IPPV if large volume (>240mls) in one time
Repeated large volume haemoptysis, or a single episode of life threatening bleeding may be an indication for lung transplant referral.
Investigations for acute bronchiectasis exacerbation
Community
Sputum: In appropriate circumstances (e.g. failure to respond to recent course of antibiotics, or if admitted for IV treatment) a sputum sample, or cough suction, may be helpful to guide therapy.
Samples should be sent for send for microscopy, bacterial culture and sensitivities. A cough suction sample (this is not the same as a nasopharyngeal aspirate) should be taken in a younger child who is unable to expectorate sputum. A nasopharyngeal aspirate may be done, if cough suction is not practical at all. However, the likely yield of identifying lower respiratory tract bacteria is much lower.
Inpatient
Sputum: as above
Oxygen saturations:
These may be normal, low or borderline (90-92%) during the day. If borderline or normal check nocturnal oxygen saturations (overnight oximetry).
If the child has significantly low saturations, appropriate oxygen therapy should be initiated.
We suggest a repeat overnight oximetry, prior to discharge, if initially abnormal, to confirm saturations have returned to normal.
Inflammatory indicators (FBC, CRP, ESR):
These are often normal
If a child is constitutionally unwell these tests may track systemic recovery
Chest x-ray:
Not routinely required
Perform if clinically indicated
Spirometry:
Where possible spirometry early on in the admission (if not done in the week or two before coming in), and repeated towards end of admission, as may be useful as a marker of recovery.
If the FEV1 has not returned to expected baseline, this may inform decision making for timing of clinical review, possibly treatment duration, and/or a decision to discharge on a further course of oral antibiotics.
Flexible bronchoscopy. This is usually not indicated during an acute admission. It may be considered if:
Child is not making expected progress during the admission
There is significant atelectasis on CXR, which is affecting overall wellbeing e.g. causing a need for O2, or other respiratory support.
If the primary team is unsure if bronchoscopy is warranted they should discuss with the SCH on call paediatric respiratory SMO
Physiotherapy clinical opinion
Further assessment of pace and extent of recovery can be obtained by seeking the opinion of the attending physiotherapist(s) over the duration of the admission.
Socio economic factors
Inpatient admission may allow for further exploration of any specific socio-economic factors the whānau /family are coping with. Where possible, solutions to these issues should be sought and implemented during the admission.
Respiratory support
Children with bronchiectasis may have severe disease or co-morbidities requiring acute or long term respiratory support. Such support may be nocturnal supplemental home oxygen, or Bilevel support. Colleagues who have a patient who may benefit from long term respiratory support should discuss the child with the on call paediatric respiratory SMO.
Children needing acute respiratory support, beyond low or high-flow oxygen (to a maximum FiO2 of 0.4), should be discussed with an HDU (at SCH or locally) in the first instance.
Discharge criteria
Conventionally, children are admitted for bronchiectasis for intravenous treatment for 10-14 days. Admission can be difficult for some whānau/families with regards to arranging care for other children at home, employers agreeing to time off work, costs incurred to attend hospital, dislocation of carer (or child) from their usual support networks.
We suggest:
Where possible, the child’s primary consultant indicate a predetermined endpoint for an adequate response to inpatient treatment
If the child reaches the agreed endpoint before 10 days of therapy, they may be able to be discharged earlier than predicted
Endpoints may include any, or all of:
Regaining their “well” FEV1
Regain of weight/ BMI centile
Cough becomes dry
Improvement of overnight desaturations
Cessation of haemoptysis for >48h
Discharge to continue home IV therapy, once a line placed and a safe home treatment plan arranged
Bronchiectasis Admission Planner
