Respiratory and Sleep

Who we are

The Respiratory and Sleep Service at PCH provides inpatient and outpatient diagnostic services and care for children up to 16 years of age with respiratory or sleep conditions.

The service comprises:

  • Respiratory Medicine
  • Respiratory Function Laboratory
  • Sleep Disorders Unit  

Conditions we manage

Our team is experienced in the investigation and management of a range of respiratory conditions, including:

  • asthma
  • chronic lung disease
  • airway
  • lung disorders
  • cystic fibrosis

We also provide sleep clinics for children with respiratory and non-respiratory sleep disorders and run a home oxygen and ventilator support service.

Services we provide

Respiratory Support Service

  • Long term (domiciliary) oxygen and ventilatory support.
  • Infant lung function
  • Skin reactivity to common allergens
    • polysomnography (PSG) – an overnight sleep study used to diagnose sleep disorders. PSG tests can be performed by PCH sleep consultants in the Sleep Disorders Unit, in the patient’s home and at selected regional hospitals (Karratha, Port Hedland, Broome, Kalgoorlie, Busselton)
    • multiple sleep latency and maintenance of wakefulness testing – daytime tests that measure sleepiness and alertness
    • overnight pulse oximetry screening – a screening test for sleep disorders that is usually performed over one night
    • simultaneous overnight pulse oximetry and transcutaneous carbon dioxide screening – a three night assessment of the efficiency of nocturnal home ventilation in neuromuscular patients
    • Actigraphy – a non-invasive test that uses a wristwatch like device worn on the wrist or ankle to monitor human rest/activity cycles over an extended period
    • blue light blocking glasses - used to address the timing of the sleep-wake cycle

Respiratory Laboratory

Spirometry, complex pulmonary function

  • Exercise challenge testing
  • Histamine or methacholine challenge testing
  • Cardiopulmonary exercise testing
  • Evaluation of arterial oxygen by oximetry
  • Flight simulation tests

Sleep Disorders Unit

Full evaluation of sleep disorders using a range of diagnostic tests including:
  • polysomnography (PSG) – an overnight sleep study used to diagnose sleep disorders. PSG tests can be performed by PCH sleep consultants in the Sleep Disorders Unit, in the patient’s home and at selected regional hospitals (Karratha, Port Hedland, Broome, Kalgoorlie, Busselton)
  • multiple sleep latency and maintenance of wakefulness testing daytime tests that measure sleepiness and alertness
  • overnight pulse oximetry screening – a screening test for sleep disorders that is usually performed over one night
  • simultaneous overnight pulse oximetry and transcutaneous carbon dioxide screening – a three night assessment of the efficiency of nocturnal home ventilation in neuromuscular patients
  • Actigraphy – a non-invasive test that uses a wristwatch like device worn on the wrist or ankle to monitor human rest/activity cycles over an extended period
  • blue light blocking glasses - used to address the timing of the sleep-wake cycle

Special services

  • Evaluation of fitness to travel by air (simulated aircraft cabin testing at 14 per cent oxygen)
  • Bronchoscopy services – flexible fibro-optic bronchoscopy can be performed on appropriate cases
  • Coordination of a multi-disciplinary Cystic Fibrosis clinic
  • Ambulatory respiratory support, including domiciliary oxygen and positive pressure support

Cystic Fibrosis

Cystic Fibrosis (CF) is a genetic illness that affects mucus-producing glands and causes a build-up of thick, sticky secretions in the lungs and gut. This leads to recurrent chest infections, impaired digestion, and poor growth. Currently there is no cure for CF. In Western Australia, CF is usually diagnosed in the first few months of life through the WA Newborn Screening Program. The management of a patient's cystic fibrosis is a multidisciplinary team approach, with close cooperation and collaboration with the patient and their family.

What causes Cystic Fibrosis?

The gene defect for cystic fibrosis is inherited when both parents have the same abnormal gene and unknowingly pass it on to their baby. A parent who has the abnormal gene but not the disease is called a carrier. The health of a carrier is rarely affected. However, for each pregnancy of two such carriers, there is a 1 in 4 chance that the child will be born with the disease. CF occurs in about 1 in every 2,500 births in WA. One in every 25 people in Australia is a carrier of the gene for CF. CF is most common in persons of a Northern European background, but it does also occur in other populations.

What problems does Cystic Fibrosis cause?

The symptoms of CF vary, and some children are less severely affected than others. Most children tend to cough and wheeze frequently and they may develop repeated lung flare-ups. Many also have digestive problems due to blockage of digestive enzymes from the pancreas, which causes the child to grow slowly. The sticky secretions in the gut may cause a bowel blockage, called meconium ileus, which occurs in about 15 per cent of babies with CF. The amount of salt in sweat is also increased and this forms the basis of the sweat test used to diagnose CF.

Although there is no cure, early diagnosis and treatment are important as recent medical advances have greatly improved the outlook for children with CF.

How is Cystic Fibrosis treated?

Treatment usually involves different strategies to help clear airway secretions including chest physiotherapy regimens, inhaled treatments and antibiotics to prevent and treat infections. Children with CF are given a high-calorie diet supplemented with vitamins and often need special medications that contain pancreatic enzymes.

Screening issues

The screening test measures the amount of a pancreatic enzyme in the blood, called IRT. Those babies with high levels of IRT then have their screening sample tested to see if they have the CF gene. Any baby who has the CF gene is then recalled for a sweat test. This screening approach will find about 95 per cent of the babies with CF. The test will also find a small number of healthy babies who are carriers of the CF gene.

Cystic Fibrosis pre-order medication form (PDF)

Technology Dependent Children 

Our Clinical Nurse Consultant for Technology Dependent Children (CNC TDC) provides specialist care to children who require long-term ventilation via a tracheostomy (breathing tube). Working closely with other members of the clinical team in the hospital and community, the CNC TDC assists these patients with their transition from Paediatric Critical Care to a ward and then to home.

Referrals

We accept referrals for children up to 16 years of age from other metropolitan or rural hospitals and health services, as well as GPs and private providers. In emergencies where patients require surgery or non-operative treatment within 48 hours, we accept referrals from the PCH Emergency Department. 

Hours

Inpatients: 24 hours a day, seven days a week.

Outpatients: Monday to Friday, 9.00am – 5.00pm.

Overnight services: Tuesday, Wednesday and Thursday nights, 4.00pm – 7.30am.

Location

Inpatient services:

  • Paediatric – Ward 2B
  • Adolescent  – Ward 4A

Our Respiratory Laboratory and outpatient clinic is located in Clinic F on Level 1.

Our Sleep Lab is located in Clinic G on Level 1.

These areas are most easily accessed via the Pink Lifts.

View the PCH map for more information.

More information

Pharmacy

Cystic Fibrosis WA (external site)

Cystic Fibrosis WA - Newly diagnosed support   

Cystic Fibrosis pre-order medication form (PDF)