Cystic fibrosis and complex respiratory conditions in children

What is cystic fibrosis?

Cystic fibrosis (CF) is an inherited condition that affects many organs in the body.  The organs most affected are the lungs and gut.  Although CF is a life-limiting condition, advances in the understanding of CF and our approaches to management have made life better for those with the condition.  Almost all children with CF will live well into adult life.

Every child with CF is affected differently.

Other complex respiratory conditions

Many other complex respiratory conditions also affect children.  These range from birth abnormalities, genetic conditions (other than CF), to surgery, infection and viruses.

Examples of complex respiratory conditions include Primary Ciliary Dyskinesia (PCD) and non-cystic fibrosis bronchiectasis.

Who we care for

We have three teams across the state caring for children and their families affected by CF and other complex respiratory conditions. These teams are based at the Royal Hobart Hospital, Launceston General Hospital, and the North West Regional Hospital (Burnie). The Royal Children’s Hospital (Melbourne, Victoria) provides additional support to our local clinics.

What to expect at the clinic

It is recommended that children with CF (or other complex respiratory condition) visit the clinic every three months to see all members of the team.  If your child is unwell, we can see them more often.  Our team will work with you and your child until they reach adulthood, at which point they will be transferred to adult care.

Due to the complexity of care and the need to see many different health doctors, cystic fibrosis and complex respiratory clinics can take a lot of time (please allow up to 2.5 hours for each appointment).

How to access the asthma clinic

A general practitioner referral is required, which your GP can submit on your behalf. 

Learn more on the Tasmanian Health Service Outpatient Clinics website

Your CF/complex respiratory team

Your CF/complex respiratory team usually begins with the following people:

Child and family/caregiver

You and your parents/caregivers are important members of our health care team, you look after yourself and your condition every day. You can tell us about your health and how your condition affects your everyday life. We will include you in decisions about your health.

Paediatrician

Your paediatrician is a key member of your health care team, they provide specialist medical care and look at your health holistically. Your paediatrician often takes the lead in providing a diagnosis or organizes referrals for tests or assessment with other health professionals.

Cystic fibrosis nurse

The CF nurse is the primary contact person for questions and concerns. The nurse will help you to navigate the health care system and promote your child and family’s views about health. They will also teach you how to keep your child in a safe environment.

There is a CF nurse in Hobart, Launceston and Burnie.

Hobart: 6166 0000

Launceston: 6777 5247

Burnie: 6493 6300

Nurse practitioner (NP)

A nurse practitioner is a registered nurse with the experience, expertise and authority to diagnose and treat people of all ages with a variety of acute or chronic health conditions. NPs have completed additional university study at master’s degree level and are the most senior and independent clinical nurses in our health care system.

Physiotherapist (respiratory)

The physiotherapist will teach you a variety of techniques to keep your child’s lungs healthy. These include airway clearance techniques, inhalation therapy, and exercises. They will provide you with an individualised physiotherapy program for your child and will also assess your child’s posture and development.

Dietitian

The dietitian will work with you to make sure your child gets the fuel they need to grow normally. These will teach you about the specialist nutritional needs of people with CF and/or complex respiratory conditions.

Social worker

The social worker will help your child and family live with CF/complex respiratory condition. They will provide parents with emotional support, information and advice. The social worker will also make sure that your child has goals that they are working towards.

Pharmacist

The pharmacist will help you understand your medication and keep your medication plan as simple, safe and helpful as possible. The pharmacist can also give support to doctors as required.

General practitioner (GP)

Your child’s general practitioner (GP) is a key member of your healthcare team. Infants with CF need basic medical care as well as CF care. Your GP will take care of some of your child's health needs. The CF team will provide care specifically related to CF.

You will receive individual information on when you should contact your CF team and when to contact your GP.

Depending on individual needs the following health professionals may also provide care:

Respiratory scientist

Respiratory scientists conduct lung function tests to help assess the health of your lungs, airways and respiratory muscles. If you need a lung function test your paediatrician or specialist doctor will discuss this with you.

Genetic counsellor

The genetic counsellor can help you understand why your child has CF.

They can assist you and other family members with carrier testing and will provide you with information about your options for future pregnancies.

Cystic fibrosis resources

Your condition explained

New diagnosis information

• Carrier screening (CF Facts)
• New diagnosis information pack (Cystic Fibrosis Australia)
• Sweat test (CF Facts)

Comorbidities (issues that may arise alongside CF)

• Bone health (CF facts)
• Continence facts (CF facts)
• Female reproductive information for people with CF (CF Fact)
• Male reproductive information for people with CF (CF Fact)
• Related Diabetes fact sheet (CF Facts)

Living with your condition

Physiotherapy and exercise

• Airway clearance general information (CF Facts)
• Autogenic drainage (CF Facts)
• Exercise (CF Guide)
• Huff and cough (CF Facts)
• Modified postural drainage and percussion (CF Facts)
• Nebulisers (CF Facts)
• Positive Expiratory Pressure (PEP) (CF Facts)

Nutrition

• CF & Nutrition XPlained (video, medicine X)
• Healthy fats (CF Facts)
• Nutrition for CF babies (CF Facts)
• Nutrition for CF children (CF Facts)
• Nutrition guide for infants (CF Food)
• Nutrition guide for children (CF Food)
• Nutrition guide for young people (CF Food)
• Snack and meal ideas for CF (CF Bites)

Management of your condition

Medications, procedures, and admissions

• Cystic fibrosis mental health roadshow information (EMPOWERED)
• Medications (CF Facts)
• PEG (Percutaneous Endoscopic Gastrostomy)(CF Facts)
• PICC lines (Peripherally inserted central catheter) (CF Facts)
• Ports (CF Facts)
• What to expect on your first hospital admission (CF Facts)

Germs and infection control

• Common infections (CF Facts)
• Pseudomonas (CF Facts)

Effects of CF on families

• Carers (CF Facts)
• CFA Mental health roadshow information (EMPOWERED, website)
• Siblings (CF Facts)
• Travelling with children with CF (CF Facts)

Tests or investigations

• Procedural anxiety (CF Facts)

Support organisations

There are other families out there, who have been through the same thing. If you would like to be in couth with other CF families as your CF health care team or Cystic Fibrosis Tasmania). For infection control reasons, the best way to contact other CF families is by phone or internet.

Australian

• Cystic Fibrosis Australia - Cystic Fibrosis Australia is the national consumer organisation for people living with CF and their families.
• Cystic Fibrosis Tasmania - CFT offers a ranges of services for CF member families in Tasmania. Please refer to the CFT membership website

International

• Cystic Fibrosis Canada
  Cystic Fibrosis United Kingdom
  Cystic Fibrosis Foundation

Research and monitoring outcomes

• Cystic Fibrosis Data Registry

View additional resources from the Child Health (Paediatrics) team

Other support

Child Health and Parenting Service (CHaPS)

CHaPS work in partnership with families of children 0-5 years of age and aim to enhance the health and wellbeing of all young children in Tasmania, through supporting their parents to understand their child’s growth and development needs.

Learn more about Child Health and Parenting Services

Family Support Services

Some families may require increased support through a community organisation due to the complexity of their situation or other vulnerabilities.

Learn more on the Strong Families Safe Kids website

School

Tasmanian Government schools can access extra support or services to meet health and disability needs. It is important to discuss any individual requirements with school staff.

Learn more on the Department of Education website