We’ve put together the answers to some of the most common questions we’re asked. If you’d like more information, or have a question that hasn’t been asked here, please get in touch.
Cystic fibrosis is New Zealand’s most common life-threatening genetic disorder that affects breathing and digestion. It leads to the lungs and digestive system becoming blocked with mucus which can cause chronic lung infections and problems with weight gain and malnutrition.
It is caused by a faulty gene that is inherited from both parents. This is known as a recessive condition. In New Zealand one person in 25 carries the cystic fibrosis gene, usually without knowing it. In New Zealand the incidence of two couples with CF genes occurs in one in 625 couples. If two carriers have a child there is a one in four chance that their baby will have cystic fibrosis. About 550 New Zealanders have CF.
Cystic fibrosis does not impair intelligence in any way, nor does it affect mobility or change the way people look. People will not know someone has cystic fibrosis unless they choose to tell them.
No, cystic fibrosis is not a contagious illness. It only occurs by inheriting the CF gene from both parents.
Cystic fibrosis is often known as ‘CF’. Other names are mucovoidosis or mucoviscidosis
All newborn babies are screened for cystic fibrosis using the heel prick blood test. This is carried out by the midwife a few days after birth. This test is for the most common faulty cystic fibrosis genes that occur in New Zealand and other inherited disorders.
A positive heel prick test for cystic fibrosis does not necessary mean a baby will have cystic fibrosis. Further testing is carried out such as a sweat test in hospital to find out if they have the condition.
Around one in 10 children with CF are diagnosed before, at or not long after birth because they have a condition called meconium ileus that causes their digestive system to become blocked with meconium which is a thick sticky substance that all babies produce before being born. Urgent surgery is needed and this can be the first sign of cystic fibrosis.
Some children with rare CF genes or who did not receive the heel prick test at birth can be diagnosed in later years. This is known as a late diagnosis and may occur with children who are born in some countries overseas where newborn screening is not carried out.
CFNZ recommends people interested in their carrier status to contact their GP. There is free carrier testing available for close relatives of people with CF or families with a strong history of the disorder. Contact the Genetic Health Service for more information:
Genetic Health Service contact numbers:
More than 2000 faulty genes have been identified that cause cystic fibrosis. There is a wide range of how people can be affected by cystic fibrosis. Some people have few symptoms and others can be unwell throughout their childhood and adult years.
Life expectancy is impossible to predict because this will be different depending on the severity of the illness. In New Zealand we have no accurate data on average life expectancy but respiratory specialists believe it to be currently in the mid to late 30s. This means that half of those living with CF will live longer than that. However, a baby born today can be expected to live longer.
Most babies with cystic fibrosis will begin physiotherapy right from birth to help clear their lungs of mucus. This is normally performed with percussion which involves clapping and vibrating the chest.
Breathing exercises in the form of games can be introduced from the age of two and from nine years most children should be able to do their own physiotherapy.
A compressor is a device that helps people with CF to take medication. It changes liquid medicine into a fine mist. You then breathe in the mist through a mouthpiece or a mask, called a nebuliser. Often the compressor and nebuliser together are simply referred to as a 'nebuliser'. If you're ever unsure, just ask the person you're speaking with to clarify.
From a young age many children are taught to use a compressor and nebuliser to inhale treatment for their lungs. Many will be prescribed hypertonic saline which helps thin mucus. Others will also need inhaled antibiotics to treat lung infections.
Most people with cystic fibrosis have pancreatic insufficiency. This is because blocked ducts in the pancreas prevent enough enzymes for the normal digestion of food, especially fatty food and vitamins A, D, E and K. Special enzymes are needed to help digest food. In New Zealand these are known as Creon® and are taken with each meal. A high-fat diet is also encouraged to maintain a healthy weight, and salt replacement may be required in hot weather or when a lot of salt is lost through sweat.
Fertility is not normally or just slightly affected in women but in most cases men are infertile because the tubes which carry the sperm (vas deferens) are usually blocked or absent. Sexual function is completely normal but men often need the assistance of reproductive technology to extract sperm from the testes to become a father. Some women with CF may be advised not to carry a child if it could harm their health.
Pre-implantation Genetic Diagnosis (PGD) or Pre-Natal Diagnosis (PND) enables two cystic fibrosis carriers to have a child that does not have cystic fibrosis. IVF is used to conceive a baby and then the embryos screened for cystic fibrosis before implantation. There is a free cycle of PGD for families who have a CF child. However, there is a long waiting list. Find out more about family planning.
It’s recommended that people with cystic fibrosis do not have contact because there is a chance they could have dangerous bacteria in their lungs that can be passed on to another person with cystic fibrosis. This is because the type of mucus in their lungs can trap bacteria causing serious infections. CFNZ has a cross infection policy for both indoor and outdoor events where people with CF are advised to stay 1.5 metres apart. Find out more about cross infection.
There is no cure for cystic fibrosis and a lung transplant can be the best option for someone whose lung function has dropped to a very low level and other forms of treatment are no longer working. Not all patients will be candidates for a lung transplant because some may have serious lung infections and ‘bugs’ that will affect the ability to recover. Some people also choose not to go on the transplant list.
Some people also experience organ rejection and sadly do not recover after a transplant but studies show 70 percent of people with CF survive for at least five years after a lung transplant.
In some cases other organs are also affected by cystic fibrosis and may also need transplantation such as the liver, kidneys or pancreas.
Families and those with cystic fibrosis can be entitled to government subsidies and benefits, as well as practical and emotional support from Cystic Fibrosis New Zealand. Contact your CF Social Worker for more information.