Cystic Fibrosis -Transplantation

The following general information has been provided as a basic guide to people with CF. For information that relates to their personal health and situation people are encouraged to discuss the issue with their CF specialist.

Lung transplantation is an option for people with advanced lung disease resulting from CF.  This surgery is now available for adults in Australia at these centres:

• The Alfred Hospital, Melbourne

• St. Vincent’s Hospital, Sydney

• The Prince Charles Hospital, Brisbane

• Royal Perth Hospital, Perth

Making the decision

Your CF specialist may discuss the option of a lung transplant with you when you have developed severe lung disease.  This may be when your lung function levels have fallen below a certain point, when you have had an increased frequency of hospital admissions or you have an increasing resistance of bacteria to antibiotics.

Referral

When you have made the decision to have a lung transplant your doctor will refer you to one of the transplant centres for assessment.  This will involve a series of clinical and laboratory tests both physical and psycho-social to determine suitability.

Lung transplantation may not be appropriate for some people with CF as there are some factors that increase the risk of complications developing after transplant.  Your CF specialist will be able to discuss these with you.

Type of lung transplantation

In CF the method of lung transplantation is bilateral sequential transplant where both lungs are removed and replaced with the donated lungs.  This removes the risk of a new lung being infected by the bacteria in the remaining lung.

Does CF “go away” after transplant?

The transplanted lungs come from people who do not have CF, so the new lungs do not have and will not develop CF.  However, after transplant the person still has CF in the pancreas, sweat glands, sinuses and reproductive tract.  The person still has to take enzymes with food and to help absorb the anti-rejection medications.

The transplanted lungs come from people who do not have CF, so the new lungs do not have and will not develop CF.  However, after transplant the person still has CF in the pancreas, sweat glands, sinuses and reproductive tract.  The person still has to take enzymes with food and to help absorb the anti-rejection medications.

Challenges

Transplanted lungs are susceptible to infection, rejection and other complications that require treatment.  Following transplantation the immune system is suppressed to help prevent organ rejection and this will decrease the ability to fight infections.  Therefore people must become vigilant about their drug treatment in that they need to take their drugs at the same time every day.  This ensures that the most constant levels of immunosuppressive drugs is maintained.  At first, this, along with the number of drugs, can be quite overwhelming.  However, people with CF tend to adjust easily as they are used to taking daily medication.  They are swapping one lot of treatment for another.  They are also used to attending outpatient clinics and generally taking an active role in the management of their treatment.  In addition their carers and families are well rehearsed at providing much needed support.

The immunosuppressive drugs may cause side effects such as diabetes, kidney problems, cancer-like tumours and osteoporosis.  Your transplant centre will be monitoring your progress.

It is important to maintain contact with your CF Clinic post transplant as they are skilled in the total management of CF which still affects you, apart from your lungs.

Waiting

There is a shortage of donor organs in Australia and the wait for lungs is (on average) between 9 – 12 months.  The waiting time is very difficult for most people as they struggle to maintain their weight and level of physical activity in an atmosphere of uncertainty.   Acceptance on the active waiting list does not guarantee that suitable lungs will be found in time.  When suitable donor organs become available the best matched recipient will be contacted.  The length of time waiting for new lungs makes little difference to priority on the list. The person with the most compatible blood group and size will be selected.  If there is more than one person considered to be suitable then the sickest person gets priority.

Life expectancy after lung transplantation

Currently in Australia over 85% of all people (not just with CF) undergoing bilateral, sequential lung transplants survive at least one year and 60% are still alive five years after the transplant.

Benefits

After recovery from the surgery many people with CF find they have more energy, can gain weight more easily and are more active than they were before.