Help regarding lung transplant

Question

Hello,
I would like to ask about possibilities for CF treatment if one already depends on LTOT (long-term oxygen treatment). My brother-in-law was diagnosed with CF 3 years ago, up till then he had been treated for asthma, which seems very strange to me, because this illness can be easily diagnosed these days. My brother-in-law is now 36. Ha has refused to be treated because of his family, he did not want to admit that his disease and kept avoiding new information even if he went through regular check-ups to Bratislava. His doctor had said at the beginning that the progress would not be as fast. During this year his state has worsened dramatically that he stayed in the hospital and only oxygen therapy for 15 – 22 hours a day can help. I am mainly interested in lung transplant and the risk of this operation – will it be successful and what complications may occur.
Thank you very much for your reply.

Answer

Hello,

If a patient needs LTOT (long-term oxygen treatmet), it can be said that the disease is at an advanced stage and it is necessary to perform tests prior to lung
transplant ( I suppose your brother-in-law has significantly decreased lung function – FEV1 less than 30 %). Not every patient is suitable for this operation. We do not perform the operation if the patient is colonised with Burkholderia cepacia genomovar 3A, colonisation with other pathogens – mycobacteria and aspergillus is also dangerous. Good state of nutrition is also necessary, serious malnutrition prevents from lung transplant as well as advanced lung disorder – the patient would not handle the operation.
There are some risks which have to be taken into account: During the operation, there might be the risk of the anesthesia, such as reactions to medications or problems in breathing. During surgery, bleeding might occur or it could lead to an infection. After transplantation, there is the risk of failure of the transplanted organ or rejection of the transplanted organ. To avoid rejection, the immune-system has to be suppressed by drugs, called immunosuppressive drugs. This long-term immunosuppression brings a higher risk of acquiring an infection.

Concerning the success of a lung transplant: The perioperative mortality could be reduced in the last years due to an improvement of the surgical and intensive care medical methods, as well as due to new drug therapeutic options. The mortality rate within the first three months after transplantation is about 10-15% in experienced centers. In case of CF a significant improvement of the prognosis can be calculated for transplanted patients compared to non-transplanted patients if suitable patients had been chosen. The statistical data of the International Society for Heart and Lung Transplantation shows a 5-year survival rate for CF patients of 53%, and a 10-year survival rate of 34%; while there are experienced centers which receive a 10-year survival rate of 51%. This emphasises the importance of a good longterm post-transplant care. Longterm survival rates of CF patients having recieved a lung transplant can be greater compared to patients having received a lung transplant for other reasons.

In general it is a very individual and very sensitive problem. Information about possibilities and risks of lung transplants specifically in case of your brother-in-law should be provided by his doctor. Please, do not hesitate to contact him. To analyse his situation in detail and to clarify if lung transplant could be an option for him, a referral to a transplant center might be helpful after having spoken with his CF specialist.

All best and good luck, Libor Fila and Jitka Brazova

03.06.2009