Idiopathic pulmonary fibrosis (IPF)
Even thought IPF patients have high scores on the waiting, this is still a deadly disease that needs to be taken seriously. Those patients are not offered a fair chance at transplant because they are often referred to us at the end stage of the disease and their condition deteriorates quickly and disqualifies them for transplants. One of the biggest issues is the lack of knowledge and misconceptions from physicians in the community. The truth is: patients with IPF should be referred as early as when they receive their diagnosis.
IPF has no cure other than a transplant, prognosis is generally poor. The median survival after diagnostic is only 2-3 years. Quality of life is far from being perfect. Patients are given a death sentence without any hope.
However, there is hope!
Reasons to start lung transplant evaluations sooner:
- It allows the transplant team to begin a complete evaluation without rushing the patient and caregivers. Evaluation is 3-4 full days of testing and this can be very stressful if done in a row.
- It allows plenty of time to address medical/surgical/social issues during work up.
- It gives transplant centers more time to monitor the patient for compliance.
Specific example: an IPF patient already on 4-6 L/min and needed a stent in his heart during work up heart cath. Unfortunately, he could not be listed right away due to the high risk of bleeding and clogging the stent. Some patients are lucky an will eventually be transplanted, others will get too sick and will die before.
- It gives more time for patients and caregivers to make arrangements for the post-transplant period.
- Post lung transplant outcomes are better if the patient was listed and transplanted before getting too sick.
IPF is very stressful for the body but transplants are even more stressful. You other organs need to be a good health in order to make it. So pulmonary rehabilitation should be started before the transplant in order to build some stamina and muscles before getting the surgery. Pulmonary rehabilitation does increase quality of life. In short, if you know someone diagnosed with IPF in relatively good health, they should be referred to transplant at least to see what it is all about. You can attend classes provided by the transplant center to learn about the process and make an informed decision.