Notes on a talk given at the Annual CFRI Conference by Jim Theodore, M.D.
Jim Theodore, M.D., Medical Director of the heart-lung transplantation program at Stanford University Medical School, delivered a sobering presentation on the criteria for selecting cystic fibrosis patients as candidates for lung transplantation. He cautioned us on the seriousness of the procedure and insisted that it required a special kind of person to be able to survive the ordeal.
He began by introducing the audience to the different kinds of lung transplants they perform. CF patients must receive a double lung transplant. In a single lung transplant, the other infected CF lung would remain behind and eventually contaminate the new lung with bacteria. He briefly touched on the controversial lobe transplants, where family members donate a lobe to a CF patient. While these are not currently being done at Stanford, other centers were having some success with this procedure (he specifically mentioned Dr. Starnes in Los Angeles).
Dr. Theodore reviewed the general indications for lung transplantation. The patient must be in the end stages of cardiopulmonary or pulmonary disease with a life expectancy of less than two years. There must be a capacity for rehabilitation, someone with good physical preserve. Except for the underlying disease, the person should be in pretty good health and free of multisystem disease. Desperate clinical situations are not in themselves indications for transplantation. A marked debilitation might mean that a patient could not survive the procedure, and if the physicians do not think the patient looks like he or she could survive, they will not subject the patient and family to the stresses and pain of the process.
More specifically, cystic fibrosis life expectancy (1-2 years for transplantation eligibility) is determined by examining a number of factors. Deteriorating pulmonary function, increasing hypoxia (inadequate supply of blood to the tissues) and hypercapnia (excessive carbon dioxide in the blood) are all considered. Dr. Theodore said that if a person is in pretty good shape but is showing some carbon dioxide retention, that is a good signal to start pushing for a transplant. If a patient is spending an increasing number of days in the hospital for pulmonary functions (e.g., monthly), and is experiencing a decrease in his or her ability to perform daily living activities, these are also signs of decline. Also, any recurring life-threatening pulmonary complications such as pneumothorax (air entering the pleural cavity-the space between the two layers of pleura lining the lungs and the chest wall-from the lungs or outside) or hemoptysis (coughing up blood) are taken into account. Finally, Dr. Theodore said the candidate must possess the ability to undertake and be committed to a major medical endeavor.
General contraindications for heart-lung transplantation include a significant systemic or multisystem disease (although with CF patients they might be more lenient). There can be no active or extrapulmonary (outside the lung) infections, and no significant hepatic (liver) or renal (kidney) disease. Steroid therapy is a major contraindication especially for pulmonary transplants because the steroids interfere with the healing process at the site where the organ is sewn in. Patients must be on 5 mg./day of steroids or less to be considered. Cigarette smokers and patients with drug or alcohol abuse or psychiatric illness will not be considered under any circumstance. And patients with previous cardiothoracic surgery must be examined on a case by case basis.
More specific to cystic fibrosis, there are several absolute contraindications. There can be no pan-resistant (organisms that do not respond to antibiotics) respiratory flora. After transplantation, patients are on maximum immune system suppression to avoid organ rejection. Any organism that can not be treated will easily spread throughout the body and cause death. Poor renal function cannot be accepted. Again, post-op patients will take cyclosporin, a drug extremely hard on the kidneys for anyone. CF patients who have absorption problems must take increased dosages. Weak pretransplant kidneys will not be able to handle this drug. Anyone with an active viral infection, such as HIV or hepatitis, or poor hepatic function (bilirubin greater than three) cannot be accepted. Finally, candidates with a lack of adequate social support or history with poor compliance with medical regime will not be considered. Dr. Theodore stressed the importance of this final statement, adding that unlike CF physicians, transplantation doctors are dictators and no negotiating is allowed.
Dr. Theodore then turned to what he called relative contraindications, less complex problems that they were willing to try to overcome. Cachexia (overly thin, weight less than 70% ideal for height) is handled with supplemental feedings. Evidence of recurrent aspergillus or other fungal colonization of the sinuses (or respiratory tract) must be addressed. Antrostomies (surgery in which openings are made into the sinuses) are commonly performed before transplantation. Dr. Theodore reminded us that the sinuses will always have CF and those infections cannot be allowed to drain down into the new lungs. Diabetes mellitus must be under control. If blood sugar is currently out of control, it will only get worse post-op when patients are on corticosteroid therapy to prevent rejection. Finally, if a patient has been on a ventilator for more than one week, the patient's muscles might have weakened and chances of becoming infected with antibiotic-resistant organisms are high. Dr. Theodore said that unlike other transplant lists, all patients have to wait their turn on the lung transplant list. Someone on a ventilator might not be able to wait.
In conclusion, Dr. Theodore discussed the major long-term problems with the transplant, which are rejection, infection, obliterative bronchiolitis (OB), hypertension and renal disease. However, he said the long-term consequences of these complications are no greater in CF patients than in other patient populations. In CF patients, long-term survival is equal to and often greater than that seen with other disorders. For selected patients with endstage cystic fibrosis, heart-lung and double lung transplantations appear to be effective forms of therapy.
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