Case 1
A 25 years old university graduate student diagnosed of cystic fibrosis with pancreatic insufficiency in childhood was started deteriorating clinically due to uncontrolled infection in chest, not responding to multiple courses of various intravenous antibiotics over 6 months. Her main complaints were cough with copious green expectorations and shortness of breath with which she could barely manage her household work. After detailed clinical examination and investigations, she was accepted on the waiting list for lung transplantation. Following repeated respiratory infections, her lung function deteriorated rapidly (Forced vital capacity, FVC 28% and forced expiratory volume in 1 s, FEV1 25%) and she became bed ridden. As it became apparent that her clinical condition was falling so fast that she will not survive a waiting for suitable cadaveric donor lungs, her family was given a choice of live related lobar lung transplantation, for which it agreed. The donors were her biological father, aged 60 years (Height: 182 cm, weight: 87 kg) and real sister, aged 24 years (Height: 176 cm, weight: 65 kg). The recipient height and weight were 164 cm and 53 Kg. The recipient, the right lobar donor (father) and the left lobar donor (sister) were taken to the different theatres with gap of half to one hour each, in that order. The recipient had clamshell incision and both the lungs were dissected. She was put on cardiopulmonary bypass (CPB) and right pneumonectomy was performed. The right lower lobectomy was performed in father through right postero-lateral thoracotomy at the same time and the lobe was brought to recipient theatre. The bronchial anastomosis (4–0 PDS, continuous) was followed by pulmonary artery (6–0 polypropelene, interrupted) and pulmonary venous (6–0 polypropelene, continuous) anastomoses. The left pneumonectomy was performed in the recipient and the left lower lobe was obtained from the sister and anastomosed in similar fashion. The ischemic time for right side was 2 hours while for left side it was 1 hour and 5 minutes. The recipient was weaned off CPB with modest inotropic support and nitric oxide and blood gases were suggestive of good gas exchange. Immunosuppression protocol in LDLLT was similar to cadaveric donor lung transplantation, consisting of tacrolimus, mycophenolate mofetil and prednisolone and was started in the patient on the day of transplantation. Recipient was weaned off ventilator in 48 hours, but had slow recovery and long intensive care unit stay due to infection with Pseudomonas, persistent pleural effusions, renal failure needing intermittent hemofiltration for a month and early rejection requiring re-intubation and methyl prednisolone pulse. She was discharged home 3 months after surgery and over another 6 moths her lung functions and exercise tolerance kept improving steadily. Over the last 12 years her respiratory function tests have fallen twice but bronchial biopsy never showed rejection. Her latest lung function showed predicted FVC of 65% and predicted FEV1 of 66%. Figure 1 shows her latest chest x-ray. She is full time, efficient office worker. Post-operative period was uneventful for both the donors who made fast and excellent recovery and were discharged home on 8th day.
Case 2
Another woman of 34 years with cystic fibrosis related end stage lung disease had complaints of cough, haemoptysis, dyspnoea, early fatigue and was requiring nocturnal oxygen therapy. Her clinical condition and lung function deteriorated rapidly following several repeated bouts of respiratory infections. Implied, that it was difficult for her to wait for cadaveric donor organs, her family was given option of LDLLT. As her mother, who already agreed to donate a lobe was only close relative alive; her altruistic friend expressed a wish to donate a lobe. She (Height: 164 cm, Weight: 50 kg) received a right lower lobe from a 24 years old altruistic friend (Height: 164 cm, Weight 72 kg) and a left lower lobe from her biological mother, aged 54 years (Height: 154 cm, Weight: 80 kg) through a clamshell incision in a way mentioned in case 1. The ischemic time for right side was 2 and half hours while for left side it was 1 hour and 20 minutes. The patient was weaned off ventilator on third day; however her post-operative course was complicated by fungal endophthalmitis requiring right side evisceration. She was discharged after 2 months. At the end of one year, she developed collapse of the right lung due to stenosis of right bronchial anastomosis which required endobronchial stenting. Her latest lung function showed predicted FVC of 78.6% and predicted FEV1 of 84% and she is leading a healthy life. Figure 2 shows her latest chest x-ray. Post-operative period was uneventful for both the donors, the friend was discharged on 5th while the mother on 7th day.