JACQUELINE ON CYSTIC FIBROSIS & LUNG TRANSPLANT

Written by,
SHANAZ MD SHAHROM

As a young baby, Jacqueline Monaghan was diagnosed with Cystic fibrosis (CF). This disease is an inherited disorder caused by mutations in a gene on chromosome 7 which involved in regulating moisture on epithelial surfaces. This mutation makes a protein called cystic fibrosis transmembrane regulator (CFTR) to become abnormal. As a result, CF patients produce thick and sticky mucus rather than the thin, watery type. This condition manifests in many organs for instance the upper and lower airways, pancreas, bile as well as reproductive tracts. In most patients, the cause of death is mainly due to lung disease. The mucus would clog the airways causing bacteria to remain in the lungs and this may lead to infection.

During her early childhood, Jacqueline was able to enjoy life as a normal young girl. However, life got more complicated when she started secondary school as she needed more antibiotics. She decided to go into medicine just as she was entering university. At that time, she had a lung capacity of 45% and was not in her healthiest state. However, she managed to finish the course. She had a part-time job but was barely able to work. Jacqueline proceeded into Psychiatry after her junior year. As time passed, her lungs were getting more damaged and she risked getting respiratory failure. At the moment, the only cure for respiratory failure is a lung transplant. She went on waiting list for lung transplant 3 years ago.

Being on the waiting list, Jacqueline felt very anxious as they could call her at any time and undergo the surgery. For patients with CF, a successful lung transplant gives them about 50% chance of surviving for the next two years. CF is an unpredictable condition; the lungs can have an acute infection or otherwise have no function for a long time. Thus, it is very difficult to perform a transplant. As soon as a lung is available, at least two donors were called to the hospital. The donors and the patient were examined base on their physical condition, blood type and other specific requirements. A match was found when a donor and a patient’s medical and biological information were the same.

On an April morning, Jacqueline received a call at 3.45a.m stating that a matching donor was found. She flew to Newcastle and was anesthetized in a hospital. The operation began at 10.00a.m that very day. After she had awakened, she felt strange and experienced a lot of pain every time she tried to breathe deeply. Her breathing became easier the next two days and only after a week was she able to breathe normally. She felt more comfortable with her new lungs.

A year after Jacqueline’s surgery, she was amazed at the things she could do, namely her daily routines. She was grateful that she had her family supporting her throughout her illness and most importantly the donor of her new lungs. Jacqueline enjoyed taking on her part-time job and appreciated her life ever since.

Jacqueline is only one of the many cases of patients involving a lung transplant. There are still many people eagerly waiting for a suitable donor in hopes of saving their lives. But for Jacqueline, she had only just begun to ‘live’.

Reference: Student BMJ Podcast Transplantation, www.student.bmj.com