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Cystic Fibrosis & Lung Transplants

Wed, 12/23/2015 - 10:26 -- IV Solutions

Though cystic fibrosis affects many of the systems in the body, it is commonly recognized that the organs most profoundly affected by mucus build up are the lungs. The mucus build up, in itself, presents a challenge in breathing and consistently taking medication and breathing treatments to loosen and clear the mucus from the airways.

Coupled with the many opportunistic respiratory infections that commonly present themselves in cystic fibrosis cases, the long term damage to the lungs can become severe and offer few options for relief other than lung transplantation.

Cystic Fibrosis Lung Transplant Prognosis

Although transplanted lungs do not have cystic fibrosis, being that they come from a donor who does not express this trait, the process comes with its own risks. More than 80 percent of cystic fibrosis lung transplant recipients live for over a year after transplantation and 50 percent beyond 5 years after transplantation. Often times people with CF fare better with lung transplants than other people with lung disease, but they are still subject to the same prognosis of any lung transplant recipient.


Lung Transplant Process

We would say there are four phases in the lung transplant process, deciding if you should get one, getting started, prepping for surgery, and recovery.

Lung Transplant Process

  1. Should I get a lung transplant?

  2. Prepping for the procedure

  3. The surgery

  4. Recovery stage

Should I get a lung transplant?

People who have mild or moderate complications due to cystic fibrosis usually do not get transplants, due to the risk being too great. But, the prognosis of lung transplantation in patients with severely damaged lungs has proven to be very beneficial and greatly improved their quality of life.

Prepping for the procedure

Since the availability of lungs for transplantation is so low, many evaluations are administered before accepting a patient for lung transplantation to make sure they are a good match for transplant and have a favorable prognosis.

  • Blood Tests
  • Diagnostic Tests
  • Immunizations
  • Psychological Evaluation

Once you have been accepted for transplant, you will be placed on a list. As soon as a donor organ is available for your case, you will be notified to report to the hospital immediately so you can be prepared for transplant. The wait on a bilateral donation is often 2 years, but priority is based on the severity of the lung condition.

Surgery

  • You will be wearing a surgical gown
  • An IV will be applied to your arm or hand as well as monitors
  • You will be taken to the operating room and general anesthesia will be administered
  • The surgeon will make a horizontal incision under the chest and replace the lungs, one at a time
  • The surgeon will close the incision and you will begin the recovery stage

Recovery stage

In the Hospital

After the surgery, you will be taken to a recovery room and closely monitored for several days. Your breathing will be assisted with a ventilator until you are stable enough to breath on your own.

Immunosuppressants will be administered to prevent your body’s rejection of the new organs. When your doctor feels you are ready, you will be moved to a private room.

At Home

It’s important that you keep the surgical area clean and dry. You will be given specific instructions for daily life and follow up visits will be scheduled to monitor your progress. It is important to look out for:

  • Fever/chills
  • Redness/swelling
  • Bleeding
  • Increased pain
  • Difficulty breathing

Considerations of a Lung Transplant with Cystic Fibrosis

It is important to realize that receiving a bilateral (both lungs) lung transplant does not address the symptoms of cystic fibrosis in other organ systems. The pancreas, intestines, reproductive tract, sweat glands, and sinuses will exhibit the same cystic fibrosis symptoms as before.

In order to ensure the body does not reject the new lungs, strong immunosuppressive drugs are administered to the patient after surgery and are often administered for the rest of their life. These make is difficult for the body to fight off infection and make avoiding illnesses an important part of your daily life.

Though the mucus that provided a hospitable environment for bacteria like Pseudomonas aeruginosa no longer builds up in the lungs, it is not uncommon for germs that stayed in the upper airways after transplantation to infect the new lungs due to the immunosuppressants.


Other Cystic Fibrosis Blogs you may like

Living with Cystic Fibrosis

There are many different facets to leading a healthy life with cystic fibrosis. Keeping your airways clear, a good exercise program, and a diet high in calories are all important to the cystic fibrosis lifestyle.

Cystic Fibrosis & Exercise

Since lung function is such an important aspect of life with cystic fibrosis, it is of the utmost importance that people with cystic fibrosis get plenty of cardiovascular exercise.


REFERENCES:

Lung Transplant for Cystic Fibrosis. WebMD. Sept 9, 2014. Accessed Sept 10, 2015

Lung Transplantation. Cystic Fibrosis Foundation. July 21, 2015. Accessed Sept 10, 2015

Lung Transplantation Procedure. Johns Hopkins Medicine. July 30, 2004. Accessed Sept 10, 2015

True Survival Benefit of Lung Transplantation for Cystic Fibrosis Patients. Journal of Heart and Lung Transplantation. Dec 29, 2008. Accessed Sept 10, 2015