How Lung Transplants for Cystic Fibrosis Patients Have Changed
For those with cystic fibrosis (CF), lung infections are the greatest contributor to death. Lung inflammation, destruction, and chronic infection occurs due to the thick sticky mucus associated with CF.
This over-proliferation of mucus occurs due to the mutated gene CFTR which is responsible for the symptoms of cystic fibrosis. Damage to the body from the gene mutation prevents the normal passage of salt and water across cell membranes. This leads to the production of thick mucus.
This sticky substance adheres to the lining of the respiratory tract and serves as a breeding ground for bacteria. It can accumulate to form mucus plugs, making it very hard to breathe. CF also adversely affects mucus production in the digestive system and other organs.
Lung transplant statistics
Since the condition is a chronic and widespread attack on the human body, a lung transplant is not a cure for CF. But, it can offer a time of relief for persons in the late-stage of the disease. A lung transplant grants 45% of patients up to 10 additional years of life.
Good outcomes from the transplant are not guaranteed if the person is carrying a chronic lung infection.
Still, long-term care after the surgery remains the key to success for patients with cystic fibrosis who have undergone a lung transplant.
Burkholderia cepacian complex
Due to concerns of excessive mortality rates after lung transplantation for CF patients with lung infections, many transplant centers refuse to accept CF patients infected with drug-resistant organisms such as. B. cepacia.
For many people with CF, infection with the B. Cepacia complex cannot be traced back to exposure with another infected person. In these cases, infection may have occurred by coming in contact with it in the natural environment.
Nevertheless, because many CF patients carry chronic lung infections, some surgical centers have continued to provide lung transplantation as an option for infected persons.
Unfortunately, lung infections are known to remain with some patients after transplantation.
Progressive respiratory failure is experienced by CP patients, particularly in the last-stage of this condition. Providing respiratory support by ventilator along with the use of an ECMO unit- like a heart and lung bypass machine- has enhanced the survival rate.
Use of this system to “bridge” CF clients in preparation for a lung transplant has been an option to prepare those with advanced CF for lung transplant surgery.
However, despite the evolving and successful use of this approach to assist CF patients in respiratory failure, the use of mechanical ventilation and ECMO are unacceptable for lung transplant surgeries at a number of centers.
From the Cystic Fibrosis Foundation in Maryland, we learn that the best approach in the treatment of CF is paying meticulous attention to drugs and treatment to hold the condition at bay. By following prescribed medication and testing regimens, people with CF are given a good chance of delaying last-stage cystic fibrosis.
The Cystic Fibrosis Foundation
You may refer to the CF Foundation site for clinical care guidelines to include infection prevention and control (most important-see the site’s attached article: For Patients: 7 Ways to Guard Against Germs in Healthcare Settings), nutrition and gastrointestinal issues, and respiratory care (great articles on treatments and therapies.
Also, be sure to scan the section on depression and anxiety-conditions. They are common among those fighting cystic fibrosis.
Lung surgery approval
While lung transplant surgery can lengthen and improve quality of life for any person in late-stage CF, getting approved for the surgery involves an extensive evaluation process.
First, you need to be considered as a successful candidate for surgery. Generally, lung transplant procedures are for those in the end-stage of lung disease. In other words, the procedure is meant for those who have exhausted all medical choices and are not expected to live more than a year if they don’t get the transplant.
Other medical conditions the patient may have will need to be explored in relation to any possible effects on surgery or recovery. (For example, active tuberculosis, hepatitis B infection with surface antigen expression, and HIV infections absolutely contraindicate transplantation.)
Who’s an ideal candidate?
An ideal lung transplant candidate is also one who has family/friends who will be there during the patient’s recovery period. Additionally, the candidate must have financial resources to pay for the transplant.
Also, the applicant needs to be someone who can be counted on to keep their post-op medical appointments and to remain attuned for any problems requiring medical consultation.
In preparation for speaking with your medical provider regarding your decision to explore the transplant process, you may wish to review information about your specific lung transplant “allocation score.” Information on this prioritization process is outlined on the United Network for Organ Sharing site (UNOS).
In deciding if you want to apply for a lung transplant, speak freely with your family members and your clinical specialists.
Your medical care providers can share with you the process involved, to include benefits and risks of a lung transplant, and outline details of the recovery phase.
If you care to do some research before these conversations, refer to the Johns Hopkins Medicine website and its Comprehensive Transplant Center article about lung transplants.
The waiting period
If you choose to apply for a transplant and your medical care provider agrees, your next step would be to get your name on the OPTN/UNOS national transplant waiting list. Your medical provider(s) will assist you with this process.
If you are accepted as a candidate for surgery, the next phase is the waiting period. The length of this process is totally out of your hands which can add to any impatience you may feel.
Choosing the right donor for you takes time. To prevent any major rejection issues, the donor’s blood type must be compatible with yours. Also, the tissue in your lungs must match the donor’s as closely as possible.
There is no way to know how long your waiting period will be. The length of time is related to your lung allocation score and the opportunity for a lung(s) that tests out as a good match for you.
While you wait for your donor lung to be available, you might check online or at the library for articles/books written by others who’ve had lung transplant surgery.
Above all, the waiting period demands that you maintain your health and stick to your established CF care habits.
Time can also be spent packing your bag to be all ready when your donor lung(s) are ready for you.
Additionally, to improve the chances for a successful outcome, you must commit to living a lung-healthy lifestyle by maintaining a healthy diet, carrying out your established care routine, and getting adequate exercise.
Complications of the surgery
Very serious complications following lung transplant include the threat of rejection and infection.
Even though you and the donor were well matched prior to the transplant, your immune system will still recognize the new organs as being “alien” tissue and will work to attack/reject your new lung(s).
This risk is greatest immediately following surgery and generally lessens over time.
Nevertheless, you will need to take anti-rejection medication for the rest of your life. This medication will curb your immune system, making you more prone to infection- especially in the lungs- and will also make you more susceptible to getting cancer.
To stay healthy, your medical provider may advise you to frequently wash your hands, regularly brush your teeth and gums, and to be careful to avoid scratches or sores on your skin. Additionally, he/she may caution you to avoid crowds and remind you to stay current with your vaccinations.
Others who have received organ transplants have reported the joy of a healthier and prolonged life outweighs these risks.
References
http://www.cysticfibrosisjournal.com/article/S1569-1993(17)30810-X/fulltext
http://www.atsjournals.org/doi/full/10.1164/ajrccm.163.1.9811076
https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Lung-Transplantation/
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4073758/
https://transplantliving.org/before-the-transplant/getting-on-the-list/
http://www.mayoclinic.org/tests-procedures/lung-transplant/details/risks/cmc-20212065
