Lung Transplant Program

Pulmonary fibrosis is a progressive disease that causes damage to the interstitial tissues, or the cells that live between the blood vessels and other structures inside the lungs. This damage causes lung tissue to scar and stiffen, which prevents it from expanding and functioning normally.

Often, the cause of pulmonary fibrosis is unknown (idiopathic), but smoking, environmental exposures, and genetics can play a role. There are medications that are designed to slow the progression of the disease. However, lung transplant is the only way to cure pulmonary fibrosis completely.

Emphysema is a chronic condition that is part of a group of lung diseases called chronic obstructive pulmonary disease (COPD). Emphysema causes damage to the walls of the air sacs (alveoli), which are critical to facilitating healthy airflow. They are located at the end of the breathing tubes (bronchial tubes) in the lungs. When they are damaged, the bronchial tubes collapse from the lack of support and air becomes trapped in the lungs.

The top risk factor for developing emphysema is smoking. There are medications that are designed to slow the progression of the disease. However, lung transplant is the only known way to cure it completely.

Chronic bronchitis is another COPD condition. Chronic bronchitis is characterized by long-term inflammation of the breathing tubes (bronchial tubes), often associated with recurrent infections.

Smoking is the most common cause of chronic bronchitis. Environmental factors, such as air pollution, may also play a role in the development of chronic bronchitis. Lung transplant is recommended for severe cases of chronic bronchitis.

Cystic fibrosis is one of the most common chronic lung diseases in young adults and children, affecting various organ systems such as the respiratory, digestive and reproductive tracts. It is an inherited (genetic) disease that causes mucus to build up in the breathing passages and restrict airflow.

Lung damage caused by cystic fibrosis can be permanent, but newer medications have shown tremendous benefit in improving lung function, reducing respiratory symptoms and improving quality of life. In cases where cystic fibrosis does not respond to medication or when the lung disease becomes too severe, lung transplantation may be recommended.

Pulmonary hypertension is high blood pressure in the pulmonary arteries in the lungs, which disrupts blood flow from the heart to the lungs. As the pulmonary arteries become narrow, the heart is required to work harder to pump oxygen-poor blood to the lungs.

Pulmonary hypertension is a rare condition that is most often the result of a complication from heart or lung disease, but can also be caused by environmental factors or chronic blood clots in the lungs. If left untreated, it can lead to serious complications, including heart failure. Lung transplantation can cure pulmonary hypertension and allow the heart to recover.

The diseases listed above are the ones most commonly treated by lung transplantation, but there are many other conditions for which lung transplantation may be recommended, including:

• Alpha-1 antitrypsin deficiency
• Bronchiectasis
• Bronchiolitis obliterans syndrome
• Eisenmenger syndrome
• Lymphangioleiomyomatosis (LAM)
• Occupational lung disease
• Pulmonary Langerhans cell histiocytosis
• Sarcoidosis

All lung transplant candidates are carefully evaluated to determine if lung transplant is the appropriate treatment, or if their condition can be managed through alternative interventions. Your transplant coordinator will guide you through your initial evaluation by gathering your medical information, previous test results, and medical history, including prior hospitalizations.

As part of your evaluation, your care team will conduct:

• A surgical evaluation, which allows your team to become familiar with your medical history and assess the risks and benefits of lung transplant surgery relative to your overall health
• Routine blood work, which will be used to find a good donor match for you, evaluate the function of other important organs, predict risk of infections, and help guide treatment following transplant
• Diagnostic tests, such as
  • Pulmonary function testing and six-minute walk tests to evaluate your lungs and physical fitness
  • Imaging, including X-rays and CT scans
  • Echocardiogram to evaluate heart function
  • Heart catheterization to look for blockages in the heart’s blood vessels and to determine if you have pulmonary hypertension
  • Gastric emptying, esophageal manometry, and pH monitoring to evaluate your digestive tract
  • Health maintenance, including tests such as colonoscopy, bone density scan, mammogram, and pap smear
  • Other tests, which may include PET scans, lung biopsy, and liver imaging/biopsy
• A psychological and social evaluation, which will assess your financial health, support/caregiver network, and mental health, all of which can affect your surgical outcomes
• A review and update of necessary vaccinations, as transplant patients are at increased risk of infection and lower response to vaccination after transplant due to medications that suppress your immune system

Determining Lung Transplant Surgery Eligibility

Your transplant team will consider all this information when determining your eligibility for a lung transplant. Eligibility requirements take into account age, physical fitness, severity of your lung disease, other medical conditions, and more.

In order to qualify for lung transplant surgery, patients must:

• Have a BMI of 35 or less
• Refrain from tobacco, alcohol and non-prescription drugs
• Be free of untreatable infections
• Have low risk of severe failure of other major organ systems

Lung transplant surgery is not suggested for all patients. If your care team determines that lung transplantation is not the appropriate treatment for you, alternative methods of care may be recommended, such as:

• Clinical investigations related to prescribed medications including corticosteroids, antifibrotic medications, and antibiotics
• Non-transplant surgical therapies, such as lung volume reduction surgery, which involves removing portions of the damaged lung
• Pulmonary rehabilitation
• Palliative care

Lung Transplant Waiting List

Once your candidacy for a lung transplant has been approved, you will be placed on the United Network for Organ Sharing (UNOS) waiting list. Unlike other organ transplant programs that apply a seniority method to determine a patient’s position on the organ donor list, lung transplant programs utilize a unique system based on patients’ transplant urgency called the Lung Allocation Score (LAS) system, which ensures that patients who most need a lung transplant receive priority over those in better health. In addition to this score, the wait time is affected by factors such as blood type, immunologic match, and the patient’s height.

While you are on the waiting list, your Mass General care team will closely monitor your health and help you create a health plan to improve your quality of life as you wait for a donor organ. This plan may include:

• Pre-transplant education sessions hosted by Mass General experts
• Lifestyle changes, such as abstaining from tobacco, alcohol, and fatty foods
• New medications
• Exercise and pulmonary rehabilitation regimens to ensure you’re in the best shape come time for your lung transplant
• Participation in our Lung Transplant Support Group, a monthly meeting of pre- and post-transplant patients and caregivers facilitated by our social work team

Once on the list, our team will begin looking for a good donor match for you.

When a deceased donor lung is available, you will receive a call from our transplant coordinator asking you to come into the hospital for the lung transplant. Since this call can come at any time during the day or night, any day of the week, it is important for the transplant team to be able to reach you promptly. You should provide our center with all of your phone numbers, as well as the phone numbers of a few emergency contacts, so that we can contact you when needed. The lung transplant surgery generally happens within 24 hours of the notification to come to the hospital.

A lung transplant is a complex operation that replaces one or both of your lungs with healthy lungs from a deceased organ donor. The operation is performed by an expert team of transplant surgeons, anesthesiologists, perfusionists and nurses who work together to replace your lungs. A lung transplant can be performed using several different approaches or types of incision, including:

• Thoracotomy, an incision between the ribs
• Transverse sternotomy, a horizontal incision across the center of the chest to divide the breastbone
• Sternotomy, a vertical incision through the center of the breastbone

The approach is determined by your specific condition, and will be discussed with you during your surgical evaluation. A single lung transplant usually takes four to six hours, while a double lung transplant can take eight to 12 hours.

During your transplant, the surgical team may use a special pump to either partially (VA-ECMO) or fully replace the function of your heart and lungs. This allows the team to operate on your heart and lungs while the rest of your body is fully supported and protected. Currently, most transplants are being performed with the help of VA-ECMO, but the final determination is made in the operating room by your surgeon. In rare cases, ECMO may be used following your transplant surgery so that your new lungs have time to recover.

After your surgery, you will recove in our cardiac surgery and thoracic transplant intensive care unit where a sub-specialized team of intensive care doctors, advance practice practitioners, nurses, and respiratory therapists will work alongside your transplant team to help you recover from surgery. Once you no longer require intensive care, you will finish your hospital stay in our dedicated unit for post-transplant patients.

Our commitment to our patients’ wellbeing extends to their after-surgery needs. Our team will help coordinate your care following discharge and even once you’ve returned home. We are very involved in our patients’ physical recovery, and we support them as they transition back to their regular lives following transplant.

Lung transplant requires long-term adjustments to ensure lifelong optimal health. Generally, you can expect to receive from your care team:

• Immunosuppressant medications to minimize risk of organ rejection
• Prescriptions for other medications to prevent infection and to treat medical conditions related to your transplant
• Recommendations for a healthy diet and exercise/rehabilitation plan
• Reminders about health maintenance (vaccinations, testing, and clinical examinations)

Preventing Lung Transplant Rejection and Infection

Rejection is the natural process of your immune system identifying your new lung(s) as an intruder and attempting to remove it as a way to protect the body. There are different types of rejection, each requiring unique testing and treatment. Every patient is different, and you will continue to work with your Mass General care team to monitor the state of your new lung(s) after transplant. Immunosuppressants, medications that suppress your automatic immune response, are a necessary step in transplant after-care to prevent rejection.

Due to the vulnerability of your immune system after surgery, you are more susceptible to infections. This is particularly true within the first year following lung transplant. Antibiotics are effective at treating most lung transplant-related infections. Your care team may also prescribe other medications to help prevent specific types of infection.

Brian Keller, MD, PhD
Medical Director, Lung Transplant

Nathaniel Langer, MD
Surgical Director, Lung Transplant

Katy Black, MD
Pulmonologist

Jason Griffith, MD, PhD
Pulmonologist

Michael Feldman, MD, PhD
Pulmonologist

Eriberto Michel, MD
Cardiac Surgeon

James Mojica, MD
Pulmonologist

Isabel Neuringer, MD
Pulmonologist

Asishana Osho, MD, MPH
Cardiac Surgeon

Judith Restrepo, MD
Psychiatrist

Jacqueline Clark, PharmD, BCPS, BCTXP

Sarah Mietz, PharmD, BXTXP

Georgina Waldman, PharmD, BCTXP

Tamara Chase, CNP

Orlaith Delgado, CNP

Robert Ober, FNP

Aliza Kosyakovsky, CNP

Joe Digrazia, RN

Ericka Gruss, RN

Nicole Prince, RN

Tonya Yurjevic, RN

Peggy Aliberti

Monique King

Yoshida Luu-Tran

Jaimee Singh

Natali Rauseo-Ricupero, LCSW

Gretchen Garlow, MS, RD, LDN

Erin Vuijk, MS, CCC-SLP

Tatiana Mendieta

To book an appointment for an evaluation for lung transplant at Mass General or to obtain more information, please call 800-876-5864 or email MGHLungTransplant@partners.org. Your primary doctor or pulmonary rehabilitation program may also call/email on your behalf to refer you to the Mass General Lung Transplant Program.

Lung transplant may be recommended for those with severe lung conditions that have not responded to other treatments, such as pulmonary fibrosis, emphysema, chronic bronchitis, cystic fibrosis, and pulmonary hypertension, among others.

Lung transplant is not an appropriate option for every patient, and your care team will help you consider all of your options. Eligibility requirements take into account age, physical fitness, severity of your lung disease, other medical conditions, and more.

In order to qualify for lung transplant surgery, patients must:

• Have a BMI of 35 or less
• Refrain from tobacco, alcohol and non-prescription drugs
• Be free of untreatable infections
• Have no risk of severe failure of other major organ systems

Your lung transplant team will conduct a comprehensive evaluation to determine whether lung transplant surgery is the best choice for you relative to your health.

Your care team will closely monitor you after surgery to assess the risk of organ rejection. If you are experiencing alarming symptoms after transplant surgery, please contact your care team immediately as these symptoms could be a sign of organ rejection. Immediate diagnosis and treatment enable quicker, more effective prevention of organ rejection.

Your care team will run tests to diagnose lung transplant rejection, and then devise a plan to help your body accept the new lung. This usually involves adjusting your immunosuppressive medications and supporting your lung function while the episode is treated. Clinical trials may also be available for certain types of rejection or when standard treatments are not successful.

If you are on the wait list, your Mass General lung transplant team will work with you to create a protocol that will aim to improve your quality of life and ensure your optimal health for when a donor lung becomes available. This plan may include:

• Lifestyle changes, such as a healthy diet, regular exercise, smoking/drinking cessation, and weight loss plan (if applicable)
• Medications
• Regular appointments and testing to monitor your health status

As a donor lung can become available at any time, we recommend that you prepare a transportation plan in advance and a bag of essential items for your hospital stay.

Your care team will discuss the qualifications in your lung transplant evaluation. Generally, patients who qualify for lung transplant:

• Are of a certain age and health status
• Have a life-threatening lung disease that prevents normal airflow and cannot be treated by medication or therapy
• Do not have severe underlying conditions, such as cancer, uncontrolled diabetes or infection, or failure of other organs like the heart, kidneys or liver
• Do not smoke
• Are able to take immunosuppressive drugs following surgery

During your evaluation, you will meet with a Mass General Transplant Center financial counselor who will review your insurance and help you obtain insurance authorization for your transplant and follow-up care. They will be able to answer all of your questions related to transplant and finances.

This depends on your specific situation. The average hospital stay following a lung transplant is about three weeks but can be influenced by a variety of factors. Some patients may need to go to an inpatient rehabilitation hospital for additional strength and conditioning before going home after their lung transplant. An outpatient pulmonary rehabilitation program is required for every patient and helps accelerate recovery after transplant, but full recovery from a lung transplant can take six to nine months or more.