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Treatment Options


Presently, there are no FDA-approved treatments for idiopathic pulmonary fibrosis (IPF) in the United States. Until approved treatments become available, there are a variety of therapeutic options that may help you live and breathe better.

Every individual diagnosed with pulmonary fibrosis has a unique experience with the disease—there is no “standard” or expected clinical course. Some patients remain in stable condition for extended periods of time; others may experience a rapid progression of symptoms; while another group may experience a stepwise deterioration over time, fluctuating between periods of stability and worsening symptoms. Accordingly, pulmonary fibrosis treatment strategies are highly individualized, based upon your medical history and other important conditions. 

Non-pharmacologic management of pf

Your medical provider may recommend important disease management strategies to help you manage symptoms and prevent future complications.

  • Don’t smoke! – If you are a smoker, please talk to your doctor as soon as possible to obtain help to quit!
  • Home pulse oximetry – While diabetes patients check their blood sugar throughout the day and adjust their insulin dose to optimize their blood sugar level, PF patients should similarly monitor their oxygen levels. Handheld pulse oximeters (available over the counter in many pharmacies) allow for at home monitoring of oxygen levels. Patients should try to maintain their oxygen saturation level above 90% throughout the day and night.
  • Pulmonary rehabilitation – Pulmonary rehabilitation is a structured exercise program for people with chronic lung diseases, including PF, with the goal of restoring a patient’s ability to function without extreme breathlessness. Typically, pulmonary rehabilitation will include conditioning; exercise training and breathing exercises; anxiety, stress, and emotional management; nutritional counseling; education; and other components. This treatment has become an important part of the standard of care for people with chronic lung diseases, and recent studies indicate that pulmonary rehabilitation improves both exercise capacity and one’s quality of life. Rehabilitation programs offer a variety of services and can be inpatient, outpatient, or home/community based. Read more. 
  • Get vaccinated  be proactive to prevent problems.  Talk to your medical professional to determine if you should receive flu or pneumonia vaccinations. Learn more about the flu shot and information on vaccines for adults.
  • Achieve and maintain a healthy weight. – Excess weight can contribute to breathlessness and some transplant centers will decline to offer lung transplantation to those individuals who are significantly overweight. If you are currently overweight, talk to your doctor about healthy ways to reduce your weight. Click here for more information about weight loss. 
  • Lung Transplantation – Pulmonary fibrosis is now the leading indication for lung transplantation in the United States; in 2013, PF accounted for nearly half of all lung transplants performed. Transplantation can improve both longevity and quality of life in patients who have no other significant health problems. Previously, it was uncommon for individuals over the age of 65 to receive transplants. As surgical techniques and outcomes have improved, more centers are performing transplants in individuals over age 65. Transplantation is not without risk; patients should discuss all of the potential risks and benefits of lung transplantation with their medical professional.

pharmacologic management of pf

Several different medications have been used for the treatment of PF. Only your medical provider can determine whether you should take any medication to treat your PF. The following list of medications are sometimes used by doctors to treat PF. Please consult your medical professional to see if any of these medications may be beneficial for you. Currently, in the US, there are no drugs that are FDA approved to treat IPF.

  • Corticosteroids (prednisone): Prednisone is used to suppress the immune system and to decrease inflammation. It mimics the action of cortisol that is produced by the adrenal glands. Prednisone has many side effects which require carefully monitoring.
  • Cyclophosphamide (Cytoxan®): Cytoxan® is a chemotherapy agent that can suppress inflammation and has been used to treat certain forms of PF. It is traditionally taken orally but may also be administered intravenously. Read More.
  • Azathioprine (Imuran®): Imuran® is used to suppress the immune system and is commonly used to treat autoimmune diseases including rheumatoid arthritis. It is also used to help prevent the body from rejecting organs following transplantation. Although a small number of individuals have had success with this drug, the effectiveness of Imuran® for PF treatment has not been confirmed in a randomized clinical trial.
  • Mycophenolate mofetil (Cellcept®, Myfortic®): Mycophenolate modulates the immune system and has been used alone or in combination with corticosteroids for several autoimmune disorders. Read More.
  • N-acetylcysteine (NAC): NAC is a naturally occurring anti-oxidant. It can be taken orally and theoretically could help protect the lung from “oxidative injury” that occurs in some forms of PF. Read More.
  • Pirfenidone (Esbriet®, Pirfenex®, Pirespa®): Pirfenidone is an anti-fibrotic and anti-inflammatory drug approved to treat mild-to-moderate IPF in the EU, Canada, and Asia. In other countries, including the US, Pirfenidone needed to undergo further clinical trials to investigate the efficacy to treat IPF in order to meet regulatory requirements.  These trials have taken place and it is anticipated in the near future that the FDA will review the results. Read More.
  • Proton pump inhibitors (Prilsec OTC®, Nexium®, others) : These drugs help block the formation of acid in the stomach, and are commonly used to prevent and treat gastrointestinal problems, including peptic ulcer disease and gastroesophageal reflux disease (GERD).
  • Supplemental Oxygen Therapy: As fibrosis inhibits the transfer of oxygen into the bloodstream, some individuals may require supplemental oxygen therapy. Supplemental oxygen helps to reduce breathlessness, enabling you to be more active. Some patients may need oxygen therapy 24 hours a day while others may only need to use it during sleep and with exertion. If your medical provider has prescribed supplemental oxygen, it is important to use it as prescribed. Many people are fearful that they will become "addicted" to supplemental oxygen. It is important to know that supplemental oxygen is not addictive. The proper amount of oxygen in the bloodstream is necessary to maintain normal body functions. Low blood oxygen levels can lead to additional health problems.

lung transplantation

WHAT TO EXPECT WHEN CONSIDERING A LUNG TRANSPLANT

“A potential transplant patient will be looked over from the hair on the top of their heads all the way down to their toenails.” —Susan Long, LCSW, Transplant Social Worker at Loyola Hospital, Chicago

Transplant Evaluation

As a potential transplant candidate, you will be evaluated by a transplant team which may include: a pulmonologist, transplant surgeon, transplant nurse coordinator, transplant social worker, dietician, psychologist, physical therapist and insurance coordinator. Your physical health, emotional health, and social support system will be closely evaluated.

Eligibility for Lung Transplant

Generally speaking to be eligible for a lung transplant a person should:
  • Be in good physical condition besides your lung function
  • Be at your ideal body weight
  • Have no other life-threatening illnesses
  • Demonstrate absolute compliance with prescribed medicines and medical recommendations
  • Have a realistic understanding of the emotional implications of a lung transplant
  • Have a very supportive social support system in place
  • Have participated in pulmonary rehabilitation program
  • Expect to participate in educational seminars and a support group
  • Consider the financial implications of the procedure/Insurance, etc

Following the Evaluation

Once you have been evaluated and a decision has been made to proceed with a transplant, you will be given a Lung Allocation Score (LAS) based on a variety of factors, including your age, severity of disease, lung, heart and kidney function, and the laboratory values from your tests. This score determines your place on the UNOS (United Network for Organ Sharing) waiting list.

Placement on the Waiting List

Once you have been placed on the transplant list, waiting can be an incredibly stressful time in your life and the lives of your loved ones. Remember to take care of your health, follow your exercise and dietary guidelines, keep up with your pulmonary rehabilitation program, and keep all of your scheduled appointments with your physicians. Transplant centers may offer support groups to individuals awaiting a transplant. It is important that your transplant team can always reach you during this time because you never know when you will receive a call that they have new lungs for you.

Transplant Surgery

Surgery can last anywhere from four to ten hours, depending on several factors including whether it is a single or double lung transplant, your current medical condition, and the institution where the transplant is being performed.

Following Surgery

After surgery, your transplant team will manage any post-op challenges that may arise. You will begin taking anti-rejection medications and immunosuppressants, and any side effects or adverse reactions will be closely monitored. Remember everyone will adjust to their new lungs and medications differently.

Before you leave the hospital, make sure you and your caregiver review the following with your transplant team: your medication schedule, follow-up appointments, diet & exercise restrictions, how to safely prepare your home for your arrival, and how to manage your pain.

Remember, a lung transplant is major surgery and it will take some time for you to feel like yourself again. Some transplant recipients will take longer to feel better than others, but do not be discouraged - lean on your support system!

A lung transplant may not be an option for all patients and some may choose to opt out of a lung transplant. Whatever the case, it is crucial to have a support system in place and discuss all of your options with a medical professional.

Ambulatory ECMO as a bridge to transplantation

Extracorporeal membrane oxygenation (ECMO) was adapted from a technique that was employed to oxygenate the lungs during cardiac surgery and has been modified for use in individuals with severely impaired oxygenation (low oxygen levels). Frequently, individuals with impaired gas exchange (removal of carbon dioxide and delivery of oxygen) and low oxygen levels can be effectively supported with mechanical ventilation (respirator). With a respirator, a tube is placed in the main airway (trachea) and oxygen is delivered at a desired concentration and volume. In most situations this method will provide adequate blood oxygen levels. However, in some unusual situations, there is severe respiratory failure (immature lungs in a premature infant, severe bacterial or viral infection, trauma, and other situations) and adequate blood oxygenation cannot be achieved with a mechanical ventilator. In these instances, ECMO has been successfully employed. ECMO was used to treat some individuals with severe viral pneumonia during the H1N1 epidemic.

With ECMO, blood is removed from the body, usually through a large vein, and is then pumped through an artificial membrane which delivers a desired oxygen concentration and removes carbon dioxide. The oxygenated blood is then returned to the body, also usually through a large vein but may also be returned through an artery in the groin. This technique was first employed in the mid-1970s in premature infants with severe respiratory failure, and has been greatly refined and improved since then.

The membranes used to artificially oxygenate the blood are more effective and less injurious to the red cells, and pumping devices have become less traumatic. ECMO has been used to support patients both prior to and following lung transplant surgery. Individuals awaiting transplant can occasionally deteriorate quite rapidly and cannot be adequately oxygenated on a respirator.  In these cases, ECMO has been successfully employed for some of these individuals. Following transplant surgery patients can develop acute rejection or primary graft dysfunction (PGD), and in very severe situations ECMO can be used.

One major drawback with standard ECMO techniques is that patients must remain in bed, cannot ambulate, and can become severely deconditioned. Recently techniques have described an ECMO method in which blood is withdrawn and returned through one cannula (a moderate sized tube) placed in a vein in the neck.  With this system, the patient can ambulate, eat, and regain some strength. Although this is a relatively new technique, it has been employed in a number of patients who have successfully gone on to lung transplantation.

ECMO is still evolving, is reserved for only the most dire situations, and is not without risk and potential for serious complications. If one looks at the history of artificial organ support systems (kidney dialysis, respirators, heart assist devices), there is an evolutionary process in which these devices are refined, improved, and simplified. It is hoped that there will be continued improvement and development of this life saving technique. 

References:

  1. Lewandowski K. Extracorporeal membrane oxygenation for severe acute respiratory failure. Crit Care. 2000; 4: 156-68.
  2. Bermudez CA, Adusumilli KR, McCurry KR, et. al. Extracorporeal mem-brane oxygenation for primary graft dysfunction after lung transplantation: long-term survival. Ann Thorac Surg. 2009; 87: 854-860
  3. Garcia JP, Iacono A, Kon ZN, and Griffith B. Ambulatory extracorporeal membrane oxygenaion: A new approach for bridge-to-lung transplanta-tion. J Thorac Cardiovasc Surg. In press

Ex Vivo Lung Perfusion - Expanding the donor pool ofr lung transplantation

Lung transplantation has experienced increasing success, but unfortunately, only a small percentage of patients can benefit from this therapy as a result of the lack of acceptable donor lungs. Lung transplantation could be offered to a larger group of patients if the donor pool could be increased by improving the lung function of the donor lungs that have been deemed unsuitable by existing criteria.

The standard selection criteria for donor lungs from the International Society for Heart and Lung Transplantation (ISHLT) include:

  • Age less the 55
  • Clear chest X-ray
  • Normal oxygen gas exchange
  • Absence of chest trauma
  • No evidence of aspiration or infection
  • Absence of purulent secretions
  • Tobacco history of less than 20 years
  • ABO blood compatibility
  • Appropriate size match with the prospective recipient

This evaluation process can be imprecise and unrecognized injury may lead to early graft dysfunction. It has also been estimated that as many as 30 – 40 % of rejected donor lungs could have been safely implanted if appropriate preservation and resuscitation techniques were utilized.

Unfortunately, as a result of the trauma and nature of the injuries that lead to brain death, there are a number of perturbations that occur that can harm the lungs and impair their function. These can include:

  • Low blood pressure
  • Leaky lung alveoli resulting in pulmonary edema
  • Elevated blood sugar
  • Low blood pH
  • Abnormal kidney function

Additionally the potential donor may have been on a ventilator for several days, and, as result, may have developed an increase in pulmonary secretions. Before explanting the lungs, attempts are made to improve these metabolic and hemodynamic abnormalities.

In order to hopefully improve and adequately assess the lung function of the marginal lungs after they are explanted, they are connected to an ex vivo lung perfusion (EVLP) system. Ex vivo implies that the lungs are perfused outside of the body.  This is a complicated process but as simply as possible, the explanted lungs are perfused with a special, dilute blood containing solution that can potentially  recondition and improve their function. The apparatus also includes a ventilator to inflate the lungs. This system is quite advantageous in that it: (1) permits the re-expansion of collapsed lung regions; (2) can remove secretions; (3) remove clots from the lung circulation; (4) improve lung ventilation; and (5) permits a better and more complete assessment of lung function.

Using EVLP, institutions in Sweden, Canada, and the United States have transplanted lungs that were initially thought to be unsuitable for transplantation. Results in the studies have revealed no significant difference in outcomes when compared to using standard techniques with suitable lungs. Also these techniques have been employed in individuals who have had cardiac death (i.e. individuals who may not have met the criteria for brain death, but their families have decided to withdraw life support). It has also been postulated that with EVLP, gene therapy could be delivered via the trachea and repair damaged tissue, reduce inflammation, and inhibit rejection.

In summary, EVLP can significantly expand the potential donor pool, improve the function of lungs that might be deemed unsuitable and ultimately decrease inflammation and rejection.

References:

  1. de Perrot M, Keshavjee S. Lung Preservation. Surg Clin N Am 2003; (13): 429-442.
  2. Yeung JC, Cypel M, Waddell TK, van Raemdonck D, Keshavjee S. Tho-rac Surg Clin 2009; (19): 261-274.
  3. Ingemanssom R, Eyjolfssson A, Mared L, et. al. Ann Thorac Surg 2009; (87): 255-260.
  4. Oliveria NC, Osaki S, Maloney JD, et. al. J Thorac Cardiovasc Surg 2010; (139)5: 1306-1315

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