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Treatment Options for Pulmonary Fibrosis

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. 

TREATMENT OPTIONS

What kinds of treatments are available for pulmonary fibrosis?

Doctors have a number of ways to treat PF, including the use of medications, oxygen therapy, non-medical treatments (such as exercise), and even lung transplantation.

The following information is meant as a general overview of some of the treatments that physicians offer their patients with PF. This information is not medical advice. Some of these treatments may be right for some people, but no one treatment is right for everyone. You should speak with your doctor before starting, changing, or stopping any medical treatment. You can also learn more by contacting the PFF Patient Communication Center (PCC) at 844.TalkPFF (844.825.5733) or pcc@pulmonaryfibrosis.org.

OXYGEN THERAPY

Oxygen is prescribed when oxygen levels go below about 88%. Your doctor may choose to measure your oxygen level in different circumstances: while sitting at rest, while walking in the hallway or on a treadmill, and during sleep. If your oxygen level drops below about 88%, your doctor may prescribe oxygen to prevent your oxygen level from dropping further. Many people report that they have less breathlessness and fatigue, and are better able to live an active lifestyle when using oxygen.

Having to use oxygen can be frustrating, inconvenient, and, for many, embarrassing. We recommend that you talk openly with your doctors about your concerns. Many people find it difficult to adjust to using oxygen, particularly when outside the home. Over time, most people find that the benefits of using oxygen greatly outweigh the downside.

In a very real sense, using oxygen to breathe is no different than wearing eyeglasses to see or using a cane to walk— it’s only the judgment of others that seems to make oxygen different from other medical devices. Contrary to what people may think when they see you wearing oxygen, you are doing something important for your health. Those living with PF can jog, work out, and play sports (with permission from their doctors) while using oxygen. Wearing oxygen is a sign of strength, not weakness.

PULMONARY REHABILITATION

Pulmonary rehabilitation includes exercise training; breathing exercises; anxiety, stress, and depression management; nutritional counseling; education; and more. The goal of pulmonary rehabilitation is to restore your ability to function without extreme breathlessness and allow you to safely exercise. It improves both exercise capacity and health-related quality of life for many people living with PF. These programs offer a variety of services and can be inpatient, outpatient, or home/community-based. While pulmonary rehabilitation programs vary, they may comprise a multidisciplinary team of nurses, respiratory therapists, physical therapists, social workers, dietitians, and others.

SYMPTOM MANAGEMENT

Breathlessness and cough are the major symptoms experienced by people living with PF. Patients may also experience fatigue, anxiety, and depression. Please talk to your doctor about these symptoms— there are treatments that might be of help.

Preventing breathlessness

  • Your doctor may recommend that you use enough oxygen to keep your oxygen level over 90% during exertion, as low oxygen concentrations are a common cause of breathlessness in patients with PF. Breathlessness may also occur in the absence of low oxygen concentrations.
  • Pulmonary rehabilitation can help you get in shape and learn strategies to minimize breathlessness.
  • Doctors sometimes treat severe breathlessness with drugs like morphine when PF is advanced. Palliative care is discussed further below.

Preventing cough

  • There are many causes of cough in addition to PF. Talk to your doctor to see if post-nasal drip or heartburn (gastroesophageal reflux, or GERD) may be contributing to your cough.
  • Low oxygen levels can trigger cough in some people.
  • Talk to your doctor to see if there are other treatments that might help your cough.

Palliative care

Palliative care, also referred to as supportive care, is medical treatment focused on relieving and preventing symptoms that are bothersome or distressing. Palliative care also helps you address advanced care planning, which allows you and your loved one to decide your goals of care as your disease progresses. It is important to understand that you can also receive “curative care” at the same time. Curative care refers to participating in research studies, being evaluated for lung transplantation, and considering new medications. This is distinct from hospice care, which is treatment administered in the last months of life.

Palliative medicine is usually administered in a team approach, involving physicians, pharmacists, nurses, religious leaders, social workers, psychologists, and other health care professionals working with you. Palliative care providers consider physical, psychosocial, and spiritual factors in their treatment approach. Palliative care is appropriate for anyone experiencing symptoms from their disease.

mEDICATIONS FOR PF

DRUG THERAPY
Drug therapy for PF is often specific for the particular type of PF a patient has. In other words, the treatment for one disease may not be the right one for another. It is important to discuss drug therapy with your physician relative to your form of PF. Some common therapies (but not all potential therapies) are listed below.

Nintedanib (Ofev®): Nintedanib is an anti-fibrotic drug that is approved to treat IPF in the United States, Europe, and Australia. In clinical trials, nintedanib has been shown to slow the decline in lung function in mild-to-moderate IPF. It is taken by mouth twice a day. For more information, please visit ofev.com.

Pirfenidone (Esbriet®, Pirfenex®, Pirespa®): Pirfenidone is an antifibrotic and anti-inflammatory drug approved to treat IPF in the US, Europe, Canada, Asia, and Australia. In clinical trials, pirfenidone has been shown to slow progression of mild-to-moderate IPF. It is taken by mouth three times a day. For more information, please visit esbriet.com.

Corticosteroids (prednisone): Prednisone is used to treat and prevent inflammation by weakening the immune system. While prednisone is not usually used to treat IPF, it is sometimes used to treat inflammation in the lungs of people living with other forms of PF. Since prednisone suppresses the immune system, it can potentially increase the frequency and severity of infections. Prednisone has many other side effects as well.

Mycophenolate mofetil/mycophenolic acid (CellCept®): Similar to prednisone, mycophenolate mofetil (MMF) also can treat and prevent inflammation by suppressing the immune system. MMF is sometimes used in combination with prednisone, but is also used alone. MMF is not indicated for the treatment of IPF, but like prednisone can be used for people living with other forms of PF. Some possible side effects include infections, diarrhea, and low blood cell counts. Pregnant women should not take MMF, and women of childbearing age need to use contraception while taking MMF.

Azathioprine (Imuran®): Azathioprine is used to suppress the immune system similarly to MMF. Some possible side effects include infections, low blood cell counts, and liver and pancreatic problems.

Several other anti-inflammatory therapies such as methotrexate, cyclophosphamide, cyclosporine, rapamycin (sirolimus), and tacrolimus have been used to treat different forms of interstitial lung disease. As mentioned previously, treatment with these agents is often tailored to specific diseases or forms of ILD and PF.

lung transplantation

Is lung transplantation an option for pulmonary fibrosis?

Yes. Lung transplantation is the replacement of one or both lungs with a new lung (or lungs) from a donor. In 2015, about 2,000 people underwent lung transplantation in the United States, and about half of those people had PF. Lung transplantation is an important therapy for some PF patients, but with only 1,000 lungs available per year for those with PF, most people living with the disease will never receive a transplant.

Transplant Evaluation

Transplant centers have strict criteria for being a candidate. The evaluation process and waiting time can take months, even years in some cases. Thus, early referral to a lung transplant center is critically important. The first visit is often just a regular medical visit with a doctor and a nurse or nurse practitioner. At that time, the transplant team might decide to order extensive testing, which can include CT scans, heart catheterizations, exercise tests, as well as visits with other members of the transplant team. 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.Talk to your doctor about whether a lung transplant is appropriate for you.

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 placed on the waiting list, about half of people receive their lung transplant within four months (as of 2014). However, there are a number of factors that can influence how long you wait for your transplant.

  • Since the availability of donors varies around the country and each transplant center has different criteria for the types of donors they find acceptable, there can be differences in waiting times between different transplant centers. Be sure to ask your transplant team what the average waiting time is.
  • People who require replacement of both lungs (called a “double” or “bilateral” lung transplant) often wait longer than those who can receive a “single” lung transplant.
  • Children and shorter adults often wait longer for a transplant than those who are taller.
  • When you are placed on the waiting list, you receive a “priority score” called the lung allocation score (LAS). This score varies between 0 and 100, with higher scores indicating higher placement on the waiting list. For most people, the LAS is between 35 and 50. On average, as a person becomes sicker, the LAS gets higher and they move up the waiting list. There are many factors that go into calculating the LAS. See the link below for the “LAS calculator,” where you can estimate your LAS.
  • 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.

    After recovery from transplant surgery, many people feel much better than they did before transplantation. Most people have no breathlessness, no cough, and require no extra oxygen.

    There are also many risks after lung transplantation, including infections, rejection, and medication side effects. While more than one out of four people live more than 10 years after their transplantation, about one out of 10 do not survive the first year. These “average” risks don’t apply to everyone. Talk to your doctors about your risks. Some people have much lower or higher risks.

    Here are some links to transplant websites that may be useful:

    LAS calculator:
    https://optn.transplant.hrsa.gov/resources/allocation-calculators/las-calculator/

    Organ Procurement and Transplantation Network (OPTN/UNOS):
    https://optn.transplant.hrsa.gov/

    Please contact the PFF Patient Communication Center (PCC) at 844.TalkPFF (844.825.5733) or email pcc@pulmonaryfibrosis.org for assistance to navigate these reports.

    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 of 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 than 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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