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These educational tools were designed to provide useful information to patients newly diagnosed with pulmonary fibrosis-with the goal of helping them and their caregivers better understand and manage their condition. The information supplied here is a guide to answer many of the most commonly asked questions regarding the disease, and its symptoms, diagnosis, and treatment.
The information is not intended to replace the advice or services of trained health professionals. You should seek the advice of your physician or healthcare professional in all matters relating to your health, particularly with respect to the diagnosis and treatment of any medical condition.
Click here to view or download the complete print version of these materials (.pdf) LET'S TALK ABOUT PULMONARY FIBROSIS (PF):
Comprehensive Information for Patients and Caregivers
PULMONARY REHABILITATION AND OXYGEN MANAGEMENT FOR THE PF PATIENT:
Helping You Make the Most of Every Day
LUNG TRANSPLANTATION: What Every Patient with PF Should Know
LET'S TALK ABOUT PULMONARY FIBROSIS (PF): WHAT IS PULMONARY FIBROSIS? Pulmonary Fibrosis (PF) is a debilitating disease—marked by progressive scarring of the lungs—that gradually interferes with a person's ability to breathe. PF belongs to a family of approximately 100 related diseases, called interstitial lung diseases, which have similar characteristics and can result in lung scarring. This scarring is most often referred to as pulmonary fibrosis.Your doctor may have referred to your condition by one of a number of names that are sometimes used to describe PF. This confusion may be due, in part, to similarities between PF and the other forms of interstitial lung disease. Only since 2001 has PF been recognized as a distinct clinical disorder, meaning that specific clinical criteria were developed to determine a diagnosis. Your physician combines clinical information derived from a medical evaluation and certain diagnostic tests to diagnose PF according to these criteria. Sometimes pulmonary fibrosis can be linked to a particular cause, such as certain environmental exposures, chemotherapy or radiation therapy, residual infection, or autoimmune diseases such as scleroderma or rheumatoid arthritis. However, in many instances, no known cause can be established. When this is the case, it is called idiopathic pulmonary fibrosis or IPF. Whether you are an PF patient, a family member of a patient, or are close to someone with PF, the CPF is always just a phone call away. Our staff have counseled thousands of patients, and we are always here to provide you with the resources and support you need. Please call (888) 222-8541 to learn more. WHAT CAUSES PF? While the cause of PF remains a mystery, what researchers suspect is that PF involves changes in the lung's normal healing process. PF patients may have an exaggerated or uncontrolled healing response that, over time, produces excessive fibrous scar tissue - or fibrosis - in the lungs. This scarring, in turn, causes the lung's tiny air sacs, called alveoli, to thicken and stiffen-rendering them less able to function and provide the body with the oxygen it needs.Exactly what sets this abnormal tissue-repair process in motion is unclear. The body's own immune response may play a major role. Researchers are investigating a number of potential risk factors that may make a person more likely to develop PF. These risks may include:
WHAT SYMPTOMS MIGHT YOU EXPERIENCE? Symptoms of PF usually have a gradual onset and may include:
As you can imagine, PF symptoms can greatly impact quality of life. Fortunately, there are things you and your doctor can do to help minimize lifestyle-altering affects of the disease
HOW IS PULMONARY FIBROSIS DIAGNOSED? Because patients with PF experience symptoms and also have an underlying pattern of scarring similar to those of other lung disorders, PF can be difficult to diagnose. In fact, it was not until 2000 that PF was classified as a distinct clinical disorder by a panel of experts sponsored by the American Thoracic Society (ATS) and European Respiratory Society (ERS). Until recently, the medical community has had no agreed-upon standards for the diagnosis of PF. Consequently, other related lung diseases were often incorrectly classified as PF. With new diagnostic standards now in place, the recognition and management of PF should be substantially improved. To diagnose PF, your doctor will take a complete medical history and perform a thorough physical examination. During this exam, the doctor will use a stethoscope to listen to your chest to try to determine if your lungs produce any abnormal sounds when you breathe. He or she may then order one or more of the following diagnostic tests or procedures: PF can be difficult to diagnose. An estimated 50% of cases are initially misdiagnosed as another form of respiratory disease.
WHAT MAY HAPPEN AS PF PROGRESSES? PF affects each person differently and progresses at varying rates. Generally, the patient's respiratory symptoms become worse over time. Activities (such as walking or climbing stairs) become more difficult.In addition:
HOW IS PF TREATED? Physicians generally follow standards of care intended to improve symptoms and hopefully slow the progression of the disease. In 2001, a panel of experts sponsored by the American Thoracic Society and the European Respiratory Society recommended that if therapy was given, it should consist of a trial of corticosteroids and an immunosuppressive agent (e.g. azathioprine or cyclophosphamide). Recent evidence has suggested that adding a third therapy called N-acetylcysteine (NAC) to this regimen may be beneficial. It is important to recognize, however, that there are no definitive studies showing that this treatment approach is effective, and there is no consensus regarding the use of this approach in the pulmonary community. For a complete listing of active clinical trials for PF, please visit www.coalitionforpf.org. Importantly, these therapies can cause side effects-some minor and some more serious. The potential risks and benefits of therapy should be discussed with your physician in detail. Be sure to contact your doctor or nurse if you have any negative reactions to any medications you have been prescribed. In all cases, doctors should consider referring their patients to a clinical trial to gain access to experimental treatments and also refer patients for lung transplantation evaluation. There remains no FDA-approved treatment for PF, and as of yet, there is no cure. Please see the Lung Transplantation section of this educational tool for more information, and consult your physician to determine if lung transplantation is an option for you. Back to Table of ContentsARE THERE ANY NEW TREATMENTS ON THE HORIZON? Researchers are developing and testing a variety of new ways to treat PF. These approaches target the various steps in the disease process. Therapies under investigation include:
As with any course of care, please consult your physician to determine what current treatment options, including participation in clinical trials, may be appropriate for you. Back to Table of ContentsA WORD TO CAREGIVERS Caring for someone who has a debilitating illness can be both physically and emotionally demanding. You are doing your best to ensure that your loved one gets the best possible care-staying informed about potential treatment options and doing all you can at home to make sure his or her day-to-day needs are met. In the process, you need to remember to take care of yourself as well.You and your loved one may be coping with a great deal of stress and anxiety as a result of your current situation. If you ignore these feelings, they can have a negative impact on your health and well-being. To help reduce the stress in your life, try to get regular exercise and learn some relaxation techniques. You may also want to join a support group or to see a counselor to help you cope with your feelings. The important thing is for both of you to ask for and get the help that you need. There are support groups specifically designed for caregivers-only. PROVIDING SUPPORT TO PATIENTS AND THEIR FAMILIES Joining a local support group can help patient and their families better cope with their disease, while sharing information and support in an open, caring environment. The CPF has helped to create more than 50 PF support groups across the country. Visit the CPF web site for a listing of support groups at www.coalitionforpf.org, or call (888) 222-8541 to learn more.WHAT CAN YOU DO? If you have been diagnosed with PF, there are a number of things you can do to take part in your own treatment and help yourself stay healthy. Caregivers may also be interested in the following information, to assist a family member diagnosed with PF:
Dozens of patients have helped create PF support groups in their communities. It's a great way to get the emotional support you need. If there is not yet an PF support group in your area, contact the CPF for your free copy of the "PF Support Group Coordinator's Kit" and we'll guide you through the process of starting an effective and lasting group. Back to Table of ContentsARE YOU A PATIENT WITH A FAMILIAL FORM OF PULMONARY FIBROSIS? The Coalition for Pulmonary Fibrosis (CPF) and National Jewish Medical and Research Center partnered to launch the first genetic counseling program for patients and families affected by FPF. The telephonic counseling program is operated by National Jewish, and funded by CPF. An estimated 10-15 percent of PF patients have a form of the disease that runs in families, known as familial pulmonary fibrosis (FPF).The genetic counseling program provides a qualified genetic counselor, who has expertise in FPF, to discuss by phone various issues surrounding FPF. These can include preparation for and interpretation of genetic tests, and various life decisions, such as having children and planning for the future. Experts recommend talking to a counselor prior to having any genetic tests, so that people are prepared to learn the results. For further information on the FPF Genetic Counseling Program or to speak with a genetic counselor, call (800) 423-8891, ext. 1097. Back to Table of Contents
PULMONARY REHABILITATION AND OXYGEN MANAGEMENT FOR THE PF PATIENT: OVERVIEW For the pulmonary fibrosis (PF) patient, managing symptoms of respiratory distress can make even the simplest activities of daily living a challenge. Not only does PF gradually rob you of your ability to breathe properly, but low oxygen levels in your bloodstream can leave you feeling weak, tired and uncomfortable, and compromise your quality of life. You may find yourself curtailing activities that previously brought you pleasure, from walking around the block or visiting the supermarket to attending family outings or enjoying concerts, plays and movies.Yet if you are a PF patient, there is no need to put your life on hold. Pulmonary rehabilitation and oxygen therapy are two treatments designed to help people with chronic lung disease become more active and vibrant while better understanding how your body uses oxygen. Through pulmonary rehabilitation, you can build your strength and endurance - resulting in a richer, more enjoyable life experience. If your oxygen levels are low, supplemental oxygen therapy can supply your body with the oxygen it needs and possibly avoid the risk of future cardiac (heart) stress. Pulmonary rehabilitation and oxygen therapy can help you live life to its fullest. Make it a point to discuss these options with your physician. Why observe life from the sidelines when you can again
be an active participant?
WHAT'S GOING ON IN YOUR LUNGS? PF is a debilitating condition that involves scarring of the lungs. The lungs' air sacs develop scar - or fibrotic - tissue, gradually interfering with the body's ability to transfer the oxygen you breathe into the bloodstream, and preventing your vital organs and tissue from obtaining enough oxygen to function normally.What happens when your lungs lose the ability to transfer oxygen into the bloodstream? The following symptoms can result:
PULMONARY REHABILITATION: BRINGING BACK LIFE'S PLEASURES Pulmonary rehabilitation programs are designed to help you be as active and functional in your daily life as possible while living with PF. Most programs include medical management, exercise training to improve endurance and strength, breathing retraining, education, emotional support and nutritional counseling. Physicians, physical therapists, exercise physiologists, nurses, respiratory therapists, psychosocial professionals and nutritionists work together to help you reach your functional goals.An important component of pulmonary rehabilitation is exercise, which helps reduce the effects of inactivity, increases your exercise capacity, and allows you to maintain activities of daily living. Emotions associated with PF - including depression and anxiety - may interfere with your daily activities. Psychosocial counseling and emotional support are also key components of pulmonary rehabilitation and can help alleviate these feelings. Pulmonary rehabilitation also addresses nutritional issues, as severe lung disease may cause either weight loss or weight gain. Nutritionists can teach you how to eat properly to maximize your health. Finally, education will help empower you - teaching you how to get the most out of life while living with PF. Pulmonary rehabilitation programs are designed to help prolong life, reduce disability and raise your level of physical and social functioning to the highest possible level. Since each individual with PF presents unique challenges, pulmonary rehabilitation programs are individually tailored to your needs. You will receive individual assessment and instruction, as well as an opportunity to take part in group activities. ARE YOU A CANDIDATE FOR PULMONARY REHABILITATION? If you answer yes to any of the following questions, you may be a candidate for pulmonary rehabilitation:
SUPPLEMENTAL OXYGEN THERAPY: ANOTHER VITAL TOOL When PF progresses to a point where your blood-oxygen levels are low, another vital tool that can help people with PF lead fuller, more active lives is supplemental oxygen therapy. Oxygen is prescribed by your physician, and can be provided by a local medical equipment company for home use, allowing you to be more active in your life.Oxygen is a gas that you can't see, taste or smell, yet it is in the air all around us and is necessary in order to survive. Why is oxygen so important? Oxygen is vital in the production of chemical energy in the cells of your body that all organs and tissues need to function optimally. Reduced levels of oxygen in your body can injure cells and may cause cell death. Indeed, this lack of oxygenation has been shown to decrease life span by making your heart work too hard. The amount of oxygen in the air is always the same - 21%. This is enough for people with normally functioning heart and lungs to thrive. However, a person with PF - whose disease prevents an adequate transfer of oxygen into the bloodstream - will benefit from breathing air with a higher concentration of oxygen because the scarring that occurs in PF patients can slow the movement of oxygen into the bloodstream, requiring the lungs to work harder. Supplemental oxygen helps increase the amount of oxygen that is available to be transferred from your lungs into the bloodstream, thereby producing more energy to be used by the cells of your body. This is why you and your doctor should discuss supplemental oxygen as part of your overall treatment plan. Supplemental oxygen can:
WHO NEEDS SUPPLEMENTAL OXYGEN? If you are unable to maintain the necessary amount of oxygen by just breathing room air, you may require supplemental oxygen. To determine if you need oxygen, your doctor will order the following tests:
Arterial Blood Gas
Pulse Oximetry You will require supplemental oxygen therapy if your results breathing room air oxygen at rest, with exertion or with sleep are:
Supplemental oxygen helps increase the amount of oxygen that is available to be transferred from your lungs into the bloodstream, thereby producing more energy to be used by the cells of your body. Back to Table of ContentsSUPPLEMENTAL OXYGEN: PORTABLE AND CONVENIENT There are many types of oxygen supply systems, and several of the newer types are smaller, lighter, and more efficient - allowing you to benefit from oxygen therapy both at home and while traveling. PF patients may only need supplemental oxygen support during activity or sleep, but patients most often benefit from a continuous oxygen delivery system that provides consistent oxygen saturation levels when in use.Your pulmonary doctor will help you determine the appropriate amount of supplemental oxygen necessary to support your needs. Of course, oxygen use also requires heightened awareness of safety issues. For example, oxygen itself is not flammable, but it will support fire. That means that if anything nearby the oxygen source should ignite, it will flame quickly. Consequently, no one should smoke near you while you are on oxygen therapy. In addition, you should keep oxygen 10 feet away from sources of ignition such as gas stoves, candles, fireplaces or space heaters. Traveling by air with oxygen presents unique challenges for pulmonary fibrosis patients. Please contact your airline prior to making travel reservations to ensure that your oxygen needs can be coordinated. Individual airlines may have different rules and regulations regarding traveling with portable oxygen. Breathin' Easy (www.breathineasy.com, 925-891-5017) also provides guides, referrals, tips and advice on traveling with oxygen. TALK TO YOUR DOCTOR! Both pulmonary rehabilitation and oxygen therapy require a prescription from your doctor. If you are an individual with PF, make it a point to discuss these options with your physician upon your next office visit. In most instances, both "prescriptions" are covered by insurance, and both can help enhance the quality of your life.There is absolutely no need to suffer with shortness of breath or dramatically decreased activity when such valuable and effective therapeutic options are available. Be proactive and find out more from your physician. And make the commitment to enjoy every single precious day. ADDITIONAL RESOURCES FOR PULMONARY REHABILITATION AND OXYGEN MANAGEMENT American Association of Cardiovascular and Pulmonary Rehabilitation (AACVPR)www.aacvpr.org 401 North Michigan Avenue, Suite 2200 Chicago, IL 60611 (312) 321-5146 aacvpr@sba.com
American Association of Respiratory Care (AARC)
Breathin' Easy
National Home Oxygen Patients Association
LUNG TRANSPLANTATION: WHEN YOUR LUNGS FAIL For certain individuals (usually 65 years old or younger, although program criteria may differ between transplant centers), lung transplantation may also be an appropriate therapy. Lung transplantation can both extend life and enhance the quality of life.PF patients should discuss the possibility of lung transplantation as a treatment option with your physician as soon as possible during the course of their care. The need for transplant evaluation early in an PF patient's disease course stems from the potentially long waiting time on the transplant list combined with the progressive nature of PF. According to United Network for Organ Sharing (UNOS), approximately 30% of PF patients currently listed for transplantation will succumb to their disease prior to transplantation.
"The course of PF is so unpredictable that you never know when you're going to take a turn and need a transplant." WHAT IS A LUNG TRANSPLANT? Lung transplantation is the placement of one (unilateral) or both lungs (bilateral) from a deceased donor into a recipient with end-stage lung disease. The donor lung must be matched for blood type and size to that of the recipient. The decision to transplant one or both lungs is dependent upon the extent of disease and the results of pre-transplant testing.In a single lung transplant, an incision is made on the side of the chest. The patient's old lung is removed and the new lung is then sewn in, connecting the blood vessels to the lung and from the lung to the heart and the main airway. At the end of the operation, the ribs are brought back together and the incision is closed with layers of stitches. Chest tubes are inserted to drain air, fluid and blood out of the chest for several days to allow the lungs to remain fully re-expanded. In a double lung transplant, an incision is usually made side-to-side across the middle of the chest just under the nipple line and both chest cavities are entered between the ribs. The lung on one side is removed and the new lung sewn in place. The opposite lung is then removed and the second new lung sewn in place. At the end of the operation, the ribs are brought back together and the incision is closed in layers with stitches. Controversy exists regarding single versus bilateral lung transplantation for certain lung diseases. Patient outcomes for single or bilateral transplantation should be discussed on an individual basis with the transplant center, in the context of their specific experience performing the procedure. BENEFITS AND RISKS OF LUNG TRANSPLANTATION It is impossible to predict how long a patient may survive after transplantation. Current survival rates are as high as 80% at one year following transplantation and 60% at four years. Further, survival statistics may vary between individual transplant centers. Individual institution's survival statistics are available for review at UNOS's web page (www.unos.org). The most critical period for survival of both the patient and the donor lung(s) is the first year after transplantation - when surgical complications, acute rejection (when a patients' immune system recognizes the lung as 'foreign' and attacks it) and infection are the greatest threats to survival. Rejection and infection are the two potential major complications of lung transplantation. Adherence to treatment plan and maintaining a close alliance with the transplant team is essential to minimize these potential complications.INFECTION Because you will be taking immunosuppressive medications, your immune system will be less able to fight off invading bacteria, fungi and viruses. You will be much more susceptible to infection, and infections are more likely to become severe.You have an important role in the prevention of infection by following instructions to avoid exposure and immediately reporting any symptoms of infection to your physician. Quality of life can be moderately to substantially enhanced by lung transplantation, and life may be extended beyond one's life expectancy prior to transplantation. REJECTION Because your transplanted lung(s) is 'foreign' to your body, your bodies' immune system will try to destroy it, just as it tries to destroy 'foreign' bacteria and viruses when they invade. This process of foreign-tissue rejection helps protect you from illness, but the process has to be "turned off" to keep your transplanted lung(s) from being destroyed.Immunosuppressive (anti-rejection) medications prescribed by your doctors will help keep the rejection process "turned off." Other medications or an increase in the dosage of your current medications may be necessary to control and treat rejection if your immune system breaks through the immunosuppressive blockade. Following your doctors' orders and taking all medications as prescribed help to prevent or control rejection. However, sometimes rejection can begin despite your adherence to your medications. The most likely time for rejection to begin is during the first three months after transplantation surgery. Symptoms of rejection are non-specific and might include shortness of breath, changes on your breathing tests or chest X-ray, or reduced oxygen saturation levels, or in rare cases fever, chills and flu-like aches. Rejection can also occur without any apparent symptoms. That is one of the reasons why regular check-ups are necessary and why some programs may elect to perform "surveillance" fiberoptic bronchoscopy with biopsies of your lung. Your transplant team will instruct you regarding whom to call to immediately report any symptoms. ARE YOU A TRANSPLANT CANDIDATE? According to the American College of Chest Physicians (ACCP), in order to be considered for lung transplantation, you must:
While criteria differs among medical centers, most single lung transplant candidates are 65 years or younger, and double lung transplant candidates are 60 years or younger. They also have an acceptable nutritional status, and a satisfactory psychological profile and support system. HOW THE TRANSPLANT SYSTEM WORKS - AN OVERVIEW Under contract with a branch of the U.S. Department of Health & Human Services, Health Services & Resources Administration (HRSA), the United Network for Organ Sharing (UNOS) maintains a centralized computer network linking all organ procurement organizations and transplant centers.In the spring of 2005, UNOS adopted a new policy for the distribution of donor lungs incorporating a Lung Allocation Score (LAS) for transplant candidates. The LAS is a numerical scale, ranging from 0 (less ill) to 100 (gravely ill), that is used for lung candidates age 12 and over. It gives each individual a "score" (number) based on how urgently he or she needs a transplant and the chance of success after a transplant. The number is estimated using lab values, test results and disease diagnosis. If a transplant center determines that a patient is a candidate for a lung transplant, it will add the patient's medical profile to the national patient waiting list for organ transplant. To view the LAS system and calculator, please visit the UNOS Web site at www.unos.org/resources/frm_LAS_Calculator.asp. Once a patient is listed, UNOS utilizes a sophisticated database and allocation system to match transplant recipients with available organs. The names and medical profiles of newly-accepted transplant patients are added to the UNOS database and the waiting list automatically updates. When an organ donor becomes available, a transplant coordinator from an organ procurement organization accesses the UNOS computer. Each patient in the "pool" is then matched by computer for the donor characteristics and ranked based on the LAS. The computer then generates a ranked list of patients for each specific organ that can be procured from the donor in accordance with organ allocation policies. Once a potential organ recipient is selected and contacted, he or she is instructed to quickly and safely make their way to the hospital. After routine pre-operative testing is performed, the transplant then proceeds. This entails a highly coordinated team effort with the timing of events at the recipient hospital dependent on events at the donor hospital. Back to Table of ContentsPATIENT EVALUATION FOR LUNG TRANSPLANT Before you can qualify for a lung transplant, you will receive an evaluation from a lung transplant team, which typically consists of transplant pulmonologists, transplant surgeons, transplant coordinators, a social worker and a pulmonary rehabilitation specialist.During the evaluation, you will undergo a number of testing procedures that are used to assess your physical and medical condition. These tests may include:
THE TRANSPLANT WAITING LIST Waiting times for organ transplants vary from region to region, and may vary depending on the institution that you choose to perform the procedure. Transplant centers also may differ in their "acceptable criteria" in choosing suitable donors for lung transplantation. Overall, the median wait times for PF patients on the lung transplant list have been reduced substantially since the adoption of the LAS system in May 2005, dropping from an estimated 275 days (for all lung transplant candidates) in 2002 to a median of 69 days (for PF patients) in 2007 (Source: UNOS). Due to the severity of their disease, patients with PF generally benefit from the new Lung Allocation System because they achieve a higher disease severity score, potentially reducing their wait time.As of March, 2008, there were over 2,100 people waiting for a lung transplant, including approximately 390 PF patients. Unfortunately, though, the Organ Procurement and Transplantation Network, estimates there are only enough donor organs to provide about 1,000 lung transplants a year. Back to Table of ContentsTHE TRANSPLANT SURGERY Once a donor lung becomes available, time is critical. Ideally the lung should be transplanted into the patient receiving the organ within four to six hours. A team of surgeons and anesthesiologists performs an operation to remove the lung from the donor. Additional surgical teams may be present to remove other organs.After the lung is removed from the donor, it is preserved and packed for transport. Although the donor is deceased, this procedure is treated like any other operation using standard surgical practices and sterile techniques. Once the operation is complete and the incisions are closed, the donor's body is prepared for burial. Organ donation is considered an incredible gift of life, and the transplant team always treats the donor body with the respect it richly deserves. Typically, both lungs are removed from the donor together. If the recipient is in need of a double lung transplant, both lungs will be transplanted. Otherwise, the lungs are usually separated after they are removed from the donor and used for two single lung transplant recipients. In the meantime, the recipient is contacted and then prepared for surgery. Preparation involves administration of general anesthesia, and placement on an artificial breathing machine (e.g. ventilator). Lung transplant surgery may require the use of a "heart-lung machine" to oxygenate blood and maintain adequate blood flow to the recipient's vital organs. The transplant of the lung begins with removal of the diseased lung and the blood vessel attachments. When the lung is placed within the recipient, the blood vessels and airways from the donor lung are connected to the recipient's corresponding blood vessels and airway. Next, the blood flow and airflow are restored. After the transplant is complete, the incision is closed. The patient then recovers in the hospital's intensive care unit. When a double lung transplant is performed, it is much like two single lung transplants. The lung that is more diseased is transplanted first and then the less diseased lung is transplanted. More than 80% of adult patients who receive a lung transplant experience no limitations with regard to their activities one to five years post-transplant. Back to Table of ContentsAFTER TRANSPLANT SURGERY Following lung transplant surgery, the patient may remain on an artificial breathing machine for the first 12 hours of recovery. However, if the donor lung is functioning properly, the ventilator may be removed at the end of surgery. Depending on their progress, some patients are moved out of the ICU within a few days. Generally, they will also begin eating within the week following surgery. The total hospital stay is usually 10 to 14 days.Because the recipient's immune system will identify the new organ as foreign, rejection of the transplanted lung is always a possibility. Powerful drugs called immunosuppressants are given starting at the time of lung transplant surgery to try to prevent rejection. Blood tests are done on an ongoing basis post-transplant to confirm that the patient is receiving the correct dosage of medication. Prior to discharge, the transplant team reviews information with the patient, gives instructions for follow-up care and medications, informs patients of potential side effects and interactions of medications, and answers the patient's questions. A prescribed rehabilitation program will continue at home including physical activity, breathing exercises, nutrition, and the continuation of immunosuppressants and other medications. Signs and symptoms of rejection and infection are also discussed with the patient and family. Frequently, the patients are instructed to buy or are given small breathing machines called microspirometers, to monitor their own breathing capacity at home. RETURNING TO THE HOME ENVIRONMENT At-home rehabilitation for lung transplantation is a gradual process, and depends on the individual. The transplant team will give specific instructions. In general, walking is recommended to restore strength and prevent lung complications, but heavy lifting must be avoided for four to six weeks following transplant surgery. Other activities, such as driving, may usually begin when the incisions have healed. Sexual activity can resume when one is comfortable.Follow-up visits are required for check-ups and begin soon after returning home. Initially, outpatient visits may occur weekly or even more often, and as time progresses the frequency of follow-up visits usually decreases. As mentioned earlier, current survival rates are an estimated 80% at one year following transplantation and 60% at four years. The most critical period for survival is the first year after transplantation, when patients are at highest risk of surgical complications, rejection and infection. The functional status of the majority of post-transplant patients is generally improved. More than 80% of adult patients who receive a lung transplant experience no limitations with regard to their activities one to five years post-transplant, while an additional 10-15% require only some assistance with their daily activities. Only a very small proportion of patients (less than 5%) require complete assistance. Patients may return to full or partial employment, and resume participation in sporting activities. Back to Table of ContentsTALK TO YOUR DOCTOR ABOUT LUNG TRANSPLANTATION - TODAY! It is important to understand that pulmonary fibrosis is a progressive disease for which there is no cure, and that lung transplantation is the only treatment option shown to extend survival for patients with PF. The American College of Chest Physicians (ACCP) provides the following initial questions that patients should ask their physician and/or transplant center:
Because of the progressive nature of PF, it is critical for you to discuss lung transplant with your doctor as early as possible to determine if you are a candidate. Back to Table of ContentsRESOURCES FOR TRANSPLANT CANDIDATES AND RECIPIENTS
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