Materiały źródłowe

• #1 Pulmonary fibrosis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/symptoms-causes/syc-20353690

  Pulmonary fibrosis care at Mayo Clinic […] Medicines and therapies can sometimes help slow down the rate of fibrosis, ease symptoms and improve quality of life. For some people, a lung transplant might be an option. […] If you have symptoms of pulmonary fibrosis, contact your doctor or other healthcare professional as soon as possible. If your symptoms get worse, especially if they get worse fast, contact your healthcare team right away. […] Living with pulmonary fibrosis? Connect with others like you for support and answers to your questions in the Transplants support group on Mayo Clinic Connect, a patient community.

• #2 Pulmonary Fibrosis: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/10959-pulmonary-fibrosis

  Pulmonary fibrosis is scarring in your lungs. It can make it hard to expand your lungs and get enough oxygen to your body. Pulmonary fibrosis usually gets worse over time, but how quickly it gets worse is different for everyone. […] Pulmonary fibrosis is permanent and usually gets progressively worse over time (called progressive pulmonary fibrosis). […] No, there isn’t a cure for pulmonary fibrosis. Treatment focuses on easing symptoms, slowing down progression, and improving your quality of life. […] Treatments for pulmonary fibrosis could include: Antifibrotic medications. Nintedanib (OFEV) or pirfenidone (Esbriet) may slow down lung scarring and preserve lung function. […] If you have a PF diagnosis, it might be harder to fight off or recover from infections. Washing your hands frequently, disinfecting surfaces and avoiding crowded places especially during cold and flu season, and when COVID-19 cases are high might help you avoid getting sick frequently.

• #3 Pulmonary Fibrosis: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/10959-pulmonary-fibrosis

  Talk to a healthcare provider if you have shortness of breath or get tired more easily with everyday tasks than you used to. Early diagnosis and treatment can help slow down the progression of lung disease. […] Healthcare providers can’t easily predict how pulmonary fibrosis will progress. Your symptoms may get worse very slowly, over years. In some cases, the disease may lead to severe symptoms quickly (over months). […] It’s important to remember that every case of PF is different, and no one can tell you what your future holds. Treatment can sometimes slow down the progression of pulmonary fibrosis and improve your quality of life.

• #4

  https://www.nhs.uk/conditions/idiopathic-pulmonary-fibrosis/treatment/

  There’s currently no cure for idiopathic pulmonary fibrosis (IPF). The main aim of treatment is to relieve the symptoms as much as possible and slow down its progression. […] As the condition becomes more advanced, end of life (palliative) care will be offered. […] If your condition continues to get worse despite treatment, your specialist may recommend a lung transplant. […] If you’re told there’s nothing more that can be done to treat you, or you decide not to have treatment, your GP or care team will give you support and treatment to relieve your symptoms. This is called palliative care. […] Your doctor or care team should work with you to establish a clear plan based on your wishes.

• #5 Comprehensive care of the patient with idiopathic pulmonary fibrosis – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21760508/

  Purpose of review: Recently, an expert committee endorsed by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and the Latin American Thoracic Society published an evidence-based guideline on the management of idiopathic pulmonary fibrosis (IPF). In the current document, we summarize and supplement this recent expert document and propose a comprehensive approach to the care and management of patients with IPF. […] We propose three pillars of care for the patient with IPF titled 'disease-centered management’, 'symptom-centered management’, and 'education and self-management’. Disease-centered management involves both pharmacological and nonpharmacological approaches. Palliative care should be an integral and routine component of the care of patients with IPF. Education and self-management strengthens the provider-patient partnership by enabling patients to set realistic goals, remain in control of his or her care, and prepare for the future.

• #6 Nursing Care Plan for Restrictive Lung Diseases (Pulmonary Fibrosis, Neuromuscular Disorders) | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-restrictive-lung-diseases-pulmonary-fibrosis-neuromuscular-disorders

  Upon completion of this nursing care plan for Restrictive Lung Diseases (Pulmonary Fibrosis, Neuromuscular Disorders), nursing students will be able to: […] Provide comprehensive nursing care for patients with restrictive lung diseases, focusing on understanding the pathophysiology, etiology, and management of conditions such as pulmonary fibrosis and neuromuscular disorders affecting the lungs. […] This plan emphasizes the importance of respiratory support, symptom management, and patient education to enhance quality of life. […] Maintenance of optimal respiratory function. […] Effective management of symptoms like shortness of breath and fatigue. […] Enhanced ability to perform activities of daily living. […] Patient and family education about disease progression and management.

• #7 Pulmonary Fibrosis Nursing Diagnosis & Care Plan – NurseStudy.Net

  https://nursestudy.net/pulmonary-fibrosis-nursing-diagnosis/

  Pulmonary fibrosis is a chronic, progressive lung disease characterized by the formation of scar tissue in the lungs. This nursing diagnosis is crucial for healthcare providers to understand, as it guides the development of comprehensive care plans for patients with this condition. […] The first step in providing care for patients with pulmonary fibrosis is a thorough nursing assessment. This includes gathering physical, psychosocial, emotional, and diagnostic data. […] Nursing interventions for patients with pulmonary fibrosis focus on improving oxygenation, managing symptoms, and enhancing quality of life. The following interventions are essential for the care of patients with pulmonary fibrosis: […] The following nursing care plans provide a framework for addressing the key nursing diagnoses associated with pulmonary fibrosis.

• #8 Nursing Care Plan for Restrictive Lung Diseases (Pulmonary Fibrosis, Neuromuscular Disorders) | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-restrictive-lung-diseases-pulmonary-fibrosis-neuromuscular-disorders

  Continuous monitoring of respiratory status and oxygen saturation. […] Assess for signs of respiratory distress or hypoxemia. […] Evaluate the patients ability to perform activities of daily living. […] Monitor for complications such as respiratory infections or heart problems. […] Impaired Gas Exchange related to reduced lung volumes and decreased lung compliance. […] Activity Intolerance related to dyspnea and fatigue. […] Anxiety related to chronic disease and breathlessness. […] Knowledge Deficit regarding disease process and self-management strategies. […] Respiratory Support: Administer supplemental oxygen as prescribed and assist with non-invasive ventilation if needed. […] Rationale: To improve oxygenation and ease breathing effort. […] Energy Conservation Techniques: Educate on pacing activities and planning rest periods.

• #9 Nursing Care Plan for Restrictive Lung Diseases (Pulmonary Fibrosis, Neuromuscular Disorders) | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-restrictive-lung-diseases-pulmonary-fibrosis-neuromuscular-disorders

  Continuous monitoring of respiratory status and oxygen saturation. […] Assess for signs of respiratory distress or hypoxemia. […] Evaluate the patients ability to perform activities of daily living. […] Monitor for complications such as respiratory infections or heart problems. […] Impaired Gas Exchange related to reduced lung volumes and decreased lung compliance. […] Activity Intolerance related to dyspnea and fatigue. […] Anxiety related to chronic disease and breathlessness. […] Knowledge Deficit regarding disease process and self-management strategies. […] Respiratory Support: Administer supplemental oxygen as prescribed and assist with non-invasive ventilation if needed. […] Rationale: To improve oxygenation and ease breathing effort. […] Energy Conservation Techniques: Educate on pacing activities and planning rest periods.

• #10 Pulmonary Fibrosis Nursing Diagnosis & Care Plan – NurseStudy.Net

  https://nursestudy.net/pulmonary-fibrosis-nursing-diagnosis/

  Nursing Diagnosis: Impaired Gas Exchange related to alveolar-capillary membrane changes secondary to pulmonary fibrosis as evidenced by dyspnea, decreased oxygen saturation, and abnormal arterial blood gas values. […] Nursing Diagnosis: Activity Intolerance related to imbalance between oxygen supply and demand secondary to pulmonary fibrosis as evidenced by dyspnea on exertion and fatigue with minimal activity. […] Nursing Diagnosis: Ineffective Breathing Pattern related to decreased lung compliance secondary to pulmonary fibrosis as evidenced by tachypnea, shallow breathing, and use of accessory muscles. […] Nursing Diagnosis: Anxiety related to chronic disease process and dyspnea as evidenced by expressed feelings of apprehension, restlessness, and increased respiratory rate. […] Nursing Diagnosis: Ineffective Health Management related to knowledge deficit regarding pulmonary fibrosis management as evidenced by verbalized misconceptions and non-adherence to the treatment plan.

• #11 Pulmonary Fibrosis Nursing Diagnosis & Care Plan – NurseStudy.Net

  https://nursestudy.net/pulmonary-fibrosis-nursing-diagnosis/

  Nursing Diagnosis: Impaired Gas Exchange related to alveolar-capillary membrane changes secondary to pulmonary fibrosis as evidenced by dyspnea, decreased oxygen saturation, and abnormal arterial blood gas values. […] Nursing Diagnosis: Activity Intolerance related to imbalance between oxygen supply and demand secondary to pulmonary fibrosis as evidenced by dyspnea on exertion and fatigue with minimal activity. […] Nursing Diagnosis: Ineffective Breathing Pattern related to decreased lung compliance secondary to pulmonary fibrosis as evidenced by tachypnea, shallow breathing, and use of accessory muscles. […] Nursing Diagnosis: Anxiety related to chronic disease process and dyspnea as evidenced by expressed feelings of apprehension, restlessness, and increased respiratory rate. […] Nursing Diagnosis: Ineffective Health Management related to knowledge deficit regarding pulmonary fibrosis management as evidenced by verbalized misconceptions and non-adherence to the treatment plan.

• #12 Nursing Care Plan for Restrictive Lung Diseases (Pulmonary Fibrosis, Neuromuscular Disorders) | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-restrictive-lung-diseases-pulmonary-fibrosis-neuromuscular-disorders

  Continuous monitoring of respiratory status and oxygen saturation. […] Assess for signs of respiratory distress or hypoxemia. […] Evaluate the patients ability to perform activities of daily living. […] Monitor for complications such as respiratory infections or heart problems. […] Impaired Gas Exchange related to reduced lung volumes and decreased lung compliance. […] Activity Intolerance related to dyspnea and fatigue. […] Anxiety related to chronic disease and breathlessness. […] Knowledge Deficit regarding disease process and self-management strategies. […] Respiratory Support: Administer supplemental oxygen as prescribed and assist with non-invasive ventilation if needed. […] Rationale: To improve oxygenation and ease breathing effort. […] Energy Conservation Techniques: Educate on pacing activities and planning rest periods.

• #13 Pulmonary Fibrosis – What You Need to Know

  https://www.drugs.com/cg/pulmonary-fibrosis.html

  Pulmonary fibrosis is the scarring of your lung tissues over time. It is also called interstitial lung disease. The air sacs and tissues in your lungs swell, scars form, and the tissues become thick and stiff. This affects how much oxygen you get and makes it hard to breathe. […] There is no cure for pulmonary fibrosis. The goal of treatment is to slow the progress of the disease and treat your symptoms. You may need any of the following: Extra oxygen may be needed if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils. […] Use oxygen at home as directed. Oxygen is usually given through a nasal cannula. This is a pair of short, thin tubes that rest just inside your nose. Tell your healthcare provider if your nose gets dry or if the skin gets red or sore. Never smoke or let anyone else smoke in the same room while your oxygen is on.

• #14 Pulmonary fibrosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/diagnosis-treatment/drc-20353695

  Our caring team of Mayo Clinic experts can help you with your pulmonary fibrosis-related health concerns. […] Treatment depends on the cause of your pulmonary fibrosis. Doctors and other healthcare professionals evaluate how severe your condition is. Then together you can decide on the best treatment plan. […] Pulmonary rehabilitation can help manage your symptoms and improve your ability to do daily tasks. Pulmonary rehabilitation programs focus on: Physical exercise to improve how much you can do. Breathing techniques that may improve how well your lungs use oxygen. Nutritional counseling. Emotional counseling and support. Education about your condition. […] When symptoms suddenly get worse, called an acute exacerbation, you may need more supplemental oxygen. In some cases, you may need mechanical ventilation in the hospital.

• #15 Pulmonary Fibrosis – What You Need to Know

  https://www.drugs.com/cg/pulmonary-fibrosis.html

  Pulmonary fibrosis is the scarring of your lung tissues over time. It is also called interstitial lung disease. The air sacs and tissues in your lungs swell, scars form, and the tissues become thick and stiff. This affects how much oxygen you get and makes it hard to breathe. […] There is no cure for pulmonary fibrosis. The goal of treatment is to slow the progress of the disease and treat your symptoms. You may need any of the following: Extra oxygen may be needed if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils. […] Use oxygen at home as directed. Oxygen is usually given through a nasal cannula. This is a pair of short, thin tubes that rest just inside your nose. Tell your healthcare provider if your nose gets dry or if the skin gets red or sore. Never smoke or let anyone else smoke in the same room while your oxygen is on.

• #16 Pulmonary fibrosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/diagnosis-treatment/drc-20353695

  Our caring team of Mayo Clinic experts can help you with your pulmonary fibrosis-related health concerns. […] Treatment depends on the cause of your pulmonary fibrosis. Doctors and other healthcare professionals evaluate how severe your condition is. Then together you can decide on the best treatment plan. […] Pulmonary rehabilitation can help manage your symptoms and improve your ability to do daily tasks. Pulmonary rehabilitation programs focus on: Physical exercise to improve how much you can do. Breathing techniques that may improve how well your lungs use oxygen. Nutritional counseling. Emotional counseling and support. Education about your condition. […] When symptoms suddenly get worse, called an acute exacerbation, you may need more supplemental oxygen. In some cases, you may need mechanical ventilation in the hospital.

• #17 Pulmonary Fibrosis – What You Need to Know

  https://www.drugs.com/cg/pulmonary-fibrosis.html

  Go to pulmonary rehab as directed. Specialists will help you safely strengthen your lungs and prevent more tissue damage. The plan includes education about your condition, exercise, breathing strategies, and ways to conserve energy. Pulmonary rehab may help decrease your breathing problems and help you function better in your daily activities.

• #18 Management of idiopathic pulmonary fibrosis | Nursing Times

  https://www.nursingtimes.net/respiratory/management-of-idiopathic-pulmonary-fibrosis-11-04-2014/

  Idiopathic pulmonary fibrosis is a life-limiting, incurable condition. […] Given that treatment options are limited and disease progression is rapid, nurses are pivotal in the care of these patients. […] Nurses are crucial in providing best supportive care, which should be tailored to disease severity and rate of progression, and patient preferences from diagnosis through to end-of-life care. […] Regular assessment for exercise-induced hypoxia and prompt referral for ambulatory oxygen assessment enable patients to engage in normal activities inside and outside the home for as long as possible. […] Supportive nurses are critical to helping patients adjust and develop coping strategies. […] NICE (2013a) recommends offering pulmonary rehabilitation to patients throughout their disease pathway.

• #19 Pulmonary Fibrosis Treatment Options | Temple Health

  https://www.templehealth.org/services/conditions/pulmonary-fibrosis/treatment-options

  Treatment of pulmonary fibrosis focuses on alleviating symptoms and improving quality of life. […] Together, our specialists work closely with your doctor to find out the cause and severity of your pulmonary fibrosis. […] This may include a combination of lifestyle changes, medications, supplemental oxygen, and pulmonary rehab. […] Pulmonary rehabilitation is a standard treatment for people with chronic lung disease. […] Pulmonary rehab does not replace medical therapy. It’s used alongside medical treatments to help patients better manage their conditions and function at their best. […] You are the most essential member of your care team. […] Temple offers a comprehensive pulmonary rehabilitation program to support you in your efforts. […] Pulmonary rehabilitation is a standard treatment used alongside medication therapy for those with chronic lung disease.

• #20 Pulmonary Fibrosis: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/10959-pulmonary-fibrosis

  Pulmonary fibrosis is scarring in your lungs. It can make it hard to expand your lungs and get enough oxygen to your body. Pulmonary fibrosis usually gets worse over time, but how quickly it gets worse is different for everyone. […] Pulmonary fibrosis is permanent and usually gets progressively worse over time (called progressive pulmonary fibrosis). […] No, there isn’t a cure for pulmonary fibrosis. Treatment focuses on easing symptoms, slowing down progression, and improving your quality of life. […] Treatments for pulmonary fibrosis could include: Antifibrotic medications. Nintedanib (OFEV) or pirfenidone (Esbriet) may slow down lung scarring and preserve lung function. […] If you have a PF diagnosis, it might be harder to fight off or recover from infections. Washing your hands frequently, disinfecting surfaces and avoiding crowded places especially during cold and flu season, and when COVID-19 cases are high might help you avoid getting sick frequently.

• #21

  https://journals.lww.com/tnpj/fulltext/2018/05000/idiopathic_pulmonary_fibrosis__a_guide_for_nurse.9.aspx

  Idiopathic pulmonary fibrosis (IPF) is a rare disease characterized by decline in lung function, dyspnea, and cough. The clinical course of IPF is variable and unpredictable. Early referral to specialists is key to ensure timely and accurate diagnosis. Two antifibrotic drugs (nintedanib and pirfenidone) have been approved for the treatment of IPF. […] The disabling and relentless symptoms of IPF, its poor prognosis, and uncertainties around the course of the disease can have a devastating impact on the lives of patients and their families. IPF has no cure; however, caregivers can help patients to better understand and manage their disease, evaluate treatment options, and access support to improve their quality of life. […] Optimal management of patients with IPF is multifaceted and evolves over the course of the disease. Two antifibrotic drugs, nintedanib and pirfenidone, have been approved for the treatment of IPF in the United States. Both of these drugs have been shown to reduce disease progression in patients with IPF by reducing decline in FVC.

• #22 Idiopathic pulmonary fibrosis: What primary care physicians need to know | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/85/5/377

  Accurate diagnosis of IPF is crucial. We recommend early referral to a center specializing in interstitial lung disease to confirm the diagnosis, start appropriate therapy, advise the patient on prognosis and enrollment in disease registries and clinical trials, and determine candidacy for lung transplant. […] The goal of this article is to delineate the features of IPF so that it may be recognized early and thus expedite referral to a center with expertise in interstitial lung disease for a thorough evaluation and appropriate management. […] Antifibrotic therapy is a choice between pirfenidone and nintedanib. […] Patients need to understand that these drugs slow the rate of decline in FVC but have not been shown to improve symptoms or functional status. […] Corticosteroids should not be used routinely in the treatment of IPF. […] Their use in IPF is discouraged, and the joint international guidelines recommend against immunosuppression to treat IPF. […] Prompt referral for lung transplant is imperative. IPF is now the most common indication for lung transplant, and given the poor overall prognosis of advanced IPF, transplant confers a survival benefit in appropriately selected patients.

• #23 How to Support Your Loved One with Pulmonary Fibrosis | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/family-and-friends/how-to-support-your-loved-one

  It is not always clear how you can help someone with pulmonary fibrosis. It is a disease that is very stressful and a diagnosis can be difficult on family, friends and caregivers. Here are some ways you can support a loved one diagnosed with PF. […] It is important that pulmonary fibrosis patients maintain a sense of independence. Resist the urge to do everything for them. Instead, talk about where you can help and where you need to step back. […] A diagnosis of pulmonary fibrosis will stir up many emotions. Patients may feel angry, depressed and anxious. It is common for patients to feel like they are losing part of their identity because they might not be able to do what they did before. It can be very reassuring to tell your loved one that you are there for them and will support them through this difficult time.

• #24 Planning for Life with Pulmonary Fibrosis | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/planning-for-life-with-pulmonary-fibrosis

  It is likely you will need oxygen therapy at some point. For example, you may start out needing oxygen only during exercise and then needing oxygen more often as the disease progresses. Supplemental oxygen can help you stay active and maintain a good quality of life. […] You will need to stay as active as possible. Many patients benefit from pulmonary rehabilitation. Ask your doctor for a referral to pulmonary rehab and about what kind of exercise you should be doing on your own. […] No one can cope with a serious illness alone. You can expect to feel a variety of emotions and need support from family and friends. You should also consider talking to a mental health professional who can help you work through your emotions. […] You will need to plan for the future. A PF diagnosis can make patients and their caregivers think about difficult topics they may not have explored before. It is inevitable to think about death and what will happen in the future. No one can know exactly what will happen, but it is helpful to make plans for whenever that day comes.

• #25 How to Support Your Loved One with Pulmonary Fibrosis | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/family-and-friends/how-to-support-your-loved-one

  It is not always clear how you can help someone with pulmonary fibrosis. It is a disease that is very stressful and a diagnosis can be difficult on family, friends and caregivers. Here are some ways you can support a loved one diagnosed with PF. […] It is important that pulmonary fibrosis patients maintain a sense of independence. Resist the urge to do everything for them. Instead, talk about where you can help and where you need to step back. […] A diagnosis of pulmonary fibrosis will stir up many emotions. Patients may feel angry, depressed and anxious. It is common for patients to feel like they are losing part of their identity because they might not be able to do what they did before. It can be very reassuring to tell your loved one that you are there for them and will support them through this difficult time.

• #26 Idiopathic pulmonary fibrosis in adults: diagnosis and management – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK553262/

  This guideline offers best practice advice on the care of people with idiopathic pulmonary fibrosis. […] The consultant respiratory physician or interstitial lung disease specialist nurse should provide accurate and clear information (verbal and written) to people with idiopathic pulmonary fibrosis, and their families and carers with the persons consent. This should include information about investigations, diagnosis and management. […] An interstitial lung disease specialist nurse should be available at all stages of the care pathway to provide information and support to people with idiopathic pulmonary fibrosis and their families and carers with the persons consent. […] Assess people with idiopathic pulmonary fibrosis for pulmonary rehabilitation at the time of diagnosis. Assessment may include a 6-minute walk test (distance walked and oxygen saturation measured by pulse oximetry) and a quality-of-life assessment.

• #27

  https://www.nursingcenter.com/cearticle?an=00152193-202101000-00007&Journal_ID=54016&Issue_ID=5737721

  Idiopathic pulmonary fibrosis (IPF) is a restrictive lung disease in which the cause cannot be determined. This article discusses restrictive lung diseases that fall under the general category of interstitial lung disease (ILD) with a focus on IPF-a fatal disease characterized by progressive fibrosis and interstitial pneumonia, dyspnea, and decreasing pulmonary function. […] The intent of this article is to provide nurses with a better understanding of the overall disorder and care needed for people who have been diagnosed with a restrictive lung disease, particularly IPF. […] Management goals for patients with ILD and IPF include slowing the fibrotic process and relieving the patient’s dyspnea. […] Nursing care for patients like AC includes educating the patient and family about the disease process and recommending community resources for support. Education should include strategies for avoiding respiratory irritants such as dust, mold, and animal dander; performing optimal hand hygiene practices; and preventing respiratory infections by avoiding crowds and people who are sick. […] Palliative care should be considered early in the disease process. Discussing expectations and wishes for end-of-life care can help patients improve their mental outlook and decrease the stress on both patients and the families.

• #28

  https://journals.lww.com/tnpj/fulltext/2018/05000/idiopathic_pulmonary_fibrosis__a_guide_for_nurse.9.aspx

  In addition to drug therapy, symptom relief and supportive care are important elements of IPF management. Common issues healthcare providers face when treating patients include dyspnea, cough, fatigue, and depression, which are often difficult to manage. Optimal symptom management requires a multidisciplinary approach focusing on patient education and self-management to develop a treatment plan and goals of patient care. […] Patient education and self-management are critical to help patients with IPF manage their disease and make decisions regarding their care. Patients frequently require support with coming to terms with an IPF diagnosis and coping with the challenges associated with the disease. Educating patients about the disease and its progression allows patients and their caregivers to set realistic goals, feel in control, and prepare for their future.

• #29 Idiopathic pulmonary fibrosis in adults: diagnosis and management – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK553262/

  This guideline offers best practice advice on the care of people with idiopathic pulmonary fibrosis. […] The consultant respiratory physician or interstitial lung disease specialist nurse should provide accurate and clear information (verbal and written) to people with idiopathic pulmonary fibrosis, and their families and carers with the persons consent. This should include information about investigations, diagnosis and management. […] An interstitial lung disease specialist nurse should be available at all stages of the care pathway to provide information and support to people with idiopathic pulmonary fibrosis and their families and carers with the persons consent. […] Assess people with idiopathic pulmonary fibrosis for pulmonary rehabilitation at the time of diagnosis. Assessment may include a 6-minute walk test (distance walked and oxygen saturation measured by pulse oximetry) and a quality-of-life assessment.

• #30 Nursing Care Plan for Restrictive Lung Diseases (Pulmonary Fibrosis, Neuromuscular Disorders) | Free NURSING.com Courses

  https://nursing.com/lesson/nursing-care-plan-for-restrictive-lung-diseases-pulmonary-fibrosis-neuromuscular-disorders

  Continuous monitoring of respiratory status and oxygen saturation. […] Assess for signs of respiratory distress or hypoxemia. […] Evaluate the patients ability to perform activities of daily living. […] Monitor for complications such as respiratory infections or heart problems. […] Impaired Gas Exchange related to reduced lung volumes and decreased lung compliance. […] Activity Intolerance related to dyspnea and fatigue. […] Anxiety related to chronic disease and breathlessness. […] Knowledge Deficit regarding disease process and self-management strategies. […] Respiratory Support: Administer supplemental oxygen as prescribed and assist with non-invasive ventilation if needed. […] Rationale: To improve oxygenation and ease breathing effort. […] Energy Conservation Techniques: Educate on pacing activities and planning rest periods.

• #31 Pulmonary fibrosis self-management plan | Asthma + Lung UK

  https://www.asthmaandlung.org.uk/conditions/pulmonary-fibrosis/pulmonary-fibrosis-sm-plan

  If youve been diagnosed with pulmonary fibrosis, including IPF, our self-management plan can help you take care of yourself and feel more in control. […] Checklist for my care – have a look at this list to check youre getting all the care and support you need. This will help you to ask your healthcare professionals about anything youre not getting. […] Flare-up (exacerbation) plan – use this page to agree a plan with your healthcare professional about what to do if your symptoms get worse suddenly (flare up). […] My goals – walking the dog? Seeing your family at the other end of the country? If something is important to you, this page helps you talk to your healthcare professional about how youre going to achieve it.

• #32 Idiopathic Pulmonary Fibrosis | AAFP

  https://www.aafp.org/family-physician/patient-care/care-resources/respiratory-health/idiopathic-pulmonary-fibrosis.html

  As frontline health care providers, you play an essential role in the early detection of idiopathic pulmonary fibrosis (IPF) and the timely referral to a pulmonologist. The disease is rare and includes signs and symptoms that make it difficult to distinguish among other interstitial lung diseases (ILDs). […] By identifying suspected cases of IPF at primary care visits, you have an opportunity to refer patients earlier, and enable diagnosis and treatment sooner. This makes education about IPF a key factor in early detection, which can potentially lead to better health outcomes. […] Family physicians play an important role in the ongoing care of their patients with IPF. Family physicians can contribute to the care of patients with IPF in the following ways: Oversee the treatment of comorbidities, Encourage participation in pulmonary rehabilitation and monitor progress, Vaccinate against influenza, pneumococcus, and pertussis, Assess emotional and mental health, Recommend support groups for patients and their caregivers, Monitor the need for oxygen therapy, Discuss treatment preferences and end-of-life care. […] Patients value a trusted source of information and may ask their family physician for information and advice. Family physicians should provide their patients with guidance for self-management of their disease and recommend credible resources for patient education.

• #33

  https://www.pulmonaryfibrosis.org/patients-caregivers/medical-and-support-resources/for-the-caregiver

  I am a caregiver for someone with pulmonary fibrosis. What do I need to know? […] The role of a caregiver includes two essential responsibilities: Do what needs to be done each day to take care of your loved one. […] Take care of yourself. The day-to-day life of a caregiver can be physically and emotionally draining. […] If you’re caring for a patient living with pulmonary fibrosis (PF), there is information available to help you on this journey. […] The Pulmonary Fibrosis Foundation’s newest resource „Caregiving for a Patient with Pulmonary Fibrosis: A Guide for Family and Friends” may help you to be a more effective caregiver while learning how to care for yourself at the same time. […] Be actively involved in your loved one’s medical treatment. […] Participate in your loved one’s diagnostic journey and treatment regimen, which can sometimes be difficult and confusing. Your support may help to alleviate anxiety and frustration for both of you.

• #34 Information for carers, family and friends – Action for Pulmonary Fibrosis

  https://www.actionpf.org/information-and-support/carer-family-friends

  Across the country, thousands of carers look after loved ones with pulmonary fibrosis. Never be afraid to ask for help. We are here to support you to look after yourself, so that you can look after others. […] Support for family, friends and carers: an overview […] Carers assessments

• #35

  https://www.pulmonaryfibrosis.org/patients-caregivers/medical-and-support-resources/for-the-caregiver

  Learn about Advance Directives like Living Wills and Medical Power of Attorney, Physician Orders for Life-Sustaining Therapy (POLST), and palliative care options, long before they seem to be needed. […] Keep up with your own medical appointments. […] Find someone you can talk to when things are hard. You can also join a support group for caregivers.

• #36 How to Support Your Loved One with Pulmonary Fibrosis | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/family-and-friends/how-to-support-your-loved-one

  Palliative care, sometimes known as supportive care, helps patients feel their best, physically and emotionally, during treatment. It’s often misunderstood as care only given at the end of life, but palliative care is recommended at the start of treatment. Help your loved one connect with palliative care so they can manage their side effects. […] It is important for the patient to think about what needs to happen so they can continue to get the care they need and want in the future and as their disease progresses. As a caregiver, you can help start these important conversations and assist with filling out paperwork, including an advance directive.

• #37

  https://journals.lww.com/tnpj/fulltext/2018/05000/idiopathic_pulmonary_fibrosis__a_guide_for_nurse.9.aspx

  The course of IPF is variable and unpredictable. Patients with IPF should be followed up with at regular intervals to assess disease progression, ensure that symptoms and comorbidities are being treated optimally, and to provide emotional support and advice in managing adverse events associated with antifibrotic medications. Regular review of pulmonary function is the practical approach to monitoring disease progression. […] Care for patients with IPF should focus on maximizing length and quality of life. The development of antifibrotic therapies was a major step forward in treating IPF. Starting either nintedanib or pirfenidone as soon as a diagnosis of IPF is made should be considered for all patients, since these drugs slow the rate of loss of lung function. Treatment of comorbidities, symptom management, and integrating ongoing education into patient care are all crucial in improving a patient’s quality of life.

• #38 Idiopathic pulmonary fibrosis in adults: diagnosis and management – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK553262/

  Offer best supportive care to people with idiopathic pulmonary fibrosis from the point of diagnosis. Best supportive care should be tailored to disease severity, rate of progression, and the persons preference, and should include if appropriate: information and support (see recommendation 1.3.1), symptom relief, management of comorbidities, withdrawal of therapies suspected to be ineffective or causing harm, end of life care. […] In follow-up appointments for people with idiopathic pulmonary fibrosis: assess lung function, assess for oxygen therapy, assess for pulmonary rehabilitation, offer smoking cessation advice, in line with Smoking cessation services (NICE public health guidance 10), identify exacerbations and previous respiratory hospital admissions, consider referral for assessment for lung transplantation in people who do not have absolute contraindications, consider psychosocial needs and referral to relevant services as appropriate, consider referral to palliative care services, assess for comorbidities (which may include anxiety, bronchiectasis, depression, diabetes, dyspepsia, ischaemic heart disease, lung cancer and pulmonary hypertension).

• #39 Interstitial (Nonidiopathic) Pulmonary Fibrosis Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/301337-treatment

  Lung transplantation is a treatment option for selected patients with advanced or progressive disease refractory to medical therapy who may be expected to have increased post-transplant survival compared to supportive therapy without transplant. […] Following transplantation, patients overall report improved quality of life with better physical, social, and general health functioning. […] Exercise and pulmonary rehabilitation may confer benefits on functional status. […] Patients with ILD can have hypoxic respiratory failure, either in the acute setting due to an exacerbation, or more chronically, requiring supplemental oxygen. […] Some types of ILD can be prevented or mitigated. […] Patients with DPLD should be evaluated initially every 3-6 months. […] The prognosis is variable and depends on the specific diagnosis and clinical, physiologic, and pathologic severity. […] Patients who demonstrate more stable disease over time can increase the intervals between visits.

• #40 Interstitial (Nonidiopathic) Pulmonary Fibrosis Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/301337-treatment

  Lung transplantation is a treatment option for selected patients with advanced or progressive disease refractory to medical therapy who may be expected to have increased post-transplant survival compared to supportive therapy without transplant. […] Following transplantation, patients overall report improved quality of life with better physical, social, and general health functioning. […] Exercise and pulmonary rehabilitation may confer benefits on functional status. […] Patients with ILD can have hypoxic respiratory failure, either in the acute setting due to an exacerbation, or more chronically, requiring supplemental oxygen. […] Some types of ILD can be prevented or mitigated. […] Patients with DPLD should be evaluated initially every 3-6 months. […] The prognosis is variable and depends on the specific diagnosis and clinical, physiologic, and pathologic severity. […] Patients who demonstrate more stable disease over time can increase the intervals between visits.

• #41

  https://www.pulmonaryfibrosis.org/patients-caregivers/medical-and-support-resources/for-the-caregiver

  Be prepared to advocate for the patient if you feel it is necessary. […] Help your loved one maintain a healthy diet and exercise program. […] Speak with your loved one’s physician about their nutritional requirements and help make sure that they are eating a healthy diet. […] See if a pulmonary rehabilitation program is recommended for your loved one to help them maintain muscle tone, which can help with fatigue and breathlessness. […] Let your loved one do everything they can for as long as possible. […] Don’t take away a pulmonary fibrosis patient’s independence any sooner than necessary, but also be prepared to take over responsibility for things your loved one may no longer be able to do now or in the future. […] Help prevent respiratory infections. […] Notify your loved one’s primary care provider immediately at the onset of a cold, cough, or flu.

• #42 Idiopathic pulmonary fibrosis in adults: diagnosis and management – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK553262/

  Offer best supportive care to people with idiopathic pulmonary fibrosis from the point of diagnosis. Best supportive care should be tailored to disease severity, rate of progression, and the persons preference, and should include if appropriate: information and support (see recommendation 1.3.1), symptom relief, management of comorbidities, withdrawal of therapies suspected to be ineffective or causing harm, end of life care. […] In follow-up appointments for people with idiopathic pulmonary fibrosis: assess lung function, assess for oxygen therapy, assess for pulmonary rehabilitation, offer smoking cessation advice, in line with Smoking cessation services (NICE public health guidance 10), identify exacerbations and previous respiratory hospital admissions, consider referral for assessment for lung transplantation in people who do not have absolute contraindications, consider psychosocial needs and referral to relevant services as appropriate, consider referral to palliative care services, assess for comorbidities (which may include anxiety, bronchiectasis, depression, diabetes, dyspepsia, ischaemic heart disease, lung cancer and pulmonary hypertension).

• #43 Pulmonary Fibrosis: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/10959-pulmonary-fibrosis

  Pulmonary fibrosis is scarring in your lungs. It can make it hard to expand your lungs and get enough oxygen to your body. Pulmonary fibrosis usually gets worse over time, but how quickly it gets worse is different for everyone. […] Pulmonary fibrosis is permanent and usually gets progressively worse over time (called progressive pulmonary fibrosis). […] No, there isn’t a cure for pulmonary fibrosis. Treatment focuses on easing symptoms, slowing down progression, and improving your quality of life. […] Treatments for pulmonary fibrosis could include: Antifibrotic medications. Nintedanib (OFEV) or pirfenidone (Esbriet) may slow down lung scarring and preserve lung function. […] If you have a PF diagnosis, it might be harder to fight off or recover from infections. Washing your hands frequently, disinfecting surfaces and avoiding crowded places especially during cold and flu season, and when COVID-19 cases are high might help you avoid getting sick frequently.

• #44

  https://www.pulmonaryfibrosis.org/patients-caregivers/medical-and-support-resources/for-the-caregiver

  Learn about Advance Directives like Living Wills and Medical Power of Attorney, Physician Orders for Life-Sustaining Therapy (POLST), and palliative care options, long before they seem to be needed. […] Keep up with your own medical appointments. […] Find someone you can talk to when things are hard. You can also join a support group for caregivers.

• #45 Idiopathic pulmonary fibrosis in adults: diagnosis and management – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK553262/

  Offer best supportive care to people with idiopathic pulmonary fibrosis from the point of diagnosis. Best supportive care should be tailored to disease severity, rate of progression, and the persons preference, and should include if appropriate: information and support (see recommendation 1.3.1), symptom relief, management of comorbidities, withdrawal of therapies suspected to be ineffective or causing harm, end of life care. […] In follow-up appointments for people with idiopathic pulmonary fibrosis: assess lung function, assess for oxygen therapy, assess for pulmonary rehabilitation, offer smoking cessation advice, in line with Smoking cessation services (NICE public health guidance 10), identify exacerbations and previous respiratory hospital admissions, consider referral for assessment for lung transplantation in people who do not have absolute contraindications, consider psychosocial needs and referral to relevant services as appropriate, consider referral to palliative care services, assess for comorbidities (which may include anxiety, bronchiectasis, depression, diabetes, dyspepsia, ischaemic heart disease, lung cancer and pulmonary hypertension).

• #46 How to Support Your Loved One with Pulmonary Fibrosis | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/family-and-friends/how-to-support-your-loved-one

  Palliative care, sometimes known as supportive care, helps patients feel their best, physically and emotionally, during treatment. It’s often misunderstood as care only given at the end of life, but palliative care is recommended at the start of treatment. Help your loved one connect with palliative care so they can manage their side effects. […] It is important for the patient to think about what needs to happen so they can continue to get the care they need and want in the future and as their disease progresses. As a caregiver, you can help start these important conversations and assist with filling out paperwork, including an advance directive.

• #47 Patient-centered care in pulmonary fibrosis: access, anticipate, and act | Respiratory Research | Full Text

  https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-024-02997-7

  Patient-centered care in patients with PF encompasses many facets, each with its own set of nuances and potential barriers to integration. […] Optimizing patient-centered care is not possible without understanding the needs of patients and their caregivers. Patient needs should be the central focus and anticipated at every stage of care. […] Palliative care aims to improve HRQOL for patients and their caregivers throughout the disease course, which is particularly relevant in PF due to the high disease burden and often progressive disease course. […] The principal symptoms of PF that profoundly impact patient HRQOL include dyspnea, cough, and fatigue. Managing these symptoms with currently available treatments remains challenging. […] Although many patients with IPF have a worse prognosis than those with lung cancer, end-of-life care is far less developed in this area, with less symptom relief achieved in IPF. An observational study in dying patients with ILD compared to patients dying with lung cancer showed that patients with ILD had lower quality of dying and death and a lower frequency of participation in end-of-life discussions. […] In conclusion, we believe that routinely addressing the domains of the Triple A Care model in clinical practice can improve the adoption of a more individually tailored approach to PF care.

• #48 Patient-centered care in pulmonary fibrosis: access, anticipate, and act | Respiratory Research | Full Text

  https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-024-02997-7

  Comprehensive care integrates individual patient needs and is highly valued for patients with pulmonary fibrosis (PF). The importance of a patient-centered care approach is rooted in the unpredictable progressiveness of the disease course in PF. The respiratory impairment associated with PF has a major impact on the quality of life for both patients and their caregivers. We believe that prioritizing patient preferences could improve the shared decision making process and may ultimately lead to better health outcomes. Despite the growing emphasis for this approach, it remains challenging to adopt it in clinical practice. […] In this narrative review, the Triple A Care Model is introduced, integrating the domains of patient-centered care described in the published literature, while also addressing the unmet needs in providing comprehensive care.

• #49 Patient-centered care in pulmonary fibrosis: access, anticipate, and act | Respiratory Research | Full Text

  https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-024-02997-7

  We propose the Triple A Care Model for patient-centered care in pulmonary fibrosis, Access, Anticipate, and Act. This model serves as a tool to deliver personalized care. We believe that by routinely identifying patients needs and evaluating whether the topics of the three domains of the model are targeted, PF care can be optimized. This model can help raise awareness of the importance of holistic care for patients with PF. Access refers to raising PF awareness among clinicians and the general public and adopting of eHealth technologies, thereby providing early access to specialized centers, information and education, and clinical trial options. Anticipate encompasses continuous assessment of patient needs, preventive measures, disease monitoring, and advance care planning (ACP). Finally, the Act domain focusses on the timely initiation of disease-modifying treatment and symptom relief, early evaluation for lung transplantation (LTx), and promptly adopting end-of-life care.

• #50 Patient-centered care in pulmonary fibrosis: access, anticipate, and act | Respiratory Research | Full Text

  https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-024-02997-7

  We propose the Triple A Care Model for patient-centered care in pulmonary fibrosis, Access, Anticipate, and Act. This model serves as a tool to deliver personalized care. We believe that by routinely identifying patients needs and evaluating whether the topics of the three domains of the model are targeted, PF care can be optimized. This model can help raise awareness of the importance of holistic care for patients with PF. Access refers to raising PF awareness among clinicians and the general public and adopting of eHealth technologies, thereby providing early access to specialized centers, information and education, and clinical trial options. Anticipate encompasses continuous assessment of patient needs, preventive measures, disease monitoring, and advance care planning (ACP). Finally, the Act domain focusses on the timely initiation of disease-modifying treatment and symptom relief, early evaluation for lung transplantation (LTx), and promptly adopting end-of-life care.

• #51 UR Pulmonary Fibrosis Foundation (PFF) Care Center – Rochester, NY – Pulmonary and Critical Care Medicine – University of Rochester Medical Center

  https://www.urmc.rochester.edu/pulmonary/patient-care/pulmonary-fibrosis-center

  The UR PFF Care Center is the only Pulmonary Fibrosis Foundation Care Center Network site in Upstate New York. […] The PFF-CCN was developed to offer patients access to world experts in scarring lung diseases, improve the accuracy of diagnoses, improve access to anti-scarring medications, and advance research of this group of potentially devastating lung diseases. […] The UR-PFF Care Center welcomes patients with scarring lung diseases who are looking to establish their primary lung care through the center as well as those who are seeking a second opinion regarding their lung disease and/or those who are interested in participating in clinical research or clinical trials. […] We look forward to developing a strong relationship with patients and their care givers as we search for the cure for pulmonary fibrosis!

• #52 Cedars-Sinai Named to Pulmonary Fibrosis Foundation Care Center Network

  https://www.cedars-sinai.org/newsroom/cedars-sinai-named-to-pulmonary-fibrosis-foundation-care-center-network/

  The Pulmonary Fibrosis Foundation has named Cedars-Sinai part of its Care Center Network, a group of medical centers that have demonstrated expertise in treating and supporting people with this deadly disease. […] Our inclusion in this network is a recognition of our excellent pulmonary fibrosis clinical care, the availability of clinical and basic science research at our organization, and our focus on providing patients with education and support, said Tanzira Zaman, MD, medical director of the Interstitial Lung Disease Program at Cedars-Sinai. […] Each site provides educational events and support groups for patients, for example. […] Inclusion in the Care Center Network is a recognition of our efforts to translate research into patient care, said Paul Noble, MD, director of the Womens Guild Lung Institute and the Vera and Paul Guerin Family Distinguished Chair in Pulmonary Medicine at Cedars-Sinai.

• #53 Idiopathic Pulmonary Fibrosis (IPF)

  https://www.upmc.com/services/pulmonology/conditions/ipf

  Idiopathic pulmonary fibrosis (IPF) is a disease marked by progressive scarring of the lungs. […] The University of Pittsburgh Simmons Center for Interstitial Lung Disease at UPMC, was created in 2001 because of a generous gift from the Simmons family. The center is dedicated to providing the highest quality of health care, education, and support for people with interstitial lung diseases and their caregivers and loved ones. […] At the University of Pittsburgh Simmons Center for Interstitial Lung Disease at UPMC, a multidisciplinary team of experts offers people with IPF the full capabilities of UPMC. […] The Simmons Center’s multidisciplinary team includes: Pulmonary physicians who are experts in the management of IPF, Cardiologists who evaluate the involvement of the heart and pulmonary vessels, Rheumatologists who assess the immune system, Pathologists who evaluate the biopsies, Chest radiology experts who evaluate imaging results, Rehabilitation and quality-of-life experts who optimize supportive therapy and assess the psychological impact of IPF, Transplant surgeons, Leaders in clinical research who provide access to novel IPF therapies.

• #54 Cedars-Sinai Named to Pulmonary Fibrosis Foundation Care Center Network

  https://www.cedars-sinai.org/newsroom/cedars-sinai-named-to-pulmonary-fibrosis-foundation-care-center-network/

  The Pulmonary Fibrosis Foundation has named Cedars-Sinai part of its Care Center Network, a group of medical centers that have demonstrated expertise in treating and supporting people with this deadly disease. […] Our inclusion in this network is a recognition of our excellent pulmonary fibrosis clinical care, the availability of clinical and basic science research at our organization, and our focus on providing patients with education and support, said Tanzira Zaman, MD, medical director of the Interstitial Lung Disease Program at Cedars-Sinai. […] Each site provides educational events and support groups for patients, for example. […] Inclusion in the Care Center Network is a recognition of our efforts to translate research into patient care, said Paul Noble, MD, director of the Womens Guild Lung Institute and the Vera and Paul Guerin Family Distinguished Chair in Pulmonary Medicine at Cedars-Sinai.

• #55 Supporting ILD patients | PulmonaryFibrosis360.com

  https://www.pulmonaryfibrosis360.com/nurses/ILD-nurses-role/supporting-ILD-patients-and-caregivers

  Nurses have an important role in supporting patients and caregivers from initial ILD diagnosis, continued management and to the final stages of care. […] Nurses have a key role to play within a multidisciplinary care team in helping patients: Understand their disease and its treatment, Manage the adverse reactions of medication, Feel supported throughout the course of their disease. […] Nurses can provide some self-care tips for caregivers, as well as help them connect with their local carer support groups. These support groups can provide advice and help for the mental and practical challenges in providing care to a loved one. […] Caregivers have an important role in providing both physical and emotional support for loved ones living with chronic conditions, including fibrotic ILD. […] Family members and friends of those with fibrotic ILDs should seek tailored support to meet their physical, emotional and spiritual needs.

• #56 Care planning in idiopathic pulmonary fibrosis | Nursing Times

  https://www.nursingtimes.net/respiratory/care-planning-in-idiopathic-pulmonary-fibrosis-27-04-2015/

  Idiopathic pulmonary fibrosis is a progressive lung disorder requiring careful management in partnership with patients to slow its progress and optimise quality of life. […] Patients with idiopathic pulmonary fibrosis require accurate diagnosis and treatment to improve their quality of life and prognosis. […] ILD clinical nurse specialists have a valuable role in helping patients to understand their diagnosis and uncertain prognosis, and to offer information about investigations and management. A CNS should be available throughout the care pathway. […] It was agreed Mr Spencers disease-specific care would aim to optimise the management of his pulmonary fibrosis. […] The CNS is integral to the multidisciplinary team in the care of patients with IPF. […] The progressive nature of IPF means care should be shared between specialist and local services.

• #57 Care planning in idiopathic pulmonary fibrosis | Nursing Times

  https://www.nursingtimes.net/respiratory/care-planning-in-idiopathic-pulmonary-fibrosis-27-04-2015/

  Idiopathic pulmonary fibrosis is a progressive lung disorder requiring careful management in partnership with patients to slow its progress and optimise quality of life. […] Patients with idiopathic pulmonary fibrosis require accurate diagnosis and treatment to improve their quality of life and prognosis. […] ILD clinical nurse specialists have a valuable role in helping patients to understand their diagnosis and uncertain prognosis, and to offer information about investigations and management. A CNS should be available throughout the care pathway. […] It was agreed Mr Spencers disease-specific care would aim to optimise the management of his pulmonary fibrosis. […] The CNS is integral to the multidisciplinary team in the care of patients with IPF. […] The progressive nature of IPF means care should be shared between specialist and local services.

• #58

  https://www.nursingcenter.com/cearticle?an=00152193-202101000-00007&Journal_ID=54016&Issue_ID=5737721

  Idiopathic pulmonary fibrosis (IPF) is a restrictive lung disease in which the cause cannot be determined. This article discusses restrictive lung diseases that fall under the general category of interstitial lung disease (ILD) with a focus on IPF-a fatal disease characterized by progressive fibrosis and interstitial pneumonia, dyspnea, and decreasing pulmonary function. […] The intent of this article is to provide nurses with a better understanding of the overall disorder and care needed for people who have been diagnosed with a restrictive lung disease, particularly IPF. […] Management goals for patients with ILD and IPF include slowing the fibrotic process and relieving the patient’s dyspnea. […] Nursing care for patients like AC includes educating the patient and family about the disease process and recommending community resources for support. Education should include strategies for avoiding respiratory irritants such as dust, mold, and animal dander; performing optimal hand hygiene practices; and preventing respiratory infections by avoiding crowds and people who are sick. […] Palliative care should be considered early in the disease process. Discussing expectations and wishes for end-of-life care can help patients improve their mental outlook and decrease the stress on both patients and the families.

• #59 Palliative Care and Pulmonary Fibrosis | Get Palliative Care

  https://getpalliativecare.org/whatis/disease-types/pulmonary-fibrosis-and-palliative-care/

  Palliative care is specialized medical care for people with serious illness. […] The goal of palliative care is to improve quality of life for both the patient and the family. […] Palliative care is provided by a team of palliative care specialists including doctors, nurses and social workers. […] While living with pulmonary fibrosis can be a major challenge, palliative care can make a big difference. […] Some palliative care treatments for pulmonary fibrosis include medications that relieve symptoms such as shortness of breath. […] Palliative care specialists are experts in treating the symptoms of pulmonary fibrosis and the side effects of treatment. […] It is important to remember that your palliative care team is 100 percent there for you. […] If you or a loved one is facing pulmonary fibrosis, ask your doctor for a referral to palliative care—the earlier the better. […] Although living with pulmonary fibrosis is a difficult journey, palliative care can ease your burden and help you achieve the best possible quality of life.

• #60 Palliative Care and Pulmonary Fibrosis | Get Palliative Care

  https://getpalliativecare.org/whatis/disease-types/pulmonary-fibrosis-and-palliative-care/

  Palliative care is specialized medical care for people with serious illness. […] The goal of palliative care is to improve quality of life for both the patient and the family. […] Palliative care is provided by a team of palliative care specialists including doctors, nurses and social workers. […] While living with pulmonary fibrosis can be a major challenge, palliative care can make a big difference. […] Some palliative care treatments for pulmonary fibrosis include medications that relieve symptoms such as shortness of breath. […] Palliative care specialists are experts in treating the symptoms of pulmonary fibrosis and the side effects of treatment. […] It is important to remember that your palliative care team is 100 percent there for you. […] If you or a loved one is facing pulmonary fibrosis, ask your doctor for a referral to palliative care—the earlier the better. […] Although living with pulmonary fibrosis is a difficult journey, palliative care can ease your burden and help you achieve the best possible quality of life.

• #61 How to Support Your Loved One with Pulmonary Fibrosis | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/family-and-friends/how-to-support-your-loved-one

  Palliative care, sometimes known as supportive care, helps patients feel their best, physically and emotionally, during treatment. It’s often misunderstood as care only given at the end of life, but palliative care is recommended at the start of treatment. Help your loved one connect with palliative care so they can manage their side effects. […] It is important for the patient to think about what needs to happen so they can continue to get the care they need and want in the future and as their disease progresses. As a caregiver, you can help start these important conversations and assist with filling out paperwork, including an advance directive.

• #62 Palliative Care and Pulmonary Fibrosis | Get Palliative Care

  https://getpalliativecare.org/whatis/disease-types/pulmonary-fibrosis-and-palliative-care/

  Palliative care is specialized medical care for people with serious illness. […] The goal of palliative care is to improve quality of life for both the patient and the family. […] Palliative care is provided by a team of palliative care specialists including doctors, nurses and social workers. […] While living with pulmonary fibrosis can be a major challenge, palliative care can make a big difference. […] Some palliative care treatments for pulmonary fibrosis include medications that relieve symptoms such as shortness of breath. […] Palliative care specialists are experts in treating the symptoms of pulmonary fibrosis and the side effects of treatment. […] It is important to remember that your palliative care team is 100 percent there for you. […] If you or a loved one is facing pulmonary fibrosis, ask your doctor for a referral to palliative care—the earlier the better. […] Although living with pulmonary fibrosis is a difficult journey, palliative care can ease your burden and help you achieve the best possible quality of life.

• #63 Patient-centered care in pulmonary fibrosis: access, anticipate, and act | Respiratory Research | Full Text

  https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-024-02997-7

  Patient-centered care in patients with PF encompasses many facets, each with its own set of nuances and potential barriers to integration. […] Optimizing patient-centered care is not possible without understanding the needs of patients and their caregivers. Patient needs should be the central focus and anticipated at every stage of care. […] Palliative care aims to improve HRQOL for patients and their caregivers throughout the disease course, which is particularly relevant in PF due to the high disease burden and often progressive disease course. […] The principal symptoms of PF that profoundly impact patient HRQOL include dyspnea, cough, and fatigue. Managing these symptoms with currently available treatments remains challenging. […] Although many patients with IPF have a worse prognosis than those with lung cancer, end-of-life care is far less developed in this area, with less symptom relief achieved in IPF. An observational study in dying patients with ILD compared to patients dying with lung cancer showed that patients with ILD had lower quality of dying and death and a lower frequency of participation in end-of-life discussions. […] In conclusion, we believe that routinely addressing the domains of the Triple A Care model in clinical practice can improve the adoption of a more individually tailored approach to PF care.

• #64 Management of idiopathic pulmonary fibrosis | Nursing Times

  https://www.nursingtimes.net/respiratory/management-of-idiopathic-pulmonary-fibrosis-11-04-2014/

  Monitoring disease progression and withdrawing ineffective therapies that could cause harm or further impair the patients quality of life should be considered. […] Palliative care teams can advise on symptom management; referral should be considered for patients who are distressed by symptoms that are difficult to manage. […] Patients need support during treatment to manage common side-effects, which can include nausea, loss of appetite, weight loss, lethargy and photosensitivity skin rashes.

• #65 Palliative Care and Pulmonary Fibrosis | Get Palliative Care

  https://getpalliativecare.org/whatis/disease-types/pulmonary-fibrosis-and-palliative-care/

  Palliative care is specialized medical care for people with serious illness. […] The goal of palliative care is to improve quality of life for both the patient and the family. […] Palliative care is provided by a team of palliative care specialists including doctors, nurses and social workers. […] While living with pulmonary fibrosis can be a major challenge, palliative care can make a big difference. […] Some palliative care treatments for pulmonary fibrosis include medications that relieve symptoms such as shortness of breath. […] Palliative care specialists are experts in treating the symptoms of pulmonary fibrosis and the side effects of treatment. […] It is important to remember that your palliative care team is 100 percent there for you. […] If you or a loved one is facing pulmonary fibrosis, ask your doctor for a referral to palliative care—the earlier the better. […] Although living with pulmonary fibrosis is a difficult journey, palliative care can ease your burden and help you achieve the best possible quality of life.

• #66

  https://www.pulmonaryfibrosis.org/patients-caregivers/medical-and-support-resources/for-the-caregiver

  Learn about Advance Directives like Living Wills and Medical Power of Attorney, Physician Orders for Life-Sustaining Therapy (POLST), and palliative care options, long before they seem to be needed. […] Keep up with your own medical appointments. […] Find someone you can talk to when things are hard. You can also join a support group for caregivers.

• #67 Planning for Life with Pulmonary Fibrosis | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/planning-for-life-with-pulmonary-fibrosis

  A social worker or someone on your palliative care team can help you work through the paperwork and navigate end-of-life decision making. […] Hospice care provides medical care, emotional support and spiritual resources for patients who are in the end stages of their illness and also offers support and resources for families or caregivers. Most hospice care is given at home, but it also can be given in the hospital or a hospice care facility. […] Palliative care is always part of hospice but palliative care can be given on its own, and not only at the end of life. It can help you cope with side effects and maintain a good quality of life, all throughout your illness.

• #68 Planning for Life with Pulmonary Fibrosis | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/planning-for-life-with-pulmonary-fibrosis

  A social worker or someone on your palliative care team can help you work through the paperwork and navigate end-of-life decision making. […] Hospice care provides medical care, emotional support and spiritual resources for patients who are in the end stages of their illness and also offers support and resources for families or caregivers. Most hospice care is given at home, but it also can be given in the hospital or a hospice care facility. […] Palliative care is always part of hospice but palliative care can be given on its own, and not only at the end of life. It can help you cope with side effects and maintain a good quality of life, all throughout your illness.

• #69 Hospice Care for Advanced Pulmonary Fibrosis | Hospice

  https://www.compassus.com/for-caregivers/how-a-doctor-chose-his-own-path-to-hospice-care/hospice-for-advanced-pulmonary-fibrosis/

  We understand the hardships that often come with advanced pulmonary fibrosis. Breathing problems, coughing fits, and fatigue are a heavy burden for patients and their caregivers. […] Hospice care for advanced pulmonary fibrosis covers a broad range of services, typically with no out-of-pocket expenses. The hospice circle of care includes: […] 24/7 access to hospice nurses […] Education and support for family members […] Managing anxiety […] Care at home, in nursing facilities, or wherever someone calls home. […] The best hospice care for advanced pulmonary fibrosis experience begins with early and honest conversations. […] Hospice eligibility requires a patients doctor and a hospice medical director to determine a life expectancy of six months or less if the disease follows a normal course.

• #70 Comprehensive care of the patient with idiopathic pulmonary fibrosis – PubMed

  https://pubmed.ncbi.nlm.nih.gov/21760508/

  The comprehensive care of the patient with IPF involves balancing the three pillars of disease-centered management, symptom-centered management, and patient education and self-management upon a solid foundation of provider-patient partnership. Constant reassessment of the individual patient’s goals of care, based on their values and preferences, is essential to the constant recalibration of these various interventions.

• #71 Idiopathic pulmonary fibrosis: What primary care physicians need to know | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/85/5/377

  Accurate diagnosis of IPF is crucial. We recommend early referral to a center specializing in interstitial lung disease to confirm the diagnosis, start appropriate therapy, advise the patient on prognosis and enrollment in disease registries and clinical trials, and determine candidacy for lung transplant. […] The goal of this article is to delineate the features of IPF so that it may be recognized early and thus expedite referral to a center with expertise in interstitial lung disease for a thorough evaluation and appropriate management. […] Antifibrotic therapy is a choice between pirfenidone and nintedanib. […] Patients need to understand that these drugs slow the rate of decline in FVC but have not been shown to improve symptoms or functional status. […] Corticosteroids should not be used routinely in the treatment of IPF. […] Their use in IPF is discouraged, and the joint international guidelines recommend against immunosuppression to treat IPF. […] Prompt referral for lung transplant is imperative. IPF is now the most common indication for lung transplant, and given the poor overall prognosis of advanced IPF, transplant confers a survival benefit in appropriately selected patients.

• #72

  https://journals.lww.com/tnpj/fulltext/2018/05000/idiopathic_pulmonary_fibrosis__a_guide_for_nurse.9.aspx

  The course of IPF is variable and unpredictable. Patients with IPF should be followed up with at regular intervals to assess disease progression, ensure that symptoms and comorbidities are being treated optimally, and to provide emotional support and advice in managing adverse events associated with antifibrotic medications. Regular review of pulmonary function is the practical approach to monitoring disease progression. […] Care for patients with IPF should focus on maximizing length and quality of life. The development of antifibrotic therapies was a major step forward in treating IPF. Starting either nintedanib or pirfenidone as soon as a diagnosis of IPF is made should be considered for all patients, since these drugs slow the rate of loss of lung function. Treatment of comorbidities, symptom management, and integrating ongoing education into patient care are all crucial in improving a patient’s quality of life.

• #73 Idiopathic pulmonary fibrosis in adults: diagnosis and management – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK553262/

  Offer best supportive care to people with idiopathic pulmonary fibrosis from the point of diagnosis. Best supportive care should be tailored to disease severity, rate of progression, and the persons preference, and should include if appropriate: information and support (see recommendation 1.3.1), symptom relief, management of comorbidities, withdrawal of therapies suspected to be ineffective or causing harm, end of life care. […] In follow-up appointments for people with idiopathic pulmonary fibrosis: assess lung function, assess for oxygen therapy, assess for pulmonary rehabilitation, offer smoking cessation advice, in line with Smoking cessation services (NICE public health guidance 10), identify exacerbations and previous respiratory hospital admissions, consider referral for assessment for lung transplantation in people who do not have absolute contraindications, consider psychosocial needs and referral to relevant services as appropriate, consider referral to palliative care services, assess for comorbidities (which may include anxiety, bronchiectasis, depression, diabetes, dyspepsia, ischaemic heart disease, lung cancer and pulmonary hypertension).

• #74 Pulmonary Fibrosis | Louisville KY | UofL Health

  https://uoflhealth.org/services/pulmonary-fibrosis/

  The UofL Physicians Pulmonary Fibrosis Care Center is one of nine pilot sites selected by the Pulmonary Fibrosis Foundation (PFF) for its newly-established Care Center Network and Patient Registry program. […] Aimed at improving the health and quality of life of patients suffering from pulmonary fibrosis, the network and registry will help provide critical insights enabling the medical research community to develop more effective therapies. […] To make progress there is a need for a multidisciplinary approach by teams of expert medical professionals, more data and to track the natural history of the disease. […] The PFF Care Center Network and Patient Registry and the UofL Physicians Pulmonary Fibrosis Care Center will provide these critical cornerstones for improved patient care and progress toward a cure.

• #75 Pulmonary Fibrosis | Louisville KY | UofL Health

  https://uoflhealth.org/services/pulmonary-fibrosis/

  The benefit of the UofL Physicians Pulmonary Fibrosis Care Center to patients is that they can contribute to the effort and partner with us in identifying new areas for intervention, becoming an integral part of their care. […] As part of the Pulmonary Fibrosis Foundation Care Center Network, the UofL Physicians Pulmonary Fibrosis Care Center provides a standardized, multidisciplinary approach to patient care. […] This model of comprehensive patient care will help identify and establish best practices, determine the impact of specific interventions and improve the quality of life of patients. […] The UofL Physicians Pulmonary Fibrosis Care Center, and all the pilot sites of the Care Center Network, participate in the Patient Registry.

• #76 Pulmonary Fibrosis | Louisville KY | UofL Health

  https://uoflhealth.org/services/pulmonary-fibrosis/

  The benefit of the UofL Physicians Pulmonary Fibrosis Care Center to patients is that they can contribute to the effort and partner with us in identifying new areas for intervention, becoming an integral part of their care. […] As part of the Pulmonary Fibrosis Foundation Care Center Network, the UofL Physicians Pulmonary Fibrosis Care Center provides a standardized, multidisciplinary approach to patient care. […] This model of comprehensive patient care will help identify and establish best practices, determine the impact of specific interventions and improve the quality of life of patients. […] The UofL Physicians Pulmonary Fibrosis Care Center, and all the pilot sites of the Care Center Network, participate in the Patient Registry.

• #77 British Journal of Nursing – Idiopathic pulmonary fibrosis: a more common condition than you may think

  https://www.britishjournalofnursing.com/content/professional/idiopathic-pulmonary-fibrosis-a-more-common-condition-than-you-may-think/

  Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive incurable lung disease that affects a significant amount of people in the UK. […] Many health professionals have a limited understanding of IPF, which can result in a delayed diagnosis and inadequate care for individuals and their families. […] This article aims to provide an overview of IPF and help to enhance health professionals’ understanding of the disease, thus contributing towards improving the care that IPF sufferers receive. […] This article is timely as a reminder to nurses of the condition and to help consider the specific care and support that this patient group needs to be offered.

• #78 British Journal of Nursing – Idiopathic pulmonary fibrosis: a more common condition than you may think

  https://www.britishjournalofnursing.com/content/professional/idiopathic-pulmonary-fibrosis-a-more-common-condition-than-you-may-think/

  Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive incurable lung disease that affects a significant amount of people in the UK. […] Many health professionals have a limited understanding of IPF, which can result in a delayed diagnosis and inadequate care for individuals and their families. […] This article aims to provide an overview of IPF and help to enhance health professionals’ understanding of the disease, thus contributing towards improving the care that IPF sufferers receive. […] This article is timely as a reminder to nurses of the condition and to help consider the specific care and support that this patient group needs to be offered.

• #79 British Journal of Nursing – Idiopathic pulmonary fibrosis: a more common condition than you may think

  https://www.britishjournalofnursing.com/content/professional/idiopathic-pulmonary-fibrosis-a-more-common-condition-than-you-may-think/

  Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive incurable lung disease that affects a significant amount of people in the UK. […] Many health professionals have a limited understanding of IPF, which can result in a delayed diagnosis and inadequate care for individuals and their families. […] This article aims to provide an overview of IPF and help to enhance health professionals’ understanding of the disease, thus contributing towards improving the care that IPF sufferers receive. […] This article is timely as a reminder to nurses of the condition and to help consider the specific care and support that this patient group needs to be offered.

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