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Chronic Care Programme Treatment guidelines Bronchiectasis n/a Chronic condition Alternative names Consultations protocols Preferred treating provider Notes preferred as indicated by option referral protocols apply Maximum consultations per annum Initial consultation Follow-up consultation Tariff codes Physiotherapy: Treating doctor: complex evaluation / Physiotherapist counselling at first visit only Initial consultation Follow-up consultation Physiotherapy: Treating doctor: one complete reassessment of Physiotherapist patient’s condition during the course of treatment Initial consultation Option/plan Provider GMHPP Gold Options G1000, G500 and G200. Blue Options B300 and B200. GMISHPP General Practitioner Physician Paediatrician Cardiologist Pulmonologist Surgeon Thoracic Surgeon New Patient Existing patient Mild / Stable Moderate to Severe / Unstable 1 3 0183; 0142; 0187; 0108 1 1 New Patient Existing patient Mild / Stable Moderate to Severe / Unstable 2 2 New Patient Follow-up consultation 1 3 2 Existing patient 4 Mild / Stable Moderate to Severe / Unstable 2 4 Investigations protocols Type Provider Tariff code Physiotherapy – postural draininage Flow volume test: Inspiration / expiration Peak expiratory flow only Maximum investigations per annum New patient Existing patient Stable Unstable Controlled Uncontrolled 6 4 6 Physiotherapist 0315 Any preferred provider Any preferred 1186 2 1 2 1192 2 1 2 Nebulizations in rooms Sputum microscopy Sputum bacteriological culture Sputum fungal culture Sputum mycobacterium Antibiotic susceptibility test: per organism Blood gases: (Panel 1: Astrup/p02. This panel includes items 4077, 4078 & 4021) CXR ICD 10 coding provider Any preferred provider Any preferred provider Pathologist 1136 1 1 1 3867 2 1 2 3895 2 1 2 Pathologist Pathologist Pathologist 3901 3915 3887 2 2 6 1 1 3 2 2 6 Pathologist or as per treating doctor 4075 1 0 1 3445 2 1 2 Radiologist J47., Q33.4 General Bronchiectasis is a disease that causes localized, irreversible dilatation of part of the bronchial tree. Involved bronchi are dilated, inflamed, and easily collapsible, resulting in airflow obstruction and impaired clearance of secretions. Bronchiectasis is associated with a wide range of disorders, but it usually results from necrotizing bacterial infections, such as infections caused by the Staphylococcus or Klebsiella species or Bordetella pertussis.[1] Rene Theophile Hyacinthe Laënnec, the man who invented the stethoscope, used his creation to first discover bronchiectasis in 1819.[2]. The disease was researched in greater detail by Sir William Osler in the late 1800s; in fact, it is suspected that Osler actually died of complications from undiagnosed bronchiectasis[3]. Bronchiectasis is a condition in which an area of the bronchial tubes is permanently and abnormally widened (dilated), with accompanying infection. Description. The bronchial tubes are the networks of branching tubes which deliver air to the tiny sacs of the lungs (alveoli). In bronchiectasis, the diameter of the bronchi is unusually large. Examination of the walls of the bronchial tubes reveals destruction of the normal structural elements, with replacement by scar tissue. Pus collects within the bronchi, and the normal flow of oxygen into the lungs, and carbon dioxide out of the lungs (air exchange) is impaired. The bronchi show signs of inflammation, with swelling and invasion by a variety of immune cells. The inflamed areas show signs of increased growth of blood vessels. The area of the lung which should be served by a diseased bronchial tube is also prone to inflammation and infection. Causes There are both congenital and acquired causes of bronchiectasis. Kartagener syndrome, which affects the mobility of cilia in the lungs[7], aids in the development of the disease. Another common genetic cause is cystic fibrosis, in which a small number of patients develop severe localized bronchiectasis[8]. Young's syndrome, which is clinically similar to cystic fibrosis, is thought to significantly contribute to the development of bronchiectasis. This is due to the occurrence of chronic, sinopulmonary infections.[9] Patients with alpha 1-antitrypsin deficiency have been found to be particularly susceptible to bronchiectasis, for unknown reasons. [10] Other less-common congenital causes include primary immunodeficiencies, due to the weakened or nonexistent immune system response to severe, recurrent infections that commonly affect the lung.[11] Acquired bronchiectasis occurs more frequently, with one of the biggest causes being tuberculosis. Endobronchial tuberculosis commonly leads to bronchiectasis, either from bronchial stenosis or secondary traction from fibrosis.[12] An especially common cause of the disease in children is acquired immune deficiency syndrome, stemming from the human immunodeficiency virus. This disease predisposes patients to a variety of pulmonary ailments, such as pneumonia and other opportunistic infection.[13]. Bronchiectasis can sometimes be an unusual complication of inflammatory bowel disease, especially ulcerative colitis. It can occur in Crohn's disease as well, but does so less frequently. Bronchiectasis in this situation usually stems from various allergic responses to inhaled fungus spores.[14] Recent evidence has shown an increased risk of bronchiectasis in patients with rheumatoid arthritis who smoke. One study stated a tenfold increased prevalence of the disease in this cohort[15]. Still, it is unclear as to whether or not cigarette smoke is a specific primary cause of bronchiectasis. Other acquired causes of bronchiectasis involving environmental exposures include respiratory infections, obstructions, inhalation and aspiration of ammonia and other toxic gases, pulmonary aspiration, alcoholism, heroin (drug use), and various allergies.[16] Prior to the widespread use of immunizations, bronchiectasis was often the result of a serious infection with either measles or whooping cough. Currently, viruses that cause influenza (flu) or influenza-like syndromes, as well as a number of bacteria may precede the development of bronchiectasis. Patients who have been infected with tuberculosis or the virus which causes AIDS (HIV or human immunodeficiency virus) also have an increased chance of bronchiectasis. A number of pre-existing conditions may cause an individual to be more susceptible than normal to infection, with increased risk of bronchiectasis developing. These conditions include disorders of cilia, and immune disorders. Cilia are the tiny hairs which usually line the bronchial tubes. Cilia wave constantly, sweeping the bronchial tubes clean of bacterial or viral invaders, and cleaning away excess secretions (mucus, sputum) which may be produced by the bronchi. When these cilia are abnormal or absent at birth, various bacterial or viral invaders may remain in the respiratory tract, multiply, and cause serious infections. Immune disorders include decreased production of certain immune chemicals (immunoglobulins) which usually serve to fight off infection by bacterial or viral invasion. When these immunoglobulins are not produced in large enough quantity, bacterial and viral invaders are not effectively killed off, and infection occurs. Other causes of bronchiectasis include an abnormally blocked (obstructed) airway. This can be due to tumor growth within the bronchial tube, or due to a child accidentally inhaling a small object which then blocks off the bronchial tube. People with the disease called cystic fibrosis (CF) often have their bronchial tubes obstructed by the thick, sticky mucus which is a hallmark of CF. Toxic exposures (breathing ammonia, for example) can harm the bronchi, and lead to bronchiectasis. An extreme allergic response of the immune system to the presence of certain fungi (especially one called Aspergillus) can also damage the bronchial tubes enough to result in bronchiectasis. Signs and symptoms Pathogenesis Dilation of the bronchial walls results in airflow obstruction and impaired clearance of secretions because the dilated areas disrupt normal air pressure in the bronchial tubes, causing sputum to pool inside the dilated areas instead of being pushed upward[4]. The pooled sputum provides an environment conducive to the growth of infectious pathogens, and these areas of the lungs are thus very vulnerable to infection. The more infections that the lungs experience, the more damaged the lung tissue and alveoli become. When this happens, the bronchial tubes become more inelastic and dilated, creating a self-perpetuating cycle of further damage to the lungs. There are three types of brochiectasis, varying by level of severity. Fusiform (cylindrical) bronchiectasis (the most common type) refers to mildly inflamed bronchi that fail to taper distally. In varicose bronchiectasis, the bronchial walls appear beaded, because areas of dilation are mixed with areas of constriction. Saccular (cystic) bronchiectasis is characterized by severe and irreversible ballooning of the bronchi peripherally, with or without air-fluid levels.[5] Chronic productive cough is prominent, occurring in up to 90% of patients with bronchiectasis. Sputum is produced on a daily basis in 76% of patients.[6] Clinical Signs and Symptoms of bronchiectasis include constant cough and the production of infected sputum (sputum is a mixture of mucus and pus), which may be bloody. In some cases, there may be wheezing and shortness of breath. The constant, lowlevel of infection may flare, resulting in increased production of sputum, worsening of the cough, and fever. The area of the lung served by the affected bronchial tube may become severely infected, resulting in pneumonia. Diagnosis The diagnosis of bronchiectasis is based on the review of clinical history and characteristic patterns in high-resolution CT scan findings. Such patterns include "tree-in-bud" abnormalities and cysts with definable borders. In one small study, CT findings of bronchiectasis and multiple small nodules were reported to have a sensitivity of 80%, specificity of 87%, and accuracy of 80% for the detection of bronchiectasis. Bronchiectasis may also be diagnosed without CT scan confirmation if clinical history clearly demonstrates frequent, respiratory infections, as well confirmation of an underlying problem via blood work and sputum culture samples.[17] Chest x ray may reveal evidence of bronchiectasis, and CT scans are particularly good at revealing the thick, dilated bronchial walls of bronchiectasis. Sputum will need to be collected and cultured (grown in a laboratory dish), in order to examine it microscopically for the specific type of organism responsible for infection. A careful search for other underlying diseases is important, looking in particular for ciliary abnormalities, cystic fibrosis, or immunoglobulin deficiencies. Treatment Treatment of bronchiectasis is aimed at controlling infections and bronchial secretions, relieving airway obstruction, and preventing complications. This includes the prolonged usage of antibiotics to prevent detrimental infections[18], as well as eliminating accumulated fluid with postural drainage and chest physiotherapy. Surgery may also be used to treat localized bronchiectasis, removing obstructions that could cause progression of the disease.[19] Inhaled steroid therapy that is consistently adhered to can reduce sputum production and decrease airway constriction over a period of time, and help prevent progression of bronchiectasis. One commonly used therapy is beclometasone dipropionate, which also used in asthma treatment.[20] Use of inhalers such as albuterol (salbutamol), fluticasone (Flovent/Flixotide) and ipratropium (Atrovent) may help reduce likelihood of infection by clearing the airways and decreasing inflammation.[21] Mannitol dry inhalation powder, under the name Bronchitol, has been approved by the FDA for use in cystic fibrosis patients with or at risk for bronchiectasis. The original orphan drug indication approved in February 2005 allowed its use for the treatment of bronchiectasis. The original approval was based on the results of Phase II clinical studies showing the product to be safe, well-tolerated, and effective for stimulating mucus hydration/clearance, thereby improving quality of life in patients with chronic obstructive lung diseases like bronchiectasis. Long-term studies are underway as of 2007 to ensure the safety and effectiveness of the treatment.[22] Advair Diskus is also a commonly used inhaled corticosteroid which has in many cases been effective in clearing the airways, reducing sputum and reducing inflammation. Treatment should involve efforts to resolve any underlying disorder. Infections will require antibiotics, obstruction may require the removal of a foreign object or tumor. Medications are available to help thin the sputum, so that it can be more effectively coughed up. Rhythmic clapping on the chest and back, while the patient assumes a number of positions (head down, primarily), may help the lungs to drain more effectively. This is called chest physical therapy, or percussion and postural drainage. In some patients, bronchiectasis eventually leads to a constantly low level of blood oxygen, despite other treatments. These patients usually have an associated increase in the size of the right side of their hearts, along with a decrease in the heart’s ability to pump blood through the lungs. Some patients with extremely severe symptoms and disability have been treated with lung transplantation. Surgery When a particular area of the lung is constantly and severely infected, surgery may be needed to remove it. When bleeding occurs from irritated bronchial tubes and overgrown bronchial blood vessels, surgery may be required either to remove an area of the bronchial tube, or to inject the bleeding blood vessel with a material to stop the bleeding. Medicine formularies Plan or option GMHPP Gold Options G1000, G500 and G200 Blue Options B300 and B200 GMISHPP Link to appropriate Mediscor formulary [Core] Blue Option B100 n/a Epidemiology Prognosis Prognosis varies widely, depending on how widespread or focal the bronchiectasis, and the presence of other underlying disorders. References 1. Hassan, Isaac (December 8, 2006). "Bronchiectasis". eMedicine Specialties Encyclopedia. Gibraltar: WebMD. Retrieved on 2007-06-22. 2. Roguin, A (2006). "Rene Theophile Hyacinthe Laënnec (1781–1826): The Man Behind the Stethoscope" (in English). Clin Med Res 4 (3): 230-35. 3. Wrong O (2003). "Osler and my father" (in English). J R Soc Med 96 (6): 462-64. doi:10.1258/jrsm.96.9.462. 4. Morrissey BM (2007). "Pathogenesis of bronchiectasis" (in English). Clin Chest Med 28 (2): 289-96. doi:10.1016/j.ccm.2007.02.014. PMID 17467548. 5. Mysliwiec, V, Pina, JS (1999). "Bronchiectasis: the 'other' obstructive lung disease" (in English). POSTGRADUATE MEDICINE 106 (1): 252-63. 6. Emmons, Ethan (January 31, 2007). "Bronchiectasis". eMedicine Specialties Encyclopedia. San Antonio, TX: WebMD. Retrieved on 2007-06-22. 7. Morillas HN, Zariwala M, Knowles MR (2007). "Genetic Causes of Bronchiectasis: Primary Ciliary Dyskinesia" (in English). Respiration 72 (3): 252-63. PMID 17534128. 8. Dalrymple-Hay MJ, Lucas J, Connett G, Lea RE (1999). "Lung resection for the treatment of severe localized bronchiectasis in cystic fibrosis patients." (in English). Acta Chir Hung. 38 (1): 23-5. PMID 10439089. 9. Handelsman DJ, Conway AJ, Boylan LM, & Turtle JR (1984). "Young's syndrome. Obstructive azoospermia and chronic sinopulmonary infections." (in English). NEJM 310 (1): 3-9. 10. Shin MS, Ho KJ (1993). "Bronchiectasis in patients with alpha 1-antitrypsin deficiency. A rare occurrence?." (in English). Chest 104: 1384-86. doi:10.1378/chest.104.5.1384. 11. Notarangelo LD, Plebani A, Mazzolari E, Soresina A, Bondioni MP (2007). "Genetic causes of bronchiectasis: primary immune deficiencies and the lung" (in English). Respiration 74 (3): 264-75. doi:10.1159/000101784. PMID 17534129. 12. Catanzano, Tara (September 5, 2005). "Primary Tuberculosis". eMedicine Specialties Encyclopedia. Connecticut: WebMD. Retrieved on 2007-06-22. 13. Sheikh S, Madiraju K, Steiner P, Rao M (1997). "Bronchiectasis in pediatric AIDS." (in English). Chest 112 (5): 1202-7. doi:10.1378/chest.112.5.1202. PMID 9367458. 14. Ferguson HR, Convery RP (2002). "An unusual complication of ulcerative colitis" (in English). Postgrad. Med. J. 78: 503. doi:10.1136/pmj.78.922.503. 15. Kaushik, VV, Hutchinson D, Desmond J, Lynch MP, and Dawson JK (2004). "Association between bronchiectasis and smoking in patients with rheumatoid arthritis." (in English). Annals of the Rheumatic Diseases 63: 1001-2. doi:10.1136/ard.2003.015123. 16. Lamari NM, Martins ALQ, Oliveira JV, Marino LC, Valério N (2006). "Bronchiectasis and clearence physiotherapy: emphasis in postural drainage and percussion." (in Portuguese). Braz. j. cardiovasc. surg. 21 (2). 17. Miller, JC (2006). "Pulmonary Mycobacterium Avium-Intracellular Infections in Women" (in English). Radiology Rounds 4 (2). 18. Evans DJ, Bara AI,Greenstone M (2007). "Prolonged antibiotics for purulent bronchiectasis in children and adults" (in English). The Cochrane Database of Systematic Reviews (2). doi:10.1002/14651858.CD001392.pub2. 19. Ötgün B, Karnak B, Tanyel K, Enocak M, Büyükpamukçu N (2003). "Surgical treatment of bronchiectasis in children." (in English). Journal of Pediatric Surgery 39 (10): 153236. 20. Elborn JS, Johnston B, Allen F, Clarke J, McGarry J, Varghese G. (1992). "Inhaled steroids in patients with bronchiectasis" (in English). Respir Med 86 (2): 121-4. doi:10.1016/S0954-6111(06)80227-1. PMID 1615177. 21. Reports, Consumer (2007-03-15). Ipratropium and Albuterol Inhalation - Drug Review. Consumer Reports of U.S.. Retrieved on 2007-06-22. 22. Waknine, Yael (2005-07-27). Orphan Drug Approvals: Bronchitol, Prestara, GTI-2040. Medscape today for WebMD. Retrieved on 2007-06-22. 23. Crofton J (1966). "Diagnosis and Treatment of Bronchiectasis: II. Treatment and Prevention." (in English). Br Med J 1 (5490): 783-785. 24. Onen ZP, Eris Gulbay B, Sen E, Akkoca Yildiz O, Saryal S, Acican T, Karabiyikoglu G (2007). "Analysis of the factors related to mortality in patients with bronchiectasis." (in English). Respir Med. 101 (7): 1390-97. doi:10.1016/j.rmed.2007.02.002. PMID 17374480