Pulmonary Hypertension

“I urge you to get involved with the ORCS by joining one of our various committees or through attending educational events." Lisa C. Cicutto, RN, PhD ...
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“I urge you to get involved with the ORCS by joining one of our various committees or through attending educational events." Lisa C. Cicutto, RN, PhD Chair, Ontario Respiratory Care Society

S P R I N G / S U M M E R

Features In this Issue Better Breathing 2005 and 2006. . . . . . . . . . . . 3 In the Spotlight Congratulations to Reny Vaughan and Carla Loftus, winners of the poster awards at Better Breathing 2005 . . . . 3 Beyond Words: the health-literacy connection Suggestions for health practitioners. . . . . . . . . . . . . . 8 Respiratory Care Perspectives A Breath of Northern Air from a Respiratory Therapist in Northern Ontario. . . . . . . . . . 9 Teen Asthma Website Launched May 3rd. . . . . . . . 11

Regular Columns Chair’s Message. . . . . . . . . . . 2 Editor’s Comment . . . . . . . . . 2 Coming Events. . . . . . . . . . . . 3 Respiratory Articles of Interest 10

Coming Up Fall 2005 Does oxygen help people who desaturate during exertion?

www.on.lung.ca

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Pulmonary Hypertension By John Granton, MD, FRCPC, Associate Professor of Medicine, University of Toronto; Pulmonary and Critical Care, University Health Network, Toronto Introduction understanding of the pathogenesis of In health, the pulmonary circulation is iPAH have translated into a variety of a high capacitance, low resistance novel therapies and treatment strategies.1,2 circuit that can accommodate large This in turn has translated into an increases in blood flow (cardiac improvement in both patients’ sense of output) without a significant change in well-being and survival. This review pulmonary pressure. In patients with will highlight these advances and pulmonary hypertension (PH), there is hopefully provide the practitioner with a progressive reduction in the cross a rational approach to evaluation of DR. JOHN GRANTON sectional area of the pulmonary patients with pulmonary hypertension. vasculature and a reduction in pulmonary vascular capacitance. The effective reduction in Diagnosis cross sectional area leads to an increase in Does this patient have an elevation pulmonary vascular resistance and, in turn, a in pulmonary pressures? reduction in cardiac output. The net result is a Pulmonary hypertension is characterized by an progressive worsening in right ventricular elevation in mean pulmonary arterial pressure performance, right ventricular dilation (RV), > 25 mmHg at rest or > 30 mmHg with exercise. and eventually right ventricular failure. However, the measurement of mean pressure Symptomatically, patients present early in the requires cardiac catheterization. Clinically, course of the disease with mild to moderate patients may have evidence of increased RV exercise intolerance owing to an inability of the volume or pressure including: right ventricular pulmonary vasculature to accommodate to the lift, loud second heart sound, presence of right need for an increased pulmonary blood flow. As sided S3 and S4, murmurs associated with the disease progresses, patients develop tricuspid and pulmonary valvular insufficiency, progressive exercise intolerance, syncope, and and elevation in the jugular venous pressure. signs of right heart failure (e.g., leg oedema, liver With more advanced disease, patients may also enlargement and ascities). have liver enlargement, ascities, and leg oedema. PH may occur in isolation (so-called In a symptomatic patient, the specter of PH is idiopathic pulmonary arterial hypertension typically raised after echocardiography iPAH) or it may complicate a variety of systemic demonstrates an elevation in pulmonary disorders (e.g., scleroderma, lupus) or cardio- pressures. Utilizing the velocity of regurgitation pulmonary diseases (e.g., congenital heart disease, through the tricuspid valve, the peak pulmonary COPD). Despite differences in etiology however, systolic arterial pressure (PSAP) may be therapies for iPAH are often effective in other measured. It should be noted however that there Continued on page 4 forms of PH. Indeed recent advances in the

UPDATE An official publication of the Ontario Respiratory Care Society, a section of The Lung Association 573 King Street East, Suite 201 Toronto, Ontario M5A 4L3 (416) 864-9911 Fax (416) 864-9916 E-mail: [email protected] Internet: http://www.on.lung.ca CO-EDITORS Dina Brooks, Ph.D., M.Sc., B.Sc.P.T. Libby Groff, RRCP, RRT, B.H.A. CHAIR, ONTARIO RESPIRATORY CARE SOCIETY Lisa C. Cicutto, RN, Ph.D., ACNP, C.A.E. CHAIRMAN, ONTARIO LUNG ASSOCIATION Robert Kelly PRESIDENT & CEO, ONTARIO LUNG ASSOCIATION Manu Malkani DIRECTOR OF ADMINISTRATION, ONTARIO RESPIRATORY CARE SOCIETY Sheila Gordon-Dillane EDITORIAL BOARD Therese Hawn, B.Sc.P.T. Lawrence Jackson, B.Sc.Phm. Elizabeth McLaney, BA, B.H.Sc.(O.T.) David Nicholas, Ph.D., MSW, RSW Mika Nonoyama, RRCP, RRT, B.Sc., M.Sc.(c) Dale Stedman, RN Reny Vaughan, RRCP, RRT Opinions expressed in Update do not necessarily represent the views of The Lung Association nor does publication of advertisements constitute official endorsement of products and services. The printing of this publication is sponsored by a generous unrestricted educational grant from GlaxoSmithKline.

ONTARIO RESPIRATORY CARE SOCIETY Vision Improved lung health through excellence in interdisciplinary respiratory care.

Mission Furthering excellence in the provision of interdisciplinary respiratory care through education, research, collaboration, provision of professional expertise and support for Lung Association efforts to improve lung health. 2

CHAIR’S MESSAGE his issue of Update marks my first message to you as Chair of the ORCS. Wow, what an exciting time for me. I would like to thank you for allowing me this wonderful opportunity! I would very much like to thank Lawrence Jackson for his tremendous efforts and leadership over the years. I am delighted that he will continue his involvement on the Provincial Committee as Past Chair. It is a time of great change at The Lung Association. Due in part to financial pressures, the organization has been restructured. Several community offices have been amalgamated. However, the commitment of the The Lung Association and the ORCS to be active in all communities across Ontario has not changed. The ORCS will continue to hold exceptional educational events across the regions of Ontario. Active involvement and networking of respiratory professionals at both the local and provincial levels continues to be extremely important. As outlined in the last Chair’s message, one of the ORCS’s priorities identified in the 3-year

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strategic plan is to strengthen our membership of both new and renewing members. The Provincial Committee is very pleased to welcome a new regional representative for the Essex/Kent Region. Carol Stallaert, a Respiratory Therapist who works in Chatham and London, has accepted this appointment for a two year term. We look forward to working with her. Thank you to the former regional representative, Michelle Girard, who has now moved to Toronto and has joined the Greater Toronto Region Executive Committee. It seems that spring is here and we are starting to come out of hibernation. With your newfound energy, I urge you to get involved with the ORCS by joining one of our various committees or through attending educational events. Don’t forget to bring your colleagues and students along. For additional information, feel free to contact Sheila Gordon-Dillane ([email protected]) or myself ([email protected]). LISA CICUTTO, CHAIR, ORCS

EDITOR’S COMMENT pring is a season of energy, growth and new beginning, especially after what seemed like a never-ending winter. I hope you take this issue of Update with you outside as you relax and enjoy the warmth of the summer. This edition includes a lead article on Pulmonary Hypertension by Dr. John Granton. We are also featuring a new column, Perspectives on Respiratory Care. In the first such column, Monique Ouellette, a respiratory therapist who works in the North reflects on her experiences and provides insights into the unique aspects of her role. We have also included an article on Health Literacy from the Canadian Health Network. As always, we have our regular columns on upcoming conferences and courses and summaries of articles of interest. The winners of the poster awards at Better Breathing 2005 are In the Spotlight. Congratulations to Carla Loftus and Reny Vaughan. The Editorial Board is delighted to welcome a new member, Elizabeth McLaney, an Occupational Therapist from St. Joseph's Health Centre Toronto. We would like to thank Janice

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Bissonnette, Kathleen Schlifer and Rachel Stack, who have left the Board, for their many contributions. Members from Southwestern, Eastern and Northern Ontario who would like to join the Board should contact the ORCS office. We are indebted to GlaxoSmithKline for sponsoring this edition of Update. As always, your input is important to us and we welcome any letters to the Editors. Contact us at [email protected]. Happy reading, DINA BROOKS, CO-EDITOR

Become an ORCS member or renew your membership for 2005-2006 Individual $40; Student $25; RHEIG add $15 Call (416)864-9911 for information or visit www.on.lung.ca/orcs

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Better Breathing 2005 etter Breathing 2005 was held at The Doubletree® International Plaza Hotel from February 3-5, 2005. About 140 people attended the ORCS sessions, which included interesting and informative lectures and workshops on topics such as knowledge transfer, lung transplantation, mechanical ventilation, COPD self-management and making the transition from ICU to rehabilitation. The ORCS Annual General Meeting was held on Friday, February 4, 2005. The Chair, Larry Jackson, presented a summary of the year’s activities and an introduction to the new Three Year Plan being developed by the Provincial Committee. Lisa Cicutto, RN, PhD, ACNP, CAE, assumed the office of Chair for a two year term beginning April 1, 2005. Larry Jackson, B.Sc.Phm., will become Past Chair on that date.

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He was presented with a gift in appreciation of his contributions to the work of the ORCS during his two year term as Chair. Bonnie Solmes, a former Chair and Social Worker at West Park Healthcare Centre, was presented with The Lung Association’s Meritorious Service Award for extraordinary volunteer service. Thirteen posters were submitted to the annual poster competition. Congratulations to the two winners of the competition, Reny Vaughan and Carla Loftus, featured in our In the Spotlight column. Planning is underway for Better Breathing 2006, scheduled for February 2-4 at the same location. Suggestions of topics and speakers are welcome and may be submitted by e-mail to [email protected].

IN THE SPOTLIGHT: Congratulations!

RENY VAUGHAN

Reny Vaughan, RRCP, RRT, Respiratory Therapist at Hamilton Health Sciences was awarded the Margaret Fitch Award for Best Poster by an Investigator at the Better Breathing 2005 conference for her poster A Quantitative Measurement of the Effect Volume Augmentation has on Vital Capacity (VC) and Peak Cough Flow in Individuals with Spinal Cord Injury.

CARLA LOFTUS

Carla Loftus, B.Sc., who is completing her undergraduate degree in Nursing at the University of Toronto, was awarded the ORCS Trainee Award for Best Poster by a Student Investigator for her poster Self-care of Chronic Obstructive Pulmonary Disease: A Survey.

BETTER BREATHING 2006 – FEBRUARY 2-4, 2006, DOUBLETREE INTERNATIONAL PLAZA HOTEL

Call for Poster Abstracts The goal of the Better Breathing conference is to provide a forum for professional education and to formulate, disseminate and implement strategies for achieving optimal respiratory health for Ontario. The Education Committee and Research and Fellowship Committee of the Ontario Respiratory Care Society invite you to submit an abstract for a poster presentation at Better Breathing 2006. The poster must relate to respiratory health and may describe: a) a recently completed (or will be completed by the presentation date) research investigation or, b) a recently developed clinical/education program. The presenting author must register for the conference and is responsible for all expenses associated with the preparation, submission and presentation of the poster. An individual may be a presenting author on only one poster per year. The deadline for submissions is October 14, 2005. To obtain the full instructions for authors and the Abstract Submission Form, visit www.on.lung.ca/orcs. SPRING/SUMMER 2005 UPDATE

COMING EVENTS May 20-25, 2005 The 2005 International Conference of the American Thoracic Society will be held in San Diego, California. Contact ats2005@ thoracic.org or (212)315-8656. May 26-29, 2005 The Canadian Physiotherapy Association National Congress 2005 Raising Our Voice will be held in Victoria, British Columbia. Visit www.physiotherapy.ca. June 2-3, 2005 The Third Biennial International Conference on Nursing Best Practice Guidelines will be held at the Hilton Suites Toronto/Markham Conference Centre in Markham. Visit www.rnao.org. June 2-5, 2005 The Canadian Society of Respiratory Therapists’ Educational Forum 2005, Compassion in Action, will be held at the Shaw Conference Centre in Edmonton, Alberta. Contact www.csrt.com. June 7, 2005* The ORCS, Eastern Ontario Region will present an educational afternoon at the University of Ottawa. Topics are Obstructive Sleep Apnea and Uses of CPAP and Non-invasive Bi-level Ventilation in the Community. June 9, 2005* The ORCS, Northern Ontario Region will present, What’s Hot: Emerging Trends in Respiratory Care at The Holiday Inn Sudbury. Topics include Respiratory Infectious Diseases, Best Practice Guidelines for Adult and Pediatric Asthma and Smoking Cessation, Coping Strategies for SOB, Management of Acute Exacerbations of COPD, Cystic Fibrosis and Prevention of RSV. June 15, 2005* The ORCS, Greater Toronto Region will present an educational evening and its Annual Meeting at The Michener Institute from 5:15 – 7:15 p.m. The speaker will be Dr. Kenneth Chapman on The Changing Face of COPD. October 5, 2005 The ORCS will hold a seminar at The Holiday Inn Barrie. October 20, 2005 The ORCS, South Central Ontario Region will hold a seminar at the Quality Hotel in St. Catharines. October 26-28, 2005 The Toronto Critical Care Medical Symposium 2005 will be held at the Metro Toronto Convention Centre. Visit www.tccms. com. The RTSO AGM is included. ...Continued on page 11

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is a wide variation in this measurement in many patients, and that the echocardiographic estimate of PSAP may both under estimate and over estimate the pulmonary pressures.a It is also worth emphasizing that it is not the pressure per se that correlates with symptoms, rather the degree of right ventricular dysfunction. In this regard, the echocardiogram is also of value for assessing right ventricular function. Pericardial effusions are commonly seen. Pericardial effusions of moderate or greater severity are associated with a poor prognosis. Why does this patient have pulmonary hypertension? Once the diagnosis of PH is suspected, the next series of steps involves determining what condition(s) may be accounting for it. The revised Venice classification for PH is provided in Table 1.3 It is important to note

TABLE 2

Diagnostic testing in patients with PH Minimal set Echocardiography

Sleep study

Pulmonary function testing Radiology Chest radiograph Ventilation / perfusion scan High resolution CT scan of the chest +/CT angiogram

Laboratory CBC, electrolytes, creatinine, urinalysis Coagulation Liver function ANA Rheumatoid factor HIV

Cardiac catheterization

ABG

Directed testing (based on clinical suspicion) Ultrasound of the abdomen Auto-immune evaluation Thrombophilic evaluation Sickle cell disease Hemoglobin electrophoresis Cardiac MRI

TABLE 1

Venice classification of pulmonary hypertension3 • Idiopathic PAH • Familial PAH • PAH Associated with – CVD – Left to Right shunt – Portal HTN – HIV – Drug / toxin – Other (T3, HHT, MPD, hemoglobinopathies) • Venous or capillary* – PVOD – PCH • Pulmonary hypertension of the newborn • PH with left heart disease • PH associated with lung disease or hypoxemia – COPD – Hypoventilation syndrome / OSA – Altitude • VTE related – proximal or distal – nonthrombotic embolism (tumor, parasite, talc) • Miscellaneous – (Sarcoidosis, Histiocytosis X, LAM, hyposplenism, compression of mediastinal vessels) *Pulmonary veno-occlusive disease (PVOD), Pulmonary capillary hemangiomatosis (PAH)

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that the previous nomenclature, i.e., primary pulmonary hypertension, has been changed to idiopathic pulmonary arterial hypertension (iPAH). From Table 1, it can be seen that the majority of conditions can be excluded through a detailed history and thorough physical evaluation. The diagnostic tests routinely performed in these patients are provided in Table 2. A high resolution CT scan with contrast angiography is useful for identifying diseases of the pulmonary parenchyma, pleura, mediastinum and vasculature. The presence of interstitial lung disease and pleural effusions are not consistent with the diagnosis of iPAH. Note that the diagnosis of chronic thromboembolic pulmonary hypertension cannot be excluded using CT angiography. A ventilation perfusion scan is required. During the evaluation of patients with PH, a cardiac catheterization is performed. Typically, only a right-sided cardiac catheterization is needed; however, some patients require both left and right cardiac catheterization to exclude the possibility of primary cardiac disease and to evaluate of the coronary arteries. During the cardiac catheterization, the presence of vasoreactivity is sought using vasodilators such as inhaled nitric oxide, intravenous

prostanoids, or adenosine. The Venice conference defines a vasodilator response as an absolute reduction in mean pulmonary pressure by at least 10 mmHg, and an absolute reduction in mean pulmonary pressure below 40 mmHg.3 Only patients who demonstrate an adequate degree of vasoresponsiveness would be suitable for calcium channel blocker therapy. The administration of calcium channel blockers in the absence of adequate information about pulmonary vascular responsiveness or in patients with significant right ventricular dysfunction, should be avoided. The cardiac catheterization also provides useful prognostic hemodynamic information. (vide infra) How disabled is this patient? Once the diagnosis of pulmonary hypertension is made and the cause identified, we typically perform measures of baseline function and assess other variables that have a bearing on prognosis and which can hopefully be modified with therapy. We find these confirmatory tests to be of great value when following patients with PH, since they enable us to better make treatment decisions and determine timing of transplantation. We typically use the Continued on page 5

The presence of pulmonary hypertension in a symptomatic patient should also be confirmed by a formal cardiac catheterization, vide infra.

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6-minute walk test as a measure of exercise performance. This test has been used in most studies of therapy of PH and has been shown to be a strong prognostic indicator. Patients who continue to experience World Health Organization (WHO) class 3 or worse symptoms and/or who have a 6minute walk distance below 350 meters, have a poor prognosis. We also follow the echocardiogram to evaluate right ventricular dimensions and performance and measure brain naturetic peptide (BNP). BNP is a marker of right ventricular/atrial distention and has been correlated to both the 6minute walk test and symptoms. Its role as a prognostic marker is being evaluated. Pathophysiology Significant advances have been made with respect to the understanding of the development of PH.4 Factors that are thought to cause the increased pulmonary vascular resistance that characterizes this disease are: vasoconstriction, remodeling of the pulmonary blood vessels, and in situ TABLE 3

Pathways that have been identified in the pathobiology of pulmonary hypertension • Mediators of vasoreactivity and signaling pathways Reduced – Prostacyclin – Nitric oxide – Vasoactive intestinal peptide – Voltage-gated potassium channels Increased – Thromboxanes – Endothelin – Serotonin • Abnormalities in signaling – Disregulation of TGF-ß signaling pathways – Abnormal apoptosis (programmed cell death) – Abnormalities in matrix proteins and proteolysis – Abnormalities in angiogenesis • Genetic – Abnormalities in bone morphogenic protein II receptor (BMPRII) – Abnormalities in activin-like kinase type 1 (ALK-1) • Viral – Herpes virus – 8 identified in some plexiform lesions

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thrombosis. Much of the research has focused on iPAH in humans or PH induced in a variety of animal models. It is important to emphasize that no animal model currently mimics the human condition. Monocrotaline, hypoxia, and flow mediated animal models have been evaluated. Using these models, a variety of perturbations in the pulmonary vasculature have been demonstrated (Table 3). An exciting area of research has involved elucidation of the gene that is responsible for approximately 50% of the familial forms of PAH.5 This gene codes for the bone morphogenic protein receptor II (BMPRII). BMPRII is an important member of the TGF-B signaling family. It is worth emphasizing that only half of the patients with the familial form carry this gene and only 10% of patients with sporadic disease are positive for it. Despite this, signaling abnormalities in the BMPRII pathway and other potential alterations in cellular signaling, growth and differentiation, apoptosis, matrix proteins, as well as a variety of other candidate genes have been identified as potential contributors to the development of PH. Hopefully, with further insights into the pathophysiology in both iPAH and PH that complicates other diseases, therapies directed at the primary etiological factor(s) that lead to the development of PH will be realized. At present, no therapy specifically targets the underlying primary vascular perturbations in patients with pulmonary hypertension. Rather, current therapies target some of the pathways that have been identified as being abnormal and contributing to the progression of disease. Indeed, in the face of conventional medical therapy, many patients continue to develop progressive disease. Treatment Options Medical therapy General principles The presence of in situ thrombosis as well as demonstrable abnormalities in clotting pathways in patients with PH has led to the recommendation for the use of anticoagulants. However, clear evidence of benefit from randomized controlled trials is lacking. In patients with secondary forms of the disease, the benefits are even less clear. On the basis of limited studies,

patients with PH are placed on warfarin and the dose is adjusted to achieve a target international normalized ratio (INR) between 1.5 and 2.5. Diuretics are often prescribed to reduce peripheral oedema, hepatic congestion, and reduce right ventricular chamber size. This in turn may lead to improvement in right ventricular performance and reduction in tricuspid regurgitation. Specific therapies In patients who have a significant vasodilator response during cardiac catheterization, calcium channel blockers remain potentially efficacious. However, fewer than 25% of patients with iPAH will have this response. Patients with other causes of PH rarely have an acute vasodilatory response. Furthermore, of the patients who demonstrate initial response to calcium channel blockers, only 50% will have a sustained long-term response. Diltiazem, nifedipine, or amlodipine are frequently used, often at very high doses. These patients are typically re-catheterized after six months of therapy to determine whether they have residual vasodilator responsiveness to allow further titration of their calcium channel blockers. A relative deficiency in prostacyclin and excess thromboxane metabolites in patients with PH lead to the early use of exogenous prostanoids. There are several routes (intravenous, subcutaneous, oral and inhaled) and analogues that have been used. At present, epoprostenol (Flolan) remains the gold standard of medical therapy to which all other therapies are compared. Indeed Flolan remains the only medication that has demonstrated a survival benefit in a prospective randomized controlled trial.6 In that study patients with predominately WHO class IV symptoms that received Flolan also experienced an improvement in quality of life and functional capacity. Unfortunately, this medication requires continuous infusion through an indwelling intravenous catheter and causes a variety of undesirable side effects. Patients must also prepare and administer their own medication each day. The drug must be kept cool and protected from light. Inadvertent interruptions in therapy can be life threatening. More recently, treprostinil (Remodulin) has been shown to be of Continued on page 6 5

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potential benefit in patients with PH. Advantages of this drug include subcutaneous administration, greater stability and longer half-life. Consequently, temporary interruption in therapy does not pose a risk to patients. Additional advantages include the ability to use a smaller infusion pump and the reduced, once every third day, drug preparation schedule. The major drawback relates to infusion-site pain, which has been limiting factor in many patients. Although efficacious, the inhaled route of administration of currently available prostanoids has been limited by their short duration of action, necessitating nebulization every 4 hours. Despite some promising early studies of an oral prostaglandin analogue (Beraprost), subsequent studies have failed to demonstrate lasting benefit.7 This drug has not been marketed. Endothelin likely plays an important role in the pathophysiology of PH.8 This potent vasoconstrictor and smooth muscle mitogen is over-expressed in the lesions of patients with PH and its serum concentration has been correlated with the severity of PH in patients. There are two receptors for endothelin: ET-A and ET-B, located on pulmonary vascular smooth muscle cells and both smooth muscle cells and endothelial cells respectively. Bosentan (Tracleer) is a non-selective blocker that has been evaluated in two prospective randomized controlled trials. Taken together these two studies have demonstrated that endothelial receptor blockade leads to an improvement in hemodynamics, six minute walk distance, and symptom scores. Followup data of the initial treated cohort of patients suggests that there is a mortality benefit compared to historical controls. More recently, selective inhibitors of the ET-A receptor are under investigation. Theoretic arguments of benefits of selective versus non-selective blockade are not yet resolved. Nitric oxide is a potent vasodilator and abnormalities nitric oxide pathways have also been described in patients with PH.9 Owing to the relative selectivity of inhaled NO on the pulmonary vasculature, inhaled NO represents an attractive therapy for PH. However, the administration of nitric oxide is limited by cost, availability and ease of use. At present, outpatient use of nitric oxide is not available. NO mediates its effects through the production of cyclic 6

nucleotides (c-GMP and c-AMP). These nucleotides are typically metabolized and inactivated through phosphodiesterases. Therefore an alternate strategy is to increase the downstream second messenger of NO through the use of phosphodiesterase (PDE) inhibitors. In particular blockade of PDE-5 appears to have selective advantages in the pulmonary vasculature. It has been fortuitous that sildenafil (Viagra) has a high affinity for PDE-5. To date, a variety of uncontrolled studies have demonstrated the potential for sildenafil in treating patients with iPAH and a variety of causes of PH. A recent multicenter, prospective blinded randomized controlled trial has recently been completed in patients with iPAH and PAH related to scleroderma. Preliminary results presented in abstract form at the recent CHEST meeting suggest that sildenafil in doses ranging from 20 to 80 mg taken three times daily, improved functional capacity and breathlessness. Changes in hemodynamics during the chronic administration of sildenafil were not realized in this study except at the highest dose. However, earlier reports have suggested an acute hemodynamic benefit with the administration of sildenafil. It is unclear if this is a dose related phenomenon, with higher doses often being used in the earlier acute hemodynamic trials. The future Recent exciting work suggests that HMGCoA reductase inhibitors (statins) may be beneficial in treating patients with pulmonary vascular disease.10 There is a suggestion from pre-clinical data that the simvastatin is capable of reversing some of the vascular changes in animal models of PH mediated through alterations in endothelial cell apoptosis. Investigators at St. Michael's Hospital have recently started working towards clinical trials of cell-based therapy. In preclinical studies using fibroblasts programmed with either vascular endothelial growth factor (VEGF) or nitric oxide synthase (NOS), preventive and reparative experiments show significant promise.11 Essentially it appears that this therapy prevents some of the pulmonary vascular drop-out that occurs in these animal models of PH and potentially restores vascular patency.

Surgery For patients who are refractory to medical therapy there are surgical options. The surgical formation of an atrial septal defect (atrial septostomy) may provide benefit in patients who have an intact atrial septum. An atrial septostomy essentially decompresses the right-sided structures and, in-turn, improves left ventricular diastolic filling and hence left ventricular stroke volume. Although patients develop a right to left shunt and hypoxemia, the net effect is an increase in oxygen delivery. Atrial septostomy is contraindicated in patients who are hemodynamically unstable or who are already hypoxic. Spontaneous closure of these defects or the establishment of too large a defect at the time of angiography remains problematic. For patients with chronic thromboembolic PH, pulmonary endarterectomy has been shown to be extremely effective (Figure 1).12 Typically patients must have a reasonable burden of disease that is sufficiently proximal to allow removal of clot. At present the only Canadian Centre that is routinely performing this procedure is the Ottawa Heart Institute.

Figure 1a Contrast enhanced, rapid sequence, CT image of a patient with chronic thrombo-embolic pulmonary arterial hypertension.

For patients who fail conventional therapy, lung transplantation remains the only suitable option. In general patients must be free from said significant limiting systemic disease such as renal failure or significant primary cardiac disease. Patients without cardiac disease may be Continued on page 7 SPRING/SUMMER 2005 UPDATE

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Figure 1b Thrombectomy specimen from the patient in figure 1a. (Courtesy of Dr. Fraser Rubens)

suitable for either double or single lung transplantation. At our centre only double lung transplantation is performed for patients with PH.13 Patients with coexisting cardiac disease are typically referred for heart lung transplantation. The survival of patients who are transplanted for PH is similar to those who receive lung transplantation for other conditions. The major long-term complications relate to the development of chronic rejection, infection, and bronchiolitis obliterans.

Conclusions For people who develop PH, the prospects for their quality of life and survival have improved. This change in prognosis stems directly from an improvement in the understanding of the pathophysiology of PH and therapies that are based upon this evolution of knowledge. At present intravenous epoprostenol remains the gold standard to which other therapies are compared. The availability of oral therapies for PH remains a significant advance for these patients. References 1. Farber HW, Loscalzo J. Pulmonary arterial hypertension. N Engl J Med 2004; 351:1655-65. 2. Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004; 351:1425-36.

6. Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996; 334:296-301. 7. Galie N, Humbert M, Vachiery JL, et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am Coll Cardiol 2002; 39:1496-502. 8. Channick RN, Sitbon O, Barst RJ, Manes A, Rubin LJ. Endothelin receptor antagonists in pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43:62S-67S. 9. Michelakis E, Tymchak W, Lien D, Webster L, Hashimoto K, Archer S. Oral sildenafil is an effective and specific pulmonary vasodilator in patients with pulmonary arterial hypertension: comparison with inhaled nitric oxide. Circulation 2002; 105:2398-403. 10. Nishimura T, Vaszar LT, Faul JL, et al. Simvastatin rescues rats from fatal pulmonary hypertension by inducing apoptosis of neointimal smooth muscle cells. Circulation 2003; 108:1640-5.

3. Simonneau G, Galie N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004; 43:5S-12S.

11. Campbell AI, Zhao Y, Sandhu R, Stewart DJ. Cellbased gene transfer of vascular endothelial growth factor attenuates monocrotaline-induced pulmonary hypertension. Circulation 2001; 104:2242-8.

4. Humbert M, Morrell NW, Archer SL, et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol 2004; 43:13S-24S.

12. Rubens F, Wells P, Bencze S, Bourke M. Surgical treatment of chronic thromboembolic pulmonary hypertension. Can Respir J 2000; 7:49-57.

5. Newman JH, Trembath RC, Morse JA, et al. Genetic basis of pulmonary arterial hypertension: current understanding and future directions. J Am Coll Cardiol 2004; 43:33S-39S.

13. de Perrot M, Chaparro C, McRae K, et al. Twentyyear experience of lung transplantation at a single center: Influence of recipient diagnosis on long-term survival. J Thorac Cardiovasc Surg 2004; 127:1493-501.

UPDATE ON THE ORCS THREE YEAR PLAN – 2005-2008 t the ORCS Annual General Meeting, held during the Better Breathing 2005 conference, the Provincial Committee presented a new Three Year Plan to the membership. Seven goals and a series of objectives for each goal were approved in principle. The Provincial Committee is defining strategies and timelines for each objective and has begun implementation. This work will continue during the next three years. The Plan includes new Vision and Mission statements which contribute to The Lung Association’s Mission, To improve lung health. The objectives focus on the Society’s key program areas and address many of the comments and suggestions made by members who participated in focus groups and a survey in the spring and summer of 2004. We appreciate the input received to date and welcome members’ suggestions for our future programs and services. Further details will be provided to the membership as the objectives and strategies are implemented.

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GOALS 1. Ensure the continued growth and success of the ORCS as an organization that supports excellence in respiratory care; 2. Be a valuable contributor of expertise and volunteer resources to the OLA and CLA; 3. Provide excellent interdisciplinary continuing education programs for respiratory health professionals throughout Ontario; 4. Provide high quality publications to the ORCS membership and the respiratory health care community; 5. Fund Respiratory Research and provide support for members undertaking post-graduate education; 6. Provide respiratory health educators with targeted programs and information through the Respiratory Health Educators Interest Group (RHEIG); 7. Institute performance indicators for ORCS programs in compliance with the requirements of the Board of Directors.

VISION Improved lung health through excellence in interdisciplinary respiratory care.

Helpline 1-800-668-7682 Helpline 1-800-668-7682

MISSION Furthering excellence in the provision of interdisciplinary respiratory care through education, research, collaboration, provision of professional expertise and support for Lung Association efforts to improve lung health. SPRING/SUMMER 2005 UPDATE

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Beyond words: the health-literacy connection Reprinted from the Canadian Health Network – www.canadian-health-network.ca any Canadians lack the basic literacy skills required to deal with the increasingly complex demands of everyday living. Almost 50 per cent of Canadian adults can’t work well with words and numbers; and 22 per cent of Canadians have serious difficulties with any type of printed materials. Another 26 per cent have problems with all but the simplest tasks requiring reading and writing.

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What do we mean by literacy? Literacy is more than the ability to read or write. Some definitions of literacy focus on the basic reading and writing skills that enable people to function in everyday situations – e.g. the ability to read and follow a recipe or the instructions for an appliance. To participate effectively in daily life, however, people also require communication skills. In this context, literacy involves the combination of thinking skills and social skills. The term critical literacy has been used to describe the more advanced set of cognitive skills, which, together with social skills, are needed to critically analyze and use information to exert greater control over life events and situations. Defining health Health means more than simply not being sick. Health is a state of physical, mental and social well being. If individuals are to reach such a state of well-being, they must be able to identify and realize their goals, satisfy their needs, and change or cope effectively within their environment. This view of health as a resource for everyday life, rather than the objective of living, was put forth in the Ottawa Charter for Health Promotion (1986). The Ottawa Charter recognized health promotion as the process of enabling people to increase control over, and to improve their health. Many factors determine our health. Low literacy linked to poorer health Not only is literacy a major determinant of health; it is also closely associated with other socio-economic conditions that influence health, such as income, social status, employment opportunities, social support, early childhood development, 8

culture and gender. Canadians who have low literacy are more likely to have poorer health, higher rates of chronic disease, and an earlier death than those with a higher level of literacy. Literacy's effects on health Literacy influences health both directly and indirectly. Most obvious are the direct effects. Less obvious, but even more profound are the indirect effects of reduced literacy on health. Some factors associated with limited literacy include: • Having difficulties speaking English • Speaking English as a second language • Being an older adult (many seniors did not have the advantage of completing their education) • Not completing high school • Living in conditions of poverty and lacking adequate resources such as income and social support. Some behaviours can serve as signs to service providers that they should be especially sensitive to the literacy level of their clients when communicating healthrelated advice. Low literacy may be a health barrier if someone: • Frequently returns for health-related services • Arrives for appointments without completed forms • Never refers to written information they have received • Avoids filling out forms and questionnaires • Relies on others to read material for them • Brings family members to appointments as a surrogate reader • Claims to have vision or other problems to avoid reading • Ignores or misunderstands advice/ instructions (e.g. medications) • Reads slowly with obvious effort • Reads something faster than expected with no comprehension • Never jots down instructions.

health concerns of people limited in literacy, and the literacy concerns of people experiencing poor health. Health and literacy practitioners are recognizing that much can be done to reduce the barriers that limited literacy imposes on one's health and well being.

Suggestions for Health Practitioners • Look for clues that someone may be having difficulty understanding your advice because of limited literacy. • Rely on face-to-face opportunities for giving advice whenever possible. • Write in clear language when using printed material. • Avoid using jargon. • Learn more about how adults with limited literacy find and use health information, services and supports. • Look for ways to make information more user-friendly and easier to access. • Be familiar with and refer clients to appropriate community-based literacy and health programs. • Challenge policies that create barriers to health when literacy is limited.

For a complete transcript of this article or to find information on other healthrelated topics, visit http://www.canadianhealth-network.ca/-- Canada’s preferred source for credible health information. Funded by the Public Health Agency of Canada, the Canadian Health Network has links to over 17,000 web-based resources from leading, nonprofit, Canadian health organizations. For more information, contact Jennifer Schenkel, Health Promotion Officer for CHN’s Respiratory Diseases affiliate [email protected].

Health literacy is an opportunity for practitioners from the fields of health and literacy to work together to address the SPRING/SUMMER 2005 UPDATE

RESPIRATORY CARE PERSPECTIVES

A Breath of Northern Air By Monique Ouellette, RRT, Respiratory Therapist, Iroquois Falls In this issue, we introduce a new column in which respiratory health professionals describe their work, demonstrating the wide range of settings in which ORCS members work and unique aspects of their experiences. If you would like to submit an article for this column, please contact the Editors or the ORCS office at [email protected] or (416)864-9911, ext. 236. here there are people, there is a need for Respiratory Services. This has been my motto from the very start of my career. I have been servicing the far northern area of Ontario for the last twenty-five years. Before then, I promised my rebellious self that the North was not for me. Once I found my way out of the “bush”, I made it a point to explore what excitement the bigger cities offered. The noise, the abundance of people, the shopping malls and the restaurants were all so different from what I had known. I truly enjoyed it but my love for the North was much stronger. Most people like myself return because they wish to be closer to their family and loved ones. I certainly do not regret coming home - the Northern Air has been good to me. In 1979, Respiratory Therapy, or at that time, “Respiratory Technology” was for the most part unknown. Most people thought respiratory therapy had something to do with breathing, as the name implies, but had no idea what we actually did. Because the profession was unfamiliar in the area, I was determined to market, convince and entice hospitals to hire Respiratory Therapists. I visited all the physicians and the CEOs within an easy traveling distance. I offered them a service they could not refuse, even when they were unsure of their need for me. The persuasion was not easy especially since the perception was that breathing problems were more common in mining towns than paper and wood towns such as Iroquois Falls, Cochrane and Matheson. Finally, an Anaesthetist, in a hospital fifty kilometres from my hometown, gave me a chance. For the first year, I was able to work there for four hours a week. Luckily, I was able to find other short-term placements at numerous locations through-

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SPRING/SUMMER 2005 UPDATE

Coalition and programs provided out the North, such as Kirkland by The Lung Association. Lake, a town 90 km from In the North, the true my home and for Home Care emergencies requiring CPR or companies. This was the ventilation are rare. We maintain beginning of my “road running” the patients until hospitals accept experience. Today, my role them and the paramedics arrive to is with the MIC’s group of Health Services covering transfer them. This may take from hospitals in Matheson, Iroquois six to eighteen hours depending Falls and Cochrane. These three MONIQUE OUELLETTE on the situation. We often use Bicommunities have seventy active beds Pap at three hospitals, eliminating the need servicing a combined population of about to transfer the patients to outside sources. 13,000. We have 14 designated physicians. I Although I am not on call, I usually try to also work casually in the sleep lab in Timmins. attend emergencies to gain experience and Because of the large geographical area, I to establish better patient care. am more often than not on the road. The There are many perks to working up summer drives can be enjoyable, however, I North. As the sole charge RT, I am well need to be aware of the moose and the known in these small communities. I am the countless bears which tread on the Northern only one wanting to attend Respiratory highways. Winters tend to be a challenge Therapy conventions; schedules are tailorwith snowstorms occurring seven months of made for me. I take holidays when I want the year. Fortunately, the North is like a holidays; I am my own supervisor and of family: there is an open door welcoming me course my own staff. anywhere I work, should I be stranded. Where there are perks there are quirks. My role is that of a sole charge RT (also Like many sole charge therapists, my role referred to as traveling RT). The vast majority includes tedious administrative tasks such of the workload is in diagnostics. I perform as numerous meetings, statistics, CQI, PFTs, ABGs, walking and nocturnal accreditation.... the list goes on. This is saturations, ambulatory BP, holter monitoring, especially true since these laborious tasks continuous loops and ECGs at all sites. I are done in triplicate for each hospital that I have acquired most of my tools through the service. It can be very lonely working by help and generosity of charitable and oneself. I have no one to bounce ideas off, service organizations. The Auxiliaries to the or consult for problems. The area hospitals Hospitals have been tremendously helpful are quite helpful in providing advice, but in providing necessary equipment. are often too busy to return my calls in a The other frequent tasks I am involved in reasonable time frame. It is also sometimes are assessments and teaching. I complete difficult to take off time that I am owed as I three, twelve-week sessions of Pulmonary do not have a replacement. Another “quirk” Rehabilitation each year. I mainly assess in- is the lack of Respiratory Therapy patients, often providing them with breathing education. I envy my southern peers who equipment such as the PEP, the Acapella® are able to attend one-day sessions easily. and the Incentive Spirometer. Outpatients I have twenty-five years in the service seen for diagnostics get information on the with six years left to go before retirement. effects of smoking and advice on methods Those have been happy years and I feel I of stopping, directions on how to use their have been an important member of a team inhalers properly and any other information and know that my contributions to these three that might help in the management of their small communities have made a difference disease. I am often asked to speak at various to their citizens. I encourage Respiratory events put on by organizations and schools. Therapists from all communities, especially I teach smoking cessation programs, attend small ones, to promote the profession Health Fairs or participate in educational because where there are people, there is a programs such as the Heart Health need for Respiratory Therapy services. 9

Respiratory Articles of Interest Vianello A, et al. Mechanical insufflationexsufflation improves outcomes for neuromuscular disease patients with respiratory tract infections. Am J Phys Med Rehabil 2005; 84:83-88. This study compared 11 consecutive neuromuscular disease (NMD) patients with respiratory tract infections and secretion retention who received mechanical Insufflation-Exsufflation and conventional chest physical therapy in an ICU and compared them with the outcomes of 16 historical matched controls who received physical therapy alone. Treatment failure was significantly lower in the mechanical Insufflation-Exsufflation group. It was well tolerated by all subjects. An interesting commentary ‘a step in the right direction’ was written about this article by M.R. Goncalves and J.R. Bach (Am J Phys Med Rehabil 2005;84:9-91) Together they support the important role mechanical Insufflation-Exsufflation plays for patients with NMD experiencing respiratory tract infection. Dolovich MB, Aherns RC, Hess DR, et al. Device selection and outcomes of aerosol therapy. Chest 2005; 127(1):335-371. This special report is a systematic review evaluating the evidence for the selection of the appropriate inhalation device for the delivery of common respiratory medications. The level of strength of the evidence for each recommendation is provided. None of the pooled meta-analyses showed a significant difference between devices in any efficacy outcome. Devices used for the delivery of bronchodilators and steroids can be equally efficacious. Monninkhof E. van der Valk P. van der Palen C. et al. Effects of a comprehensive selfmanagement programme in patients with chronic obstructive pulmonary disease. Eur Respir J 2003; 22:815-820. In other conditions such as asthma, interventions based on self-management principles have been recognized to improve health outcomes. However, studies on the role of self-management in the care of individuals with COPD have been inconclusive. This article provides the results of a large randomized controlled trial that examined the effects of comprehensive self-management intervention 10

in 248 individuals with stable COPD. The intervention consisted of a selfmanagement education and a fitness programme in addition to usual care, compared to a control group receiving usual care. The main outcomes were health related quality of life measured with the St. George’s Respiratory Questionnaire, symptoms from a two-week diary and 6 minute walk test. There was no beneficial effect of the intervention for any of the outcomes. Surprisingly, the intervention group had an almost double the rate of acute exacerbation, the majority of which were self-treated at home and did not lead to deterioration in quality of life. The authors conclude that this study failed to show positive effects of a self-management programme among individuals with COPD who are already receiving close to optimal treatment. Bourbeau J. Julien M. Maltais F. et al. Reduction of hospital utilization in patients with Chronic Obstructive Pulmonary Disease. Arch Intern Med 2003; 163:585-591. This article provides the results for a large multicentered randomized controlled trial conducted to assess the effects of selfmanagement on health status and the use of hospital services among a population of 191 individuals with COPD. The intervention consisted of patient education, the provision of written teaching material and a training program, supported by the counselling from a case manager. The control group received usual care. Main outcome measures were health related quality of life measured by the St George’s Respiratory Questionnaire (SGRQ), 6 minute walk test, frequency of acute exacerbations and health care utilization by patient report and standardized monthly interviews. Results indicate favourable results in the intervention group with respect to the number of hospital admissions, the number of unscheduled family physician visits and in the impact subscale of the SGRQ. The authors’ conclusion supports the implementation of this comprehensive approach in the long term care of patients with moderate to severe COPD.

ONTARIO RESPIRATORY CARE SOCIETY PROVINCIAL COMMITTEE 2005-2006 CHAIR Lisa Cicutto, RN, PhD, ACNP, CAE PAST CHAIR Lawrence Jackson, BScPhm CHAIR, RESEARCH & FELLOWSHIP COMMITTEE Paula Burns, RRT, RRCP, PhD(c) CO-CHAIRS, EDITORIAL BOARD Dina Brooks, BScPT, MSc, PhD Libby Groff, RRCP, RRT, BHA CHAIR, EDUCATION COMMITTEE Rob Bryan, RRCP, RRT CHAIR, MEMBERSHIP & PROGRAM PROMOTION COMMITTEE Miriam Turnbull, RRCP, RRT, MBA REGIONAL REPRESENTATIVES EASTERN ONTARIO Harold Joubert, RRCP, RRT ESSEX/KENT Carol Stallaert, RRCP, RRT GREATER TORONTO Paula Cripps-McMartin, RRCP, RRT, CAE NORTHERN ONTARIO Julie Duff Cloutier, RN, BScN, MSc, CAE SOUTH CENTRAL ONTARIO Sheila Dedman, BSc, BHScPT SOUTHWESTERN ONTARIO Justyna Minda, BScPT RESPIRATORY HEALTH EDUCATORS INTEREST GROUP REPRESENTATIVE Dale Mackey, RRCP, RRT, CAE COMMUNITY SERVICES LIAISON Cathy Relf, BScPT ONTARIO LUNG ASSOCIATION BOARD OF DIRECTORS REPRESENTATIVE Nancy Hanson

Compiled by Reny Vaughan, Larry Jackson DIRECTOR OF ADMINISTRATION Sheila Gordon-Dillane, BA, MPA and Stefania Costi. SPRING/SUMMER 2005 UPDATE

New Teen Asthma Website Launched on World Asthma Day

n May 3rd, The Lung Association launched a new interactive, educational and fun website for teens with asthma – www.teenasthma.ca. The theme for World Asthma Day 2005 was “The Unmet Needs of Asthma”. Past investigations found a lack of resources for Canadian teens with asthma. We went to the people who know best what teens want – teens! The results of several teen focus group studies steered the development from the initial concepts to the final website. Funding was provided by the Government of Ontario.

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ORCS Membership

COMING EVENTS

ORCS membership is open to health care professionals and students in many disciplines including nursing, respiratory therapy, physiotherapy, occupational therapy, pharmacy, pulmonary function technology, social work and nutrition. The membership fee for April 1, 2005 to March 31, 2006 is $40. The student fee is $25. Members wishing to join the ORCS Respiratory Health Educators Interest Group, add $15. Please complete the form below and send it by fax with a credit card number or by mail with your cheque to the Ontario Respiratory Care Society, 573 King St. E., Toronto, ON, M5A 4L3.

October 29–November 3, 2005 Chest 2005 will be held in Montreal. Visit www.chestnet.org.

Name: ___________________________________ Profession: ______________________________________ Preferred mailing address: ❏ Home ❏ Business Home Address: ____________________________________________________________________________ ___________________________________________________________ Postal Code: __________________ Bus. Address: ____________________________________________________________________________ ___________________________________________________________ Postal Code: __________________ Position: ______________________________________ Organization: ________________________________ Home Tel: _____________________________________ Bus. Tel:

__________________________________

Fax: ______________________________ E-mail: ________________________________________________ Membership Category: ❏ Individual

❏ Student

❏ Respiratory Health Educators Interest Group Option

❏ Two year renewal (to March 31, 2007) Amount Enclosed $________ ❏ Cheque enclosed payable to the Ontario Respiratory Care Society Charge my: ❏ VISA ❏ Mastercard Credit Card No. _____________________________________________________ Expiry Date: ____________ Name of cardholder: ___________________________________ Signature ____________________________

Changing Your Mailing Address? Please help us keep our mailing lists up-to-date by notifying us of any changes in your address or employment. SPRING/SUMMER 2005 UPDATE

Continued from page 3

November 9, 2005* The ORCS, GTR’s annual fall seminar, Respiratory Emergencies, will be held at the Ramada Hotel and Conference Centre in Toronto. November 17-19, 2005 ASED 7, Rising to the Challenge: Better Asthma Care Through Education will be presented by the Canadian Network for Asthma Care (CNAC) at the Hyatt Regency Hotel in Calgary, Alberta. For information, go to www.cnac.net/ased. November 23, 2005 The ORCS, Southwestern Ontario Region presents Innovations in Respiratory Care: are you ready? at the Best Western Lamplighter Inn in London. February 2-4, 2006 Better Breathing 2006 will be held at the Doubletree® International Plaza Hotel in Toronto. *For further information on ORCS programs, call (416) 864-9911, e-mail [email protected] or visit on.lung.ca/orcs.

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Indicated for the maintenance treatment of asthma in patients where the use of a combination product is appropriate. See Product Monograph for patient selection, warnings, precautions and adverse events.

Mississauga, Ontario L5N 6L4