From the Guidelines
For a patient with CREST syndrome in the pulmonary setting, education should focus on collaborative self-management skills, including an action plan for early recognition and treatment of exacerbations, to improve quality of life and outcomes. When educating the patient, consider the following key points:
- Emphasize the importance of self-management skills, as recommended by the American Thoracic Society/European Respiratory Society statement on pulmonary rehabilitation 1
- Teach the patient about the importance of regular monitoring, including pulmonary function tests and echocardiograms, to track disease progression
- Explain proper use of prescribed medications, such as vasodilators for pulmonary hypertension and immunosuppressants for interstitial lung disease
- Instruct on oxygen therapy, including proper flow rates and when to use it, if prescribed
- Emphasize the importance of pulmonary rehabilitation exercises to maintain lung function and build exercise tolerance
- Teach breathing techniques, such as pursed-lip and diaphragmatic breathing, to manage dyspnea
- Discuss the importance of avoiding respiratory irritants like smoke and air pollution
- Educate about recognizing signs of respiratory infection or worsening pulmonary symptoms that require immediate medical attention, as outlined in the joint ACCP/AACVPR evidence-based clinical practice guidelines 1
- Collaborative self-management strategies, including goal setting, problem solving, and decision-making, can be implemented through pulmonary rehabilitation, as described in the American Thoracic Society/European Respiratory Society statement on key concepts and advances in pulmonary rehabilitation 1
From the Research
Education for Patients with CREST Syndrome in the Pulmonary Setting
- The patient should be informed about the characteristics of CREST syndrome, including calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias 2, 3.
- It is essential to educate the patient about the potential complications of CREST syndrome, such as pulmonary hypertension, which can be a major cause of mortality 2, 3, 4.
- The patient should be aware of the symptoms of pulmonary hypertension, including dyspnea, cyanosis, and peripheral edema, and the importance of seeking medical attention if these symptoms occur 2.
- Patients with CREST syndrome should be educated about the available treatment options for pulmonary hypertension, including vasodilators, such as sildenafil and beraprost, and the potential benefits and risks of these treatments 5.
- The patient should be informed about the importance of regular monitoring of their condition, including pulmonary function tests and echocardiograms, to detect any changes in their condition 4, 6.
- Patients with CREST syndrome should be educated about the potential for pulmonary fibrosis and the importance of seeking medical attention if they experience any symptoms of respiratory distress 6.
- The patient should be aware of the potential for other complications, such as digital gangrene and primary lung carcinomas, and the importance of regular follow-up with their healthcare provider 3, 6.