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Sickle cell disease is one of the most common genetic diseases in the world. (1) It is characterized by the production of abnormal hemoglobin mainly responsible for vaso-occlusive clinical manifestations and chronic hemolysis with anemia. (2) It is therefore a chronic disease with major acute complications, such as acute chest syndrome. The treatment for this syndrome will be based on oxygenation, hydration and analgesia. At the physiotherapy level, we will have an action on the prevention and treatment of the syndrome by incentive spirometry. (3,4) In fact, it is currently the only physiotherapy treatment that has proven its effectiveness and is recommended for sickle cell patients. (3) As part of prevention, it is recommended to prescribe incentive spirometry during vaso-occlusive crisis. It has been shown to reduce the risk of atelectasis and significantly limit the risk of developing ACS. (5) In treatment, it makes it possible to regain normal chest amplification and therefore to allow ventilation of unventilated areas. (3.4) However, in order to increase therapeutic efficacy, patient compliance is essential.
Adherence to treatment is a major problem in chronic diseases. Currently, it is estimated that 80% of patients with chronic conditions do not sufficiently follow their therapy, which limits the optimization of benefits. (6) This is the case in sickle cell patients, especially with hydroxyurea which is their disease-modifying treatment. Lack of adherence is the most common cause of primary failure of this treatment.
During various treatments, we noticed the patients' lack of compliance with spirometry. Indeed, we explained to the patient how to do the incentive spirometry, so that he could practice it several times a day as recommended. When we returned the next day, or after a weekend, most of the time the patients had little or no observance. So I wanted to know if this concerns a majority of patients with sickle cell disease. Indeed, it appears important to assess compliance in these patients in order to improve the effectiveness of treatment and reduce the risk of ACS.
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| Measure | Description | Time Frame |
|---|---|---|
| To take stock of the observance of sickle cell patients with inciting spirometry | Measuring compliance through the questionnaire | Through study completion, an everage of 10 minutes |
| Measure | Description | Time Frame |
|---|---|---|
| To take stock of patients' knowledge of their pathology and incentive spirometry | Assessing knowledge about their pathology and treatment via the questionnaire | Through study completion, an everage of 10 minutes |
| Measuring adherence to medical follow-up |
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Inclusion Criteria:
Exclusion Criteria:
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Patient with sickle cell anemia
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Centre hospitalier intercommunal de Créteil | Créteil | 94000 | France |
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| ID | Term |
|---|---|
| D000755 | Anemia, Sickle Cell |
| D056586 | Acute Chest Syndrome |
| D010349 | Patient Compliance |
| ID | Term |
|---|---|
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
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Measuring the number of consultations via the questionnaire |
| Through study completion, an everage of 10 minutes |
| Assessing the impact of the disease on quality of life | Measuring the patient's quality of life via the questionnaire | Through study completion, an everage of 10 minutes |
| D006425 |
| Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D012120 | Respiration Disorders |
| D010342 | Patient Acceptance of Health Care |
| D000074822 | Treatment Adherence and Compliance |
| D015438 | Health Behavior |
| D001519 | Behavior |