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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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