Background
Sickle cell disease and important aspects of care
Group medical appointment | Individual medical appointment | |
---|---|---|
Number of patients | 6–8 | 1 |
Duration of appointment | 90 min total | 15 min per professional |
Severity of disease | Various severities of disease | One severity of disease |
Professionals | Treating physician, nurse, clinical geneticist, social worker | Treating physician, nurse, clinical geneticist, social worker |
Clinical examination | Behind a screen in conference room or in another room (before or after the appointment) | In physician’s office |
Privacy | Confidentiality protected by the group | Completely |
Hypotheses
Methods/Design
Participants
Interventions
Care-as-usual: IMA
Intervention: GMA
1. Measurement of weight and height of all participants. |
2. Introduction by the appointed chairman with special attention to procedure, privacy and allotted time. |
3. Individual interview by treating physician specifically focusing on individual disease symptoms and complications. |
4. During the GMA disease-related topics are discussed, in accordance to patient questions or introduced by the chairman: |
Disease and therapy |
Early identification of sickle cell crises and crises provoking factors |
Interventions to influence sickle cell crises provoking factors. |
Pain treatment regimens, importance of treating pain early and adequately |
Consequences of functional asplenia and importance of anticipating possible consequences (antibiotic prophylaxis, vaccinations, early treatment with broad spectrum antibiotics in case of fever) |
Recognition of important or alarming disease symptoms: aggravation of anemia, splenic sequestration, severe sickle cell crises needing hospital attention, infections and respiratory problems, gallstones, symptoms suggestive of cerebral infarctions, cognitive deterioration, priapismus, bone infarctions |
Education with regard to pathophysiological effects of treatment options (hydroxycarbamide, chronic blood transfusions) |
Importance of healthy lifestyle (enough rest, healthy diet, consciousness of limitations) |
Importance of informing school and others of disease |
Regular dental consultations and importance of vaccinations according to national guidelines |
Lifestyle |
Avoidance and early treatment of sickle cell crisis provoking factors (cold, fever/infection, pain, high altitude, dehydration/enough fluids, fatigue and stressful situations) and underlining specific social and physical circumstances with risks: swimming lessons, alcohol, menstrual period, intensive sporting activities |
Encouragement of individual self-empowerment, self-efficacy |
Frequent contact with Sickle Cell Comprehensive Care Center is encouraged |
Support and guidance with regard to job and career choice |
Genetics |
Explanation of different sickle cell genotypes |
Inheritance of the disease and importance of genetic counseling |
Explanation of meaning of coinheritance of alpha thalassemia and persistent fetal hemoglobin levels (HbF) |
Options with regard to prenatal diagnosis and preimplantation genetic diagnosis |
5. At the end of the GMA diagnostic vena punctures and more private individual consultations are performed and, if necessary, physical examinations |
Previous evidence of GMA-effectiveness
Innovative character
Outcomes
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Self-report (Morisky Medication Adherence Scale) [23];
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Variations in adherence (Medication Adherence Report Scale) [24];
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Blood values suggesting adherence to prescribed medication (folic acid, hydroxycarbamide, iron chelation therapy) such as: folic acid (nmol/l), MCV (fl), fetal hemoglobin percentage (HbF %), ferritine (mcg/l);
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Attendance at outpatient clinic visits or imaging examinations (% of total planned).