Table 2 Structure of (pediatric) sickle cell disease group medical appointment

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