Sickle cell disease Haematological Disorders Sickle-cell anaemia without crisis
Conditions
Interventions
Conventional therapy including hydroxyurea and exchange transfusion. This includes the use of 5-hydroxyurea or exchange transfusions as prescribed by haematology team caring for the patient. This is s
Sponsors
University College London (UK)
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1. Patients with a diagnosis of homozygous sickle cell disease 2. Aged 18 and over
Exclusion criteria
Exclusion criteria: 1. Age outside inclusion criteria 2. Impaired left ventricle (LV) function 3. Valvular abnormalities 4. Sickle cell crisis within 2 weeks of recruitment. 5. Acute chest syndrome within 4 weeks of recruitment 6. Principal exclusion criteria to perform an MR scan: 6.1. Permanent pacemaker 6.2. Intracerebral aneurysm clip 6.3. Pregnancy 7. Physical diabilities which don't permit the patient to ride on a bike 8. Principal exclusion criteria to perform a cardiopulmonary exercise test: 8.1. Myocardial infarction (35 days) 8.2. Syncope 8.3. Uncontrolled heart failure 8.4. Uncontrolled Asthma 8.5. Respiratory failure 8.6. Resting saturations <85% 8.7. Uncontrolled arrythmias 8.8. Active endocarditis, myocarditis, pericarditis
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 1. Cardiac Magnetic Imaging can be used to assess pulmonary vascular parameters in SCD patients 2. The pulmonary vascular responses to exercise are abnormal in patients with SCD Timepoints of measurements: 0 months (baseline) and 12 months (end) | — |
Secondary
| Measure | Time frame |
|---|---|
| Treatment of SCD with transfusion therapy and 5-hydoxyurea, designed to reduce the proportion of sickle red cells, alter vascular responses to exercise Timepoints of measurements: 0 months (baseline), 6 months (mid) and 12 months (end) | — |
Countries
United Kingdom
Outcome results
None listed