Please select the arm or intervention descriptions that belong to the clinical trial below.
Title: Long-Term, Open-Label, Multicenter, Extension Study of Bosentan in Patients With Pulmonary Hypertension Associated With Sickle Cell Disease Completing a Double-Blind ASSET Study (AC-052-368 or AC 052-369)
Brief Summary: This study will assess the safety and efficacy of bosentan therapy (in a study known as ASSET) for patients who have high blood pressure in the lungs associated with sickle cell disease. That form of hypertension places people at risk for complications, including shortness of breath, pain, pneumonia, and death. Previous studies have shown that bosentan can be helpful in reducing pulmonary hypertension.

Patients ages 16 and older who have completed the 16-week treatment in the ASSET 1 or ASSET 2 study and who are not pregnant or breastfeeding may be eligible for this study. The research will be conducted in about 25 hospitals in the United States and Europe. Up to 30 participants will be enrolled. The screening visit will involve a physical examination, blood sample of about 3 teaspoons for laboratory tests, and a pregnancy test. Patients' doctors will give them bosentan tablets (62.5 mg each), to take one in the morning and one in the evening. After 1 month, patients will be told whether the dose should be increased to 125 mg tablets to take twice a day. Two weeks after the increase in dose, a blood test will be done to analyze the drug's effects on the liver. After the start of treatment, patients will return for visits every 6 months, when there will be a 6-minute walking test to measure exercise capacity and evaluate shortness of breath. There will be follow-up for patients up to the end of the study and for 28 days after the last dose of bosentan is taken, to collect information about side effects.

Some patients on bosentan have had changes in liver function and red blood cell count. Side effects commonly reported are headache, flushed appearance, inflammation of the throat and nasal passages, and gastrointestinal symptoms. If patients have sudden worsening in breathing in the first few weeks after taking bosentan, they should immediately tell their doctors, because it may be necessary to change the treatment.


Options:
A: Arm Label: Blood Transfusion Trial Cohort; Type: ACTIVE_COMPARATOR; Interventions: Biological: Single blood transfusion | Arm Label: Standard Care Trial Cohort; Type: ACTIVE_COMPARATOR; Interventions: Behavioral: Standard care | Arm Label: Standard Care Observational Cohort; Type: ACTIVE_COMPARATOR; Interventions: Behavioral: Standard care | Intervention Type: BIOLOGICAL; Name: Single blood transfusion; Assigned to Arm(s): Blood Transfusion Trial Cohort | Intervention Type: BEHAVIORAL; Name: Standard care; Assigned to Arm(s): Standard Care Observational Cohort, Standard Care Trial Cohort
B: Intervention Type: DRUG; Name: Bosentan; Assigned to Arm(s): N/A
C: Arm Label: 1; Type: NO_INTERVENTION; Interventions: N/A | Arm Label: 2; Type: EXPERIMENTAL; Interventions: Drug: Bosentan | Intervention Type: DRUG; Name: Bosentan; Assigned to Arm(s): 2
D: Arm Label: 1; Type: NO_INTERVENTION; Interventions: N/A | Arm Label: 2; Type: EXPERIMENTAL; Interventions: Drug: bosentan | Intervention Type: DRUG; Name: bosentan; Assigned to Arm(s): 2

Answer: B