Table 2. Medications
Class/Agent Dose Notes
Medications for Sickle Cell Disease-associated Pulmonary Artery Hypertension
Prostacyclin agonists
Epoprostenol
Veletri
®
, generic
• Initial: 2 ng/kg/min by
infusion pump
• Increase by 1-2 ng/kg/min at
≥15 min intervals
http://www.veletri.com/
pdf/veletri_full_prescribing_
information_2nd_gen.pdf
Iloprost
Ventavis
®
10 and 20 mcg/mL
• 6-9 inh daily by nebulizer at
≥2 hour intervals
• Initial: 2.5 mcg/dose
• Max: 5 mcg/dose
http://www.4ventavis.com/pdf/
Ventavis_PI.pdf
Treprostinil
Tyvaso
®
• Initial: 3 inh (18 mcg ) QID
(Q4H)
• Increase by 3 inh every 1-2
weeks
• Max: 9 inh (54 mcg ) QID
(Q4H)
https://tyvaso.com/pdf/Tyvaso-
PI.pdf
Endothelin Receptor Antagonists
Ambrisentan
Letairis
®
• 5 mg daily PO
• Max: 10 mg daily
• Embryo-Fetal Toxicity
• Tablets should not be split,
crushed, or chewed
http://www.gilead.com/pdf/
letairis_pi.pdf
Bosentan
Tracleer
®
• Initial: 62.5 mg BID ×
4 weeks PO
• Then 125 mg BID
Hepatotoxicity and Teratogenicity
http://www.tracleer.com/patient/
prescribing-information.asp
Macitentan
Opsumit
®
• 10 mg daily PO • Embryo-Fetal Toxicity
• Tablets should not be split,
crushed, or chewed
http://opsumit.com/sites/
opsumit/files/OPSUMIT-Full-
Prescribing-Information.pdf
Medications for Patients with Sickle Cell Disease and an Increased Risk for Mortality
Hydroxyurea
Droxia
®
• Initial: 15 mg/kg/day PO
• Increase by 5 mg/kg/day
every 12 weeks if the CBC
is acceptable
• Max: 35 mg/kg/day
• CBC every 2 weeks
• Cytotoxic; avoid dermal exposure
packageinserts.bms.com/pi/
pi_droxia.pdf
