What key counseling points should be provided to a patient with Pulmonary Arterial Hypertension (PAH) before initiating sotatercept therapy?

From the Guidelines

A key counseling point for a patient starting sotatercept for pulmonary arterial hypertension (PAH) is that the medication will be administered once weekly, with the fourth dose increased unless side effects are problematic. This dosing strategy is used to optimize efficacy while managing potential side effects, as seen in the STELLAR trial 1. The medication works by restoring balance to dysregulated bone morphogenetic protein signaling, and the gradual dose escalation allows the healthcare team to assess the patient's response and adjust treatment accordingly. Some key points to consider when counseling the patient include:

• The importance of following the prescribed dosing schedule to ensure optimal efficacy and minimize side effects
• The potential for side effects, such as nose bleeds and bleeding of the gums, and the need to notify their healthcare provider if these occur
• The need for daily monitoring of blood pressure, as the medication may lower blood pressure
• The recommendation to administer the medication with food to optimize its benefit Patients should understand this dosing schedule so they can anticipate the planned dose increase and recognize that this is part of the normal treatment protocol, not an indication that their condition is worsening. They should also be aware that if they experience problematic side effects, their provider may decide to maintain the initial dose rather than increasing it at week four, as supported by guidelines for pharmacologic therapy for PAH in adults 1. Additionally, patients should be informed about the potential risks and benefits of the medication, as well as the importance of regular follow-up appointments to monitor their condition and adjust treatment as needed, in line with recommendations for the management of PAH 1.

From the FDA Drug Label

Advise patients to read the FDA-approved patient labeling (Patient Information and IFU). Discuss the following with patients prior to and during treatment with WINREVAIR. Erythrocytosis Caution patients that WINREVAIR may raise Hgb to levels that increase their risk of thrombotic events Inform patients that Hgb levels will be assessed before at least the first 5 doses and then periodically, as dosage may need to be adjusted [see Warnings and Precautions (5. 1)]. Severe Thrombocytopenia Caution patients that WINREVAIR may cause platelet count to decrease, which if severe could cause bleeding. Serious Bleeding Inform patients of the possibility of serious bleeding, which is more likely to occur if they have low platelet counts or while on prostacyclin background therapy and/or antithrombotic agents.

The key counseling points for a patient initiating sotatercept based on the methods used in and findings from the STELLAR study include:

• Erythrocytosis: The medication may increase the risk of thrombotic events due to elevated hemoglobin levels.
• Severe Thrombocytopenia: The medication may cause a decrease in platelet count, leading to an increased risk of bleeding.
• Serious Bleeding: The patient should be informed of the possibility of serious bleeding, especially if they have low platelet counts or are on certain background therapies. 2

From the Research

Key Counseling Points for Sotatercept

• The medication sotatercept may increase the risk of nose bleeds and bleeding of the gums, and patients should notify their healthcare provider if the bleeding is severe and persists 3.
• There is no mention in the provided studies of the need for daily monitoring with a blood pressure cuff or the requirement to administer the medication with food to optimize the benefit.
• The STELLAR study is mentioned in the context of sotatercept improving 6-minute walk distance, pulmonary vascular resistance, and NT-proBNP levels in patients with PAH 4.
• Common adverse events associated with sotatercept include epistaxis and telangiectasia, with some patients experiencing serious bleeding events 3.
• Patients should be informed of the potential risks and benefits of sotatercept, as well as the importance of reporting any adverse events to their healthcare provider 3, 4, 5, 6, 7.

Administration and Monitoring

• Sotatercept is administered subcutaneously, with the dosage and frequency determined by the healthcare provider 3.
• Patients should be monitored for adverse events, including bleeding and other potential side effects 3, 4.

Patient Education

• Patients should be educated on the proper use of sotatercept, including administration, potential side effects, and the importance of reporting any adverse events to their healthcare provider 3, 4, 5, 6, 7.