Cablivi is administered through subcutaneous injection meaning that it is injected under the skin, typically in the thigh or abdomen. It is provided with plasma exchange (PEX) and immunosuppressive therapy, for instance, corticosteroids or rituximab treatment of aTTP.

The regular dosing regimen for Cablivi is as follows :

Loading dose: The patient will first receive a loading dose of initial 10 mg administered as a subcutaneous injection. This dose is usually given on the first day of treatment, followed by a second 10 mg dose on the next day (Day 2).

Maintenance dose: Cablivi is administered usually as a subcutaneous injection of 10 mg once daily during plasma exchange therapy, which often lasts for approximately 30 days after the loading doses.

Treatment post-plasma exchange: Post completion of plasma exchange, Cablivi treatment is normally continued for at least 30 days to minimize recurrence.

The treatment regimen may be different according to the individual patient’s response and his healthcare provider.

The mechanism of action of Cablivi is through its inhibition of von Willebrand factor which plays a central role in the development of thrombotic thrombocytopenic purpura (TTP). It helps the platelets to become attached to the blood vessels walls at sites of an injury during normal blood clotting. However, in TTP, vWF are abnormally cleaved thereby resulting in large, ultra-large vWF multimers that bind to platelets causing them to form microvascular clots.

Cablivi (caplacizumab) works by binding to the A1 domain of vWF, specifically preventing the interaction between vWF and platelets. By inhibiting this interaction, Cablivi reduces the formation of these microclots, thereby preventing further platelet consumption and reducing the risk of organ damage due to reduced blood flow.

In essence, Cablivi directly targets the pathological process driving aTTP, helping to normalize platelet levels and improve blood flow to affected organs, which is crucial in managing the disease.

As with all medications, Cablivi may cause side effects, but not everyone who takes the drug will experience them. Common and less serious side effects include:

Headache: Some patients may have mild to moderate headaches during treatment with Cablivi.

Injection site reactions: Common reactions include redness, pain, swelling, or irritation at the injection site.

Fatigue or tiredness: Some patients may feel unusually tired or fatigued, especially in the early stages of treatment.

It raises a risk of bleeding, primarily when used in combination with other anticoagulant or antiplatelet drugs. Patients should be monitored on signs of bleeding, for example, bruising and nosebleeds, more severe complications of bleeding, which may be associated with drug use.

Risk of infections: Cablivi also raises the risk of infection primarily when used in combination with immunosuppressive therapy. Patients should be followed up for signs of infections, and appropriate measures can be taken to prevent infections or treat them.

Pregnancy and breastfeeding: The safety of Cablivi during pregnancy and breastfeeding is not established. Cablivi should only be used during pregnancy when the potential benefits outweigh the risks, and it should not be used during breastfeeding.

Impairment of the kidneys and liver: Patients with the diseases of the kidneys or the liver should have their Cablivi dosages reduced or monitored very closely while on the medication. Because there is no dosing information for such patients, a careful management approach is mandatory.

Combination therapy: Cablivi is a combination therapy where plasma exchange, along with immunosuppressive agents, is commonly used. Interactions with these treatments need to be monitored closely for optimal outcome in the patient.

1. Anticoagulants: Cablivi, an inhibitor of the von Willebrand factor, can increase the risk of bleeding when taken with other anticoagulants like warfarin, rivaroxaban, or apixaban. Monitoring for signs of bleeding is crucial.
2. Platelet Aggregation Inhibitors: Co-administration with drugs like aspirin or clopidogrel, which reduce platelet aggregation, may enhance Cablivi’s bleeding risk, requiring careful assessment of bleeding tendencies.
3. CYP3A4 Inhibitors: Strong CYP3A4 inhibitors (e.g., ketoconazole, ritonavir) can elevate Cablivi plasma levels, increasing the risk of adverse effects like bleeding, necessitating monitoring and possible dose adjustments.
4. CYP3A4 Inducers: Medications that induce CYP3A4 (e.g., rifampin) can reduce Cablivi concentrations, potentially compromising its therapeutic effectiveness.

These interactions emphasize the need for close monitoring and adjustment when using Cablivi alongside other medications.

Induction phase:

First dose: 10 mg subcutaneously on Day 1.

Second dose: 10 mg subcutaneously on Day 2.

Maintenance phase: 

10 mg subcutaneously daily for the total duration of plasma exchange treatment, approximately about 30 days.

Post-treatment period:

The treatment is then continued for at least about 30 days after plasma exchange treatment to avoid relapse.

Cablivi should be prescribed by a healthcare provider experienced in treating rare blood disorders such as aTTP and due to potential severe bleeding complications, careful monitoring of response to therapy – including regular blood tests and assessments of platelet levels and organ function – shall be performed by the prescribing doctor.

1. What is Cablivi?
   Cablivi (caplacizumab) is a medication used to treat acquired thrombotic thrombocytopenic purpura (aTTP), a rare blood disorder characterized by blood clot formation in small blood vessels.

2. How does Cablivi work?
   Cablivi works by inhibiting von Willebrand factor (vWF), which plays a crucial role in platelet aggregation and clot formation, thereby helping to restore normal blood flow.

3. Who is a candidate for Cablivi treatment?
   Cablivi is indicated for adults diagnosed with aTTP, particularly those experiencing recurrent episodes of the condition.

4. How is Cablivi administered?
   Cablivi is administered through subcutaneous injection, typically done in a healthcare setting, during the initial phase of treatment.

5. What is the recommended dosage of Cablivi?
   The typical dosage is 11 mg, initially given as an injection once daily for the first 30 days, followed by a transition to once every two weeks.

6. What are the common side effects of Cablivi?
   Common side effects include headache, injection site reactions, nausea, fatigue, and mucocutaneous bleeding.

7. Can Cablivi be used during pregnancy?
   The use of Cablivi during pregnancy should be discussed with a healthcare provider, as safety data in pregnant women is limited.

8. What precautions should be taken while using Cablivi?
   Patients should be monitored for signs of bleeding or thrombosis, especially during the initial treatment phase, and any unusual symptoms should be reported to a healthcare provider immediately.

9. What should I do if I miss a dose of Cablivi?
   If a dose of Cablivi is missed, it should be administered as soon as remembered. However, if it’s close to the next scheduled dose, skip the missed dose and continue with the regular schedule.

10. Are there any serious side effects associated with Cablivi?
    Serious side effects can include severe bleeding and allergic reactions. If you experience symptoms such as difficulty breathing or swelling, seek medical attention immediately.

11. Can Cablivi interact with other medications?
    Yes, Cablivi can interact with other medications, especially those that affect blood clotting. Inform your healthcare provider about all medications and supplements you are taking.

12. How is the effectiveness of Cablivi monitored?
    Effectiveness is monitored through regular laboratory tests, including blood counts and assessments of platelet levels, to ensure that treatment is effective and safe.

13. Can Cablivi be used in elderly patients?
    Yes, Cablivi can be used in elderly patients, but they may require closer monitoring for potential side effects, especially related to bleeding.

14. What lifestyle changes can support treatment with Cablivi?
    Maintaining a healthy lifestyle, staying hydrated, eating a balanced diet, and regular follow-ups with healthcare providers can support treatment effectiveness.

15. Is Cablivi suitable for patients with liver or kidney problems?
    Patients with liver or kidney issues should discuss their conditions with their healthcare provider, as dosage adjustments or closer monitoring may be necessary.

16. What should I do if I experience significant bleeding while on Cablivi?
    Seek immediate medical attention if you experience significant bleeding, such as unusual bruising, prolonged bleeding from cuts, or blood in urine.

17. What storage conditions are recommended for Cablivi?
    Cablivi should be stored in a refrigerator at 2°C to 8°C (36°F to 46°F) and protected from light. Do not freeze.

18. Is Cablivi covered by health insurance?
    Coverage for Cablivi may vary depending on insurance providers. Patients should check with their insurer for details about coverage and copays.

19. Are there alternatives to Cablivi for treating aTTP?
    Other treatments for aTTP may include plasma exchange and immunosuppressive therapy, but the choice of treatment should be made by a healthcare provider based on individual patient needs.

20. How can I learn more about Cablivi?
    For more information, consult your healthcare provider or pharmacist, who can provide more personalized guidance and answer any specific questions about your treatment plan with Cablivi.