Name of the Procedure:

Injection of C1 esterase inhibitor (human), Cinryze, 10 units
Technical term: HCPCS J0598

Summary

In simple terms, the injection of Cinryze involves delivering a specific protein into the bloodstream to help prevent or reduce the symptoms of a condition known as hereditary angioedema (HAE). HAE is characterized by severe swelling in various parts of the body.

Purpose

This injection is used to treat hereditary angioedema (HAE), a genetic condition that causes painful, recurrent episodes of swelling in different parts of the body. The goal of the Cinryze injection is to replace the deficient or dysfunctional C1 esterase inhibitor protein, thereby preventing or reducing the frequency and severity of swelling attacks.

Indications

• Diagnosis of hereditary angioedema confirmed through diagnostic testing.
• Patients experiencing frequent and/or severe HAE attacks.
• Situations where prophylactic treatment to prevent HAE attacks is necessary.

Preparation

• No specific fasting or dietary restrictions are typically required.
• The patient may need to inform the healthcare provider of any current medications, especially blood thinners.
• Baseline diagnostic tests might include blood tests to confirm C1 esterase inhibitor deficiency.

Procedure Description

1. The patient will typically be seated or lying down comfortably.
2. Using aseptic technique, a healthcare professional will prepare the injection site, usually on the upper arm or thigh.
3. Cinryze comes in a powder form that must be reconstituted with a provided diluent.
4. Once reconstituted, the solution is drawn into a syringe.
5. The syringe is used to inject the solution intravenously or subcutaneously, depending on the specific protocol.
6. The injection process is monitored closely to manage any immediate adverse reactions.

Duration

The procedure generally takes about 15-30 minutes, including preparation and the injection itself.

Setting

This procedure can take place in various settings such as a hospital, outpatient clinic, or specialized infusion center.

Personnel

• A registered nurse or a trained healthcare professional administers the injection.
• A medical doctor or healthcare provider supervises and provides orders for the treatment.

Risks and Complications

• Common risks: Mild pain or discomfort at the injection site, bruising, and swelling.
• Rare risks: Allergic reactions, which could include rash, itching, or more severe symptoms like difficulty breathing.
• Possible complications are managed by immediate medical intervention if necessary.

Benefits

• Reduced frequency and severity of HAE attacks.
• Improved quality of life due to fewer and less severe swelling episodes.
• Benefits can be noticed within hours to a few days after the injection.

Recovery

• Patients can generally return to normal activities shortly following the injection.
• Post-procedure instructions might include monitoring the injection site for signs of infection or adverse reactions.
• Follow-up appointments will likely be scheduled to assess the effectiveness and manage any ongoing treatment needs.

Alternatives

• Alternative treatments include other C1 esterase inhibitors (e.g., Berinert, Haegarda).
• Antifibrinolytics and kallikrein inhibitors may also be used.
• Each alternative has its own pros and cons related to efficacy, frequency of administration, and side effects.

Patient Experience

During the procedure, patients might feel a slight pinch or discomfort at the injection site. Post-injection experiences can vary, but most patients resume normal activities shortly after. Pain management and comfort measures may include applying a cold pack to the injection site if there is any swelling or discomfort.