Name of the Procedure:

Injection, idursulfase, 1 mg (HCPCS Code: J1743)
Common Names: Idursulfase Injection, Elaprase Injection

Summary

Injection of idursulfase is a medical procedure used to treat Hunter syndrome, a rare genetic disorder. This treatment involves administering idursulfase, an enzyme replacement therapy, directly into the bloodstream to help break down complex molecules that build up in the body due to the illness.

Purpose

Medical Conditions Addressed:

• Hunter syndrome (Mucopolysaccharidosis II)

Goals/Expected Outcomes:

• Reduce symptoms and prevent disease progression related to Hunter syndrome.
• Improve the patient’s quality of life by managing the buildup of glycosaminoglycans (GAGs).

Indications

Symptoms/Conditions:

• Respiratory issues, joint stiffness, and growth delays associated with Hunter syndrome.
• Accumulation of heparan sulfate and dermatan sulfate in tissues.

Patient Criteria:

• Diagnosed with Hunter syndrome.
• Typically administered to male patients, as the condition is X-linked.

Preparation

Pre-procedure Instructions:

• No fasting required.
• Review of current medications by healthcare provider to avoid drug interactions.
• Routine blood tests and liver function tests may be recommended.

Diagnostic Tests:

• Genetic testing to confirm Hunter syndrome.
• Baseline assessments including physical exams and possibly imaging studies.

Procedure Description

Steps:

1. Preparation: The patient is positioned comfortably, and an IV line is established.
2. Administration: Idursulfase is infused into the vein over a period of 3 to 4 hours.
3. Monitoring: Vital signs are monitored throughout the infusion to watch for any adverse reactions.

Tools/Equipment:

• IV catheter
• Infusion pump
• Heart rate and blood pressure monitors

Anesthesia/Sedation:

• Not typical for this procedure, but medications may be given to manage potential allergic reactions.

Duration

• Typically takes between 3 to 4 hours for each infusion session.

Setting

• Administered in a hospital, outpatient clinic, or specialized infusion center.

Personnel

• Healthcare providers involved include:
  • Physicians or specialist nurses trained in enzyme replacement therapy.
  • Clinical pharmacists to prepare the medication.
  • Nurses to monitor the patient during the infusion.

Risks and Complications

Common Risks:

• Fever, headache, and skin reactions at the injection site.

Rare Risks:

• Serious allergic reactions (anaphylaxis).

• Respiratory distress.

  Management of Complications:

• Emergency care with epinephrine and antihistamines for severe reactions.
• Supportive care for minor side effects.

Benefits

• Significant reduction in GAG buildup in tissues.
• Improvement in breathing, joint mobility, and overall physical function.
• Benefits might be observed after several months of regular treatment.

Recovery

Post-procedure Care:

• Observation for any delayed allergic reactions.
• Keep hydrated and resume normal activities as tolerated.
• Follow-up visits scheduled to monitor progress and adjust dosing if necessary.

Recovery Time:

• Minimal recovery time; patients can typically go home the same day and resume normal activities quickly.

Alternatives

• Symptomatic treatment approaches such as physical therapy, surgical interventions for specific complications, and supportive care.
• Pros and Cons: Alternatives may not address the root cause (enzyme deficiency) and might be less effective in halting disease progression compared to enzyme replacement therapy.

Patient Experience

During the Procedure:

• Patients may feel pressure at the IV site and could experience mild discomfort.
• Monitoring and support provided throughout to ensure comfort.

After the Procedure:

• Patients might experience mild fatigue or warmth, which usually resolves shortly.
• Most are able to carry on with daily routines soon after the infusion.

Pain Management:

• Over-the-counter pain relief may be recommended for minor discomfort.
• Healthcare team provides reassurance and addresses any concerns about side effects promptly.