Approved by the Food and Drug Administration in 1991, Alglucerase is a synthetic hormone used for managing the symptoms of Gaucher's disease. It is a modified version of human Î²-glucocerebrosidase enzyme, whereby the non-reducing ends of the oligosaccharide chains are terminated with mannose residues. Approved as an enzyme replacement therapy, Alglucerase is administered intravenously to treat Type 1 Gaucher's disease - a genetic condition in which the body fails to produce enzymes needed to break down lipids.
Lipid build up in the body can cause symptoms such as persistent weakness, easy bruising and bleeding, anemia, joint and bone pains, liver and spleen enlargement, and weakening and fracturing of bones. Alglucerase is thus administered to improve the condition of the bones, liver, spleen and blood cells in patients with Type 1 Gaucher disease. It is important to note, however, that alglucerase does not offer complete cure for the condition.
Patients treated with alglucerase (brand name Ceredase) have reported a range of side effects. Some of these side effects were related to mode of administration and they include pruritus, discomfort, and swelling, burning or sterile abscess at the location of venipuncture. Other side effects include slight fever, abdominal discomfort, chills, vomiting and nausea. These side effects are mild hence do not necessitate medical attention.
Some patients have exhibited symptoms of hypersensitivity after alglucerase therapy. Onset of these symptoms tend to occur during or shortly after infusion. They include flushing, pruritus, urticaria/angioedema, chest pains, respiratory symptoms and abdominal cramping. Some patients also suffered from hypotension immediately after alglucerase treatment. Pretreatment with antihistamines and reduced infusion rate have been adopted to minimize the side effects of alglucerase therapy.
Call your doctor for advice or report these side effects to the FDA through the toll-free line 1-800-FDA-1088
Alglucerase is administered by intravenous infusion over 1-2 hours. The dosage should be customized for each patient. The starting dosage could be as little as 2.5 units/kg of the patient's body weight 3 times a week. Based on severity, some patients can receive up to 60 units/kg given as frequently as once per week spread through a period of 3- 4 weeks. The severity of the disease may require that the therapy is initiated with relatively high doses or slightly higher frequency. Once the patient's response is established, a reduction in the dosage should be adapted for purposes of maintenance therapy. Progressive reduction can be implemented at 3-6-month intervals while monitoring the patient's response.
Alglucerase should never be shaken during infusion. In addition, each bottle should be visually examined for discoloration or suspended matter before use. Any bottle exhibiting discoloration or presence of particulate matter should be discarded. Never administer expired alglucerase.
During use, the appropriate amount of alblucerase injection should be diluted with 0.9% sodium chloride IV solution to a volume not exceeding 200mL. Aseptic technique should be used when diluting the dose. When diluted to 100 to 200mL, alglucerase remains stable for up to 18 hours when stored at 2-8 degrees centigrade.
Relatively low toxicity, in combination with the extended time course of response makes it possible for small dosage adjustments to be made periodically. As such, the dosage administered in a single infusion session may be increased or decreased slightly to fully utilize each bottle as long as the patient's monthly dosage is not altered.
While certain medications should never be used on combination at all, there are cases where two different alglucerase may be used in combination with other medications if there are no interactions. It is important that you disclose any medication you are currently taking to your doctor before the onset of this therapy. Your doctor will advise you to avoid alcohol and tobacco products when you are on alglucerase treatment.
Approximately 13% of patients treated with alglucerase developed IgG antibody to the medication during the first year of treatment. Approximately one-quarter of patients who developed IgG antibodies exhibited symptoms of hypersensitivity. Thus, patients with antibody to aglucerase injection have a higher risk of experiencing hypersensitivity. However, not all patients who exhibit hypersensitivity have detectable antibody. Decreased efficacy has been reported in less than 0.5% of treated patients due to antibodies to alglucerase injection.
Alglucerase treatment should be directed by healthcare providers knowledgeable in management of patients suffering from Gaucher disease. Treatment should be conducted with caution in patients who have exhibited hypersensitivity symptoms to the product. Such patients should be pre-treated with antihistamines before continuation of the therapy.
Alglucerase injection should be used with caution in individuals with androgen sensitive malignances such as prostate cancer as well as individuals with prior allergies to hCG.
Alglucerase injection is prepared from the placental tissue that may host the causative agents of some viral diseases. Stringent manufacturing steps have been put in place to reduce the risk of transmission of such agents. However, the risk of contamination from latent or slow acting viruses like the Creutzfeldt-Jacob disease have not been tested and eliminated. That said, the risks and benefits of alglucerase treatment should be assessed prior to the therapy.
There is no substantial data on the effect of alglucerase injection to unborn babies as well as a woman's reproductive capacity. Therefore, this treatment should only to administered to expectant women as a last resort.
Since the drug may be passed on to human milk, caution should be taken when administering alglucerase to nursing mothers.
Since hCG has been detected in alglucerase injection, healthcare providers should be on the lookout for signs of early virilization in males 10 years and below. A single case of precocious puberty has been linked to alglucerase treatment. However, due to introduction of manufacturing steps aimed at reducing the level of hCG in alglucerase injection, the likelihood if this occurrence has been reduced significantly.
This drug should be stored in tightly closed bottles in the refrigerator. However, it should not be frozen. Keep the drug away from sunlight.
Alglucerase injection (brand name Ceredase) is a synthetic enzyme designed for long-term therapy in patients suffering from Type I Gaucher disease. The treatment is administered to patients who exhibit signs and symptoms that are severe enough to in any of the following symptoms:
Alglucerase injection acts as a catalyst to glycolipids hydrolysis (glucocerebroside) inside the reticuloendothelial system's lysosomes. This way, the enzyme restores the deficiencies found in patients with Gaucher's disease. The drug works by containing the complications while relieving the symptoms associated with Gaucher disease.
Alglucerase is administered through intravenous infusion over the course of 1-2 hours per dose. The drug is administered on a case-to-case basis depending on the state of the patient. The dosage may vary from 2.5 units per kilogram of body weight, given up to 3 times a week to a maximum of 60 units per kilogram administered once per week or month. The most common dose is 60 units per kilogram every fortnight. In patients with severe conditions, the therapy may be administered at a high amount or frequently at the onset of the treatment. Upon reaching a positive response, the dosage should be reduced to maintenance treatment levels. Gradual dosage reduction can be done at 3-6-month intervals while carefully monitoring the patient's response to reducing dosage.
Tests performed with over 1000 patients treated with alglucerase have shown cases of side effects, some of which are linked with the administration method. However, most of the drug's side effects are severe enough to warrant medical intervention. On rare occasions, patients treated with this drug have exhibited symptoms of hypersensitivity. Patients who exhibit symptoms of hypersensitivity should be withdrawn from the drug and these symptoms treated before resuming the therapy. Finally, patients are advised to inform their healthcare providers of any prescription or over the counter medication they may be taking before onset of alglucerase therapy.