Stay Connected

GET UPDATES
Learn more about Strensiq® (asfotase alfa) and HPP.

Please enter the following information:

Thank you for signing up to receive the latest information about Strensiq® (asfotase alfa).

Your registration on Strensiq.com ensures that you will be among the first to receive news and updates about Strensiq and hypophosphatasia (HPP).

Safety
Information
Click for Important Safety Information

Strensiq® promoted substantial improvements in hypophosphatasia (HPP)-related rickets in patients with perinatal/infantile- and juvenile-onset hypophosphatasia.1

  2. Home >
  3. Clinical Data >
  4. Skeletal Improvement
 

RGI-C scale

Hypophosphatasia-related rickets was assessed using the RGI-C scale.1,14

 
 

Radiographic Global Impression of Change (RGI-C) Scale1,14

 
 

 

RGI-C evaluates change in paired radiographs of the chest and bilateral wrists and knees1,14

Scores measure change from baseline at prespecified time points14

 
 
 

Radiographic examples

 
 
An example of substantial healing of hypophosphatasia-related rickets10,15,a

There were insufficient data to determine the effect of Strensiq on fractures in patients with perinatal/infantile- and juvenile-onset hypophosphatasia1

aHypophosphatasia-related rickets was assessed in patients with perinatal/infantile-onset hypophosphatasia in Studies 1, 2, and 3 using the RGI-C scale.1
bRadiographs are from a patient in Study 1.10
 
 
Example of a responder showing substantial healing of hypophosphatasia-related rickets10,15,a

aRadiographs are from a patient in Study 1.10
 
 
 

Results in juvenile-onset hypophosphatasia

100% (8/8) of patients with juvenile-onset hypophosphatasia treated with Strensiq® had substantial or complete healing of hypophosphatasia-related rickets.1,a

 
 

Patients with substantial or complete healing of hypophosphatasia-related rickets1,a

 
 
 
 
 
Example of a responder showing substantial healing of hypophosphatasia-related rickets10,15,a

aRadiographs are from a patient in Study 3.16
 

INDICATION & IMPORTANT SAFETY INFORMATION

INDICATION

STRENSIQ® is indicated for the treatment of patients with perinatal/infantile- and juvenile-onset hypophosphatasia (HPP).

IMPORTANT SAFETY INFORMATION

WARNINGS AND PRECAUTIONS
  • Hypersensitivity Reactions, including anaphylaxis, have been reported in STRENSIQ-treated patients. Signs and symptoms consistent with anaphylaxis included difficulty breathing, choking sensation, nausea, periorbital edema, and dizziness. These reactions have occurred within minutes after subcutaneous administration of STRENSIQ and and have been observed more than 1 year after treatment initiation. Other hypersensitivity reactions have also been reported in STRENSIQ-treated patients, including vomiting, fever, headache, flushing, irritability, chills, skin erythema, rash, pruritus and oral hypoesthesia.
    Inform patients and/or caregivers of the signs and symptoms of hypersensitivity reactions and have them seek immediate medical care should signs and symptoms occur. If a severe hypersensitivity reaction occurs, discontinue STRENSIQ treatment and initiate appropriate medical treatment. Consider the risks and benefits of re-administering STRENSIQ to individual patients following a severe reaction. If the decision is made to re-administer the product, monitor patients for a reoccurrence of signs and symptoms of a severe hypersensitivity reaction.
  • Lipodystrophy: Localized lipodystrophy, including lipoatrophy (depression in the skin) and lipohypertrophy (enlargement or thickening of tissue), has been reported at injection sites after several months in patients treated with STRENSIQ in clinical trials. Advise patients to follow proper injection technique and to rotate injection sites.
  • Ectopic Calcifications: Patients with HPP are at increased risk for developing ectopic calcifications. Events of ectopic calcification, including ophthalmic (conjunctival and corneal) and renal (nephrocalcinosis, nephrolithiasis), have been reported in the clinical trial experience with STRENSIQ. There was insufficient information to determine whether or not the reported events were consistent with the disease or due to STRENSIQ. No visual changes or changes in renal function were reported resulting from the occurrence of ectopic calcifications.
    Ophthalmology examinations and renal ultrasounds are recommended at baseline and periodically during treatment with STRENSIQ to monitor for signs and symptoms of ophthalmic and renal ectopic calcifications and for changes in vision or renal function.
  • Possible Immune-Mediated Clinical Effects: In clinical trials, most STRENSIQ-treated patients developed anti-asfotase alfa antibodies and neutralizing antibodies which resulted in reduced systemic exposure of asfotase alfa. In postmarketing reports, some STRENSIQ-treated patients with initial therapeutic response subsequently developed recurrence and worsening in disease-associated laboratory and radiographic biomarkers (some in association with neutralizing antibodies) suggesting possible immune-mediated effects on STRENSIQ’s pharmacologic action resulting in disease progression. The effect of anti-asfotase alfa antibody formation on the long-term efficacy of STRENSIQ is unknown. There are no marketed anti-asfotase alfa antibody tests. If patients experience progression of HPP symptoms or worsening of disease-associated laboratory and imaging biomarkers after a period of initial therapeutic response to STRENSIQ, consider obtaining anti-asfotase alfa antibody testing by contacting STRENSIQ Medical Information at Alexion at 1-888-765-4747 or by email at medinfo@alexion.com. Close clinical follow up is recommended.
ADVERSE REACTIONS

Overall, the most common adverse reactions (≥ 10%) reported were injection site reactions (63%). Other common adverse reactions included lipodystrophy (28%), ectopic calcifications (14%), and hypersensitivity reactions (12%). Possible immune-mediated clinical effects have been identified during post-approval use of STRENSIQ.

DRUG INTERACTIONS

Drug Interference with Laboratory Tests:
  • Laboratory tests utilizing alkaline phosphatase (ALP) as a detection reagent could result in erroneous test results for patients receiving treatment due to the presence of asfotase alfa in clinical laboratory samples. Inform laboratory personnel that the patient is being treated with STRENSIQ and discuss use of an alternative testing platform which does not utilize an ALP-conjugated test system.
  • Elevated serum ALP measurements detected through clinical laboratory testing are expected in patients receiving STRENSIQ due to circulating concentrations of asfotase alfa and may be unreliable for clinical decision making.
SPECIAL POPULATIONS
  • Pregnancy & Lactation: There are no available data on STRENSIQ use in pregnant women, the presence of STRENSIQ in human milk, or the effects on the breastfed infant or on milk production, to inform a drug associated risk.
Please see STRENSIQ (asfotase alfa) full Prescribing Information.
 

Additional information on trial design and endpoints

 
 
 

Information on growth improvement

 
 

Please enter the following information:

*Indicates a required field.

Please review our Terms & Conditions in addition to our Privacy Policy. By submitting your registration, you are agreeing to the terms of use and our Privacy Policy.

You can unsubscribe from further communications from Alexion Pharmaceuticals, Inc. at any time.

Please enter the following information:

*Indicates a required field.

*Preferred method of contact is only in response to unsolicited request for medical information