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

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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).

Strensiq® has been evaluated in 99 patients 1 day to 58 years of age with perinatal/infantile- and juvenile-onset hypophosphatasia (HPP).1,a

Most patients were treated with Strensiq® for more than 2 years (range: 1 day up to 6.5 years for some patients).1

Study 1
(ENB-002-08/ENB-003-08)1,10

Study Design

  • Prospective, single-arm trial and extension (N=11 patients with severe perinatal/infantile-onset hypophosphatasia)
  • Patients who completed trial ENB-002-08 were enrolled in the extension (ENB-003-08)
  • Inclusion Criteria: Onset of hypophosphatasia symptoms <6 months of age, with biochemical and radiographic evidence and a medical history of hypophosphatasia as well as the presence of any of the following: rachitic chest deformity, vitamin B6-dependent seizures, or failure to thrive

Patient Characteristics

  • Age: 3 weeks to 3.3 years at baseline

Endpoints

  • Survival and Ventilation-Free Survival compared with historical controls
  • Change in hypophosphatasia-related rickets (as measured by the Radiographic Global Impression of Change [RGI-C] scale)
  • Growth (as measured by height and weight z scores)
Study 2
(ENB-010-10)1,9

Study Design

    • Prospective, open-label trial (N=59 patients with perinatal/infantile-onset hypophosphatasia)
    • Open-label, nonrandomized study of the safety and efficacy of Strensiq in patients up to 6.5 years of age
    • Inclusion criteria: Onset of hypophosphatasia symptoms <6 months of age, with biochemical and radiologic evidence and a medical history of hypophosphatasia

Patient Characteristics

  • Age: 1 day to 6.5 years at baseline

Endpoints

  • Survival and Ventilation-Free Survival compared with historical controls
  • Change in hypophosphatasia-related rickets (as measured by the RGI-C scale)
  • Growth (as measured by height and weight z scores)
Study 3
(ENB-006-09/ENB-008-10)1,9,11

Study Design

  • Prospective, open-label trial and extension (N=13; 5 patients with perinatal/infantile-onset hypophosphatasia and 8 patients with juvenile-onset hypophosphatasia)
  • All patients with juvenile-onset hypophosphatasia entered the extension study (ENB-008-10) and were treated for ≥48 months

Patient Characteristics

Age: 6 to 12 years of age at baseline

  • 38% (5/13) of patients had onset of symptoms <6 months of age
  • 62% (8/13) of patients had onset of symptoms ≥6 months of age

Endpoints

Change in:

  • Hypophosphatasia-related rickets (as measured by the RGI-C scale) compared with historical controls
  • Growth (as measured by height and weight z scores) compared with historical controls
  • Mobility (as measured by the 6-minute walk test [6MWT]b)
  • Gait (as measured by the Modified Tinetti Performance-Oriented Mobility-Gait [MPOMA-G] scale)
  • Running speed and agility (BOT-2)
  • Bone mineralization
Study 4
(ENB-009-10)9

Study Design

  • Randomized, open-label, dose-ranging trial and concurrent control study and extension (N=19; including 10 adult patients with perinatal/infantile- or juvenile-onset hypophosphatasia)

Patient Characteristics

10 adult patients with perinatal/infantile- and juvenile-onset hypophosphatasia were 26 to 58 years at baseline

  • 10% (1/10) of patients had onset of symptoms <6 months of age
  • 90% (9/10) of patients had onset of symptoms ≥6 months and ≤18 years of age

Endpoints

Change in:

  • Alkaline phosphatase substrate levels (inorganic pyrophosphate [PPi] and pyridoxal-5’-phosphate [PLP])
  • Mobility (as measured by 6MWTc)
  • Bone mineralization

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.
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