How it works

Hypophosphatasia (HPP) prevents healthy bone mineralization,1,2 while STRENSIQ promotes it3

STRENSIQ is the first and only approved enzyme replacement therapy indicated for perinatal/infantile- and juvenile-onset HPP,3 supported by 7 years of clinical data.4-8

Plasma and protein molecules
Alkaline phosphatase How STRENSIQ works Need to treat

About alkaline phosphatase (ALP)

HPP is a rare, inherited metabolic disorder in which deficient ALP activity disrupts bone mineralization throughout life.1,2

Hydroxyapatite crystal
Hydroxyapatite crystal

ALP is essential for bone mineralization10

ALP cleaves phosphate (Pi) from inorganic pyrophosphate (PPi) so it can combine with calcium (Ca2+) to form hydroxyapatite crystal.10

ALP is essential for bone mineralization10

ALP cleaves phosphate (Pi) from inorganic pyrophosphate (PPi) so it can combine with calcium (Ca2+) to form hydroxyapatite crystal.10

How STRENSIQ works

STRENSIQ replaces the deficient tissue-nonspecific alkaline phosphatase (TNSALP) enzyme3

  • By replacing ALP, STRENSIQ enables bone mineralization and reduces toxic levels of systemic PPi and pyridoxal 5ʹ-phosphate (PLP)3
    • STRENSIQ cleaves Pi from PPi so it can combine with calcium to form hydroxyapatite crystal, which is necessary to make healthy bone3,10
    • Reductions in plasma PPi and PLP did not correlate with clinical outcomes3
DRAG THE SLIDER TO SEE THE DIFFERENCE
Strensiq® replaces the deficient tissue-nonspecific alkaline phosphatase (TNSALP) enzyme (1)
Strensiq® replaces the deficient tissue-nonspecific alkaline phosphatase (TNSALP) enzyme (2)

Need to treat

Dangers of low ALP

In HPP, low ALP activity leads to harmful accumulation of the substrates PPi and PLP, impairing bone mineralization and leading to accumulating physical burden, pain, and poor quality of life.1,11,12

Without STRENSIQ, deficient ALP activity and disrupted bone mineralization will not improve.1,11-14

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Important Safety Information and Indication for STRENSIQ® (asfotase alfa) 40mg/mL vial
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 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.
Indication

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

Please see STRENSIQ (asfotase alfa) full Prescribing Information.
Important Safety Information and Indication for STRENSIQ® (asfotase alfa) 40mg/mL vial
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 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.
Indication

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

Please see STRENSIQ (asfotase alfa) full Prescribing Information.
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References

1. Rockman-Greenberg C. Hypophosphatasia. Pediatr Endocrinol Rev. 2013;10(suppl 2):380-388.

2. Whyte MP. Hypophosphatasia. In: Bilezikian J, Martin TJ, Clemens T, Rosen C, eds. Principles of Bone Biology. 3rd ed. Academic Press; 2008;2:1573-1598.

3. STRENSIQ [package insert]. Alexion Pharmaceuticals Inc.

4. Whyte MP, Rockman-Greenberg C, Moseley S, Denker AE, McAlister WH. Sustained radiographic and functional improvements with asfotase alfa treatment for up to 7 years in children with hypophosphatasia. Poster presented at: 13th International Congress of Inborn Errors of Metabolism; September 5-8, 2017; Rio de Janeiro, Brazil.

5. Whyte MP, Simmons JH, Moseley S, et al. Asfotase alfa for infants and young children with hypophosphatasia: 7 year outcomes of a single-arm, open-label, phase 2 extension trial. Lancet Diabetes Endocrinol. 2019;7(2):93-105.

6. Hofmann CE, Harmatz P, Vockley J, et al. Efficacy and safety of asfotase alfa in infants and young children with hypophosphatasia: a phase 2 open-label study. J Clin Endocrinol Metab. 2019;104(7):2735-2747.

7. Whyte MP, Madson KL, Phillips D, et al. Asfotase alfa therapy for children with hypophosphatasia. JCI Insight. 2016;1(9):e85971.

8. Högler W, Rockman-Greenberg C, Petryk A, Zhou S, Whyte M, Bishop N. Long-term efficacy profile of asfotase alfa in the treatment of patients with hypophosphatasia: a pooled analysis. Poster presented at: 9th Biennial International Conference on Children’s Bone Health (ICCBH); June 22-25, 2019; Salzburg, Austria.

9. Feng X. Chemical and biochemical basis of cell-bone matrix interaction in health and disease. Curr Chem Biol. 2009;3(2):189-196.

10. Orimo H. The mechanism of mineralization and the role of alkaline phosphatase in health and disease. J Nippon Med Sch. 2010;77(1):4-12.

11. Weber TJ, Sawyer EK, Moseley S, Odrljin T, Kishnani PS. Burden of disease in adult patients with hypophosphatasia: results from two patient-reported surveys. Metabolism. 2016;65(10):1522-1530.

12. Rush ET, Moseley S, Petryk A. Burden of disease in pediatric patients with hypophosphatasia: results from the HPP Impact Patient Survey and the HPP Outcomes Study Telephone Interview. Orphanet J Rare Dis. 2019;14(1):201.

13. Whyte MP, Leung E, Wilcox WR, et al. Natural history of perinatal and infantile hypophosphatasia: a retrospective study. J Pediatr. 2019;209:116-124.

14. Szabo SM, Tomazos IC, Petryk A, et al. Frequency and age at occurrence of clinical manifestations of disease in patients with hypophosphatasia: a systematic literature review. Orphanet J Rare Dis. 2019;14:85.

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