Enzyme Replacement Therapy (ERT) for HPP

In 2015 an Enzyme Replacement Therapy (ERT) was approved in Europe for long-term enzyme replacement therapy in patients with paediatric-onset hypophosphatasia to treat the bone manifestations of the disease.

Supportive care
Earlier supportive care for hypophosphatasia was aimed at dealing with the systemic manifestations of the disease:

• Analgesics for the management of bone, muscle and joint pain.
• Surgery to reduce elevated intracranial pressure or repairment of fractures.
• Vitamin B6 for seizures.
• Dental care to maintain good dental health
• Treatment of hypercalcemia/hypercalcuria
• Respiratory support

Surgery
Treatment strategies for surgical repairs may differ for patients with hypophosphatasia. Fractures and pseudofractures are common for patients with hypophosphatasia and may require different types of surgical interventions.

Unsuitable (contraindicated) treatments
High doses of Vitamin D, calcium supplements and biphosphonates should not be given if hypophosphatasia can be suspected, as it has been shown that these agents may worsen symptoms of hypophosphatasia.