XLH and Surgery – Important Considerations

XLH and Surgery – Important Considerations
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X-linked hypophosphatemia (XLH) is a rare, progressive, genetic disease caused by mutations in the PHEX gene. The disease is characterized by low levels of phosphate — an important mineral for the formation of healthy bones and teeth — in the blood.

Although there is currently no cure for XLH, there are several treatment options available to manage the symptoms. For example, doctors may use surgery to correct severe skeletal deformities.

Here are a few points to keep in mind about surgery.

Discuss your medical history with the doctor

Before surgery, tell your doctor about allergies to any medications, and the current treatments you are taking.

If you have been taking medication for diabetes or using blood thinners, be sure to inform your doctor.

Carry reports of all diagnostic tests, such as X-ray imaging and blood tests, and discuss them in detail with your doctor.

Prepare yourself for surgery

It is important that you prepare yourself for the surgery. Your doctor or a team of surgeons will inform you about do’s and don’ts before and after the procedure. You may have to undergo a pre-surgery checkup to ensure you are in good health for the operation.

Avoid strenuous physical activity after surgery

Surgery in XLH patients usually involves fixing bone deformities or replacing joints, which can take between six and 12 weeks to heal. During this time, you should avoid strenuous physical activity, such as lifting weights.

Parents of children who have undergone surgery for XLH should apprise the school of the situation and ensure the child does not carry heavy loads to and from school.

It also is very important to walk carefully and protect against falling.

Use appropriate aids and adaptations, and have a friend or family member nearby to help you move around, especially in the days just after surgery.

Comply with therapies after surgery

After the surgery, your doctor may prescribe medication, such as antibiotics and pain relievers. Doctors may also prescribe phosphate supplements and active vitamin D, or put you on a Crysvita (burosumab) regimen to promote bone development.

They may also recommend physiotherapy and occupational therapy to protect and strengthen bones and muscles. For a speedy recovery, make sure you comply with your doctor’s advice.

Have regular follow-ups

Doctors will regularly assess the physical and functional outcome of the surgical correction after the operation. For example, they will follow the outcome of osteotomy to correct bent legs for up after 12 months to assess whether the deformity has been fixed or has worsened.

 

Last updated: March 12, 2020.

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XLH News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. 

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Özge has a MSc. in Molecular Genetics from the University of Leicester and a PhD in Developmental Biology from Queen Mary University of London. She worked as a Post-doctoral Research Associate at the University of Leicester for six years in the field of Behavioural Neurology before moving into science communication. She worked as the Research Communication Officer at a London based charity for almost two years.

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