Tips for Maintaining a Personal Health Record When You Have Adrenoleukodystrophy

Tips for Maintaining a Personal Health Record When You Have Adrenoleukodystrophy

Adrenoleukodystrophy (ALD) is a rare disease characterized by the accumulation of saturated very-long-chain fatty acids (VLCFAs) that can destroy the myelin sheath and damage the adrenal glands, leading to symptoms that include both neurological problems and adrenal insufficiency (Addison’s disease).

A number of treatment options aim to slow disease progression, and keeping a track of these therapies and outcomes with their use can be tedious. This is where a personal health record (PHR) comes in handy.

Here are a few pointers on what PHRs are, and how they can be useful in managing your treatment.

Why is a PHR needed?

An ALD diagnosis involves a multitude of tests and decisions regarding treatment approaches, which complicates the tracking of disease progress and therapy effectiveness. A PHR can help consolidate this information. It also allows your entire medical history to go with you, if you switch doctors or are traveling.

Finally, PHRs can be very useful in providing vital information to caregivers and first responders in case of emergencies, such as adrenal crises.

PHRs generally are available in two formats — standard or standalone, and tethered.

What is a standard PHR?

A standard PHR is a collection of the many medical reports you may have accumulated over time. These include results that you provide of diagnostic tests, progress reports, and notes from doctor visits.

Creating a standalone PHR can be quite time consuming, and requires due diligence especially if you are just starting out. However, you are in control of the information that goes into a standalone PHR. You also have the flexibility of creating a standalone PHR as a physical collection of documents, or you can opt for a web-based service that can be accessed across all your devices.

What is a tethered PHR?

A tethered PHR ties into your healthcare provider’s electronic health record (EHR). A tethered PHR offers instant access to your medical history for both you and your doctor, and is usually kept up to date by your healthcare provider after every visit. Some tethered PHRs may also offer additional analytic tools to help your doctor make informed decisions.

While tethered PHRs can save you the hassle of collecting and updating information, access to them may be limited based on time and location depending on the healthcare provider’s policies. Check with your healthcare provider about such limitations.

You may also be required to pay a nominal fee to access a tethered PHR, but remember that a patient’s legal medical record is protected under the Health Insurance Portability and Accountability Act Privacy Rule. This law ensures that patients have the right to view, request changes, and get copies of their stored medical information as needed.

What information should go into a PHR?

A PHR, regardless of whether it is standalone or tethered, should contain the following information:

  • Your name and complete contact details
  • The contact details of your caregivers or family and friends to contact in case of an emergency
  • Details of your current health insurance plan, and the contact details of the agent or insurance provider
  • A description of your symptoms, the age at onset, and their severity
  • A calendar containing information about upcoming doctor visits
  • Information about prescribed diagnostic tests and their results
  • The type of ALD established at your diagnosis
  • Information about genetic counseling sessions, if any
  • Information about aids and adaptive devices you may be using and their effectiveness
  • Information about any known family history of ALD
  • Details of any ongoing therapies, such as Lorenzo’s oil, corticosteroid therapy, or stem cell therapy
  • Any other information you think would be relevant, such as the details of a medical alert ID or an emergency steroid wallet (ESW).

How much does it cost to maintain a PHR?

A standalone PHR that is maintained by you should be free or inexpensive unless you opt for a web-based service, which may have a one-time access fee or an annual recurring fee.

The cost of tethered PHRs is tied to a hospital’s EHR and may be covered in your treatment plan. In some cases, you may be charged to access additional information or retrieve data beyond a certain time period, so check with your healthcare provider.


Last updated: Jan. 14, 2019


Adrenoleukodystrophy News 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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