10. Long-term Management: Educate yourself!

10.1. Seek the help of a specialist

In addition to seeing a primary care physician (GP) regularly, people diagnosed with adrenal insufficiency should consult with an endocrinologist (a specialist for hormone disorders) on a regular basis.

They should request that the endocrinologist

• Prescribes an emergency injection kit (Solu-Cortef 100mg with syringe and needles) and demonstrates its use.
  
• Provides a letter that describes the condition and importance of immediate medical attention and treatment in case of an adrenal crisis. (see: Sample letter, under development).
  
• Explains the need for taking extra hydrocortisone in the case of an illness, accident, acute trauma or before a surgical procedure (“stress-dosing“), how to prevent and prepare for an adrenal crisis.

Patients should also inform their doctor/specialist if they are prescribed any new drugs as those may influence the cortisol metabolism and may require adjusting of the daily hydrocortisone dose. A pharmacist may also be able to address any concerns.

In primary adrenal insufficiency of autoimmune origin the doctor should enquire about symptoms and signs of other autoimmune disorders and undertake relevant testing every 6–12 months if needed.

10.2. Be your own advocate

Remember: Adrenal insufficiency is a rare condition.

Not every health care professional has encountered a patient with adrenal insufficiency during his or her career and therefore may not be up-to-date on treatment guidelines and / or protocols.

Everyone diagnosed with adrenal insufficiency should play an important part in the management of this chronic condition to ensure his/her own wellbeing.

Every person living with adrenal insufficiency and their family and friends should know and understand:

• Continuous (life-long, daily) rigorous cortisol replacement therapy is essential for life.
• Medication requirements can change and doctors should monitor for any new symptoms which might require adjustment of doses.
• During physical or mental stress the usual cortisol dose may need to be increased.
• Stress dosing is required in circumstances such as accident, injury, fever, diarrhoea, vomiting, influenza or extreme emotional stress to prevent an adrenal crisis.
• Symptoms of under-replacement and the beginning symptoms of an adrenal crisis.
• When and how to give an emergency injection of Solu-cortef.
• Medical staff must always be notified when undergoing planned or emergency surgical procedures.

10.3. Adrenal Crisis: Don’t stress, but be prepared

Approx. 8 – 10 % of all people living with adrenal insufficiency will experience at least one adrenal crisis per year.
Therefore, everyone diagnosed should:

• Always carry a medical alert bracelet and an emergency letter from their doctor explaining their condition and including the contact information of their GP and endocrinologist
• Always carry spare medication (tablets).
• Always carry an emergency kit consisting of Solu-Cortef, syringes and needles for injection
• Order repeat prescriptions in plenty of time – ideally maintaining a month’s reserve supply – to ensure they do not run out of essential medication.
• Take an extra supply of medication (e.g. double what they would normally need) when travelling or on holidays plus the injection kit.
• Carry the medication and injection kit in their hand luggage when travelling by plane, along with a doctor’s note explaining why they need to carry medications, needles and syringes.

More on the topic and helpful tips can be found in our Library.

> NEXT CHAPTER

> CHAPTER OVERVIEW
Anatomy/Physiology | Types of Adrenal Insufficiency | Symptoms | Testing/Diagnosis | Treatment | Stress dosing/Sick day Management | Adrenal Crisis | Quality of Life and Risks | Other Conditions and Drugs | Long-term Management | Suggested Reading | Literature/References

Author: Gisela Spallek, MD PhD
Edited by Maria Stewart, Director of AIC and deputy editor Des Rolph, Associate Director of AIC