EFMODY

Posology Treatment should be initiated by physicians experienced in the management of CAH. As maintenance therapy the dose must be individualised according to the response of the individual patient. The lowest possible dose should be used.

g. surgery, infection, trauma). As the treatment has a modified release profile, blood tests are used to monitor clinical response, assessment of the evening dose should be done with a morning blood test and assessment of the morning dose should be done with an early afternoon blood test.

During excessive physical and/or mental stress it may be necessary to increase the dose of Efmody, and/or add additional immediate release hydrocortisone especially in the afternoon or evening. 5). Treatment in CAH Recommended replacement doses of hydrocortisone are 10-15 mg/m2/day in adolescents aged 12 years and over who have not completed growth, and 15-25 mg/day in adolescents who have completed growth and adult patients with CAH.

In patients with some remaining endogenous cortisol production a lower dose may be sufficient. At initiation the total daily dose should be split into two doses with two thirds to three quarters of the dose given in the evening at bedtime and the rest given in the morning.

Patients should then be titrated based on their individual response. The morning dose should be taken on an empty stomach at least 1 hour before a meal and the evening dose taken at bedtime at least 2 hours after the last meal of the day.

Changing from conventional oral glucocorticoid treatment to Efmody When changing patients from conventional oral hydrocortisone replacement therapy to Efmody, the identical total daily dose should be given, but the dose should be given in two doses with two thirds to three quarters of the dose given in the evening at bedtime and the rest given in the morning.

When changing patients from other glucocorticoids to Efmody an appropriate conversion factor should be used, and the patient monitored for response carefully. Conversion to Efmody might elicit symptoms of adrenal insufficiency or overreplacement during dose optimisation.

A starting dose exceeding 40 mg per day of hydrocortisone is not recommended. 4). e. a doubled daily dose of hydrocortisone) to allow for easy return to the normal replacement dose of Efmody once additional hydrocortisone is no longer required.

8%). 2%) were reported in the long-term extension study DIUR-006 for patients that were receiving a mineralocorticoid. Tabulated list of adverse reactions The commonest reactions reported to Efmody in the pooled population in the clinical trial population are tabulated below.

Frequencies are defined as very common (≥ 1/10), common (≥ 1/100 to < 1/10), uncommon (≥ 1/1,000 to < 1/100). Table 1-Tabulated summary of adverse reactions seen in clinical trial programme MedDRA system organ classification Event Frequency Endocrine disorders Adrenal insufficiency including acute events Common Increased appetite Common Decreased appetite Common Metabolism and nutrition disorders Impaired fasting glucose Common Insomnia Common Abnormal dreams Common Depressed mood Common Psychiatric disorders Sleep disorder Common Headache Common Dizziness Common Carpal tunnel syndrome Common Nervous System Disorders Paraesthesia Common Nausea CommonGastrointestinal disorders Abdominal pain upper Common Acne CommonSkin and subcutaneous tissue disorders Hair growth abnormal Common Arthralgia Common Muscle fatigue* Common Myalgia Common Musculoskeletal and connective tissue disorders Pain in extremity Common *Includes muscular weakness Description of selected adverse reactions Adrenal Insufficiency (including acute events).

Events of acute adrenal insufficiency were reported during the clinical trial programme but none were considered related to Efmody. 4). Rare instances of anaphylactoid reactions have occurred in patients receiving corticosteroids especially when a patient has a history of allergies to medicinal products.

4) - it is unclear if these relate to hydrocortisone therapy using current replacement regimens. Historical cohorts of adults treated from childhood for CAH have been found to have raised cardiovascular risk factors and a higher risk of cerebrovascular disease than the general population - it is unclear if these relate to hydrocortisone therapy using current replacement regimens.

Adrenal crisis Acute adrenal insufficiency may develop in patients with known adrenal insufficiency who are on inadequate daily doses or in situations with increased cortisol need. Therefore, patients should be advised of the signs and symptoms of acute adrenal insufficiency and of adrenal crisis and the need to seek immediate medical attention.

Sudden discontinuation of therapy with hydrocortisone risks triggering an adrenal crisis and death. 9%) solution for infusion, should be administered according to current treatment guidelines. Pre-operatively, during serious trauma or during intercurrent illness Pre-operatively, anaesthetists must be informed if the patient is taking corticosteroids or has previously taken corticosteroids.

Parenteral administration of hydrocortisone is warranted during transient illness episodes such as severe infections, in particular gastroenteritis associated with vomiting and/or diarrhoea, high fever of any aetiology or extensive physical stress, such as for instance serious accidents and surgery under general anaesthesia.

Where parenteral hydrocortisone is required, the patient should be treated in a facility with resuscitation facilities in case of evolving adrenal crisis. In less severe situations when parenteral administration of hydrocortisone is not required, for instance low grade infections, moderate fever of any aetiology and stressful situations such as minor surgical procedures, there should be high awareness of the risk of developing acute adrenal insufficiency.

2). Patients with CAH are at risk of life-threatening adrenal crisis during infection so clinical suspicion of infection should be high and specialist advice should be sought early. Immunisation Treatment schedules of corticosteroids for people with CAH do not cause immunosuppression and are not, therefore, contraindications for administration of live vaccines.

Undesirable effects of corticosteroid replacement therapy Most undesirable effects of corticosteroids are dose and duration of exposure related. Undesirable effects are therefore less likely when using corticosteroids as replacement therapy.

1.