Statement of Principles

concerning

CUSHING'S SYNDROME

No. 34 of 2009

for the purposes of the

Veterans’ Entitlements Act 1986

and

Military Rehabilitation and Compensation Act 2004

Title

1. This Instrument may be cited as Statement of Principles concerning Cushing's syndrome No. 34 of 2009.

Determination

2. The Repatriation Medical Authority under subsection 196B(3) and (8) of the Veterans’ Entitlements Act 1986 (the VEA):

(a) revokes Instrument No. 250 of 1995 concerning Cushing's syndrome; and

(b) determines in its place this Statement of Principles.

Kind of injury, disease or death

3. (a) This Statement of Principles is about Cushing's syndrome and death from Cushing's syndrome.

(b)              For the purposes of this Statement of Principles, "Cushing's syndrome" means an endocrine disorder resulting from an excess of endogenous or exogenous glucocorticoids.

(c)               Cushing's syndrome attracts ICD-10-AM code E24.0, E24.2, E24.3, E24.8 or E24.9.

(d)              In the application of this Statement of Principles, the definition of "Cushing's syndrome" is that given at paragraph 3(b) above.

Basis for determining the factors

4. On the sound medical-scientific evidence available, the Repatriation Medical Authority is of the view that it is more probable than not that Cushing's syndrome and death from Cushing's syndrome can be related to relevant service rendered by veterans or members of the Forces under the VEA, or members under the Military Rehabilitation and Compensation Act 2004 (the MRCA).

Factors that must be related to service

5. Subject to clause 7, at least one of the factors set out in clause 6 must be related to the relevant service rendered by the person.

Factors

6. The factor that must exist before it can be said that, on the balance of probabilities, Cushing's syndrome or death from Cushing's syndrome is connected with the circumstances of a person’s relevant service is:

(a)               having a neuroendocrine neoplasm at the time of the clinical onset of Cushing’s syndrome; or

(b)              having an adrenocorticotrophic hormone (ACTH) secreting neoplasm of the pituitary gland at the time of the clinical onset of Cushing’s syndrome; or

(c)               having micronodular or macronodular adrenal hyperplasia at the time of the clinical onset of Cushing’s syndrome; or

(d)              having an adrenal neoplasm at the time of the clinical onset of Cushing’s syndrome; or

(e)               having glucocorticoid therapy as specified before the clinical onset of Cushing’s syndrome, and where the glucocorticoid therapy as specified has ceased or decreased, the last dose of the therapy was received within the one month before the clinical onset of Cushing’s syndrome; or

(f)                being treated with medroxyprogesterone acetate or megestrol acetate for a malignant disease or human immunodeficiency virus infection, for at least one month before the clinical onset of Cushing’s syndrome, and where such treatment has ceased, the last dose of the treatment was received within the one month before the clinical onset of Cushing's syndrome; or

(g)              inability to obtain appropriate clinical management for Cushing's syndrome.

Factors that apply only to material contribution or aggravation

7. Paragraph 6(g) applies only to material contribution to, or aggravation of, Cushing's syndrome where the person’s Cushing's syndrome was suffered or contracted before or during (but not arising out of) the person’s relevant service.

Inclusion of Statements of Principles

8.                  In this Statement of Principles if a relevant factor applies and that factor includes an injury or disease in respect of which there is a Statement of Principles then the factors in that last mentioned Statement of Principles apply in accordance with the terms of that Statement of Principles as in force from time to time.

Other definitions

9.                  For the purposes of this Statement of Principles:

"a drug from the specified list" means:

(a)              amprenavir;

(b)              atazanavir;

(c)              darunavir;

(d)              fosamprenavir;

(e)              indinavir;

(f)               itraconazole;

(g)              ketoconazole;

(h)              lopinavir;

(i)                nelfinavir;

(j)                ritonavir;

(k)              saquinavir; or

(l)                tipranavir;

"a high or very high potency topical glucocorticoid" means:

(a)          betamethasone dipropionate 0.05%;

(b)          betamethasone valerate 0.1%;

(c)          clobetasol proprionate 0.05%;

(d)          diflucortolone valerate 0.1%;

(e)          fluocinolone acetonide 0.025%; or

(f)           another topical glucocorticoid of equivalent potency;

"a neuroendocrine neoplasm" means a non-pituitary neoplasm that secretes polypeptides functionally equivalent to adrenocorticotrophic hormone (ACTH) or corticotropin-releasing hormone (CRH) including oat cell or small cell lung carcinoma, carcinoid tumour, islet cell tumour, tumours of the thymus, medullary carcinoma of the thyroid, and phaeochromocytoma;

"an adrenal neoplasm" means a benign or malignant tumour arising from the adrenal gland;

"equivalent glucocorticoid therapy" means a glucocorticoid in the following table, at the doses specified in the table, or a therapeutically equivalent dose of another glucocorticoid:

"equivalent inhaled glucocorticoid" means:

(a)          8000 micrograms of triamcinolone;

(b)          1600 micrograms of budesonide;

(c)          1000 micrograms of fluticasone; or

(d)          a therapeutically equivalent dose of another inhaled glucocorticoid;

"death from Cushing's syndrome" in relation to a person includes death from a terminal event or condition that was contributed to by the person’s Cushing's syndrome;

"having glucocorticoid therapy as specified" means:

(a) taking:

(i) hydrocortisone, orally or by injection,

(A)        to a cumulative dose of at least 1500 milligrams, and

(B)        at a minimum dose rate averaging 50 milligrams per day, or

(ii) equivalent glucocorticoid therapy, orally or by injection; or

(b) inhaling at least 2000 micrograms of beclomethasone, or equivalent inhaled glucocorticoid, daily, for at least 6 months; or

(c) using an ocular or intranasal glucocorticoid at above the recommended maximum therapeutic dosage level, daily, for at least 6 months; or

(d) applying a high or very high potency topical glucocorticoid to at least 20% of total skin surface area, daily, for at least 6 months; or

(e) using a glucocorticoid concurrently with a drug from the specified list, daily, for at least 30 days; or

(f) using glucocorticoid containing enemas, daily, for at least six months;

"ICD-10-AM code" means a number assigned to a particular kind of injury or disease in The International Statistical Classification of Diseases and Related Health Problems, 10th Revision, Australian Modification (ICD-10-AM), Sixth Edition, effective date of 1 July 2008, copyrighted by the National Centre for Classification in Health, Sydney, NSW, and having ISBN 978 1 74210 016 6;

"a neuroendocrine tumour" means a non-pituitary neoplasm that secretes polypeptides functionally equivalent to adrenocorticotrophic hormone (ACTH) or corticotropin-releasing hormone (CRH) including oat cell or small cell lung carcinoma, carcinoid tumour, islet cell tumour, tumours of the thymus, medullary carcinoma of the thyroid, and phaeochromocytoma;

"relevant service" means:

(a)              eligible war service (other than operational service) under the VEA; or

(b)              defence service (other than hazardous service) under the VEA; or

(c)              peacetime service under the MRCA;

"terminal event" means the proximate or ultimate cause of death and includes:

(a) pneumonia;

(b) respiratory failure;

(c) cardiac arrest;

(d) circulatory failure; or

(e)               cessation of brain function.

Application

10. This Instrument applies to all matters to which section 120B of the VEA or section 339 of the MRCA applies.

Date of effect

11. This Instrument takes effect from 6 May 2009.

Dated this twenty-fourth day of  April 2009

The Common Seal of the  )

Repatriation Medical Authority )

was affixed to this instrument )

in the presence of:   )

KEN DONALD

CHAIRPERSON