Table 4 Key-interventions to treat patients with HH

Screening

1. First-degree relatives of HH patients must be screened.

Treatment/management

Phlebotomy

2. HH patients with raised ferritin levels must start treatment with (bi)weekly phlebotomy (removing 400–500 ml of blood).

3. The ferritin target level for HH patients ‘on treatment’ is between 50–100 μg/L.

4. HH patients without indicators of significant liver disease (AST and/or ALT elevation), but with elevated ferritin, must also proceed to phlebotomies.

5. HH patients with advanced liver fibrosis or cirrhosis can safely undergo phlebotomy and must also be treated.

6. HH patients undergoing phlebotomies must be advised to take adequate hydration before and after treatment and avoid vigorous physical activity for 24 h after treatment.

Examinations

7. Screening for liver fibrosis or cirrhosis in HH patients must be performed and can be performed using either transient elastography or biopsy.

General issues

8. HH patients without evidence of iron overload must be monitored annually and treated when the ferritin rises above normal.

9. HH patients must be immunized against hepatitis A and B.

10. HH patients with cirrhosis must receive yearly influenza and 5-yearly pneumococci vaccination.

11. HH patients with cirrhosis must be screened every 6 months for focal liver lesions (by ultrasound and serum alpha fetoprotein).

12. HH patients must be assessed and managed for complications (liver disease, diabetes mellitus, joint diseases, endocrine deficiency (hypothyroidism), cardiac disease, porphyria cutanea tarda and osteoporosis).

13. Fasting glycemia and/or HbA1c must be measured yearly in HH patients.

14. HH patients who have complaints compatible with osteoarthritis, must undergo physical and radiological evaluation.

Diet/lifestyle

15. HH patients in the iron depletion phase must avoid the intake of alcohol.