Treatment
Treatment for juvenile hemochromatosis is similar to that for other forms of hemochromatosis and iron overload, and focuses on reducing the amount of iron in the body in order to prevent complications of iron overload. However, if the disease is not discovered early enough, or if progress is not well controlled, further treatments may be aimed at the symptoms of organ damage which may develop. Phlebotomy
Phlebotomy, the removal of blood from the body, is the main treatment for juvenile hemochromatosis. One unit of blood, the amount typically given during blood donation, is typically removed per session, and it is generally recommended that this be done once weekly until acceptable levels of iron are in the blood, which may take years. After these levels are reached, phlebotomy will be continued, but less often than once weekly, perhaps every few months. Chelation Therapy
In the event that phlebotomy is not an appropriate option or is not enough on its own to reduce iron levels, chelation medications, those that bind and remove certain metals from the blood, may be utilized. Examples of chelators specifically for iron include deferoxamine and deferasirox. Dietary Modification
It is recommended that those with juvenile hemochromatosis refrain from eating iron supplements, vitamin C supplements, and uncooked/undercooked seafood and shellfish, and reduce or eliminate consumption of alcoholic beverages and red meat. Additional Treatments
If the disease is advanced enough, further treatments can be aimed at the complications of the disease, depending on which are present:
Diabetes may be treated with oral medications and/or insulin as indicated.
In such cases, closed reduction followed by immobilization in a thumb spica cast and serial radiography may be all that is required for effective treatment. For Bennett fractures where there is between 1 mm and 3 mm of displacement at the trapeziometacarpal joint, closed reduction and percutaneous pin fixation (CRPP) with Kirschner wires is often sufficient to ensure a satisfactory functional outcome. The wires are not employed to connect the two fracture fragments together, but rather to secure the first or second metacarpal to the trapezium. For Bennett fractures where there is more than 3 mm of displacement at the trapeziometacarpal joint, open reduction and internal fixation (ORIF) is typically recommended.Regardless of which approach is employed (nonsurgical, CRPP, or ORIF), immobilization in a cast or thumb spica splint is required for four to six weeks. Prognosis
If intraarticular trapeziometacarpal fractures (such as the Bennett or Rolando fractures) are allowed to heal in a displaced position, significant post-traumatic osteoarthritis of the base of the thumb is virtually assured. Some form of surgical treatment (typically either a CRPP or an ORIF) is nearly always recommended to ensure a satisfactory outcome for these fractures, if there is significant displacement.The long-term outcome after surgical treatment appears to be similar, whether the CRPP or the ORIF approach is used. Specifically, the overall strength of the affected hand is typically diminished, and post-traumatic osteoarthritis tends to develop in almost all cases. The degree of weakness and the severity of osteoarthritis does however appear to correlate with the quality of reduction of the fracture.