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MANAGEMENT AND TREATMENT
It is very difficult to know exactly where to start; FA is very complicated but there are many day-to-day considerations, outside formal medical treatment / intervention. People with FA are at a higher risk of Cancer than the rest of the population and their chromosomes break down very easily. As a result, every (reasonable) measure should be taken to avoid carcinogenics. Some suggestions may not be feasible or practical but "every little bit helps". This list is not exhaustive and will be constantly updated but here are a few helpful pointers:
1. Avoid cigarette (also cigar and pipe) smoke. Did you know that tobacco smoke contains over 4000 chemicals, many of which are carcinogenic or toxic?
2. Try to avoid fuelling the car, whilst an FA-sufferer is in the vehicle. If you can't avoid this, close all windows and vents, and switch off "blowers".
3. Try to avoid open fires, wherever possible; oil or gas central heating are preferable. This includes bonfires (and fireworks) - is it possible to watch from further away or better still, from inside, looking out a window?
4. Industrial smoke also contains carcinogens, so if at all possible, it is better to avoid industrial areas and try to live in a more rural, less industrial setting. (You will notice that the word "try" is used extensively within this page of advice).
5. Try to avoid food that is "burnt" to any degree, even slightly. Charring food actually produces small quantities of potent carcinogens, similar to those in cigarette smoke.
6. Minimise exposure to Ultraviolet radiation; avoid UV lamps (sunbeds, etc.) and cover up when in the sun, preferably with clothing and hat, or high-factor sun protection.
7. Avoid building work, where possible, due to some of the products involved.
8. Where possible, avoid carcinogenic chemicals: Arsenic is associated with lung and skin cancers and is found in fungicides and herbicides; Asbestos is associated with lung cancer and although no longer used,it can be found in certain types of older contruction, primarily roofing, floor tiles and some fire-resistant materials; Benzene is associated with Leukaemia and Hodgkin Lymphoma and is found in solvents, fumigants (pest control), paints, detergents and adhesives; Cadmium is associated with prostate cancer and is found in metal paintings and coatings; Chromium is associated with lung cancer and is found in paints, pigments and preservatives; Ethylene Oxide is associated with Leukaemia and is found in ripening agents and fumigants; Radon is associated with lung cancer and is found in cellars and poorly ventilated places. (Information obtained from http://en.wikipedia.org/wiki/Carcinogen and corroborated with other websites).
Androgens:
Approximately half of FA patients respond well to androgens (male hormones), which stimulate the production of red blood cells, and often, platelets. Sometimes white cell production is stimulated as well. This treatment may be effective for many years, but most patients eventually fail to respond. It is essential that the use of androgens is considered in the context of an eventual bone marrow transplant, as their use may affect adversely the ultimate success of a transplant.
Growth factors:
Haematopoietic (blood-stimulating) growth factors are also used. G-CSF stimulates the production of white blood cells and seems to be effective in FA patients. Other growth factors may be effective in combination.
Bone marrow transplantation:
At the present time, this is the only long-term cure for the blood defects in FA. This treatment has many risks associated with it, and the risks are compounded in FA patients because of their extreme sensitivity to radiation and chemotherapy. The consensus of the Doctors who participated in March 2003 in the development of the handbook Fanconi Anemia: Standards for Clinical Care (in corroboration with FARF) is that, if a transplant centre has had experience with fewer than 5 matched sibling donor transplants for FA, strong consideration should be given for referral to a transplant centre with significant experience in transplants for FA. FA patients often experience complications which are not routine for other transplants, such as a marked increased risk in organ toxicity and in graft-versus-host disease (GVHD) and development of glucose intolerance, with most FA patients requiring insulin therapy.
Information provided on this page about medications, treatments or products should not be construed as medical instruction or scientific endorsement. Always consult your Specialist / Medical Professional before taking any action based on this information.
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Fanconi Anaemia FAmily Support