Most cases of CMC are first recognized in childhood. Babies or children may have repeated episodes of oral thrush or sometimes thrush in the gullet affecting swallowing ( Candida oesophagitis ).
Repeated episodes of oral thrush or a single episode of Candida oesophagitis indicate that the immune system cannot defend the body against Candida. Further testing of the immune system has to be done . This shows that the person’s immune cells (lymphocytes) do not respond as they should to Candida. This is the reason for the recurrent episodes. Those that occur in childhood or in infancy are almost always genetic. The precise genetic defect has not been identified but the pattern of inheritance suggests that affected children have got two copies of an abnormal gene, one from each parent, just as in Cystic Fibrosis. Occasionally, there are reasons other than CMC, which account for the recurrent thrush. Blood tests help in deciding whether someone has CMC or not.
Although most cases of CMC occur in childhood, thee are a number of cases that first appear in adulthood, sometimes as late as 50 years of age. Cases that occur in adulthood are not so clearly linked to a genetic defect and it is not known why these patients develop CMC.
In some of those with genetically acquired CMC other problems occur as the children get older. Although the immune system is unable to fight off Candida, it appears to be overactive in certain body tissues. If this happens, damage to endocrine organs such as the adrenals and ovaries , and also to the skin can occur in adolescence or early childhood. Some patients develop pale patches on their skin (vitiligo), other patients lose some or all of their hair, other patients need various forms of hormone replacement. The precise reason for these additional diseases occurring later in life is not known. These problems only rarely occur in patients who develop CMC as adults, which also suggests that there is a different cause of their CMC.
The treatment of oral thrush, Candida oesophagitis or vaginal Candidiasis is fairly straightforward. Oral anti-fungal antibiotics such as fluconazole and itraconazole are highly effective in treating the problem. Usually a course of one or two weeks therapy is given to start with. If the Candida infection recurs quickly, as it usually does in CMC patients, then continued long term treatment with one of these drugs is appropriate. They have very few side effects and patients have been treated for many years with them quite safely. One of the concerns about treating with any antibiotic or anti-fungal is that resistance may appear over time. Resistance is now an increasing problem in other patients who get Candida infections , such as Intensive Care patients , leukemia patients and patients with AIDS. It is though, very unusual in patients with CMC. However, if breakthroughs occurs on therapy, the most likely reason for it is the development of resistance.
The underlying reason why patients with CMC get many episodes of Candida is not known. There is no cure in sight for this disease at present. In the long term it might be possible to consider gene replacement therapy, as this has been tried for Cystic Fibrosis and other genetic defects. The first step, however, will be to demonstrate what the actual defect in CMC is. This requires additional research.
Information supplied by Dr David Denning – Manchester