So i thought I would try and explain briefly what MODY is for any of you guys are confused (i'll admit it is confusing).
There are 2 types of monogenic diabetes, neonatal diabetes and MODY
neonatal diabetes is usually diagnosed within the first 6 months of life. it is often diagnosed as type 1 as not alot of doctors are aware of the condition. It can be treated with oral diabetes medications from the sulphonylurea group of drugs. Children who are treated with insulin have the chance of stopping and going onto tablets. Anyone diagnosed at 6 months old or younger shoudl be tested for this.
MODY- stands for maturity onset diabetes of the young. which is misleading as it implies type 2 in young people, that is NOT the case. MODY is not type 1 or type 2, but shares similarities with both types.
It is a monogenic type of diabetes, which means a single gene is involved. the gene is dominant. So if a parent has the gene the children will have a 50% chance of getting the gene and therefore getting diabetes.
there are 6 identified genes that casue MODY the 2 most common are MODY 2 and MODY 3
MODY 2 is caused by a defect in the glucokinase gene, this type is almost like a faulty thermostat, the level is set too high, so fasting blood sugar is elevated but these people tend not to see spikes in blood sugar and only have slightly elevated HbA1c's. it is thought that these people do not get complications as they have very stable blood sugars. these people will have higher fasting numbers but will pass glucose tolerance tests.
MODY 3 is the most common form of MODY and is due to a defect in the HNf1alpha gene. peoplw with this type of MODY have normal insulin secretion as children but this reduces with time and diabetes is usually diagnosed by 25 years of age (but can be alot earlier or later) as the diabetes progresses.
people can often initially manage on oral sulphonyureas to which they are very sensitive to, needing alot smaller doses than type 2's. but as the diabetes progresses insulin is needed in alot of people, the time frame differs for each individual, some people can go 30 years before insulin is needed, some only 3 years. when insulin is used it tends to be very small doses due to people with MODY being very insulin sensitive and also natural insulin production will still exist.
There is always a family history of diabetes although it may have been diagnosed as type 1 or type 2 in other relatives. a feature of people with MODY 3 is they spill glucose into the urine at normal blood glucose levels.
people who have been diagnosed with type 1 who are found to actually have MODY have the chance to stop insulin and go onto tablets. but this must be done under supervision of experienced doctors.
people with MODY are very unlikely to get DKA due to natural insulin production, they may have very high blood gluocse levels but don't get the assosiated high ketone levels.
in the US gene testing for MODY may or may not be covered by insurance, but it can be expensive.
in the UK it is free on the NHS
I have tried to explain in simple terms as I can, if anything is unclear please ask, or any more questions I will try to answer.
Nikki age 23
dxd MODY 3 at 15