Hello folks -
I was invited over here via Twitter, thought I'd say hello and introduce myself :-)
I'm one of those rare creatures…an actual confirmed MODY patient! I have HNF4α (also known as MODY 1, although doctors in the UK tend not to use that terminology) - I had the genetic testing done by the lab in Exeter, along with several of my family members.
I was diagnosed as "type 2" aged about 25, not overweight and with a very strong family history of diabetes - my Mum and both of her sisters were all previously thought to be freak cases of type 2. It's also very likely that their mother (my Grandmother) had it, although she was never diagnosed, and her brother (my Great Uncle) was thought to be type 1 but we now suspect he may have had the family MODY too. I had the genetic test for MODY done via my GP surgery after reading an article about it in Diabetes UK's Balance magazine a few years ago, and once I was diagnosed the rest of the family followed.
I'm treated with a tiny dose of Gliclazide (40mg a day), which works really well and gives me really good control - I have a very strong dose response to Gliclazide, which I think is a feature of some types of MODY.
I'm just a patient, definitely *not* a doctor or a medical expert in any way, but I'm reasonably well-versed in MODY (you need to be when you spend as much time as I do explaining to doctors what it is!) so hopefully I may be able to provide some information, support and solidarity! I don't give out medical advice though, I know my limits…so don't be too disappointed if my answers are often "consult your doctor".
So, anyway, hello, it's nice to meet you all :-)
I'm also MODY-1, with a transcription of HNF4α.
I was diagnosed a little later in life than you - mostly because my doctors relied on fasting blood sugars far too much (and ignored symptoms that I reported.)
I was the first in my family to have genetic testing done for confirmation...
Still waiting for others to follow suit.
At the moment, I'm controlling with strict diet and exercise...
If you don't mind me asking,
what do you consider good control, and what is your diet like?
Hello Knightshade, nice to meet you! I think you're the first 4α I've "met" who wasn't my family :-)
Re. control I've been pretty lucky so far (I'm still young...ish!), HbA1C generally between 5 & 6. I eat a healthy balanced diet, but don't follow any particular special diet - I'm pretty happy with how I manage it at the moment.
I'm sure you already know, there's a huge amount of variation in MODY patients, even within the same family - my Mum had really good control from just diet for years and years, others are on insulin.
Your the first person that I've talked to with the HNF4α form of MODY as well...
Guess that we are a rare breed :-)
So far, my HbA1c has always been in that 5-6 range as well... even without medications.
Going to a moderate carb diet (~80g/day) I've brought it down to 5.3, and usually keep my 2 hour pp numbers < 120 mg/dl
I've been curious about the variation of MODY's manifestation - in that the rest of my family was diagnosed as Type 1 in their teens and being insulin dependent.... mine has manifested itself much milder....
From everything that I've read, with HNF4α insulin production declines at a rate of 1-4% per year... I've been curious if that means that it is only a matter of time until mediations (and eventually insulin) are needed.
There does seem to be such a huge variation between individuals that it's certainly difficult to predict when you might need to move on to medications! The impression I get from what I've read is that most (all??) patients do need them eventually.