My Diet with POMC - The POMC Series

Hello there everybody!

How is everybody today? Hope all is well. Today's post in The POMC Series is all about my diet and how I cope with the hyperphagia caused by POMC deficiency disorder. I've always been very quiet and conscious of my diet but recently I've realised that my diet is actually a lot better than I thought. This post is not meaning to be bragging rights at all, like "oh my diet is so much better than yours" because the fact is it's not better than anybody's my diet is mine because this works well for me, I'm not always excessively hungry every second of the day nor do I skip or miss meals. 

I've heard from discussions recently that some people with POMC tend to keep an extremely low calorie diet and that seems to be going alright for some people and kudos to you if you've found a diet that seems to fit you and your body or needs because that's not an easy thing to do whatsoever, I mean take your average person, you try and find a diet that is satisfying, healthy and gives you all the nutrients you need at 1,000 calories or even much less in some circumstances... it's an exceptionally difficult thing to do, so well done! 

This post is also not going to be a complete run down of every single tiny thing I eat and drink each day because... well that would just be boring for me to write and probably for you to read, however if you would like me to do something like that in the future contact me privately and I shall look into putting something like that together to post. 

Anyway, I shall get on with the post right now. Overall, I've been to countless dieticians, exercise specialists and such, I've spoken to at least 5 different endocrine specialists who deal with my POMC from the RVI, my main doctors and nurses form Cambridge and each doctor and nurse has given me a different answer (with obvious similarities in the case of the specialists) to the question: "Which diet is best for me?"
Over the years I've tried many diets from carb-free, low fat, vegetarian, vegan and gluten free but none worked. Obviously, a dietician will tell me low fat, low sugar, low everything pretty much with a very low calorie intake, however doctors have told me (those from Cambridge and the RVI) that if I can maintain an exercise regimen with roughly equal output to my intake of calories and energy I should be able to manage my weight slightly easier, although it'll still have little effect on weight gain as I've been told with POMC it's extremely difficult to lose and much too easy to gain weight as the body retains as much energy from food as possible. Therefore from all the information I've gathered I've managed to set up a diet of roughly 1,200 calories per day, I've also recently been made aware that a high protein diet, the right kind of protein that is, can help in some circumstances with feeling hungry so often, I was recommended that snacks like nuts and Skyr yoghurt which is high in protein and very low in fat are snacks that may help. 

However, in terms of breakfasts I am, as I've said before unable to eat bread, it's just something about the texture and taste that makes me feel sick so I don't eat much of that whatsoever. I also struggle to eat on a morning at the best of times so breakfasts for me tent to be things such as homemade fruit smoothies and cereals like those high in fibre such as bran and oaty cereals, this allows me to get my fibre as well as getting fruit and natural sugars, my grandfather is friends with a man who often gives us fresh fruit and veg that he grows which is much nicer than supermarket produce, so it's very much homegrown stuff in the smoothies, skimmed or almond milk that kind of thing. Very tasty and surprisingly filling. If you make a larger one as well you could always take that with you to school, uni or work and make it last throughout the day. Stops me snacking at least. 
Again lunches tend to be kept very low in fat, small homemade pasta pots, soups, rice boxes usually keep me going, I love vegetables so grilled veg couscous is one of my favourite things, I usually have some kind of desert to go along with it and I enjoy making fruit boxes so I'll just make a mini fruit salad with different types of fruit or a small pot of mixed seeds and low fat vegan chocolate mixed in, although that one is a treat as I love seeds and chocolate!
Snacks tend to include a lot of nuts and seeds as I mentioned, sometimes I'll just buy a bag of carrots and snack on those until I'm satisfied, more yoghurt or crunching ice cubes are pretty much all I do for snacks.
Dinners for me are often much more protein based with fish playing a big part, definitely a fan of omega 3 over here ha! I enjoy experimenting with spices as does Simon so I'm quite happy to try different kinds of meats, vegetables and so on, usually dinner times are just a concoction of things we have thrown together (I make a mean shredded chicken salad as well!). It's really important for me that I keep an extremely low fat, low sugar and salt diet, protein is higher on my priority list than anything else, usually it's meat I crave anyway but honestly I do manage to stay under 1100 calories almost all of the time. Sometimes I go out to eat but that I on occasion every now and then, never a regular occurrence unless it needs to be as I tend to make my own lunches so I know exactly what is going into by body. When I do go out I love things like Japanese food so Nudo and YO! Sushi are two of my favourites and a lot of things on YO! Sushi also have the calories and nutritional value next to them on the menu so you know exactly what you can get and how it'll all add up at the end of the night. Most of the time I avoid things that are greasy or fast food because I know it'll make me feel sick as well as not do anything for my weight gain. 
in terms of drinks I only drink fizzy drinks they are now a no-go, I mainly rink water and flavoured water, always still. 

I've heard people many times over the years claim that food is the enemy with POMC warriors and that's all well and good if that is how you like to view it but in my personal opinion that's not how I ever want to view my condition. I see my body as doing me a favour... go with me for a sec. See, my body stores as much energy as it can because it doesn't work properly, therefore it's wires are crossed and thinks that I'm going to starve if I don't eat or get hungry a lot of the time (now I know this is the incorrect way of thinking about it but it helps me understand myself a bit better). So, because my body thinks it is helping me by storing all the energy and such from food it's up to me to control my own diet and exercise in order to guide my body in the right direction, almost like helping a child who has gotten something a bit wrong and needs some help, if you understand what I'm saying? That was very long winded ha. 

Once again, I thank everybody for reading this post and coming back to my blog, I hope if anybody is reading this with POMC or a child with POMC/LEPR or Prader Willi Syndrome recently diagnosed I hope I've helped a little.
xo. 

Life Update and Exciting Future Projects!! The POMC Series

Hi again everybody! 

Image from google.com

So, it's been over a week since I made my return to blogging and explained that the focus and style of the blog would shift significantly and oh my gosh the support has been absolutely amazing! I truly cannot thank everybody enough for all of the comments, likes, shares, retweets and such. Most of all I would like to thank everyone who has taken the time to read the posts, to talk about them with their families, I've managed to get in contact with some of you whom have been reading the blog and honestly your stories are so touching and heart-warming. I'm really glad some of the posts have helped you guys and I'm overjoyed that the posts have given some even a slightly better understanding of Pro-opiomelanocortin deficiency disorder! That's the whole purpose of the blog. 

This post will be slightly shorter than usual but I just wanted to give you guys a very quick update on how life has been recently. Therefore, I shall begin with last week, I had been waiting on a phone call from my nurse at Cambridge hospital to find out whether or not I was eligible to take part in a drug trial, last Thursday she called me and turns out that because I only have one gene faulty in one pathway I'm not eligible just yet although she did say that if the drug works well on those with 2 faulty genes and doesn't need any development the tablet should be available to all of those with genetic obesity diseases. I was obviously devastated but that was actually what drove me to get my blog up and running and get so involved with fundraising and awareness raising for this condition; even if someone is not entitled to a drug to help them medically, their lives could be made better if there was more understanding and knowledge in the general public of this disease. 
Obviously you guys know how it went after starting the blog, I developed The POMC Series which is a series including any posts related to POMC and in the first week of blogging I've gained well over 1,000 views, I've connected with some amazing people and managed to do a couple of things I never dreamed I could with this page!

Now, since starting the blog up again a couple of things have happened, both of which I'm extremely excited and hopeful about these two things. First off, after posting the initial post about returning to blogging the President of the University Student's Union and vice president of wellbeing and engagement took and interest in it, who may I say are two of THE most amazing, kindest men I've ever had the true pleasure of meeting, made some absolutely wonderful comments about the blog and was far too kind about me. The president has always made me feel extremely comfortable and welcomed at the university and we met last year on freshers week and had to drag me to get my freshers band I was so nervous! Anyway I approached him after he made the comments and explained what my aim was and how could I work with the university in order to raise awareness and give a friendly face for those who may feel isolated or excluded from others. I had a meeting Tuesday passed which was very emotional and touching for us all I think and we have managed to set out some aims and overarching factors that we want to include or happen. 
Currently, we are scheduling another meeting for  sometime later in the first semester to discuss more ideas as we are hoping to maybe do something around the second semester, this is an extremely exciting turn of events and I'm feeling very confident and determined to carry this on!!

The second exciting thing that's happened is that on one of my posts that was shared by the Student Union president an amazing young woman and journalist has approached me and asked if she could conduct an interview with me about my condition and what has driven me to get to university, this opportunity is so great. I'm aware that this fabulous lady has worked at a number of newspapers and is an exceptionally talented and competent journalist so working with her this Monday is going to be very inspiring and enlightening. She currently writes for the university's newsletter so I will be making sure to ask permission to post that right here, ladies and gents! Keep your eyes open for more information about that. 
So, all in all everybody it's been a bit of a hectic week! I'm so excited to get all of these things underway. To be honest other than those major things all I have been up to is spending time with my friends, spending time with Simon, I had a day out the other day buying all of my stationary for uni which I'm always super happy about! I'm still trying to read the books I got from the Uni library to prepare for the next year (again massively excited about! ONLY 24 MORE DAYS!!), I've been planning, writing and posting blog posts for you all and finally me and Simon are getting ready to head off to York next week for 3 days which will be a long needed break away so I shan't be posting those few days although i will have a lot to post about when we get back!!

Exciting times are a'coming! 
Thanks for reading guys and I'll keep you up to date on everything going on, I'll also be posting a few more POMC related posts so look out for those! 
Have a great day! 
xo

Keep Fighting - The POMC Series

Hey again,

Hope everybody is well, this next post will be a short one as I'm lacking in energy today but definitely have a couple of posts coming up that you should find interesting.

I wasn't going to post today as me and the family went out for the day but I've decided that if qany day is a good day to demonstrate the reality of how POMC can affect people it's today.

Beautiful Whitby Town - North Yorkshire

Today was a really bad day, to be honest. I woke up feeling pretty good, well rested and such and I knew me and the family were heading off to Whitby for the day, one of my most adored places I've ever been to, and I was visibly excited. After a cup of tea I went to get s cold shower because I could feel myself beginning to overheat, despite windy and pretty cloudy weather, my body temperature kept going up and I was just sweating more and more so I took 5 minutes, tried to keep myself hydrated as much as possible and got myself dressed quite quickly. 

Me and Simon got into the car and went to Subway for our lunches, we decided to go there as I'm not keen on just grabbing whatever I can wherever I can, I've learned that food is best planned well in advance so I know what I'm putting into my body compared to the energy I'll be using up, I just feel it's the most sensible decision for me. Other people are different though. Anyway as I was driving I honestly felt like my body was slowly turning to lead, as if I weighed twice what I do now but I pushed it to the back of my mind and hoped I was going to get a good day out of today. 

Nanna and Granda, who we went with, eventually got home and I packed a bag with inhalers, water, phones and everything else we would need and headed off. While I was in the car I could feel my legs turning numb, my granddad's car is very low to the ground compared to mine and I find it extremely difficult to get out of, the seat belts also don't fit around me without an extender so it's not the best car to travel in. Anyway  like I mentioned I felt the numbness creep up my legs and a dull ache hit my lower back which was there all the way to Whitby, along with the overheating feeling which was exacerbated with the weather turning sunny and hotter the closer we got to the coast. By the time we got to Whitby there were no parking spaces so me and Simon tried to get out to walk on ahead; I don't think I managed getting 12 metres before I was sweating in pain with my back and my knees started to ache. I've always been self-conscious of looking like the stereotypes version of an obese person, someone who is so large they cannot walk properly, mainly because when I've tried to explain that I'm not simply over-eating the wrong things without exercising and have a genuine medical condition I am met with looks of disbelief and dubious scrutiny. At this point I walked a little further and had to stop where my anxiety got the best of me as I was convinced everyone was looking at me. I broke down and cried on Simon, insisting I go home and we go for a walk privately around our local area later today when I feel better. So, not only did I not get the day at Whitby we all wanted but I feel I've held back my family and boyfriend, something I'm not unused to feeling. 

Somehow though me and Simon are still determined to do that walk, determined that pain won't stop me from cooking family dinner later and it will not stop me being a happy person overall. The point of this post was not to complain or whine or make people feel sorry for those of us with such problems, it was to remind parents of children with POMC or any genetic obesity disease and those directly dealing with POMC/genetic obesity themselves that no matter how hard the days might seem, how much you want to give up fighting or feel hopeless always remember that the condition you have is there to be fought and we are all warriors in this battle, nobody is alone with a strong support network around them of family, friends, partners and even medical professionals who help you cope. You are a strong person with better things to do and live for, than to give in to anything at all! Even if is is easier said than done at times, please keep fighting! 

Thanks for reading. xo

My Story - Adolescence to Adulthood - The POMC Series

Hello again lovelies!

This is the second instalment of my story, recounting my journey from birth to present while living with POMC. I must give a warning for this post as it will contain slightly more specific information on my development through my teenage years and so and information is, once again very sensitive and personal; however, please feel free to continue to share and like these posts as every little helps!

My first day at comp - 11 years old

I ended the last post by claiming that at the beginning of adolescence was where the real battle began, hopefully you'll understand why. After finishing primary school the next big thing was on to comprehensive and I can honestly say that was the worst time of my life, no exaggeration. By 11 years old I fully understood what I was expecting to happen to my body in the process of "becoming a woman" if you catch my drift, all of the usual things happened I gained more weight as I aged, my body changed shape so I got more of a feminine figure and I started to hold my weight differently. I mainly help my weight in my lower stomach, things and face which did absolutely nothing to help the curve in my spine, that got so bad that even to this day I cannot walk 6 metres without feeling the numbness and pain creep up my calf, thigh and up to my hip and lower back. Honestly, the pain is agonising, I get sweaty, sickly and dizzy with how much it hurts. All of this at 11 years old, although Ive admittedly gotten better with dealing with it, it hurts nonetheless. Yet, somehow at 11 I had reached a massive 16.5 stones in weight, my blood pressure, sugar and cholesterol had all stayed on a level of someone who weighed around 7 stone; at least I had that. 

My 13th birthday

The bullying continued into secondary school, I couldn't walk through the corridors without having some form of abuse shouted at me, some nasty remark. Comments like "You should go and kill yourself" and "you're so ugly you should've been aborted" were regular occurrences, almost daily to be honest. I never got anymore beatings but I feel like a beating a day would have damaged me less than the cruel comments I got did.

By my 13th birthday I'd contemplated suicide and self harm on a number of occasions, never believing I would do anything, only wishing I had. I was put on anti-depressants at 14, I never ever left the house, and to show how serious it had become I developed a sensitivity to the sun that is now so severe I cannot be out in the sun for more than one minute without blistering, literally. All because I couldn't bare getting abuse thrown at me as soon as I left the door. I dropped countless days at school, didn't do anywhere as well as I could've done, teachers allowed me to leave the classroom 5 minutes before bell went so I could get to my next class without  getting abuse, I was allowed a toilet pass to leave whenever I needed to as by this point I had developed severe anxiety as well. But I had managed to find an outlet in playing the piano, clarinet and singing lessons. 

From ages 13-17 (the picture to the right is me at 16, almost 17) my weight gain did continue but at a much slower rate, despite that being the most inactive time of my life, like I said I didn't go out at all, probably ate worse than I should have as I simply didn't care about myself by that point. Everything developed as usual expect one aspect of my body. I hadn't developed certain aspects of puberty, menstrual cycles being one of them, at 15 I was put on Medroxyprogesterone so as to induce them. That's been going pretty well ever since no changes there really. I did however get diagnosed in January 2014 with Polycystic Ovary Syndrome via a routine blood test, and an ovarian cyst indicative of Endometreosis in 2015 as I was rushed into hospital with severe lower abdominal pain in late April 2015. Doctors have told me this cyst can be removed but nobody is willing to operate on a woman of my BMI, I get me with a resounding "lose weight first" even after explaining my situation. Sometimes it seems impossible to get professionals to listen to you no matter how much you tell them. Recently, I have discussed my Gyno concerns with a professional who specialises in POMC and I was told that they are not aware whether or not POMC can cause such problems and have advised I continue to keep an eye on it. If any female with POMC does read this I would advise going to see a specialist about the issues right away. Keep it under control as soon as you see it. 

After finishing school/sixth form in 2014 and beginning a course at New College Durham, who may I say were reluctant to believe my condition was real, despite being registered disabled because of it, bullying had died down significantly in a more mature environment although had not fully been resolved. By 18 ears old my hunger had not lessened but my diet and exercise regimen was improving massively, I was forcing myself not to give in to my hunger, some days were terrible others not so bad. I would usually keep myself busy and whenever I felt hungry remind myself of how disgusting I was... clearly my mental health and self-esteem.image was not on the up at all, I still never left the house or went anywhere really. Not much else can be said for my 18th year. I just plodded on. 

By 19 years old I had started to seek help for my body image, using a number of self-help leaflets, talking more to friends and family, being open with lecturers and teachers about what I needed and gradually my opinions on myself improved, I did really well in college and managed to secure a place at the University of Sunderland studying social work, which as it turned out I wasn't suited to, and changed to Sociology. All the while this is going on I was in a relationship with the most wonderful man I could've ever wished for, he has dealt with a lot and will hopefully be posting about being in a relationship with someone who has POMC sometime in the future. 

Very dark! Me and the best friend on my first night out earlier this year :)

I am now a 21 year old woman, my self-esteem has never been better, I'm engaged to the man I love, I'm getting out and doing things and I have a life that 13 year old me never dreamed I could while having this condition. However, in recent months, this past year, I've noticed my weight has increased more than it should have, in the last 5-8 months I've gained 5 stone while maintaining an exercise regime, I have developed a condition where my knees have turned in opposite outwards directions of one another (pigeon toes) and my spinal curve is stopping me sleeping as it's impossible to find a sleeping position. I have also developed sleep apnoea, a condition where I can stop breathing at any point throughout the night because my throat can close and there is significant pressure on my chest, something that people with POMC must look out for, I had also developed an overactive hypothalamus, meaning my internal thermostat has stopped working properly and I have a tendency to dehydrate extremely quickly and overheat. I am also now double the weight I was when I was 16, terrified of dying young because of this awful incurable and untreatable (as of yet) disease, all support and help goes towards stopping young children with this feeling so scared, maybe there will be a treatment soon enough! 

POMC is, as I have been told by professionals specialising in this area, supposed to lessen with age  in their exeperience, the weight gain in their experience has in some circumstances evened out as has eating habits in some, I am living proof that this disease does not play by the rule book. Bear in mind also that I am one of the lucky ones, there are people out there much worse off that I am, those are the people that need this support and awareness most of all! 
Please if anybody had any questions, comments or so on, do not hesitate to contact me. I'm available all the time so if you want to share your story of living with POMC or a genetic obesity disease get in touch and I'll be more than happy to help! 

Have a great day all! 
xx

My Story - Infancy to Late Childhood - The POMC Series

Hi All!

I hope everybody is well and I'm so pleased people are continuing to come to read the blog, the support has been awesome, thanks guys! Today's post in The POMC Series is very very personal and sensitive to me and will outline my life from birth to diagnosis, development with POMC in terms of the medical side of things to give people a better idea of how somebody with POMC can develop and survive. Let's begin.

I was born on December 14th 1995 in the early afternoon, from what my mother has told me  her pregnancy was good and there was nothing to make her think anything would be wrong, in fact she once describes being pregnant as "the healthiest time of [her] life". My birth was pretty normal and everybody was happy to have a bouncing baby lodging with them now, I was quite average for the first few weeks of life until at 9 weeks old my mother took me to the local GP surgery explain she felt I was gaining weight too quickly as I'd reached 16oz. She was told she was being an overly anxious young mother and my weight would level out, so she went home and thought she would keep and eye on me. 

Me at 9 months with my amazing Nanna

By 1 year old I had hit all of the appropriate milestones by that point, I was walking at 9 months, but I did not like to be touched and didn't cope well with certain noises like the grass cutter,  I was extremely picky about how I would play with my toys and being removed from my routine would upset me hugely. This lead my parents and grandparents to take me for an assessment, which lead on to an Autism diagnosis. However, those diagnosed with POMC have a tendency to have sensory issues, a sensitivity to certain noises and textures, not only textures of food. In my case I couldn't be touched or eat foods such as crunch veg/fruit or bread because the texture doesn't agree with me. 

I eventually went to nursery at 3 years old and the image to the right shows how much weight I had gained, which is clearly more than the average child. I was, at that point, not under the care of any doctor and had not received a diagnosis, doctors repeatedly told my mother she was overfeeding me but didn't look any further into it than that (although I was cute! I mean come on, say 'no' to that face!). When I went to my local nursery issues started arising, each year I remember being forced into a classroom and being told to take off my shoes so I could be weighed for a health check up, along with all the other children, being nearly 4 years old now I can remember this and it was unpleasant to say the least, I remember hearing what a man was saying to the teachers as I got off the scales "This girl is far too fat, phone her mother", so my mother was called and raised holy hell with them, she thought the situation was a barbaric as I did, my whole family at this point was convinced something was wrong but again, doctors simply told my Mam to get me moving more and feed me less. Thing is, I was always dancing, running and playing around, I thought I was "normal" until I went to nursery, but bullying, which I will get to in less retail later and more detail in another post, absolutely crushed my idea that I was average as did this traumatic experience with the scales. 

Me at 4 - close to my diagnosis

At 4 years old I was changing my personality, I stayed away from people at nursery more and went outside a little less, mainly playing in my back garden because I realised people were looking at me because I was fat, still it didn't stop me being happy. One day I took poorly and was taken into Durham hospital Treetops ward for children, I had had a number of tests done one of which being blood. A doctor who later became my regular paediatrician at the RVI told my mother something in my bloods was missing, he then told her to take me to the RVI immediately, this was obviously a distressing time for my family not knowing what was going on. It was at the RVI after numerous, extensive tests and genetic analysis, nights spent in the RVI, days of nursery and school, that Mam was finally told I had POMC deficiency. I don't know if I was told right away but I do remember being told that I "wasn't like everybody else" by family and thinking "Mammy and Nanna are crying so I have to be brave" but after being told in as much detail as you can tell a 4/5 year old, sitting behind our armchair and crying silently. 

After my diagnosis I was regularly at the RVI in Newcastle where I got fab treatment from the Doctors and nurses who even let me paint up a wall for Christmas, which was so so fun! The focus for doctors at this point was keeping my weight maintained as they knew very little about the POMC in 1999, so with regular hospital appointments and check up it got to the point where I was told I needed to go to Cambridge Addenbrooke hospital to meet the chief doctor, as I called her, who studied this disease. I stayed two nights in Cambridge hospital where I was tested every 5 minutes for blood pressure both pre and post exercise, I was given blood test after blood test, an ECG and many more tests. At the end of the experience I was told that after all of that the doctors were surprised to find that everything was in full working order, my blood pressure was perfect, resting and exercising heart rate was extremely good, blood sugar was completely normal, as was cholesterol. Basically, the only thing wrong was POMC affecting my weight gain, thankfully I was healthy. I was 6 years old when I had my first Cambridge visit. 

When I started normal school at age 4, as mentioned previously bullying did become a problem, a major problem. The older I became the more weight I gained and to be brutal it was a free for all among the students with me. I was taunted day in and day out at school, nobody would play with me, I honestly did go through primary school without one true friend, mainly I was excluded to the point where on a break and dinner time I would push myself into a corner of the playground and read and read and read until the bell went. My school was particularly unhelpful and unsupportive as at one point in my school life teachers would berate me for having time off of school, some days my legs would hurt so much because of the rapid weight gain, I couldn't walk properly and didn't feel up to school at all, some days I just didn't go because at 6 years old I couldn't take the bullying anymore. There was one day I was in school at 6 where a group of boys came up to me and just started beating me because of how I looked; a fighter to the last I kept telling them I would win but they didn't stop until a teacher called them as they didn't know where they were. Safe to say my Mam and Nanna went up to their parents house and I didn't get that badly beaten ever again. 

For all of the parents of children with POMC do not EVER let any school, establishment,  adult, or child take advantage of a child the way my school was allowed to, one teacher would come over to my lunch table every day, as I took in packed lunch because I was picky with eating certain foods and textures, and she looked into my lunch box everyday she saw me to tell me what I was eating was disgusting and would make me fatter. Right here on this blog is the first time I  have ever disclosed that information. From that point on I have never been able to comfortably eat in front of anybody. 

Me at 8 years old, drawing away my troubles.

At 8 years old I was well into my primary education now and I was learning quickly that there were certain things I couldn't do, because of my weight I had developed exercise enduced asthma, I wasn't able to participate in P.E., I wasn't able to always go out with neighbouring children to play games due to pressure on my knees, back and legs, I had developed a curvature of the spine and a condition called Lordosis, walking became more difficult and the spinal curve made and still does make my legs go numb when walking for more than 10 minutes at a time. I was, although I didn't know it at that point, becoming depressed and more and more isolated so I found my pleasures in reading and drawing as you can see to your left.  

My regular hospital appointments continued, as did a steady weight gain, excessive hunger and the crowd of people at school, in the GP surgery and outside of the house, who would berate my weight, tell me to get more active and eat less. My family tried feeding me different things, less calories, watching me at all times with food, but they had no idea that the less they gave me to eat the more starving I became. It was when I started secondary school however that the real battle would begin...

Thank you all for reading this post, I apologise for the length of it, I hope this will help some of you understand how difficult it can be for children with POMC. In my next post I will move on to my journey from adolescence to adulthood, you can expect that out at some point tomorrow. 

Thank you all. XO

What is POMC? The POMC Series

Image from Google.com

Good morning my lovelies!

I'm very excited for the first post in what I've called 'The POMC Series' which will explain in as much detail as possible and as accurately as I am aware how the condition works and affects my daily life and such. I will be referring to my development a fair bit throughout the writing of these posts unless I have information I am allowed to use regarding other people; I'll refer to myself as I have no right to assume how other people have developed with this condition or how they cope. Hopefully I'll be able to get interviews and written articles from those I've been connecting with. Right, now I will move on to the crux of the matter and start with all the science-y stuff! YEY!

What exactly is POMC?

POMC is a gene in the body that everybody* has and contributes to their genetic makeup, it gives instructions to the body to make a protein called pro-opiomelanocortin, which after production is split into smaller pieces called peptides. These peptides all have a very different role in the body, mainly sticking proteins into different areas of the body to alert signalling pathways to control different body parts/systems. Think of it like a car, if you put the keys in it signals for the ignition to come on, then the engine, if something in that process within the car goes missing the job can’t be done. 

How does POMC work?

Pro-opiomelanocortin produces another protein called adrenocorticotrophic hormone (ACTH) which sticks to something called a Melanocortin receptor (MC#R) of which there are 5  in the body. ACTH sticks to MC2R which releases cortisol that is in control of maintaining blood sugar levels, protects the body from stressors and stops inflammation; people who have this part “missing” are at great risk of developing kidney problems, adrenal insufficiency, blood sugar will skyrocket and are often insulin resistant. This is associated with the POMC deficiency disease in some cases, however in my case doctors are unsure as to why only my POMC gene and MC4R (which I will get to later) is non-existent. In terms of health my blood sugar often goes quite low and I am at risk of hypoglycaemic shock. I also have significant resilience to stress. 

Three more peptides of POMC similar to ACTH are alpha- beta- and gamma-melanocyte stimulating hormone (a-MSH etc.) Alpha is responsible for producing pigment in skin and hair and attaches to MC1R, suggesting that those with this receptor inactive or missing should have red hair, green eyes and pale skins as they will lack melanin. I do not have this as I am a natural blonde with olive skin and very green eyes although I have connected with individuals who have shown typical features suggestive of a lack of MC1R.

Beta plays a role in weight gain regulation by sticking to MC4R, which I mentioned earlier. It signals the receptor to help keep even ground between energy taken from food and energy stored in the body, those missing this so tee more energy from food than is necessary and will find it difficult if not impossible to release any large amount of that energy. As a child the Professor whose care I was in explained it to me in a particular way:

Imagine a light switch in your head, in most people when that light switch is on that tells them when they are hungry or not, yours doesn't turn off, so that means that your body always thinks your hungry and will look after the energy you get from food better than most people would. This means that it’s hard for that energy to get out of your body and makes it much harder for you to lose the weight. 

As I got older more and more doctors explained it to me, they would explain that because the energy from this faulty receptor stays within the body it’s almost like fighting with myself to try and release that energy as it’s hellbent on storing it. I like to think of it that my body is storing the energy for a Walking Dead type of affair and then it’ll miraculously start to work and I can be as badass as Rick Grimes… that’ll never happen, right? Anyway back on topic. 

It’s suggested that MC3R is involved with another hormone to regulate sodium within the body and keep cholesterol levels down, once again it is important to note that each case is different with some overarching similarities in the conditions but I have perfectly normal if not healthy levels of cholesterol, some individuals are not as lucky as us by any means and are at risk of very serious complications such as heart attacks, fatty livers and so on. 

The final MCR is MCR5 which unfortunately I know very little about. Although not a great deal is understood about all of this by myself I am doing by best to try and describe it as well and accurately as possible, the information I have obtained has been through extensive phone calls from highly trained and experiences nurses, Doctors and informative websites specialising is rare genetic obesity diseases.

Other information

So basically, this condition is characterised by extremely early onset obesity, that weight gain is not likely to stop and can be very rapid, in the last 6 months I have gained 5 stone despite doing over 6 hours per week exercise and eating a diet of less than 1200 calories per day. That is an example of how serious the condition is. 

• It causes extreme unrelenting hunger and excessive eating, as a child and I know this is true for others who have the condition, the fridge and food was controlled massively, I was monitored and watched in the kitchen a lot of the time but nobody could understand (obviously at the time I didn't know it was the best thing for me) that I was desperately hungry and just wanted to stop that feeling. 
• The main production of these hormones are centred around the pituitary gland, responsible for hormone production. 
• It affects the endocrine system.
• Symptoms can be different from boys and girls, for example I spoke to a nurse yesterday who said gynaecology issues in girls may or may not be related to the POMC deficiency, which are definitely present in myself but I'm unsure about other women who have this. 
• Weight gain can continue despite rigorous exercise and good diet. 
• There are 50 officially recorded cases of POMC, my dad and me being 2 of those and it is estimated it is only between 100-500 people globally that may have the condition or some variant of it. 
• It is not curable and as of right now there is no treatment whatsoever, although some drugs are being tested right now and have seen extremely hopeful results for some, I am not eligible for the trial this time but hopefully next time. 

I feel I need to point out this this disease is extremely scary as I and those others suffering with it have uncontrollable weight gain that, frankly could be fatal to those suffering from it if it puts too much strain on our hearts, so please again just try to get the word out, help however you can share the posts or talk about it with friends because it all helps raise awareness. All of the information is as accurate and updated as possible, I obtained the info from highly trained and experienced doctors, nurses and practitioners as well as websites specialising in rare genetic obesity disorders. 

Thank you all for the amazing reception that the previous post received, I was so overwhelmed and really feel like we can all help raise awareness for this awful condition!! 

Have a fab day everyone!