Digestive problems!

Friday 14th November 2014.

Hello! 

A bit of education with how it affects Dregan specifically !!

Myotonic Dystrophy is very well known for affecting the digestive system of its suffers, although it is something that many people do not talk about. According to the specialist who gave the presentation last weekend on this issue, it is one of the most common symptoms - also the most embarrassing! And, also, one that has likely been affecting the person for a long time - so might even seem 'normal' to them!

If you think about muscle wasting, and how it can affect the digestion, it is quite complex!

1. Chewing the food! 

The tongue is a major muscle affected by DM, in fact, it is used as one of the diagnostic tools for the disorder - so if the tongue is unable to do its job properly (rolling the food into a swallow-able ball), even just getting the food into you can be a major problem. Not to mention weakness of the muscles that help you chew and swallow - facial and mouth muscle weakness.

Up until Dregans diagnosis, his issues with eating were put down to 'Dyspraxia' - despite us mentioning at MANY appointments that he can't seem to bite his food. How does Dregan eat? Well, he is quite picky about the foods he eats - he will avoid messy food and gravy/soups/etc. this was always put down to 'Autism' - however, we now know it is because he struggles to actually get the food down his throat! This is a VERY common problem, and it is liquids and drinks which often cause the most issues! He pushes the food into his mouth, and squishes it up. 

2. Swallowing.

THIS is the scary one! The number 1 cause of death for people with DM1 is Pneumonia. Until last weekend, we thought this was because of the immune system not being strong enough. but we were very wrong! This is because of the inability to swallow food and drink. The food actually goes down the windpipe into the lungs, instead of down into the correct tube, causing infection in the lungs. So, keep an eye on it? Surely you will know? WRONG! The majority of people don't even know it has happened! THIS is why it is so dangerous and scary. It causes death in over 50% of suffers, early on in life, and is a major contributor to the reduced life expectancy of people with the disorder. This terrifies me!

For Dregan, this is a problem. He gets hiccups almost every meal, and also, throughout the day. He struggles to chew and swallowing is a big problem. Not only food, but drink and saliva. He cannot swallow tablets, so medication have to be in liquid form, and he struggles with this too! It is quite painful watching him if you ask him to swallow for you :-( 

Also, what is the treatment for Pneumonia - if symptoms even show themselves in time? ANTIBIOTICS! Well, Thanks to Dregans critical illness in April/May this year, he is now VERY allergic to Antibiotics. He cannot take anything from the 'Cillin' or 'Cef' family, or Metrinidozole. So, he would be in trouble if he was to get this illness - terrifying thought, given his difficulties.

We were told that when swallowing becomes a severe problem, a lot of people have a feeding tube placed directly into their stomach. I simply can't imagine that for Dregan!

This issue is a very serious worry for us!

3. Digestion!

Once the food has actually arrived where it is supposed to - the digestion begins. people with DM can often have an inability to DIGEST FAT! Also, digesting certain minerals is a problem, and so the liver is often impaired, insulin resistance, diabetes can all be seen in a number of patients with the disorder. The first thing you may notice, is that you cannot put on weight - or that you put on too much weight, depending where the difficulty lies. Also, you may find you are having a lot of tests on your liver, and the first thing you may know is that your GP is asking 'Are you an Alcoholic!' You need to see a specialist, because this liver damage will not improve without the correct treatment, and you may just be told all your tests are negative 'we don't know' what it it is. In the words of the specialist doing this talk 'Before you know it you're in Liver Failure' - the is REALLY important! And comes down, again, to YOU educating the Health Professionals. Your GP probably doesn't even know what Myotonic Dystrophy is - it is so unknown that if you are aware you have the disorder, it is down to you to make sure you receive the help you NEED. Great for people like Dregan, who don't have the cognitive ability to understand or fight for their help!

So, inability to digest fat! The majority of sufferers will be underweight. Might not seem a problem for a healthy person, but for someone with DM it can cause an onslaught of issues. Your muscles will become even weaker, and you are prone to other illnesses. So take suppliments, surely thats the obvious route? WRONG! Trials and clinical tests have proven that it actually makes NO difference. Trials have been done and shown that, unfortunately, it does not help. So, what do you do? You tell me?

Dregan has always been skinny, when he was ill in hospital he lost a LOT of weight, which he has managed to gain back, Unfortunately we struggle to get him to put more weight on. He gets ill if he eats too much dairy, and is unable to digest milk as it causes sickness and addiction . This is something we have known for a long time, but now we have PROOF! People might start listening to us now! He is awaiting an appointment with specialist dieticians and speech therapists to see if there is anything we can do to help. At the moment, making sure he tries to sit a bit straighter when eating, watching he doesn't choke, and keeping a close eye on anything that may seem to indicate illness...

In the meantime, he is currently in the 0th percentile for children his age and weight. That's not just a bit underweight - that is actually clinical starvation. BUT HE EATS ALL THE TIME!!!! I am keeping a very close eye on him, and basically, we have been advised that 'healthy eating' guides might not be relevant for him just now - just get the fat into him! Dregan is very happy about this ;-p

4. Intestines!

The intestines are long muscles - can you see where this is going? Yep, moving the food from the stomach to the anus can be a struggle. Pain, inability for the food to move, etc... This can cause constipation, bowel obstruction, etc. Many patients are diagnosed with 'irritable bowel syndrome' but it is much more than this, and can have serious consequences if not seen by a specialist.

5. Pooping!

Constipation, Diarrhoea, Pain and discomfort, all part of the problem. The Anal Sphincter can be quite badly affected, meaning that it is hard for a person to stop 'leakages' - this can be embarrassing and is often not spoke about very much.

Dregan has these issues. He was not toilet trained until late in his development, and pooing has always been difficult for him - he simply did not know when he needed to go. It was, again, put down to 'sensory perception disorder. and 'Autism'. Later on in life, it has been put down to 'poor self care' and 'learning difficulties'. Having the diagnosis has not changed the difficulties - BUT, it has changed how WE as CARERS can deal with it. It simply is not his fault - so we have more soiled underwear to deal with sometimes, and he cannot tell when he needs to go to the toilet always - BUT, we now know it isn't him being lazy. We change OUR behaviour, to support him, and learn to read the signs and try and send him to the toilet. I don't think there is much we can do about it really.....

Also, we can see a lot of these difficulties in Dregans brother, Xander. Especially the digestion issues. These have been apparent from him being very little, and are documented on many reports. Without getting him tested, we won;t know, but at the moment, I don't think this is a valid reason to test him. We shall see how Xander progresses.

What made me decide to write about this today? Simple, Dregan just came into my office, and handed me something he found in his room. We had a little discussion, and out he walked. Suddenly he was panicking and saying 'don't come out' - yes, you guessed, he needed the toilet...URGENTLY! He didn't know until it was almost too late. Luckily, on this occasion he made it in time as he was passing the bathroom when the urge hit - but this is a problem that will only increase for him. And he doesn't make it every time! I have no idea what support we can get for this!

So, on that note - time to start thinking about what to eat for tea!!

x