So I’m just going to run through a couple of quick slides on nutritional considerations for people living with SMA. So firstly, what we frequently see here at Great Ormond Street Hospital, so our referrals mainly kind of fall into two types of, yeah, issues. So firstly, we see children that are having issues with weight gain, so that’s faltering growth and poor weight gain. So often they present with, yeah, faltering growth due to difficulties with posture and swallowing problems. Often we look at things like, you know, how can we optimize their diet, which I’ll go through in a bit more detail in a few more slides. But we also can look at things like you touched on Sanah, like oral nutritional supplements as well as things like enteral feeding. I won’t dive too much into enteral feeding today, but if you’ve got any questions, do let us know. The other side we look for is weight management. So just making sure that people are not at risk of obesity just because that does impact things like mobility and can also increase the risk of obesity-related comorbidities such as high cholesterol, blood pressure and diabetes. 

Research has shown that people that have got SMA have got altered body composition. Findings from some studies have shown that fat mass, but not fat free mass, increase with age, suggesting that growth can sometimes be driven by increase of body fat rather than growth and maturation of skeletal muscles. So that’s just where that weight management just needs to be closely monitored to make sure that we’re optimizing things like ability to move and things such as that. It also just means that we need to be mindful when looking at growth charts and comparing to things like BMI. So what we essentially want to be looking at is the balancing act of weight control. So when we talk about weight control, there are two sides of this seesaw we can think about it like. So calorie intake, so that’s things such as meals, drinks and snacks. Often things that we see, you know, with people, for example, with faltering growth, is meals are often less calorie dense than calorie burn. 

So on the other side of the balance we see your calorie burn that’s impacted by things like body function, daily activity and exercise. Some studies have shown that people that have got SMA have got a reduced energy expenditure compared to other people. So we just need to be mindful of that as well as reflecting the unique body composition that I talked about earlier on. Other things in this kind of equation that we need to consider that are specific to SMA, I think, We know that we’ve had some studies showing that nusinersen increases resting energy expenditure and can have some positive impacts on fat free mass. However, the research is still pending, so we will keep you guys updated with what comes with that. We also see that sometimes nusinersen and risdiplam can actually increase things such as appetite. So again something that we need to be mindful of that scale, as well as other side effects that can come along with some other medications such as constipation and vomiting, which again can impact the intake side. And I did kind of touch on the differing amount of calories. So when we look at the scale, we talk about the calories needed for body function. Everyone needs a certain number of calories to sustain basic life function, so things like breathing, thermoregulation, metabolic homeostasis. And that differs for people that are sitters, non sitters, walkers and things like that. 

So firstly I’m going to touch on strategies to help gain weight. So often what we find is the calorie intake is less than the calorie burn. So often this is due to things such as small portions. People are finding difficulties with fatigue and swallowing, as well as needing to modify the foods in order to make them easier to chew and swallow. In terms of kind of this presentation, the one thing I really want to emphasize is that finding something that works for you and your lifestyle is super important. Graeme is going to touch on evidence in regards to specific diets and things like that a little bit later on, but nutrition is very personalized and very individualized, and I think these are really good examples of some things that you can take away and kind of adapt into your current eating and drinking patterns. 

So firstly, the main thing, like I said, that I often see are children struggling with fatigue and low appetite… So some strategies to help with that is we find that often smaller, more frequent meals are better tolerated. Sometimes looking at a plate full of, you know, all this food can be quite overwhelming, especially when you don’t have the energy for it. That also can be quite good if you’re struggling with things like posture. So obviously, as you eat a little bit more, that can overload things like your tummy. We often find that sometimes people have difficulties with breathing as well. They eat too much. So splitting it up into six small meals throughout the day can be a good strategy around that. I’m going to touch on an important point around food fortification a little bit later. Some people find as well having a savoury meal followed by a sweet. So dessert doesn’t just have to be for the end of the day. It can be, you know, you have lunch dessert, you can have breakfast dessert. And that’s just kind of maximizing your appetite throughout the day as well. In terms of chewing and swallowing, again, that takes energy and you’re burning energy as you’re getting it in. So what we want to make sure is every mouthful that you’re getting is as efficient and calorie dense as possible. 

 So we find that sometimes making foods a little bit softer… So for example, just making sure meat’s super tender, making sure that you’re using sauces, oils, gravies, and things in periods of fatigue, because that can help with things like, you know, chewing and also swallowing and it going down a little bit easier. Adding extra sauces and gravies as well can mean extra calories. Like you touched on Sanah as well, there are also lots of options out there for oral nutrition supplements which we find if you kind of try these first side strategies, it is really good to go to your doctor and discuss. So those are high energy milkshakes that can be had between your meals and they supplement your intake and they just, again, are really beneficial in periods of low appetite as well as fatigue, just because, you know, they’re liquids, easy to swallow and yeah, often don’t take a huge amount of appetite to drink. The other thing that can be quite good is you’re opting for full fat options and things like full fat yoghurts, full fat milks and things like that. Which kind of brings me on to my next point. 

So, like I said, having as much energy and as small a package as possible is super important. So you don’t necessarily need to change and flip the entire meals that you’re eating, but you just need to make sure that every mouthful is as calorie dense as possible. I’ve got a couple of pictures on the slide here of things like olive oils, butters, margarines, avocados, nut butters, sauces, full fat yoghurts and things like that. So try to add a source of energy to each meal and each snack. So, for example, if you’re having something like a cracker with cheese, making sure it’s full fat cheese, maybe adding a layer of butter under it, just making sure that each mouthful you’re kind of getting as much energy as possible. And, yeah, nutritious liquids I have touched on as well, but having sources of fluid that have got energy in them, again, can be good in periods of fatigue and low appetite. Drinking them away from your meals is super important though, or drinking them just after as like a meal top up, just because we can find that sometimes nutritious fluids can fill up us up super quickly and then kind of not leave room for lots of other nutrients for us to get in our diet as well. 

 So another thing that we get a lot of questions on is protein. So protein is super important for growth and repair, and we need protein in order to help sustain the muscle mass that we have got. Aiming to have a sauce at each meal and snack is super important as well. Or if you’re able to do that, yes, fantastic. So typically when you think of protein, you think of kind of meat, fish and poultry. But I do just also want to highlight that there are lots of other vegetarian options out there as well. And I know Graeme’s going to touch on that as well. But things such as tofus, tofu, lentils, beans, chickpeas, so, such as hummus, so that again can be used as a spread or as a sauce, dressing, things like that, cottage cheese, yoghurts, nuts, seeds. Soya products are also really high in protein as well. So lots of milks out there these days. So soy milks have got a good amount of protein to them as well. And yeah, eggs as well are a great source of vegetarian protein. 

So on the flip side, what about if I want to lose weight? So in this instance, the energy burn needs to be greater than the calorie intake. Now this can be a little bit difficult if your exercise and daily activity is a little bit limited in terms of things like mobility. But what we have found is that you can actually achieve a good calorie deficit just by changing things such as your meals, snacks and drinks as well. So a lot of weight loss can actually be attributed to changing your calorie intake. And now again, one thing that a lot of people find is that changing one thing at a time can be really useful. So there’s obviously a whole lot of information out there on how to lose weight and how to, or how to maintain your weight. So just focusing on one or two things at a time and kind of making sure that you’re making those changes in a good sustainable way is a good way to kind of slowly, you know, lose your weight without it kind of feeling overwhelming or unrealistic. So firstly, I just want to touch on things such as changing the proportion of your plate. So again, you don’t necessarily need to make massive changes as to what you’re currently eating. But looking at proportions is super important. So here I’ve put the plate model on this slide. 

It’s something a lot of people are familiar with. But making sure that half your plate is non starchy vegetables. So that’s things such as salads or aubergines, zucchinis, carrots, tomatoes. A quarter of your plate is then carbohydrates. So that’s things like your potatoes, your sweet potatoes, rice, grains, breads. And then the other quarter of your plate is protein. If you are hungry and going for seconds, try to aim to prioritize the non starchy vegetables followed by a lean protein. So you want to be aiming for about five plus fruits and vegetables a day. The second is aiming for balanced regular meals and snacks to prevent overeating. So this is something that we often find, people try to just cut down their meals significantly, then they get over hungry and then they kind of want to obviously eat the high energy foods out there. But aiming for a good balance, again, referring to this plate model of fruits and vegetables, carbohydrates and lean protein. 

Some things to consider when constructing your snacks of things such as fibre, so looking at the quality of your carbohydrates. So whole grain or seeded varieties have got some more fibre in that that can help with things such as constipation as well as satiety. So they can actually help you feel fuller for longer. Other important nutrients are things such as calciums, and I’ll touch on that a little bit later. So maybe looking for something like low fat yoghurt or low fat cheese and adding that as a snack. Iron and vitamin C as well. So that’s where things such as your meat products and your meat alternatives can come in, and opting to have them with a source of vitamin C, so oranges or spinach, tomatoes, things like that, just to help maximize the absorption. Another tip that could be quite good is aiming to drink low calorie fluids, aiming to drink about six to eight glasses a day. So water is the first choice, followed by things such as low fat milk. Other things that can be quite good are fruit smoothies made with low fat, yeah, milk or water or yoghurts, low fat yoghurts, and avoiding high calorie fluids, so any fluid that has got kind of added energy to it. So things such as fizzy drinks, we know have got a lot of added sugar to them, don’t really provide us with much in the way of nutrients. So they’re quite a good, easy change. 

 Fruit juice as well. And yeah, if you do kind of feel like you want to make the transition slower, looking for low sugar or no sugar alternatives to that. One other thing that people find really useful is reducing the amount of fat. So fat per gram has actually got the highest number of calories. So this is quite a good way that we can, you know, see quite a bit of change with, you know, small kind of changes. So overall, reducing the amount of fat that you’re using. So removing things like visible fat from meat, opting for your low fat options, so skimmed or trim milk. Cooking with less oil, or looking to cook with methods that don’t need as much oil, so things such as boiling, stir frying rather than things like deep frying or, you know, kind of lathering a whole lot of oil to roast. Roasting can be good. But again, just using less oil. And kind of on the topic of heart health, you want to be making sure that you’re reducing your saturated fat. So that can come from things such as cakes, biscuits, coconut oils and things like that. So you want to aim for things like polyunsaturated and monounsaturated fats. So those come from things like your plant based oils, so put a little picture of avocado here just because that… You know, swapping to those kind of more heart healthy oils can also be really good as well. 

Well now in terms of exercise and things like that, I’m unfortunately not an exercise expert for people with SMA, but I’m sure that you guys have got some great physios and support out there as well. But I’ve just popped a couple of ideas for some exercises that you might be able to do while sitting that can also help with that calorie burn side. So one thing that I just wanted to touch on briefly as well is, What about bone health? So we find that children with SMA have got a high prevalence of low bone mineral density. So on this graph here you can see that as the bone grows, you know, peak muscle mass, you know, we’re kind of aiming to achieve that, that kind of plateaus around about the 20 to 30 mark and then it plateaus off. In terms of, yeah, for children with SMA, obviously the lack of mobility and not being able to kind of walk on your bones to help kind of make that structure can mean that we do find a lot of fractures as well as decreased mineral bone density as a child ages. 

So what we really want to focus on is taking in enough of the bone nutrients essentially, so things such as calcium, vitamin D and protein. So in terms of calcium, so I’ve just popped a couple of examples on here and like I said, these can be really useful to add into things such as snacks. So you can see here there’s the kind of recommendations from the British Dietetic Association of how much calcium you should be aiming per day, as well as kind of what foods can, you know, can give you that, those that calcium. So it doesn’t just have to be from milk and milk products. So as you can see here, things such as tofu, calcium fortified cereals and white bread can be quite good as well. So we live in a day and age where luckily manufacturers are kind of aware of where, you know, some nutritional problems do sit. And sometimes they do add additional nutrients to our products. So looking on the back of a nutrition label and seeing whether or not calcium’s added to it is a good thing to do as well. 

And also just lastly on vitamin D, so vitamin D is critical for calcium and phosphorus absorption which is important for our bone health. So we know that high risk groups of people that… Yeah, so infants, children, adolescents who spend little time playing outside, pregnant breastfeeding people, those over 65 years of age, people with darker skin tones and people living in the UK or other northern climates, especially at this time of year haven’t seen a whole lot of sun for the last couple of weeks. So to get adequate amounts, so yes, sunlight is one of the ways that we can do that. But when you are limited, you know, supplementation is a good way to go as well. So sometimes through the autumn, winter, that can be good just to routinely get into as well as, yeah, you can get a small amount from your foods as well. And I’ve popped a couple of pictures here so things like oily fish, cod liver oil, egg yolk, meat and milk and fortified cereals and things like that as well. But yes, I will hand back over to you Sanah to introduce Graeme.