We recently shared Alix McLean Jennings’ story about how she worked with a nutritionist to bring her nine-year-old daughter Cassie, who has Marfan syndrome, up to a healthy weight. Today, in The Marfan Blog, we share the first part of Alix’s Q & A with her nutritionist, Hien.
Q: How can proper nutrition improve your quality of life?
A: In every way! Nutrition affects every organ system in the human body, as well as our emotional and psychological state. In a nutshell, nutrition enhances our well-being by giving us more energy on every level--a physical level, an emotional level, and a mental level. In addition to having more energy and increasing our vitality, it also increases our resilience to tolerate stress in our lives.
Q: If you are better nourished, will you have more stamina, less fatigue, and feel more balanced?
A: Yes, yes, absolutely you would have more energy. You would have more sustained energy. And you definitely would feel more balanced and grounded--the word I like to use is anchored--in your body and in yourself. If you think about individuals who don't have nutritional issues but, let's say, they missed a meal or they went too long without eating, we know how hunger can affect their mood. Now, imagine that on a longer term basis in someone who's chronically malnourished. Yes, absolutely.
Q: I have been struggling to gain weight for so long that food feels like the enemy. Do you have suggestions for how to combat this feeling?
A: Really hard question. It's normal to feel that way. I think that those feelings stem from the frustration of needing to be so focused on food and nourishment and then perhaps not getting the results that you want. I think the best way to deal with that is to be educated about your nutrition issues, whether there are digestive issues or a metabolic issue, or whatever the person's ailment is as to why they're not able to gain weight. It would be important to seek professional help to understand their problems and the nature of their issues, and to understand why their past efforts were not successful.
Then, of course, it would be important to get the appropriate recommendations to create the success they're looking for and that's what's really going to alleviate that feeling where food is so difficult.
Q: Will being better nourished help me feel more in control of my eating? I feel like I have to force myself to eat. I want to be able to pay less attention to eating and feel like I have more time to focus on more pleasurable activities.
A: Yes, being better nourished will definitely do that, but it's really more going to be a product of you as a person continuing to weight restore. As a person gets closer to their natural healthy body weight, all the biological mechanisms that regulate eating and appetite will start to normalize. Hunger cues will start to come back, fullness cues will start to work more properly. The person will have more of an appetite and so feeding will feel less mechanical and less force-fed and more intuitive. That will help the person to feel more in control.
Q: Assuming I have no major issues with my organs, what is the best way to add calories for weight gain?
A: If a person is getting all of their calories orally, the best way to add calories is to choose nutrient-dense foods. A nutrient-dense food is a food that has the most nutrition, the most macronutrients—which are carbs, proteins, and fats—and calories for the smallest volume. For example, whole milk will be more nutrient-dense than skim milk. It will have more calories and more fat than skim milk. Also, try to eat smaller, frequent meals. Instead of having three larger square meals, have five to six smaller volume meals that have higher calories per meal. That is going to be the best way to add the calories. Small, frequent meals that are high in calories.
If a person is getting their calories orally as well as through a tube feeding, then it's going to be to run the tube feedings at night and to find the right balance between the tube feeding and the oral intake. Obviously, that's something that's going to require a little bit more advanced knowledge from either the patient (or parent) or a professional working with the individual to figure out what the best balance is between tube feeding calories and food calories.
Q: Is it just as good getting calories from junk food as healthy food?
A: No, it’s not as good, but it’s better than not getting calories. The first priority is to re-feed, which is the process of getting someone re-nourished. (The process of getting weight back is called weight restoration.) In order of preference, first is to re-feed with more nutritious, high calorie, nutrient-dense foods. Second is to use junk food, and then third is not at all. I don't want to undermine the value of junk food. My philosophy is that if there is a solid basis of healthy calories coming in, there's room for the junk food as long as the entire diet is not junk food. The issue with junk food is that the person may be getting the calories, but they will not be getting the nutrients and the micronutrients, which are the vitamins and the minerals that will really help the body to heal.
Q: Can I add a lot of calories to my diet without adding a lot of sugar?
A: Yes, absolutely. The best way to do this is to add more fat and to add complex grains, like quinoa and whole grains, which are high in calories. You want to add fattier proteins. Basically, try to stick to a diet that is higher calorie whole foods like fattier meats, nuts and seeds, avocados. Not every single food item has to be extremely high calorie, but there should be a lean towards those types of foods. Sugar is okay. I would just try to limit it.
It also depends on a person's overall caloric needs. In my experience with people who are malnourished and are weight restoring, depending on the age and the individual, some people end up needing well over 3,000 calories a day. If that is the case, you can only eat so many nuts and seeds and there's certainly room to add more ice cream, candy, chips, and sweets, as long as there’s a good basis of what I call real food.
Q: How do I know what a healthy weight is for me?
A: When working with children and adolescents, I often use growth charts to get an idea. It's not absolute proof, but it gives you an idea of what a healthy percentile would be. In general, I think that this is something that would best be determined with a professional. If there isn't an established history or a way to track the history (some adults don't have their growth charts anymore), then I would take a look at where the individual's weight is right now and what kind of health issues they're having that are nutritionally related. I would work on gradually re-feeding and weight restoring until those issues resolve.
For example, if a person is complaining of feeling fatigued because they're so malnourished, I would gradually weight restore until their energy level is improved. Or it would be until whatever their physical ailments that are directly nutritionally related improve—until their appetite comes back or their GI issues improve, that sort of thing. I'd also look at physical indicators to help someone find out what a healthy weight is for them.
The other indicator is looking at something called the hyper-metabolic process, which would require professional input.
Q: What is the hyper-metabolic process? What happens to metabolism when a person is trying to gain weight?
A: Hyper-metabolism is when a person’s metabolism speeds up a lot so that it’s a lot faster than “normal.” When a person is malnourished, their metabolism slows down and that's why they have low energy--because their engine is not running very well. When a person starts to re-feed and gets more nourished, their metabolism starts to speed up. It's like money. When you don't have a lot of money, you spend less. When you have more money, you feel free to spend more. When the body knows it's not getting a lot of calories, it's going to conserve calories, which means it's going to slow down the body’s systems. Your heart rate slows down. All of your body's basic functions slow down. For example, the bodies of girls in their teen years who are malnourished might not have enough energy to regulate their hormones so they might not get their periods. Your body will pull the energy from its functional systems to slow its metabolism down.
Then, as a person re-nourishes, the metabolism speeds up. In some cases, it can speed up drastically. It depends on the person's history, genetics, age and if they're hitting a growth stage. During adolescence this would be more severe. As a person re-feeds, their metabolism speeds up until it reaches a peak point. Then, as a person reaches their healthy weight, the metabolism slows down and normalizes. This really varies from individual to individual. It needs constant monitoring. Back to the last question--one of the indicators that a person's reached a healthy weight is when they are no longer hyper-metabolic.
Q: How do you know if your metabolism is that fast?
A: By monitoring how much food you need to gain weight. If a person doesn't need a lot to gain their weekly goal weight, then their metabolism is speeding up but they're becoming normal metabolic, which means their metabolism is speeding up to what would be typical for that person's age. If their metabolism is speeding up super-fast and their body requires much more than "normal" to gain weight, then they're hyper-metabolic. It's looking at the relativity of how much food is needed to gain weight.
Q: You still need to take in all those calories to gain the weight if you're hyper-metabolic, but then after you weight restore, would you be able to take in fewer calories?
A: Yes, the metabolism slows back down as you approach your goal weight. During that period of hyper-metabolism, it might feel more like force-feeding. Hopefully the person's body will be getting healthier and stronger so their appetite comes back and it won’t feel like force-feeding.
For more information on GI issues and nutrition in Marfan syndrome and related disorders, please register for our annual conference. These will be included among the workshop topics.