In recent blog posts, we 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. Then, we featured the first part of Alix’s Q & A with her nutritionist, Hien Nguyen-Le. Today, we offer the second and final part of the interview with Hien, the nutritionist.
Q: Why am I not hungry when I am so thin?
A: When a person's body is malnourished, as we said it affects every system in the body, including the neurochemical system that regulates appetite, hunger, and fullness cues. Because that system gets broken, our body experiences skewed hunger and fullness cues. If hunger becomes suppressed, then a person who is chronically malnourished doesn't experience hunger. Their body is hungry, but they don't experience hunger cues as much. Then fullness becomes premature. Hunger becomes a delayed reaction and fullness becomes premature. That’s called early satiety or premature fullness, which means that when a person who is malnourished starts to eat they'll complain about feeling full pretty quickly. They might feel full after a small amount of food is eaten.
Q: Will my appetite come back if I gain weight?
A: Absolutely. Yes.
Q: How can I make myself eat if I'm not hungry?
A: That is a tough one. The first thing is to find palatable foods. If you're not hungry, try to find the things that you like the most. The second thing is psychological acceptance and having a good understanding of the re-feeding process and knowing that this is temporary, that this is something that you need to do to get to the other side.
How do you learn to do your homework when you don't feel like it? The short answer is using will power to a certain degree, but really understanding that this is what you need. It might not be what you feel like doing, but it's what you need. It’s difficult but it might just be accepting that sometimes we need to do things to take care of ourselves that we don't want to do. I wish there were a better answer, but it's a hard thing and it's about accepting that and understanding the process. I wish there was a way to make somebody suddenly want to eat, but there isn't.
Q: When is it time to get a G-tube (gastric feeding tube)?
A: The short answer is it's time to get a G-tube when a person is unable to consume adequate nourishment orally for whatever reason, whether it's psychological or physiological. Some of the physiological or medical reasons might be a swallowing or chewing issue, food sensitivities, or texture issues. Or, it might be that a person is too hyper-metabolic—they’re eating orally, but they just physically can't seem to get down enough to make them gain weight. Or there might be behavioral developmental reasons, like a very young child who doesn’t understand why they need to eat when they don’t feel like it.
Q: How does being underweight play a role in lack of stamina?
A: When a person is underweight, their body is not getting the proper nutrition to sustain life. The body will defend itself by slowing its metabolism down. Think of metabolism as your body's engine. Your metabolism is your body's core system to operate everything—for your heart to beat, for your lungs to expand as you breath, for your blood to flow, for your brain to think, for your digestion to digest. All of that requires cellular energy which comes from food. When we're not getting enough of that energy and we're underweight, everything has to slow down in order to preserve life. Otherwise, we would be on our way to a quicker death. Starvation is just a slow death.
Our body, in its defense mechanism, slows everything down so that it doesn't need as much since we're not getting as much. It's trying to stretch it out. It's like getting every bang for your buck. When our body's metabolism slows down, we're naturally just going to feel more fatigued and more tired and not have the energy or endurance we need. Some people who are malnourished will talk about having lots of energy in short spurts and then they'll crash. That's due to some of the biochemical responses in malnutrition where when the body is under stress, and being starved is a stress to the body. It will trigger an adrenalin response.
Your body will increase its secretion of adrenalin to give you more energy to deal with the stress, like the fight or flight response. You can feel great for a short period, but then it crashes because it's not real energy, so to speak. I don't want to make it sound like people who are malnourished never have energy, but as a whole their energy looks like a roller coaster where they have an energy high and then they crash. Eventually they are flat-lined.
Q: Is there a best time of day to add calories or does it only matter the number of calories you consume in a 24-hour period?
A: What matters is the overall consumption in a 24-hour period, but I highly recommend that you spread out meals regularly. If you try to do it all at once you're not going to be able to get as much in because there is a volume threshold and you’ll get full too quickly.
Q: What is the right amount of protein to get daily?
A: This is going to vary from person to person but general nutritional recommendations are about one gram of protein per kilogram of body weight, for adult or child.
Q: Should you be concerned about making sure you get the right amount of carbs or fat?
A: No. Protein is critical because protein is not an energy source for the body. Fat and carbohydrates are energy sources. Protein is used structurally as a basis for enzymes, neurotransmitters, and the building blocks of every single cell. We definitely want to prioritize getting enough protein because if you just re-fuel with calories that are carbs and fat, the carbs and fats will be converted to protein in the body since the body needs that protein. If you get enough protein, then the carbs and fat that you're eating will be spared to be used as an energy source. In general, I would focus on whether you’re getting enough calories. And as long as about 20 percent of those calories come from protein--whether it’s poultry, seafood, red meat, whatever you like most--you should be fine. Then the rest of the calories should come from carbs and fats, but leaning more heavily towards fat so that it’s more nutrient-dense. If tube feeding is the means for re-feeding, then look at the nutritional information for the optimal ratio.
Q: Are there any supplements I should be taking?
A: In general, I would recommend a whole foods based vitamin and mineral supplement. Then, I would recommend additional supplements based on the individual's unique needs. For example, if someone is anemic, I would recommend iron. If someone is low in magnesium or zinc or vitamin D, I would suggest additional supplementation besides a multivitamin.
Q: Would you know if you’re low in something through a blood test?
A: Yes, I would recommend that their doctor order a vitamin and mineral panel as part of their medical workup.
Q: Are there things a person with Marfan syndrome or a related disorder should consider in weight restoration that may be different than the general population?
A: As a whole, I would say that if a person with Marfan syndrome has specific GI issues, that wouldn't be different than anyone else with those same GI issues. If someone with Marfan syndrome had digestive issues, I don't know that I would look at that any differently than someone else who also had digestive issues. I would still treat based on that person's individual medical profile.
Q: If I look for a nutritionist in my area, how do I make sure they have the knowledge or background they need to help me gain weight?
A: You want a nutritionist who is experienced in malnutrition and the process of re-feeding and weight restoration. You want a nutritionist who understands the biological stages of the weight gain process and what happens to hunger and fullness cues and who is comfortable working with people who are on tube feedings.
Q: Is it important to have a nutritionist who knows something about Marfan, and if not, should you have the nutritionist talk to any of the person's specialists?
A: I think it would be preferred if the dietitian knew about Marfan, but it’s not critical as long as he or she is willing to collaborate with the rest of the medical team.
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.