Last week you learned how our metabolic patients are diagnosed and how their low protein diets are managed during infancy.
But what happens when they start solid food?
There are very few foods that are truly protein free. You may think of fruits as protein free, however, they do have a small amount of protein. There is protein in all vegetables as well, although some are higher than others. Any grain based foods will also contain protein in some amount including rice, pasta, crackers, and bread. Nuts, beans, legumes and products made with these foods are higher typically in protein than fruits and vegetables. And of course, all animal based products including dairy items are very high in protein. There are very few patients with protein based metabolic disorders that are allowed to have any animal product, including eggs, cheese, yogurt, ice cream or even pudding.
The base diet for these patients is focused on fruits and vegetables. Even with foods that are lower in protein, they still need to be weighed and calculated. The gram scale doesn’t get put away once they are a toddler. As a child transitions off of breastmilk or formula, they will remain on metabolic formula to supplement their diet.
Think about the nutrients found in higher protein foods – iron in meat, calcium and Vitamin D in dairy, healthy fats in nuts, fiber and vitamins in beans. If you aren’t getting those in your diet, where are they going to come from? There are many options as children age to help maximize the supplementation of their diet with different metabolic formulas. But they will remain on some type of formula for supplementation throughout their life.
Remember that patient with PAH deficiency?
They have done well through their infancy, levels have been maintained, they like their formula and the caregivers are excited to transition to table foods and get them off of infant formula.
We initiate solid foods as we would with any child, starting around 5-6 months old with cereal and baby foods. The only difference is that the cereal and baby foods are weighed and provide a specific amount of phenylalanine. The metabolic formula is adjusted to supplement the diet and provide the additional nutrients to maintain growth and development.
The caregivers become very proficient in math, calculating out daily menus that provide specific amounts of phenylalanine at every meal and snack through the day. If a child doesn’t finish their breakfast, the amount of phe not eaten has to be added into the next snack or meal so that at the end of the day, they full amount is received. If they leave a little or get a little extra, it usually isn’t a problem every once in a while.
The problems arise when they get too much on a daily basis and their levels are too high, or they get too little on a daily basis and they have slowed growth and development.
Communication between the team and the caregivers remains very important!
For some of the protein disorders, the protein calculation must be even more exact, and serious complications can arise very quickly if those needs are not met. Some of those patients go through their life with a feeding tube so that their treatment needs can be met during illness or even when they are being a typical stubborn toddler who doesn’t want to eat. Getting too much or too little is not safe for those disorders and must be dealt with immediately with changes to their treatment plan and formula.
Yeah, but what about as they get older?
Things continue to get more complicated as a child ages. The amount of food a 2 year old wants to eat in a day vastly changes by the time they are 10 or 12 or 16 years old. For instance, we’ll say our patient is allowed 275 milligrams of phe a day (or 5 ½ grams of protein). The caregiver splits this up between 3 meals and 3 snacks. A typical breakfast for this patient provides about 25 milligrams of phenylalanine. So our 2 year old eats 2 tablespoons of Froot Loops and 2 tablespoons of cantaloupe along with their metabolic formula.
Not too bad! But now that 2 year old is a 12 year old who still only gets the same amount of cereal and fruit. They are probably going to be hungry fairly soon! How do the caregivers, and the patients, work around this? One thing that does help is the development of specialized low protein foods that are made for metabolic disorders. There are many options including low protein pasta, rice, bread, meat substitutes and cheese alternatives. These foods are not sold in the store however, they must be ordered online.
And they are fairly expensive. Here is a comparison between “regular” foods in the grocery store and the low protein equivalent that families have to order –
16 oz elbow macaroni = $1.79
16 oz box spaghetti = $1.00
2 pound flour = $1.99
White bread (18 slices) = $0.99
Chips Ahoy cookies = $3.00
8 oz shredded cheddar = $3.79
24 count American slices = $4.49
Total = $17.05
16 oz elbow macaroni = $13.98
16 oz box spaghetti = $9.99
2 pound “baking mix” = $13.33
White bread (18 slices) = $14.61
Sugar cookies = $13.00
8 oz shredded cheddar = $6.33
24 count American slices = $9.37
Total = $80.61
The low protein foods are 4 times that of regular foods in the store! These foods can be very cost prohibitive for most families. But if they are able to get them, they can definitely change the amount of food at a meal or snack and help make a child’s daily intake much more acceptable.
Another thing that has helped our protein patients is the increase in gluten free foods. Gluten is the wheat protein in food so when that is taken out, it decreases the protein in that food. Some patients are allowed enough protein that gluten free foods, which are in most stores, are part of their diet.
Dairy alternatives also help. For instance, almond milk is very low in protein – 1 gram of protein per cup versus regular milk at 8 grams per cup. Many patients can use these types of products to have more options for dairy type foods including cheese, yogurt and ice cream.
How do the kids react?
While the calculating and mixing and measuring and meal planning is overwhelming enough, then factor in normal psychological and social development as a child ages.
The toddler may not mind that they have “special milk” or “their pasta” or that Mom mixes up a “special cheese” for them. A 10 year old in school who has to go through the lunch line and gets handed a tray with “special” food may not feel the same way. They may feel isolated from their friends who are grabbing slices of pizza or eating a cheeseburger. They may not want to open up their thermos of metabolic formula because it smells funny and they are afraid people will laugh at them. They may be upset that they are different and have a disorder that changes what they can eat or drink.
If you think of all the things that are influenced by, or influence, our diets, imagine that changing because you can’t eat like everyone. Going to birthday parties is different because you have to take a dairy free, egg free low protein cupcake your Mom made and you can’t have the birthday cake everyone else is having. Imagine going to camp and having to take your own cooler with formula and food because you can’t have what everyone else is having at lunch. Even holidays with your own family are different because while everyone else is having turkey and stuffing, you are having a low protein pasta with vegetables.
Trips are hard because you have to make sure and take your metabolic formula, your gram scale, have your phe calculating app on your phone up to date and have food packed because you can’t just stop at a fast food restaurant for lunch. The diet is for life, nothing changes the need for protein restriction, for formula supplementation, for having blood drawn and levels assessed.
Children, and adults, will rebel at formulas, sneak foods, stop checking levels. These are normal compliance issues that happen with chronic disease. When you live with something daily, it becomes too hard to deal with some days and that’s understandable. We do our best to help our patients own their disease, to accept the steps needed for treatment and to embrace them. We encourage talking with friends, telling people about their disorder, setting up a support system of friends and family that will give them the boost they need on a bad day.
There is nothing more heartwarming than having a patient tell you that their friends pick restaurants based on whether or not there will be low protein or vegetarian options so they can eat too!
This is just the tip of the iceberg when it comes to treating patients with metabolic conditions. Hopefully though you will get an idea of the work and effort it takes the patients and their caregivers every day as well as how much we care for and support our patients. This is an amazing group of patients to work with and the Metabolic Treatment Team takes their job to heart. We enjoy seeing “our babies” grow into young children and adolescents and adults who have jobs and families of their own.
So think about protein the next time you sit down to a meal. If you are really adventurous, try to go a whole day with only 5 or 6 grams of protein TOTAL.
You will see what our patients deal with daily and how important it is that we continue this work with metabolic conditions. We can hope for enzyme replacement therapy or gene manipulation, but until that day is here, our families are living with this complicated treatment.
Having more people aware of these rare diseases and the effort needed for treatment will hopefully open the door for new and exciting advances in the years to come!