Managing PKU

PKU
Photo by Julie Johnson on Unsplash

Phenylketonuria (PKU) impacts the way a person metabolizes protein. People who live with PKU have low or no ability to process phenylalanine (PHE), an amino acid that comes in most forms of protein. As a result, the PHE can build up inside the body. Infants, children, and adults with this buildup have a risk of health and mental problems that come from this buildup. For example, PHE accumulation can cause seizures, hyperactivity, developmental disorders, and several other health issues.

Thankfully, this disorder is quite rare, and medical professionals have an easy way to test for this disorder in infants. The newborn heel prick test reveals whether or not an infant has this disorder. When doctors and parents realize quickly enough that the child has the disorder, they can develop a plan that will manage the disorder and allow the child to live a healthy life.

Treatment and Management

Individuals with PKU must maintain a low-protein diet to avoid mental and physical health problems. Any more than just a few grams of regular protein can cause health issues. Because this disorder has no cure, the individual will have to keep up with this restriction for his or her entire life to achieve optimal health. Additionally, those with this disorder should arrange regular blood tests with their doctors. With blood tests, they can monitor their PHE levels and make any necessary dietary adjustments. Some people with the disorder may take medication to manage their PHE levels, but most manage their PHE through diet alone.

PKU Oral Supplement

Although the PKU diet limits protein intake, even people who can’t have PHE need protein to develop and function normally. Doctors have formulated PKU oral supplements to help individuals with this disorder fulfill their protein needs. These supplements come without PHE, giving those with PKU a healthy way to get protein without risking health problems. Also, infant formula for babies with PKU gives parents a safe way to feed their children without causing a PHE buildup.

Your best Wholesale Supplement Options

AAA Wholesale Company provides medical and pharmaceutical supplies for the healthcare community. We have a wide range of PKU products to help patients manage their protein intake. From adult oral supplements to baby formula, we have your needs covered, and since we sell these products wholesale, you can save some money in the long run. Take a look at our PKU formulas here, and if you have any questions, please feel free to contact us at any time.

The Differences in PhenylAde and PhenylAde 60

PhenylAde and PhenylAde 60 are medical, nutritional supplements for those who suffer from Phenylketonuria (PKU). PKU is a rare, genetic disorder caused by a defect in the gene that helps create the amino acid phenylalanine hydroxylase. Amino acids are the building blocks of protein. Individuals without the necessary enzyme to process phenylalanine cannot eat foods that are high in protein. If they do, a dangerous buildup of phenylalanine can develop that results in serious health problems.

People with PKU need to follow a special, restricted diet that limits phenylalanine, which is found mostly in foods that contain protein.
Because their diet is limited, people with PKU need to get essential nutrition through a phenylalanine-free formula.

PhenylAde Essential Drink Mix

PhenylAde Essential Drink Mix is a phenylalanine-free, powdered medical food for the dietary management of those who have been diagnosed with PKU. It can be used for toddlers, children, and adults. It is also safe for pregnant women and women of child-bearing age. Some of the features of PhenylAde are as follows:

• Phenylalanine-free
• Complete with essential vitamins and minerals
• Low in saturated fat
• Provides 25 grams of protein equivalent and 13 grams of fat per 100 grams of powder
• Contains a 4:1 ratio of omega-6 to omega-3 fatty acids
• Provides 1.5 grams of soluble fiber per serving
• Available in chocolate, vanilla, orange cream, strawberry, and unflavored
• Available in cans or pouches

PhenylAde 60 Drink Mix

PhenylAde 60 Drink Mix is a phenylalanine-free, powdered medical food for the dietary management of PKU in children, adults, pregnant women, and women of childbearing age. This drink mix provides 60 grams of protein equivalent per 100 grams of powder. It can be mixed with just three ounces of water as opposed to PhenylAde Essential Drink Mix which requires seven ounces of water. Additionally, it has only 49 calories per serving. It is only available in vanilla and unflavored. Therefore, the differences are the amount of protein provided, calories per serving, the flavors available, and the amount of water required.

Medical Supervision Is Required

Due to the nature of PKU and the severity of health issues that may occur with too much protein intake, these products must be used under medical supervision by a physician or registered dietician.

At AAA Wholesale Company, we have PhenylAde and PhenylAde 60 in stock and ready to ship. We offer great prices always! Visit us at the above link or contact us by filling out this short form. We look forward to working with you!

Signs You Need a Low Protein Diet

Image by Foundry - https://pixabay.com/en/users/Foundry-923783/
Image by Foundry – https://pixabay.com/en/users/Foundry-923783/

Why do some people need a low protein diet? What function do dietary supplements like Phenylade serve? What are the symptoms of the disease that forces people to change their diets, and how can treatment help? These are big, scary questions, especially if you suspect you or someone you love may have PKU, the disease that forces patients to start low protein diets. We’ve outlined PKU and its symptoms below. Children rarely go undiagnosed, and we explain why. We also give adults a list of potential symptoms to consider as they talk with their doctor.

PKU and a low protein diet


PKU prevents your body from fully digesting protein. Left in the system, certain amino acids build up. Like any chemical, a high enough concentration of these amino acids eventually becomes harmful. While most cases are caught before symptoms develop, PKU patients who go untreated, develop serious mental, emotional, and neurological problems. These range anywhere from delayed development, all the way to seizures, which can be deadly.

In order to treat PKU, patients must go on a low protein diet. Since their bodies cannot absorb what is needed from regular protein, their doctors prescribe specialized dietary foods, such as Phenylade. Products like Phenylade augment a low protein diet so patients can receive vital nourishment without ingesting dangerous amino acids.

Diagnosing Children


Today, most children are diagnosed at birth. The vast majority of hospitals run tests to ensure infants don’t suffer from PKU before they even leave the hospital. This early detection circumvents many of PKU’s developmental and emotional side effects, allowing children to grow up without battling the vast majority of these symptoms. Special formula for infants with PKU prevents them from consuming indigestible amino acids the same way Phenylade supplements adults’ diets.

Symptoms in Adults


There are undiagnosed adults with PKU. Although very few children go undiagnosed, many adults were born before regular testing for PKU became common in hospitals. In addition to the handful of symptoms listed above, adults may develop and notice any number of startling symptoms. For instance, patients with PKU often have unusually pale coloration, including fair hair, blue eyes, and stark white skin. Melanin, which adds pigment to these areas, comes from the amino acids PKU patients cannot absorb. Your body gleans many important nutrients from protein, and without them, you may face a host of symptoms. Patients may also have unusually small heads and musty smelling breath or urine. Hyperactivity is another sign of PKU, along with weak bones and eczema. Psychiatric disorders and intellectual disabilities may also stem from PKU. If these symptoms sound familiar, speak with your doctor right away. The sooner patients go on a low protein diet, the fewer symptoms they suffer.

Thanks to supplements like Phenylade, PKU patients can live long, healthy lives. If you feel like the symptoms above fit you, be sure to speak to a doctor right away. Early diagnosis can make a tremendous difference. Always ask for PKU testing for newborns, and monitor your own health carefully. You may find warning signs that you should be on a low protein diet.

 

Adding Some New Nutrition to Your Diet

 

Image by maxmann - https://pixabay.com/en/users/maxmann-665103/
Image by maxmann – https://pixabay.com/en/users/maxmann-665103/

Nutrition is the key to a healthy life. It fuels your body and gives you energy to keep fit and pursue your goals. Unfortunately, getting the right nutrition can be difficult, and the consequences have immediate and long-term consequences. Below are some ideas to help get the right nutrition from two of the trickiest portions of your plate.

Vegetables

Vegetables are the cornerstone of good nutrition. However, there are plenty of reasons people don’t want to eat them. People say they don’t like the texture, or they don’t like the flavor. Children often take this dislike to new levels of dietary rebellion. The good news is: there are plenty of ways to overcome those obstacles.

There are innumerable methods to hide vegetables in food. One idea is to imitate a smoothie, and then get creative. A blender will turn most vegetables into a highly nutritious puree, and tougher vegetables are easy to blend after a short boil. This veggie mix can be used in everything from meatballs to pasta sauce. After all, tomato sauce is nothing but seasoned tomato puree. Any sauce is an opportunity to add some vegetables to your meal. Finley chopped cauliflower can masquerade as breadcrumbs with the right seasoning, and many shredded vegetables go well in baked goods. Essentially anything you eat can be a secret vehicle for vegetables.

An easier trick is to simply try cooking vegetables a new way. Steamed and boiled vegetables quickly loose flavor and become soggy. Many people who believe they hate all vegetables really just hate vegetables when they are mushy. Roasted vegetables appeal to many picky eaters who do not believe they like vegetables. A little olive oil and some generous seasoning can go a long way towards turning the worst part of a meal into the highlight of your dinner.

Protein

The most complicated part of nutrition is often protein. Many people do not know the best sources of protein, consume too much protein, or do not consume enough of this vital dietary element. Any of these can cause severe health problems and increase your chances of developing additional conditions. Lean protein, particularly from fish, is usually the healthiest option. Protein from nuts and other vegetarian sources is also healthy, but it’s important to talk with your doctor and check for certain conditions before giving up all meat.

For some, however, protein is an even more complicated issue. Phenylketonuria (PKU) is a rare genetic condition found in infants and adults that prevents the body from breaking down a specific amino acid. This causes the amino acid to build up to dangerous levels inside the body, resulting in seizures, hyperactivity, stunted growth, and further symptoms. If not treated, the condition can cause brain damage. Individuals with PKU must be prescribed special dietary supplements to ensure they receive the necessary protein without the hazardous amino acid they cannot properly digest. PhenylAde and PhenylAde60 are both good options for those with PKU. These two supplements are so popular, there are actually recipes online to turn daily doses into tasty shakes.

Every individual’s nutritional needs are a little different, and it’s important to consult with your doctor before making any serious changes to your diet. Still, there is always room for improvement, whether you’re looking to add more vegetables to the table or seeking the right source of protein.