What is a PKU diet?
A PKU diet centers around foods that are low in phenylalanine to keep this build-up to a minimum. Avoiding protein-rich foods is one of the basics of the PKU diet, as these choices are a major source of phenylalanine. Of course, your body still needs protein, so specially formulated medical foods help fill that gap.
What happens if you don't follow the PKU diet?
Even women with less severe forms of PKU may place their unborn children at risk by not following the PKU diet. Babies born to mothers with high phenylalanine levels don't often inherit PKU. But they can have serious consequences if the level of phenylalanine is high in the mother's blood during pregnancy. Complications at birth may include:
What foods can you not eat on a PKU diet?
Foods such as red meat, chicken, fish, eggs, milk, yogurt, cheese, nuts, soybeans, and beans are too high in protein to be part of a PKU diet, except when the dietary restriction required is minimal. Your dietitian will instruct you as to which foods—and how much of them—you can eat. Even low-protein foods cannot be eaten in unlimited quantities.
What happens if you eat aspartame if you have PKU?
Without the enzyme necessary to process phenylalanine, a dangerous buildup can develop when a person with PKU eats foods that contain protein or eats aspartame, an artificial sweetener. This can eventually lead to serious health problems.
Can you eat protein with PKU?
Diet. The main treatment for PKU is a low-protein diet that completely avoids high-protein foods (such as meat, eggs and dairy products) and controls the intake of many other foods, such as potatoes and cereals.
How much protein can a person with PKU have?
In most people with PKU, the natural protein or dietary phenylalanine is restricted to 25% or less of a regular intake to maintain blood phenylalanine concentrations within European PKU Guidelines target ranges [1].
What protein does PKU affect?
Phenylketonuria (commonly known as PKU) is an inherited disorder that increases the levels of a substance called phenylalanine in the blood. Phenylalanine is a building block of proteins (an amino acid ) that is obtained through the diet.
Can people with phenylketonuria eat meat?
Children and adults with PKU have to eat small amounts of Phe and they cannot eat meats, dairy, eggs, beans or nuts. Someone with PKU cannot eat pizza, birthday cake, regular bread, yogurt, cheese, ice cream, chocolate, hot dogs, hamburgers, or turkey on Thanksgiving!
Do you outgrow PKU?
There is no cure for PKU, but treatment can prevent intellectual disabilities and other health problems. A person with PKU should receive treatment at a medical center that specializes in the disorder.
Can PKU go away?
There currently is no cure for PKU, but the condition is controllable through proper diet and supplements. What kinds of diets do children with PKU need to adopt? PKU is caused by the body's inability to metabolize phenylalanine, which is found in high protein foods such as poultry, meat, eggs and dairy products.
What three things can PKU lead to if left untreated?
Untreated PKU can lead to:Irreversible brain damage and marked intellectual disability beginning within the first few months of life.Neurological problems such as seizures and tremors.Behavioral, emotional and social problems in older children and adults.Major health and developmental problems.
What foods should you avoid if you have PKU?
Foods that contain large amounts of phe must be eliminated from a low phe diet. These foods are high protein foods, such as milk, dairy products, meat, fish, chicken, eggs, beans, and nuts. These foods cause high blood phe levels for people with PKU.
What gender is PKU most common in?
377 adult individuals with PKU were identified, resulting in a period prevalence of 10.13 in 2015 (per 100,000 individuals). Most adult PKU patients were female (58.1%) and the mean age of adult PKU patients in 2015 was 50.9 ± 20.4 years ( Table 1). ...
How does PKU affect the brain?
PKU affects the brain. High blood Phe levels can cause disruptions in neurotransmitters like serotonin and dopamine, which are important for mood, learning, memory, and motivation. In addition to disrupting neurotransmitter balance, Phe itself can be directly toxic to the brain.
Can PKU eat chocolate?
Eating chocolate is a pleasure many children take for granted, but not for two siblings, Kathryn Connolly, 10, and sister Ruby McKenzie, 7, who both suffer from rare genetic condition phenylketonuria (PKU). It is so potentially harmful that their mother Sarah refuses to let them have so much as a single chocolate.
What happens if you can't break down protein?
Signs and Symptoms Loss of muscle mass, impaired liver performance and anemia are common, since the body cannot properly absorb essential vitamins. Other common signs that you are not digesting protein properly include fatigue, vomiting after consumption of protein and an impaired immune system.
How much protein does a PKU need daily?
formula. Most patients with classic PKU can only tolerate < 500 mg Phe per day (10 g natural protein) to maintain adequate Phe control. Compliance with a life-long Phe-restricted diet is challenging and dietary adherence decreases with age.
How much protein can a child with PKU have?
For children with PKU, the prescribed target total protein intake was 3 g/kg/day (2) and median natural protein intake at weaning was 5 g/day (range: 3–7 g/day).
What foods should be avoided with PKU?
Foods that contain large amounts of phe must be eliminated from a low phe diet. These foods are high protein foods, such as milk, dairy products, meat, fish, chicken, eggs, beans, and nuts. These foods cause high blood phe levels for people with PKU.
How can PKU be treated in relation to the diet or intake of phenylalanine?
The main way to treat PKU is to eat a special diet that limits foods containing phenylalanine. Infants with PKU may be fed breast milk. They usually also need to consume a special formula known as Lofenalac. When your baby is old enough to eat solid foods, you need to avoid letting them eat foods high in protein.
Why is it important to follow a diet for PKU?
It's especially important for young children with PKU to follow the diet carefully because their brains are developing quickly. High levels of phenylalanine in a child's body can cause seizures and irreversible damage to their brain, resulting in permanent intellectual disability.
What is a PKU diet?
A PKU diet is the key treatment strategy if you have the genetic disorder phenylketonuria (PKU), in which you are missing an enzyme that allows for the conversion of phenylalanine to tyrosine (both amino acids).
How does PKU work?
The PKU diet works by providing: 4 . Adequate calories for proper growth (in children) or to maintain a healthy weight (in adults) Enough protein and phenylalanine to meet but not exceed your essential amino acid needs.
How to find the amount of phenylalanine in a serving?
Multiply the number of servings you'll eat by the amount of protein per serving to figure out the total amount of protein. Multiply the total amount of protein by 50 to get the amount of phenylalanine in that food.
What fruits have phenylalanine?
Fruits: Most fresh or frozen fruits are low in phenylalanine, with the exception of raisins, which are high and should be limited, and bananas and honeydew melon, which are considered medium phenylalanine fruits. Keep your portion of these to half a cup and eat them in moderation. Keep in mind if you eat dried banana chips (or most dried fruits), it's easy to eat large portions, so make sure you limit those to about half a cup as well.
What is the best way to make up for missing protein?
To make up for the protein and other nutrients you'll be missing from your diet, you'll need to add a specially designed, phenylalanine-free medical nutrition food. In fact, about 70% to 85% of your protein will come from such supplements, 6 like Phenyl-Free.
How much protein should an infant eat?
The protein needs for infants are dependent upon their age but average around 2.5 grams of protein per gram of body weight.
How much protein is needed for PKU?
Due to the spectrum of severity in PKU, individual daily protein intake can vary from 1 gram to 23 grams of protein. The diet is mainly made up of man-made low protein foods with a minimal amount of natural foods. To show you how severe the diet is, below are natural foods and the protein content of these. This shows how individuals ...
What are the symptoms of PKU?
Individuals will start to have symptoms ranging from depression, anxiety, poor concentration, intellectual disability, cognitive defects, liver failure, seizures, mental retardation and would end up institutionalised. Many untreated PKU individuals require constant nursing care.
Why are supplements necessary for PKU?
The supplements are provided through Pharmaceutical Benefits Scheme (PBS) and are necessary for an individual with PKU to ensure they are receiving all the vitamins & minerals that the diet alone cannot provide. The diet remains the same regardless of the type of supplement an individual takes.
What is PKU in medical terms?
Individuals with Phenylketonuria (PKU) are unable to properly metabolise one of the amino acids in protein. As a consequence, they must follow a strict low protein diet, perform regular blood tests to monitor levels and take a dietary supplement to avoid severe and irreversible brain damage.
What do you do if you have PKU?
Individuals with PKU need to conduct regular blood tests and send them to the laboratory for testing. They also need to attend the metabolic clinic at a major teaching hospital to monitor and ensure levels are kept in range. If levels are not closely monitored, diet is not maintained and supplement is not taken then the consequences are severe.
How many grams of protein are in a banana?
To put this in perspective: 120g banana (average sized) is 2 gram s of protein. 50g of boiled potato is 1 gram of protein. Vitasoy Rice milk is 0.3/100ml, that equals to 1 gram protein for 1 cup (250ml) 30g of protein of hot chips is 1 gram of protein. 75g of avocado is 1 gram protein.
How many grams of protein are in 20g of green peas?
20g of green peas is 1 gram of protein. 50g of boiled white rice is 1 gram protein. Next time you are in the supermarket, turn over the items you are looking at and look at the ‘protein’ part of the nutritional label. As you will see, most items are high in protein and cannot be consumed by an individual living with PKU.
What is the diet of a PKU?
This diet is based on: An important part of your diet will be consuming your prescribed amount of medical formula. Your medical formula includes the vitamins, minerals, and protein you need to stay healthy.
What is the diet of a PKU patient?
In addition to caloric intake, everyone must also consume enough protein , essential amino acids, and other nutrients such as vitamins and minerals. As someone with PKU, your carefully monitored diet is customized for you by your clinic team.
What foods are not PHE?
Foods such as red meat, chicken, fish, eggs, milk, yogurt, cheese, nuts, soybeans, and beans are too high in protein to be part of a PKU diet, ...
What is a low protein diet?
Your individualized low-Phe diet will include fruits, vegetables, fats, sugars, and low-protein medical foods, such as low-protein pasta and breads. Foods such as regular pasta, bread, rice, and starchy vegetables (e.g., potatoes, peas, corn) are likely to be limited in your diet.
Can you stray from low phe diet?
Plan ahead! You may stray from your low-Phe diet if do not have the right foods in the house or a strategy for meals outside your home
Can you eat unlimited amounts of low protein food?
Even low-protein foods cannot be eaten in unlimited quantities. All foods must be measured to make sure that Phe intake stays within the daily limit. Work with your dietitian to figure out what works best for YOU in managing your diet. Jen, 38 years old.
What happens if a child has PKU?
If PKU in a child is not diagnosed and treated, phenylalanine will build up in the bloodstream until it reaches levels that can cause brain damage. It can also cause permanent damage to organs and tissues around the body. PKU can lead to: Developmental delays.
How is PKU caused?
PKU is caused by a defect in a gene known as the PAH gene. This defect changes the way that phenylalanine is broken down by the body during digestion. PKU is passed on to children when each parent has 1 mutated gene. This means that neither parent has any symptoms of PKU, but both are carriers of the faulty gene.
What is phenylketonuria (PKU) in children?
Phenylketonuria (PKU) is a rare metabolic disorder. Children with PKU can’t process an amino acid called phenylalanine. Phenylalanine is in many common foods. It helps the body make protein. It's also important for brain growth. It's normally changed to tyrosine, which helps create all of the body's proteins. But with PKU, the amino acid can build up in the bloodstream and cause harm. PKU is found with a simple blood test. All newborn babies in the U.S. are screened for PKU.
What are the symptoms of PKU in a child?
If a baby is not tested and has undiagnosed PKU, he or she may show signs or symptoms at several months old. Some babies with PKU may seem more drowsy and listless than normal. They may have feeding problems. As they continue to take in protein and phenylalanine through their diets, they may have growth, mood, behavior, and thinking problems, as well as other problems. Symptoms can range from mild to severe based on how much phenylalanine is in the blood.
How is PKU diagnosed in a child?
All newborn babies in the U.S. are screened for PKU with a blood test. If you adopt a child from another country, he or she may need to be screened for PKU and other genetic diseases.
What are possible complications of PKU in a child?
This can cause low birth weight, slow growth, small head, behavior problems, and heart disorders. These mothers are also at risk for pregnancy loss.
How can I help prevent PKU in my child?
Women with PKU who are of childbearing age should be careful to follow a strict low-protein diet. Your healthcare provider may advise genetic counseling. You can discuss with a counselor the risk for PKU in a future pregnancy.
Where do people with PKU get their protein?
Where do they get their protein if they can’t eat it? They drink it! The special medical formula that people with PKU drink provides all the building blocks of protein except phe. It also gives them the vitamins and minerals they need. This special formula is a lot like the protein drinks that body builders use.
How to help someone with PKU?
Friends play a very special role in helping the person with PKU. It is very hard to constantly be faced with food temptations when you are trying to stick to a special diet. Understanding the challenges of living with PKU and offering a listening ear to frustrations will be really helpful.
How does phenylalanine affect the baby?
When a woman becomes pregnant, she supports her baby by sharing nutrients from her diet, as well as oxygen through the placenta and the umbilical cord. Phenylalanine, like other amino acids, can cross the placenta to the baby. There is a 1.5:1 gradient of PHE across the placenta. This means that the baby is exposed to a higher concentration of PHE than what is in the pregnant woman’s blood. Because of this gradient, blood PHE levels that are safe for non-pregnant adults are harmful to the baby. The higher the blood PHE level in the Mom, the greater the damage there is to the developing baby.
How to get a healthy PHE?
In order to get blood PHE levels in the safe range, Mothers will need to drink a special drink (formula) in order to provide adequate PHE-free protein for the baby to grow. They will also need to carefully watch their dietary intake of phenylalanine by keeping daily food records and doing blood work twice a week (or more) to monitor their blood phe levels.
How often do people with PKU test their blood?
People with PKU test their blood every month by pricking their fingers and applying blood to a special blood dot card. The card is sent to the lab where the level of phe in their blood can be determined. A good phe level is 6 or less for life.
What happens if you eat too much protein?
If the person with PKU eats too much protein, phe builds up in the blood. Remember the groceries and how the refrigerator was locked? Phe will build up in the blood like the groceries building up outside the refrigerator door.
What are the symptoms of high phe?
Other symptoms related to high phe levels include poor judgement, skin problems (eczema), headaches, lack of energy, mood swings, lack of ability to concentrate, loss of short term memory, poor performance in school or inability to hold a job, muscle weakness and poor personal relationships. High phe levels are not safe.
Overview
Symptoms
- Newborns with PKU initially don't have any symptoms. However, without treatment, babies usually develop signs of PKUwithin a few months. Signs and symptoms of untreated PKUcan be mild or severe and may include: 1. A musty odor in the breath, skin or urine, caused by too much phenylalanine in the body 2. Nervous system (neurological) problems that may include seizures …
Causes
- A gene change (genetic mutation) causes PKU, which can be mild, moderate or severe. In a person with PKU, a change in the phenylalanine hydroxylase (PAH) gene causes a lack of or reduced amount of the enzyme that's needed to process phenylalanine, an amino acid. A dangerous buildup of phenylalanine can develop when a person with PKUeats protein-ric...
Risk Factors
- Risk factors for inheriting PKUinclude: 1. Having both parents with a gene change that causes PKU.Two parents must pass along a copy of the changed gene for their child to develop the condition. 2. Being of a certain racial or ethnic descent. PKUaffects people from most ethnic backgrounds worldwide. But in the United States, it's most common in people of European ance…
Complications
- Untreated PKU can lead to complications in infants, children and adults with the disorder. When women with PKUhave high blood phenylalanine levels during pregnancy, it can harm their unborn baby. Untreated PKUcan lead to: 1. Irreversible brain damage and marked intellectual disability beginning within the first few months of life 2. Neurological problems such as seizures and trem…
Prevention
- If you have PKUand are considering getting pregnant: 1. Follow a low-phenylalanine diet. Women with PKU can prevent harm to their developing baby by sticking to or returning to a low-phenylalanine diet before becoming pregnant. Nutritional supplements designed for people with PKU can ensure enough protein and nutrition during pregnancy. If you have PKU, talk to your hea…
Benefits
How It Works
Duration
What to Eat
General Guidelines
Recommended Timing
Infants and Babies
Considerations
A Word from Verywell
- Many teens and adults go off the PKU diet because they miss the variety in their diet. Doing this increases your risk of cognitive and behavioral problems, which can impact your school, work, or relationships. The good thing is, you can always go back on your diet, and even making some simple swaps can be helpful. If you've been "off diet" for a wh...