It is a rare disorder in which the body is unable to process certain proteins properly. It is classified as an organic acid disorder, that leads to an abnormal buildup of particular acid known as organic acids. Abnormal levels of organic acids in the urine (Organic Acidurias), blood (Organic Academia) and tissues can be toxic and can cause serious health problems. Normally the body breaks down proteins from foods into smaller parts called amino acids. Amino acids can be further processed to provide energy for development and growth. Isovaleric Academia has an inadequate level of enzymes that helps to break down a particular amino acid called Leucine.
Abnormal genetic mutations cause Isovaleric Academia. Enzyme isovaleric gives instructions for making a co-enzyme that play an essential role in breaking down proteins from the diet. Especially, this enzyme helps to process the amino acid leucine, which is part of many proteins. If there occurs a deletion in the gene of enzyme Isovaleric, then it reduces the activity of this enzyme, and the body is unable to break down leuicne properly. In this way, an organic acid called Isovaleric acid and related compounds build up to toxic levels in the body and damage the nervous system and brain, and causing serious health problems.
Symptoms of IVA (Isovaleric Academia) are:
The signs and symptoms of this disorder become apparent within a few days after birth
- Poor feeding
- Weight loss
- Breathing difficulties
- The distinct odor from the feet
- Lack of energy can progress to coma
- A medical condition called metabolic acidosis-
- Low white blood cell count.
- Low platelet count.
- Elevated ammonia levels in
- Encephalopathy- a disease of the brain that causes structural and functional damage.
- Hypothermia- low body temperature than normal.
- Severe acidity in adults.
- Presence of ketone bodies in the blood where patients may eventually lapse into a coma and sometimes death (in severe cases).
How Is Isovaleric Acidemia Diagnosed?
- GENETIC TESTING- it is suggested for diagnosing Isovaleric Academia as this test look for changes in the genes that cause Isovaleric Academia.
- BLOOD AND URINE SAMPLE- Is advised to rule out the harmful toxins and acids. Certain toxins and acids build up in the body when a child has an organic acid condition, so studying the levels of these substances in your baby’s body can help to determine Isovaleric Acidemia.
- Moreover, in the blood high level of acylcarnitine is the sign of the IVA (Isovaleric Acidemia).
- In the urine high level of isovalerylglycine is the sign of the IVA (Isovaleric Acidemia).
This herbal formulation consists of various herbs. which are known for their purgative and carminative properties. “Avipatti” mean to get rid from troubles. It removes excess pitta dosha from the body. Avipattikar churan is used for many problems like hyperacidity, gastritis, burning sensation, loss of appetite, piles, urinary problems, and kidney stones. It improves overall digestion and treats loss of appetite. It neutralizes the acid secretion in the stomach.
Take ½ – 1 teaspoonful with plain water, twice daily before meals.
Giloy capsules of Planet Ayurveda are prepared from the pure herbal extract of Tinospora cordifolia. Giloy is an anti-arthritic, anti-pyretic, digestive, anti-inflammatory, anti- cancerous and excellent blood purifier. And it is also used to cure, cardiac debility, skin problems, anemia, jaundice, etc. Giloy capsules are very effective, free from chemicals, preservatives, colors, , 100% pure and natural. It also supports a healthy immune system. Giloy capsules of Planet Ayurveda can help you to deal with the condition called Isovaleric Acidemia effectively byincreasing your immunity.
1-2 capsules, twice or thrice daily with plain water, after meals.
Punarnava mandur is very helpful in many health conditions. It has excellent property to improve blood count, blood quality, removes the toxins from the blood, and helps in decreasing urea/creatinine and cholesterol levels. Punarnava mandur is a natural diuretic. Thus it is helpful in ascites, renal failure, liver failure, fluid accumulation, puffy eyes, swelling around the joints, fatty liver. It helps to increase hemoglobin hence beneficial in treating anemia.
1-2 tablets twice daily with lukewarm water, after meals.
Manjistha is considered to be the best blood purifying herb and it is also used as immune regulator. It detoxifies the blood, dissolves obstructions in blood flow, and removes coagulated blood.
It also has healing properties that protect gums from receding. It is used to treat reoccurring skin infections or skin diseases such as pigmentation anomalies, leucoderma, etc. Manjistha works very well both internally and externally to promote the skin glow and lusture. Planet Ayurveda manjistha powder is free from chemicals, preservatives, colors, yeast, binders and is 100% natural.
½ to 1 teaspoonful twice daily with plain water, after meals.
Praval Panchamrit Ras
Praval panchamrit ras is a classical Ayurvedic mineral formulation. It is a potent rasa aushadhi that contains five bhasmas, which acts as elixir of life. The five bhasmas are- praval bhasma, mukta bhasma, shanka bhasma, sukti bhasma & kapardika bhasma. Basically, they are calcium compounds thus, widely as a calcium supplement and they also act as rasayana. It is good for the stomach and liver. Praval panchamrit also have antacid property. It works well in malabsorption syndrome like Isovaleric academia if it is presented with bloating and abdominal discomfort.
1-2 tablets with honey or water twice or thrice daily.
InIVA (Isovaleric Acidemia) children should be given low protein diet. This is customized to reduce the amount of leucine your baby receives.
High- protein foods need to be limited including- fish, meat, milk and dairy products, eggs, pulses, nuts, etc.