Pulmonary Interstitial Glycogenosis (PIG)

What is Pulmonary Interstitial Glycogenosis (PIG)?

PIG is caused by having too much glycogen (a type of sugar) in the lungs. The glycogen makes it harder for the lungs to move oxygen. The exact cause of PIG is unknown. Some think it could be a problem with normal lung growth during pregnancy. Sometimes it can happen in babies who also have other problems like heart disease.

PIG only happens in babies. It can cause trouble breathing and low oxygen levels. Some babies can also have trouble growing. They often need the hospital to help with breathing after birth or with illness.

Diagnosis

PIG is possible when babies have long lasting trouble breathing.

• A chest x-ray may show interstitial markings.
• A lung scan (chest CT) may show thickening of the interstitial spaces in the lungs.
• A lung biopsy is sometimes needed for the right diagnosis. This will show high levels of glycogen in the lung tissue.

Treatment

Treatment of PIG is to support breathing. Children may need oxygen to help keep their oxygen levels normal. They may need positive pressure to help them breathe oxygen in and carbon dioxide out.

Sometimes doctors try medicines called steroids to block inflammation in the lungs. Your medical team will help decide which medicines to use and how long to use them.

What Does Pulmonary Interstitial Glycogenosis Mean for My Child?

Most babies with PIG get better as they grow older. Many children are able to live without oxygen or extra support. Some children may still need help with breathing during sickness or with sleep.

It is important to protect your child’s lungs from infection by making sure they get the recommended vaccines.

What to Watch Out For

• If your baby is working hard to breathe, talk to a health care provider. If possible, ask to see a lung specialist who is experienced with these conditions.
• If your child needs to wear oxygen, work with your health provider to get the correct amount to give your child. This amount may change from time to time.
• If your child struggles with feeding or gaining weight, talk to your health care provider.
• Try to prevent infections from common childhood illnesses. Wash your hands often and ask your friends and family to let you know if they are sick before they are around your child.
• Get your child’s vaccines and yearly flu shot.

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This information is for educational purposes only. It should not be used as a substitute for the medical advice of one’s healthcare provider.