What is common variable immunodeficiency in children?

Common variable immunodeficiency (CVID) is an immunodeficiency problem that causes a child to have a low level of antibodies and a decreased responsiveness to some vaccines. This makes it hard for the child’s body to fight infections. The child then becomes sick with infections that keep coming back. Most people with CVID become sick and are diagnosed as adults. But in some cases, the disease may become obvious after a child is 2 years old, during childhood or puberty. The symptoms of the disease are very different for each child affected. This is why it is called a variable group of disorders.

What causes CVID in a child?

Experts don't know what causes CVID. The disorder causes a decrease in the number of immunoglobulins (antibodies) in the child who has it. Immunoglobulins are made by the body. They are needed to fight infections. In some cases, more than one person in a family may be affected.

Which children are at risk for CVID?

The only known risk factor for CVID is a family history of the problem.

What are the symptoms of CVID in a child?

Each child may have slightly different symptoms. Symptoms may include:

• Infections that keep coming back in the eyes, skin, ears, sinuses, and lungs. The more these infections happen, the greater the risk of scarring and lasting (permanent) damage to the lungs and breathing tubes.

• Inflammation in the joints of the knees, ankles, elbows, or wrists

• Stomach and bowel problems

• Increased risk of developing some cancers, especially lymphomas and gastrointestinal cancers

• Autoimmune diseases

How is CVID diagnosed in a child?

A diagnosis of CVID is often made based on a complete health history and physical exam. In addition, multiple blood tests may be ordered to help confirm the diagnosis. Testing for low serum IgG concentrations is key to diagnosing this health problem.

How is CVID treated in a child?

Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is.

Treatment may include:

• Immunoglobulin replacement therapy. IV or subcutaneous infusions of immunoglobulin (antibodies) may be given to help boost the child’s immune system and replace the immunoglobulins that are needed.

• Medicine. Antibiotics to treat and prevent infection as prescribed by your child’s healthcare provider.

• Routine blood tests.

• Postural drainage of the lungs. These are special exercises or treatments for the lungs that are used in cases where frequent infections have led to scarring in the lung. Postural drainage may help with lung infections and removing secretions.

What are possible complications of CVID in a child?

Infections, and the results of those infections, are the greatest complication of CVID. With correct treatment, the number and severity of infections should be reduced. Adults with CVID have an increased risk of developing cancer.

How can I help my child live with CVID?

CVID is a lifelong health problem that can lead to a reduced ability to fight infections. The current therapies can reduce the number and severity of infections. It's important to help your child stay out of situations that have a greater risk for infection. Stay away from others who are sick. Have your child wash their hands often with soap and water. Include your healthcare provider in the discussion about school attendance and after-school activities. Most children are able to take part in all activities. But they should use caution. They should not do activities when the risk for infection is higher. It's also important that you work closely with a specialist who is familiar with CVID and the newest treatments.

When should I call my child’s healthcare provider?

Call the healthcare provider if your child’s symptoms get worse or if your child has new symptoms.

Key points about CVID in children

• CVID is an immunodeficiency problem. It causes a child to have a low level of antibodies and a decreased responsiveness to some vaccines. This makes it hard for the child's body to fight diseases.

• Children with CVID have infections that keep coming back. These can affect the eyes, skin, ears, sinuses, lungs, joints, and GI tract.

• The joints and skin are also often affected by inflammation and rashes not caused by an infection.

• Treatment includes immunoglobulin replacement therapy, medicines, routine blood tests, and postural drainage of the lungs.

• It is also important to help your child stay out of situations that have a greater risk for infection and wash their hands often with soap and water.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.

• Before your visit, write down questions you want answered.

• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.

• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.

• Ask if your child’s condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if your child does not take the medicine or have the test or procedure.

• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.