Primary Immunodeficiency

  • Dr. Sagar Bhattad,

    Consultant - Paediatric Immunology & Rheumatology

Immune system is our army – what if, it fails to protect us?


The Almighty has provided all living beings including humans with an efficient immune system that protects us from infections. The orchestra of the immune system is made up of players who defend the human body against pathogens like bacteria, virus, protozoans and many more. It is akin to an army that is made up of various levels of defense, each member coordinates with the other to defend the kingdom, the kingdom of human health. Unfortunately, nature fails to provide the necessary armamentarium to some children. These children, in the absence of one of these soldiers, cannot effectively fight pathogens and thereby get repeatedly ill. The group of diseases in which children are prone to recurrent infections due to a defect in the immune system are called PRIMARY IMMUNE DEFICIENCY DISEASES.

You may have come across families who say “My child has been unwell for years. He falls sick very often. He has been admitted thrice/many times in various hospitals. He is not growing well.” Some of these families may have children with problems in the immune system. These children get hospitalized on several occasions. They are treated with a variety of antibiotics. However, one wonders why should a child fall repeatedly ill? These families do not find a proper answer to their ongoing problems, despite consultations from several doctors. Majority of such children suffer from a dreaded group of diseases called “Primary immune deficiency diseases”. Unfortunately, physicians and paediatricians are not well versed with these diseases and this may result in undue delay in the diagnosis

Magnitude of the problem

Primary Immune Deficiency diseases are by no means ‘rare’. As per the research conducted worldwide, 1 in 1200 individuals suffers from an Immune Deficiency.

What does it mean?

If these figures are extrapolated to the city of Bangalore, around 5000 children have Immune Deficiency Disease. Unfortunately, the majority of these children remain undiagnosed and suffer for years. There is an urgent need to increase awareness amongst doctors and the public about these diseases.

What are the symptoms?

  • Chest infection – children with problems in the immunity are prey to frequent chest infections. They may require hospitalisations for treatment of these ailments repeatedly.

  • Ear discharge – Recurrent episodes of ear-discharge can be a feature of underlying immune defect.

  • Loose motions – Repeated episodes of loose motions/diarrhoea or diarrhoea that take unusually longer days to resolve, warrants evaluation by an immunologist.

  • Not gaining weight and height – Healthy children gain weight and height as per age norms. If a child fails to achieve his/her potential, a problem in the immune system must be sought for.

  • Death of children in the family – Some immune deficiencies are serious conditions and if untreated, such children die at a young age. If you come across families who have lost children due to infections, one must advise them to meet an immunologist.

How can these children be diagnosed?

If you come across children who remain unwell, kids who visit doctors very often, kids who are often hospitalised, ask them to contact an immunologist. By performing a few blood tests, the doctor would determine if there is any problem in the immune system of the child. These tests are readily available at few hospitals in Bangalore.

Commonly asked immunological tests are serum immunoglobulins (IgG, IgA and IgM), however, the battery of tests would vary based on the problems in a given child.

Is there a treatment for these children?

Children who have problems in immunity require special care and treatment. Injections that boost their immunity (immunoglobulins) can be given. For children with a more serious defect in immunity, bone marrow transplant can be offered. If treated in time, these children lead a healthy and normal life.

Can they grow normally?

Children with immune deficiencies, who have been on regular treatment and follow-up have grown to become engineers, doctors, business tycoons and so on. They contribute to the development of society and the nation, like all of us. Hence, providing the right treatment at the right time is the need of the hour.

Message

  • Children with frequent infections need appropriate evaluation.

  • Frequent episodes of ear discharge or lung infection and/or failure to grow normally warrants assessment of immune functions and consultation with an Immunologist. Simple blood tests can offer a solution to the ongoing problems.

  • Children with immune defects, if treated appropriately, grow out to become productive citizens.

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