Health tips to prevent infections in infants with a primary immunodeficiency

What do you need to bear in mind if you look after a child with primary immunodeficiency?

Primary immunodeficiencies are a group of genetic disorders in which the immune system functions improperly. There are more than 300 different types of primary immunodeficiency illnesses (PI), from fairly minor illnesses to other more serious ones that may prove fatal if not treated properly.

Regardless of the age at which the illness begins, it should be considered a chronic process for life. The initial symptoms resemble common illnesses (sinus infection, ear infection, fever, etc.) and this can lead to a delay in their diagnosis.

The following warning signs may cause suspicion of an immune deficiency:

1. More than two serious sinus infections in the space of a year.
2. More than two x-ray confirmed cases of pneumonia in the space of a year.
3. Serious skin infections and/or recurrent deep abscesses.
4. Stunted or delayed weight and height gain despite feeding correctly.
5. Two or more serious infections.
6. More than eight serious ear infections in the space of a year.
7. Fungal infections in the mouth after the first year of life.
8. Complications after being vaccinated and/or infections from microorganisms that normally do not cause illness.
9. Infections that do not get any better with normal treatment and require intravenous antibiotics to eradicate them.
10. Family history of illnesses in which the immune system functions improperly.

These infants are more susceptible to recurring, lingering, serious and unusual infections. At the same time, they are more likely to suffer from autoimmune diseases, allergies and abnormal tissue growth.

For this reason the necessary precautions must be taken to prevent infections. The strictness with which these measures must be applied will depend on the seriousness of picture presented and will have to be agreed with medical personnel:

• Correct hand hygiene is required, especially before eating, after going to the toilet or taking part in outdoor activities.
• Toys and personal belongings should be cleaned often.
• Avoid contact with people with infectious symptoms (fever, cough, sneezing, etc.).
• Use disposable tissues.
• Avoid crowds.
• Avoid smoking areas.
• Take precautions with pets and other animals.
• Take precautions during exposure to sunlight.
• You must ensure correct hygiene of foods to avoid poisoning.
• Avoid raw or under-cooked food.
• Avoid drinking water from an unknown source or that has been kept in the same container for a long time.
• The administration of live attenuated vaccines (rotavirus, oral polio, MMR, chicken pox, etc.) should be avoided, always following your paediatrician's advice.
• Family members should keep up to date with vaccinations.
• Follow your paediatrician’s treatment plan correctly.

The treatment chosen is up to your paediatrician and will depend on the type of PI and other factors. Most infants will, however, need the following treatment:

• Treatment with intravenous or subcutaneous gamma globulin.
• Prophylaxis with antibiotics, antivirals or antifungal medicines.
• Early and aggressive treatment of infections.

There are currently excellent collaboration channels between the biggest PI organisations, which provide optimum connections between medical staff and scientists (ESID - European Society for Immunodeficiencies), nursing staff (INGID - International Nursing Group for Immunodeficiencies) and patients (IPOPI - International Patient Organisation for Primary Immunodeficiencies).

In Catalonia, the Associació Catalana de Dèficits Immunitaris Primaris (ACADIP - Catalan Association for Primary Immunodeficiencies) aims to support patients and their families, and to campaign for the scientific community to increase research into new PI treatments. You can find further information on the association’s website: www.acadip.org.