Immune system failure, which is among the rare diseases, is much more common in Turkey than in other countries. The immune system or immune system is a system that forms the defense mechanism against diseases, recognizes and destroys pathogens and cancer cells, and protects the body from harmful substances. Patients with immune system failure experience Covid-19 much more severely. Immune system failure can be treated if detected early. In order to detect it early, it is necessary to be conscious of the observed findings.
ATTENTION TO RECURRENT INFECTIONS AND SEVERE ALLERGIES
Member of the Board of the Turkish National Allergy and Clinical Immunology Association Prof.Dr. Sevgi Keleş made the following statement on the occasion of the 2022 World Rare Diseases Day: “Although the first finding in primary immunodeficiencies is usually recurrent infections, patients may sometimes apply to allergy clinics or hematology clinics with severe allergic findings or low blood cells. For this reason, if there is an allergy that is more severe than normal, diarrhea that does not go away and does not respond to treatment, or severe low blood cells, these people should be evaluated for immune deficiency.
PIY DISEASE IS NOT RARE IN TURKEY DUE TO INTERNATIONAL MARRIAGE
Stating that the number of primary immunodeficiencies in our country is not known exactly, Prof. Dr. Sevgi Keleş said, “It is estimated that the forms resulting from consanguineous marriages are 5 to 10 times more common in Turkey than in other countries. To give an example, the frequency of severe combined immunodeficiency, which is the most severe form of primary immunodeficiency, in our country was found to be one in ten thousand (1/10000) in studies conducted in three different centers. The incidence of this disease in the USA (between one in 65 thousand and one in 80 thousand) is reported as 1/65,000-80,000. This shows that PIDs are not rare in our country.”
LOW AWARENESS OF DISEASES
Today, more than 450 genetic disorders that cause primary immunodeficiency have been identified. It is thought that this number will reach 3000 in the near future. As can be seen from the numbers, these diseases are not rare, they are just diseases of which awareness is low. In particular, some genetic disorders may cause susceptibility to infections only with certain microorganisms. During the Covid19 pandemic process, 9 genetic disorders were found to cause severe COVID19 infection.
Immune Deficiency Patients Have More Severe COVID-19!
Referring to the study conducted by Rockefeller and including some centers from Turkey, Prof. Dr. Sevgi Keleş said, “It has been observed that the individuals with this genetic disorder are actually individuals who do not have a known disease before and they only have a severe COVID19 infection. This situation indicates that primary immunodeficiency is an equation with many unknowns; It is a sign that there are many undiagnosed cases and they are not rare. These patients should be very meticulous about the recommended mask and distance rules, especially during the Covid-19 pandemic. Apart from this, it is of great importance for them to follow up and treat them regularly, and to decide by asking their physicians which vaccine to have when a Covid-19 vaccine or a revaccination is recommended. It should not be forgotten that Covid-19 infection can be severe in some primary immunodeficiencies, and the treatment of these patients should be planned in line with the recommendations of their physicians.
ATTENTION TO THESE FINDINGS!
In order to increase the awareness of both families and physicians on this issue, 10 warning signs for PID were determined by the Jeffrey Modell Foundation, which was founded in the USA by Fred and Vicki Modell, who lost their 15-year-old son due to immune deficiency, and has many diagnostic and research centers around the world. There are some differences in these warning signs in terms of the frequency of infection in adults and children. Findings that are stimulating for PID in adults and children were determined as ten items. Patients with at least two of these findings should definitely apply to immunology clinics and be screened for immune deficiency.
10 WARNING FINDINGS OF IMMUNITY DEFICIENCY IN CHILDREN
- 4 or more middle ear infections per year
- 2 or more middle ear infections per year
- 2 or more sinusitis per year
- Infection with normally harmless bacteria
- 1 or more pneumonia per year
- Chronic diarrhea with weight loss
- Recurrent skin or organ abscesses
- Long-term oral thrush or skin fungal infection
- Using intravenous antibiotics to cure the infection
- Recurrent viral infection (herpes – warts)
- Having family members with immunodeficiency
10 WARNING FINDINGS OF IMMUNITY DEFICIENCY IN ADULTS
- 2 or more sinusitis per year
- Antibiotic use for 2 months or more
- 2 or more pneumonias per year
- Growth and developmental retardation
- Recurrent skin or organ abscesses
- Long-term oral thrush or skin fungal infection
- Using intravenous antibiotics to cure the infection
- 2 or more deep tissue infections
- Having family members with immunodeficiency
TREATMENT DEPENDS ON THE TYPE OF THE DISEASE
Stating that the treatment of these diseases varies according to the type of the disease, Prof. Dr. Sevgi Keleş “While preventive antibiotics are sufficient in some types, immunoglobulin replacement therapy, known as immune serum, in severe forms; In some types, stem cell transplantation, which is known as bone marrow transplantation among the people, is required. prof. Dr. Sevgi Keleş said, “Which treatment will be given to which patient is decided according to the patient’s complaints and the results of laboratory and genetic tests. Especially in severe forms, early diagnosis and treatment is life-saving. When these patients are diagnosed early and treated appropriately, they become completely healthy.”