About primary immunodeficiency diseases
Primary immunodeficiency diseases are inborn immune deficiencies that affect the functioning of the immune system. An immune system that does not function proper the way it is supposed to causes recurrent infections to the body. The primary immunodeficiency diseases tend to weaken the immune system hence the recurring infections and other health complications. People who suffer from the primary immunodeficiency diseases were born without some of the body's immune defenses making them to be more prone to germs and organisms that cause infections and diseases. Some types of primary immunodeficiency diseases are mild and can be unnoticed for many tears while others are so severe that they are noticed on birth.
Types of primary immunodeficiency diseases
There are many types of primary immunodeficiency diseases and it is important for people to have an idea of the common ones so they can be in a position to identify the type that may be attacking them. The following are the common types of primary immunodeficiency diseases.
- T-cell deficiencies: T-cells originate from the bone marrow and are responsible for the fighting of viruses and fungal infections. Lack of the T-cells leads to frequent infections that can affect many parts of the body.
- B-cell deficiencies: these are the cells that are responsible for the production of antibodies. Lack of these cells makes the body unable to fight foreign substances and the body may be unable to produce enough immunoglobulins.
- Defective phagocytes: this happens when the white blood cells fail to work properly. Defective phagocytes affect the production of oxygen transporting compounds.
Exposure to the risk factors
The main and known risk factor for primary immunodeficiency diseases is having a family history of the disorder. If a family member especially a parent has suffered from the primary immunodeficiency diseases then the children are at risk of contracting the diseases. It is important fro a person to investigate the family history so that if they are at risk, early management of the primary immunodeficiency diseases can begin.
Appearance of primary immunodeficiency diseases symptoms
Most of the primary immunodeficiency diseases have similar symptoms. The following are the main and common signs of primary immunodeficiency diseases.
- three or more new ear infections in one year
- more than one case of pneumonia within one year
- recurrent viral infections
- chronic unexplained diarrhea
- weight loss
- two or more unexplained sinus infections in one year
- regular deep abscesses of the skin and internal organs
- regular thrush and fungal infections
Diagnosis of the primary immunodeficiency diseases
The best and sure way to know if you have the primary immunodeficiency diseases is by having a clinical diagnosis. This will include laboratory test that will help in the determination of your primary immunodeficiency diseases status. The following are ways in which this can be done;
- identifying infections: if you have been having a recurring infection that has not been responding to treatment, your doctor will have a test done to identify the germs that may be causing the infection.
- Prenatal testing: samples of the amniotic fluid, cells and blood are tested for any abnormalities. A DNA test may be done to test for the genetic defects. This makes it possible for the parents to prepare for their babies treatment and care upon birth.
- blood test: blood test can be used to reveal abnormalities in the immune system. A blood test can identify the exact type of primary immunodeficiency disease that is present.
After the identification of the primary immunodeficiency diseases, the next step would be to prevent and treat the infections that are caused by the diseases. The treatments that are administered are meant to manage the infections, to boost the immune system and sometimes to cure the primary immunodeficiency diseases. The infections are managed using antibiotics and treating the symptoms. The immune system is boosted through immunoglobulin therapy, growth factors therapy and gamma interferon therapy. Stem cell transplantation and gene therapy are used to cure the primary immunodeficiency diseases.
- If a patient suffers side effects from the drugs administered, he/she should inform the doctor for a change of prescription.
- If a patient is being treated with Hizentra, they should not be vaccinated for mumps, rubella and measles as they do not work well with Hizentra.
- Since Hizentra is made from human blood, there is risk of transmission of infectious agents including viruses.
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