Primary Immunodeficiency Syndrome

Primary Immunodeficiency Syndrome General Information

Primary Immunodeficiency Syndrome Symptoms

Primary Immunodeficiency Syndrome can destroy very easily the organism of a patient in a matter of months if medical intervention is not received in time. The recurrent infections are longer lasting, more severe and harder to cure than in people with a healthy immune system. Primary Immunodeficiency Syndrome leads to the appearance of other more severe medical disorders and this is why the signs and symptoms are various and very different, depending on what part of the patient’s immune system does not work properly or is missing. The biggest problem of immunocompromised people is that they are more prone to get infections than other people. Their vulnerability to different microorganisms is obvious and very easy to detect. There are some particular signs that are experienced by people who suffer from Primary Immunodeficiency Syndrome, although some patients accuse unique and undetectable symptoms. Besides the persistent and recurrent infections, individuals born with this medical condition present some particular problems of organs, for example heart, skeletal and muscle system, skin or brain. Developmental delay can occur as a result of permanent infection (an example is the problem in the facial development). Other patients are more prone to develop tumors, such as lymphoma, sarcoma and other specific types of cancer. The immune system can sometimes attack the body’s own tissues by mistake, instead of attacking the harmful germs; this happens in autoimmune disorders. All these signs and symptoms range from very mild to very severe and interfere in the patient’s quality of life.

Primary Immunodeficiency Syndrome Treatment

Primary Immunodeficiency Syndrome is usually diagnosed before the new born reaches his first year of life, especially if the signs and symptoms are noticeable (if the syndrome is in a serious phase). In some cases, this type of medical condition starts milder and this is why it can be discovered later in life, during young adulthood or adolescence. Primary Immunodeficiency Syndrome is inherited, being passed down from parents to children. In order to avoid this, young couples should talk to their health care provider before conceiving a child if they know they suffer from this medical disorder. If the disease is diagnosed in time and a proper treatment is applied, the expectances are truly positive. Otherwise, the patient’s life can be seriously threatened by the persistent and recurrent infections.

The main aim of any treatment option applied by the doctor is too improve the quality of life and to help the individual’s organism fight against the infections. Usually, the therapies are strong because the doctors want quick responses so that any existent infection must be stopped from advancing and doing more harm. The methods to treat Primary Immunodeficiency Syndrome depend on what the abnormality of the immune system is. Usually, in order to cure this medical condition, health care providers must follow three steps: firstly, they must clear up the already existent infection, then they must prevent exposure to new infections and finally, they should try correcting the immune deficiency. Several broad-spectrum antibiotics and wide range of infection-fighting antimicrobials are prescribed as a first stage of curing this type of medical condition and to combat a range of germs. There is no treatment that will work in all the cases of Primary Immunodeficiency Syndrome. Doctors will try to combine several therapies to obtain good results and to satisfy the patient’s needs. In most cases, prophylactic antibiotics are the first treatment option.

It is very important to reduce the exposure to certain pathogens that may be dangerous. One of the most common used treatment option when antibody deficiencies occur is the immunoglobulin replacement therapy that can be done intravenously (into the vein or IV) – doctors call it IVIG or under the skin (subcutaneous) – known as SCIG. If these do not work, SCID (hematopoietic transplantation of stem cells) is needed. An infection may fail to respond to common medications; this is an alarming sign that the patient’s health status is seriously damaged and that he/she requires hospitalization. Once the infections reach a chronic level, the health care provider must prescribe medicines that prevent any further complications and relieve the symptoms, for example Ibuprofen, Advil and Aspirin. These ease general aches of the body and other symptoms such as fever. Some new discovered drugs proved to have extraordinary effects in treating Primary Immunodeficiency Syndrome. The first choice is Vicaglobin, followed by Gammaglobulin and Privigen. In addition, if the patient suffers from cancer, autoimmune disorders or other associated conditions, special attention is needed. People may present chronic infections of the respiratory tract and in this case bronchial drainage is the main option.