Introduction
Agammaglobulinemia, a common immunodefiency, is a rare disorder which causes repeated development of infections which mainly affects males. It commonly appears during first four years of life. It’s a genetic malfunction that blocks the normal growth of mature immune system cells called B lymphocytes.
History
Agammaglobulinemia results in low production of immunoglobulins into the bloodstream causing more illness and infection. Immunoglobulins are important for your body’s immune system for it to defend you from certain illnesses. With less production of immunoglobulins, you are now more susceptible to infectious diseases.
With agammaglobulinemia, there is repeated development of infections caused by bacteria such as Haemophilus influenza and Streptococcus pneumoniae which commonly affects lungs causing bronchitis, unexplained asthma, pneumonia and sinusitis which will be explained further. Infections can also occur because of Staphylococci which can cause otitis media or infection of the middle ear and conjunctivitis which is infection of the conjunctival sac of your eyes.
Features
Bronchitis is an inflammation of the main air passages of your lungs making it difficult for the individual to breathe. This can manifest with productive cough with sputum and hearing hoarse crackles over the affected lung. Pneumonia can also appear in patients with agammaglobulinemia due to the infection. Invasion of the bacteria Streptococcus pneumoniae can be the culprit for the pneumonia to occur.
Asthma can also occur in agammaglobulinemia patients. Due to the enlargement of the bronchial sacs of the lungs because of an allergen, asthma can happen in patients which will deprive patients from oxygen which will eventually lead to death. Because of the respiratory diseases mentioned above these will may all lead to bronchiectasis which means collapse of the lungs which may lead to death.
Conjunctivitis means inflammation of the conjunctival sac. The eyes have been infected due to the presence of Staphylococci in the eyes. The immunosuppression of the individual has paved way into allowing the infection to occur. Otitis media, meaning infection of the middle ear has also occurred because of the lack of the immunoglobulin to defend the bacteria to invade the system. In individuals with agammaglobulinemia, individuals are more likely to develop these diseases if left untreated.
Tips and comments
Aggressive antibiotic therapy is required for patients suffering from agammaglobulinemia. This will prevent further infection from occurring at the same defending your body from more penetration of the bacteria. Patients will also have to receive intravenous immunoglobulin to further facilitate more treatment. It is important to really monitor patients receiving intravenous immunoglobulin to avoid adverse reactions to occur in the patient. Rigorous monitoring of vital signs must be done to detect abnormalities when this process is ongoing.
Antibiotics must be taken religiously for patients with agammaglobulinemia. This is to facilitate more protection against disease at the same time this will prevent superinfections to happen. If antibiotics are not taken religiously as prescribed the physician, the bacteria will tend to develop drug resistance making the antibiotics less effective and making the patient more susceptible to bacterial invasion.
Agammaglobulinemia is a serious disease. It should be treated immediately to prevent more disease to occur in the patient. With the right treatment course, agammaglobulinemia will be managed well and the patient will probably develop good prognosis in the long run.
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