How can those affected by immune deficiencies be helped?
Good medical care is important for improved well-being and often allows a near-normal life.
A common therapy for many primary immune deficiencies is the administration of immunoglobulin (antibodies) in order to compensate for the lack of patients' own antibodies. To do this, the missing immunoglobulins are given by infusion, the aim being to protect the affected person against infectious diseases (otitis, sinusitis, bronchitis, pneumonia etc.).
The immunoglobulins are extracted from the blood of 10,000 to 60,000 healthy blood donors, and then purified. The immunoglobulin preparations available in Switzerland meet the highest requirements regarding protection against the transmission of pathogenic organisms. This is achieved by strict selection of the blood donors and rigorous inactivation of the possibly present pathogenic organisms. Since 1994, no transmission of pathogenic organisms of any kind via immunoglobulins has been observed in Europe and America. However, it must be added that a minimal residual risk (e.g. pathogenic organisms unknown at present) can never be fully excluded in immunoglobulin preparations – as in other blood products.
Immunoglobulin replacement therapy
Today, immunoglobulins are administered to the body in two different ways: During intravenous immunoglobulin therapy (IVIG), the immunoglobulin solution is administered into a vein in the arm. This infusion takes several hours and is given by a nurse usually in a specialised medical centre. The therapy is repeated every three to five weeks. In doing so, the dosage is adjusted to the patient's condition and to the immunoglobulin concentration in his or her blood before the infusion.
Subcutaneous immunoglobulin therapy (SCIG) is a type of therapy that affected persons can perform themselves at home or in the workplace after intensive training. In this case, the immunoglobulin solution is administered in the fatty tissue under the skin using a pump. This type of therapy is generally performed at weekly intervals. The patients treated this way depend less on professional help for their therapy.
Aim of immunoglobulin replacement therapy
In the case of antibody deficiencies, predominantly the upper and lower respiratory pathways are affected by infectious diseases. This means, the aim is to prevent or reduce the frequency of infections of the nose, the throat, the sinuses, the middle ear, the bronchial tubes and the lungs. However, the affected persons cannot be completely protected against all infections, despite optimal therapy with immunoglobulins. Therefore, additional treatment with antibiotics is necessary for bacterial infections.
Even if the effectiveness of a vaccination and, with it, the achieved protection of patients with an immune deficiency cannot be fully predicted, every patient with an immunodeficiency should receive the recommended vaccinations. This includes vaccines against influenza (seasonal flu) and hepatitis B (jaundice), and also against pneumococci (respiratory infections). The vaccinations can increase protection against dangerous infectious diseases. However, the use of live vaccines is prohibited in immunodeficient people.
Stem cell therapy and immunomodulation
Severe primary immune deficiencies or those that do not respond to the usual therapy require other therapeutic approaches, including removal of the spleen (splenectomy), stem cell transplantation, gene therapy or treatment using immunomodulating medications.