Primary and secondary immunodeficiencies

Immunodeficiencies constitute a group of over 250 diseases which cause the immune system to lose either all or part of its functionality.
Usually, the immune system defends the human body from pathogenic microorganisms (including bacteria, viruses and fungi) which can lead to the contraction of infectious diseases.
In the event that a part of this system fails or is not present, the patient may suffer from infections more often and subsequently need more time to recover.

Primary Immunodeficiencies (PIDs) occur when the immune deficiency has genetic origins.

As well as being very numerous, a characteristic of primary immunodeficiencies is that they present non-specific symptoms which make accurate diagnosis more difficult.
Studies suggest that diagnosis takes place on average 5.5 years after the first symptoms are detected in adults, while the figure is 2.5 years for children.

With regard to immunodeficiencies associated with antibody defects, replacement therapy with immunoglobulins is the preferred treatment. It is crucial that such therapy is applied quickly in order to avoid irreparable damage caused by infections to organs and systems.

Secondary immunodeficiencies (SID) can be caused a range of factors such as malignant tumours, especially those affecting the haematopoietic and lymphoreticular system.Metabolic diseases and/or malnutrition represent other possible causes.

Immunoglobulin therapy is used to treat hypogammaglobulinemia linked to chronic lymphocytic leukaemia (CCL) and multiple myeloma (MM), which are often brought on by concomitant immunosuppressive therapies.

Such patients may obtain positive results from immunoglobulin replacement therapy as a supplement to the standard treatment for their primary disease.

Dysimmune neuropathies

Dysimmune neuropathies are a set of neurological diseases comprising chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), Guillain-Barré syndrome (GBS), and Lewis-Sumner syndrome.

Such diseases are rare but debilitating, and may affect one's ability to walk or to hold onto objects. They may also lead to insensitivity, tingling or pain in the hands and feet.

These diseases are often progressive, and they might include recovery and relaspse phases or become chronic. There is, at present, no cure for such deseases, although there are treatments available which can substantially improve their symptoms.

Immunoglobulin therapy

The treatments available at present can vary according to how serious the disease actually is. One possible treatment is the administration (intravenous or subcutaneous) of immunoglobulins.

Intravenous immunoglobulin administration has proven to be a turning point in the treatment of such diseases, substantially reducing cases of infection and, as a result, improving patients' quality of life.
With intravenous replacement therapy, one can quickly reach a high plasma immunoglobulin peak, and a level of protective circulating antibodies is maintained with an infusion every 3 to 4 weeks in a hospital environment.

Subcutaneous immunoglobulin replacement therapy is a treatment which has been attempted previously, and it has regained traction in recent years thanks to a number of small modifications that have improved response in patients with primary immunodeficiencies.
Medication is infused via a portable infusion pump and a small needle into the subcutaneous tissue of the abdomen, shoulders or thigh.

Given that the infusion site is the subcutis, the quantity of immunoglobulin which may be infused is lower than is possible with intravenous administration, and it is absorbed more slowly. As a result, it is necessary to perform multiple short-term home infusions with an infusion pump (every 7-15 days) each month.
However, these short and frequent administrations mean that the subcutaneously-infused immunoglobulins survive and remain in circulation longer than those infused intravenously, and stable serum levels can be maintained for longer, as can a typically flat pharmacokinetic pattern which lacks the peak of an intravenous infusion.

Home treatments with subcutaneous immunoglobulins for diseases leading to antibody deficiencies, or which require immuno-modulation, have a series of benefits with respect to standard hospital intravenous therapy:

• Access to veins not necessary.
• Hospital admission for particularly vulnerable patients not required, thus potential exposure to infections is avoided.
• The possibility of carrying out infusions at home with a portable infusion pump reduces the number of necessary visits to the hospital and allows patients to save both time and money.
• Infusion times are reduced: the subcutaneous infusion takes between half an hour and an hour, much less than the 3 to 4 hours needed for the intravenous administration of immunoglobulins.
• Subcutaneous infusion of immunoglobulins allows the patient to move around and perform normal everyday activities, provided that the infusion pump is small and easily portable.
• Side effects following subcutaneous immunoglobulin infusion are typically local and minor to moderate with respect to those associated with intravenous immunoglobulin infusion (muscle aches, fever, chills, headache, nausea, vomiting, anaphylactic shock).
• This technique seems to bring financial benefits both to families with relatives affected by primary immunodeficiencies and to the health service itself.
• Frequent subcutaneous immunoglobulin infusions allow a deposit to be formed locally which enables an almost constant level of IgG over time, providing longer protection against infection while maintaining constant levels of defence.

CANÈ Medical Technology S.p.A. has developed a number of portable infusion pumps for the subcutaneous administration of immunoglobulins, with medication levels varying in accordance with the amount of subcutaneous immunoglobulin to be administered.

The aim of the company is to supply infusion pumps which can improve the quality of life of patients needing subcutaneous immunoglobulin infusions.

Canè Medical Technology has established a position as the leading European leader of infusion pumps for the subcutaneous treatment of primary immunodeficiencies.