In general, treatments for immunodeficiency disorders focus on:
- Treating and preventing infections
- Boosting the immune system
- Treating other symptoms
- Helping people live as normal a life as possible.
In some cases, the immunodeficiency may be linked to a serious illness, such as an autoimmune disorder or cancer, which also needs to be treated.
The treatment used will depend on the severity and type of immunodeficiency, as well as many other factors.
Talk to your doctor
It is important to talk to your doctor about which treatment is best for your particular type of immunodeficiency.
Antibiotics are medicines that kill bacteria or stop them from growing or multiplying, therefore helping the body’s immune system to fight bacterial infections.1
People with an immunodeficiency often need to take antibiotics to treat infections. Some people with immunodeficiency may need to take antibiotics for long periods of time to help prevent infections.1
There are different types of antibiotics available2:
broad-spectrum antibiotics (work against a wide range of bacteria)
narrow-spectrum antibiotics (only work against a few types of bacteria)
If your doctor decides that antibiotics are required, they will choose one that best treats the infection that you have. They may need to do a test to identify which type of bacteria has caused the infection, and in some cases, they may start you on a broad-spectrum antibiotic while waiting for the results.1
As with all medicines, it is important to follow the instructions given by your doctor, nurse or pharmacist.
Antibiotics are prescribed by a doctor and are only effective against bacterial infections.
1. IPOPI. Primary Immunodeficiencies and Infections. Available at: https://ipopi.org/wp-content/uploads/2018/03/WEB_IPOPI_Infections.pdf 2. NPS Medicine wise. What are antibiotics and how do they work. Available at: http://www.nps.org.au/medicines/ infections-and-infestations/antibiotics/for-individuals/what-are-antibioticsand- how-do-they-work
Immunoglobulin replacement therapy
Immunoglobulins (also known as antibodies) are special proteins that the immune system produces to fight a specific infection. The immune system makes a different antibody to protect against each different type of infection, because they are specifically designed to attach to each germ - much like a lock and key.
Antibodies are a vital part of the body’s defence against both bacteria and viruses, and also help protect the body from getting infections more than once. However, in most immunodeficiency disorders, the body produces too few antibodies, or none at all.1
Immunoglobulin replacement therapy involves taking antibodies from human plasma (donated from healthy donors) and giving them to a person with an immunodeficiency. This provides the person with some immune protection against infections, but the protection is not long-lasting, so this treatment must therefore be given regularly.1
There are two main forms of immunoglobulin replacement therapy, based on how they are administered1,2:
Intravenous immunoglobulin (IVIg)
- Given straight into the blood through a vein
- Usually in a hospital day clinic by a doctor or nurse
- Most people receive a dose once every 3 to 4 weeks. The dose and how often it is given depends on a person’s weight and immunoglobulin levels.
Subcutaneous immunoglobulin (SCIg)
- Slowly infusing liquid immunoglobulin under the skin
- Can be done at home by patients themselves, or by parents or carers, after training from a nurse or doctor4
- Usually administered weekly depending upon the patient’s dose and immunoglobulin levels
NOTE: Although immunoglobulin replacement protects against many common and serious infections, it does not prevent all infections.3
Therefore, if you are being treated with immunoglobulin replacement therapy, you should still take care to avoid infections.
1. IPOPI. Treatments for primary immunodeficiency: a guide for patients and their families. Available at: https://ipopi.org/wp-content/uploads/2017/07/IPOPI-Treatments-for-PIDs-download.pdf 2. ASCIA. Immunoglobulin replacement therapy in primary immunodeficiencies. Available at: https://www.allergy.org.au/patients/immune-deficiencies/ immunoglobulin-replacement-therapy 3. IDF. Immunoglobulin therapy and other medical therapies for antibody deficiency. Available at: http://primaryimmune.org/treatment-information/immunoglobulin-therapy/
Other therapy options
Although everyone experiences infections from time to time, people with an immunodeficiency tend to experience infections more frequently and the infections may be severe.
- Stem cell transplantation
- Granulocyte-colony stimulating factor (G-CSF)
- Gamma interferon
- Peg-adenosine deaminase (ADA)
- Gene therapy1
NOTE: These treatments are not suitable for all patients with an immunodeficiency. Your doctor will prescribe a therapy suited to your particular immunodeficiency
1: IPOPI. Treatments for primary immunodeficiency: a guide for patients and their families. Available at https://ipopi.org/wp-content/uploads/2017/07/IPOPI-Treatments-for-PIDs-download.pdf
Managing symptoms of immunodeficiency
- Skin Symptoms
- Autoimmune Symptoms
- Lung Symptoms
- Skin conditions are common in PIDs and skin can also become infected
- Non-infectious skin conditions can also occur, including eczema (red itchy rash), psoriasis (red raised, scaly skin which may be itchy and painful) and cutaneous granulomas (lump or bumps in the skin)
- Treatment for most mild skin conditions involves applying a moisturising cream (to lock in moisture and lock out unwanted bacteria) and topical steroids (to calm down the inflammation), can help the skin to heal1
- In some people with an immune deficiency, the immune system may attack the body itself - causing autoimmune symptoms such as pain and swelling in the joints (arthritis), skin rashes, a loss of red blood cells (anaemia) or platelets (cells involved in blood clotting), diarrhoea and kidney disease
- Corticosteroids (or ‘steroids’) are commonly used to treat these symptoms, though as these medicines suppress the immune system, they can also increase the risk of infections
- Always use corticosteroids under the guidance of a doctor and follow the instructions given with these medicines
- People with PIDs may suffer chronic (long-term), recurrent respiratory infections, which can damage the lungs
- One type of lung condition that can result from recurrent infection is bronchiectasis, which results in a build-up of mucus and bacterial growth, leading to repeated infections and a mucus-producing cough
- Physiotherapy in people with bronchiectasis can help clear the airway of excess mucus
- Immunoglobulin replacement therapy and antibiotics are also used to help prevent or reduce the severity of chronic lung disease1
1. Immune Deficiency Foundation. Patient and family Handbook for Primary Immunodeficiency Diseases Australasian Edition https://primary immune.org/wp-content/uploads/2016/03/Australasian-PFHB_2015.pdf Accessed July 2018