Improving the lives of people with

Primary Immune Deficiency

How is a PID treated?

If your diagnosis follows a prolonged period of illness and you are finding it more and more difficult to fight infections – even with antibiotics, a Consultant Immunologist may feel it is appropriate for you to receive regular infusions of something called immunoglobulin. This is made from human blood plasma that has been extensively checked throughout its manufacturing process to ensure it has absolutely no ‘nasties’ in it like HIV Aids or Variant CJD. But what the immunoglobulin does contain are some antibodies your own body can’t make, either at all – or in very small quantities. It’s those antibodies that help to fight infection that an otherwise healthy person’s body will create automatically. At this point, it must be made clear that whilst Immunoglobulin therapy sustains life in patients, it cannot necessarily replicate an intact or otherwise fully operating immune system. Yes, this therapy is a fantastic help but having it does not necessarily mean that because you are taking it as prescribed, you can then go on to lead an otherwise entirely normal life. Consequently, some patients will unfortunately continue to have breakthrough infections which need to be treated promptly and appropriately.

How often you infuse will depend on you as an individual.

To begin with, you’ll have to go to your nearest immunology clinic to get accustomed to the process and to see how your body is coping with the therapy and whether you’re having any side effects.

Of course, having to go to hospital on a regular basis to infuse can be disruptive for anyone but on the longer term, your Consultant Immunologist and the Clinical Nurse Specialist assigned to you will tell you and teach you how to infuse at home. No-one will rush you, no-one will bully you and if you choose to continue going to your clinic to infuse, that’s fine as well. But be open-minded to this option of ‘doing it yourself’. Thousands of patients do infuse themselves at home and this means they can do it while reading a book, watching TV or talking to friends on the phone. In fact, the rest you’re giving yourself while infusing won’t do you any harm at all!

Just one last thing about the treatment of a PID.

If your diagnosis is of a serious antibody deficiency, it is probable that your immunologist will consider prescribing replacement immunoglobulin therapy (IgG) for you. This is usually needed for the rest of your life and initially is given to you in hospital. Many people chose to learn how to give themselves their own IgGIgG cannot be taken by mouth as the antibodies it contains would be destroyed by the digestive system and will not get into the parts of your body that need the antibodies to combat infections. The most popular way of having IgG given is by subcutaneous infusion. This is where one or two needles are put into the fat of the stomach or upper leg and attached to a pump which pushes the IgG in. This leaves a red lump for up to a few days which slowly goes down as the IgG disperses into your body. Other people have their IgG delivered by an intravenous infusion (IV). Some people learn to put their own needle into a vein and  give themselves their IgG by IV infusion at home. IgG infusions are usually given between once a week to once every three weeks. However, some people now use a daily injection to give their IgG to themselves. Your Immunology Nurse Specialist will discuss these options with you and you will be able to decide, together with your family and friends, which method may suit you best.

People who do not need replacement immunoglobulin therapy will have a discussion with their immunology team about a treatment plan. People with milder antibody deficiencies may be offered prophylactic antibiotic therapy. This is where antibiotics are taken all the time in order to try to prevent infections. They may also be advised to have standby antibiotics at home to be used when an infection “breaks through”. This is because people with a mild antibody deficiency can become unwell quite quickly and it is important to nip infections in the bud before they become very serious.

People with other types of Immune Deficiency will have treatment plans which involve such things as anti-viral and or anti-fungal treatments. People with Severe Combined Immune Deficiency (SCID) will be offered a bone marrow transplant followed by a treatment plan for the rest of their lives.