Maj-Lis Hellström, founder in 1978 of PIO, the Swedish organisation for patients with primary immunodeficiency, and an officer of the IPOPI board for eight years, has put together a collection of stories from 14 countries. 49 authors mainly patients and parents, but also doctors and nurses have contributed to this book.

In reading the book you may recognise your own problems and situation if you are a patient or parent, but also, hopefully, find support, comfort and hope. As a student or a doctor you should find much to learn; how it feels being a PID patient or parent; their side of the story. You will also find good case studies for your practice.

This book should be on the reading list for medical students and student nurses. For people in general, this collection of experiences will be very useful and important in gaining some idea of primary immunodeficiency and its impact on affected families. It should lead to a better understanding and ability to help.

The book will be distributed through t.he IPOPI member organisations. Others who are interested can require copies from info@ipopi.org. For multiple copies a contribution to postage costs may be asked.

Sections from the book:
Table of Contents
Article: “It’s alright for you to be ill – You are used to it.”
Article: “You should stop worrying, take your son home, and feed him.”

The book has been published in Sweden by Andersson & Strand Advertising Agency and Certus Printing firm Ltd, 1999. The printing of this book was made possible by the support of Baxter and Bayer.

The World Health Organization (WHO) recognizes over 150 primary immunodeficiencies including X-Linked Agammaglobulinemia (Bruton’s Disease), Common Variable Immunodeficiency (also called Hypogammaglobulinemia), Selective IgA Deficiency, and Severe Combined Immunodeficiency (boy-in-the-bubble disease).

Some disorders such as Selective IgA Deficiency can be quite common, occurring as often as 1/500 to 1/1000 individuals. Others may be as rare as 1 individual affected per million. Untreated primary immune deficiencies are characterized by frequent, some times life-threatening infections and debilitating illnesses.

Because of advances in medical understanding and treatment of primary immunodeficiency diseases, many patients who in the past would not have survived childhood are now able to live nearly normal lives. Most primary immunodeficient patients require life long therapies including intravenous gammaglobulin infusion, aggressive antibiotic therapies, or bone marrow transplantation.

Five classes of primary immunodeficiency diseases have been identified:
B-cell (B-lymphocyte) disorders (such as X-linked agammaglobulinemia, common variable immunodeficiency, and selective Immunoglobulin A deficiency);

Combined T- and B-lymphocyte disorders (such as severe combined immunodeficiency, i.e. SCID, the Wiskott-Aldrich syndrome and ataxia telangiectasia, the DiGeorge Syndrome and chronic mucocutaneous candidiasis),

Phagocytic disorders (such as chronic granulomatous disease) and Complement disorders (such as C2 deficiency and C3 deficiency).

Diseases with recurrent fever and inflammatory manifestations.

Secondary immunodeficiencies result from environmental factors, some therapies etc. One example is the Acquired Immune Deficiency Syndrome (AIDS), which is caused by the HIV virus. Other immunodeficiency diseases occur or are acquired as the result of having cancer, severe nutritional disorders, burns, infections, exposure to radiation or organ transplantation.

This is a list of primary immunodeficiencies. It is by far not complete as WHO lists over 150 different diagnoses.