The Patient Handbook: An Introduction

Patient Handbook in English

Patient Handbook in other languages

Severe chronic neutropenia (SCN) is the name given to a group of conditions in which neutropenia is the primary problem. The severity and symptoms of the neutropenia differ widely among the various sub-types of neutropenia and even from patient to patient within each disease type. This handbook is designed to give you a better understanding of SCN. It has been written to answer many of the questions you may have about neutropenia and treatment for it. We hope that you find it useful in helping you and/or your child in coping with the disease. The purpose of this document is to give you information and to empower you to go back and ask questions to your physician. There is an ongoing process of investigation and research into severe chronic neutropenia and its management. Consequently, this handbook is not all-inclusive and further updates may be obtained from the registry web site.

The staff members of the Severe Chronic Neutropenia International Registry (SCNIR) wrote this handbook. The SCNIR was established in 1994 under the sponsorship of Amgen Inc., Thousand Oaks, CA, USA. On July 1st, 2000, the SCNIR has become an independent organisation dedicated to research and education in chronic neutropenias. We would like to thank the Advisory Board members of the Registry for their contributions and also, Debra Scarlata and Carol Fier of Amgen Inc. for reviewing the contents.

For the purpose of this document and the website "you" refers to you/your child.

Throughout the text there are words and phrases that appear in Italics, these are explained further in the glossary at the back of the handbook.
In the web page, linked words are connected to the online glossary.

The Handbook was written for the Severe Chronic Neutropenia International Registry
by
Audrey Anna Bolyard, R.N., B.S.
Tammy Cottle
Carole Edwards, R.G.N/R.S.C.N., BSc.
Sally Kinsey, M.D.
Beate Schwinzer, Ph.D.
Cornelia Zeidler, M.D.

Contents List

Introduction
How Blood Is Formed
What Is Neutropenia?
Types Of Severe Chronic Neutropenia (SCN)
Severe Congenital Neutropenia
Cyclic Neutropenia
Shwachman-diamond Syndrome
Glycogen-Storage Disease Type 1b
Idiopathic Neutropenia
Autoimmune Neutropenia
Other Conditions Associated With Neutropenia
Diagnosis Of Severe Chronic Neutropenia
Blood Count Monitoring
Other Blood Tests
Bone Marrow Aspirate/trephine Biopsy
Cytogenetic Evaluation
Investigations In Other Conditions
Treatment For Severe Chronic Neutropenia
Granulocyte-Colony Stimulating Factor (G-CSF)
Bone Marrow Transplant
Other Treatments
Long Term Management Of SCN
Bone Marrow Monitoring
Pregnancy
Psychosocial Effects
The Severe Chronic Neutropenia International Registry
Support Groups
Frequently Asked Questions And Answers
Addendum A
Glossary


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