IDF Patient & Family Handbook for Primary Immunodeficiency ... - IDFA

More documents

Recommendations

Info

22 Selective IgA Deficiency Treatment of Selective IgA Deficiency It is not currently possible to replace IgA in IgA deficient patients, although research toward purification of human IgA is ongoing. However, it remains to be seen if replacement of IgA by any route (IV, oral, or topical) will be beneficial for humans with IgA deficiency. Treatment of the problems associated with Selective IgA Deficiency should be directed toward the particular problem. For example, patients with chronic or recurrent infections need appropriate antibiotics. Ideally, antibiotic therapy should be directed at the specific organism causing the infection. Unfortunately, it is not always possible to identify these organisms, and the use of broad-spectrum antibiotics may be necessary. Certain patients who have chronic sinusitis or chronic bronchitis may need to stay on long term preventive antibiotic therapy. It is important that the doctor and the patient communicate closely so that appropriate decisions can be reached for therapy. As mentioned above, some patients with IgA deficiency also have IgG2 and/or IgG4 subclass deficiency and/or a deficiency of antibody production. However, these laboratory findings do not always predict a greater frequency or severity of infections. For patients with IgA and IgG2 deficiency who do not respond adequately to antibiotics, the use of replacement gamma globulin may be helpful in diminishing the frequency of infections (see chapter titled Specific Medical Therapy). There are a variety of therapies for the treatment of autoimmune diseases. Anti-inflammatory drugs, such as aspirin or ibuprofen, are used in diseases that cause joint inflammation. Steroids may be helpful in a variety of autoimmune diseases. If autoimmune disease results in an abnormality of the endocrine system, replacement therapy with hormones may be necessary. Treatment of the allergies associated with IgA deficiency is similar to treatment of allergies in general. It is not known whether immunotherapy (allergy shots) is helpful in the allergies associated with Selective IgA Deficiency, although there is no evidence of any increased risk associated with this therapy in these patients. The most important aspect of therapy in IgA deficiency is close communication between the patient (and/or the patient’s family) and the physician so that problems can be recognized and treated as soon as they arise. Expectations for Selective IgA Deficiency Patients Although Selective IgA Deficiency is usually one of the milder forms of immunodeficiency, it may result in severe disease in some people. Therefore, it is difficult to predict the long-term outcome in a given patient with Selective IgA Deficiency. In general, the prognosis in Selective IgA Deficiency depends on the prognosis of the associated diseases. It is important for physicians to continually assess and reevaluate patients with Selective IgA Deficiency for the existence of associated diseases and the development of more extensive immunodeficiency. For example, rarely, IgA deficiency will progress to become Common Variable Immunodeficiency with its deficiencies of IgG and/or IgM. The physician should be notified of anything unusual, especially fever, productive cough, skin rash or sore joints. The key to a good prognosis is adequate communication with the physician and the initiation of therapy as soon as disease processes are recognized.
Severe Combined Immunodeficiency chapter 5 Severe Combined Immunodeficiency is an uncommon primary immunodeficiency in which there is combined absence of T-lymphocyte and B-lymphocyte function. There are a number of different genetic defects that can cause Severe Combined Immunodeficiency. These defects lead to extreme susceptibility to very serious infections. This condition is generally considered to be the most serious of the primary immunodeficiencies. Fortunately, effective treatments, such as bone marrow transplantation, exist that can cure the disorder and the future holds the promise of gene therapy.
Page 1 and 2: Patient & Family Handbook For Prima
Page 3 and 4: Patient & Family Handbook For Prima
Page 5 and 6: Patient and Family Handbook i Prefa
Page 7 and 8: Patient and Family Handbook iii Abo
Page 9 and 10: The Immune System and Primary Immun
Page 11 and 12: The Immune System And Primary Immun
Page 19 and 20: Common Variable Immune Deficiency c
Page 21 and 22: Common Variable Immune Deficiency 1
Page 23 and 24: X-Linked Agammaglobulinemia chapter
Page 25 and 26: X-Linked Agammaglobulinemia 17 Clin
Page 27 and 28: Selective IgA Deficiency chapter 4
Page 29: Selective IgA Deficiency 21 Clinica
Page 33 and 34: Severe Combined Immunodeficiency 25
Page 39 and 40: Chronic Granulomatous Disease 31 De
Page 41 and 42: Chronic Granulomatous Disease 33 Di
Page 43 and 44: Chronic Granulomatous Disease 35 Tr
Page 45 and 46: Wiskott-Aldrich Syndrome 37 Definit
Page 47 and 48: Wiskott-Aldrich Syndrome 39 Maligna
Page 49 and 50: Wiskott-Aldrich Syndrome 41 Treatme
Page 51 and 52: Hyper IgM Syndrome 43 Definition of
Page 53 and 54: Hyper IgM Syndrome 45 Treatment of
Page 55 and 56: DiGeorge Syndrome 47 Definition of
Page 57 and 58: DiGeorge Syndrome 49 Therapy for Di
Page 59 and 60: IgG Subclass Deficiency and Specifi
Page 61 and 62: IgG Subclass Deficiency and Specifi
Page 63 and 64: Ataxia Telangiectasia 55 Definition
Page 65 and 66: Ataxia Telangiectasia 57 General Tr
Page 67 and 68: Hyper IgE Syndrome 59 Definition of
Page 69 and 70: Hyper IgE Syndrome 61 Inheritance o
Page 71 and 72: Complement Deficiencies 63 Definiti
Page 73 and 74: Complement Deficiencies 65 Treatmen
Page 75 and 76: Other Important Primary Immunodefic
Page 77 and 78: Other Important Primary Immunodefic
Page 79 and 80: Inheritance chapter 15 Many disease
Page 81 and 82:
Inheritance 73 Types of Inheritance
Page 83 and 84:
Inheritance 75 Types of Inheritance
Page 85 and 86:
Inheritance 77 Reproductive Options
Page 87 and 88:
Laboratory Tests chapter 16 Laborat
Page 89 and 90:
Laboratory Tests 81 Major Categorie
Page 91 and 92:
Laboratory Tests 83 Summary Laborat
Page 93 and 94:
General Care 85 General Health Meas
Page 95 and 96:
General Care 87 Exercise Participat
Page 97 and 98:
General Care 89 General Care During
Page 99 and 100:
General Care 91 General Care During
Page 101 and 102:
Specific Medical Therapy 93 Introdu
Page 103 and 104:
Specific Medical Therapy 95 General
Page 105 and 106:
Specific Medical Therapy 97 Hematop
Page 107 and 108:
Specific Medical Therapy 99 Granulo
Page 109 and 110:
Specific Medical Therapy 101 PEG-AD
Page 111 and 112:
Specific Medical Therapy 103 Gene T
Page 113 and 114:
Infants and Children with Primary I
Page 115 and 116:
Page 117 and 118:
Page 119 and 120:
Adolescents with Primary Immunodefi
Page 121 and 122:
Page 123 and 124:
Page 125 and 126:
Page 127 and 128:
Adults with Primary Immunodeficienc
Page 129 and 130:
Page 131 and 132:
Page 133 and 134:
Health Insurance chapter 22 Having
Page 135 and 136:
Health Insurance 127 Who Are the
Page 137 and 138:
Page 139 and 140:
Page 141 and 142:
Health Insurance 133 LIFETIME MAXIM
Page 143 and 144:
Glossary 135 Gamma globulins: The p
Page 145 and 146:
Glossary 137 Sepsis: An infection o
Page 147 and 148:
Reference 139 NIH Clinical Trials w
Page 149 and 150:
Reference 141 International Patient
Page 151 and 152:
Reference 143 Manufacturing Compani
Page 153 and 154:
Reference 145 Books For Youth Balkw
Page 156:
This publication has been made poss
show all

IDF Patient & Family Handbook for Primary Immunodeficiency ... - IDFA

You also want an ePaper? Increase the reach of your titles

Delete template?

Save as template?