All children with serious chronic illness need the support of parents and family members.Requirements for parents of boys with WAS and the decisions they need to take, can be extremely serious.
Progress in the field of clinical nutrition and antimicrobial therapy, prophylactic use of immunoglobulin replacement therapy and bone marrow transplantation have significantly improved SVR outlook for patients' lives.
Due to increased blood loss, iron deficiency anemia is common, requiring the introduction of additional quantities of iron.If you have symptoms of infection should be a thorough examination to detect bacterial, viral or fungal infection, and determine the most effective antimicrobial therapy.
Since CBO abnormal antibody responses to vaccines and to invading microorganisms, patients suffering from frequent bacterial infections can be shown preventative immunoglobulin replacement therapy.Note that
low platelet counts, most physicians prescribe IVIG as subcutaneous immunoglobulins may cause intradermal or subcutaneous hemorrhage.Immunoglobulin replacement therapy is particularly important if the patient has been treated with splenectomy.
Eczema can be severe and permanent, in connection with which the patient may be in need of constant care.Avoid excessive washing of the skin, as frequent washing can cause dryness of the skin and worsen the eczema.
When bathing, use bath oil and after washing several times a day should apply a moisturizing cream.Creams with steroid drugs often help with limited application to chronically inflamed areas, but their excessive use should be avoided.Do not use strong steroid creams, eg fluorinated steroids, on the face.If any nutrients lead to a deterioration of eczema or food allergies are identified, it should be as far as possible to eliminate dangerous foods from the diet.
In some cases, for the treatment of low platelet count and bleeding may require platelet transfusions.For example, if severe bleeding can not be stopped by conservative measures, platelet transfusion is indicated.In intracerebral haemorrhage usually require immediate platelet transfusions.
Patients CBO performed surgical removal of the spleen (a lymphoid organ in the abdomen that filters the blood);shows that this operation removes low platelet count (thrombocytopenia) is more than 90% of cases.Surgical removal of the spleen does not treat other disorders that are typical for the CBO, and it should only be used for the elimination of thrombocytopenia in patients with particularly low platelet counts.
After removal of the spleen is greatly enhanced the effectiveness of substitution administration of high doses of immunoglobulins to increase the platelet count in males, patients with SVR.Splenectomy patients increases the sensitivity to certain infections, particularly infections boiling circulating blood and meningitis caused by encapsulated bacteria, for example, S pneumoniae and H influenzae.
If you are removing the spleen, it is extremely important that the child be prophylactic antibiotics and possibly immunoglobulin replacement therapy, sometimes throughout their lives to prevent these serious infections.Symptoms of autoimmune diseases may require treatment with drugs that further suppress the patient's immune system.In this case, the substitution may help the administration of high doses of immunoglobulins and systemic administration of steroids, and it is very important how to reduce the dose of steroids to the lowest level as soon as possible, providing symptom control.
Boys with CBO, like other children with primary immunodeficiency diseases involving T and / or B-lymphocytes, should not receive live virus vaccines since there is a possibility that a vaccine strain of the virus may cause disease.Sometimes there are complications from chicken pox infection.They can be prevented by appointment (immediately after contact with sick) antiviral drugs, substitutive administration of high doses of immunoglobulins or hyperimmune serum against shingles.
only means of ensuring a permanent cure for WAS is bone marrow transplantation or cord blood stem cells, and the search for an HLA-matched donor should be undertaken immediately after setting CBO diagnosis.Because patients have some residual T-lymphocyte function in spite of immunodeficiency, prior to transplantation of the patient preparation is needed with drugs that suppress the immune system, and / or total body irradiation.
If a patient boy has healthy siblings from the same parents, the entire family should be tissue typed to identify HLA-identical sibling (a good tissue compatibility), which can be a donor for bone marrow transplantation.Bone marrow transplantation from an HLA-identical sibling gives excellent results when the CBO with a total success rate (cure) of 80-90%.This procedure is PREFERENCE-tive for boys with severe clinical manifestations of NWO.
question of bone marrow transplantation from an HLA-matched sibling in patients with milder clinical forms, such as an isolated thrombocytopenia, is more complex and needs to be discussed with an experienced immunologist.The success of the transplant from a matched donor-unrelated significantly increased over the past two decades.
transplantation of fully matched-unrelated donors are now as successful as matched sibling transplants if they are performed in patients aged up to 5-6 years before the emergence of significant complications, such as severe viral infections or cancer.The success rate of fully matched-unrelated donor transplants decreases with age, making it difficult to decision to transplant teenagers or adults with WAS.
fully or partially compatible cord blood stem cells have been successfully used to restore the immune system and correction of violations of platelets in patients with multiple CBO;You can consider the possibility of applying this method when there is no matched sibling or fully matched-unrelated donor.In contrast to the very good results of transplants from HLA-matched donor, haploidentical bone marrow transplantation (used as a donor one of the parents) are far less successful than transplants from HLA-matched donor.
Thirty years ago, the classic Wiskott syndrome - Aldrich syndrome was one of the most severe primary immunodeficiency disorders with a life expectancy of only 2-3 years.
Despite the fact that it remains a serious disease in which the possible life-threatening complications, many patients are males survive to adolescence or even adulthood, live productive lives and have families of their own.The oldest of the patients who have received bone marrow transplants, now in their twenties and thirties and seem to be cured, without developing malignancies or autoimmune diseases.
Article courtesy of a worldwide organization IPOPI, working to improve the lives of people with primary immunodeficiency.
Copyright 2007 owned fund Immune Deficiency Foundation, USA."Guidelines for the primary immunodeficiency diseases for patients and their families", from which the material is taken under the license, was developed Immune Deficiency Foundation with support from Baxter Healthcare Corporation.
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