Many people with X-linked agammaglobulinemia find it challenging when it's time to move (transition) from pediatric to adult health care services. The Sibling Support Project is a nonprofit organization that serves the concerns and needs of siblings of people with special health, developmental, and mental health needs. Some children may benefit from support outside of the immediate family including therapists or doctors. Parents are encouraged to make time for activities especially for healthy siblings and to talk with and encourage healthy siblings to address their concerns and feelings. Siblings can help with the care of their sibling with the chronic illness. Parents can work with their other children by having clearly defined roles and responsibilities within the family structure. Although studies have shown that siblings of children with chronic illness tend to develop strong levels of compassion and empathy, these studies have also shown that siblings can develop feelings of jealousy and anger and high levels of anxiety. As with similar disorders, it is easy for healthy siblings to feel as if they have been pushed aside. X-linked agammaglobulinemia may demand a lot of time and focus from parents. Will my insurance cover treatment for X-linked agammaglobulinemia? How can my doctor learn about X-linked agammaglobulinemia? Should I tell my child's school the diagnosis of X-linked agammaglobulinemia? What is the life expectancy of someone with X-linked agammaglobulinemia? Where can I get financial help for X-linked agammaglobulinemia? Should individuals with X-linked agammaglobulinemia quit smoking? Should my child with X-linked agammaglobulinemia take all of his antibiotic medication if he's feeling better? Should my child with X-linked agammaglobulinemia be vaccinated?Īre there any drugs that should be avoided by people with X-linked agammaglobulinemia? Is proper sleep important for people with X-linked agammaglobulinemia? Is exercise important for people with X-linked agammaglobulinemia? How do children with X-linked agammaglobulinemia go from pediatric care to adult care? Is eating right important for people with X-linked agammaglobulinemia? What can I do for siblings of kids with X-linked agammaglobulinemia? 3 pts presented lung cirrhosis.Ĭonclusions: Searching for an underlying immune defect in pts with chronic lung disease is very important.ĭelayed or suboptimal replacement therapy may result in chronic pulmonary disease or bronchiectasis.Can my child with X-linked agammaglobulinemia play sports? Clinical diagnosis was established at 3.9 yrs (2.0 - 5.4 yrs), replacement immunoglobulin therapy was induced at 5.3 yrs (2.3 - 15.7 yrs). Pts with bronchiectasis developed first symptoms at mean age 1.8 yrs (0.6 - 4.7 yrs). The most common complications were the lower respiratory tract infections: 71% pts suffered from bronchitis, 51% pneumonia, 30% bronchiectasis (10 pts). Replacement immunoglobulin therapy was induced at mean age 3.5 yrs (range 0.11 -12 yrs). Total number of B cells was decreased (range 0 - 391 cells/μL, mean 0.65). All pts presented normal number of CD3+, CD4+, CD8+ cells. All immunoglobulin classes were below 2 SD. Clinical diagnosis was established at 3.9 yrs (range 0.11-12 yrs). Results: First symptoms of XLA occurred at mean age of 1.3 yrs (range 0.2 - 7 yrs). The retrospective analysis of personal, laboratory and clinical data was carried out. Materials and methods: The group under study comprises 33 boys meeting definitive criteria for diagnosis of XLA. XLA is characterized by virtual absence of all immunoglobulin isotypes and of circulating B lymphocytes. XLA is PID caused by Bruton's tyrosine kinase gene mutation. PID is identified as the underlying cause of bronchiectasis. Pts with PID suffer from recurrent and/or severe infections that frequently involve the lung. Primary immune deficiencies comprise a heterogeneous group of genetically determined disorders that affect development and/or function of innate or adaptive immunity.
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