WHAT IS IPEX SYNDROME?
• IPEX (which stands for immunodysregulation polyendocrinopathy enteropathy x-linked) syndrome is a condition that affects the immune system of a baby in the first six months of life.
• Most patients with IPEX syndrome are males, and the disease can be life-threatening in early childhood.
• IPEX is characterized by the development of multiple autoimmune disorders, which means the immune system malfunctions and attacks the body’s own tissues and organs.
• Although IPEX syndrome can affect many different areas of the body, autoimmune disorders involving the intestines, skin and endocrine glands occur most often.
• The symptoms of IPEX syndrome include severe diarrhea, diabetes, skin conditions (such as eczema, erythroderma, or psoriasis), anemia and thyroid disease. It can cause failure to gain weight and grow at the expected rate.
• Treatment of IPEX syndrome consists of medications that limit immune system functioning. The only real cure is through a bone marrow transplant.
Source: National Institutes of Health
HOW TO HELP
A fund has been set up to help support the Latham/Johnson family during the grueling process of finding and obtaining bone marrow transplants for the boys.
You can help by donating to:
The Benefit Fund for Bryson and Tré Johnson
Citizen’s National Bank in Sweetwater, Tenn.
Two Sweetwater, Tenn., brothers fighting an unthinkably rare, life-threatening genetic disease will have the chance to be treated by doctors at the leading research hospital for such disorders.
Tré Johnson, 2, and Bryson Johnson, 1, and their family are headed to Cincinnati Children’s Hospital on Sunday to begin what will likely be a year’s worth of grueling evaluation and treatment for IPEX syndrome — a disease that causes the boys’ immune systems to malfunction and attack their own tissues and organs.
It also means their immune systems fail to protect their bodies from infections. There are only 150 to 200 known cases of the disease in the world.
Symptoms of IPEX syndrome include diabetes, thyroid disease, skin conditions, severe diarrhea and anemia, among other problems. It stunts growth and leaves the boys vulnerable to infections like pneumonia. A full bone marrow transplant is their best hope for survival.
The family, which is uninsured, learned in the last couple of weeks that TennCare had approved the boys’ evaluations at the Ohio hospital. Those tests will include whole-body scans, extensive blood work, and scopes in their small lungs and gastrointestinal systems.
“The thought of dropping everything and moving for what is potentially a year is stressful — but other than that, everything is going smooth,” said the boys’ grandmother, Melissa Latham.
Jasmine Latham, their single mom, has had to quit her job to stay with them.
Because the boys are on TennCare, getting approval for the expensive treatments in Ohio has been difficult, but the Lathams’ doctors at T.C. Thompson Children’s Hospital at Erlanger have advocated heavily for the transfer, saying it offers the boys the best chance for survival.
At this point TennCare has approved the evaluations, and doctors are hopeful the agency will cover the transplants.
Tré is expected to be admitted to the hospital for a life-saving bone marrow transplant the final week of August. Doctors will not start work on Bryson’s transplant until after they see how Tré does.
Besides side effects of their constant medication, the boys’ health has been relatively good these past few months, said their grandmother.
Last week, a relative organized a biking and concert benefit in Sweetwater, where $600 was raised to help the family pay for the costs of travel and living expenses during the boys’ treatment.
Contact staff writer Kate Harrison at firstname.lastname@example.org or 423-757-6673.
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