Every three to four minutes, someone in the U.S. is diagnosed with a blood disease such as lymphoma, leukemia, or sickle cell anemia. As reported in the Pittsburgh Post-Gazette, there is hope, and there are cures through programs such as ‘Be The Match’. Donors on the list are matched with patients, resulting in life-saving blood cell transplants.
About Jax
Missy Ramirez, Jax’s mother, is a psychologist at the Pine-Richland School District. Missy has dedicated her after-work hours to a program called ‘Be The Match’. Over the last few years, Missy has signed up 6,000 donors by attending over two hundred events.
She is hoping to find a matching donor for Jax, her 8-year-old son, who has been living with a rare autoimmune disease called IPEX (Immunodysregulation polyendocrinopathy enteropathy X-linked syndrome).
The syndrome is the result of a mutation in the FOXP3 gene on the X chromosome. IPEX affects boys as they have only one X chromosome, while girls have two. Therefore, if one X chromosome is affected, the remaining healthy chromosome will compensate.
The IPEX syndrome affects approximately 1.6 million people worldwide. According to Dr. Hey Jin Chong, IDDT Director at UPMC’s Children’s Hospital, there have been just a few cases of IPEX reported in the entire Pittsburgh area during the last decade.
The syndrome will characteristically present with dermatitis (eczema), type 1 diabetes (autoimmune endocrinopathy), and autoimmune enteropathy. Dr. Chong, the Immune Dysregulation director at the UPMC hospital, is Jax’s doctor. Dr. Chong told the Gazette that the prognosis is poor for patients who have not been able to find a donor. The doctor stated further that he has had many patients with autoimmune issues. He emphasized that they survived by being able to find a donor and receive a bone marrow transplant.
Jax’s mother added that due to his diverse ethnic background – German, French, Brazilian, Hispanic, and Black, it has been extremely difficult to locate a donor.
However, as Missy points out, there is always hope as so many people are not aware of their ethnic background.
Missy’s campaign is called: Be the Match for Jax. It involves sixty volunteers plus ten more individuals in the area coordinated by Missy who provide public information about the campaign and the disease.
Creating a Normal Life for Jax
It isn’t easy for Jax to appear normal when he must take so many medications, each with their own symptoms.
Also, because of his immune frailties, Jax goes to cyber school. He is, therefore, mostly isolated from his classmates. Missy acknowledges the intense amount of care needed to maintain his current lifestyle.
The family and his 6-year-old brother Lincoln are always planning outdoor activities such as trips to McConnells Mill, walks into town, and family fun sleigh riding at night. Jax’s parents strive to be creative in their planning of outdoor activities.
When asked, Jax will tell you that he showed his class tricks he can do on his mountain bike. Another favorite place for Jax to go is the town skate park. Jax and his mother take his little brother along.
In most discussions, Jax mentions that due to his Type 1 diabetes, he has to ‘have needles stabbed’ in his leg once a day. Jax also gets infusions administered by a nurse every six weeks. His parents try to distract Jax by naming his shots ‘power juice’.
About the Registry
Be The Match reports that fewer than 50% of the list of registry members are available for transplants when needed. Missy hopes to eventually see her activism at a national level because of the critical need for donors.
Ronald Francois, an account manager for Be The Match, reports that seven people out of ten do not have a matched donor in their family. The organization was formed in 1987 and has arranged 125,000 transplants, thereby saving about 7,000 lives each year.
Please visit Be the Match for Jax on Instagram and Facebook for future events or to join the registry text “jaxramirezpa” to 61474 for details.
