When you’re a parent and something terrible happens to your child, your natural inclination would be to do something about it. That’s what local Chicago mother of four, Sonia Green did when she learned her first child, Harrison was suffering from some unusual infections at a very young age.
At 14 months old, Harrison cut his lip and the wound turned black. Several months later, he went through other recurring infections, one in his blood and several in his ears and lungs. When his mother saw this, she suspected a deeper cause to his infections. It turns out she was right.
A simple blood test revealed that Harrison had XLA, a form of Primary Immunodeficiency (PI). He will need to take therapy for the rest of his life, but his mother’s persistence to get answers has saved him years of misdiagnosed illness. Three of Green’s four sons would soon get diagnosed with XLA as well.
So what is XLA and PI? Green talked to Brandi Walker about what these conditions are, how she has dealt with her sons being diagnosed with them, and how she plans on continuing to increase awareness of PI.
Brandi Walker: Tell me a little about yourself.
Sonia Green: I’m a mom of four boys, ages 11-16: Davis (11), twins Holden and Langford (12) and Harrison (16). My boys are all miracle kids, who were born after years of infertility and loss, including a stillbirth. Three of the four have a primary immune disease called x-linked agammaglobulinemia (XLA), but with treatment, they are able to be active kids. We went through a lot to become parents, and now have a busy and happy life, taking kids to soccer and theatre practice and doing a lot of goofing around at home. I’m a law professor at John Marshall Law School in Chicago, an avid reader and a huge soccer fan. My husband is an attorney who works from home, and we share the work and joy of caring for the boys. In addition to our boys, we have a cat, a dog, and eight gerbils.
B.W.: For those who are not familiar, what is PI and XLA?
S.G.: Primary immunodeficiency (PI) is a group of more than 200 rare, chronic disorders in which part of the body’s immune systems is missing or does not function properly. X-linked agammaglobulinemia (XLA) is one type of PI. XLA is a condition that affects only males. Males who have XLA produce B-cells, but the B-cells never mature and so are unable to do their job. Their immune systems are basically only half effective. Without B cells, XLA patients do not produce immunoglobulins and are unable to fight off certain infections. Without treatment, patients are prone to multiple, recurring infections of the lungs, sinuses and other areas. While there is no cure for PI, there is an effective treatment: the patients get infusions of immunoglobulin from donated human blood plasma, which is distilled to form a clear liquid medicine that provides them with what they are lacking. They are all on a low dose of antibiotics, just prophylactically. The infusion medicine they get (IVIG – intravenous immunoglobulin) needs to be administered often since their bodies use up the added immunoglobulin, so each of my sons has to have an infusion every 28 days, and the infusions last for several hours.
B.W.: How much of an ordeal has your sons health been for you and your husband?
S.G.: It has varied over the years. When the boys have had their infusions and medicine and are feeling healthy and energetic, we feel like a normal family. My boys play soccer (two, on very competitive teams with tournaments across the country) and their interests range from programming (Harrison) to theatre (Holden). There are many days when we don’t think about PI or XLA. It helps that we have educated our schools, our friends and community so they know what to watch for to keep our kids healthy. Then, we have their treatment days. On these days, our house becomes a home health center, and we have a nurse who comes to infuse all three boys. Our home health nurse is like a family member to us, and she can usually start their IVs quickly. The infusions last a few hours, so we have one day out of 28 when we are definitely different. At worst, we have had days and weeks when the boys, despite their treatments, get very sick, oftentimes with mystery ailments that require a hospital stay. About two years ago, Holden got a skin infection that turned very bad very quickly, and he needed a hospital stay and IV antibiotics. That week turned our whole world upside down, and was definitely an ordeal, but we were lucky to have friends step up to help us out. The other part of it is just getting our boys’ treatment and dealing with insurance coverage: that adds a whole layer of stress on top of the medical issues.
B.W.: How do you plan on continuing to increase awareness of PI?
S.G.: I am really involved in advocacy for patients with PI because there are still many people who are undiagnosed, and because the public needs to be more aware about the often silent conditions, demonstrated by the fact that 70-90% of people with PI worldwide don’t know they have it. I’ve given interviews and posted on blogs where I can. I share information with other parents via email and in person, and I’ve talked with physicians. My sons and I have gone to DC to advocate for patients with PI: we want to make our legislators aware of these conditions, and we want to put a human face on them. My boys have done presentations at their schools, too: they know it’s important to raise awareness. We are really grateful to websites like this one that take the time to share information about these conditions!
B.W.: Where could people go to find more information about PI and XLA?
S.G.: Luckily, there are many great resources for patients with PI. One of my favorites is http://myigsource.com/. This site has information about the various conditions, emotional and treatment related information and financial and insurance resources. For us, the patient advocates we’ve reached through that site have helped us with some really tough insurance problems. They were also kind enough to send us magazines and introduce us to IGI the IVIG bear: a stuffed animal who comes with supplies that kids can use to start pretend IVs on him. They have learned a lot playing with IGI! The Immune Deficiency Foundation has also been a wonderful resource as well, for information, support, and advocacy tools (primaryimmune.org). Now, we also have some great Facebook groups where parents and caregivers can exchange information and sometimes just send each other virtual hugs.
B.W.: What advice do you have for other parents dealing with their children’s health?
S.G.: Have wine on hand! No, seriously – my best advice is to create a support network right away: use resources that are available for patient support, find other parents whose kids have similar conditions, and talk to others whose kids are older. For us, meeting a family with a son who had XLA and is about 15 years older than my oldest has been huge: we see all the amazing things he’s been able to do (and now he’s a dad himself!) so we know what is possible. I would also advise parents to establish a good connection with their physicians, and to make sure that their team is working together. Don’t be afraid to insist on that. Part of the job of having a child with any special medical issues is being an advocate for your child, and it’s not pushy or wrong to be the best advocate you can be. Take time too to enjoy your child, and don’t wish that s/he were different. We tell ourselves and our boys this: everyone has something. Everyone. Every person has some issues and the only thing to do is accept what you have, find the best treatment, and don’t let the condition define you.