Green and growing: 7-year-old Harrison Green (left) is hooked up to his medicine, which enables his body to create antibodies. Two of his brothers, David (20 months, second from right) and Holden (3, far right), also have XLA, a genetic abnormality. Langford (3, in mom’s lap) is the only one who doesn’t. In spite of the need for constant monitoring, the family tries to keep life normal.
Harrison Green watches his cartoons during a regular summer afternoon at his River Forest home. While his friends are outside playing, the 7-year-old is getting his “sticks,” as the family calls them. Harrison has a rare immune deficiency condition and receives an intravenous injection of a medicine compound that includes donated adult blood plasma. The procedure, which Harrison and his parents, Sonia and Colby, are all too familiar with, lasts about an hour.
Harrison Green and his “sticks.”
Harrison’s condition is called “X-linked agammaglobulinemia, or XLA. It’s an inherited genetic abnormality that prevents the body from producing antibodies to fight off infections.
It afflicts only males and is among the rarest of immune deficiencies, affecting 1 in every 100,000 male newborns, though it can go undiagnosed for years.
Two of Harrison’s younger brothers, Holden, who’s 3, and Davis, who’s 22-months-old, also have condition. Their other brother, Langford, Holden’s twin, is the only one who doesn’t have it.
Outwardly, the boys are as normal as any other kids.
“He’s never asked, ‘Why me? Why am I different?'” Sonia says of Harrison. “There were days when I was hurting for him because other kids were outside playing, and he was inside getting his treatment, but it never really bothered him. And the other guys, they know that they get sticks, so they know that they have something else. It’s just been a part of our lives.”
David and Langford Green.
X-linked agammaglobulinemia prevents the body from producing antibodies, the proteins secreted into the blood, or lymph system, to attack viruses and bacteria.
The condition is caused by a mutated, or deficient, X chromosome, which can result in the absence of the B cells, a particular antibody-producing white blood cell.
Women are only carriers of the condition. Since women carry two XX chromosomes, if one mutates, they have an extra X chromosome that can produce the B cells. Men, having an X and Y chromosome, can inherit a mutated X gene with no “spare.”
Sonia wasn’t aware she was a carrier until Harrison was diagnosed as an infant. Boys have a 50/50 chance of inheriting XLA from their mothers. No one else in her family has the condition.
Since XLA sufferers can’t produce their own antibodies, treatment involves receiving a medicine compound consisting of gamma globulin, or immunoglobulins, which help produce antibodies. The medicine is given intravenously.
Harrison gets his treatment from his home care nurse once a month. The other two siblings get infusions done by their parents every two weeks. Harrison’s one-a-month treatment is a newer procedure, but the parents have decided, for the time being, to stay with the two-week treatment for Holden and Davis.
Because of their condition, the boys are more susceptible to certain illnesses, such as sinus infections or a fever, than other kids. If the boys get a fever, the family gets concerned because it could indicate a serious bacterial infection.
They’ve been to the emergency room on more than one occasion when Harrison has had a fever, his parents said.
Because of Harrison’s vulnerability, teachers and other school officials, the Greens said, had to be educated on his condition and how to respond to it.
Getting their ‘sticks’
Harrison entered the second grade at Willard Elementary School in River Forest this fall. His brothers attend Keystone Montessori School in River Forest. Nurses and other officials at both schools are aware of the boys’ condition, and they promptly call their parents if the boys get sick.
They do receive antibiotics every day along with their infusions, their parents said. They can’t receive the normal battery of immunizations, however, because their system can’t produce antibodies.
When it’s time for their home infusions, which they get with a needle, the family calls it “getting their sticks” because the boys often put it that way. “When do we get our sticks?”
Harrison gets his sticks in his arm with an IV and machine pump. It lasts about an hour and a half. Holden and Davis get theirs through a “button” in their stomachs. Some diabetic patients, for instance, receive medications via a button, which is surgically placed in a person’s stomach. The younger boys have the buttons because of their small size, Sonia said.
They’ve tried, she says, to make the situation fun for the boys. When it’s time to get their sticks, the boys watch TV and eat candy. Harrison has grown to like it.
“I love them,” he said.
Even Langford, the only son without the condition, gets a stick, and lets his brothers know when it’s medicine time. He may take a little drop of medicine himself, which won’t harm him, and holds his baby brother’s hand when he gets his sticks, Sonia said.
Harrison usually watches cartoons-Tom and Jerry is one of his favorites-when he’s getting his treatment. Sometimes he has to explain his condition to his friends.
“I say, ‘It’s like getting a shot,'” he said. “I tell them it’s medicine that keeps me from getting sick.”
Sonia and Colby said they’ve tried to make things as normal as possible for the boys. Though they’re young, they have a pretty good understanding about their situation.
“We try not to make a big thing about it,” Sonia said. “Our whole goal is not to have them feel different or scarred, or anything. [They’re] basic understanding is that [their] immune system doesn’t make certain things, and they need to be put in. Their immune systems don’t work the way immune systems should. What they have to get on a regular basis is an infusion of what their body isn’t producing. They just know. They all line up and say ‘medicine time.'”
Teamwork
The parents have had to become amateur doctors to an extent. They’ve developed something of a teamwork approach. Sonia is more of the medical expert and advocate while Colby is more of the medical technician, setting up the pumps and giving the younger boys their sticks.
“That’s really my area,” he said. “I’m usually the one who sticks the younger guys. Mainly, what I work with them on is just trying to get them to understand that it’s going to hurt for about a second, and then we’re done hurting. I think I’ve gotten them there pretty much.”
Colby is the medical technician of the family.
And the condition hasn’t prevented the boys from having a regular life, their parents insist.
“There’s nothing they can’t do except when they’ve got the IV in them for a couple of hours,” Colby said.
But things weren’t always so normal, especially since nether Colby or Sonia had ever heard of X-linked agammaglobulinemia before Harrison’s birth.
Harrison was diagnosed with XLA when he was 22 months old. From the time he was 16 months he suffered several infections, including staph, his parents recalled. He also had a cough that wouldn’t stop. It was then that an allergist at their hospital suggested checking the B-cells in his immune system, which produces antibodies. They discovered he didn’t have any.
The family was sent to an immunologist, who diagnosed the condition.
“When he told us, he said, ‘Your son has this condition, and he’s going to need treatments,'” Sonia recalled. “And when he said that Harrison was going to need infusions, I thought he was going to need a blood transfusion and was going to be hooked up to something. I mean, we’re calm about it now, but at the time, it was pretty devastating.”
The doctors tested Sonia and found she was a carrier. After Harrison’s diagnosis, he started treatments immediately, she said. Other than the injections, he seemed healthy.
“At first, he didn’t like getting stuck,” Sonia said.
When she and Colby chose to have more children, they decided to use in vitro fertilization.
They decided to test each embryo to see which of the twins was female. They eventually found that they were having two boys. When the twins were born, both were tested, and one, Holden, had the condition.
Holden and David consult with their nurse, Lois Messina.
Sonia, however, was, and remains, healthy. Carriers like herself show no symptoms. When the twins were 7 months old, she became pregnant with Davis, who would also be diagnosed with the condition.
The condition is considered X-linked because the female carrier could pass along a mutated gene to her son. Another difficulty with the condition is that most boys who have it look relatively healthy.
If infant boys do get sick, it’s rarely viewed as a problem with their immune system, the Greens said.
“That’s what makes me want to talk about it,” said Sonia. “[Harrison] turned out fine, but if we would have known about this earlier, it would have been even better.”
Most families they’re aware of have just one son with the condition. Their situation is unique because they have three.
The long-term prognosis for those with XLA is good. Prior to gamma globulin treatments, which began in the 1950s, 90 percent of patients died before the age of 8.
Now, individuals with XLA, with treatment, can live long and productive lives. Sonia and Colby said they’ll talk to the boys about managing the condition as adults down the road. Right now, they’re letting the boys be boys.
“As long as they get their treatments, they can do pretty much anything other kids can do,” Sonia said. “As long as they get the treatments the prognosis is good, but it’s a lifelong condition. It’s something they’ll have to manage.”
CONTACT: tdean@wjinc.com
