Well This Is Just Swell
Chapter 23: Hereditary Angioedema
Much of Sunday was spent in bed for Hilery. Cooter had excused her from going to church due to the hangover. He felt sorry for her, but part of the reason for the night before was so that she could learn the consequences of drinking too much.
Bo had called, but Cooter hadn’t been willing to wake Hilery first thing in the morning. It had been a check-in call, but he understood Cooter’s reasoning for not waking the young woman.
Monday, however, Hilery was up bright and early. She was fixing breakfast when there was a knock on the door. She answered it, spatula in hand and was immediately greeted by a bouquet of cream roses.
“Hey gorgeous.” Bo said as she took them. “How ya feelin’?”
“Better than yesterday,” she said, embarrassed by the show of affection. She remembered most of Saturday night, thanks in part to Cooter cutting off her alcohol intake when he did, but she was slightly troubled. Bo knew her deepest, darkest secret now… and so did everyone else for that matter.
The fact that she was now his object of affection, his only object of affection, was going to take some getting used to. She smelled the roses and smiled. It figured that he would remember what her favorite flowers were. After a few moments she realized herself, opened the door wider, and stepped out of the way.
“Come on in.” she said.
“Don’t mind if I do.” Bo replied, stepping past her. She shut the door and he looked around. “Pancakes and sausage?”
“Yeah, you hungry?”
“Kinda. I left right after chores this mornin’, before breakfast.” He enjoyed her cooking, but the truth was, he hadn’t been able to wait to see her.
“Pull up a chair.” She went back to the stove and flipped the sausage one last time before turning the burner off and removing the food to a plate. “Did Cooter know you were comin’?”
“Yeah. He did. He sorta invited me to breakfast,” he admitted.
“Well that explains why he said to cook extra,” she chuckled. She placed the plate of pancakes and the plate of sausage on the table before grabbing three more plates out of the cupboard.
“Good mornin’,” Cooter said, entering the room. He was freshly showered and shaven, but dressed for a day at the Hazzard Garage.
“Mornin’ Cooter.” Bo replied.
“Mornin’ cousin.” Hilery said as she took her seat.
After grace was said the three of them began to dig in. Then Cooter posed a question. “So what are you two lovebirds doin’ today?”
Hilery shot him a dirty look and Bo chuckled. She cut a piece of sausage in half and stuck it in her mouth, chewed, and swallowed before answering. “I have a doctor’s appointment today; the allergist again.”
Cooter nodded and mulled that over as he chewed.
“Bo, you can come with me if you like. He’s meeting me at Doc Appleby’s office.” Truth was, she didn’t want to be by herself when she got any bad news. She figured that was why Cooter had invited him over for breakfast instead of lunch or dinner.
“Sure thing, hun. Does this have anythin’ to do with those swellin’ episodes you’ve been havin’?” He cut his pancake in half as he asked.
“Yeah.”
Bo was seated in the extra chair in the exam room as Nurse White looked Hilery over, gathering the preliminary information that all nurses gather before the patient is seen by the doctor. He hated doctors’ offices, but he knew Hil needed him to be there with her. As she finished, Doctor Johnson walked in. He took a seat on the stool and opened her chart. He reviewed it silently for a few moments before closing it.
“Are you on any new medications, new vitamins…?”
“No, Doc.” Hilery replied. “Although… my uh… father told me a few nights ago that my mother had had problems with swelling. She died of it when I was six months old.” She blushed when Bo looked at her questioningly. “Bo, I’ll explain later.”
He nodded, not saying a word.
Doctor Johnson took his glasses off and pinched the bridge of his nose. “I know what you have.” He looked back up at her after making a few notes in her chart. “You have Hereditary Angioedema. As your C1 esterase inhibitor levels are normal, and it’s functioning normally, you have type III. All three forms of Hereditary Angioedema involve swelling. The swelling can be extremely painful, and can be, as you have discovered, extremely dangerous if they involve your throat.”
“As such, it is strongly recommended that you don’t take any combination birth control pills. High estrogen levels seem to be a trigger for most women, and they do not tolerate the pill very well. However, I would like to try you on progesterone pills. It’s another type of birth control, but it will help lower your estrogen levels. It is, however, not as reliable as combination pills for birth control purposes. Hopefully it will bring you some relief.”
“There are other treatments available. These are treatments I would not recommend to a young woman of childbearing age, unless there were no other options. It involves androgen therapy. Androgens are male hormones. As you are type III, your insurance may be a little reluctant to pay for them. How they work is they trigger the liver to produce more C1 inhibitor. This sometimes helps those with type III. Also, androgen treatment tends to damage the liver, and can damage the reproductive organs in a young woman. This is one of the reasons that I want to try to progesterone pills first. Going off the pills if they work for you will be risky, however, it will have to be done if you are ever going to have children.”
“Any children you have may inherit the disease. There will be a fifty percent chance of a child inheriting it from you.” He paused, studying her.
“Another treatment which may help you, especially if you find yourself in the Emergency Department and on a respirator again, would be fresh frozen plasma, or FFP. It contains C1 inhibitor as well as whatever is missing in type III patients. The medical community doesn’t know why type III patients swell. There is still much research to be done on that subject.”
“There are other treatments in the works, but they’re many years away from being approved by the FDA.”
When the doctor finished speaking, Hilery looked up, tears streaming down her cheeks. She didn’t want something which she could pass on to her children, and she sure didn’t want to live the rest of her life in fear. She had been hoping it was something that was curable.
Bo got out of his seat and sat down on the exam table next to her, wrapping his left arm around her. She leaned into him, resting her head against his chest.
The doctor handed Hilery a prescription and shook both of their hands before leaving the room.
The Duke boy just held her for a few moments; processing everything they had been told.
“I’m so sorry,” Hilery whispered. “I know you wanted to have kids someday.”
“Hey now, there’s nothin’ to be sorry for Hil. It’s not the end of the world, and the doc said there’s treatments available. As far as havin’ kids, we’ll cross that bridge when we come to it. He said there was a chance any kids we have won’t have it, and if you decide you don’t want to have any yourself, there’s always adoption.”
Hilery pulled back and looked at him. She didn’t smile, but she did hug him. “You always did know what to say to make me feel better.”
“Yeah, although I thought I’d lost that touch at the Church Social.” He blushed. She looked at him curiously.
“I tried to tell ya how I felt then, only ya took it the wrong way.”
“Ah.”
“C’mon, let’s go fishin’.”
Hilery raised an eyebrow. “You can’t fish, your arm is in a cast still.”
Bo shrugged. “Doesn’t mean I can’t enjoy watchin’ you fish.” The young woman couldn’t help but to chuckle before she kissed him. It was a slow, tentative kiss, the type that fit so well for a newly budding romance.
“Very true,” she whispered.
“Now you have got to tell me how you talked to your father, when the man’s been dead for almost eight years now, and I thought your mama died with him.”
She chuckled again and gently punched his good arm. “Bo, I was adopted.”
“Really?” This was news to him. He was very surprised. “Then who…?”
“Cooter’s only my second cousin by adoption.”
“Well that much I kinda figured Hil.”
“Honey, he’s my birth father.”
Bo was dumbfounded. Dating Cooter’s daughter would be quite an adventure, and he’d definitely have to make sure to watch his Ps and Qs. He would have watched them before, but now he was going to have to be even more careful. Finally he gave a smile which became a laugh a few moments later.
“What?”
“Oh, just realized, even though it turns out Cooter is your father, I still don’t have to worry about anymore shotgun wielding daddies! Bar fights maybe, but no shotguns!”
“True, Bo, true… but that’s only because he knows I’ll shoot your behind if you break my heart.” He stopped laughing at that. She did have a good point, and one hole in his shoulder was enough to last a lifetime.
Please take a minute to click on the HAE Appendix link below to read more about Hereditary Angioedema.
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