Living With a Rare Condition, a 25-Year-Old Faces One of Life’s Hardest Decisions

What Is Autoimmune Autonomic Ganglionopathy (AAG)?

AAG is an autoimmune disease, meaning the body’s own immune system mistakenly reacts to parts of the body, causing serious difficulties.

In this condition, the immune system affects the autonomic ganglia—parts of the nervous system that control automatic body functions such as reflexes, digestion, and more.

AAG disrupts the way the body processes nerve signals, which can interfere with many involuntary physical functions.

Symptoms of AAG

Because the autonomic nervous system controls so many processes, symptoms can vary widely.

According to Cleveland Clinic, common symptoms may include constipation, dilated pupils, dry mouth or dry eyes, lightheadedness or fainting, urinary retention, and low blood pressure upon standing.

The clinic also notes that about two-thirds of patients have high levels of a specific antibody called ganglionic acetylcholine receptor (g-AChR) antibodies, which researchers believe may influence symptom severity.

Is AAG Treatable?

Because AAG is so rare, there is no universally standard treatment. Doctors typically create personalized plans based on each patient’s needs.

While Annaliese’s case is very severe, the condition can present in many different ways.

Although AAG currently has no cure, treatments aim to manage symptoms and improve quality of life. These may include plasma exchange, intravenous immunoglobulin (IVIG) therapy, corticosteroids, or immunosuppressive medication, according to Cleveland Clinic.

Many patients are able to manage their symptoms long-term with ongoing medical care.

Annaliese’s Story

The condition has significantly affected Annaliese’s life.

“I’ve been sick since being a child really, I lived in and out of the hospital ever since I went in for nine months,” she told news.com.au.

“It actually took until I transitioned to the adult hospital that we found the diagnosis, and it came back that I did have this autoimmune disease that was causing it all along.”

The condition has resulted in multiple surgeries, and she has lost several metres of her bowels over time.

“My stool would back up so much that I would throw it up or drain it out my tummy,” she explained. “I was put on something called Total Parenteral Nutrition or TPN and that’s basically a bag of nutrition that’s delivered directly into your bloodstream through like, a line in your chest.”

Because her condition can progress unpredictably, Annaliese has spoken honestly about wanting to consider voluntary assisted d.y.ing if her symptoms become too overwhelming. For her, having this option brings a sense of peace.

“For me, I don’t want to have to wake up every day with anxiety about the pain that I know is ahead for me,” she said.

“The pain of starving to death when they can’t feed me anymore, or the horror of sepsis. Knowing I can go when the time is right is just a huge relief.”

Source: unilad.com