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Normalizing the Itch: Pruritus and Alagille Syndrome

For people living with Alagille syndrome (ALGS), one challenging aspect that is often overlooked is the chronic itch and its impact on daily life.

ALGS is a rare genetic disorder that affects various organ systems, primarily the heart and liver. When impaired liver function causes the build-up of excess bile in the body, patients may experience pruritus, or chronic itching. With so many symptoms and manifestations and varying symptom severities between patients, many patients with ALGS downplay their experience with pruritus, believing their condition isn’t severe enough to warrant attention. 

But here’s the truth: all levels of itch deserve acknowledgment and treatment. 

Chronic itch is more than just a physical sensation; it can significantly affect a person’s mental and emotional well-being. 

Pruritus doesn’t take a break. 

Imagine waking up each day, already fatigued from a night of restless sleep because of incessant itching. The urge to scratch is a persistent battle throughout the day, disrupting focus and productivity. The constant distraction also makes engaging fully in social activities challenging, leading to feelings of isolation. 

Despite efforts to resist, scratching often becomes inevitable, leading to raw, irritated skin and, sometimes, open wounds. The cycle perpetuates itself as injuries from scratching exacerbate the itch further, creating a loop of discomfort and frustration. Insecurity about the visible scratches resulting from pruritus can compound the emotional toll of living with Alagille syndrome.

Friends and family may find it challenging to grasp the full extent to which their loved one experiences pruritus. This could be, in part, because it can be difficult for patients to openly discuss the condition. When living with a chronic health condition like ALGS, patients might find themselves censoring their daily experiences, concerned about “burdening” their loved ones. Moreover, there’s often a fear of being perceived or treated differently due to their health condition, which can add to the decision not to share challenges. This internal grappling between the desire for support and the fear of being a burden can create a profound sense of isolation, deepening the gap between people living with ALGS and their support networks.

By fostering open conversations about life with pruritus, we can normalize the itch. It’s common to feel overwhelmed by the physical and emotional discomfort of chronic itching, and if you’re dealing with this symptom of ALGS, you aren’t alone. Up to 88% of patients living with Alagille syndrome report pruritus as a symptom, and it is a commonly reported factor in patients’ decisions to receive a liver transplant for ALGS. 

In today’s interconnected world, individuals and families affected by Alagille syndrome can find support and resources through online communities (like The Mighty!), patient advocacy groups, and nonprofit organizations dedicated to rare genetic disorders. These platforms offer a space for sharing experiences, accessing information, and building a supportive network. By sharing personal experiences and stories, patients can help others understand their challenges. This can increase empathy and support from friends, family, and the community.

It’s also essential to approach conversations with your health care provider openly and honestly. Start by outlining the itch’s duration, severity, and specific characteristics, including any factors that exacerbate or alleviate the symptoms. 

It’s also helpful to discuss the impact of the itch on your daily life, including sleep disturbances, emotional well-being, and any coping strategies you’ve tried. Talk to your doctor about any questions you have, or any clarification you desire about ALGS, pruritus, and different treatment options. 

If you are living with chronic itch from pruritus, consider asking your health care provider these questions:

  • Are there potential triggers that can make the itch from pruritus worse?
  • How else can the itch be treated?
  • Are there any tests that could further evaluate or monitor this symptom?

Your health care provider may also have questions for you. One way you can prepare for your next appointment is to consider your answers to these common questions about chronic itch: 

  • How often does chronic itch prevent you from falling or staying asleep?
  • How often does scratching cause skin damage (scratches/open wounds/scars)?
  • Where do you experience the itch most often, and where on your body is the itch most intense?

By collaborating openly with health care professionals and seeking support from loved ones, patients can work toward alleviating the chronic itch associated with ALGS. No one should have to endure any level of chronic itch, and by seeking understanding and effective management strategies with the help of their health care provider, individuals can reclaim a sense of comfort and well-being.

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