Recognized as both “butterfly skin” and “the worst disease you’ve never heard of,” epidermolysis bullosa (EB) casts a profound impact on the lives of those affected by its relentless assault on the delicate skin and mucous membranes. This group of genetic disorders presents an ongoing battle with extreme fragility and blistering, underscoring the crucial importance of raising awareness and providing support for individuals facing the daily hurdles posed by this condition.
There are different types and subtypes of EB. The mildest form, known as EB simplex, typically involves blistering in areas prone to friction, such as the hands and feet. In contrast, junctional EB and dystrophic EB can be more severe. Junctional EB affects the skin’s junctional layer, leading to widespread blistering and open sores. It can also involve the internal organs, causing complications.
Dystrophic EB, on the other hand, affects the deeper layers of the skin, resulting in severe blistering, scarring, and deformities. It can significantly limit mobility, require extensive wound care, and cause complications like difficulty eating or breathing.
The impact of EB on daily life, therefore, can vary depending on the form and severity, with more severe forms often requiring specialized care, daily bandage changes, and precautions to prevent injuries and infections.
Currently, there is no known cure for epidermolysis bullosa. Patients focus on managing their condition through various strategies aimed at minimizing symptoms, preventing complications, and improving quality of life. This includes meticulous wound care and working closely with a multidisciplinary healthcare team.
Unfortunately, not many people are familiar with EB which can lead to misconceptions and a lack of support for patients. We hope to help increase awareness by sharing some examples of the challenges EB patients face on a daily basis.
1. Limited Activities
The hallmark symptom of EB is the formation of blisters and wounds on the skin in response to minor friction or trauma. These blisters can be painful, slow to heal, and can easily become infected. Patients with EB must be extremely cautious to prevent injuries and manage wounds carefully.
Depending on the severity, individuals may need to avoid activities that increase the risk of skin blistering, wound formation, and associated complications. Gentle handling is crucial to minimize the potential for skin trauma and blistering, making it essential to avoid rough play or excessive pressure on the skin.
Activities that involve contact with rough surfaces, such as rough carpeting, sandpaper, or rough fabrics, should also be avoided to prevent discomfort and potential harm to the delicate skin.
Aside from these, engaging in activities that involve intense physical contact, running, jumping, or repetitive friction can significantly increase the likelihood of blisters and wounds. This includes contact sports such as football, basketball, or rugby.
Avoiding activities can lead to social isolation or a sense of being left out. EB patients may feel excluded from social gatherings, sports, or events that their peers can participate in, which can contribute to feelings of loneliness and disconnect from their social circles.
2. Laborious Bandage Changes
The daily bandage change process for individuals with EB can be a laborious and meticulous undertaking. It involves carefully removing the existing bandages, which can be adhered to fragile and sensitive skin, requiring utmost caution to prevent further damage or pain.
Once the bandages are removed, the wounds and affected areas must be treated with great care. Gently cleansing the wounds and applying appropriate medications or dressings are essential to promote healing and prevent infection.
The final step in the bandage change process involves the application of new bandages. They need to be applied securely to protect the wounds while ensuring a comfortable fit. The bandages must be carefully positioned to avoid unnecessary friction or pressure that could exacerbate existing injuries.
This comprehensive process requires patience, skill, and attention to detail, often necessitating the involvement of caregivers. It also requires a significant investment of time and effort on a daily basis, which can range from 30 minutes to a few hours depending on the severity, to provide optimal care and support for patients.
For individuals with EB, this means dedicating a substantial portion of their day to the process. It may require adjustments to work, school, or social commitments. It can also impact their ability to engage in other activities or pursue interests. These can lead to a sense of frustration and can be physically and mentally draining.
And since bandage changes often require the assistance of caregivers or healthcare professionals, the reliance on others for this essential aspect of care can impact the independence and autonomy of the individual with EB, potentially affecting the overall quality of life.
3. Treatment Expenses
The burden of medical costs for individuals with EB and their families can be substantial, given the ongoing need for specialized care and management of the condition.
EB patients require a constant supply of specialized, more expensive dressings and bandages to protect their fragile skin, promote healing, and prevent infection. Aside from these, patients often require various ointments, creams, and medications to manage their symptoms, prevent infections, and alleviate pain.
These specialized treatments often require frequent and generous application. Bandage changes may need to be performed on a daily basis or even several times a week. As a result, the expenses incurred from these essential procedures, coupled with the ongoing need for prescription medications, can substantially add to the financial strain experienced by individuals and their families.
In addition, the involvement of a large care team comprising healthcare professionals incurs costs, and travel expenses for accessing specialized care can quickly accumulate, particularly for those living in areas with limited accessibility.
The cumulative impact of these financial challenges can lead to stress, difficulties in accessing care, and potential debt. Exploring insurance coverage, seeking financial assistance from support organizations, and government assistance programs can help alleviate the burden, but it’s important to acknowledge the indirect costs such as loss of income due to caregiving responsibilities.
Addressing the financial burden requires a comprehensive approach that encompasses access to affordable medical care and support systems to mitigate the broader economic challenges faced by EB patients and their families.
4. Visible Manifestations
Individuals with EB often experience unwanted attention and reactions from the public due to the visible manifestations of their condition. Stares, questions, and mistaken assumptions about their appearance are unfortunately common occurrences.
People may approach them with curiosity and ask probing questions, assuming their condition is the result of a burn or accident. These interactions can be emotionally challenging for patients as they constantly find themselves having to explain their condition, which can be exhausting and sometimes intrusive.
In some instances, people move away from them mistakenly perceiving their condition as contagious. This reaction stems from a lack of awareness and understanding about EB. It can be disheartening for patients to experience this fear-based response, as it reinforces a sense of exclusion and can lead to feelings of isolation and self-consciousness.
Additionally, children with EB may encounter teasing or bullying from their peers, further intensifying the emotional impact of their condition. The visibility of their wounds and bandages can make them targets for cruel remarks and taunts, causing them to feel ashamed or embarrassed about their appearance.
In response, some patients may go to great lengths to hide their wounds, wearing clothing that covers their skin or avoiding social situations altogether. The fear of being ridiculed or ostracized can have a profound effect on their self-esteem and social interactions, contributing to a sense of isolation and a reluctance to fully express themselves.
Without a cure, individuals with EB face daily challenges in managing their fragile skin, undergoing laborious bandage changes, coping with the financial burden of specialized care, and enduring the emotional impact of their disease. Increasing awareness and support for those with EB is crucial to improve their quality of life and foster a more inclusive society.
Ben’s Friends has a free and safe online support community for patients affected by EB as well as their caregivers and family. Join our Living with Epidermolysis Bullosa community so you can get in touch with people who truly understand what you’re going through.