Spring 2024 Intern Articles

About

This publication compiles a series of articles authored by Scleroderma United’s Spring 2024 Intern Cohort. These articles were written as part of an educational program intended to encourage and guide student interns in learning about scleroderma and related medical/healthcare topics.

The information presented in these articles should not be considered as a substitute for professional medical advice, diagnosis, or treatment. Readers are strongly encouraged to consult with qualified healthcare professionals regarding their individual medical conditions, as inaccuracies may inadvertently be present in intern articles. The content is not intended to endorse any medical treatments or approaches. Readers are advised to independently verify information and seek professional advice before making any decisions based on the content of this publication.

Authors

We are very happy to publish articles authored by the following interns: Aania Garg, Anaiya Khan, Analiese Godin, Angela Liu, Alex Nguyen, Anika Deshmukh, Anoushka Bellum, Anusha Rahman, Aryan Krti Iyyappan, Christina Zhu, Christopher Yeow, Davia Williams, Diya Joshi, Elma Cesic, Elyse Da Costa, Franny Khine, Heqing Feng, Imaan Mehmood, Isha Parikh, Jadey Ding, Jaguar Ngo, Jia Hui Lin, Jisara Wuttikreekiat, Joy Wei, Kaitlyn Flanagan, Karson Yuen, Liya Khan, Liyana Haque, Martin Nguyen-Khoa, Medha Nandikur, Mia Szczesniak, Minhanh Nguyen, Mya Silveyra, Nathan Morales, Navya Dubey, Nichu Shibu, Nithusha Ranjith, Nuzhat Azim, Prisha Phatnagar, Sarika Rahman, Sehrish Hirani, Shria Ajay, Sonia Lee, Sonal Jain, Sophia Neuenhaus, Sophie Choi, Sophie Citron, Tharmitha Ramkumar, Tisya Mahajan, Tonique Swaby, Valeria Bianchi, and Valeriia Svyrydova.

Scleroderma's Silent Battle: The Importance of Understanding Neuropsychiatric Symptoms

By Aania Garg from New York City, USA

Beyond the Visible

Scleroderma, a complex autoimmune disease, systematically affects multiple organs in the human body, including the lungs, heart, and gastrointestinal tract, leading to a wide range of symptoms that vary significantly from patient to patient. While the external changes in scleroderma, such as hardening of the skin and Raynaud’s phenomenon, are the most visible and often the initial indicators for diagnosis, patients frequently endure neuropsychiatric symptoms, which although less apparent, are profoundly debilitating and significantly impair their quality of life.

These psychological effects often receive less attention in clinical settings compared to the physical aspects of the disease. The reasons for this oversight are manifold but largely stem from a historical focus on visible symptoms that are more easily quantifiable. However, the spotlight should be shifted onto these hidden struggles, and a holistic approach to scleroderma treatment that acknowledges and addresses the full scope of scleroderma’s impact is crucial for providing comprehensive care and improving overall patient outcomes.

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Understanding Neuropsychiatry

Neuropsychiatric symptoms refer to mental health problems that occur in conjunction with chronic illnesses, including cognitive dysfunction, mood disorders, and anxiety. These symptoms arise as a direct consequence of the disease’s impact on the body and are compounded by the emotional strain of living with a chronic, visibly altering condition. The persistent pain, significant lifestyle adjustments, and the social stigma associated with physical changes can trigger or exacerbate these psychological issues.

Research indicates that approximately 60% of scleroderma patients experience depressive symptoms, with a similar percentage reporting anxiety disorders. This high prevalence highlights the significant impact of scleroderma on mental well-being. Biologically, the chronic inflammation and immune system dysregulation common in scleroderma are believed to influence neurotransmitter functions and brain chemistry, potentially leading to neuropsychiatric conditions. Psychologically, the constant stress of managing a chronic illness and the fear of social rejection due to physical symptoms contribute significantly to mental health declines.

Resources for Support

Many patients affected by scleroderma have found strength and practical help through various support systems and coping strategies, which have proven to be critical components of managing both the symptoms of the disease and the psychological stress it can induce.

Online communities and support groups are particularly valuable resources. These platforms offer a space where patients can share experiences, treatment tips, and emotional support from others who truly understand the realities of living with a rare chronic illness. For example, the Scleroderma Foundation’s online forums provide a structured environment where patients can connect, share information about managing symptoms, and discuss coping strategies such as meditation, exercise, and diet modifications tailored to their needs. Additionally, reaching out to family and friends for support can help significantly. Even though it might be tough, loved ones are only there to support you, and can offer practical help, emotional support during tough times, and

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transform the landscape of scleroderma treatment and truly empower our patients in their journey toward wellness.

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Exploring Challenges in Diagnosing Scleroderma

By Anaiya Khan from Newark, Delaware, USA

Scleroderma is a rare autoimmune disease with skin thickening as its hallmark that can also affect internal organs and, potentially, shadow your entire body Scleroderma is challenging to diagnose due to differences in symptoms that may imitate other diseases. No tests can confirm scleroderma so the diagnosis relies on individual evaluation and testing. However, diagnostic uncertainty can result in a lengthier delay in appropriate treatment of up to one’s entire body.

Diagnosing scleroderma poses challenges for medical professionals because of understanding the difficulties associated with diagnostic uncertainty, such as the many symptoms and the absence of definitive tests, we seek to uncover potential solutions. The goal for the future is to advocate for improved diagnostic approaches to ensure timely and accurate identification of scleroderma, to ensure the patient is getting the right treatment and medications.

The symptoms of scleroderma can range from coughing to Arithisis. Scleroderma symptoms vary widely and can affect different parts of the body physically and mentally. This variability makes diagnosis challenging because symptoms may not always be clear or consistent due to the lack of specific diagnostic tests. There are no specific markers or tests that definitively diagnose scleroderma, adding to the difficulty of accurately

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https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5412724/ https://newsnetwork.mayoclinic.org/discussion/hold-for-zebra-evergreen-needs-image-text-connect-link-x-2-reviews-consumer-health-what-do-you-know-about-scleroderma/ https://www.aad.org/public/diseases/a-z/scleroderma-symptoms https://scleroderma.org/ https://patient.boehringer-ingelheim.com/more-than-scleroderma/understanding-scleroderma/testing-for-scleroderma/blood-and-urine-tests

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Photo Source here

What comes to mind when thinking about rare diagnoses? For some people, it's easy to dwell on the negative aspects and the difficulties of managing. Naturally, being diagnosed with a rare disease is a shocking, devastating, overwhelming, and life-changing event; however, it is vital to have an optimistic outlook and to embrace the positive aspects. Many individuals diagnosed with Scleroderma have done just that, and it has changed not only their outlook on their "new" life but has given them the opportunity to find their purpose.

Positive Aspects

Scleroderma is a rare, terminal autoimmune disorder that can have a significant impact on diagnosed individuals' quality of life, causing physical symptoms as well as emotional and psychological effects. The symptoms range in severity from hardening and thickening of the skin to various effects on the internal organs. Despite the challenges that come with this disease, many individuals have found ways to manage their symptoms which has led to them living fulfilling lives. These individuals have learned to embrace the positive aspects of their diagnosis, such as developing increased self-awareness and self-care. They have achieved this greater level of self-care by listening more closely to their body's needs and prioritizing their health,

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both physically and mentally. By embracing their Scleroderma diagnosis, many individuals have learned to embrace gratitude, pursue everything they are capable of doing, and experience new things. This shows that many individuals are taking advantage of their changed lives to develop a mindset shift that is more positive and more appreciative. Because of this, many individuals have learned to change their perspectives in life and their priorities for the better. Diagnosed individuals also have the opportunity to connect with others who are going through similar experiences and the chance to raise awareness about rare diseases. This has resulted in improved relationships and social support for many individuals.

Shared Experiences

In fact, many individuals with Scleroderma have shared about their changed lives and newfound positivity from their diagnosis. For example, Amy Gietzen, who was diagnosed with Scleroderma in 2011, has written many articles focusing on how her diagnosis has led to her finding her purpose in life. She said that in the beginning, being diagnosed was naturally one of the scariest moments for her; however, now it "has become the single greatest factor" in her life, helping her find her "true self" (Gietzen, 2023). Looking at the positive side of things is crucial for patients' mental health and also in understanding that everything happens for a reason, especially when dealing with health challenges. Amy even states that "Scleroderma has carved out an identity" for her (Gietzen, 2023). Yogita Sharma, who was diagnosed with Scleroderma in 2016, also shared about her experiences with Scleroderma United. She discussed that since her diagnosis, she has learned to count her blessings, live life to the fullest, and fully express herself. Prior to her diagnosis, she was more of an introvert; however, Scleroderma has given her the opportunity to come out of her shell (Scleroderma United, n.d.). It is truly inspiring to hear stories from diagnosed individuals like these who are coping positively with their diagnosis and looking at their lives through an optimistic lens despite their disease and its challenges.

Support Groups

Another key component to staying positive is support and communication. Because of this, many people have found support groups and organizations to be highly beneficial in positive outcomes. One of the most important aspects of support groups is that they offer a sense of community and belonging. Scleroderma patients often feel isolated and misunderstood, and support groups can offer a space where they can connect with others who

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understand first-hand what they are going through. Amy shared in her article that after being diagnosed, she began connecting with others who were diagnosed, joined organizations, and began educating others about Scleroderma. From this, she said she felt like she "became part of a broader community" and "wasn't alone anymore" (Gietzen, 2023). Scleroderma support groups have proven to be a safe and supportive environment where individuals can share their experiences, challenges, and successes with others who are going through similar situations, which can help many stay positive and motivated despite their disease. These support groups can be found online, through social media, or in many communities across the globe.

While living with a Scleroderma diagnosis can certainly be challenging, it is inspiring to see how individuals can transform their experiences into opportunities for growth, self-improvement, and positivity. It is important to acknowledge the positive effects that come with a diagnosis; hence, it is crucial to encourage those diagnosed with Scleroderma or other rare diseases to develop a positive outlook on their "new" life as well as encourage these patients to seek support and connect with others who share similar experiences. By discovering hope through an overwhelming and difficult time, many individuals can learn to navigate their journey effectively and find their purpose in their "new" life.

References

Gietzen, A. (2023, July 26). How a scleroderma diagnosis changed my purpose in life |... Scleroderma News. https://sclerodermanews.com/columns/how-scleroderma-diagnosis-changed-purpose-life/%20

Gietzen, A. (2023, March 15). Accepting all of my feelings about scleroderma | Scleroderma News. Sclerodermanews.com. https://sclerodermanews.com/columns/accepting-all-my-feelings-scleroderma

United, Scleroderma. Yogita Sharma - Scleroderma United. (2023, September 23). https://www.sclerounited.us/yogita-sharma/

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Methotrexate and Emerging Treatments for Juvenile Localized Scleroderma

By Alex Nguyen from Scarborough, Ontario, Canada

The condition known as scleroderma is linked to an overabundance of collagen synthesis, which results in fibrosis of the skin and other organs involved. Systemic sclerosis (SSc) and localized scleroderma (LS) are the two primary types; LS is six to ten times more frequent in children. Dermatologists also commonly refer to LS as morphea. Life-threatening conditions such visceral organ fibrosis and vasculopathy are common in SSc patients, whereas they are rare in LS patients. However, extracutaneous involvement of LS patients can result in significant deformity and functional disability.

Treatment for juvenile localized scleroderma (jLS) is dependent on the severity of the condition. A pediatric rheumatology research network subgroup (CARRA) has generated classifications for the disease severity. Low-severity is diagnosed in patients with morphea that is not associated with subcutaneous atrophy or scalp hair loss. These patients are given topical medications (corticosteroids, tacrolimus, imiquimod, vitamin D analogues) or phototherapy as treatment. Patients with deeper tissue involvement and all other subtypes fall under the category of moderate to high severity. Clinical exams might not be able to identify this, and low-severity cases may develop this later.

Pediatric rheumatologists and dermatologists did a randomized treatment of methotrexate in combination with corticosteroids on patients with

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moderate to high severity jLS. The goal of this treatment is to suppress inflammation and reduce the risk of major morbidity development. It might be difficult for dermatologists to identify flare-ups or treatment effectiveness in children who have entered the pubertal growth spurt since they may be in remission from the medication. Because 15% to 53% of jLS patients experience relapses, long-term monitoring is typically required.

Additionally, it was discovered that certain jLS patients who did not exhibit methotrexate tolerance benefited from mycophenolate mofetil. In adults, phototherapy is successful; however, no evidence was found for individuals at a premature age. As alternatives for nonresponders, several immunomodulators—including biologic agents—are being investigated. Surgery can be used to treat atrophy and other abnormalities in emergency situations, but it should only be taken into consideration when the illness has become inactive as a result of post-operative flare-ups.

Despite being thought of as self-limiting in the past, juvenile localized scleroderma are now more often acknowledged as chronic relapsing-remitting illnesses that call for ongoing care and long-term monitoring. There is evidence for the use of biologics and small molecules in scleroderma in addition to corticosteroids, mycophenolate mofetil, and methotrexate. In order to lower long-term morbidity, extracutaneous symptoms must be recognized. Unfortunately, there do not seem to be any other known medications that can stop juvenile scleroderma from getting worse. Additional outstanding study has unveiled the therapeutic arsenal that targets adult tissue fibrosis. In the near future, new methods and technology will be needed to assess how well these therapies work on children.

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I. Introduction

Scleroderma, a rare, chronic autoimmune disease, most commonly affects adults aged 20-50. The disease, in which there is an excess of collagen and connective tissues within the body leads to the hardening of skin due to a change of cellular structure and vascularization of the body's tissues. Despite not being an inherited condition, a trend of genetic predisposition is seen in some individuals who have associated family members with the same condition.

Emotional and psychological impact upon individuals remains an often uncharted area within Scleroderma research. Major themes emerging from live interviews of patients afflicted with scleroderma were a deep change in personal identity, including mental readjustment and processing of grief through the loss of mobility and changes in physical facial features. Australia’s limited research on this facet due to a preference for a purely biological approach often pathologizes and minimises the experience of grief in individuals, generating difficulty for individuals in accessing community and maintaining a tangible sense of autonomy. Women remain disproportionately affected in this regard, their condition often causing further difficulties navigating culturally idealised body image and

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Toby reported that maintaining clear headedness when feeling overwhelmed helped him maintain clarity when making important decisions about treatments or interacting with their loved ones and health care professionals. The verbal expression of scleroderma experiences, both in person and through social media was also seen as imperative in assisting loved ones and caregivers in maintaining clear communication and empowering the patient’s autonomy in concurrence with their diagnosis, leading to decreased emotional distress. Similarly, connecting to other individuals with Scleroderma as well as SSc societies and reputable organisations, not only leads to accessing greater information, emotional support and strength against daily struggles, but generation of funding for clinical research on the condition.

V. Gender Perspective: Coping with Changes in Physical Appearance

Women remain disproportionately affected by scleroderma and as such, face unique challenges related to changes in physical appearance. Amin K’s (2011) scientific journal, focusing on body image concerns in systemic sclerosis, found in a study of 489 patients (432 female, 57 male,) that dissatisfaction with appearance alongside social discomfort was strongly correlated with the extent of skin involvement. The standardised regression coefficients were = 0.02, p = 0.001; 0.02, p = 0.020, respectively), as was skin involvement in the face (0.18, p = 0.016; 0.23, p = 0.006, respectively). Additionally, greater social discomfort was increasingly associated with younger age (-0.017, p<0.001) and upper-body telangiectasias (0.32, p = 0.021), relaying severe concern for the psychological wellbeing of young women with Scleroderma growing into adulthood in regards to social functioning and self esteem. Dissatisfaction with appearance was associated with hand contractures (0.07, p = 0.036,) symptoms that occur typically as a result of Raynaud's syndrome, reveal how body image concerns in women with Scleroderma must be further investigated and prioritised in treatment, alongside consideration of how overwhelmingly appearance correlates with identity and autonomy in contemporary society. Cultivating self compassion and challenging societal beauty standards remains a cornerstone to empowering women to navigate such challenges with resilience and emotional release.

VI. Conclusion

Scleroderma presents significant challenges to patients' psychological well-being and that of their families. Through examination of the psychological impact of the disease and the implementation of targeted strategies, both health professionals and caregivers can support patients in coping with

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symptoms and maintaining a sense of resilience.

Citations

Galetti, I.(2021) Living with systemic sclerosis: the point of view of patients, Current Medical Research and Opinion, 37:sup2, 1-4, DOI: 10.1080/03007995.2021.1992369

Milette.K, Thomb. BD, Maiorino.K, Nielson W.R, Körner.A, & Peláez.S (2019) Challenges and strategies for coping with scleroderma: implications for a scleroderma-specific self-management program, Disability and Rehabilitation, 41:21, 2506-2515, DOI: 10.1080/09638288.2018.1470263

Jewett LR, Hudson M, Malcarne VL, Baron M, Thombs BD; Canadian Scleroderma Research Group. Sociodemographic and disease correlates of body image distress among patients with systemic sclerosis. PLoS One. (2012) doi: 10.1371/journal.pone.0033281. Epub 2012 Mar 23. PMID: 22457749; PMCID: PMC3311623.

K. Amin , A. Clarke , B. Sivakumar , A. Puri , Z. Fox , V. Brough , C.P. Denton , E.M. Peter & M.D. Butler (2011) The psychological impact of facial changes in scleroderma, Psychology, Health & Medicine, 16:3, 304-312, DOI: 10.1080/13548506.2010.540250

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Understanding Social Support Needs of Scleroderma Patients: Insights from a Recent Study

By Anoushka Bellum from Folsom, California, USA

Abstract

In the journey of living with a rare disease like scleroderma, support and understanding from close relationships can make an immense difference. It is important to delve into the social support needs of scleroderma patients, shedding light on their experiences and the crucial role of support networks. By highlighting these ideas, scleroderma patients will feel more comfortable to share their struggles and gain more information on how to live with this condition.

Patient Perceptions

One poignant instance from an article from the National Library of Medicine encapsulates the emotional impact of diagnosis: "‘I’m so sorry, you’re so young, you have scleroderma,’ turned out to be the second most impactful statement on my life... ‘I’m giving her 6 months and that’s pushing it’...turned out to be the first." These words, spoken by a doctor, marked the beginning of a transformative journey for the patient, highlighting the shock and uncertainty that often accompany a rare disease diagnosis. The study revealed that patients often receive various forms of support from their close relationships, including emotional, informational, and instrumental support. Emotional support emerged as particularly crucial, providing patients with

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strength and resilience in the face of adversity. However, the study also underscored the importance of relational factors, such as communication style and active engagement, in shaping the support received.

Moreover, the study emphasized the need for interventions that address scleroderma as a collective challenge. By harnessing the support of close relationships and empowering both patients and their supporters with relevant information and skills, healthcare professionals can optimize rehabilitation efforts to improve patient outcomes. The journey of living with scleroderma is not without its challenges, but with the support of knowledgeable healthcare professionals and understanding from close relationships, patients can navigate this journey with resilience and hope. As the patient in the study aptly stated, "She is clothed in strength and dignity and she laughs without fear of the future," embodying the courage and determination to face the unknown.

Social Support

Another article by Taylor and Francis Online delves into the social support dynamics experienced by people living with scleroderma, looking at how patients perceive and use support from their close relationships to cope with the physical and psychological challenges of the condition. The study's focus group conversations with 19 patients illustrate the complex nature of social support, which includes emotional, informational, and instrumental aspects. Emotional support is highlighted as especially important, highlighting the value of empathy, understanding, and friendship in managing the intricacies of scleroderma. The study also highlights relational aspects such as communication style, active participation, and complementarity that influence the effectiveness of support received.Honest communication, careful selection of support sources, and strong connections defined by motivation and involvement are regarded as improving support, whereas avoidance of interaction and misunderstanding impede it.

The findings highlight the importance of targeted therapies that promote communal coping skills among scleroderma patients. Understanding patients' complex experiences within their social networks allows rehabilitation specialists to better promote successful support systems. Key techniques for rehabilitation experts include encouraging patients to seek out supporting individuals with specific knowledge and abilities, establishing open communication, and providing resources to both patients and their

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close relationships.

Conclusion

In conclusion, the studies offer valuable insights into the social support dynamics of scleroderma patients. By recognizing the multifaceted nature of support and addressing relational nuances, healthcare professionals can better assist patients in navigating the challenges posed by this rare disease.

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Julia was only 5 years old when her parents noticed something peculiar about their daughter’s body. “I said something to our pediatrician like her toes are blue,” her mother explained, “That’s not normal.” Julia, who was once a bright bubbly child, became so weak it became hard for her to enjoy her favorite hobby, gymnastics. And the cause of it all? Scleroderma.

For background, scleroderma is a very chronic autoimmune disease, affecting 20 people out of every 1 million and is even rarer in children, with only 2 cases out of 100,000. It results in thickened skin from a collagen overproduction and depending on each case, can spread to vital organs such as the lungs, kidney, and heart, limiting movement and functionality. Juvenile Systemic Scleroderma is when thickening tissue spreads in an adolescent's inner body with the possibility of fatality and is exactly what Julia had to endure.

Children with JSSC usually first show signs with Raynaud’s phenomenon. This is when fingertips and toes are particularly sensitive to the cold and turn blue, but in warmth are red and painful. Weakness is also common, since as tissue thickens in the muscles, it can hinder joint mobility. Julia’s mother recalls Julia not being able to do a simple bike ride they’ve done a number of times

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saying, “She's like I can't pedal anymore my legs are so tired.” Drastic weight loss and decreased appetite from the inability to swallow food are also common. Sudden changes in a child’s ability to perform movement such as loss in flexibility, decreased stamina, rashes on fingers, complaints of always feeling tired, and not being able to stand up without help are significant indicators of scleroderma in children.

Diagnosing a child with scleroderma starts with consulting with a rheumatologist. The processes that would follow include a physical examination to analyze the skin thickening, a conversation on family background, a skin biopsy, an electrocardiogram or echocardiogram to monitor the heart, pulmonary function tests to monitor the lungs and a gastrointestinal tract exam to track esophagus and stomach function. The purpose of these tests are to see if the thickened skin is damaging the organs so it’s vital that parents act quickly.

Juvenile Systemic Scleroderma confines little ones to their homes. Simple tasks like brushing your teeth or using a spoon becomes difficult and lack of social activities are detrimental to the child’s way of life. This is why family is essential to nurture and support them. To begin, exercise is crucial for the first step back to proper health. Simple stretches and walks help to slow down thickening of tissue and help to relearn how to move again. Second, continuous aid and supervision is key. Friends and families should help the child with tasks that might seem like second nature and that way, everyone learns how to live cooperatively. Lastly, the barrier with social interaction can be bridged with other forms of communication. Calls between friends and visits to their houses are still very much possible and a great way to interact and get support.

It’s also still very possible to still be creative and enjoy hobbies. Julia, for example, was able to express creativity and her experience through songwriting and singing. Her mom described that meeting Julia being able to meet with a songwriter to publish her own song was “a really meaningful experience”. Parents similarly should continue to encourage and support their children through their ambitions.

Living with Juvenile Systemic Scleroderma may seem difficult at first. However with a strong group of support and the right actions, anything can be possible.

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{Links}

Julia’s Story

Making a Difference for Kids with Scleroderma

Kids vs adult

Scleroderma in Children

Juvenile Systemic Scleroderma Center at UPMC Children's Hospital of Pittsburgh

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Introduction

Living with scleroderma brings about a host of challenges, from the tightening of the skin to complications affecting our internal functions. Yet, among these concerns, we often overlook the impact that scleroderma can have on our muscle health. Recent research regarding skeletal muscle abnormalities in the bodies of scleroderma patients has discovered a new area of research for systemic sclerosis that can help to further understand the severity of the disease and potentially lead to discovering more in-depth muscle involvements and newer treatments.

This article explores the complexities of muscle problems in scleroderma, analyzing their impact, significance, and solutions for patients diagnosed with the autoimmune disease to use in their daily lives.

Understanding Muscle Problems in Scleroderma

Not only does scleroderma heavily affect the skin of patients, but it can continue downwards past the skin and damage the areas underneath, such as blood vessels, joints, and muscles.

Scleroderma’s muscle involvement in patients is extremely complex and can be subtle.

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Despite being subtle, the health and mobility of the muscular system are crucial for our overall well-being. Thus, it is important to invest time and effort into check-ups of muscle function to maintain personal well-being. Common experiences can include body weakness and fatigue.

Spotting the Signals of Muscle Problems

Some indications of muscle problems in scleroderma include weakness, fatigue, joint stiffness, muscle pain, decreased mobility, increased creatine kinase levels, muscle atrophy, and Raynaud's phenomenon. When experiencing any of these symptoms, it's important to talk with a healthcare professional as early as possible because detecting muscle issues early is key to effective treatment.

Consequences of Untreated Muscle Issues

If left untreated, muscle problems in scleroderma can lead to more complications, like myositis and Sjögren's syndrome, which can seriously affect our long-term health. Thus, identifying early symptoms and arranging for regular check-ups are vital for keeping things under control and maintaining a healthy lifestyle. It's important to prioritize our health and speak more openly about body irregularities in order to achieve a healthy and active life.

Dealing with Muscle Challenges in Daily Life

Adapting to muscle issues in our daily lives takes some adjustment and support. Whether it's changing our routines or using assistive personal devices, finding ways to maintain our independence and comfort is crucial. Even if changes to your daily life are difficult, prioritizing your well-being and bodily health take a higher priority. However, if one is feeling the mental stress of a sudden change in their lifestyle, take the time to reach out to health professionals or supportive virtual groups.

Future Research and Solutions

It's worth noting the emerging research into the role of physical activity and exercise in managing muscle involvement in scleroderma. While it may seem unreasonable to participate in more constant physical activity when battling muscle problems, studies suggest that exercise routines can improve muscle strength, function, and overall quality of life for scleroderma patients.

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Working closely with healthcare professionals to develop safe and effective exercises can be a valuable part of managing muscle problems in scleroderma. By integrating more physical activity into our daily lifestyles, patients can further enhance their ability to deal with the challenges created by muscle involvement in scleroderma.

Conclusion

In summary, muscle problems in scleroderma are a significant yet often overlooked part of the condition. Understanding, detecting, and managing these issues early on can lead patients to take better care of themselves and improve their quality of life. By raising awareness, seeking early treatment, and taking more open approaches to health, scleroderma patients can make life better for themselves and may even find unexpected happiness from newly discovered activities.

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Cardiovascular System

Scleroderma may affect the cardiovascular system, leading to various complications. Increased blood pressure in the arteries supplying the lungs defines pulmonary arterial hypertension (PAH), one of the most difficult conditions. Untreated PAH can lead to fatigue, shortness of breath, chest pain, and fainting, affecting both life expectancy and quality of life. Two more cardiovascular symptoms of scleroderma are arrhythmias and pericarditis, or inflammation of the sac surrounding the heart.

Conclusion

A complicated autoimmune condition, scleroderma, can affect many bodily systems and organs. Its effects on the gastrointestinal tract, cardiovascular system, musculoskeletal system, and skin can cause a wide range of problems and symptoms that lower the prognosis and quality of life for people who are affected. Patient outcomes are enhanced by early diagnosis, multidisciplinary care, and focused therapy, which lessens the effects of scleroderma on the body.

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Social Life and Scleroderma

By Christopher Yeow from Singapore, Singapore

How many times has this happened to you: you make plans with your friends, but on the day, you are struck by an illness- whatever it is, and are forced to cancel your plans. You’re not just upset to have to cancel, but also worried that your friends will think less of you and may not invite you to things in the future. For patients with scleroderma, these feelings are all too common.

Let’s slow down a sec though: What is scleroderma you may ask? Scleroderma- or systemic sclerosis, is an autoimmune disease that causes the hardening and tightening of one’s skin. To the layperson, this is often where their understanding of Scleroderma ends. In reality however, Scleroderma- like many other chronic illnesses, causes a plethora of other physiological symptoms. Such symptoms often have no correlation to each other: ranging from generalised discomfort and fatigue to more targeted issues like gastrointestinal and even cardiovascular complications. Due to a lack of understanding and assumptions, these symptoms, which are already a physiological nightmare, often end up causing social complications for patients; who may develop a reputation as a bad friend or lazy colleague without understanding or empathy for the hardships they could be facing, or why they are acting the way they are. This article seeks to highlight some examples of misconceptions that patients with scleroderma have had to tackle in order to highlight the importance of education and understanding

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who would otherwise be left to ruminate with their thoughts. For patients, however, a point brought up by Luisa Ruiz Kulig that I think is important to bear in mind is that unlike something obvious like a broken bone for example, most effects and symptoms of scleroderma are invisible to everybody that isn’t the person suffering from it. These symptoms often cause scleroderma patients to miss work or be unable to keep up with work. Luisa recounts this in her testimony, highlighting that people would not believe her when she spoke about her symptoms as she was not bleeding, or displaying any symptoms that would be visible to an onlooker. This pushed her to do things like not drink water throughout the day so she wouldn’t have to use the toilet as her colleagues, who did not believe she was being affected by her symptoms, would not accommodate her laboured movement to or from the toilet. She was also met by much doubt when she told her colleagues that she was unable to come into the office because her symptoms were too bad. She eventually had to give up work altogether, as her symptoms became too much for her to handle. Conversely, Elaine Wiley details how her employer has provided her many resources which she can use freely to make her working life easier. She is also allowed to work from home, which allows her the flexibility to plan her medical checkups.

Whether it involves unprofessionalism, flakiness, or any other negative association you may have, the assumptions we make about people with scleroderma can have incredibly negative effects on their mental health. While it is easier to be ignorant and turn a blind eye, let me encourage you to choose kindness over impatience, and to educate yourself before making assumptions. Nobody wants to be a burden, and assuming the worst in those around us, especially those who are struggling will only serve to hurt them.

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Unveiling the Xist Ribonucleoprotein Complex: Unraveling Sex-Biased Autoimmunity

By Davia Williams from Wylie, Texas, USA

Abstract

Autoimmune disorders, encompassing conditions where the immune system mistakenly attacks the body's own tissues, pose a significant burden on individuals' health and healthcare systems globally. Despite considerable advancements in understanding these disorders, there remains a notable gap in comprehending why certain autoimmune diseases exhibit a marked sex bias in both prevalence and severity. This discrepancy has spurred researchers to delve deeper into the intricate mechanisms underlying autoimmune pathogenesis, with a particular focus on the role of the Xist ribonucleoprotein complex.

Understanding the Xist Ribonucleoprotein Complex

The Xist ribonucleoprotein complex, traditionally recognized for its involvement in X chromosome inactivation, has emerged as a key player in autoimmune disorders. This complex, critical for achieving gene expression balance between males and females by silencing one of the two X chromosomes in the latter, has garnered attention for its potential implications beyond chromosomal regulation. Recent studies have unveiled its association with autoimmune pathogenesis, suggesting a broader role in immune system dysregulation.

Study Findings: Unveiling the Role in Sex-Biased Autoimmunity

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Dou, D. R., Zhao, Y., Belk, J. A., Zhao, Y., Casey, K. M., Chen, D. C., Li, R., Yu, B., Srinivasan, S., Abe, B. T., Kraft, K., Hellström, C., Sjöberg, R., Chang, S., Feng, A., Goldman, D. W., Shah, A. A., Petri, M., Chung, L. S., Fiorentino, D. F., … Chang, H. Y. (2024). Xist ribonucleoproteins promote female sex-biased autoimmunity. Cell, 187(3), 733–749.e16. https://doi.org/10.1016/j.cell.2023.12.037

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A Parent’s Guide To Tackling Pediatric Scleroderma In Their Child

By Diya Joshi from Weston, Florida, USA

What is Scleroderma?

Scleroderma is a rare autoimmune disease, meaning that an individual’s cells mistake themselves as foreign invading cells, which then causes the cells to attack themselves. This causes inflammation and destruction in different areas on the skin and adversely affects internal organs and blood circulation.

Since this is a chronic disease, the pain only worsens over time. Unfortunately, because there isn’t a cure for it, all of the treatments/medications that are taken to combat it, are targeted to weakening and slowing down the development of Scleroderma within an individual.

Types of Scleroderma

There are two main types of Scleroderma and they have their own subcategories of areas they impact. Localized Scleroderma is far more common in children than Systemic. Localized targets only the skin in different ways, while on the other hand systemic affects the whole entire body. Here is a more detailed description of the two:

1. Localized Scleroderma
   

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• Damages the skin and tissues beneath the skin like muscle and bone
  
• Can be morphea which affects the skin in patches, linear which goes straight down an arm or leg, or en coup de sabre which is a straight line on one’s forehead
  
• Systemic Scleroderma
  
• Affects blood circulation and internal organs
  
• Can be diffuse cutaneous which involves the skin thickening all throughout different areas of the skin and involves issues within different organs’’ functionality, it can be limited which only affects the organs’ functions but does not adversely impact the skin. Or it can be a mix of both which is limited cutaneous systemic, also known as CREST Syndrome which involves calcinosis, Raynaud’s phenomenon, esophageal motility dysfunction, sclerodactyly, and telangiectasia according to Cleveland Clinic.
  

Scleroderma in Children

As stated by the National Institutes of Health, Scleroderma is most commonly seen in individuals who are anywhere from 30 to 50 years of age. In fact around 2% of people with it happen to be younger than 10 years of age and 7% are 10-19 years of age.

Since there are only about 5,000 to 7,000 children in the United States with this medical condition as said by experts at Cleveland Clinic, many parents that have a child with this medical condition are unsure what the next step may be and how to deal with this as a parent.

It is understandingly hard to accept that your child has such a harmful disease, especially when it is this rare. According to Stanford Medicine, gene and environmental factors may play a role in why your child has Scleroderma but the cause is primarily unknown.

Although this condition, like all, comes with many extremities and implications, it is not nearly as limiting as many other conditions and there are many ways to comfort your child if they are struggling with it through various medications and treatments. Children are strongly encouraged to play sports with Scleroderma, to improve their mobility and flexibility as their skin is prone to tighten over time.

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Common Symptoms

Scleroderma can affect individuals in varied intensities with varied symptoms so it can be hard to detect if your child has it, especially since it is such a rare disease. These are a few common symptoms seen in patients with this medical condition are the following:

• Pain/tightness in the joints
  
• Sores on the fingers, swelling, thickness, and pale color on fingers
  
• Dark/shiny areas on skin
  
• Heartburn and difficulty swallowing
  
• Limited lung function
  
• Calcinosis
  
• Muscle weakness, immobility
  
• Arthritis
  
• Swollen blood vessels
  
• Behavioral/learning complications
  
• Rayaud’s Phenomenon
  

If your child has been exhibiting some of these symptoms, it is strongly advised that they are taken to see a doctor and there they may be referred to see a rheumatologist or dermatologist depending on the severity of the symptoms. There they will be properly diagnosed, supervised, and given special treatment for the kind of medical condition they have.

Antibody, blood & urine, skin biopsy, echocardiogram, and pulmonary function tests may be taken in order to determine specifically what kind of Scleroderma your child has and what area it is specifically targeting in your child’s body, as stated by Columbia University Irving Medical Center.

Treatments & Medications

Unfortunately, there is no cure for Scleroderma yet, but there are some treatments and medications that can be used to tackle it. A few of them include the following:

• Calming moisturizers
  
• Being a part of a support group
  
• Physical therapy
  
• Immunosuppressive drugs
  
• Mycophenolate
  
• Phototherapy
  

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• Methotrexate
• Cyclosporine
  
• Corticosteroids
  

Medications aid patients differently based on the type of symptoms present, severity of the symptoms, and type of Scleroderma they have. It is advised to start off with one to two of these treatments/medications and progress as needed.

Works Cited

• U.S. Department of Health and Human Services. (2023, December 15). Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases. https://www.niams.nih.gov/health-topics/scleroderma#:~:text=Age.,affect%20African%20Americans%20more%20severely.
  

• Medical Professional, C. C. (n.d.). Pediatric systemic scleroderma: Symptoms, causes & outlook. https://my.clevelandclinic.org/health/diseases/4910-pediatric-systemic-scleroderma
  

• Scleroderma in children. Stanford Medicine Children’s Health. (n.d.). https://www.stanfordchildrens.org/en/topic/default?id=scleroderma-in-children-90-P01729
  

• Scleroderma in children. ColumbiaDoctors Children’s Health. (2021, July 1). https://www.columbiadoctors.org/childrens-health/pediatric-specialties/rheumatology/treatments-conditions/scleroderma-children#:~:text=In%20children%2C%20scleroderma%20usually%20begins,eventually%20turning%20brown%20or%20white.

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• social media about scleroderma you are helping bridge the divide between those that may not know about scleroderma at all and those that do. The more people that know the more they can do.
  
• Take a STEM class- by taking a STEM class you can learn more about the details behind human biological processes, and in that way learn more about the scleroderma experience. Talking with like-minded people that are also interested about those topics can spark new ideas as well.
  
• Watch videos, read articles, do research yourself- by doing research yourself you can help contribute to this field through first hand ideas. Addressing different aspects of the scleroderma experience will help the greater body of researchers find a cure and possibly interest those around you as well.
  

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Healing is Pain: The Battle of the Scleroderma Warriors

By Elyse Da Costa from Toronto, Ontario, Canada

Scleroderma is a rare autoimmune disease characterized by the thickening and hardening of the skin. Despite the ambiguity surrounding the disorder, there exists numerous non-for-profit organizations that have been developed to spread awareness about the disease. Grassroot organizations such as Project Scleroderma, the National Scleroderma Foundation, and the Scleroderma Research Foundation have all pioneered their own set of advocacy strategies to illuminate the stories of those fighting against ongoing symptoms. Some Scleroderma symptoms could include tightening of the skin in such a way that grasping becomes difficult. Other side-effects could include progressive damage to internal organs such as the heart, lungs, kidneys and respiratory tract. Despite the detrimental byproducts of Scleroderma, there is unfortunately no cure. Although some might see this diagnosis as the “start of the end,” many Scleroderma patients are proving the exact opposite. On the contrary, these survivors are channeling their pain by facing it head-on!

The Artists’ Guidebook to Life

Scleroderma is a disease associated with a spectrum of different side-effects, from shortness of breath to crippling joint movements. Due to such

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her own art gallery!

Through these mediums, many artists have found a way to cope with debilitating symptoms while taking care of their own mental wellbeing. Facing illness with a courageous and headstrong attitude allows Scleroderma patients all over the world to use their talents to the best of their ability. It speaks volumes on the artist's mentality of perseverance: “I can, so I will.”

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What is CREST syndrome?

By Franny Khine from Brooklyn, New York, USA

Every year, 300,000 people, aging from ages 30 to 50 years old are diagnosed with Scleroderma, a disease formed by the rapid growth of fibrous tissue. One form of scleroderma is CREST syndrome. CREST is an acronym for calcinosis, raynaud’s phenomenon, esophageal dysmotility, and sclerodactyly, which are all clinical features seen in a patient with this disease.

Calcinosis is the act of calcium depositing in the skin and subcutaneous tissue. It may be caused by dystrophic calcification and is associated with calcium and phosphorus levels.

Raynaud’s phenomenon is the spasm of blood vessels in response to cold or stress. It decreases the blood flow in the fingers and in other parts of our body.

Esophageal dysmotility causes acid reflux and a decrease in the motility of our esophagus. It makes the process of passing food and water more difficult, which causes problems when swallowing.

Sclerodactyly is the creation of tension through the thickening and tightening of the skin on the fingers and hands.

Telangiectasias is the dilation of capillaries, which causes red marks on the surface of skin. Although they can be associated with diseases, they are

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Embracing Beauty: Navigating Makeup and Skincare for Facial Changes

By Heqing Feng from Nashville, Tennessee, USA

Scleroderma, a complex autoimmune condition, can profoundly affect an individual’s skin. Excessive collagen production leads to progressive thickening and hardening of the skin, further resulting in tightness and stiffness in facial features. Some scleroderma individuals may also experience microstomia, which is tightness around the mouth, and blepharophimosis, the narrowing of the eyelids. It can be uneasy to witness one’s facial skin alter as the symptoms progress, constantly reminding themselves and others of the presence of their disease. So, how about putting on some makeup?

While makeup is not necessary if you are comfortable with your skin, it can be a helpful tool if you find yourself struggling with the changes in your appearance – feeling embarrassed to be seen in public, weary of others' stares or questions about your skin – while also grappling with concerns about whether makeup might worsen your skin condition or whether you are “psychologically weak” by trying to hide your skin conditions. This article will guide on applying makeup for individuals with scleroderma.

The Power of Makeup and Skincare

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First and foremost, there is no need to feel ashamed about wanting to wear makeup. Everyone experiences moments of self-consciousness about their appearance, especially when they feel different from others. Wanting to enhance one's appearance and cover imperfections is entirely natural. However, it can indeed be challenging to find makeup products tailored specifically for individuals with scleroderma and to learn techniques for applying them effectively to achieve a polished look. Here are some tips to improve your skin appearance:

• Before applying makeup, use gentle, hydrating skincare products to prep your skin. Oil-based cream will help retain moisture and act as a protective layer on your skin. This can reduce dryness and improve adherence to the foundation.
  
• Select makeup products with safe ingredients suitable for sensitive skin. Hypoallergenic and fragrance-free formulations will minimize the risk of adverse reactions on scleroderma-affected skin. Avoid using products with parabens, which will disrupt hormone function and potentially aggravate skin conditions, and alcohol solvents, which might strip the skin of its natural oils and lead to dryness.
  
• Using concealing foundation and camouflage creams can effectively cover redness and marks, evening out the skin tone. Stippling brushes or sponge applicators can be used to accommodate changes in skin texture, helping promote even coverage of the skin without applying excessive pressure. Alex, a makeup artist with scleroderma, has been creating tutorials on product selection and application techniques. Her videos can be viewed here.
  

There are also plenty of online resources to support individuals with scleroderma to improve their skin conditions.

SRUK (Scleroderma & Raynaud's UK): Skin Camouflage offers guidance on using camouflage techniques to conceal skin changes due to scleroderma. It provides tips on selecting appropriate products and techniques for effective coverage, and also advice on maintaining skin health and managing self-esteem.

Changing Faces supports individuals with visible differences, such as those from medical conditions like scleroderma, by providing counseling services, peer support, and practical advice.

American Academy of Dermatology (AAD): Scleroderma Self-Care also

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provides comprehensive information on mitigating skin symptoms of scleroderma through self-care, including advice on skin care routines, protective measures, and suitable skincare products to reduce the discomfort from scleroderma.

Thriving with Scleroderma

Fighting scleroderma is a long and arduous battle, so it is crucial to maintain a healthy mindset. Beyond skincare and makeup, finding supportive communities and online forums can provide comfort and encouragement. You are not alone in the battle, and connecting with others through platforms like Scleroderma United will help foster a sense of belonging and acceptance. Also, engaging in hobbies like outdoor adventures, arts and crafts, or culinary not only adds excitement to life but also shifts your focus from what you are losing due to the disease to what you are accomplishing. By embracing a comprehensive approach to wellness, you will be empowered to navigate the complexities of living with scleroderma with resilience and positivity.

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Diagnosed with Scleroderma: What’s Next? Ways of Taking Care of Your Mental Health

By Imaan Mehmood from Ajax, Ontario, Canada

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changes. Consistent physical pains, financial difficulties, and major disruptions to social life and work are changes that may come with scleroderma that are correlated with declines in mental health. In addition, this diagnosis comes with a lot of uncertainty. Patients often describe not understanding what exactly their diagnosis will mean for them, and how it will manifest in their body. This uncertainty stems from the "the unknown cause of scleroderma, the unpredictability of this disease, and the variability of this disease course from patient to patient”, which is an added stressor that many patients face. Amongst all this uncertainty, there is one thing you can be sure about: we all have the potential to take charge in our medical journey and make the best of what we have.

Keeping your Mind Healthy

Taking care of your mind alongside your body is crucial for combatting this disease. Firstly, your relationship with your doctor matters. Finding a doctor that will share in your concerns and figure out innovative ways of managing the disease is incredibly important. Also, building a strong support system is crucial for being able to express what you are going through. There are a number of local and international organizations that are built around a strong and supportive community. In fact, many scleroderma patients feel a great amount of empowerment and confidence through advocacy work and involvement within their community. This often becomes a means of forming long-lasting relationships as well. Importantly, mindset is extremely important. Many people with scleroderma around the world are combatting this disease with sheer strength of mind, alongside keeping their physical health in check. Yogita Sharma, an associate consultant living in Delhi, India, was diagnosed with systemic sclerosis in her late teen years. Yogita emphasizes how having support and developing a strong mindset were crucial in battling her declining self-esteem following the onset of the disease. She stresses that people with scleroderma should still “go anywhere and do anything” that they want to the best of their abilities. Yogita actively works in an NGO to support others with scleroderma at a global level. There are many Scleroderma Stories of inspiring individuals that have implemented unique ways of not only coping with the disease, but thriving despite it. As always, if you are experiencing negative thoughts, difficulty coping with the disease, and other symptoms related to your mental health, this is completely normal. Make sure to reach out to a professional that can understand your struggles and help you navigate these tough times.

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Having scleroderma does not mean you will have to stop doing what you love. It is important to find encouragement and enable ways for yourself to continue pursuing your passions and interests despite the difficulties that come with the disease. With the right care, support, and choices, there is no doubt that your life can be fulfilling and active.

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The Further Impact of Chronic Physical Illnesses

By Isha Parikh from Plano, Texas, USA

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Disorder, any type of anxiety can impact a person’s mental health and attitude. According to Forbes Health, another considerable mental health disorder that impacts about 280 million people worldwide is depression. Depression is more than just feeling sad or overwhelmed; it’s a condition that can affect your physical and mental well-being. Depression can cause a constant feeling of sadness and a loss of interest, which can interfere with a person’s daily life. Anxiety disorders and depression are only a few examples of mental health disorders, but they can change someone’s attitude and life because of the lack of compassion from other people or significant changes in their life.

How do these mental health disorders originate in people with chronic physical illnesses though? Living with these illnesses usually results in a loss of independence or the feeling of being misunderstood. In addition to this, receiving news as big as having a disease that will impact the rest of one’s life can affect a person if they don’t have people around them to help. Without understanding or compassion, a feeling of loneliness is created, further intensifying the negative impact on their mental health. Not only do mental health disorders originate after being diagnosed with a chronic physical illness, but they can also lead to some. For example, depression increases the likelihood of developing heart conditions or diabetes. With a mental health disorder, a person with a chronic illness may find it harder to go outside, eat a proper amount of nutrients, exercise, or do other things that can better their well-being.

Now that we’ve talked about the mental health disorders that are associated with chronic physical illnesses and their impacts, how can we combat these disorders? The American Psychological Association says that being surrounded by understanding and friendly people can lift your mood by 15%. For people with chronic physical illnesses, there are a lot of support groups that can help. A lot of other things that can help boost your mood are going to therapy, breathing exercises, talking to someone you trust, keeping track of your day in a diary, and pursuing a hobby. Overall, chronic physical illnesses can crucially impact a person’s physical health, but with an adequate amount of help, a person’s day or even life can be changed.

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Are COVID-19 Vaccines Effective for People with Scleroderma?

By Jadey Ding from Melbourne, VIC, Australia

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Moderna and AstraZeneca require at least two doses to ensure the individual has long-term memory and protection of antibodies against COVID-19 and allow the continuous development of memory cells. As autoimmune diseases already decrease one's immune system due to less leukocytes, COVID-19 vaccines may be doing more harm than good for individuals with SSc.

People with scleroderma are given immunosuppressants as treatment for their skin thickening caused by the overproduction of collagen. An immunosuppressant is a class of medicine that can either “inhibit or decrease an immune response’s intensity”5 and reaction rate.

Specific immunosuppressants, such as Mizoribine, can inhibit the proliferation of lymphocytes6. Lymphocytes play a key role in antibody production, meaning it directly influences one’s response to vaccines. Hence, a person who is immunocompromised is unlikely to adapt to the abnormal immune response caused by the vaccines, causing them to not only be unprotected due to the reduced immune response, but feel more uncomfortable. It is important to know that “death may also occur”7 due to a weakened immune system, as the body cannot withstand any more damage due to the little immune cells available to tackle additional unknown pathogens.

People with SSc may also enter a renal crisis following the vaccination. One patient after their first dose of Pfizer (a common COVID-19 vaccine) developed persistent headaches, nausea, and sudden acute vision loss with extreme high blood pressures8. After- effects of COVID-19 vaccines can also include rapid progression of skin thickening9, which was seen from a 70 year old man after receiving the first dose of AstraZeneca. As there are new COVID-19 vaccines constantly entering the market, there are many unexpected and unfound detrimental aftereffects, such as relapses10 or stable patients after vaccinations. Other adverse reactions include physical pain, such as sore arm, muscle ache and joint pain, as well as chills and fevers11, which builds on top of the pain and discomfort of SSc patients.

It has been suggested that SSc Covid-19-infected patients can consider TCZ infusion if they are undergoing other treatments and experiencing mild symptoms12. TCZ is a recombinant humanised anti-interleukin-6 receptor monoclonal antibody, which has a main use in the treatment of rheumatoid arthritis. In infected hospitalised SSc patients, preventive anticoagulation (substances that prevent blood clots)12 is advised. Other methods of lowering the chance of infection for SSc patients include social distancing, washing hands often, and wearing masks in crowded spaces.

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Substantial amounts of data have proven COVID-19 vaccines to be effective, however, health officials and professionals must consider the individual’s health first. Therefore, for SSc patients, it is not the vaccine itself being ineffective, but the fact that they are immunocompromised, meaning they cannot produce the maximum number of antibodies from the vaccine. As a result, their immune reaction to the vaccine is smaller compared to a healthy individual, hence why they would have less protection against COVID-19, making the vaccine less effective.

Sources

• https://ourworldindata.org/coronavirus
  
• https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8875950/
  
• https://doi.org/10.1016/j.jtauto.2023.100212
  
• https://www.who.int/news-room/feature-stories/detail/how-do-vaccines-work
  
• https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8987166/
  
• https://doi.org/10.1016/j.jaci.2007.06.016
  
• https://www.cdc.gov/coronavirus/2019-ncov/vaccines/recommendations/immuno.html
  
• https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8321963/
  
• https://doi.org/10.1016/j.jaut.2022.102812
  
• https://link.springer.com/article/10.1007/s13760-021-01775-2
  
• https://doi.org/10.1016/S2665-9913(21)00416-1
  
• https://doi.org/10.1136/annrheumdis-2020-217407
  

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Empowering Oneself in the Face of Scleroderma

By Jaguar Ngo from Brisbane, Queensland, Australia

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Scleroderma has on their life, they thrive in achieving their goals and doing amazing things.

Thank You Warriors

In closing, my time as a Scleroderma United intern has taught me so much. It taught me the emotional, physical, and financial toll that chronic diseases like Scleroderma can have on individuals. It taught me the transformative power of community in overcoming adversity. But most of all, it taught me that regardless of the circumstances one faces, the empowerment to shape one’s destiny and future is within reach to all.

To all the Scleroderma warriors out there, your enduring resilience and courage continues to inspire us all.

Sources

https://www.sclerounited.us/

https://www.niams.nih.gov/health-topics/scleroderma

https://sclerodermawarriors.org/

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How the Scleroderma patient experience is reflected in the stages of Grief and the Biopsychosocial Model; protective and risk factors as patients proceed through the stages of grief.

By Jia Hui Lin from Melbourne, Victoria, Australia

What is Scleroderma?

Scleroderma is a rare autoimmune disease that causes the skin and internal organs to thicken. In Australia, 6000 individuals have scleroderma, compared to 2.8 million people with eczema.

The five stages of grief

Kubler-Ross’s model proposed that individuals proceed through 5 stages (denial, anger, bargaining, depression and acceptance) when dealing with grief. While it has its merits, this model could be applied to patients with Scleroderma.

How patient experience reflects the five stages of grief

Scleroderma stories share insights about the sentiments of Scleroderma patients from pre-diagnosis to the onset of disease, and finally, life adjustments and management of the condition to live a fulfilling life despite the disease.

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This risk factor could be addressed by initiatives developed to support patients with chronic illnesses, such as the Health Care Card in Australia and the Social Security Disability Insurance in the United States. Healthcare professionals should also consider the patient's financial situation when developing treatment plans to ensure it is long-term financially feasible for the patient.

Importance of holistic approach to a rare chronic disease

As a chronic illness, Scleroderma has a holistic impact on patients. Aside from biological symptoms, scleroderma is also accompanied by a mental health toll and social interruptions for patients. Therefore, when developing treatment options and long-term care plans for patients with scleroderma, medical professionals and allied health practitioners should adopt a holistic approach to ensure these different factors are considered, as they all are important considerations for patients' quality of life.

The theory underpinning this is the biopsychosocial model, which suggests that illness is an interaction between biological, psychological and social factors. This could be addressed in a clinical setting by incorporating patients into developing the treatment plan to ensure patients' needs are accurately and adequately addressed. Another way to apply it in a clinical setting would be using a multidisciplinary treatment team to provide comprehensive patient support.

It is also important to note that each patient’s financial situation, values, support level needed, and disease severity are different. Therefore, doctors should tailor treatment methods to each patient. A fixed treatment plan for all patients would fail to address that.

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Pediatric Scleroderma: Scleroderma in Children

By Jisara Wuttikreekiat from West Vancouver, BC, Canada

Scleroderma is an autoimmune disease that causes inflammation and fibrosis (thickening) in the skin and other areas of the body. While the condition itself is rare in adults, it is possible for children to develop the disease, which leaves a significant impact on families when their child is diagnosed with an unfamiliar disease. Therefore, understanding the distinct clinical features, diagnostic approaches, and therapeutic considerations specific to pediatric scleroderma becomes a discussion that could not be ignored.

Overview

Pediatric scleroderma includes two major groups of clinical entities, systemic sclerosis and localized scleroderma. Localized scleroderma often affects only the skin and not major organs. On the other hand, systemic scleroderma affects the skin and may affect the tissues under it, including blood vessels and major organs. This implies that those diagnosed with systemic scleroderma are more likely to be fatal. According to the National Library of Medicine, although both are uncommon in children, the estimated annual incidence of localized scleroderma is 1–3 per 100,000 children while systemic scleroderma is 1 per million children.

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Clinical Features

The most common clinical feature presented on children diagnosed with scleroderma is thickened or hardened skin, often in a symmetric distribution. Another common feature is changes of color in fingers or toes in responding to stress or cold. Although more difficult to spot on children, symptoms such as joint pain, stiffness, and swelling can be developed as a result of skin tightening. However, for systemic scleroderma, the child might experience affects in their internal organs such as gastrointestinal, respiratory or cardiac symptoms. Those symptoms include: abdominal pain, bloating, diarrhea, constipation, shortness of breath, cough, and chest pain.

Diagnostic Approaches

The first step is to recognize the symptoms and head to a clinic for a professional to do physical examinations for the conditions present. After that, depending on the results, laboratory tests might be necessary to provide experts with the information needed to confirm their diagnosis. Blood tests are used to elevate inflammatory markers, autoantibodies such as anti-nuclear antibodies, anti-Scl-70 antibodies, and anti-centromere antibodies. Moreover, imaging scans may be used to evaluate internal organ involvement such as lung fibrosis in the case of systemic scleroderma. It is extremely important to let an expert or a professional to provide accurate diagnosis and information regarding the treatments moving forward.

Therapeutic Considerations

Unfortunately, there is currently no cure for this disease and as a result, patients are encouraged to undergo therapy. Medications are used to alleviate symptoms, control inflammation, and prevent organ damage while physical therapy helps with joint contractures, mobility, and in general, improving life’s qualities. Overall, with current technologies, there are not a lot of treatment options for scleroderma, let alone pediatric scleroderma.

In the end, given the rarity of scleroderma and the panic it often causes in adults who aren't familiar with it, imagine the added worry for parents when their child is diagnosed. Therefore, the purpose of this article was to ease this stress, by explaining the signs of scleroderma in children, what it can do, and how it can be treated.

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Introduction: What is Linear Scleroderma?

Linear scleroderma is a kind of scleroderma that presents a distinct problem in pediatric dermatology and rheumatology: unlike the more well-known diffuse type of scleroderma, which usually affects adults with broad skin involvement and/or internal organ issues, linear scleroderma mostly affects children and adolescents. This form of the condition is characterized by localized skin thickening along linear tracks. These all mean that this condition's pathogenesis, clinical manifestations, and management strategies are significantly different from those of its counterparts, or other subtypes. Thus, linear scleroderma requires a focused understanding and approach. The importance of early detection/specialized care in managing linear scleroderma (particularly in children) means that not only clinicians, but also the general public, should recognize and address it in order to mitigate its potential impact on growth, development, and overall quality of life.

Diagnosing Linear Scleroderma:

Linear scleroderma in younger patients has a specific clinical profile, including thicker skin bands, joint stiffness, and the possibility of growth disruption. In contrast to the diffuse or systemic types of scleroderma that are

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more common in adults, linear scleroderma is characterized by localized skin involvement and limited systemic symptoms. The linear distribution of skin thickening along with joint stiffness distinguishes this subtype, often affecting specific areas on the body such as the limbs, face, or trunk. Moreover, the potential for growth disturbances in affected areas further underscores the unique nature of linear scleroderma in children. Despite these distinguishing characteristics, however, the diagnosis of linear scleroderma in pediatric patients is still extremely difficult (because of the disease's rarity, as well as its symptoms overlapping with other rheumatic illnesses). For example, the Children’s Hospital of Philadelphia states that linear scleroderma is often mixed up with Parry-Romberg syndrome because both conditions are characterized by the same progressive loss of subcutaneous fat, and both occur in childhood. Therefore, these diagnostic difficulties highlight the need for a comprehensive evaluation by an experienced team (for example, a medical team comprising of pediatric specialists, rheumatologists, and dermatologists) in order

to accurately differentiate linear sclerosis from other conditions and enable prompt management!

Treatment for Linear Scleroderma:

When treating linear scleroderma, a team-based approach guarantees thorough evaluation, precise diagnosis, and customized treatment regimens catered to the specific requirements of the child. Regular evaluations of skin involvement, joint mobility, and growth factors, along with close observation of the disease's course, allow for prompt modifications to treatment plans aimed at maximizing benefits and reducing drawbacks. There are also many therapeutic options available for treating children with linear scleroderma including topical treatments, systemic medicines, and physical interventions.

• Topical therapies, like calcineurin inhibitors and corticosteroids, (as discovered in Cunningham’s 1998 clinical trial) are frequently used to treat localized skin irritation and lessen skin thickness.
  
• When treating children who have refractory symptoms, systemic medicines like biologic agents, as explained by the National Scleroderma Foundation may also be appropriate (but their safety and efficacy in juvenile patients should be carefully considered).
  
• Finally, physical therapies such as splinting and physiotherapy are crucial for preserving joint mobility, which lowers the risk of long-term functional impairment.
  

In the end, though, it's critical to weigh the benefits and drawbacks of each

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course of treatment in relation to the child's overall health and the severity of the illness.

Psychosocial Aspects:

A comprehensive approach to care that takes into account both the medical and psychological/social elements of the condition is also very important, since linear scleroderma can have a significantly negative influence on both a child's physical and mental health. The need of giving children and their families continuing assistance and counseling is underscored by the physical changes and possible functional limitations that may have an impact on social interactions, body image, and self-esteem. As Rheumatology Advisor mentions, psychosocial therapies, such peer support groups and cognitive-behavioral therapy, can help kids be resilient and manage the emotional difficulties brought on by their conditions. Furthermore, advocating for social inclusion and educational adjustments as well as creating a supportive atmosphere at home and at school are crucial elements of holistic care for kids with linear scleroderma.

Conclusion:

Children with linear scleroderma have a wide range of long-term prognoses, which are influenced by factors such as therapy response, extracutaneous symptoms, and the degree of skin involvement. With early and active treatments, some children may have illness remission or stabilization; however, other children may have complications, including joint contractures, disparities in limb length, or functional impairment, which require continuous monitoring and care. Frequent follow-up appointments and care are necessary to identify any problems early, correct them, and modify treatment plans as necessary. Ultimately, even though children with linear scleroderma will likely face many obstacles, their long-term prognosis and outcomes can definitely be improved with early detection, prompt management, and comprehensive treatment!

Sources:

https://www.hopkinsmedicine.org/health/conditions-and-diseases/scleroderma/types-of-scleroderma#:~:text=Diffuse%20scleroderma%20is%20a%20subtype,%2C%20kidneys%2C%20lungs%20and%20heart.

https://www.sruk.co.uk/scleroderma/understanding-scleroderma/scleroderma-systemic-sclerosis-diffuse/

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https://www.chop.edu/conditions-diseases/parry-romberg-syndrome

https://www.chop.edu/conditions-diseases/linear-scleroderma#:~:text=Linear%20scleroderma%20is%20a%20progressive,one%20side%20of%20the%20body.

https://pubmed.ncbi.nlm.nih.gov/9704831/

https://www.rheumatologyadvisor.com/ddi/scleroderma/#Scleroderma_Manageme

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Exercise and Scleroderma Patients: Biological Functioning Improvement

By Kaitlyn Flanagan from Toronto, Ontario, Canada

The diagnosis of the autoimmune disease Scleroderma can bring on many daunting perceptions, but exercise is the answer. The progression of Scleroderma can leave individuals searching for treatment options to better their bodies and regain some of the physical strength they had before diagnosis.

Many symptoms are brought on through the diagnosis of Scleroderma, posing a need for physical treatment options to make patients feel better about themselves. Individuals in the study for Identifying barriers and facilitators observe that rehabilitation is a challenge due to the involvement of the skin, musculoskeletal system and internal organs for patients.

The pathology of this autoimmune disease generates an unpredictable nature in which patients can improve. In 50-60% of Scleroderma patients, it is indicated that there is a consistent usage of a physical therapist for a period of time. This deciphers that although the disease is rare, a non-pharmaceutical intervention like exercise can be conducted at home as a safe way to improve circulation and therefore promote better biological functioning.

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The management of pain and need for improved mobility makes a great difference in biological functioning when physical activity is engaged in. The range of exercise is endless, but the starting point for all should be low impact activities. Scleroderma patients not only feel better moving their bodies physically, but their mental clarity will also improve.

So what low impact exercises can I start doing today?

As a newly diagnosed patient it is very important to get into a physical routine to avoid the arisal of any new symptoms, particularly build up of scar tissue or fibrosis.

However the following physiotherapy workouts were designed through Scleroderma & Raynaud's UK, an incredible source for patients to combat their diagnosis right away:

Face: lifting eyebrows, winking eyes individually, frowning your forehead and grinning without showing teeth

Back: sit on a chair and twist, lie on your back and twist knees to both sides and stretching your arms behind you

Neck: tilt your head to each shoulder and rotate your head to each side

Wrists: extending or flexing wrist by placing palms together and lowering or raising them down the chest

Shoulders: standing up hold a stick and face upwards until you feel a stretch

Furthermore, higher impact exercises are also available for those that feel they need greater strides in mobility to fit their specific needs. One individual demonstrated the way in which your body can improve over the course of engaging in multiple marathon runs through his Run Greg Run campaign. The evident love for running can be translated to all Scleroderma patients looking to better their bodies.

What exercises are next if those are too easy for me?

The consultation of a physician before pursuing strenuous exercise is highly encouraged, as it is always best to know your limits and stay within them. Experimenting with the following aerobic exercises can improve symptoms significantly:

• Exploring your neighborhood by walking or running
  
• Trying out a new yoga class
  
• Cycling at your local gym
  
• Diving into a swimming class
  
• Hitting the slopes through skiing or snowboarding
  

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Although any activities described can have positive effects on patients, the best way to allow biological function improvement comes down to a daily routine to stick by. Setting a routine can be hard when fluctuations in physical limitations occur due to the flare ups of symptoms linked to the disease. These challenges make it especially important to move your body in any way you can without being too hard on yourself.

The important takeaway is that the progressive symptoms are proven to become suppressed when diagnosed Scleroderma patients are actively engaged in physical activity. Learning to incorporate exercise into a routine will have exponential effects on those impacted by Scleroderma of all forms.

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Close Relationships Promote Internal Healing In Scleroderma Patients

By Kaitlyn Flanagan from Toronto, Ontario, Canada

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Navigating the Complexities of Chronic Illness through the Lens of a University Student: Understanding the Connection between Physical Health and Mental Well-being

By Karson Yuen from Toronto, Ontario, Canada

Living with a chronic illness presents a unique set of challenges that extend beyond the realm of physical health, often intertwining with one's mental well-being. For university students grappling with chronic conditions, the journey is multifaceted, encompassing not only the management of symptoms but also the emotional upheaval that accompanies the chronicity of their health condition. In this article, we'll explore the relationship between chronic illness, anxiety, and depression, shedding light on the complexities faced by students navigating these intersecting realms. Moreover, we'll explore practical strategies and resources aimed at fostering resilience and promoting holistic well-being within the university setting.

The experience of living with a chronic illness as a university student is akin to traversing a labyrinth, fraught with twists and turns that demand adaptation and resilience. From managing coursework and extracurricular activities to attending medical appointments and coping with fluctuating symptoms, the balancing act can be overwhelming. However, what often remains unseen is the toll that chronic illness takes on mental health, manifesting in the form of

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offers practical tools for challenging negative thought patterns and developing adaptive coping mechanisms. Additionally, psychotropic medications, prescribed under the guidance of a healthcare provider, can help alleviate symptoms of anxiety and depression, enhancing students' ability to engage fully in academic and social pursuits.

In conclusion, the journey of university students grappling with chronic illness is marked by intricacies that extend beyond the confines of physical health. By recognizing the interplay between chronic illness, anxiety, and depression and implementing comprehensive support strategies within the university setting, we can empower students to thrive academically and emotionally. Through collaborative efforts and a commitment to holistic well-being, we can cultivate a university environment that nurtures resilience and fosters the growth and development of all students, irrespective of their health status.

Additional resources if you need support:

https://www.beyondblue.org.au/docs/default-source/resources/bl0124-chronic-physical-illness-and-depression-fact-sheet-lr_12-18.pdf?sfvrsn=e21646eb_2

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Supporting Scleroderma through Stories

By Karson Yuen from Toronto, Ontario, Canada

Scleroderma, a condition that involves the hardening and tightening of the skin and connective tissues, is more than a medical term; it's a reality that thousands live with every day. While scientific definitions provide clarity, they often fail to capture the human experience behind the diagnosis. It's through personal stories that the true impact of scleroderma comes to light, presenting the emotional and physical journey of those affected. By exploring these stories, it sheds light on the courage, challenges, and resilience that define the lives of individuals with scleroderma.

In Issue 5, we feature a tapestry of personal stories from those battling scleroderma. Each narrative offers a unique perspective, from the shock of initial diagnosis to the rollercoaster of emotions that ensues. These stories are not just about the struggles but also the victories, big and small, that punctuate this journey. Through them, we gain a deeper understanding of the day-to-day realities and the indomitable spirit of those living with scleroderma.

No one should navigate scleroderma alone. The stories we've gathered underscore the vital role of support systems—family, friends, and specialized scleroderma communities. These networks not only provide emotional solace but also practical advice for managing daily challenges. Shared experiences forge a bond that empowers individuals, highlighting the

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transformative power of community in the face of adversity.

Amid personal anecdotes, a wealth of medical and therapeutic insights emerge. From cutting-edge treatments to tried-and-true home remedies, our storytellers share their journeys through the medical landscape of scleroderma. These narratives vividly demonstrate the importance of ongoing research and innovation in improving the quality of life for those with scleroderma, showcasing the hope that new discoveries bring.

Raising awareness about scleroderma is a recurring theme in the stories we've collected. Individuals and groups alike are championing the cause, seeking to enlighten the realities of living with this condition. Their advocacy efforts aim to improve care, boost research funding, and ensure that no one faces scleroderma in the shadows. This section highlights the pivotal role of advocacy in driving change and fostering a more understanding and supportive community.

Perhaps the most inspiring aspect of these stories is the resilience and hope that shine through. Despite the undeniable challenges, individuals with scleroderma find ways to grow, change perspective, and uncover joy in life's moments. This section celebrates the strength and optimism that fuel the journey, offering encouragement and inspiration to us all.

The mosaic of personal stories presented in this article reflects a collective strength and courage that transcends scleroderma. These narratives are a testament to the human spirit's ability to confront adversity with resilience and hope. As readers, we're called to support scleroderma research, raise awareness, and join the community in solidarity. Together, we can make a difference in the lives of those touched by this condition.

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Empowering Wellness Through Movement: Navigating Scleroderma Through Physical Activity

By Liya Khan from Newmarket, Ontario, Canada

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Patients living with SSc are at a greater risk of developing pulmonary hypertension, pulmonary fibrosis, kidney, and bowel disease due to scar tissue obstructing the lungs, elevated blood pressure, and lack of mobility in the stomach. SSc also causes blood vessel abnormalities, damaging internal organs, and immune system dysfunction - impairing the balanced function of B cells, T cells, and macrophages. Patients also commonly experience acid reflux, heartburn, the Raynaud phenomenon and telangiectasias.

Localized scleroderma can manifest as thickened patches, lines and spots of skin on local body parts, such as the limbs or face. It can be characterized by the unbalanced formation of collagen, made up of fibroblasts, which are unnecessarily being activated, causing tissue damage and the malfunction of certain organs and body parts.

Investigating the Impact

A study showed that due to factors such as joint pain, limited range of motion, fatigue, dyspnoea, and abdominal pain, individuals with scleroderma tend to have relatively low levels of daily physical activity. These factors are the result of the experienced inflammation, lack of blood circulation and compromised organ systems.

Exercise is progressively being regarded as a non-pharmaceutical rehabilitation intervention for patients with scleroderma, as regular fitness may lead to anti‐inflammatory benefits, improve circulation, help maintain a range of motion and improve overall functioning to support daily activities.

A systematic review conducted by Sophie Liem and colleagues, examined randomized controlled trials and observational studies which compared different forms of exercises and their effects of body functions; oxygen uptake, aerobic capacity and muscle strength. The participants were 18 years of age and older with scleroderma.

Studies found that mobility hand exercises done at home for 50 minutes, 5 days/week, improved hand functional capacity, mobility, and grip strength through significant improvements in the Dreiser’s index, HAMIS, and Cochin hand function scale (all tools for hand function assessment). Orofacial exercises, such as mouth-stretching and oral-augmentation exercises, for 6-20 minutes, twice daily, were shown to significantly improve oral aperture by approximately 10.7 mm.

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Studies that focused on aerobic training, showed that treadmill walking, high-intensity-interval training followed by brisk passive recovery, and ergometer cycling for approximately 20-30 minutes at moderate to high intensities can increase values of VO2 peak, peak exercise oxygen saturation and metabolic equivalents. Muscle-strengthening exercises showed that resistance training with optimal repetitions 30-50 minutes, 2-3 days a week, can eventually improve values of VO2 peak, improve muscle strength, function and endurance, time to exhaustion, resting heart rate, and reduce fatigue. Individuals who engaged in both aerobic and muscle-strengthening exercises, showed converging benefits and reported greater quality of life.

Scleroderma Chronicles: Navigating My Personal Health Journey

Through reading Scleroderma Stories issues 4 and 5, physical activity is represented as a realistic therapeutic intervention, promoting overall well-being and improving quality of life for individuals across all spectrums of health. Many of the interviews highlight routines of hand exercises to maintain hand mobility and reduce tractions, and walking exercises to keep active.

Santos, a scleroderma warrior, shared how yoga and consistently working out clinically benefitted her lungs. Other individuals share how they replace higher intensity activities, like running, with moderate intensity activities, like walking, to stay active in a safe manner. It's important to note that not every condition is the same and individuals with scleroderma have compromised organs and functional capacities to different severities, affecting their level of ability to exercise.

There are many stories similar to Iddon and Romero, of individuals living with scleroderma that had to stop exercising upon disease onset or unaware of how to incorporate practical fitness in their routine but want to. When starting your fitness journey from scratch, it's important to start with finding your current “why”, take time to improve your fitness knowledge, set realistic goals and slowly work towards them. Cohen notes that physical activity, from a mental health perspective, plays a significant role in a healthy and positive mindset. You should also start your fitness journey with a similar positive mindset to set proper and lasting intentions.

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Although the benefits of physical activity look clear, it's important to consider specific ways individuals with scleroderma can pragmatically incorporate it into their lifestyle. Whether it's simply taking walks or doing hand exercises consistently, it's important to make sure your body is being heard and to take steps in finding a fitness regimen that is fitted for you.

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Individuals with scleroderma (SSc) can benefit greatly from conventional therapeutic interventions, such as achievable fitness regimens, to support their unique health needs. These interventions can help reduce feelings of social alienation by promoting community engagement and shared activities tailored to their abilities, sustaining pre-SSc symptoms behavior. By incorporating structured exercise into their routine, individuals can adapt to lifestyle changes upon onset and sustain overall health and well-being.

Scleroderma is characterized by a complex interplay of fibrosis, inflammation, vascular damage, and immune system dysfunction. SSc can affect the ability to engage in physical activity due to joint stiffness, fatigue, and potential organ involvement causing pulmonary challenges, cardiovascular strain, and abdominal constraints, which may limit range of motion, mobility, and endurance.

Systematic literature reviews, particularly investigating the effect of different types of exercise therapy, such as hand exercises, orofacial exercises, aerobic and muscle-strengthening exercises, and uniform management of physical impairment are required to design and assess treatment modalities for patients with scleroderma.

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Mental and Physical Health Concerns for Optimal Well-Being

Through reviews evaluating psychiatric symptoms and quality of life (QoL) among patients with SSc, it was found that psychological distress was associated with physical HRQoL, perceived physical health, and quality of life. Regression analyses revealed that a low level of SOC, a measure of an individual's resilience confronting stress and coping capacities, was associated with high levels of psychological distress and depressive symptoms in SSc.

A systematic review found that orofacial exercises (e.g. mouth-stretching and oral-augmentation exercises), mobility hand exercises, aerobic training (e.g. treadmill walking, ergometer cycling), and muscle-strengthening exercises may improve oral aperture, hand functionality, oxygen uptake, resting heart rate and overall strength respectively. Rehabilitation therapy, such as fitness, has resulted in improved quality of life for decreasing disabilities. It was particularly noted that evidence on the effect and safety of exercise therapy in SSc is relatively limited.

Scleroderma Interviews: About Natasha Sisodiya

“My name is Natasha Sisodiya, and I turned 51 in February. I have been married for 26 years and have two boys, Shaan (19) and Taran (16) and was born in India and raised in Saudi Arabia and Canada before moving to San Francisco in 1992. In August of 2022, my family and I moved to Los Angeles. By profession, I am an accountant but am currently not working.

I have a passion for hiking, exercising, and cooking healthy food. After being diagnosed with scleroderma, my passion for cooking arose as I had to watch my diet carefully. It led me to create an Instagram page (@bytesofyum) to share my healthy cooking and support me during my journey.

I am still passionate about hiking and would love to hike somewhere overseas like Kilimanjaro in Africa someday.”

Natasha was diagnosed with systemic scleroderma in 2012 but still explores her fitness journey with a conventional approach. She has been actively engaged in aerobic activities, particularly walking/hiking, 4-5 times a week and strength training 3-4 times a week, for almost 3-5 years respectively. Upon scleroderma onset, she describes having to reduce overall fitness

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Scleroderma is an autoimmune disease that causes fibrosis and inflammation in the skin and other areas of the human body. This is due to the immune system's response triggering tissues to think they are injured, leading to an overproduction of collagen. Collagen is a protein required for healthy connective tissues that support and hold human organs in place. Scleroderma can be divided into two categories: systemic sclerosis and localized. Systemic sclerosis affects multiple systems within the human body and induces damage to blood vessels and internal organs including the heart, lungs, and kidneys. In comparison to localized scleroderma which only affects the skin and structures directly beneath the skinny causing fibrous, waxy patches on the skin.

Medline Plus defines the family of genes associated with developing this autoimmune condition as the human leukocyte antigen (HLA) complex. This complex is located on the short arm of chromosome 6 and transports processed peptide antigens which use alloantibodies to fight against leukocytes. The Yonsei Medical Journal illustrates the HLA complex to assist the human immune system by differentiating between the body’s proteins and proteins produced from unknown sources including bacteria and viruses. In addition, each HLA gene consists of different normal variations to

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fight a wide range of unknown proteins, and these normal variations increase the risk of producing scleroderma. Examples of these genes include IRF5 which influences diffuse cutaneous systemic scleroderma and STAT4 which influences limited cutaneous systemic scleroderma.

We strive to engage the audience in complications related to being diagnosed with scleroderma at the molecular level. Two common compilations caused once a patient is diagnosed with Scleroderma include Raynaud’s Phenomenon (RP) and Scerldeorma Renal Crisis (SRC).

Raynaud’s Phenomenon

Raynoud’s phenomenon (RP) is when small arteries that supply blood to the skin begin to narrow. This results in areas of the body including fingers and toes to respond to cooler temperatures and stresses by feeling numbness. By limiting blood flow to affected areas, we develop cold-induced vasoconstriction: a physiological process to redirect blood flow and protect the body from excessive heat loss. The Front Pharmacol Journal describes that this constriction is controlled by the reflex sympathetic release of norepinephrine and results in RP when it is exaggerated. RP involves local, neuronal, and hormonal mediators that contribute to the over-exaggeration of blood-carrying arteries in response to norepinephrine. Norepinephrine binds to adrenergic receptors (ARs) on the vascular smooth muscle cells (VSMCs) to evoke effects. There are three ARs: α1, α2, and β2 in which α1 and α2 are responsible for mediating cold-induced vasoconstriction. Moreover, exacerbating these ARs can lead to RP.

Scleroderma Renal Crisis

Scleroderma renal crisis (SRC) is a complication of scleroderma (specifically systemic sclerosis) that causes severe hypertension and progressive renal failure, hypertensive encephalopathy, congestive heart failure, or microangiopathic hemolytic anemia. The National Library of Medicine has found that 10%-25% of patients diagnosed with diffuse scleroderma experience this disease and 75% of diagnoses for this condition are made as soon as the first 8 months of developing scleroderma. Most models of pathogenesis suggest that SRC is associated with vascular endothelial injury and changes in cellular/humoral immunity. Moreover, scleroderma has antibodies including T helper lymphocyte type-2 (TH-2) activation, cytokine

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Privilege, scleroderma, and running a marathon – it’s the little things that matter

By Martin Nguyen-Khoa from Melbourne, Victoria, Australia

“There was an inevitability about their road towards one another which encouraged meandering along the route.”

– Zadie Smith

Something that has defined my 20s so far – despite still being 3 months from 22 – is this pervasive, hypersensitive awareness of my privilege. Almost everything that has contributed to who I am now has been a result of privilege. There are the more obvious signs: attending both a private and a selective school during my secondary years, having financial support from my parents even to this day, being able to afford non-essential luxuries.

But there are also the more subtle ones which I argue are the most significant. For one, I have the privilege to decide. To decide what I want to do with my life and what I want to use my privilege for. And I don’t ever want to sound conceited in saying this, as if privilege is some divine purpose that I’ve been granted. I’m still getting used to the language I use when discussing this. But I feel so lucky and so fortunate every single day to live the life that I live.

And I want to dedicate my life to using this privilege for good – within my own

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life and within the wider community.

Scleroderma? What’s that?

Like most people, I had never heard of scleroderma prior to beginning my internship at Scleroderma United. I began to learn that it was a rare autoimmune disease that’s brought about by excessive collagen production, which causes a thickening of the skin around the body. Beyond limiting movement and noticeable cosmetic differences, I further learned that scleroderma often severely impacts the health and wellbeing of the diagnosed individual. Patients can experience shortness of breath, hypertension, fatigue, mood alterations, reduced vascular system functioning and if severe enough, fatal organ failure.

I knew that if I was going to raise awareness and raise funds around this debilitating disease, I needed to do something that actually challenged me. So I asked my friends to donate, because for every $10 that was donated, I’d run a kilometre. I thought people would donate in smaller amounts. I’d raise maybe a maximum of $100. Running 10 kilometres would be a good challenge anyways, considering the last time I ran 100 metres was four years ago. $100 all donated to Scleroderma United’s noble missions. That’s great, right?

I raised $732 within 48 hours. I was going to be running exactly 73.2km. That’s a full marathon and a bit. Why didn’t you instead promise one push up for every $10? Why didn’t you promise 1 kilometre of bike riding for every $10? Naturally, I was worried, and my close friends were worried – not just for my health and wellbeing – but about whether I was going to have the time to do this right and to see it through to the last kilometre.

And then I watched Greg Cohen’s documentary, ‘Run Greg Run’.

Can’t stop, won’t stop

Greg Cohen was an avid fitness-enthusiast, being both an NCAA Division 1 wrestler and a CrossFit trainer. One day, he couldn’t feel his left hand at all. He was initially diagnosed with bilateral carpal tunnel, and then Hashimoto’s disease (an autoimmune thyroid disease). Eventually, he was diagnosed with limited systemic scleroderma.

For all of the symptoms of scleroderma, even just from the ones I listed earlier,

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fitness at Greg’s level should’ve been a long-lost venture. But then, he signed up to run four marathons within a year.

If that wasn’t inspiring enough, this comment in his interview to the Daily Express US might seal the deal:

“[And] I think the other thing is I think about people that aren't as fortunate as me, who are in worse positions with the illness, whether they have severe lung involvement and they have to be on oxygen or their body doesn't allow them to do the things that I'm able to do. I run for those people as well.”

A reminder of how to be great

I never knew about Greg before choosing to run for my own fundraiser. But as you can imagine, his documentary deeply resonated with me. Not because I was also running to raise funds and awareness, but because he was also willing to use his position to do good. To help others and to really challenge himself for this cause.

I’m in no way placing myself at Greg’s level of commitment and dedication. To be able to acknowledge that there are people less fortunate than himself even while suffering with a debilitating disease is awe-inspiring; running four full marathons with scleroderma might elevate him to the status of a god.

But for someone like myself, who is healthy and able-bodied, it is a stark reminder that I have the privilege to decide what I want to do with my life and how I’ll give back to people more disadvantaged than me.

And luckily for me, I can decide how to do that while running 73.2 kilometres.

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Methotrexate: Is it really the right drug for me?

By Medha Nandikur from Frisco, Texas, USA

Scleroderma, also known as systemic sclerosis, is an autoimmune disease and there is no known cure for this terrible disease. However, there are ways patients of Scleroderma or other Autoimmune diseases treat the disease to reduce symptoms. One of these ways is using a commonly known drug known as Methotrexate.

Methotrexate is a drug that is in the antimetabolie family and it treats different types of immune system problems and it is also used to treat cancer. It is usually prescribed by a dermatologist or rheumatologist and it is given in the form of a pill or an injection. The pill form is small and yellow and it is taken from 5mg to 25 milligrams a week by Autoimmune patients. If patients take high amounts of this drug, that would be because this drug is mostly a chemotherapy drug that is used to treat cancer

The other form of Methotrexate patients take is in the form of an injection. The injection is usually from 7.5 to 25 mg similarly to the pill and it is injected underneath the skin. A study from Verywellhealth randomly assigned 384 patients with a methotrexate pill or injection after a 24 day period, patients who took the injection show that they prefer the injection over pill due to a better experience in taking it and having to take in less of a dosage

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compared to the pill.

However, both the injection and the oral pill can have both pros and cons to it. For some patients, this pill is a lifesaver while other patients experience horrible side effects. So if you decide to take the pill or not, be aware of the effects of this drug and decide if methotrexate is really the best thing for you.

A lot of the time, Methotrexate is a game changer for patients with autoimmune disease. For example, an article written on bezzyRA by Alexis Rochester says that after taking methotrexate for one month Alexis saw benefits regarding her joints in which she was able to move them with ease. Alexis saw that her swelling was getting better as well. Methotrexate is also said to reduce body inflammation as well as kill cancer cells since this drug is often used to treat cancer. Elaine from Elaine’s autoimmune adventure says that methotrexate helped her reduce her hair loss and made her hair a lot healthier and stronger.

Even though it helped Elaine through her autoimmune journey, some patients do experience hair loss along with other normal or life-threatening side effects as well. Aimee’s article by Versus Arthritis discusses how methotrexate made her very sick and unable to sleep. “I had lots of physio and occupational therapy to help me regain movement in my ankles and wrists” says Aimee. She did not have a good experience with methotrexate in which she decided it was not the drug for her. Other patients experience different side effects such as seizures, increased swelling, vomiting and/or more.

Remember, every body is different and every Autoimmune patient experiences different things. Some patients may experience multiple side-effects and some may experience nothing at all. Everyone has different experiences and perspectives of Methotrexate like shown in this article. Whether you take methotrexate or not depends entirely on your experience of this drug.

Summary:

Methotrexate is a drug that autoimmune patients take to relieve their symptoms. However, this drug has both pros and cons. Patients should talk to their specialist to discuss if methotrexate is really the right drug for them.