For many people, appearance or comfort is the most important consideration when choosing a hat to wear. But for people living with Xeroderma Pigmentosum (XP), an inherited disease that causes an individual to be extremely sensitive to ultraviolet radiation, health and safety must take precedence. As part of her ongoing efforts to raise awareness and support for individuals with XP, Dr. Laura Niedernhofer (College of Biological Sciences) partnered with Professor Lucy Dunne’s (Apparel Design) technical design studio to help design a better hat option for people living with XP.

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Dear Patients and Family members

Thank you for your participation in our research at the National Institutes of Health on the DNA repair conditions xeroderma pigmentosum, trichothiodystrophy and Cockayne syndrome. During these difficult times we would like to provide you with some information to help you and your families stay healthy.

COVID-19 is a new disease caused by the novel coronavirus infecting humans, and there is much that is still not known or understood about it. Currently, physical distancing, frequent hand washing, use of hand sanitizers and wearing face coverings such as tightly woven cloth face masks or even UV blocking face shields are the best ways to prevent spreading the virus. With physical distancing, staying 6 feet apart, these measures help from contracting the infection.

We do know that people with neurological problems such as XP neurologic disease and Cockayne syndrome can experience a temporary worsening in their symptoms when they are suffering from any infections that cause flu-like symptoms or pneumonia. Therefore, we might expect if XP patients with neurological problems or Cockayne syndrome patients develop symptoms from the coronavirus infection, they may get worse while the respiratory symptoms or pneumonia persist. TTD children, who often have immune problems, may be more vulnerable to the respiratory complications of COVID 19 and physical distancing is very important for them. The World Health Organization has also advised on the importance of treating Vitamin D insufficiency because of increased time indoors. We would recommend all of our patients continue on supplementation with oral Vitamin D.

We understand that social isolation, reduction in physical activity, unpredictability and changes in routine can all contribute to increasing stress. People living with DNA repair conditions often have some aspects of physical distancing. However, missing out on school activities, therapies or being required to attend school classes and work from home can feel isolating.

Several types of light bulbs including unshielded florescent and halogen bulbs emit higher levels of UVR (ultraviolet radiation) than is safe for people with XP and photosensitive TTD. These are often found in areas like cafeterias and gyms. Recently, high UVR (UVC bulbs) emitting light sources have been proposed for use in public areas as a means to protect from the coronavirus. It is important for people with XP and photosensitive TTD to be aware of all of these light sources in the environment. If necessary, re-check public areas with a UV meter to assess if it is safe.

We are thinking of you during this difficult time and are here for you if questions or concerns arise. Please contact us at the email and phone numbers listed below.
Phone and Email:
Dr. Kenneth Kraemer: 240-760-6139 kraemerk@nih.gov
Dr. John DiGiovanna: 240-760-6138 jdigiovanna@nih.gov
Debby Tamura RN: 240-760-7355 tamurad@mail.nih.gov
COVID-19 is an emerging, rapidly evolving situation.
• What people with cancer should know: https://www.cancer.gov/coronavirus
• Get the latest public health information from CDC: https://www.coronavirus.gov
• Get the latest research information from NIH: https://www.nih.gov/coronavirus

As part of the NIH Intramural Program, CCR has shifted all non-mission-critical laboratory operations to a maintenance phase in order to promote physical distancing and diminished transmission risk of COVID-19. Effective Monday, March 23, 2020, only mission-critical functions within NIH research laboratories will be supported.

Below are some websites that provide updated information on COVID 19 and living with the social consequences of the infection.

https://faq.coronavirus.gov/ – information from the Centers of Disease Control and Prevention
https://directorsblog.nih.gov/2020/04/07/dealing-with-stress-anxiety-and-grief-during-covid-19/ – a wonderful conversation between NIH Director Dr Francis Collins and Dr Joshua Gordon director of the National Institute of Mental Health (NIMH) on dealing with stress, grief and anxiety during COVID 19.

https://www.nimh.nih.gov/news/science-news/2020/supporting-mental-health-during-the-covid- 19-pandemic.shtml – link to the NIMH website section addressing some of the emotional issues occurring while living with COVID 19 pandemic. There are links to other sites for additional information on dealing with stress.

Below are some websites pertaining to services for children with disabilities.

https://www.americanbar.org/groups/litigation/committees/childrens-rights/articles/2020/are- special-education-services-required-in-the-time-of-covid19/ – Link to the American Bar Association statement on special education services and COVID 19. Contain ssuggestions for parents of special needs children who are experiencing disruption of services.

https://www2.ed.gov/about/offices/list/ocr/frontpage/faq/rr/policyguidance/Supple%20Fact%20Sheet%203.21.20%20FINAL.pdf. This is a statement from the Federal Department of Education describing the responsibilities of local educational districts to children with special needs.

https://www.parentcenterhub.org/ Online center for parents who have children with disabilities. Provides information on COVID 19 and special education needs.

Everyone has sun-sensitive skin. It’s the reason we tan and sunburn. It’s why freckles, age spots, and skin cancers appear.

Some people are born more sun sensitive than others. People who have an extreme sensitivity to sunlight are born with a rare disease known as xeroderma (zer-o-der-ma) pigmentosum (pig-men-toe-sum). They must take extreme measures to protect their skin from ultraviolet (UV) light. Anything that emits UV light, including the sun and some lightbulbs, can damage their skin.

When people with xeroderma pigmentosum (XP) go outdoors during daylight, they need to cover up. Dermatologists recommend that they wear clothing which offers maximum protection from UV light. To protect their face and eyes, they should put on UV-protective sunglasses, goggles, or a face shield. Beneath this outer protective layer, they have on sunscreen, which is necessary should any skin become uncovered.

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Book Review: Midnight Sun by Trish Cook (soon to be a major motion picture)

The book Midnight Sun is told from the perspective of a girl named Katie who has Xeroderma Pigmentosum (XP). But, the story is greatly exaggerated. Katie says that even if the sun touches her skin, she will die. So she stays locked inside, only going out at night. Here are some parts of the book that I, as person who has XP, found amusing and disturbing.

Describing XP, Katie says, “If sunlight so much as glances off my skin, I’ll get skin cancer, and my body can’t repair the damage so my brain starts to fail.” That’s not how XP works. There are several different forms of XP. Some people do get skin cancer, but it doesn’t happen that quickly. Other people have neurological issues, but it’s not related to sunlight.
In her mind, Katie finds herself relating to the Greek goddess Cassiopeia: “According to Greek mythology, she’s chained to her throne, forever stuck there as punishment for her boastfulness and vanity. I wonder if sometimes I did something to deserve XP, something terrible that requires me to do penance in my house and my room all day, every day, until the sun sets.” I can understand how Katie feels that way sometimes. Sometimes I also feel like I’m stuck to my disability. Like I can’t get away from it. But, I think Katie could have found a better way to meet her needs. She could have found a way to live with her disability rather than being trapped by it.
Even the doctor in the book doesn’t get XP right. She says that when Katie does get barely touched by sunlight, it might be a “triggering event” that leads to brain damage and even death. I think if kids with XP were to read that, they might feel that they didn’t have a life anymore. They might feel like they have no hope. So I want to tell people that there is hope! There is no such thing as a “triggering event” that turns XP instantly deadly, so you can live your life! What happens to Katie doesn’t happen to real people with XP.
A lot of the story is about Katie’s relationship with her first love, Charlie. She doesn’t want to tell him that she has the disease. She thinks to herself, “Charlie Reed already has enough on his plate without adding me and my weirdo disease to it.” She literally has chances to tell him about XP at least three times, but she keeps it a secret. I don’t really understand that. I think you should give people a chance, because they might surprise you. Having XP doesn’t make you alone in the world. But in the story, Katie makes herself alone.
Finally, after having a crisis, Katie finds that she does have friends, and Charlie loves her even after he finds out about XP. It does bug me that in the end of the story, Charlie “saves” her from XP by showing her all the things she has been “missing” by staying indoors. And then she dies anyway. It’s like life’s not worth living if you have XP. That’s just not true.
So here are some facts about XP from someone who has it: We do go outside. It’s true we take precautions to avoid sun damage. We can wear clothes with special UV-blocking fabric. We cover as much of our bodies as we can. We also use sunscreen–a LOT of sunscreen. People who are very sensitive to the sun also wear a hood or face covering to protect themselves. It can be a hassle, but it keeps us sun safe. Most people with the type of XP Katie has do go to school, have friends, and enjoy all different kinds of activities during the day like anybody else. We just have to be a more careful. XP is a serious disease, but that doesn’t mean our lives are tragic.

If I could say one thing to the writer of the book, and the people making the movie, I would say, get your facts straight! Try to consider how real people with XP live and feel, and how this movie might affect them. Don’t portray us as just a disease, with death as the only option to escape. We are people, we have real lives, and we are worth getting to know.

If you want to learn more about XP, there are many resources, like the Xeroderma Pigmentosum Family Support Group (xpfamilysupport.org).

Originally posted at https://titaniumamyblog.wordpress.com/

A 17-year-old girl with a rare condition that prevents her from exposing her skin during daylight hours had her night in the sun on Friday when she was crowned homecoming queen of Dana Hills High School.

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