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Stay-At-Home Stories: Ariana Covarrubias

In response to the COVID-19 pandemic, stay-at-home orders were implemented across the nation to keep us indoors in order to protect ourselves and others. Our Stay-At-Home Stories blog series highlights some of the ways in which our EB Community has been passing the extra time at home and how the pandemic has affected them.

Today’s Stay-At-Home Story belongs to Ariana Covarrubias, a student at California State University Los Angeles and past debra of America Spirit Award honoree.

As a college student, it has definitely been a challenge to go forward with my academic learning while staying at home. However, with the lack of the in-person college experience I was able to gain long-lasting skills such as self motivation and organization skills. Due to my brother and I having EB, my family has taken extra precautions to keep us all safe. We've minimized our outings to only essential things and we've increased our disinfecting routines. Unfortunately though, my EB has noticed the change. Due to the temporary closure of clinics and other medical services, I had been unable to see my doctors until just recently. This has taken a toll on my skin as it's been harder to combat infections and improve the healing of my chronic wounds.  

A message that I'd like to share is to always keep others in mind when going out and/or having social outings, as not everyone has a strong immunity to overcome the virus. I hope everyone will continue to stay safe and gain strength from our current obstacles.

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Watch Ariana’s recent YouTube video below where she bravely talks more about the impact that the pandemic has had on her EB and mental health: 

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Ask the Nurse: Esophageal Strictures

Each month, debra of America’s “Ask the Nurse” series will highlight a commonly asked question received by our Acting Nurse Educator, Shawn Tavani, R.N. This month’s post focuses on esophageal strictures. Let’s get started! 

QUESTION: My 4 year old son who has Recessive Dystrophic EB (RDEB) is complaining of “food getting stuck in his throat” when he tries to swallow it. What is happening? 

ANSWER: He most likely has an esophageal stricture. 

Esophageal strictures are a serious complication of EB, with the RDEB subtype being the most prone to them. The esophagus is lined with the same tissue as the mucosa, which is also prone to blistering and sloughing and will form strictures of scar tissue where an area is affected. The act of eating can be difficult due to these issues with the mouth, throat and esophagus. Because we can’t see those areas with the naked eye, we have to depend on signs from those affected to know that they are having issues. Often, children will complain of it hurting when they swallow, will swallow hard, eating slower, and food may even get “stuck” at the area where the esophagus has narrowed from a stricture. They may even vomit the  food item because it won’t go down the esophagus. If forceful enough, the lining of the esophagus may also be vomited up as well as blood.  

To test for a stricture, a radiology test called a barium swallow can be performed. On the films, you can see the narrowed areas.  Sometimes there is only one stricture, and sometimes there are more. A procedure called an esophageal dilatation can be done to “reopen” the esophagus so that food can once again, move down to the stomach to be digested. A person may need to have repeat esophageal dilatations throughout their life to maintain an open esophagus, but the frequency can vary from person to person.   

To read more commonly asked questions, click here.

If you have an EB care-related question for our EB Nurse Educator, please email: nurse@debra.org or call toll-free at 866-DEBRA-76 (866-332-7276)

*The information provided herein is intended to educate the reader about certain medical conditions and certain possible treatments. It is not a substitute for examination, diagnosis, and medical care provided by a licensed and qualified health professional. If you believe you, your child, or someone you know, suffer from conditions described herein, please see your healthcare provider. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision.

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Ask the Nurse: Corneal Abrasions

Each month, debra of America’s “Ask the Nurse” series will highlight a commonly asked question received by our Acting Nurse Educator, Shawn Tavani, R.N. This month’s post focuses on corneal abrasions. Let’s get started!

QUESTION: My daughter won’t open her eye and says it hurts. She also says light is bothersome. Is this a sign of a corneal abrasion? What do we do to treat it? How can we prevent it from happening in the future?

ANSWER: Yes, this sounds like she is experiencing a corneal abrasion.

Corneal abrasions are a common complication of Epidermolysis Bullosa and can occur in all types of EB. While mostly seen in DEB and JEB, there have been cases of eye involvement in EBS as well. Any rubbing, itching, or other trauma done to the eye can potentially cause an abrasion.

Prevention is key in reducing the occurrence of corneal abrasions. Using lubricating gel ointments and drops can reduce their occurrence for many people. Using the ointments or drops before bedtime is recommended and, if needed, they can be reapplied during the day, especially for people who tend to have chronic corneal abrasions. Protective contacts, applied by an ophthalmologist, can also be used in preventing corneal abrasions in those who are more prone to them as well. Because corneal abrasions can cause progressive scarring and reduced vision, prevention is key. 

Corneal abrasions are extremely painful and often debilitating while the cornea heals. Because the eye becomes light sensitive, staying in a darkened room is often more comfortable for a person experiencing a corneal abrasion. The use of lubricating drops, pain relievers, and even prescription eye drops can aid in the healing of these abrasions. Occasionally, an ophthalmologist may also use a contact “bandage” to assist the healing process and make the eye more comfortable. Your ophthalmologist should be notified of an occurrence and a plan should be devised so that treatment can begin quickly to alleviate the discomfort and begin the healing process. A corneal abrasion will gradually resolve within a week and the person can continue their normal activities.

To read more commonly asked questions, click here.

If you have an EB care-related question for our EB Nurse Educator, please email: nurse@debra.org or call toll-free at 866-DEBRA-76 (866-332-7276)

*The information provided herein is intended to educate the reader about certain medical conditions and certain possible treatments. It is not a substitute for examination, diagnosis, and medical care provided by a licensed and qualified health professional. If you believe you, your child, or someone you know, suffer from conditions described herein, please see your healthcare provider. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision.

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Ask the Nurse: Pioneering Skin Graft Procedure

Each month, debra of America’s “Ask the Nurse” series will highlight a commonly asked question received by our Acting Nurse Educator, Shawn Tavani, R.N. This month’s post focuses on the revolutionary skin graft procedure that was recently covered in The Atlantic, the New York Times, NPR, and other major news sources. Let’s get started!

QUESTION: I recently saw a new piece where a little boy in Germany had much of his skin replaced with gene therapy altered skin grafts. I would like to know more and see about having this done for my child. How do I get this treatment for him?

ANSWER: This is incredible and exciting news in our world. The experimental skin grafting procedure was performed on one individual with Junctional Epidermolysis Bullosa, and is currently not a research trial open for enrollment. The results of this procedure serve as a great advancement in the scientific world, and hopefully hold promise for the future of EB Research. Mike Jaega, President of DEBRA International, released a statement regarding the particular trial and what may happen next:

“This is without doubt an incredibly interesting trial and a positive step forward that has had remarkable results. There has been a huge amount of interest and positive awareness since this story broke into the public domain. As someone with EB, I think it should be noted that this was a trial with a Junctional EB patient and the young man had to have extensive operations and monitoring. The results of this trial are an enormous step for those suffering from Junctional EB, but that doesn’t automatically mean it will transfer to other types and subtypes of EB. We need to support the next step in this process to explore if or how this trial can help other types of EB. The fantastic thing is that all these trials are a step in the right direction; one monumental step in finding viable treatments for EB around the world. It’s a hugely exciting time for research but we must keep our feet on the ground while we continue to reach for the stars. Gene and cell therapy trials, and even innovative ideas producing new dressings and appliances to help people with EB, are all important." Click here to continue reading. 

DEBRA International is a consortium of 56 national debra organizations worldwide. Brett Kopelan, Executive Director of debra of America, serves on the Executive Committee of the DEBRA International Board of Directors.

To read more commonly asked questions, click here.

If you have an EB care-related question for our EB Nurse Educator, please email: nurse@debra.org or call toll-free at 866-DEBRA-76 (866-332-7276)

*The information provided herein is intended to educate the reader about certain medical conditions and certain possible treatments. It is not a substitute for examination, diagnosis, and medical care provided by a licensed and qualified health professional. If you believe you, your child, or someone you know, suffer from conditions described herein, please see your healthcare provider. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision.

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Ask the Nurse: Dealing with Biofilm

Each month, debra of America’s “Ask the Nurse” series will highlight a commonly asked question received by our Acting Nurse Educator, Shawn Tavani, R.N. This month’s post focuses on biofilm. Let’s get started!

QUESTION: My son has a medium sized wound that isn’t healing with the use of a foam dressing and Aquaphor. I’ve even tried using some Polysporin on it but it just won’t heal. I have noticed a “film” on top of the wound as well. What should I do?

ANSWER: From your description, you may be dealing with a biofilm presence on the wound. Biofilms are a major obstacle to wound healing. Biofilm are complex communities of microorganisms, containing bacteria and fungi. The bacteria are embedded in a thick, slimy barrier of sugars and proteins which protects the bacteria from external threats. In order for wound healing to progress, the biofilm needs to be dealt with first. Debriding of the wound and would cleansing is the best method for ridding of the biofilm. Keep in mind that it is very difficult to rid of all of the biofilm at once as any non-visual remnants of the bacteria/biofilm has the potential to regrow and form a new biofilm on the wound. To avoid this, debridement may need to be done regularly.

The use of antimicrobial dressings and agents to kill microorganisms can help prevent biofilms from reforming. The most commonly used antimicrobial agents impregnated into bandages are silver and honey. Topical antibiotics can be used as well. The best indicator of knowing if the biofilms are gone is the progression of wound healing.

To read more commonly asked questions, click here.

If you have an EB care-related question for our EB Nurse Educator, please email: nurse@debra.org or call toll-free at 866-DEBRA-76 (866-332-7276)

*The information provided herein is intended to educate the reader about certain medical conditions and certain possible treatments. It is not a substitute for examination, diagnosis, and medical care provided by a licensed and qualified health professional. If you believe you, your child, or someone you know, suffer from conditions described herein, please see your healthcare provider. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision.

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Ask the Nurse: Feet

Each month, debra of America’s “Ask the Nurse” series will highlight a commonly asked question received by our Acting Nurse Educator, Shawn Tavani, R.N. This month’s post focuses on feet. Let’s get started!

QUESTION: My son has EBS and has been getting more blisters on his feet. While I know how to treat them once they appear, what can we do to prevent them?

ANSWER: For many with EBS, blistering on their feet is life altering. We often focus on blister treatment instead of prevention, but prevention can be an option for keeping blisters at bay.

Most people only think of using bandaging after a blister exists to aid in the healing process, but bandaging can also be used as a preventative measure on the feet. While there is not one specific product/bandage that will work for everyone, bandages can provide “padding”, especially in areas more prone to blistering on the feet, and may possibly reduce the amount of blistering that occurs. Non-adherent dressings are options that are used most often. They can be placed anywhere on the feet and either secured with rolled gauze for more protection, or with a retainer-net dressing like Tubifast or Surgilast. For some, a sock may work well enough too. Keeping the foot dry is also important in reducing the occurrence of blistering as sweat can make the feet moist and more suceptible to blistering. That may require the changing of socks during the day or even the use of antiperspirant products. Another preventive measure to try is the use of balms and sprays that runners use to protect their own feet from blisters.

While there is not one perfect option for everyone, you can individualize what works for your son. Hopefully some of these options will lessen his blister occurrence and make him more comfortable.

To read more commonly asked questions, click here.

If you have an EB care-related question for our EB Nurse Educator, please email: nurse@debra.org or call toll-free at 866-DEBRA-76 (866-332-7276)

*The information provided herein is intended to educate the reader about certain medical conditions and certain possible treatments. It is not a substitute for examination, diagnosis, and medical care provided by a licensed and qualified health professional. If you believe you, your child, or someone you know, suffer from conditions described herein, please see your healthcare provider. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision.

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Ask the Nurse: Infections

Each month, debra of America’s “Ask the Nurse” series will highlight a commonly asked question received by our Acting Nurse Educator, Shawn Tavani, R.N. This month’s post focuses on infections. Let’s get started!

QUESTION: My daughter developed a bad chest wound that I think is now infected. What should I do?

ANSWER: Skin infections are an unfortunate common complication of EB, even though caretakers try their best to keep wounds clean and infection free in order to encourage wound healing. Infections will occur in most people with EB at some point and should be addressed by a medical professional for treatment. Even with diligent wound care, other factors beyond your control may contribute to an infection developing. And unfortunately, topical antibiotic treatment may not be enough to stave off an infection. An infection may require oral antibiotics or a hospital admission for IV antibiotics.

So how can you tell if a wound is infected?  There are some key signs to look for in determining whether an infection is present. 

When a wound is infected, a person may have a fever or experience unusual tiredness. Meanwhile the wound may exhibit redness and swelling, yellow or green foul smelling drainage, and it may be painful and warm to the touch. You will also notice a delay in healing of the wound. It is extremely important that a physician examine the wound for evidence of an infection and perform a culture to not only confirm that there is an infection, but to also test for antibiotic sensitivities so that they can prescribe appropriate antibiotics.  It is vital that an infection be treated appropriately and quickly, as the infection could spread into the blood stream and cause further systemic complications.

To read more commonly asked questions, click here.

If you have an EB care-related question for our EB Nurse Educator, please email: nurse@debra.org or call toll-free at 866-DEBRA-76 (866-332-7276)

*The information provided herein is intended to educate the reader about certain medical conditions and certain possible treatments. It is not a substitute for examination, diagnosis, and medical care provided by a licensed and qualified health professional. If you believe you, your child, or someone you know, suffer from conditions described herein, please see your healthcare provider. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision.

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Ask the Nurse: Anemia

Each month, debra of America’s “Ask the Nurse” series will highlight a commonly asked question received by our Acting Nurse Educator, Shawn Tavani, R.N. This month’s post focuses on anemia. Let’s get started!

QUESTION: What is anemia and why is my child, who has EB, more prone to having it?  How do I know she has it?

ANSWER: Anemia is a common problem among those with EB. It is a condition where the body has a lower hemoglobin than normal. A blood test called a Complete Blood Count can be run to check for anemia.  Hemoglobin’s responsibility is to carry oxygen to various tissues in the body and supports new tissue growth and healing.   People with EB often have a lower hemoglobin test result due to wound bleeding, internal bleeding from wounds along the GI tract, and malabsorption issues of nutritional needs that can affect hemoglobin levels, such as the absorption of iron.

Signs of anemia are fatigue, low energy, trouble concentrating and focusing, pale skin, tiring easily with activities, and delayed healing. If your child is showing these symptoms, please contact your pediatrician to address these concerns. Oral iron can be given to increase iron levels if there is an iron deficiency causing the anemia, which often is a contributing cause in EB people. Many with extremely low iron levels or those who have failed on oral iron treatment due to absorption issues, can also be give iron infusions on a regular basis if necessary.   

To read more commonly asked questions, click here.

If you have an EB care-related question for our EB Nurse Educator, please email: nurse@debra.org or call toll-free at (866) DEBRA76 (866-332-7276) 

*The information provided herein is intended to educate the reader about certain medical conditions and certain possible treatments. It is not a substitute for examination, diagnosis, and medical care provided by a licensed and qualified health professional. If you believe you, your child, or someone you know, suffer from conditions described herein, please see your healthcare provider. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision.

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Ask the Nurse: Vaccinations

debra of America is very excited to introduce our new “Ask the Nurse” series! Each month, we will highlight a commonly asked question received by our Acting Nurse Educator, Shawn Tavani, R.N. Let’s get started!

QUESTION: My infant daughter has EB. Can she receive the routine recommended vaccinations? Are there any special instructions we should give our pediatrician about injections? 

ANSWER: Vaccinations during early childhood provide an important defense against serious illnesses. Routine vaccinations may be given at the appropriate, scheduled times as directed by your pediatrician. Alcohol or another cleansing agent should be gently dabbed onto the area where the injection will be given. Following the injection, there should be no vigorous massaging to the area. Gentle pressure should be held on the injection site to be sure the medication doesn't leak out. Your baby may have a mild reaction to some vaccinations, including a slight fever (under 102 degrees F), fussiness, and redness in the thigh area where the shot was given. These symptoms typically last up to two days and should be discussed with your pediatrician.

To read more commonly asked questions, click here.

If you have an EB care-related question for our EB Nurse Educator, please email: nurse@debra.org or call toll-free at (866) DEBRA76 (866-332-7276).

*The information provided herein is intended to educate the reader about certain medical conditions and certain possible treatments. It is not a substitute for examination, diagnosis, and medical care provided by a licensed and qualified health professional. If you believe you, your child, or someone you know, suffer from conditions described herein, please see your healthcare provider. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision.

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Ask the Nurse: DEXA Scans

Each month, debra of America’s “Ask the Nurse” series highlights a commonly asked question received by our Acting Nurse Educator, Shawn Tavani, R.N. This month’s post focuses on DEXA Scans. Let’s get started!

QUESTION: Hi. My daughter’s physician wants her to have a DEXA Scan. What is this test and why does she need to have it done?

ANSWER: A DEXA (dual energy x-ray absorptiometry) Scan measures bone mass density or how “strong” your bones are. In people with EB especially, but not limited to the more severe types of EB, their bone mass density can be lower than normal, which means their bones are not as strong and can lead to bone fractures. Your physicians may refer to terms such as osteopenia (a precursor to osteoporosis where bone mineral density is low), and osteoporosis (causes bones to become weak and brittle, more prone to breaks and fractures) if the bone mass density is low. There are many reasons why people with EB can have lower than normal bone mass density including inactivity, decreased muscle mass, nutritional deficiencies, chronic inflammation, and delayed puberty. It is important to have the DEXA scan done so that your physician can determine if your daughter has a lower bone mass and can treat her in a timely and appropriate way.

To read more commonly asked questions, click here.

If you have an EB care-related question for our EB Nurse Educator, please email: nurse@debra.org or call toll-free at 866-DEBRA-76 (866-332-7276)

*The information provided herein is intended to educate the reader about certain medical conditions and certain possible treatments. It is not a substitute for examination, diagnosis, and medical care provided by a licensed and qualified health professional. If you believe you, your child, or someone you know, suffer from conditions described herein, please see your healthcare provider. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision.

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Ask the Nurse: Wound Healing & The Flu

Each month, debra of America’s “Ask the Nurse” series will highlight a commonly asked question received by our Acting Nurse Educator, Shawn Tavani, R.N. This month’s post focuses on wound healing & the flu. Let’s get started!

QUESTION: I was doing OK with my wounds healing but when I caught the flu, my wound healing slowed down. Does the flu affect my wound healing?

ANSWER: That is a great question. Having an illness, such as the flu, along with EB, can complicate the healing process. Your immune system was previously focused on wound healing, but with the flu, the immune system becomes weakened and refocuses on the flu itself. The flu becomes its primary target. Because of this refocus, wound healing can slow down during illness such as the flu, and there could be an increased susceptibility to infection of wounds.  During this time, there are a few things you can do to help yourself: get some rest, be diligent with wound care, and make sure you are getting adequate nutrition while you are ill. Hopefully by incorporating these recommendations you will start to feel better, your body will recover, and you may see your wounds heal as they were prior to becoming ill.

To read more commonly asked questions, visit: debra.org/nurse

If you have an EB care-related question for our EB Nurse Educator, Shawn Tavani, RN, email: nurse@debra.org or call toll-free at 866-DEBRA-76 (866-332-7276)

*The information provided herein is intended to educate the reader about certain medical conditions and certain possible treatments. It is not a substitute for examination, diagnosis, and medical care provided by a licensed and qualified health professional. If you believe you, your child, or someone you know, suffer from conditions described herein, please see your healthcare provider. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision.

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A 2016 DCC Photo Journey

Thank you to all who attended & supported the 2016 debra Care Conference! Ari Espay, award-winning photographer, joined us at this year’s Conference to capture some of its special moments on camera. Check them out below:

Group Photo: 550 attendees strong!

Social Time: Friends, Family and Fun!

Keynote Speaker: John F. Crowley

PHOTO CAPTION: John F. Crowley, father of two with Pompe Disease, National Chairman of Make-A-Wish America and Chairman & CEO of Amicus Therapeutics Inc, inspired our EB Community as the 2016 DCC Keynote Speaker.

General Sessions: Presentations & Discussions

PHOTO CAPTION: M. Peter Marinkovich, MD, presenting on Clinical Trials of Gene Therapy for RDEB.

PHOTO CAPTION: Jakub Tolar, MD, PhD, discussing Bone Marrow Transplants.

PHOTO CAPTION: Anne W. Lucky, MD, providing an update on Genetic Classification of EB & Interdisciplinary Care. 

PHOTO CAPTION: Joe Murray, Jennifer Bernstein, Andrew Tavani, and Brett Kopelan sharing tools to becoming a successful EB Advocate.

Kids’ Activities: Made to be Awesome

PHOTO CAPTION: Superman brightens up Monday morning! 

PHOTO CAPTION: Professor Brainius’ Wild and Wacky Science Show!

PHOTO CAPTION: Painting Party!

PHOTO CAPTION: Antonio Delgado’s Sing-a-long.

PHOTO CAPTION: Miki the Monkey monkeying around for the kids! 

PHOTO CAPTION: Last but not least, Texas Sheriff's Association surprise visit!

Sponsors & Exhibitors: Thank you!

PHOTO CAPTION: Thank you to all our generous sponsors for supporting the 2016 DCC: Molnlycke Health Care, Amicus Therapeutics Inc., Hollister, McKesson Patient Care Solutions, Fibrocell, CCS Medical, Crawford Healthcare, Curefini, Edgepark, PolyMem, Puracyn, befriend EB, The Butterfly Fund, and US EB.

We can’t wait to see you at our next debra Care Conference in 2018!