Raising Marley HOW WE NAVIGATE EPIDERMOLYSIS BULLOSA EB

Raising Marley HOW WE NAVIGATE EPIDERMOLYSIS BULLOSA (EB) Presented by: Shauna-gay Case

Marley Case (The Jamaican EB Warrior)

What is EB? "The Worst Disease You've Never Heard Of" —is a rare connective tissue disorder with many genetic and symptomatic variations. All forms share the prominent symptom of extremely fragile skin that blisters and tears with any friction RARE PAINFUL GENETIC PERVASIVE DEVASTATING DEBILITATING ISOLATING AGONIZING COMPLEX EXPENSIVE According to stats, 1 in 20, 000 Americans are born with EB. Here in Jamaica, those stats are seemingly much lower. While doctors have heard of a handful of cases in recent years, Marley seems to be the only living local with the condition.

Marley's First Picture

How EB manifests There are 3 main types of EB: EB SIMPLEX JUNCTIONAL DYSTROPHIC Marley's initial diagnosis was Dominant Dystrophic but he has not been tested for confirmation. No two EB patients are alike. Visit debra. org for unparalled info on all things EB EB IN MARLEY • skin blisters • scarring • constant itchiness • loss of fingernails • fused fingers and toes • fecal hoarding • esophageal tearing • acid reflux • occasional light sensitivity

Skin is everywhere EB therefore manifests both internally and externally

Medical Support DERMATOLOGY Marley has been seeing top dermatoplogist, Dr. Michael Fitz-Henley since birth. He continues to provide invaluable advice and support and has pointed us to information and resourses that guides how we navigate Marley's skin care. PAEDIATRICS Dr. Palomino-Lue Marley's paediatrician while we lived in Kgn. The lady is a gem! Dr. Jason Mungal. Singh Marley's current paediatrician. Him and his staff are so accommodating and Marley sees his visits with Doc as a treat. PHYSIOTHERAPY Andrea Cameron - I wish we met her sooner. NHF We have. been approved to purchase some of Marley's supplies directly from the NHF warehouse/pharmacy, reducing ongoing costs significantly

Bath & Bandaging Sessions

Baths are critical and complex Marley needs warm saline baths. We have used donated saline packs but typically we eyeball it with purchased sea salt. Others incorporate chlorine but we've stuck to sea salt. We also try different herbs, use aloe vera etc and at times we add some antiseptic solution (Dettol) to his baths. Baths often last as long as 3 hours as it encompasses unbandaging, actual soak and bath as well as bandaging Baths are done every other day

OLD PIC OF A BIG BLISTER RECENT BATH PIC

Bandaging /Wound Care Management • Marley requires constant bandaging. Bandaging sessions are best done with at least 2 people. • It involves the use of injection needles (we call poppers. Diabetic needles) to pop each blister. • antibiotic and antibacterial creams, steroids occasionally • different types specialized bandages all of which are not local • conforming gauze (bought through NHF (retail is far too expensive) • moisturizing agents (vaseline and aquaphor • antihistamine creams (hydrocortisone etc)

Nutrition & Marley's Journey to Solid Foods Nutrition has been a big concern for us from the very beginning. Marley could not be breastfed. We 'drip fed' him from a nipple bottle until he turned 6 years old. • Green Smoothies have been a staple. • Fibre is essential and he gets vitamins • We stay away from processed foods and sugary foods. • No candy, no cow's milk. His dairy intake include yogurt and occasional ice-cream as a treat to soothe his blistered tongue

Itchiness and Pain Management • That Marley experiences more pain than even an adult can tolerate goes without saying. EB can be extremely painful • We give him fairly standard pain medication (Panadol and Ibuprofen). We are wary of strong meds • Itchiness is the bane of his existence. It frustrates Marley and it is the issue I yearn to solve for him as a parent. • Itchiness is a byproduct of skin healing and also a cause for blistering. . . a gnarly cycle • Creams, antihistamines and diet so far have been the course of treatment

Skin Care & Maintenance EXTERNAL MOISTURIZING Moisturizing is super important for Marley. We try to keep his skin hydrated as much as possible. We use lotions for sensitive skin, Aquaphor and homemade salves and creams. INTERNAL MOISTURIZING Hydration via lots of water makes a big difference. Marley typically gets copious amounts of it as well as his favorite, coconut water everyday. One other helpful thing we've done is to add a spoon of coconut oil to his smoothie. Research shows lots of benefits to ingesting coconut oil and I believe it is helping his skin.

Physiotherapy & Finger Fusing Despite efforts to prevent fusing, Marley's fingers have severely fused. He currently uses his thumbs and he still has relative use of his left index finger. Dr. Cecil Aird has seen him and has reached out to surgeons abroad for possible surgery going forward. This is an area of admitted confusion for us.

Marley's Struggles • SOCIAL ANXIETY Marley quite understandably developed a fear of interacting with children; particularly young children. He yearns to play with them but he physically tenses up when he sees little children doing regular things like running or playing with balls etc. • ANTICIPATORY ANXIETY Marley lives in fear of pain. As he gets older, this fear seems to be intensifying. This makes unavoidable tasks like bathing him extremely difficult. He will scream and sometimes refuse to be touched and baths turn into a negotiating session. We are learning to navigate that with prayers prior to and during baths, deep breathing exercises and playing his favorite worship songs to shift the tension.

Bandage Donations Wound Care Supplies are costly. Some bandages are so specialized that we actually have never tried them and in other cases, we sensibly ration out what is difficult to source. Still, donations from people here and abroad have really blessed us. In 2015, a good samaritan, Sasha Palmer created a Go. Fund. Me and that helped us tremendously. Gratitude abounds in all cases.

How we Cope as a Family

• Prioritize family time • Accept that we are a dynamic family • Intentional about our words and I don't allow negative talk from others • Manage expectations of each other. . . empathy is golden • Valuing individual time too because if we're not careful, everything will revolve around Marley's condition

How I Cope • God Time/Self Care - Note that self-care will often look different for us than for other moms/people and that is ok • Affirmations • Maintainng a Gratitude Practice • Journaling • Godly Circle • Managing Expectations and Acceptance (disassociation) • Protecting my Mental Space - toxicity needs to be cut off

Gratitude is a powerful catalyst for happiness. It’s the spark that lights a fire of joy in your soul. Amy Collette

Empowering Marley

• • • Focus on what he can do vs on what he can't Prioritizing Joy Validating his feelings Ensuring inclusion Having him repeat affirmations Facilitating openness, aunthenticity and vulnerability Creating a Faith-filled atmosphere Teaching/homeschooling Marley Being Sensitive to his Anticipatory Anxiety Hyping up every little thing he does, encouraging him to be proud of his small wins

Encouragement for all you other moms #YOUMATTER!!!

"Until you have a child with special needs you have no idea of the depth of your strength, tenacity and resourcefulness ANONYMOUS

MY PRAYER

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