EB Related Eye, Mouth and Nose issues

EB can affect all aspects of the body; including the eyes, mouth, and nose.

Here are some tips from others with EB for those areas:

MOUTH

Those with EB are often affected by wounds in the mouth that can be painful, and slow healing which makes eating, drinking, and speaking difficult.  Some find swishing with salt water can be helpful.  Others find that magic mouthwash or Carafate can provide temporary relief from the pain

EYES

Eye Issues are a common and painful complication in those with EB.  When the eyes become dry, they can become damaged easily, causing corneal abrasions.   Keeping the eyes lubricated daily, but especially at night.  It is recommended that those with EB use preservative-free eye drops or eye ointment frequently to prevent abrasions.   Everyone has their own preference on which eye drops/ointment they use.  When corneal abrasions are frequent or begin to interfere with daily life, bandage contact lenses are often used.  These lenses are just like contact lenses and not only provide immediate pain relief and protection while the abrasions heal. Many with EB wear them daily to prevent corneal abrasions.  The most common brands used in those with EB are Air Optix Day and Night Aqua 30-day lenses. 

NOSE

Just like the rest of the body, the nose can be affected in those with EB.  The inside of the nose can blister or develop wounds.  Dry weather can cause nose bleeds as well.  Using saile nose spray or saline nose gel to help keep the inside of the nose moist to help reduce wounds and nose bleeds from developing.   When wounds become chronic inside the nose, scabs can occur, along with inflammation.  Over time the opening of the nose can scab closed, interfering with breathing.  Using a humidifier while you sleep, along with saline spray/gel can help keep the inside of the nose clear.  

Infections in EB: How to prevent them and how to treat them

While skin infections in EB are common, there are many things one can do to prevent infections. The following advice was given to me by an infectious disease doctor.

Prevention

1. ALL wounds need to be bandaged. Open wounds can easily become contaminated with bacteria causing an infection to start. Keeping wounds covered with bandages as they heal, not only prevents infections but also protects the wounds from further damage while they heal.

2. Bandages need to be changed daily and the skin needs to be cleaned daily. Some bandages need to be changed multiple times a day if there is heavy drainage.  The longer infection drainage sits on a wound, the more infected it becomes and the longer it takes to heal.

3.  Only use bleach when you have an active staph or strep infection, and no more than 3 times a week. Since it is cytotoxic it will kill both good and bad bacteria.

4.  Take showers, not baths.  Sitting in a tub of water with open wounds allows bacteria to spread to other open wounds.  A shower doesn't have to be like it sounds.  It can be sitting in an infant bathtub, making up a bowl of warm water and soap (or whatever you use in the bath) and gently pouring it over the body to clean it.  It could also be letting a toddler sit in the bathtub with just enough water to play in, and using a bowl or pitcher of warm, soapy water to clean them with.  But however you do it, clean the bathtub before and after bath/shower time.

5. Wash the clothing, bedding, towels, and blankets of those with EB separate from everyone else in the household to prevent the spreading of bacteria.

6.  Frequently clean your child's toys and other objects in the house they touch a lot- the TV remote, computer mouse/keyboard, door knobs, light switches, the toilet, fridge handle, etc.

7. Change clothing after coming home from school, work, and medical appointments.

Signs of an infection

• red or swollen wound, sometimes it will feel hot
• foul odor
• excessive drainage )more than usual)
• excessive pain and/or itching (more than usual)
• a fever

(Greenish-blue drainage can indicate a pseudomonas infection. Cloudy, pea-green, or yellowish drainage can indicate a strep or staph infection.)

Only a wound culture performed by a doctor can tell you what type of bacteria is causing the infection. 

The typical treatment of an infection

Treatment depends on the person's age, previous history of infections, and recommendation of their medical team.

Typically skin infections are treated with topical antibiotics, such as Polysporin or Bacitracin. Sometimes a prescription-strength topical antibiotic such as Bactroban is necessary. Only use Bactroban as prescribed. Overuse of it can cause your body to become resistant to it and it will no longer work when you really need it to. The same thing applies to oral antibiotics. Use those only when necessary so your body doesn't become resistant to them either.  In addition to topical and oral antibiotics, there are other home remedies that you can do to help eliminate the bacteria. 

For Pseudomonas Infection, you will want to use an acetic solution and the most common thing to use is Vinegar. 

The acetic acid formula is: Mix 1 ounce of 5% vinegar in 19 ounces of water.

Vinegar baths or compresses can be used at any age. Be sure you rinse well with clear, clean water after a Vinegar bath, otherwise, excess itching and irritation may occur. 

For a Staph or Strep-related infection, (including MRSA) you can take a bleach bath or apply bleach compresses to the infection area to help kill the bacteria no more than 3 times a week. Bleach is cytotoxic (meaning it kills both bad and good bacteria. When you kill off the good bacteria, things like pseudomonas and yeast start to take over) 

Bleach Bath Formula for Adults and Children OVER the age of 1:

Mix 1 teaspoon bleach in 1 gallon of water.

For Children and Infants UNDER the age of 1, 

Mix 1/2 teaspoon bleach or less to 1 gallon of water.

No matter what age, rinse well with clear, clean water after a bleach bath, otherwise excessive itching and irritation may occur.

Chlorhexidine is commonly used among those with EB, but it is also cytotoxic

NEVER mix Vinegar and Bleach together in the same bath. A chemical reaction can occur and produce a toxic fume causing respiratory issues.  In addition, adding other agents such as soap, salt, or bubble bath can alter the PH levels of the bleach (or vinegar) solution making it less effective against the bacteria.  It is also vital to use the correct ratio of water to bleach, or vinegar to water.  Too much water can dilute the bleach (or vinegar) making it less effective, while too much bleach (or vinegar) can cause stinging upon contact with the open wounds

How WE treat infections.

1- cover all open wounds

2- clean the wounds and change bandages at least once a day, sometimes more for heavily draining wounds.  

3- we start with topical antibiotics and/or antimicrobial dressings before we use oral antibiotics UNLESS we have multiple infected wounds and/or a fever is present.  

Some examples of antimicrobial dressings: Xeroform (NOT safe for use on Infants) Mepilex AG, Polymen AG or Biatain AG (also NOT safe for use on infants or young children) Hydrofera Blue Ready Foam,  and dressings impregnated with medical grade honey,   There are also topical antimicrobial agents: silver gel (NOT safe for use on infants or young children) MediHoney, essential oils that have been safely diluted for the age of the person.

Since we switched from baths to showers and followed the above steps, we have seen a significant reduction in the occurrence of infections. 

Blended diet 101

Whenever I tell people we did blended diet g-tube feeds for 12 years vs using pre-made, sugar filled commercial formula, they look at me like I have two heads.  I have gotten a lot of comments as well. 

The top 3

“OMG!!! that is too much work; i don’t have time to do that”

“My child can’t handle real food”

“How can this be better than scientifically made, nutritional complete out there?”

I have done previous posts on blended diet but I thought I would do an update on blended diet, providing step by step directions. 

Step one: get a blender.  For those that do blended diets, it is recommended in getting a commercial grade blender, such as a Vitamix or a Blentec.  We have a Vitamix blender.

Step two: start with basic ingredients: yogurt, applesauce (or any type of baby food), a grain (baby rice cereal or oatmeal is a great starter) and a liquid.  You can use water, milk, juice or formula.  

Step Three: combine all ingredients in the blender and blend until it becomes a liquid. Or smoothie type texture. 

Step Four: use a syringe and the bolus extension to push the blends through your child’s g tube. Then flush with a bolus of water (we used 20cc) amount will vary on your child age, weight and form of EB.  A nutritionist will help you determine how much free water your child needs per day.

Once you get comfortable with blending basic items, you can branch out.  Some people just blend up what they made for breakfast/lunch/dinner for their family, and feed that to their tubie.  The goal of a blended diet is to provide a healthy, well balanced whole food alternative to commercial formulas.  Each blend can be tailored to your child’s specific dietary needs.  

In EB, the goal is a high calorie, high protein, and low sugar diet.  Those with EB need anywhere from 100-300% more calories than a child without EB that is the same age, along with 115-200% more protein than a child without EB what is the same age,  Those with EB also need a lot of fluid in their diet to replenish what is lost from wound drainage and to aid in healthy kidney function and reduce constipation.  

A nutritionist can help you figure out what your child needs in their diet, as well as how much free fluid they need per day.

We use this calculator to determine what our child needs from a nutritional standpoint and we use the results from his previous blood work to see if we need to add any additional nutrients.  

I have done a tremendous amount of research on the nutritional content of a wide variety of food to figure out which combine of foods would get us the highest calorie, yet low volume blends (our child is volume sensitive)  Bur there are many apps out there that all your have to do is add the ingredients you are using, and it will calculate the nutritional value of those items.  One app I like is called Track.  All you have to do is add the food and quantities and the app calculates the calorie total and breakdown of the nutrients.   

For our blends we included the follow ratio in each batch:

3 cups of vegetables

3 cups of fruits

1 cup of whole grain (oatmeal, pasta, brown rice, quinoa) 

1 cup of  an animal based protein (eggs, cook chicken, cooked fish, 

1 cup of a plant based protein (seeds, tofu, lentils, beans peanut butter)

1 cup of a dairy product (yogurt, heavy cream, kefir, cottage cheese) 

A liquid base: milk (cow, goat, almond, rice) broths (chicken, vegetable, bone),  various juices (pear, prune, apple)  this amount can vary depending on how thick or thin you prefer the blends. 

.  

I blended all those items together then divided it up into the serving sizes we give per feed (this will vary from person to person).  For us it was 6oz.   I then continued to blend that ratio of those items until I had enough to make 7 days worth of feeds.  You can use Tupperware containers or Ziploc bags or breast milk bags to store each serving.   Once the blends are diving into individual servings, I then add one or more high calorie, low volume additions to EACH servicing size to increase the calories for that serving size.  I kept 2 days worth in the fridge and froze the rest, pulling out a day's worth at a time. 

*High calories low volume additions: peanut butter powder, flaxseed oil, olive oil, heavy cream, (benecalorie, duocal or liquidCal or beneprotein- these are all commercial made additives that can be easily mixed into blends.) 

One of the benefits of blending food is you can pick and choose what you want to use.  We used either fresh or frozen fruits and vegetables, depending on what was in season, and what his need was based on his blood work.  We aimed for using things high in iron, vitamin D, vitamin C and foods known to reduce inflammation.  I created a spreadsheet of how many calories, and which vitamin/nutrients each food was high in.  My favorites were kale, spinach, sweet potatoes, cooked apples, blueberries, mangos,  bananas, boiled chicken, hard boiled eggs, bone broth, kefir, brown rice, tofu, almond milk, prune juice, and peanut butter.  But I tried to use a variety of each category of food.  It is easy to make it a vegetarian diet, or a diary free diet as well. 

We saw consistent weight gain, quicker wound healing, less inflammation/pain and no issues with constipation.  He had more energy as well.  It also allowed him to eat anything he wanted, because he was getting all the good, nutritious food through his tube. No more fighting at meals to get him to eat fruits and vegetables.  

I’ll be honest, this is a lot of work, especially in the beginning when I was trying to figure out what to use, how much, etc.  But once I figured out how many calories he needed per day and the ratio of vitamins, minerals and nutrients, it became much easier and was just a part of my weekly routine.   But overall it was worth the extra work. I made our child MUCH healthier by being on a blended diet.

Hospital Guidelines

STOP!!! 

PLEASE READ BEFORE TOUCHING ME!!

I have Epidermolysis Bullosa.  It is a connective tissue disorder that causes fragile skin, internally and externally.

Here are the ways you can SAFELY care for me

1.  If you have not worked with an EB patient before, please ask my parents how to care for me,

2. I CAN NOT have ANY tape, even paper or hypoallergenic tape used on my skin.  Mepitac tape the ONLY safe tape that can be used on my skin.

3.  A Blood Pressure Cuff MUST be used over clothing or bandages, otherwise the pressure will rip my skin

4.  When taking blood, the tourniquets MUST be placed over clothing or bandages because the pressure will rip my skin.  Blood draws may be difficult and  can only be taken by an EXPERIENCED NICU nurse or while using IV radiology to find the best vein and avoid multiple sticks.   Do not rub the skin with alcohol wipes.  Gently dab the skin with an alcohol pad.  

5. DO NOT slide me during bed transfers.  Lift me by rolling me from side to side into your arms or let me parents show you how to move me.

6. For diaper changes, it usually takes two people.  DO NOT grab my feet or ankles.  Instead, one person should lift me with one arm under the back of my knees, and the other should change the diaper.  DO NOT rub the skin with wipes.  Use warm water to clean the area and gently pat dry. 

7. Coat exam gloves and any tubing that comes into contact with the skin, externally and internally with a non-petroleum-based lubricant.  

8.  Temperatures CAN NOT be taken rectally or in the ear.  Under the arm is the safest method, using a non-petroleum-based lubricant.  Infrared Thermometers can also be used, but are not always accurate in EB patients due to temperature regulation issues. 

9. PLEASE USE common sense and ask questions if you are unsure about my care.  

Created by ©Garrett's House http://http://ingarrettsmemory.blogspot.com/

 

Personal Stories: Piper Rose

 🌹Piper Rose, my Leap Year Baby🌹

My daughter Piper was born via c-section at 39 weeks right at the start of the COVID madness. 2/29/2020 at 2:29pm   I had a normal and healthy pregnancy, but her birth was anything but normal.  When the doctor pulled her out of my womb, her lower body was completely raw.  The hospital staff was completely shocked and horrified.  No one had ever seen anything like it. As they attempted to clear her airway, blood started pouring out.   She was whisked away straight to the NICU before I could even see her.  

The doctor stitched me up as quickly as he could as I laid there sobbing.  I was alone in the delivery room, and had no idea what was going on with my baby girl.  

Once I went to recovery, all the nurses were quiet around me, none one would tell me how my baby was doing; I was terrified she was dead, 

Eventually, the neonatologist came into recovery to speak to me.  A nurse came with him as well.  I was sitting there with tears streaming down my face,  afraid of the news I was about to receive.  

They had good news and bad news.  The good:  Piper was alive and they think they knew what was wrong.  The nurse had seen it before, but only once in her 25 years career.  The bad: they thought Piper had a rare skin condition called Epidermolysis Bullosa or EB for short.  I had never heard of it before, but it sounded very scary.  

The doctor explained she had very fragile skin and would need to be wrapped in protective bandages to prevent infection.  I asked if Piper would get better; the doctor said he didn’t know.  Some get better, some get worse, some die.  And worst of all, there was no cure :( 

They said I could see her as soon as I felt up to moving.  I just laid there in shock at the news I just received.  I was full of so many emotions.  I was all alone and had no idea if my baby would live or die.  

A few hours later I felt I could move into a wheel chair and a nurse wheeled me up to the NICU.  There was my baby, in this incubator covered in bandages and wires.  But she was beautiful with her Fuzzy blonde hair and blue eyes.  She was a whopping 9 pounds, 9 oz and was 21 inches long. Aside from the skin issue, she was perfectly healthy.  They wouldn’t let me hold her just let, so I sat there rubbing her head telling her how much I loved her.  

The next few days, there were so many ups and downs.  They sent off blood for genetic testing to confirm her diagnosis.  The doctors began researching EB to learn how to best care for Piper.   I had also enlisted my best friend, Ava,  to research EB. I couldn’t bring myself to look on-line for fear of what I might learn.  Ava found so much on line about EB; some good, some bad, some terrifying.  She got us a list of the correct bandages and ointments to use, links to videos on how to bandage an EB baby, the best clothes to buy, diapers to wear and bottles to use.   She found support groups online with so many helpful people also going through the same thing.  

Over the days I learned how to carefully bandage my baby to protect her skin from getting worse or getting an infection.  She struggled to breastfeed, so she was fed with a special bottle.  At first she started out eating amazing, but slowly started to struggle when she was around 10 days old, her breathing began to get “noisy” but no one knew why.    Around that time, her doctor learned about some hospitals that had an EB clinic.  He contacted one of them and spoke to the dermatologist there.  She had dealt with EB for many, many years.  She gave the doctors some good tips and suggestions but feared Piper had the worst form of EB, Junctional Hertliz,  based on his description of her.  

A couple of days later Piper stopped eating altogether and a feeding tube had to be placed down her nose into her stomach.  It was so hard to watch my baby fight  for her life.  I felt she was fading right before my eyes. 

When she was 2 weeks old, we still had no answers.  The unknown of  COVID was delaying everything.   The doctor felt she might receive better care at a hospital with an EB clinic.  I was all for anything to help my baby get better.  He contacted the nearest hospital with an BB clinic, but because of the uncertainty of  COVID, they weren’t accepting transfers.  The dermatologist however, offered to watch a bandage change via video conference before deciding to override the transfer request.

The next day, everything was set up and the doctor at the EB clinic watched me carefully unwrap, clean and rewrap my fragile baby.  Afterwards, we had a meeting and the dermatologist felt her hospital couldn’t provide any better care than Piper was already receiving.  She said transferring her would be risky and she might not survive the transfer.  She also said that in her many years of experience of treating EB patients, she felt Piper had  the Junctional Hertliz form of EB,  and she likely wouldn’t live past four months.  I asked about having a feeding tube placed, only to be told she might not survive the surgery.  It felt like no matter what we did, she was going to die anyway. 

That was devastating news to hear.  The thought of my baby girl dying was more than I could bear. I spent the next few days holding my baby girl, trying to decide what was best for her.  I wanted her to live, but what kind of life would she have???  Would she be in pain everyday of her life?.  Would she ever walk?  Eat without a feeding tube?  Here I was a brand new parent, all alone holding my fragile baby girl, feeling totally lost.  

Over the next few days, Piper started to decline.  Blood work showed she was anemic and needed a blood transfusion.  Two days later her breathing got worse and she spiked a fever; more blood work was done, and it was discovered she had a blood infection.  Her doctor called the EB clinic for advice on how to best treat the infection, and the doctor asked to speak to me. She said I could choose to  treat the infection, and Piper might survive, or I could decide to make her comfortable and let her go.  It was an awful decision to have to make.  

I sat there in the NICU rocking my baby girl while trying to decide what to do.  But I never got to make the decision; at 2:50am on a Thursday, while holding my baby in my arms, she looked up at me and smiled, closed her eyes and took her last breath.  I waited too long to decide.  She was 21 days old.  

The autopsy revealed that her epiglottis was severely inflamed and it closed off the opening in the back of the throat that leads to the upper airway.  There was evidence of silent reflux, mucus plugs and swelling in her upper airways.  The NICU doctor didn’t know what any of that meant in terms of being related to EB or if it would have been treatable.  I later learned what it all meant and And the what if’s still haunt me to this day.

The day of Piper’s funeral, her genetic results came back, but I couldn’t bring myself to open them.  I put them in a safe place and tried to move on with my life.  Opening them wouldn’t change anything and I worried the results would make me question every decision I had made in those 3 weeks.  

On Piper's first birthday, I decided to open the results.  Much to my shock,  Piper didn’t have Junctional Hertliz EB like the doctor at the EB clinic suspected.  She didn’t even have Junctional EB.  The results listed her as having generalized severe EB simplex.  I was completely floored.  I had no idea what any of this meant, so I began to research the hell out of EB and specifically, EB simplex.  I couldn’t believe what I was reading.  Most with EB simplex survived.   I joined social media groups and read other people’s experiences.   I researched her specific Keratin 5 mutation. I spent hours on-line looking for any information I could on her mutation, c. 1421c>t, trying to understand what it meant,  and why it caused EB. 

I found another EB family with the same mutation.  The mom has EB, and 3 of her kids were also born with EB.   Sadly she too has an EB angel.  Knowing it wasn’t just Piper, made me feel less alone, but it still sucked. 

 I met with a genetics doctor to help understand how Piper got EB.  She explained how one gets EB simplex, and recommended I be tested.  It was discovered I didn’t have the mutation.  I tracked down her “father” and demanded he be tested.  He didn’t have the mutation either.  The doctor explained that her EB was caused by a spontaneous mutation.  A fluke of nature.   I learned that silent reflux was common in EB and totally treatable.  My baby could have lived.  The more I researched, the more angry I got.  I was so, so angry, why Piper??  Why me??   How could the doctor have been wrong?  Why did I listen to her??  My baby should have lived.  I was more angry at that point, than I had been anytime in the past year.  I felt like I had failed my baby.  I blindly trusted the doctors because that is what we are taught to do.  But the doctors was wrong, so very, very wrong.

The genetics doctor explained the odds of having another baby with EB would be very, very small.  But I didn’t want another baby, I wanted Piper.  I wanted my baby in my arms.

This entire experience has opened my eyes to so much in this world.  It has also caused me to trust doctors a lot less.  I am currently going to nursing school to be a NICU nurse.  I hope I never get an EB baby in my NICU, but if I ever do, I’ll give him or her  the best care I can.   Everything I do is for Piper.  I don’t want her death to be in vain.

 

Thank you to Garrett’s House for allowing me to share my story.  I miss my baby every single day and I wake up everyday to keep her memory alive. 

❤

Lynnea, Piper’s mommy.