Kourtney (Koko) was born September 20, 1997 in Abbotsford.  At birth she was diagnosed with a rare skin disorder called Epidermolysis Bullosa. She lived a life of pain, however, it did not stop her from achieving dreams and goals. 

She attended school and graduated from Abbotsford Christian in 2015. She began post secondary education but was unable to complete due to skin cancer which resulted in an amputation of her right hand. 

Still, this didn’t slow her down. 

She loved to travel to USA. Favorite destinations included Hawaii, Colorado and Arizona.  But her most favourite destination was to Disneyland. She visited the happiest place on earth 8 times! Kourtney loved to shop. She kept the Amazon drivers busy! She also scoured bakeries from Chilliwack to Vancouver to find the most delectable grilled cheese or croissant.  Kourtney had such a full summer.  She didn’t let her failing health stop her from enjoying every minute of it.

She loved going to church and apologetic conferences.  Her faith in Jesus was what gave her strength through her very difficult life on earth.  

On September 6th Kourtney fell ill and despite every effort made, her body could no longer keep up with the demands of EB.  After a brave 3 week battle at Abbotsford Hospital, Kourtney passed away 5 days after her 25th birthday.

She was predeceased this year by her Grandpa Dave Kujawa and her dog, Lexi. 

She is survived by her parents Todd and Janelle and sister Shae. Maternal grandparents Harold and Charlene Lutzer. Paternal grandma Bev Kujawa. Numerous aunts, uncles and cousins. Special mention to Uncle Wonderful (Meldon Lutzer) and his wife, Charlotte, cousins Nate, and Clarise (Toby) Funk.

She had numerous caregivers and educational assistants who became family to her. Special mention to Tammy Seppela, Kourtney’s preschool teacher who later became her EA and forever friend.

Also a big thank you to her medical team at BCCH , RCH and most recently ARH. We have nothing but grateful for the care she received . Special mention to Dr. Courtemanche, Dr. Burrill and Dr. Chew. 

Although there is so much sorrow we know she is dancing in heaven. 

In Lieu of flowers donations to DEBRA Canada, a charity committed to supporting people and families who are affected by EB. 

Kourtney’ Birth Story

 

If there ever was a birth story that should be shared it’s Kourtney’s. It’s been 23 years and I remember every detail like it was yesterday. We called her our butterfly from day one, as her skin is a fragile as a butterfly wing. There is also a rare phenomenon called the butterfly effect which I believe sums up her 23 years. The butterfly effect is the “phenomenon whereby a minute localized change in a complex system can have large effects elsewhere - even the flutter of butterfly wings.”

If anyone’s life on earth has had a large effect it would be Kourtney’s. To know her is to love her.

As a child I was obsessed with babies. I was the third child of six and have clear memories of looking after my two youngest siblings. In fact looking back I remember being so sad that my mom was not going to have anymore babies that it triggered my first bout of depression.

One memory sticks out the most for me though happened one morning at our daily family breakfast. No matter how many times we rolled our eyes and complained, daily devotions were a priority in our family. This one particular morning my dad asked us to go to our rooms and bring back something we wanted to give to Jesus. We had to place it in the middle of the table using a Lazy Susan as a makeshift alter. My dad placed his wallet, my sister put her curling iron, my brother his ghetto blaster, and I remember clearly bringing in one of my dolls (picture the story of Abraham and Isaac. Read Genesis 22 if you don’t know it).

Todd and I were the ripe old ages of 26 when we got married. I was established in my career as a nurse aTodd was finishing up his PDP to be a teacher. We never really talked a lot about when we would have children. All we knew was I wanted at least 4 and more. We didn’t expect to get pregnant 5 months into our marriage.

Todd graduated from his program in December and after his first day on the job he announced he was going to buy a mountain bike. I told him we should think about buying a house because I was pregnant. In true Todd fashion he was not excited nor was he disappointed . He just asked if he would still would get his bike.

We bought a house, Todd got his bike and we prepared ourselves for the birth of our baby. I had a relatively easy pregnancy and I worked past my due date.

September 20, 1997 my water broke at 4:20 am. I woke my mom up in Saskatchewan and told her I couldn’t get off the toilet because I was peeing. (I was not a maternity nurse at the time). She told me to get to the hospital. I grabbed a baby diaper and got dressed for the hospital.

I had a few contractions that I breathed through on the way to the old MSA hospital. I remember being upset with Todd for coming to a complete stop at a 4 way stop! It was 4:30 on a Saturday morning after all. On arrival to the hospital the ER nurses laughed when I arrived my pants completely soaked. No need for assessment and they brought a wheelchair and whisked me up to maternity.

I was gbs positive but before they started the IV I had to go to the bathroom. I said to Todd I felt like I had to have a huge dump and the nurse overheard me. She ordered me back into bed; I was 8 cm dilated. After a few puffs of Etonox I was fully dilated. The real work began. Pushing.

I pushed for 2 hours. It was a workout for sure. But it wasn’t pleasant for anyone due to the fact that I took castor oil the night before. As Kourtney was starting to crown and Dr. Driedger was getting gowned for delivery I remember giving one big push. Kourtney was born on the bed; no one there to catch her or to protect my perineum. I believe that was the first instinctual way that I protected my fragile skinned baby girl .

As she was placed in my arms all I saw was her “grandpa Harold chin” but the doctors and nurses were immediately concerned. The skin on her right foot was completely sheared off. It was though she was wearing a red slipper. There was loose skin hanging from her hand and a blistered area on her nose. They took her to the NICU right away and Todd went with her as I got stitched up with a very complex 3rd degree tear.

Thankfully the pediatrican on call that day had been at Children’s Hospital and diagnosed her right away with Epidermolysis Bullosa. He said it can range from mild forms to very severe forms and at this point he could not even speculate what kind she had.

In 1997 internet access was limited so we had no clue what we were dealing with. When I first saw Kourtney in the NICU she was getting skin cut out of her mouth to protect her airway. After that they fed her with a bottle that she took easily. She was strong and feisty from day one.

When I finally went back to my room for a rest I could not sleep. As I looked out at Mount Baker from my window I saw my baby on an altar very similar to the makeshift breakfast table alter my dad created years ago. I thought maybe the Entonox was making me hallucinate. It seemed so real. From that moment forward that vision gave me so much peace that my baby belonged to Jesus and I was not in control.

Because it was a weekend and Kourtney was stable there was no rush to get her to Children’s Hospital to see a dermatologist . The NICU was full and because Kourtney did not require any tubes she was able to come to my room for the night. It was the worst night of my life as I learned very quickly how fragile her skin was. My hospital arm bad rubbing against her caused blisters. The tears began to come but Todd was, and always has been, a strong pillar of strength through it all!! He heard a song on a radio that became Kourtneys song.

Take my life and let it be.

Take my feet

Take my hands

Take my eyes

Take my voice.

(every part of her body that was and has been affected by EB).

Now, 23 year later. This has been a lonely difficult road. No matter how hard we tried to prevent it from happening the disease became more debilitating and disfiguring with age. I could get into the details but if you go to the https://www.debracanada.org/ you can read all about it.

Our life with Kourtney has been full of miracles from the first air conditioner we put into our house, the birth of Shaelyn and a lump sum of money we used to purchase our second home. There have been divine appointments that clearly we know the hand of God has been so evident in our lives.

We have blessed beyond measure by Dr. C. He refers to Kourtney’s birthday as “their anniversary” - since he has been her doctor from the time she was born. It’s rare to have a doctor that is so invested and willing to do anything for her.

Most of all it has changed my perspective of who God is. We live in a world where people will not open their hearts to Christianity because it’s going to change their lifestyle or they view people who go to church hypocritical. It could have easily been our story. . However, we could never live our lives without God and the message of Hope. We live moment to moment knowing we don’t have the energy, and the stamina to try and control this awful disease.

23 years later. Our lives have become richer, our perspective changed and an unbelievable love for this beautiful woman I call my daughter!!!

Happy Birthday Koko!!!!

Happy 19th Shaebay

Happy 19th birthday to our sweet Shae.Looking through our past blogs I was surprised to note there wasn’t a story about Shae and her birth. I love telling this one. It’s a long one but so worth it. 

Kourtney’s condition is genetic. That does not mean there is a family history of the disease but that Todd and myself both carry a recessive gene. Alone the gene doesn’t cause any issues, but together, recessive genes can cause numerous genetic disorders including cystic fibrosis and in our case Epidermolysis Bullosa Recessive Dystrophic or RDEB. It’s amazing that one small gene called collagen 7 can cause such significant problems.

The risk for Todd and I to have another child with EB was 1/4. Although the odds were in our favour we were undecided if we would even have more children. This was difficult for me to even comprehend as I always saw myself with a quiver full of children. Did we want to risk having another child with EB?

We saw Kourtney suffering with EB but we noticed she was so social and talked from the time she woke up to the time she went to bed. It was an obvious choice that she needed a sibling. Todd and I both decided that even if her sibling would have EB that Kourtney would not be lonely and isolated. 

Conceiving was never an issue for us. I was on the pill when I got pregnant with Kourtney. So once we decided to pull the goalie (and the back up one) . . . we were expecting!! Dealing with the first semester nausea was difficult enough never mind the bureaucratic  nightmare that ensued when we simply requested chorionic villi sampling (CVS) to determine if our baby would have EB.   Because we were adamant about not aborting our baby, the doctors at BC women’s genetic lab refused to test us. This started a chain of letter writing that clearly stated that it was important for us to be prepared. This process alone took over a month (19 years ago we didn’t even own a computer).

Unfortunately, by the time the approval process went through I was too far along in my pregnancy to have CVS done. This meant I would need an amino done instead which came with more complications. Todd and I felt strongly we needed to have an amino as it would impact where we would deliver our baby, prepare us and have an army of people praying for us.  At 14 weeks we had the amino done.

The doctor doing the amino was so so kind. When we requested an ultrasound pictures of our baby and she was so happy to give them to us. She said most people don’t want them because they are planning to abort based on the results. I don’t remember much more about that procedure except being left on the bed all by myself. Everyone else was  gathered around Todd examining the kidney stone he passed into a coffee filter. That was the easy part however the hardest part was waiting for the results.

2 weeks later I was laying down with Kourtney when my Motorola sized cellphone went off. I will never  forget the nutcracker ring tone as I picked up the phone. It was the genetic doctor. In her broken English she told me the fetus’s DNA matched Kourtney’s to a tee. Our fetus would have the same condition as Kourtney. We had a decision to make. All I remember is feeling the movements of my 16 week old baby and saying, “There is no decision. I will not have an abortion.”  She went on to say she did not agree with my decision and I told her it that it did not matter if she agreed or not.

 I felt such a pit of despair right away but my older sister Gaylene said to me “God is sovereign and doesn’t make mistakes.”  Those words carried us through the pregnancy and to this day resonates with our family when we are faced with difficult challenges.

I continued to have my prenatal appointments at BC women’s hospital. Eventually I put a stop to those when I drove into Vancouver with a full bladder for an ultrasound,  got stuck in traffic, and peed my pants. When I arrived at the ultrasound I was told my bladder was too full and needed to let some out. After the ultrasound I saw the most uncaring and unkind doctor.  I tried to lighten the situation by telling her about my incontinence and she failed to see the humour in it. I decided after that I was not going to have my baby there. I needed a community to support and pray for us, family close by and people who knew us. 

Thankfully we have amazing team in Abbotsford that agreed for me to deliver in Abbotsford hospital. Dr Driedger and Dr Anquist will always be the men wearing superhero capes in Shaelyn's birth story. Children’s hospital was not happy with my decision and was concerned about my fetus well being. Which was a bit ironic considering that at 16 weeks they thought  abortion was the only option. (At one of Kourtney’s appointments Todd was in the bathroom at children’s hospital. He overheard our dermatologist speaking with a resident telling them that she did not agree with our decisionnot to abort. It made him physically ill and was not able to share their conversation with me until a few years later.) It was a satisfying moment when I told the uncaring and unkind OBGYN that I wasn’t delivering at BC women’s.

Kourtney’s fun little personality kept me going throughout the rest of the pregnancy.  We had so many people praying for us we felt so carried throughout it all. Even a well meaning couple encouraged me to take one of their products that would change the DNA code of my baby. Thankfully I didn’t as my mom said, “ If your baby comes out healed we want to give the glory to Jesus not to the company.” 

There were some sweet moments during my pregnancy. During one of Kourtney’s dressing changes we read a bible story to her about a man who was healed of Leprosy. His name was Naimen. Kourtney was so interested in the story as we related it to her skin condition. Todd and I decided if we were to have a boy we would name him Naimen. This in no means meant we were expecting God to completely heal our baby but somehow it gave us peace. We were realistic and knew the amino carried a 100% accuracy rate. We were prepared and ready to receive another glorious gift of a child with EB into our family.

At 38 weeks I was to be induced. March 8 the was my scheduled induction date. But there was no room at MSA inn that day. On March 9 th I was doing Kourtney’s dressings and got a call to come in. Todd came home from work and we went in together. Dr Cindy Shaw was the Doctor on. She broke my water and gulped when she saw meconium in the amniotic fluid. This was an issue because all babies with meconium were suctioned before they took their first breath so they wouldn’t breathe in the meconium. Because EB affects the mucous membranes, suctioning her mouth could cause blisters in turn causing airway obstruction.Yikes. But while I was waiting for my labour to kick in, two men came into our room to this day I still am not sure who they are (they were actual people but I never met them). They said they heard someone needed prayer and they came in and prayed for all of us and prayed for a miracle. 

I got started on oxytocin and my labour progressed quickly. I went from 2- 10 cm in 10 minutes and started pushing. Dr Shaw was calm and started singing a song on the radio. I wanted to scream at her (well I actually did). My irritation worked in my favour as I pushed Shaelyn out in about 10 minutes. The thick meconium was suctioned and she came out screaming. Todd was the first one to see it was a “Shaelyn”. The  cord was cut, she was taken to the warmer, I was almost hanging out of my bed trying to take a closer look. No signs of EB! The paediatrician said  after his examination, "You must believe in God, there are no signs of EB." There was nothing but tears of joy and praising God  in the moments that followed. I was a bit more sceptical as I looked her over with my trained EB eye. It took many days before I was totally convinced.  19 years later I am still in awe of her and the gift that Shaelyn is not just to our family but to so many people (Shaelyn's name means gift).

We did follow up DNA tests and they were compared to the amino results and they were different. She is documented as a medical miracle. We decided that we would handle the dermatologist, the ob gyn and the genetic doctor with grace. We wrote letters to them with a picture of our very healthy baby girl. It was a beautiful time in our lives. God is always with us but revealed his power to us in a way that not many people have experienced. Winning a lottery would have never felt that good. 

Although we have never experienced the big miracle for Kourtney there have been many amazing events along the way to makes us feel God's presence  in her life. It doesn’t always help with heartache we feel when she is going through a rough time. But knowing God intervened in such a miraculous way reassures us that God is sovereign and doesn’t make mistakes. 

Shaelyn’s birth story is one many will never forget. She has blessed us in so many ways. We are looking forward to see what this year of being an adult brings for her!

We love you Shae! Happy 19th!

  

Fearfully and wonderfully made.

It's anti bullying day. There is a song that keeps going through my head that I sang in elementary school and is still be sung today especially on remembrance day but I see it as fitting on Anti- bullying day. 

"Let there be peace on earth, and let it begin with me."

What I am about to share is not about my daughter, Kourtney, being bullied but about the mixed message that are being relayed to our society about acceptance.  We support 0 tolerance for bullying, rainbows on doors of classrooms and on city sidewalks symbolizing acceptance and inclusion, to love and accept everyone based on race, colour, gender, etc. The message of tolerance is clear. So why is it that our gender (not our sex) is a choice and important to how we identify? My concern is that we are encouraging transformations that require such drastic measures with little or no guarantee that this will bring self worth and acceptance. I want to share where I believe one can find their true identity. Ultimately the only way you can love and accept others and have "peace on earth"

Kourtney suffers from a rare genetic disease called Epidermolysis Bullosa https://en.wikipedia.org/wiki/Epidermolysis_bullosa_dystrophica

Kourtney is in a wheelchair and needs help with every aspect of daily living including bathing, eating, toileting etc.  This summer my daughters  and a caregiver went to the PNE ( a fair in Vancouver BC). They were grateful I was not there, as the mama bear would have created a ruckus.

Kourtney is limited in her activities and she lives for amusement park rides. She is also restricted in foods she can eat so melt in your mouth cotton candy is a fair favourite.  It was a very hot day.  Kourtney wears 4  layers of dressing to protect her fragile skin which makes her prone to dehydration.  On very warm days she always has a Gatorade bottle handy that is syringed out and put into her g-tube to keep her electrolytes balanced. Naturally she has to use the bathroom more often. 

As Kourtney and company got to the bathroom they were confused by line of apparently able-bodied people who stood in line to use the disabled washroom. Surely they were in the wrong lineup. Not wanting to lose their place Shaelyn (Kourtney’s sister)  checked the sign. Yes, it was disabled washroom alright, but also a transgender washroom.  All the regular bathrooms in this particular area could only be accessed by stairs. It was a very long wait and during that time one transgender said "**** it" and went to the bathroom of their choice because they didn’t want to wait in the lineup. In that moment their time was more important than their identity" I could go on about the injustice of a disabled bathroom being doubled as transgender but that’s not where I want to spend my energy.  Unfortunately, Kourtney did not have a "choice" but to wait for the disabled bathroom.

I am  sure I would have been locked up if I told Kourtney she could chose to have EB.  We could never ignore that her DNA contained a small gene that made her skin blister.  We always told her she had EB but EB didn’t need to control her.  Even with the disease process taking over her mobility, her nutrition, her sight and so many physical deformities, Kourtney didn’t fall  victim to her EB, because EB didn’t define her.  Her identity was not a part of her DNA. Despite her circumstances she is one of the most confident self-assured people you will ever meet. 

Kourtney has endured 10 plastic surgeries and an amputation to open her fingers widen her mouth and remove cancer. She has had numerous skin grafts taken from her thighs and abdomen. These surgeries were only performed to maintain hand function, open her mouth wider and remove skin cancer. Even with Kourtney having a high pain tolerance the surgeries were brutal and not without complications. I am not sure why anyone would want to take a perfectly functioning body and put it through unnecessary surgery. I also realize that there are hormone treatments one can go through and or a combination of both (being peri menopausal I understand hormone changes and would never wish that on anyone).  To sum it up changing your gender involves pain and suffering. It does not come with a lifetime warranty of self worth and acceptance. 

Despite the numerous hand surgeries Kourtney’s fingers still formed  into mittens. To this day if I knew that the end game would have been the same I would have never had signed consents to put her through all those surgeries. 22 years ago there was not a lot of information on RDEB so I only knew what we were being told by our doctor (who has been Kourtney’s biggest advocate).  Even if there was a cure for EB today, Kourtney would still have all the physical traits of EB, the scars, the clubbed hands, the alopecia.  Likewise, even with surgery and hormone changes there will always be signs and scars of the "transformation" surgery. That’s why I believe there needs to be more to this in how you identify.

The only way I have been ever able to rationalize Kourtney’s genetic disorder is believing in the words of Psalms 139: 13-14

For you created my inmost being;

    you knit me together in my mother’s womb. 

I praise you because I am fearfully and wonderfully made;

    your works are wonderful.

Believing that we are made in God's image has been so meaningful to me as  I raised Kourtney. I  have had these verses on speed dial through all the challenges. If I believed that our identity was in our human nature I would have never made it through. Identifying with Christ (Galatians 2:20) has been what has been instilled into Kourtney’s heart and has turned her into the person she is today. If only people would know that  we as humans are not strong enough to bear the weight of their own struggles. God wants us to rely on his word and the promises he has given. I believe if our identity is with Christ that it makes us more accepting of ourselves and in turn more accepting of others."If we want peace on this earth it has to begin with us"

Koko & Shae

As 2020 is upon us I have been doing a lot of praying,pondering and planning.  I will be turning 50 this year and have been thinking of the best way to bring in this new decade of life. I thought about creating an Instagram account about turning 50 but decided that I am not that interesting. Being a shift worker, coupled with the unpredictable behaviour of Kourtney’s health, makes it difficult for me to committment to many extracurricular activities. In my heart I have felt unsettled in my current stage of life.

Since November Kourtney has had a ton of pain in her knees. Sadly, her pain doctor retired in the summer. We quickly realized that there is not a lot of expertise or comfortability for medical staff in prescribing pain medication. So we went through a vicious cycle of controlling her pain that resulted in Kourtney sleeping through most of the day. I spent many of those days sitting with her while she slept. Every year I make Christmas gifts. To pass the time I decided to get crafty.  I went into full pom pom and macrame mode. It was so satisfying!! Then my sister, Sharon, inspired me to make a natural spray to add to the gifts. I knew it was a hit when Shae and her boyfriend, Ethan, swiped it for their cars.

Seeing Kourtney in such a state has been depressing to say the least. Of course our biggest fear was that cancer was going to be found again.  I could feel the “cloud” forming over my head and I was going down with her. Both Kourtney and I needed inspiration to get through this. On December 26th I had to take time off work due to a neck injury. Needless to say Kourtney and I became a fun pair over the holiday season. My thoughts were consumed with how to make Kourtney feel better. We tried antibiotics however after just one dose she had a reaction that caused even more blistering on her already broken down skin.

The pain in her knees was so bad that even the pressure of cream or coconut oil caused her to shudder with pain. Although the coconut oil is healing and feels good on Kourtney’s skin she hates the smell. It's a known fact that pain heightens one’s sense of smell so it’s extra important that scents are “Kourtney approved” before putting them on her. I went into mixing mode and made a cocunut based spray that was soothing for Kourtney but also was pleasing to her senses. It smelled so good I started using it on my hands as a soothing spray. Little did we know that it would become a kickstart to this new business venture.

After the holidays we went and saw Dr. C in Vancouver. We showed him her knees and he wasn’t too worried about them!  He creatively adjusted her pain medication which now seems to be working. As I redressed Kourtney’s knees I used the “KOKOnut” spray on her knees. We didn’t realize it but we drew a crowd into the room. People were saying how good it smelled in the room. (Keep in mind that we just removed some dressings that don’t always smell so good.)  Everyone from doctors occupational therapists and clerical staff were wanting me to bring some in for our next visit!!!! Kourtney and I both acknowledged that perhaps there may potential, not just for business opportunity, but to help other EB families as well. 

So let me introduce you to KOKO&SHAE!!! “making scents of suffering”