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”

27 Dresses

This is not about the movie.

For those following me on Instagram and Facebook you know that illness and injury are a regular part of family life.  Most recently, Kourtney became really sick a few weeks ago.  This has resulted in countless appointments, procedures and hospital stays. Last month Shae came down with gastro in Mexico, had to be hospitalized and we missed our flight home!  Christmas Eve was spent with Kourtney in the ER with post op pain from an esophageal dilatation. Going back to October 2018, Todd was hospitalized after a bike injury and in November he was hospitalized for kidney stones and needed to get them surgically removed. Oddly enough, none of this has hindered our spirits. We keep on making plans and trucking along.

Our next adventure was my nephew Carson’s wedding in Edmonton.  At first Kourtney did not want to go. But after my extended vacation in Mexico with Shaelyn she wanted to join in the fun. I booked our flights and hotel with no cancellation insurance because surely, what could possibly go wrong? (I live with my glass always half full and now and my wallet half empty.)  But alas I have to be thankful that Kourtney is being treated here rather than risking the trip to Edmonton.

On a brighter note there is a comical side to this whole story that I would love to share.

Over these past few years I have been gaining weight. I have tried everything to lose it.  It’s so difficult to be on a diet or “change your lifestyle” when the rest of the family is not on board.  I had lower back pain aggravated by lifting weights and trying to many complicated and frustrating workout regimes.  In January Todd tipped out the scale and decided to eat lower carb meals and exercise more regularly. This made it easier for me to jump on board. Although I have not lost the 30 pounds Todd has, I feel so much better eating healthier and exercising regularly. I do have an end game - Shaelyn’s grad. We are getting some family photos done and I want to be able to look at the picture and not shudder. My jeans were starting to feel looser and my selfies (which is a whole other story) weren’t reflecting a double chin.

Feeling somewhat more confident, the dress hunt began. Prior to Shae’s grad we had a few weddings to go to, including the recently cancelled trip to Edmonton. Dresses are difficult to find. My first trip was to the basement to my own personal stash of dresses, most of them casual sun dresses. My Facebook friends flooded my feed with suggestions; The Bay, Winners, boutiques, and cross border shopping.  But I was working lots and working out.  No time for shopping (can’t believe I said that).  Then I started online shopping and my obsession began.

My first stop online was Shein.  Shae had bought some items on their website.  The clothes were cute and inexpensive so I took my chances and ordered a lot of dresses tops and summer clothes. There were free returns so why not? (but what I didn’t realize was postage wasn’t paid for shipping back to China). What I should have kept in mind that Shein’s clothes are made for tiny Asian women, not a curvy middle aged Canadian woman. Thankfully, I have a tiny  sister I was able to offload most of the dresses on to. I kept a few of them although they are wearable but still tight. This only made me more obsessive about finding the right dress. I knew Aritzia has a good return policy so I went a little crazy in my on line ordering. I probably spent over $ 1,000 dollars (which in Aritzia money is not that much, but still silly for me).  I found one cute pant suit and a cute casual dress, but it would have not been too compatible with the Edmonton weather. All the rest went back. I was determined to find the right one. Then Kourtney got sick.

Kourtney’s little episode did not hinder our plans for Edmonton. Kourtney continued to feel well. The hunt for the perfect dress for Carson and Mandi’s wedding was still on. Over the Easter weekend Aritzia was having a sale and I found the perfect dress. I ordered it on Saturday, hoping I would get it on Tuesday. In my experience Aritzia delivers the next day but with Easter holidays I gave them a day of grace. But as things have gone in the past couple of weeks the dress did not arrive Tuesday. Later that day, Tuesday April 23, our Edmonton plans were terminated as it was recommended that Kourtney start a 2 week course of IV antibiotics. The dress did not arrive until Thursday morning, an hour or so after our plane was supposed to take off. The irony - it fit perfectly, it was reduced by 60% and it would have been perfect for the snowy weekend in Edmonton; bittersweet moment. Sometimes I wonder why I bother planning as much as I do.

There is a word that I believe that follows me every step of the way.  It’s the word Grace. I believe it’s the God given ability that I can still wake up in the morning not fearing what the day weeks, months hold. It allows me to see life through rose coloured lenses knowing God is in control of every situation. I have to be truthful and say it has been a journey to get to this point. Even these past couple weeks, I don’t want to leave Kourtney’s side because I am worried about what the day will bring.  But Grace shows up in so many shapes and sizes.

The medical team we have is incredible and spread throughout different hospitals and health authorities. (Abbotsford doctor, New West Minister doctor and of course Dr. C, Vancouver doctor) They are all available through text or email and willing to help out. That’s where I believe that I see Grace in our village of family and friends and a home I love. I can’t forget to mention Todd and sweet Shaelyn. I feel so, so fortunate to travel this journey with Todd supporting Kourtney and I every step of the way. (Not one question was ever asked about the online purchases).  Shaelyn has become my right hand person and enjoys driving me around and the best little back seat driver there is. (She often forgets who taught her to drive) But oh I how love her. It’s these things that keep my rose coloured glasses from fogging up.

Our life has been an ongoing story of hardship, challenge and disappointment; it is also a story of Hope.  Our life is anything but “picture perfect” but it is full of Purpose and Meaning.  The way we look at it is that our lives have a Purpose, planned by God and we choose to accept it; the good, and the bad.  It gives me the Hope I need to get out of bed in the morning knowing He has our lives in the palm of His hand.

1 Corinthians 10:13 New International Version (NIV)

No temptation has overtaken you except what is common to mankind. And God is faithful; he will not let you be tempted beyond what you can bear. But when you are tempted he will also provide a way out so that you can endure it.

The Ramp

         “Poetic justice” are the words I would describe the events of this last weekend. This particular week had the potential to break me, but instead I had to console myself that A- my life is not normal, B- I am doing well despite how I see myself and C- I love shopping. (OK, I already knew about the shopping but it is a coping mechanism that I have perfected over the years.

         The month of October is EB awareness month. For those who take time to read this, my hope in writing is that even through the worst circumstances we are able to see humour and find joy through our trials.

         Kourtney has had an electric wheelchair since kindergarten. Because I am such a big bad mama bear I have always insisted that Kourtney walk as much as possible. She was only allowed to use her chair for long distances and to get around the school. I never wanted a ramp for our van nor did I ever want to make our home accessible; no wide doors and no stair lifts. The reason I did this was for her to keep some walking muscle memory.  It also allows her to go places that aren’t wheelchair accessible like my parents home of her Uncle Wonderful’s.  Of course the number one reason is so she can transfer onto the rides at Disneyland!

         Over the years though, Kourtney has become increasingly dependant on a wheelchair when going out places so we purchased a manual fold-up wheelchair. Having this gave her the ability to go out with friends in their own cars. With Todd and I both working we would have needed a 3rd vehicle to get Kourtney around.

Although an accessible van is ideal, they are expensive, and puts limits on the number of passengers can fit in the van. This makes it difficult to bring caregivers on family trips and outings. Bringing a caregiver has been our way of life for years now. Not only is the help necessary, it also provides companionship for Kourtney. Over the last few years Kourtney has been requesting a ramp for our van or a new van that is accessible.

         Kourtney presented an insightful case for a wheelchair accessible van. She says that people stare at her more and are more likely to  talk to the people pushing the chair instead of her. Over the past few years she has developed a social anxiety. She doesn’t like to go to places like church or gatherings as she feels she is stared at and ignored. The Handi-dart is unpredictable and taxis are so expensive and unpredictable.  This evidence was enough for Todd and I to look into options for her.

         The best and cheapest way for us to get her electric wheel chair into the van was to buy a 5 foot long metal ramp. It is clumsy and somewhat cumbersome, but for now it works. The electric wheelchair alone takes up a lot of space and the 5 foot long ramp has to fit in the van as well. This means that our 7 passenger van becomes a 4 passenger vehicle. Which brings us to last weekend.

         For the past 15 Thanksgiving long weekends (missing only about 3 years) my family has met in Sylvan Lake, Alberta for a family reunion. Sylvan Lake is a 1100 km drive through the Rocky Mountains from Abbotsford. Over the past 7 years we have taken a caregiver with us. As Kourtney has gotten older she has grown apart from the Saskatchewan group of relatives and was undecided whether she wanted to go. As we have learned to do, we gave Kourtney the option to stay home. After some thought she reluctantly obliged to go and but negotiated that she take her electric wheelchair. This meant we couldn’t take a caregiver with us. This did not sit well with me. With our increase in funding it is rare for me to do a dressing change, mix her medication for the day or even get her up in the morning. For those who know, Kourtney and I have such similar personalities that we usually end up in some kind of disagreement while I am doing her care. My one consolation was that Todd would be there to help out as well. It’s so much easier when we are able to tag team her care. I do not take Todd’s willingness to help for granted. Not many men would be so willing to be a part of the care of their 21 year old daughter.

       A week before our road trip Todd fell off his bike and “did something” to his upper thigh. His pain was intense and he couldn’t put weight on it so we had to call an ambulance to take him to the hospital. X-rays ruled out a broken femur but the pain continued. Todd managed to work and asked the family doctor if he was fit to go on the trip. The doctor reluctantly gave him the go ahead. This prompted a serious one to one talk with Todd and I. I did not want to be Kourtney’s caregiver and his as well. I gave him the option to stay home with Kourtney. By then though, Kourtney’s mind was made up that she was going. This put some added pressure on Todd to go but I also made it clear that I didn’t need two patients. Famous last words.

         Todd drove all the way to Sylvan Lake. Although we broke the trip up into two days, his leg pain intensified. To be honest I found it hard not to do the old eye roll when he was waking me up at night because he was in pain. Lack of sleep is my biggest catalyst for a decline in my mental health. I stayed accountable to my sister Sharon to not allow resentment to take over my compassion. To help me keep perspective this is the man who once stayed over night on a mountain with a broken ankle, walked around for a weekend with a broken fibula, endured kidney stones and corneal infections. Some of the worst pain known to man (other than childbirth and EB).

         Todd still managed to help out with Kourtney, which I was so thankful for. But we knew the 11.5 drive home would have killed him. So we made plans to fly him home on Sunday. This meant that I would have to do all the driving home. With snow in the forecast I was uneasy about driving through the mountains. I felt overwhelmed. The one thing I knew though is that when I need the girls to support me they step it up. Both of them knew how uneasy I was and I started the trip out tired. 

        

         You see, the night before I had to make a 3 hour return trip to Calgary to drop Todd off at the airport.  Todd’s pain was so extreme that he had to use a wheelchair to get around the airport. When he arrived in Abbotsford he called for an ambulance to pick him up as transferring into a vehicle was too much. So, I drove to Calgary and back, had to pack and get Kourtney ready that night. My sleep was troublesome as I envisioned Todd throwing a blood clot or losing circulation to his leg. I kept my phone close by and waited to hear from him. Although nothing sinister happened as I imagined, there were still no answers.  They sent him home after more X-rays and an ultrasound booked for the next day. This frustrated me more as I lay in bed, wide awake.

         After a short sleep I woke up to a fresh layer of snow. Thanks to my brother and brother-in-law they helped me pack our van. Shaelyn and I managed to get the wheelchair in, but I knew that getting it out was going to be tough as we had piled our luggage and bags on top of the ramp. The tears flowed as I anticpated the long drive ahead. I was able to console myself that we could spend the night in Revelstoke or Kamloops if it became too much. But once I got behind the wheel I went into stealth driving mode.

         I love having a GPS on while I drive. I love seeing the km’s go down and try to beat the suggested arrival time. As we drove through sleet and snow, dry roads and wet roads, our destination to Revelstoke was approaching. After 6 hours of driving we needed a pit stop. Kourtney slept for most of that 6 hours and like clockwork, 5 min from Revelstoke she said she had to poop. This was an unusual request as Kourtney rarely needs to even pee on a road trip, never mind #2. But remember the electric wheelchair and ramp?  How the heck would I get it out? I had visions of Shae and standing with Kourtney on the side of the road helping her pee. The best solution was for Kourtney to make a run for it (no pun intended).  As my mind was formulating the exit plan I concluded that Tim Horton’s bathrooms on a holiday = line ups. My best option was the Denny’s. I made Shae go in first to get us a seat. What occurred next was a well-deserved reward for big bad mama bear who made her daughter walk. I helped Kourtney out of the van and said, “RUN!”  Run she did. The bathrooms weren’t close to the entrance but adrenaline took over. She just made it. As Kourtney was serving up her own Grand Slam I was trying to get my land legs back. It turned out I had to do the deed as well. Like all moms we put our needs on the back burner, I had to hold it all in as  I had to find a place to sit Kourtney down. Shae was patiently waiting for us and when it was her turn  she  made sure she didn't  use the stalls we did.

         None of us felt hungry but we also knew that a dump and dash was not a good way to thank Denny’s   for their patronage. We started to giggle and totally enjoyed our time together. The best part was that I forgot about the hour gain from Alberta to BC. That was enough to give me a second wind to push it through to Abbotsford. In 5 more hours we made it home. I usually unpack as soon as I get home but I went straight to bed. Requested that no one talk to me and no one including the animals sleep with me.  Most of my night was spent waking up in fear that I had fallen asleep at the wheel but it sure felt great to have a good rest.

         The rest of the week has been spent going with Todd to doctor’s appointments. Still no answers except that he could rule out fractures of the upper and lower leg, tendon tears, blood clots or compartment syndrome. The pain is getting better but ever so slowly. He’s on and off crutches and looking forward to getting back to work and biking!

         The girls and I giggle about our trip and are so thankful we stayed safe and no accidents inside the van. I will always remember this trip as it was a confirming moment that pushing Kourtney to walk has been a good thing. We did manage to do a lot of shopping and time with our family was very special.

Happy fall y’all.

No signs of cancer since July! We are so excited we are going to Disneyland in November!!!

Phonenix

In less than 24 hours Todd, Kourtney and I will be boarding an airplane to Phoenix for a DEBRA (dystrophic epidermolysis bullosa research association) of America conference. Yes, it will be hot and no, Kourtney can’t handle the heat. However, since it is an EB conference, the planners take the heat intolerance into consideration.  All events will take place in a conference centre; no outdoor activities and a full 3 days of speakers, food and entertainment. Summers in Arizona are a lot like a prairie winter. These places are designed to hold conferences inside. We are thankful for the opportunity as DEBRA Canada has sponsored us to go. 

However, our hearts are heavy.  That's why I am reaching out to anyone reading the blog.  WE need lots of prayer coverage as we just got the results that Kourtney's amputated arm has cancer again.  Just when we had reached the new normal we were aghast to see the familiar signs. It broke my heart as  Kourtney had known about it for a while before she showed it to me. She knows that when she tells me it will make it real and then we have to face what lies ahead.We were so discouraged. We had so many hopes for a summer free of Doctors appointments and gruelling waits for pathology results.  We had also hoped to avoid the incredible fear of seeing Kourtney suffer.  We had so many plans and though we are still going through with them, there is a cloud over our heads that has reappeared.

As always Dr. C’s office is so accommodating. I called Monday and they had an appointment for Kourtney on Wednesday, my birthday. Over the past few years I  plan the day of my birthday. This way I have no expectations and it takes the pressure off of my family.  My plan includes my family, spa appointments and being with friends. This year was no exception, but those plans were put on hold because family always comes first (I have to be careful here because Kourtney feels bad that this appointment took place on my birthday) Kourtney and I face things together and in complete honesty 

being there for Kourtney was a birthday well spent.  I believe that I was born to be a mama bear, a caretaker and to champion people no matter what they are facing.  That’s who I am. (by the way I did have a "me day" after) The icing on the cake was Kourtney asking for the birthday girl to hold her hand when Dr. C decided to remove the growth in his office.

I am beginning to read Dr. C's facial expressions and so far all of the them have been accurate. This time there wasn’t worry on his face.  More of an "aha ".  When he amputated  her hand he cut the margins close. So he called this a  “local" reoccurrence. We did not have to wait for the pathology reports; he knew it was cancer, but the pathology reports would determine the next move. Yesterday we got the results. At this point he cleared the margins but barely so we watch and wait. 

We always leave his office feeling like a load is lifted. He knows so much and has seen it all before. Although we were all too tired to do anything after, my heart was full as the people I love the most were with me. Todd, Shae and Marijke. So thankful for these wonderful people.

As we go to this conference we are praying that Kourtney finds Hope, companionship and a renewed courage to keep going. We don’t want this recent diagnosis to overshadow the good that can come out of this conference.There will be opportunities for Kourtney to interact with other adults with EB  and gather information on the newest treatments for her type of cancer. Todd and I are looking forward to being with Kourtney and hopefully encourage and give HOPE to other children and their families.

Shaelyn made the decision not to come with us (just in case you were wondering). She will be busy with VBS in Mission and looking after the pets. We completely trust her and know she will be ok here. This gives Todd and I the rare opportunity to have 1:1 time with Kourtney. We will give you updates as the conference proceeds.

Ellie

With Kourtneys amputation behind us, the Kujawa’s have reached a new normal again. This seems to occur when Kourtney's feisty spirit returns, and well . . . she is back. Our household has more joy, more laughter and more peace than it ever has. We have learned to appreciate each other more and won’t take each breath we breathe for granted.

This has resonated with me especially in the last month as two of my coworkers have passed away. Two totally different disease processes but both so aggressive and unpredictable. Both of them have left big empty spaces for their spouses and family. As coworkers it has been difficult to process. Work continues, people still have babies and they need nurses to deliver them. But the hallways aren’t the same because we won’t see these two ladies walk them anymore.
Part of processing my grief is to pay tribute to both of these ladies. For Marnie I was able to make a video of her work friends and capture her personality that way.  Ellie, on the other hand, has been more difficult.  I don’t have many pictures of her as she didn’t socialize beyond work. Like most of us 40 plus moms we have been in the thick of raising children.  So the best way I thought I could pay tribute was to write something about her.

My first recollection of Ellie was asking her about her signature she signed it as "P. Cranfield."  She told me the P was for Petranella. I was impressed by her matter of factness. (I grew up with a middle name that I got made fun of and was generally ashamed of my name. But that was Ellie;  matter of fact.

To be honest ,Ellie was not my most favourite person in the beginning. There was no rhyme or reason. Ellie was not out to impress anyone, her black was black and white was white. She worked part time and I was full time. 10 years ago we both had young children and came to work tired and overwhelmed. It wasn’t until we became Facebook friends that I started appreciating her more.

Ellie took out her frustrations on Facebook. But despite her rants, she was one of my most diligent followers of our journey with Kourtney. I could always count on Ellie responding with “I am praying“, sending love or acknowledging my frustration with the system. Our friendship continued to grow but the bond grew more when her eldest daughter started getting sick and was later diagnosed with Lupus.

During that time Ellie began to work full time, that’s when I truly started appreciating Ellie. Ellie was an experienced maternity nurse.  She never complained about her assignment, nor did she ever try to be anything more then she could be. Although I couldn't always follow her charting, one thing about Ellie, she cared for her patients. Ellie received more individual gifts from patients than anyone on our unit.

In this last year her oldest daughter spent months in the hospital. Ellie became her best advocate and dealt with her frustrations on Facebook. She didn’t come to work as a victim.  Her situation made her strive to be a great nurse and one of the most compassionate people I know. I feel so fortunate that this is the Ellie I remember, and have shed many tears over.

As Ellie’s life has gone, not only was her daughter sick, her husband became ill and Ellie was having unusual stomach pains. The stomach pains turned into a hysterectomy and eventually a diagnosis of ovarian cancer.  From what I understood from Dr. Google, the prognosis was poor. However, this was not the diagnosis Ellie was told. The tumour was contained and the doctors and oncologists felt they got it all before it spread. Chemo was only precautionary to kill off the cancer cells. 3 months of chemo and the plan was for Ellie to come back to work in October. As a group of nurses we wanted to help.

During this time I was going through all our problems with Kourtney, but I felt I needed to help Ellie. I started collecting money and was so happy to provide the family with many meals. Ellie and the rest of the family were so appreciative. I stayed in contact with Ellie and was planning on providing the family with more meals for her next round of chemo. But in a sudden twist of events this was not to be.

Exactly one week after Ellie and I communicated I got a frantic phone call from a coworker. Ellie’s cancer had metastasized to her brain. She was admitted in our hospital and had only days to live. 3 of us nurses went down to see her.  We were so confused when we saw Ellie. She was herself, chatty and as her usual self complaining about her watch being on the wrong hand, correcting her husbands pronunciation of medical terms and proud that she still had the wifi password. She mentioned she was planning to come back to work. We all thought that I received the wrong news.

As I was talking to Ellie her husband motioned to me that things were not as it seemed. Through text message I learned that Ellie was not aware of her prognosis. She was clinging on to hope that radiation could kill off the cancer cells. No one actually could confirm how much time she had left so her husband wanted to keep it positive and give Ellie hope.

On Saturday Ellie received so many visits from coworker as the news spread of her admission. Her husband texted me and said the doctor came in and said she had days left. My coworkers still continued to question me as Ellie continued to be herself, still blissfully unaware of her prognosis. That night things changed.

As the inter cranial pressure increased in her brain Ellie had a violent night of projectile vomiting. Sunday morning I got a text from her husband, Ron, that she had become unresponsive. I left my duties at church to get up to the hospital. Although Ron’s request was for family only, I had brought some food and my faithful coworkers made a giant card for Ellie. Ron graciously allowed myself and two other coworkers to come in and say goodbye. I will never forget hearing her children weeping for her, just wanting to hear her voice one more time. It was not to be.
Ellie passed away 30 minutes later, peacefully, with her family at her side. 

Although seeing her family so sad and upset will replay in my memory for a long time, I am grateful I got to see her. I am thankful I got to know the real Ellie rather then just the Facebook Ellie. I will miss her Facebook posts, our private messages but most of all I will miss seeing her at work. She left far too soon at the pinnacle of her career, and as a wife and mom. I miss you Ellie and you will remain in our hearts and minds forever!!