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!!

1 More Sleep Until Disney

Yesterday  Kourtney, Marijke and I went to children's for one last dressing change. Kourtney only needed a light sedation as her pain has been minimal and basically the site is all healed up. This means that now the torch will be handed back to me. The familiar nurses were sad to see Kourtney go. They loved all the "spectators" that came to see Kourtney's dressing change. I assumed we looked like rednecks coming from the country to the big city. We brought in our coffees.  All we needed was popcorn for the event. However, the nurses loved the company. They did the dressing change without any help from me. They said that usually parents don't come in because they don't have the fortitude to watch a dressing change. It was nice to be a spectator and not have to involve myself unless I was asked. We said our goodbyes, hoping our next time there would only be to visit.

Although it's all over, a whole new chapter of life begins as we look into prosthetics, physio and regular surveillance of her skin for cancer hot spots. As of now we know she is completely free of cancer so we can breathe. We aren't sure what is in store for Kourtney on a personal level. At this point school may be out of the question. We want her to start a vlog (video) of her life and every day accomplishments. I do think it would be a great way of communicating and educating the world on disabilities and EB.

Yesterday after Kourtney's change we took the opportunity to go shopping for last minute Disneyland accessories. It's strange but if I am going to have a panic attack it will be in a mall. Although I hid it well, the worry and fear that came over me caused my heart to race and I had to sit down. I felt worthless, exhausted and defeated. I always seem to talk myself out of it as quickly as it sets in but the cloud lasted longer this time. Although the countdown to Disney is on, Kourtney still needs a blood transfusion. She lost so much blood with her first surgery that her body isn't keeping up with her needs. So now less than 24 hours before we fly Kourtney will receive the gift of life. No stress at all.

The good news is that the suitcases are packed and just awaiting final touches. This Disneyland trip has been a complete act of faith. We didn't put any cancellation insurance on the trip as it was unlikely we would get covered due to Kourtney's surgery. I do think if we had cancelled we would have waited a long time for the surgery with nothing to look forward to.  It would have made Kourtney's recovery much more difficult.

So just to be clear, I am not busting Kourtney out of the country against medical advice. I know I have complete support and trust of the medical team. They have all worked diligently to get Kourtney  ready to go. I have gone through every worst case scenario in my head and this is what makes me worry. Kourtney still has her picc line in. So should she need IV access it will be comforting to know it's there. We have great medical coverage as well so I feel it will be ok.

Kourtney is tough and I believe she will be covered in prayers when we go, right?????

Home.

We came home today to flowers, cards and a very clean house. ( thank you Diella). The love, support and solidarity shown for Kourtney and our family has been amazing. Shae stole my heart as many times she is the forgotten trooper of the bunch. . We are so fortunate to have family and friends that live close thar were available  for her. But what thrilled me most would  be most patents worst nightmare. Shae had a sleepover ( with our permission) , like I said the house was clean when I came home. She even cooked breakfast for her friends ( yes there was 2, and the dishes were cleaned.up) She knew how important it was to me to come home to everything in order. She held it together until we got home, but the tears flowed when she first saw Kourtney. The two girls have worked through their  sibling rivalry and have developed a strong connection. I love that.

So how is Kourtney doing? Her pain has been minimal which is a huge relief after the pain crisis she went through 5 weeks ago. She was able to crack a few jokes about her missing hand today which is alwways a good sign. She has had to restrain me from strssing out about every little thing. But she wants me around, so in my opinion she asks for it. Her and I have an amazing bond and although most of the time we clash I would not ever ever let her go through this  with anyone but me by her side.

As for me the dull ache is gone. November 2017  is when I had to come to terms with taking Kourtney in to see Dr C.  I could not bring myself to make that phone call. My first day back to work after being in Disneyland I could not not shake this feeling of despair I was feeling. My wise friend Marlene started asking the right questions which caused the jailbreak of tears. She held me accountable for making the call to Dr C's office. ( little did she know she would be facing a cancer diagnosis as well- love you Matlene) When I got home the bond Kourtney and I share prevailed, as soon as I returned home. She looked at me and said, " we need to show Dr c my hand". This is how it unfolded, and now that dull ache has disappeared. I hoped it would have been a different outcome but deep down I knew amputation was the only answer.

Secretly I hoped that the tumour board would have had a case study for us to be a part of or would have suggested something different. So about the tumour board......Dr C is a good man however like most surgeons they take pride in the choices they make and the work they do. Dr C likes to pat himself on the back and is not always humble about the work he is done. Which is how a surgeon needs to be. This may come across as arrogance to some people but because we know him and have faith in him we appreciate it. After Kourtney's surgery Dr C said he just met with the tumour board about Kourtney's situation and their recommendation was for Kourtney to have her hand amputated. So he said " I am glad I made the right decision" As my daughter is back in her room post op!! It was humour that only we could appreciate.

But it's over. The cancer is gone, the pungent odour, the pain and the despair it has caused. I could draw parallels to this but I will leave it up to the pastors in my family to do this. We pray and hope that it won't show up for a long time, and Kourtney can start living again.

We will take one day at a time and not live in fear of what the future holds. Nobody knows what tomorrow will bring all we have is today.

Thanks for sharing along in our trial. We know it's the only way we can get through these terrible bleak moments.

 “Praying psalm 30 - (selected verses)
Weeping may last through the night, but joy comes with the morning.

Hear me, Lord, and have mercy on me.  Help me, O Lord.

You have turned my mourning into joyful dancing ... that I might sing your praises to you and not be silent. Oh Lord, my God, I will give you thanks forever!

”