Fall

I have to say Fall and winter have never been my favourite times of the year. The cool, rainy weather acts as a deterrent to Kourtney's wound healing, the lack of sunshine takes an emotional toll on all of us school routines become mundane, life just seems to "fall" apart. This fall feels different although we were hit early by the cold virus,  it is one of the first years in Kourtney's history that the grey cloud (called EB) hasn't been lurking over our heads.

Although the "effects" of the disease are more visible than ever  (more blisters on her face, her stooped walk, her mitten hands and wrist contractures), I feel that we have finally have stayed one step ahead of this wretched Monster called EB.

So what is different? For 2 years, I took pictures of back/shoulder/and leg wounds that would not heal. Due to the nature of the pictures I could not show them to anybody,  except for Todd and some of Kourtney's doctors. I am not talking about small scrapes, infact to measure the size of them we would refer to the ones especially on her back as continents on a map. (Africa and SouthAmerica) It was devastating to Todd and myself when the continental drift would take place, in other words the skin would heal, reblister and then just become one big wound. I spent hours looking at the pictures, to see if there were any signs of healing and attempted to come up with the right dressing to heal the wounds. One day Todd finally had to tell me to stop taking pictures because it was depressing for us to see her skin in this shape and he said it.....it's not getting any better.

During the 2 years of being a photographer, not one doctor ever offered me any hope that these chronic wounds would heal nor did any doctor ever suggest something that would work.  I would lay in bed at night consumed with the fact that this monster had robbed my Kourtney of any kind of normal teenage existence. The pain was so excruciating, dressing changes were miserable, morning meltdowns were an everyday occurrence. I tried so hard to accept that this was the way it was going to be. A lot of people wondered how I could work fulltime. Hindsight is always 20/20 but I believe I needed to work full-time to provide an emotional balance for me.My mother's heart broke watching my daughter in such a state, it was very difficult to watch.

Then last "fall" happened. November 1st was the day we took Kourtney into Children's. We were not given a lot of hope that Kourtney was going to survive, and if she did her quality of life would be greatly affected.  The doctors at Children's sent us home with our tails between our legs with no followup care. We were bludgenoned by the news, and after a week of having Kourtney at home, we could no longer keep her "comfortable". Her pain was so out of control, she was too weak to even sit up in her wheelchair and she was loosing weight. I phoned Dr C. at Children's, his response, "take her to Abbotsford" as there was "no room at in the INN" at Children's hospital. This was clearly God's hand leading us there.  The doctors at Abbotsford hospital collaborated as a team to improve Kourtney's quality of life. They told us that Kourtney's recovery would be long and arduous. But the fact they were even talking about "recovery" was the best news we had yet. Even after we were sent home from the hospital we were still not sure if Kourtney would fully recover......

Now we are almost at the 1 year milestone. I haven't had to take any pictures of her skin because the chronic wounds, although scarred, are completely healed. Kourtney has gained 20 pounds, her dressing changes although still extensive are not painful like they used to be and she has not needed a blood transfusion or iron infusion  in 11 months (she was getting these every 2 months for 4 years prior!!) She is able to go out more and socilaizes a lot more then she ever has. She just turned 16 and will be able to drive with an adapted vehicle. This gives us a lot of hope for independance down the the road as she steps into adulthood. Once again the fact that we can even think about her driving is miraculous.

Starting this week Kourtney will be going weekly into Children's Hospital for serial casting of her right foot. Kourtney is having a very difficult time walking as her right foot is contractured. The Hope is that the serial casting will be able to straighten her foot out, increasing her mobility and transferring skills.

And just a little about me.....I have recently taken a part-time position at work. I feel I have been "checked" out for awhile, and I need to be home more with my family. Shaelyn is at such a transitional age and I want to be there for her (12 going on 17). I want to be around to break up the sibling rivalries,  attempt to be the voice of reason in a teenage world where life does not make sense, I want to be around to hear about friendship issues and their days at at school. I still have been working out at the gym, and have taken back my responsibilities in the kitchen (Todd was doing a lot of the cooking). I feel I can maintain a better balance now that the monster seems to be under control. Life is good right now! I feel I can finally breathe!

Summer Finale

Girls Night in Downtown Vancouver

My cousin Melanie's family. Makenna, Ashlyn, Jayden and Carter.

My cousin Melanie.

My mom and sister Gaylene.

Sharon and Todd.

Fishing lessons with my dad and Aunt Esther.

Kelly!

My cousin Susan and daughter Sydney.

Meldon photo boming this picture.

The cousins waiting for the wedding of their cousin! (I think this could be a new meaning for Gangman Style.

The Future Actor!

Our favourite BC family!

My bros wives. Can you tell which ones are the sister in laws?

Our 17th anniversary!.

Two of these farm boys are married to the above two ladies.

A visit to the "historical Lutzer village"

The cousins!

Emerson

Todd's project- back yard deck. AKA "the Stucture"

And so fall begins.

I feel like I need to pinch myself to wake myself up. This summer was one of the best we had yet. Never could I have ever imagined back in November that Kourtney would have been so active. She kept up with all the activities in Sask,(sang 3 times, sat in 34 degree weather just to be with her cousins, numerous shopping trips etc) and finished up the summer by going to the PNE with all her young caregivers. Kelly, Meaghan, Jordyn- what you have given to Kourtney and to our family has far exceeded any expectations. You 3 have respected our family, shown incredible amount of love and grace to Kourtney and have made Shaelyn feel apart of the "inner circle" as well. You will be missed as you pursue your goals of being nurses. You all 3 will make incredible nurses- because you know how to care - unconditionally.

We now welcome Brittany onboard! Looking forward to spending time with you!!!!

Happy Father's Day to Todd

 Last year I blogged about my relationship with my dad. This year, after the events of the last couple of
 weeks I have decided to focus on the dad to our girls.

2 weeks ago our lives yet again took unexpected turn. Our dear, sweet kitty Fresca was killed by a car. Fortunately we were all out of the house when he met his untimely death. We highly expect (due to the timeline) that the driver of the car had some compassion and immediately called the SPCA. Fresca  had been tatooed (with all of our information) on his first visit to the veterinarian, so Todd got the call.
Todd called me at work.  His voice was unrecognizable, and my heart stopped beating when he told me "something bad had happened." As I felt all the blood drain from my face, Todd choked out the details that Fresca had been killed by a car. My stomach instantly went into a knot when I thought about Shaelyn. Todd told me if he told Shaelyn the news at school he would be unsafe to drive home. We both thought that it would be best to get Shaelyn through her day at school before we gave her the news. I thought I would be ok to get through the rest of my workday, but Todd kept sending me texts, of this precious kitty and our girls. As I was getting these pictures, it became painfully obvious that Todd was very emotional about this kitty as well. I have really never seen so much grief in my husband and that's when I knew I needed to be home not only to give Shaelyn the news but to support Todd as well.

My grief almost instantly turned to anger, as I felt it was just another sucker punch to our family. As much as I could soothe my emotions by telling myself "it was just a cat" and "I don't even like cats", it is what Fresca represented to our family that was so suddenly taken away - JOY-

Last fall when Kourtney was sick Shaelyn was really depressed. She quit eating,she hated coming to the hospital to visit, to add insult to injury we had forgotten to pick her up at school on several occasions. She was carrying so much resentment and guilt about her sister being so sick and her being well. We attempted to pacify her grief by taking her shopping, buying a new pet frog and promising an iPhone at Christmas so she would never be left at school again. Her pet frog met his untimely death only a couple of weeks after his arrival and the promise of an iPhone did not soothe her grief. When Todd had kidney stones and stayed in the ER for an entire day Shaelyn came to me in tears wondering if "Dad was going to die as well." I said no, and in only a way Shae can do, through her tears she said, "I think I need a new kitty, because it won't die." (These words replayed so many times in my head when I got the news that Fresca had been killed).

I talked to Todd after his admission in the ER, and his words were, "there would be nothing better for our family than to have a new kitten to hold, watch and play with. What we didn't realize was that Shaelyn had already picked out her kitty from her friend's farm. As soon as we gave her the go ahead, the next thing we knew we were picking up a 4 month old barn cat. We had not discussed gender, type of cat or even litter training. Todd and I both had mixed feelings when we first laid eyes on this long haired, male barn cat, that had already stolen Shaelyn's heart. On our first day of ownership we had just settled Kourtney into bed when Todd and I collapsed into bed. When our heads hit the comforter, it became evident that Fresca had used our bed as a litter box.  That night we attempted to lock him up in the garage but he escaped, clawed up our custom blinds and found him happily licking himself in our dog's bed. Lexi (our dog) was beside herself. We thought we had made the biggest mistake of our lives.

 Todd ended up taking Fresca to the vet in order to get some clairity and advice on what to do with this new creature. After a very expensive vet bill, we understood more about a male cats and what do with a new kitty and within a week Fresca wormed (or shall I say dewormed himself) into our lives.

When Kourtney's pain was out of control, she would hold the kitty and as the pain medication started to work she would fall asleep with the kitty at her side. On many occasions he was the center of attention as we would watch our cat Jenny hiss at her and Lexi play with him like a stuffed animal. He brought us so much JOY during our pain, and we all smiled when he entered the room.

In January, Todd took some time off to help with Kourtney's care and help with the transition to her new school.  He was also emotionally spent from his concern over Kourtney's condition, trips to the hospital after work and many sleepless nights. Todd,  began exercising regularly, made some of his best meals, came up with a new plan for giving Kourtney's meds that allowed him to sleep more and he worked tirelessly in the yard.  But what began as a 4 week leave turned into a full blown "teachers worst nightmare!"  The 4 week leave turned into 10 weeks.  Todd won't let me post the details here but believe me when I tell you it was horrible to watch him have to go through it.  He contemplated changing schools, careers, and moving out of Abbotsford.  I soon noticed he would spend a lot of time outside but nothing would get accomplished.  He spent countless hours researching rocks walls, patio stones and designs for our backyard but was not able to make any decisions. This was not like him!  During all this we were going through the process of hiring our nanny, and the application got denied. It was just another "thing" that we had to go through, and Todd shut down even more.

I saw this all happening, however I didn't know how to steer Todd in the right direction. I knew that sleep deprivation was becoming a big problem. I would encourage him to sleep downstairs, so he wouldn't have to wake up for Kourtney several times a night. Even on his nights off it was obvious that it wasn't just Kourtney that was keeping him awake. I knew my husband was showing signs of depression, but I was unable to help.

When we told Shaelyn the news that Fresca was killed, she cried for days . . . and so did Todd. I have never seen Todd cry so much in our entire 18 years together. At one point even sweet Shae, said I am more worried about dad than I am upset about losing Fresca. I knew that Todd's tears were not just because of losing Fresca but an accumulation of the events of the last 6 months.  Once again I am so thankful for the great family Dr. we have. I was able to text him and ask for an "emergency" appointment for Todd. We got in right away. It was great timing, because if there's one thing our family can do well it is putting on happy faces while we cover up our grief. We have all gotten good, especially Todd, at "sucking it up". At the Dr. visit Todd handed him a list of all the thoughts and feelings that were bouncing around in his head.  Dr. Kornelsen looked at the list for only a few seconds before chuckling.  His eyes had found the one that said, "I'm 43 and my best friend is a cat."  Dr. Kornelsen with a LOL (laugh out loud), said "that statement alone tells me a lot". He went on to discuss how Todd was dealing with depression.  I don't think anyone was surprised by this, we all saw it coming, it just took losing Fresca for Todd to have a complete breakdown.

Shaelyn had a memorial for Fresca. The Vissers , Grandpa/Grandma Kujawa  as well as Fresca's vet (get this Dr. Katz) came for the memorial. Her comment, was, "Why would Fresca ever want to leave such a beautiful yard?"  Her recommendation was to invest in an invisible fence if we ever got another kitty.

Well we now have a new kitty named "Gracie- Jane". She is Fresca's 5 week old sister, and is a clone to Fresca. Shae got to pick her out as well. She actually was already spoken for, however, the owners took sympathy on our family and we got the trump card for this new little creature. She has filled the gap alright, but never can anything/anyone, replace the Joy, that Fresca's short-lived life brought to our family during one of the most painful circumstances we have had to endure.

Once again we are reinventing how to live so that our circumstances don't consume our entire lives. It is not an easy road that we are going down. We feel we have been blindsided on so many occasions, that we almost are in a state of panic when we get an email or the phone rings. We don't want to live in fear of what the next day will bring, however, we don't want to get to comfortable because as soon as we do it just feels that is when we get pounced on.

The one thing I can say about our family is that we are resilient. We have learned to live above our circumstances. At times we have to admit that we aren't OK, we need to lean on people for our support. A breakdown is important, because it serves as a reminder that it is just not Kourtney that is afflicted with EB; our whole family is. When we lost Fresca we shuddered when we thought about Kourtney's prognosis in November. We couldn't beleive the void that losing Fresca left, we couldn't imagine if it had been Kourtney.

So this Father's day, I want to encourage Todd and all the dad's out there, that it is ok to cry, it is ok to breakdown. Never should a man have to take so many sucker punches and try to deal with it. It was a huge turning point for the girls as well as myself to see this different side of Todd. We all felt the impact of his depression and to see their dad breakdown revealed to us that this man is human indeed.

Happy Father's Day Todd! Love ya!

A little bit of this and a little bit of that

There is so much on my mind that I have had a difficult time putting my thoughts into words. The following maybe scattered, so bear with me.

How is Kourtney doing?

Kourtney was wondering when I was going to blog because she was wondering how she was doing (with that sense of humor she is obviously doing well). We hear her giggle more which  means she is bugging Shaelyn or she is making someone grin with her witty sense of humour. She feels loved and respected at school and just recently sang at her school talent show. (She was more nervous about walking up the stairs to walk on stage than actually singing.) Kourtney has some amazing caregivers in her life, as well as some special friends who are committed to her and bring all of us a lot of joy. She is doing very well, (ahem) comparatively speaking.

Not to be a Debbie Downer, but it 's like the pink elephant in the room that you know is there but nobody talks about. Being a teenager with a disfiguring, debilitating disease is very difficult. There, I said it. We still are faced with a 15 year old girl who requires one to one care, sleepless nights, long dressing changes, many meltdowns and resistance to physio. The years of being undernourished and countless infections as well as the course of the disease has left her body with irreversal damage. Sometimes I don't even know where to begin to get Kourtney seen by the right doctors. For 4 years I  took Kourtney faithfully to weekly hospital visits, with little change to Kourtney's condition. But just as Kourtney has anxiety about walking up stairs, I have similar anxiety about going into Children's Hospital. The thought of explaining Kourtney's history to a resident, watching her in excruciating amounts of pain getting bloodwork, attempting to excuse her lack of motivation for not exercising and just the thought of the drive in makes me break out into a cold sweat. There are so many things that I should/need/could be doing for Kourtney that I just get overwhelmed thinking about it. So instead I have taken the stand to let life with EB happen.

To my biggest loser friends . . .

In my new quest to let EB life happen I feel like the dark cloud that had been hovering around my head is gone.  I'm not sure exactly what has given me a new lease on life; the antidepressant that has dulled all my emotions or the fact that I quit trying to control a disease that is not mine.  I did something I never thought I would do . . . I joined a gym!  It may not shock you as many of you know I am an avid runner and biker.  I love exercising outdoors . . . even in the rain.  The thought of a sweaty body beside me on the elliptical isn't my idea of fun.

Although I was active it was becoming obvious to me that I needed to do something different in my exercise routine. *Almost* 3 years ago I turned 40. I did a celebratory "weigh in" on that warm, sunny day in July and much to my despair I hit a new decade of weight as well. It was devastating for me to tip the scales on my 40th birthday. I justified the weight gain on antidepressants and my metabolism slowing down. For the next 2 years I did my best to stop the scale from climbing further into the next decade, with fancy diets, yoga and even stopping my antidepressants. No matter how much I exercised, it was my love for food that stopped me from losing my middle age weight.

One year ago in June I did my 30 day challenge. I exercised everyday for 30 days, and tracked my food intake using my fitness pal. I lost 10 pounds. I kept my weight off, Kourtney got sick, and with that stress I lost another 5lbs. During that time I was encouraged to go on antidepressants. The last time I went on an antidepressant my appetite increased and I was emotionless. I fought going back on them due to unwanted side effects. But the tears wouldn't stop and I came to realize I had to go back on them so I could be strong for Kourtney.

So as Kourtney got better the and the antidepressants kicked in the weight slowly started to settle again around my middle.  My back and shoulders were sore from doing dressings changes in awkward positions and getting Kourtney up and down the stairs, in and out of the van, as well as many nights of sleeping in the parent hospital beds. Circumstances that followed in February and March left me emotionally spent.  I felt so rundown that I had no motivation to exercise.

After spring break Angela and I decided our 40 year old bodies needed some weight training so we decided to join the gym together.  We had a complimentary personal trainer assessment when we signed up for the gym.  Like two school girls joined at the hip Angela and I wanted to have the same routine so we requested to do the assessment together. It became painfully obvious that Angela was very experienced with weights and was a whole lot stronger then I was. The personal trainer was obviously concerned about my lack of experience and gave me a yoga ball and 5 pound weights to work with, meanwhile Angela was pumping iron. This gave me another reason to hate the gym even more.  I am not coordinated, I don't like others watching me when I exercise, and I don't like being new at something.

So instead of of trying to follow the personal trainer's stick figure training program, I started taking the classes offered at the gym.  I found out how out of shape I actually was, and in many instances felt like I was going to throw up.  But then something happened during my "ride" class. It was a twist of fate (actually I thought it was a God thing). The instructor in the ride class said she had an extra spot in her HIT (high instensity training) class. One has to understand that this class is very sought after and it's not very often that a space comes up, so I jumped at the opportunity. My first class was awkward.  I became painfully aware how weak my muscles were and how uncordinated I was. The instructor, Jody, was so encouraging. Somehow I managed to to weasle my way into regular classes, ( I actually wonder if I was that pathetic or if it was my look of desperation) and I have now been doing the HIT classes for a month. It is the first time in years that I have stuck to a regular workout regime and have seen some great results (I am slowly becoming stronger and a bit more cordinated. I started the class being 15 pounds down from my 40th birthday.  Interestingly enough . . . and this is what I want to reinforce to you, my biggest loser friends. My size in clothes did not change with the 15 pound weight loss. (the lulu hoodies were all still the same size), but now that I have changed the way I exercise, my clothes are much looser, especially my shirts.

SO what I am trying to get at to all my friends who are involved with the biggest loser. (It's a work thing).  It is a long, slow battle. Don't be discouraged if you aren't seeing results on the scale, but be proud of yourself that you are taking steps to make yourself healthier. In our automated world we don't know how to be patient. We expect results quickly but what we have to come to terms with is the metabolism of a human body. It's not something that can easily be changed especially at the tender age of 40. Just be patient, and slowly make little changes in your life. Continue to be active, incorporate exercise in your daily routine, and keep track of what you eat. Don't be discouraged and don't give up.

Give A Shout Out to Megan Barron.  Click HERE for her blog.

Megan has the same type of EB as Kourtney. She just graduated from Duke University, and just recently started her own blog. This young lady has been an inspiration to our family. She has received a lot of publicity for her post about "public prayers". Just so you understand what she is saying is not overstated. Kourtney has had this happen on many occasions and I think Megan does a phenomenal job expressing how it makes not only herself feel but everyone else who has been in the "prayer sandwich".

AHHHHA

Yesterday was the day I was expecting life to fall apart again for us. The EB we know doesn't usually give us this much breathing room. As much as  I tell myself "not to worry" it has become embedded into my mind to have a life filled with crisis.
In my 2 recent reading of articles about EB, I have read that death can occur from cardiomyopathies.(translation, heart problems, valves as well as the heart muscle can become large and weak). As many of you may remember, this was a huge area of concern for the doctors in November as Kourtney's heart was as she would described was pounding and beating really fast.  She fainted many times as well would go ashen white.  The cardiologist was concerned that her heart was enlarged from the last echo cardiogram that was done a year prior. One of the doctors wrote cardiac failure on the notes.
Yesterday we had a followup appointment with the cardiologist. The traffic was fine on the way in which always makes a dreaded trip a little more manageable. I was so nervous, but Kourtney reassured me that her heart was fine, and was angry that she had to go to the hospital. Once we arrived I wish we had a tape recorder, because it is always the same explanations about Kourtney's skin,  how much pressure, will I hurt her? and usually "aww poor girl". After 15 years  one would think that would become easier but once again it makes us realize that "normal" to us is abnormal to anyone who comes into contact with Kourtney. The appointment went well, it always helps Kourtney when there is a cute resident that spent a lot of time trying to figure out Kourtney's cardiac history (because in fine Children's hospital form there were no records that the cardiologist saw us in November). Kourtney is always able to express herself so well, I fill in the gaps where needed. Her heart is * normal*. The pounding fast heart rate has been corrected and there has been no damage.
 I had a long talk with the cardiologist about the reasons why cardiomyopathies occur with children/adults with EB. He has studied this as well, so he assured me was that usually it is due to malnutrition and not absorbing the micro nutrients needed for heart function.( so in November Kourtney was well on her way for heart issues). WE saw Dr Cautermanche later he was patting himself on the back for being the superhero and ordering the micro nutrients. He said it took an old plastic surgeon to figure it out. We thanked him over and over again. You would think being a surgeon he wouldn't need so much affirmation, but this guy seems to. (sorry to the men out there but I have to remember that despite the letters behind his name he is still a man).
My mind could not stop thinking about Kourtney handling the conversation with the resident, coupled with a few incidents and conversations I have had with her last week. As much as I want to take credit for this resilient, self confident, bright child, I am going to step out and say "she has raised me well"  She has made me realize what a selfish life I led, prior to her birth. She has taught me that beauty comes from within(and that is not a cliche),face your fears, and pain is a perception. (I had a hang nail, and complained how much it hurt- she said "poor baby"- it didn't hurt so much after that). We feel that Kourtney's life as well quality of life has taken on a new meaning. Yesterday it was made very clear that Kourtney's life was spared,  because God has not accomplished everything through her that needs to be. We don't know what that looks like or how long we will go before another crisis ,but we need to live each day, knowing we are not in control. We live each day knowing we are in the centre of God's will. It is a beautiful place to be.