Update

Abbotsford Hospital doesn't have wifi, my 3g doesn't work the greatest as well, so that is why blog posts aren't coming daily. Keep checking, we are attempting to stay ontop of it.

Yesterday morning I woke up and realized that Kourtney and I both had slept for 6 hours without any interruption. I was so relieved until I found Kourtney in a pool of her feed, it had leaked out onto the bed, and of course this meant dressing change. It was one of the better dressing changes because Kourtney was so medicated that I was able to really clean her up well. She sat in the shower for at least an hour, all the dressings slid of easily, and her skin wasn't looking too bad. I thought Kourtney was starting to turn a corner......
But then Kourtney started having incredible pain in her leg and spiked a fever. The fever went down instantly with Tylenol, however the pain was a bit more difficult to stay ontop of. She was started on Iv antibiotics and given Albumin( this is what was so low when she was admitted) to see if this may help more with the wound pain she is having. Lack of Albumin causes swelling in the tissues.

During the late afternoon she perked up nicely, as was her usual happy self, with a will to push through all this. She knows that the only way through this is stay well emotionally and the best way for her is the continued visits from friends and family-that seems to fill her love tank.
I came home for a sleep last night and Todd stayed with her. It was nice to sleep in my own bed, and wake up to Lexi calling for me at 0600.
I have spoken with most of the Kourtney's doctors from Children's. They all feel Kourtney is getting the best care in Abbotsford, and at this point there would be not much more they would be doing for her their then what they are doing locally. We actually think we are getting better care, although it is very difficult not to be under the watchful eye of Dr Davis and Dr Cautermanche. They both have poured a lot of time into Kourtney and know her well.

At this point what we know.....is Kourtney is stable, she is showing signs of improvement however the road to recovery  for her is going to long and slow. I am so happy her pain is under control and we are able to change her feeds to see if they are absorbed better.

Shaelyn is doing a  better. My mom has always had a way of making us "talk". That's why I became a coffee drinker at the tender age of 10, she would pour me a cup and make me tell her about my day..She was able to get Shae talking about the issues( but Shae isnt' drinking coffee yet). She is happy and lighter, more worried about her Grade 6 immunizations then anything else right now.

Staying Put

Kourtney will be staying in Abbotsford for the rest of this week and into next week as well.  Her pain continues to be managed with morphine but she is weary and worried about what the future holds.  We are still waiting for the team meeting at Children's Hospital; Dr. Courtemanche has heard from a few key doctors but is frustrated that some remain silent. They won't be silent for long as Janelle plans to rattle a few cages tomorrow!

Today, however, we are thankful for many things!

• Janelle's mom arrived from Regina
• Lots of friends and family came to visit (as well as a few Abbotsford Heat hockey players)
• Cups and cups and cups of coffee
• Tomato basil soup from Safeway
• Another good dressing change
• Excellent care from the doctors and nurses

Tonight Kourtney will be starting on a different type of feed as the dietitian here has concluded that Kourtney has not been properly absorbing some key nutrients in her current feed. Dr. Courtemanche has also recommended supplemental zinc and selenium.  Who knew that those elements could be contributing to her low albumin levels?

So, we sit and wait.  Sitting and waiting is not so bad though when surrounded by such good friends.

Holding Pattern

Kourtney is still the Abbotsford hospital waiting a bed at Children's as well as a "plan" for her. I am not in huge hurry to go.....as I am surrounded by so many friends, family coworkers. We have had the party room all day, even the members of our local hockey team the Abbotsford Heat, came and got a picture with Kourtney. (our young nurses were a bit excited to have them there as well).

What they are thinking is the "hunch" I have been having for awhile. Kourtney is not absorbing her feeds.This would cause a lot of her problems as well. So right now in our local hospital they have changed her feeds to something that is absorbed better. I do think this will "steer" us in the right path but unfortunately I don't think this will fully solve the issues, and at this point am afraid to speculate what is ahead of us.

My mom came today.....we have made a very nice room for her downstairs, I have a feeling she is going to be here for awhile.

Tired, exhausted, but so relieved that Kourtney is comfortable.

Quick Update

I knew Kourtney wasn't well on Sunday when even Uncle Wonderful couldn't move her off the couch to go to the mall and play the "wheelchair game" they have come to enjoy. She did have a good day and was restless at night.  So. . . I thought maybe her hemoglobin had come up. I ventured to take her for a walk in her wheelchair.

We got about a half a block when she turned pasty white and became nauseous.  Meldon and I took her back to the house, where it took her a while to recover, and she started having major pain.  I deliberated all day what I should do.  My dear friend Angela came over and discussed with Kourtney what would make her feel better.  Kourtney said, "I want to go to the hospital."

Due to a bed crunch at children's hospital, Dr. Courtemanche encouraged us to have her assessed at Abbotsford Hospital. Thanks to some wonderful people I work with, who I can truely say are my friends, we got directly admitted and were assessed quickly by a paediatrician. After they drew some blood work, my suspicions were right, her hemoglobin was OK, however now her albumin is scary low.  What does this mean now???  We are really not sure.  Kourtney will be transferred to Children's after a team meeting where they will come up with the most reasonable options.

It is a relief having her in Abbotsford, especially knowing her pain is so well controlled with the morphine. The dressing change was totally and completely peaceful.

She is in the right place.  I am really happy that I can be in our local hospital for now. 

Clarification

I know we have a lot of people praying for us and reading the updates on Kourtney.  I feel that I need to clarify a few misconceptions that may be circulating in the community.  I want to make it clear that in no way are we keeping Kourtney "comfortable", nor have we "given up" Hope that she will get better.  However, we have taken all of our cards off the table to make sure we are giving Kourtney every chance for her body to get better.

At this point we have withdrawn her from school and have not attempted for her to continue with her virtual school.  Her body is weak; we want her to preserve any energy she has into healing.  Her body is working very hard to keep up so until we know what we are dealing with Todd and I are in agreement that this is a necessity. We do know and fully understand that is it important to keep her emotionally well.  I believe all the visitors we have had as well the company of our caregivers have helped keep her spirits up. I realize soon that her brain will need more stimulation but I know that right now I would just become terribly frustrated if I tried to encourage her to do school work. She has started some physio as well and recognizes how important it is to keep moving.

What we do know is that Kourtney is not yet in "system" failure.  Her kidneys and liver are fine. Her heart is "OK" at this moment, but because the heart is a muscle the more it works the bigger and weaker it gets (opposite from what we would think - not like when we exercise). Kourtney's heart is beating so fast related to the anemia and malnutrition issues that the possibility of damage is a reality.
So what are we doing now?

To preserve our sanity Todd and I decided to switch Kourtney's feeds to the daytime instead of night. Todd (I seem to have the Lutzer gift of sleep) was very sleep deprived as Kourtney's feed alarmed almost every night due to Kourtney rolling on her tube as well a very finicky feeding pump. In addition Kourtney had to have her first round of her daily double at 0500 am. Sometimes it was so difficult for her to walk to the bathroom that she wouldn't make it on time. Keep in mind that there were dressings that got soiled. I think her feeds were running right through her as it seemed to come on so quickly.  This could be why her nutrition is so poor.  So now that Kourtney is home we are doing "intermittent" feeds during the day.  So far so good.  Kourtney said she sleeps better, and now Todd does as well. We are hoping that doing this may help slow down her bowels a bit too and increase absorption of her feed.  So far there have been no emergencies.

We as well have restarted her on an injection called EPO.  We had taken her off because the hematologist believed it wasn't working.  However, since she hasn't been on it she hasn't been making any red blood cells which she always have made in the past. EPO is what Lance Armstrong was using to "blood dope".  It stimulates red blood cell production and kicks them into high gear. We are holding out hope that this will help.

I think Todd looks a little bit like Lance Armstrong!!  

We still have not heard about a team meeting . . . but I do feel that the doctors feel we have to give this time to play out before there are any moves.

Once again, we ask for your prayers.  It is very difficult to have life in a holding pattern, especially around the holiday season. But as I have said before it is way more Peaceful around our house. The break has been much needed.  I have started getting out and enjoyed some Canadian Black FridaySales!!

The lazy rainy days of . . . November

I haven't been out of the house for two whole days;  probably a record for me. This morning was rough as Todd and Shae went off to school and I knew it was going to be just Kourtney and I to face the day. The happy humming of the vaccum as our dear and faithful cleaner Anna was busy making our house spic and span, and visits by very special people made the day less quiet. There is more Peace in our home now then there has been in years. There is no more pushing Kourtney to walk straight, do homework or negotiating for 5 more minutes of sleep. No more outbursts of tears saying she can't do this anymore. No matter how much I encouraged her the more I realized that a regular routine had become way too difficult for her.  I try to wake her up around 8, we have moved all her dressings and supplies to her bedroom. It just seems so much simpler now.  This morning I did most of her morning routine while she was on the bed. We were going to go out for a walk, but the rain didn't let up.  I am realizing that Kourtney's stress level, was affecting our whole family more then we really knew.

There isn't a lot to report from a medical perspective, except Kourtney is swallowing better after her dilation. The radioloigist who did Kourtney's dilation is now on my very small list of doctors who will never touch her or any child with EB ever again. Kourtney has had two dilataions done before and never has she experienced the pain afterwards that was inflicted on her. (She wasn't intubated so it was not the tube that caused it).

I have emailed all the doctors and have requested a meeting to discuss what needs to be done regarding Kourtney's care. The one thing that gives me HOPE right now (call me crazy) is a blog post from August 2009 . . . The Bone Marrow Transplant.

In all honesty I believe it will happen one day but it will happen at Children's Hospital and it will be covered by our health care. I believe there will be a clinical reason to get it done and that reason is her anemia issues that I blogged about a month ago. A bone marrow transplant would replace her tired worn bone marrow with healthy under worked bone marrow.

It seems like the perfect script.....but once again I have no control. Kourtney's is God's and I have to place this in HIS hands. Right now I am enjoying the blanket of tranquility over our family right now and for that I am thankful.

Continue to pray

Kourtney's morning started out with a dizzy spell. The rest of the day was better.  She had more awake time than sleep time and required nothing for pain.  During the dressing change she was quite miserable . . . she hasn't been that miserable in a long time.  I may be a sign that she has a little more energy).

I called for a team meeting with Kourtney's Doctors at Children's. So far I have only heard back from the hematologist. He told me he didn't want to alarm me in some ways a bit of redemption for me. He attempted to explain what he meant, offered me some Hope that all is not lost. He isn't 100 % sure of  his suspicion that Kourtney's bone marrow is completely depressed.  (I don't know if one bullet to the brain is easier than 4 stabs to the heart.) If he had told me this on Friday, would I have felt better?  I'm not sure.  I know I fear the unknown more than the known.  He also reminded me that Kourtney's nutritional status can be causing a lot of these symptoms and agreed there needs to be a team meeting.

Please continue to hold our family in prayer. Little Shae has no appetite, (and for you that know her that is so unusual, she has lost quite a bit of weight).  Todd is busy this week with school, I am thankful he is continuing to work despite what is going on at home. He is my Rock. Most of the times I have Peace. Last night I was able to get out to a movie.  I'm so thankful for my friends Angela and Jeanne who have been there every step of the way on my journey. Thanks to my other friends who have come to visit. Keep them coming!

Kourtney is doing OK emotionally. There are moments that she will come up with some of the funniest things, and then moments later she will be overcome with emotion and fear. However, she knows how awful she has felt, and I do believe that the break from her highly regimented routine is a welcome change, not only for her, but for all of us!!!!

PEACE

Last night I asked one thing to pray for and that was PEACE. We were all able to get some rest tonight. We had many visitors yesterday, and we appreciated it. Anyone please come for a visit. You don't have to bring anything (coffee is nice) or a great Netflix suggestion. Just let us know before you come. Kourtney remains low on energy as well is very scared! She knows how unwell she is. We just want to have her rest. She had a lot of her friends come yesterday including her 18 year old friend Monique (HAAHAA). Today, pray that there will be joy in the day and in our journey! Also pray for wisdom for Todd and I in the weeks to come. We don't want to feel constantly panicked but if we feel Kourtney needs to taken in to hospital again that it will be clear. Yesterday was one of the most difficult days I have had in years, but thanks to amazing, amazing friends you guys helped get our whole family through it!!!

It's like a knife went through my heart!

This will be probably one of the most difficult blogs that I will have to write. A part of me is numb, the other part of my mind is going 100mph and trying to bring Hope to this situation. However, since they could not isolate where the bleed came from the hematologist highly suspects her bone marrow is not working anymore and even the transfusions will only last for 3 weeks at a time. When he told me this it was like a knife went through my heart. He told me like I knew this was a commonly known fact and I should have known this and in some ways that was even more difficult for me to take. Kourtney was in the room sleeping so I was the only one who absorbed this information. We also learned that Kourtney's nutritional status is not good. She is not making use of all the feed we are giving her. Not sure why, but once again it seems like Kourtney's body is so overwhelmed that she is starting to shutdown. At this point it will be "wait and see" how Kourtney handles this transfusion. If she holds on to it then we will have more Hope that this was an acute blip with EB. If she doesn't hold on to it then it will be clear in our minds that we will have many decisions to make regarding her care. This was a blindside for me and not something I was prepared for. Please pray for strength wisdom and a whole lot of HOPE. WE are exhausted, scared, but also feel that although we are walking through the dark valley we know and feel that it is so out of our realm of control. Thanks for your prayers!

Water to wine....

We found out a couple days before the procedure that the anesthesiologist that was going to do Kourtneys procedure was the one who bullied us into the bath that damaged Kourtney's skin 4 years ago! I didn't have enough emotional energy to fight this and decided that all I could do was pray that God would turn water to wine or that God would give Kourtney and myself some sort of grace and love for this obnoxious woman. I prepared Kourtney for this and told her that she hopefully had learned her lesson to listen to this mama bear! On arrival to the hospital kourtney told me she was having bad thoughts and was convinced this dr was going to poison her.... I explained to her that her job is her livelihood and that would not to this! (but then I started having bad thoughts!!) On arrival to surgical daycare our nurse was kind,fastidious, professional, but didnt crack a smile. We went through our regular procedures, no blood pressures, temperature under the arm and yes she leaves her shoes on when she gets weighed, and do you have an attachment for her gtube? Then I asked who was the anesthesiologist, the nurse started into a fit of giggles when she told me it was Dr Lauder (the anesthesiologist that knows Kourtney the best and took Kourtney under her wing after all of this happened) the nurse excused herslef from laughing by saying she just loves Dr Lauder...somehow I knew there was more to that story from the nurses reaction however,due to the professional code ethics I will never know what kind of shakedown went on! All I know that God must have have a sense of humour by taking the murky water and turned into a fine wine. Sometimes it is the smaller miracles (and very funny)that help me get through these very tough times, the best part is that I didn't have to anything but have complete Faith and trust that God will work things out! Still after the procedure there are still no solid answers! Kourtneys hemoglobin dropped again significantly so she was transfused last night! Kourtney will have her heart checked out as well due to her abnormally high heart rate! The weird thing that her skin is not the issue at this point, infact it is better then it ever has been! I am trying to stay positive...... The other day Kourtney had visits from Kim and Kelly.....if you guys are reading this you have been missed more then you know! You gave Kourtney friendship and showered her with so much love! Also Jennifer M thanks for your visit, you are a true friend to Kourtney,a rare jewel!,, Please hold us in your prayers!

Routine

It is November 13th today....the new school year is well underway, and there is no routine. I feel like we have been in a holding pattern for the last month, as Kourtney has struggled with being unwell. I keep hoping and praying that we will settle into a new normal.....and we haven't. I don't do well not being in a routine. Today I had a meltdown.

Most likely it was because  my mom left today (she will be back in 34 days with my dad), its pouring rain,  pitch dark outside at 430, and my jeans are starting to feel tight again, as I have been able to keep up with my exercise routine. I don't know how much I should be pushing Kourtney to be exercising, studying, her body is so weak. She just yawns and yawns and yawns when we do homework with her, and when she stands up she is short of breath and goes so pale. A part of me just wants her to watch TV or netflix. If I push her I feel guilty if I don't push her I feel guilty and ashamed of myself for lack of discipline......but I can't do it all. That is the way I feel right now. Kourtney is so irritable that even the slightest wrong move can send her into orbit. We both know that I know her skin the best.

I also had a long chat with the hematologist today. I am not sure why he thinks that her hemoglobin of 79 is fine, and nothing we should not worry about. He didn't think her anemia was a problem until we went through her history and then it became evident that a hgb was too low for her. Although he is such a nice guy, and has always been receptive to my suggestions, I don't think he fully understand anemia of EB- I find that exhausting. But then I talked with Dr Cautermanche, he had received the report from the Abbotsford Hospital, and acknowledged my concern. He gets EB. He calls himself the plastic surgery paediatrician quarterback and understood my complete frustration ! He rallied the troops and......

Thursday Kourtney will have a scope. Please pray that they will find the source of the bleeding and that it will be simple fix......like changing her medication. For Kourtney to be poked and prodded it just causes so much damage, even the scope has the potential for problems. Then she will be admitted overnight, and be transfused. Please pray that she will be able to hold on to these red blood cells and she will feel better!! She will also see a cardiologist and see if they can get to the bottom of fast heart rate. 

I made the mistake of investigating on the internet....not a good thing. I have to take this all one day at a time. I am exhausted mentally and should have not gone close to the internet.

Please pray for an answer on Thursday.....it is not a great way for her to live.....we all want her well.

I want to say she is better.

I want to tell you that Kourtney is better......but she isn't. She continues to be dizzy, short of breath, and pale. I phoned the hospital where I work to see who the paediatrician on call was, and it was one of my favourites. I took the liberty and called him,he is such a good guy he met us there, and avoided long ER wait.

He checked her heart out right away, because it was galloping. It was fine. They did a hemoglobin....and it has dropped again!!! Frustrating that they didn't figure out what the problem was while we were there.

We will hang out tonight here, and then take her in this week for another transfusion as well as a scope.

Yep I am scared!!

Home

So we got home at 9 pm! Todd and I had to learn to look after Kourtney's line before we left! Never in a million years did I imagine that I would be doing daily heparin flushes, and 1 minute hub scrubs, for Kourtney! Yes the line is still in, she still needs her scope to investigate her bleeding as well keep a close watch on her blood levels!

Coming home is bitter sweet.  I have to be honest! In the hospital I had nothing else to do to tend but to tend to Kourtney.  It made me realize how easy she is to look after (when it is just her and I).  No early morning routines,  I could take my time with the dressings, no housework, no laundry, just her and I watching NETFLIX. Both Kourtney and I were getting antsy and were wanting to come home.

But as soon I walked through the door the overwhelming feeling hit me as Shaelyn's piano didn't get practiced, tears that she doesn't understand her math, the house in the same state of disarray when I left, lots of unpacking from the hospital stay, and the list goes on, figuring out how I was going to catch Kourtney up with her homework . . . and the list goes on.

But last night as I laid beside my snoring husband, just knowing he was close by, made me realize I was indeed Happy to be Home.

Hospital

I really am sorry for not updating you all sooner. However, blogging on my iPad is a bit tough as Kourtney's laptop has been in use with our new addiction . . . "NetFlix".  Kourtney has been in the hospital since November 1st.

Yep, my suspicions were right the other day when I blogged about her not feeling better . . . nobody knows how bad I wanted to see Kourtney doing well after her her transfusion.  I had a week off work which was a relief because then I got a pretty good idea of what was going on with Kourtney. She had missed a couple of days of school prior to the transfusion so I wanted to help her catch up. I started studying good old Chemistry 10 and learned all about radioactive decay and tectonic plates. The more time I spent with Kourtney the more I realized that she was unwell.  She couldn't stay awake when I was going through her Science and it seemed that every bone in her body ached.

On Halloween night I took Shaelyn and her friend out trick or treating.  When I got home Kourtney was whiter then a Halloween ghost and was unable to concentrate on studying for her Science quiz.  I took her to the bathroom that night, and noted her stool to be black. A part of me wanted to pretend that I didn't see it, but its smell and consistency just reassured in my mind there was a bleed going on. Thursday morning she was shaking and continued to be pale. When I saw her in this condition I became nauseated knowing that she was most likely bleeding internally. Todd went to school to arrange for a TOC, and I started  packing supplies for a stay in the hospital.  I phoned Dr. Courtemanche, and he said he would make arrangements for her.

I started second guessing myself when we drove in as Kourtney seemed to settle down somewhat.  Upon arrival at Children's Hospital we were triaged right away as Kourtney's heart rate was 176 bpm, and her colour was fading very quickly. The whirlwind of events that occurred was amazing.   We were seen by a doctor in emergency within 30 minutes (the wait would have been closer to 3 hours). The anesthesiologist was called and after two tries the IV went in the anesthesiologist said, " I am sweating like a pig."  Kourtney, you can really make the guys at the top of the totem pole sweat.

As the blood work results started slowly drifting in my suspicions were confirmed.  Kourtney's hemoglobin dropped significantly since her last transfusion . . . there was a geyser inside somewhere and we had to figure out where . Dr. Courtemanche got us admitted to "his" floor; the floor where surgical patients go but because of what happened years ago, he is pretty darn protective of our little princess. He made it clear  that "no residents" were to see her. What a relief!

Kourtney seemed to stabilize through the night when they started her on a medication through the IV called Pantoloc. Unfortunately, her IV fell out during the night and the next option was to put in a Central Line.  This would avoid the need to poke her over and over and over again for bloodwork, IV's as well as any procedures that need to be done.  We have avoided this over the years because she is such a high risk for infection so the chances of the central line (which goes right into the superior vena cava - the heart vein)getting infected are increased, which could cause another set of problems (that I don't want to think about and have to really give to GOD). However, Dr. Courtemanche and I felt it was absolutely necessary to get to the bottom of this. He felt very uncomfortable sending her home without knowing how approximately one cup of blood got lost in only 9 days post transfusion.

So as she was taken down to interventional radiology to get the PIC line (central line in) we both felt unsettled.  I really didn't understand what was involved. Kourtney senses when I am uneasy, and played off my fear, and broke down when she was going to get up on the table. I told her she didn't have to do this but she would continue to feel crappy and the only way to make her better was to get this line in so we could find out what is making her so unwell.  With that she marched up the table, and continued to be  miserable, that is until Dr. Cassidy walked in. We met him 9 days ago when Kourtney had her last transfusion.  This man with the strong Scottish accent put Kourtney's IV in, was so kind and gentle with Kourtney, as well he is a "wee bit handsome".  Kourtney in her misery didn't see him come in and when I said, "Kourtney look who is here!", Kourtney looked up and smiled. You know that smile when you know she is truly happy . . . or being totally deceitful. Those of you who know her well, you know the look.  Everyone in the room saw the connection. Dr. Cassidy took her little hand and held it talked her through putting the mask on and he held her arm as she was getting her line in. I came in to help with the dressing and thanked him for coming in. He said, "No it is my pleasure. For everything that she goes through she is so pleasant."

Now she has the line in we have learned a lot about what is going on in her body. It is amazing that blood work provides so much information about what is going on. We know her liver and kidneys are OK (Dr. Courtemanche made sure I knew this cause he knows that I was a dialysis nurse forever and knew it would easy my mind).

One possible explanation though came up on her blood test. She had a positive Coombs test which could cause these symptoms, however, she did not have blood in her urine,  just in her stool. She could have had a delayed reaction to the blood transfusion, and there was an antibody in her blood that she was rejecting which could cause hemolysis (or breakdown of red blood cells-causing her hemoglobin to be low)   I know its confusing . . .

But her stool also tested positive for "occult" blood which means there is bleeding higher up. So at this moment we are still waiting for arrangements to be made for a scope of her stomach and her small bowel. While we are here Kourtney and I both feel that a dilatation of her esophagus may be a good thing as she is experiencing a lot of problems swallowing lately (not as bad as before but it is getting worse).

What I find amazing is how Dr. Courtemanche and Dr. Davis have supported us and been here everyday to see how she is doing. The internal specialist is new to the hospital, and we have found him to be agreeable and quite lovely as well. He has respected what I have told him and acknowledges that I know Kourtney's history the best. As long as they listen to Mama, there is no Mama Bear!

The most random thing that occurred was Kourtney's "old" teacher's aide, Tammy, was flying from Calgary to Vancouver the same day and same hour Kourtney was admitted. The Vancouver airport is close to the hospital so Todd was able to pick her up. Kourtney introduced her to the nurses as her Grandma, who then commented on what a young grandma Kourtney had. It was quite amusing. It made us realize how much more we missed her.

We have had many special friends come out to visit and have made some new ones.

We are still here until they can arrange the scope and dilatation together. Although its not the greatest place to be it has eased my mind greatly knowing that she is getting the best care.