November Update

I haven't emailed since the start of Kourtney's school. When Kourtney first started school in September I noticed that her blisters were looking infected but this time they looked different and Kourtney was in a lot of pain. We had to put her on Tylenol with codeine just so she could walk because her knees were blistered and infected looking. The skin swabs came back as strep infection and she was treated accordingly. She even sang and danced at our church homecoming despite being in an enormous amount of pain. (check out the attachement) . The next few weeks we struggled with esophagus an eye problems. The nurses were doing great with Kourtney at school and I was enjoying the freedom of having Kourtney out of the house and in a safe environment.

We then had our annual family reunion in Alberta. I noticed that Kourtney was starting to look infected again and we had to take her to the hospital in Innisfail (where Sharon and Blair live) to get more antibiotics. This time around Kourtney became very very ill. She could hardly move out of bed and she was very lethargic. I took her to 2 Dr's in Abbotsford and they didn't know what to do. ( At this time the teachers were on strike. Todd wasn't getting paid so I worked full time, as we were unsure how long the strike was going to last. It ended up lasting 11 days. I ended up taking Kourtney to Children's Hospital. The dermatologist and the nurse spent 2 hours with us. I gave them a list of my concerns and walked away feeling like I was beginning to make resolutions. One of things that came out of our meeting was that Kourtney's strep infections were not being cleared up because one of her caregivers was colonizing strep in their throats and reinfecting Kourtney. (Step A in the throat usually has no symptoms but on the skin can become deadly as Strep A is what causes flesh eating
disease). So we had to swab everyone who walks into the house. It ended up that it was Todd and Shaelyn who were the carriers. Everyone thought for sure it was me bringing it home from the dialysis unit. I was pretty convinced it wasn't me because I do a lot of swabs at the
unit and I never see strep A. Also we wash our hands a lot at work and we wear gloves.

So started the long treatment of antibiotics, 6 weeks for Kourtney and 2 weeks for Shaelyn and Todd. Todd and Shaelyn are both cleared. Shaelyn was a little trooper in faithfully taking the antibiotics. She swallowed capsules and was so happy taking them because she knew it would help Kourtney. Kourtney will be done her 6 week course in a couple of days. The antibiotic seems to have warded off colds this last 6 weeks as she hasn't had any. No esophagus problems and no eye problems. Last Saturday I did some reflecting and cautiously said that things were starting to feel a bit normal. Normal for me is sleeping through the night sending Kourtney to school daily and me having a little bit of time to myself. She had a fall at school during that
time and had to get 2 stitches in her head. That even felt normal. BUT THEN..............

Last Monday I received a phone call that Kourtney had fallen at school again. I went up to the school. Kourtney got knocked down by a boy and fell onto her bottom blistering her bum and back of her legs . I have never have seen her bum that badly blistered. The pain she had was incredible. She could hardly move her bum was sore. The kicker of it all was that there was no adult present with her when she fell and her friends had to look for the nurse. I find this very difficult to take as Kourtney has never been allowed to walk in a hallway by herself and why wasn't the nurse present? The next blow came when all 3 nurses that are trained to look after Kourtney were sick or unavailable for the whole week and I had to look after her with no help for the full week. I had to give up shifts at work , couldn't take Kourtney anywhere so I was stuck at home for the whole week. Todd ended taking Friday off to give me a hand as Shaelyn ended up with an ear infection and needed more antibiotics. As I was home all week I began thinking that I really should start getting paid when the nursing hours are not being used up and when I do the training of the nurses (which is ongoing). This has been met with a lot of resistance so far but I am willing to start fighting for being compensated when my child isn't getting the care she has been approved for and we have to give up shifts or take days off when she can't be in school or have the necessary help at home.

I guess what it boils down to is we need some prayer as we fell very frustrated once again. Kourtney's skin was looking good with the antibiotics but now because of the pain and her being home all week has really broken down again.

We also have to decide if we should keep the same nurse with Kourtney (she has two nurses and the one that Kourtney had this last fall with I have had red flags about all along while the other one has been awesome and professional).

Also, in my heart I felt that I need to homeschool Kourtney. I just feel that I need to have her home with me at least a year and get things back on track. I think this has been reinforced since her fall which was in my mind pure negligence and not just "one of those things". I realize that the socialization part is very important but it would prevent having weeks like this where I don't have a structure for her at home and ends up being frustrating for Shaelyn Kourtney and myself.

Pray for wisdom.


Some things that have been positive is that Kourtney has started piano lessons. Despite the contractures and webbing in her fingers she has managed to use a key board and has done quite well (music comes from the soul). Kourtney has blossomed in her writing and printing skills over the last 3 months. She has improved greatly in these areas. Also Kourtney and I will be attending a skin disorder camp in Philidelphia. I will go as a volunteer, we have been accepted and looking forward to going. Also we will start taking Kourtney to a physchologist that deals with chronic pain management. I am looking forward to getting some new ideas for her. Also Kourtney will be having surgery December 19th.

Anyways I know that you will keep us in your prayers. The stress has really taken its toll on everyone. We are ready for some relief soon.

Thanks for being interested!!!! Janelle

Who Am I?

KOURTNEY CHARLENE KUJAWA

I can do all things through Christ who strengthens me.
Phillipians 4:13

Recessive Dystrophic Epidermolysis Bullosa(EB) is a genetic skin disorder. There are 4 types of EB, ranging from mild to very severe. Kourtney's type is severe.

EB is characterized by blistering of the skin with only a slight amount of friction. Kourtney's EB also affects her mucous membranes such as the eyes (cornea), the mouth, esophagus and rectal area.

Kourtney's blisters are significant in the fact that her blisters cause scarring. Repetitive blistering and scarring of affected areas cause contractures of the hands, webbing of the toes and strictures (or narrowings) in her mouth and esophagus.

How does it affect Kourtney:

• Kourtney has dressings on all her extremities to protect her skin. The bandages are changed twice a day, taking 1 hour each time

• Kourtney's hands have developed contractures and her fingers have become fused together. She will require plastic surgery for the rest of her life to maintain proper hand function. The plastic surgery requires skin grafts from Kourtney's thigh or abdomen and placing them onto Kourtney's hands. Surgery is painful and intense.
• Kourtney has had a difficult time eating due to being tongue tied, and having a small mouth. People with EB have very high nutritional needs as it takes many calories to heal. In the past many children with EB died because of malnutrition. At 2 1/2 Kourtney ended up getting a feeding tube placed into her to help meet her daily calorie requirements. Kourtney can eat small amounts of food by mouth. We call her our social eater (chocolate is her favourite).
• Kourtney's esophagus is also prone to blistering. Sometimes she cannot even swallow her own saliva. Kourtney's airway is not challenged but swallowing is painful and uncomfortable.
• Kourtney's corneas have also had blisters on them. The cornea refracts the light coming into the eye, so when there are blisters on her cornea, her eyes become very sensitive to light. The pain is excruciating. We have been able to relieve the eye problems by using many lubricants in her eyes at night.
• Kourtney uses an electric wheel chair for long distances because she gets blisters on her feet in addition to lacking the energy to walk long distances.

What Kourtney can do:

• She can walk short distances and likes to run and play soccer with a beach ball or play volley ball with a balloon
• Loves to swim, the chlorine does not bother her; neither does the sun
• Can sing and dance; has been helping out with the worship band at church for 2 years
• Great sense of humour and ability to converse with adults
• Very intellectual and bright
• Has learned to knit
• Loves to do crafts
• Plays computer games and game boy

Despite the constant challenges, Kourtney remains a very positive and happy child.

Back to School

Just thought I should send a update since school is almost upon us. Kourtney has had a great summer. We were away a full 3 weeks. It was a great time spent with family. Once again we felt the providential hand of God when Kourtney got sick in Regina. Kourtney started complaining of sore legs and pain around one of her open blister sites. This was very unusual for her to complain so much about a blister. I was pretty sure the area looked more red than usual ones so I thought she was getting an infection. Kourtney then started developing fevers. We let her rest and hoped she would start showing signs of a cold or gastroenteritis or something that would show it was more viral than bacterial.

We were having "the last supper" at Gaylene's house before we were to leave back to BC. Kourtney became more and more lethargic and hot over the course of the evening. We decided we didn't want to travel the next day before getting her checked out so I phoned a pediatricain we had taken Kourtney to when she was 6 months old. I didn't think that he would remember us after 7 years but I thought I would give it a shot. Not even 10 minutes later I got a call from him. He remembered us and advised us to come to the hospital. The wait in the hospital would have been 4-5 hours but we waited 10 minutes and were seen by the pediatrican right away. The doctor ordered antibiotics for her and we once again were reminded how God's hand has been so evident in our lives. We traveled to Calgary the next day and Kourtney recovered well. Why didn't the prayers work for the Rough Riders that night??

The rest of the summer went well. Trips to fairs, walks around the neighborhood, and daily swims in our little pool. It was a nice relaxing summer for the girls.

We had one trip to Children's to see the plastic surgeon. We were delighted to hear there is a new cream out that helps heal sores. Our plastic surgeon is not a band wagon person so we know that this would be something that has been well researched. Now it is just a matter of getting funding for it. For you that are interested the cream is call REGRANEX. You can google it if you are interested. It is used for diabetic foot ulcers. Kourtney will be having surgery on both hands and her mouth all at the same time. Probably in November sometime.

Oh yeah, it was very nice to tell the Doctors that she has had no more issues with her esophagus and has been eating quite a bit, for Kourtney. She was started on a medication called Losec and that seems to have helped heal things up.

We are asking for prayer as school starts. I am anxious about all the changes as far as nurses go. It will be all new to everybody and I am praying for understanding from the teacher and principal as we try to work all the wrinkles out. I will be going to the school with Kourtney for the first week to establish a routine with her. There will be 3 nurses looking after her so, I want to make sure everyone is on the same page and the care Kourtney receives is consistent from day to day.

Also next weekend Kourtney will be singing in a children's band (4 other children) at our outdoor church fall kick off. They are expecting at least 3,000 people at it. Kourtney said she is a little freaked out but very excited about it. Please pray for protection before the performance and during it as they are doing a lot of jumping around.

Shaelyn is starting preschool 3 mornings a week. I still can't comprehend what my day will be like without a child attached to me. I think I might be lonely.....for the first few times, I am sure I will get over it rather quickly.

We are also going to a family reunion in Sylvan Lake in October. We are flying there and are looking forward to seeing our family all together.

Thanks for your prayers.

Janelle

July 1 Update (Whew!!!)

The school year has come to an end for Todd and Kourtney. Shaelyn and I feel a little off schedule now that we have the two of them home. I guess they will have to learn to adapt to our routine.

This last month has been extremely difficult on Kourtney. The warmer days have brought much discomfort at school and her skin has broken down like I have never seen it before. The frustration for me has been the fact that it is happening at school and not at home. It isn't that she is being bumped either, but the fact she is scratching herself nonstop.

I have really had to reevaluate the whole school thing as this has been such a tough year for Kourtney and our stress level has been huge. She missed 35 days of school and was brought late every morning and picked up early every day. This seemed to help a lot as far as her not getting run down but I found myself getting extremely tired not having a good break from her for the day and also trying to keep up with her studies. Another thing was having a teacher's assistant that was up at the school who was assigned to Kourtney, yet Kourtney was not there for a good part of the year. It was mentioned that the teacher's assistant could come to our home to help with her studies but I would have to be home during that time. I knew this would not be good as Kourtney will do anything to make sure I am involved with her when someone else is over, plus there are days that grocery shopping or going for walks gives me a mental health break.

We had asked for a nurse to go to school with Kourtney but we were turned down as the committee did not feel that Kourtney required a nurse. I felt adamant that we needed a change for Kourtney this next year as I was completely exhausted and could not go on with the same system. I honestly felt that keeping her home might be an option because I felt I would like to keep her environment stable. But on the other hand the social interaction was necessary for Kourtney and the break mandatory for myself.

As June was drawing close to an end I noticed Kourtney becoming more and more frustrated with her teacher's assistant. I put myself into Kourtney's shoes and realized how agitated I would be if I had someone like her following me around everyday. The more I prayed, the more signs I received that we needed a change.

In my many conversations with my mom she finally told me that I needed to call Carol Clark (she looks after special need children in the district). I said I had tried but she hadn’t returned my call (after a week). My mom told me she would take it to the Lord . . . sure enough an hour later Carol Clark called and apologized for not calling back (that is what I call a heavenly nudge). She agreed we needed a change and she absolutely agreed that Kourtney needed a nurse at school with her. So we arranged for a meeting. I called our nursing supervisor to invite her to the meeting for the next week. She couldn't make it but she said that the next day she was presenting cases to the committee that approves nursing support for special needs children. She said that it was a long shot but she would try for approval again.

Having a nurse at school with Kourtney would allow me more freedom. If Kourtney was ever sick the nurse could come home and be with Kourtney and I wouldn't have to be there. Plus we wanted to put Kourtney in a twice weekly swimming program but I would be fully responsible for the dressings after swimming, and that would just burn me out, plus Shaelyn loses out on time with me also.

The nursing supervisor called me the next day. FULL TIME NURSE FOR KOURTNEY AT SCHOOL!!!!

I cried tears of relief and joy and the summer months became much more relaxing for me. Kourtney was ecstatic with the news of the change and the fact she will be in a swimming program.

Persistence in prayer and listening to that still small voice is what this is all about. I am not sure why sometimes life's little lessons are more difficult for some to learn than others but I have such a thankful heart and very thankful for the small miracles. Still waiting for the big one . . . I still pray everyday for Kourtney's complete and total healing.

Thanks for all your prayers. We are headed to Alberta, Saskatchewan and than to Penticton (our annual summer vacation). We are looking forward to reuniting with friends and family and just being together with our little family.

Love with a sigh of relief,

Janelle

May 1 Update

Hi just a quick note to let you know that we have had a great week with Kourtney. She seems to have the sparkle back in her eyes. It is only through your prayers that we get over the humps.

I also have to made a conscious effort to be thankful in every situation. I am so thankful that Kourtney has had 2 full weeks at school without any major concerns. We have even walked to school everyday (Kourtney in her wheelchair). We also sang this weekend in our church sunday school program. I am so thankful for the opportunity to do this as a family. We were also at mom and dad Kujawa's tonight celebrating birthdays and Mothers Day. What a joy to see cousins playing together, giggling and enjoying being together.

I am so thank-ful for all of you who pray diligently for our little family. I think it is so awesome to be able to email and share not only the pain but the joy we encounter as a family.

Tonight Todd, Kourtney and I were talking. Kourtney said, "Will I have EB forvever?" I said, "Yes you will but when you get to heaven you won't." Kourtney responded, "The first thing I will do is ride a tricylcle, then wrestle with you."

Kourtney is starting to acknowledge her feelings about not being able to do things like the other kids. I let her vent but than I have her focus in on the things she can do. Pray for her spirit, that she will not carry bitterness, and pray for Todd and myself as parents that we will learn the best ways for Kourtney to vent her frustrations but not keep focusing on them..

I feel blessed to have you all praying for us.