Peace

Today has been better so far than yesterday morning! Kaitlyn was pretty relaxed/"out of it" yesterday afternoon and evening due to all the meds she had that morning. She didn't have any more episodes for the rest of the day which is really great though. Her peace and comfort is our top priority.

We've had a good morning so far except her toe sensor for her pulse/ox decided to stop working! The machine is fine but the sensor doesn't work so we have the adult finger sensor on her toe until we can order a new one and have it shipped overnight. Luckily her toe is big enough now to pick up her pulse with the adult finger sensor. It seems to read a little low (just a couple numbers lower) but at least it gives us a range for where she is at with her oxygen. You realize quickly how much you depend on those machines when they stop working!

I found the following in Helen Steiner Rice's "A Touch of Love" and really liked it. I hope you do too!

In seeking peace for all people
There is only one place to begin
And that is in each home and heart-
For the fortress of peace is within. 

Rough Morning

Kaitlyn is having a rough morning today. She woke up around 6am and started crying in pain. She normally doesn't cry this loud so we checked everything out and gave her some morphine to see if that would help. It helped the pain but she also started to have seizure-like episodes. She's had about 6 at least this morning in just the past two hours. We gave her diazepam for the episodes but she continues to have them but not as severe.  Please keep Kaitlyn in your thoughts and prayers today.

We had a great Thanksgiving and were able to gather with family and have some fun. Kaitlyn even had a small laugh for Gigi! Something Kaitlyn hasn't done in a few months. We have so much to be thankful for. Hope everyone had a wonderful Thanksgiving! 

Changes

Thank you to everyone who donated and participated in the 11/11/11 Challenge to raise money for Niemann-Pick Disease research through the Niemann-Pick Disease Foundation. We are so grateful to have so many generous, caring friends and family out there and it really means the world to us! One day with your help, there will be a cure. THANK YOU!!!!!

Snuggled in her new blanket from Aunt Jess

Kaitlyn and her birthdate- so sweet!

Kaitlyn is doing a lot better than she was a few days ago. We aren't sure why (most likely all of your prayers!) but we sure are thankful! Since it seemed that her bowels/intestines were slowing down, we switched her from Pediasure to Elecare. Elecare is more broken down and easier to digest. So far she has been able to process it better. She has had a decrease in the number of wet diapers that she has each day so we are keeping an eye out for fluid retention.

This is what she thinks of how she's been feeling

Her heart rate hasn't been fluctuating as much the past few days. She's actually been able to sleep pretty well at night. During the day she is pretty lethargic but has a hard time taking good naps. Could be disease related or it could be "little brother" related! She went for a number of days with little to no seizure-like episodes, which we were told can also happen at the end of life but she's had a few off and on, these past few days. It's hard to figure out whether that's a good thing or a bad thing?!

Fun with Gigi!

One thing that is starting to bother her more is her saliva. As her disease progresses, she has lost her ability to swallow efficiently, which is why she has the G-tube for feeding. But she still has saliva in her mouth that gets built up and she's having a hard time controlling it so she coughs and chokes on it. We keep the suction machine close by and are using it a lot more frequently. She is also having a hard time sitting in her floor chair cause she is in a more upright position, causing her saliva to pool in the back of her mouth rather than on the side if she were laying on the floor. This is why if she were to get sick with a cold it would be really hard on her body to manage all the extra drainage. There is medication that we have used in the past called Robinul that helps decrease the saliva but it also can cause constipation and other issues. So right now the benefit doesn't out weigh the risk of making her other systems compromised.

Yes- he really IS this happy 99% of the time!

  
We know that this disease is very unpredictable so we are trying to take one day at a time and be thankful for each day that we have, and especially soak up the good ones! :-)

Thank you for all of the messages you all have left, either on the blog, through Facebook, or email. It really means so much to us to know that Kaitlyn has such great support and that she has touched so many of your lives. She is truly an angel from God sent to make us feel more in our hearts and learn what matters most. We are so lucky to be her parents.

Be still my heart

Monday night Kaitlyn was up most of the night. Her heart rate was high and her oxygen concentrations were starting to dip. I gave her meds to try to get her heart rate down so that she could rest but nothing really worked too well.

Today she started to have fluctuations again in her heart rate during the day. So far we had only seen it happen at night. We are obviously very worried for her. We know that there isn't anything that we can do but keep her comfortable. Hospice has told us that if her heart rate and oxygen levels fluctuate that we should alternate diazepam (Valium) and morphine.

Pray that tonight she gets some rest!

On a different note, we have been thinking about how we can help to further the research that Dr. Wasserstein and the group up at Mount Sinai are doing on NP Type A. They have never studied an actual body of a child with Type A after they have passed away. I called Dr. Wasserstein to discuss what would benefit them the most. She said that samples of cells from different parts of the body would help them in their research. Especially to see if sphingomyelin is stored in the intestines or not. Something that has never been proven. Dr. Wasserstein said that it would be minimally invasive and the samples of cells can be done by a pathologist locally after she passes away and then sent up to them in NYC. Chip and I decided that we are going to do this. I feel really good about this decision.

Please continue to pray for Kaitlyn. We really just want her to be comfortable and pain-free. Please also pray for me and Chip that we will be able to have the strength and courage to be the best parents we can be to Kaitlyn and Cole during this time and to remember that she is still here! :-)

The Power of Prayer

Snuggle snuggle

It is truly a blessing that Kaitlyn had a good weekend. The love and prayers that we have received from everyone is really the only explanation as to why she had such a good weekend. We had a couple of scary days with fluctuating heart rates and not having bowel movements like she should last week. All signs that her systems are shutting down. We know what the future holds for Kaitlyn. We know that she will pass. We just don't know when. It could be days, it could be weeks or even months from now.

So glad that Daddy is back in town!!!!

We just want her to be happy and comfortable. It is all about quality of life. We used to think that we wanted her here for as long as possible, which of course we still do, but as long as she wants to be here. I have made sure that she is aware that it is up to her and God. We will be okay. 

Reading with Cousin Makayla

Trust me, it took quite some time for me to get to this point. There have been plenty of times where I was angry at God and doubted my faith. I no longer have any doubt in God and in the goodness of others. Kaitlyn and all of you have taught me that.

Lots of family time

We cherish every moment we have with her. Please continue for peace and comfort for Kaitlyn during this time. Your prayers are working!

Cousin Daniel being cute as always!

On Friday, when I was really worried about Kaitlyn, I reached out to Faith Massey, our friend and photographer, who has generously donated her talents over the past year to us so that we can capture precious moments with Kaitlyn. She dropped everything and came over on Saturday for a quick photo session at our home. I can't tell you how much these photos mean to us. They are everything. Faith also put together a video of the photos too.

Click here to watch video. (make sure you have your volume turned on or use headphones to hear the music)

She is amazing. Here is also a link to her website Images by Faith Portrait Art and her Facebook Page.

Here are some of the photos!

Another Rough Night

Yesterday Kaitlyn had a good day with family and friends at home, but last night her heart rate got really high again. The only thing that seems to be keeping her heart rate lower is the diazepam. Please keep praying. We can feel the love and prayers from everyone.

Big Prayers Needed

Big prayers are needed for Kaitlyn today. Last night her heart rate was high and she was very restless all night. Normally when she is sleeping, her heart rate is in the 80s and 90s. Last night it was in the 120s and 130s consistently, even when she was given clonazepam and morphine.

This is another sign that it is taking a lot of work to keep her body going. We have heard from other parents who have lost their children to NPD, that this is common at the end of life. What that means, time wise, we don't know. We just pray that she isn't in any pain and that peace and happiness would fill her during this time.

"So do not fear, for I am with you; do not be dismayed, for I am your God. I will strengthen you and help you; I will uphold you with my righteous right hand."
(Isaiah 41:10)

Ten little fingers, ten little toes

The day that Kaitlyn was born, I remember laying on the c-section table and all I could think about was whether she was ok. As they were stitching me up, I could see her under the warmer being assessed by the nurses. Chip was over with them and I remember asking him, "Is she okay?" "Ten fingers? Ten toes?"

He responded, "10 fingers, 10 toes! She's perfect!"

Kaitlyn's toes- 10 days old

How silly it seems now to me to have asked that question. Of course I wanted my baby to be healthy and  "normal", both inside and outside but 2 years, 4 months later I could care less if my baby had 2 toes or 11 toes!

Kaitlyn has perfect little toes. She never had her toenails painted so we decided to have some girl fun and do them up! The color is called "starlet". A perfect name for our little girl's first pedicure. :-) She IS a starlet!*

Kaitlyn's starlet toes! 2 years, 4 months old

As you go about your day, please keep Kaitlyn in your thoughts and prayers. This week she has been having trouble with bowel movements. She hasn't had any unless we give her a suppository. Her urine is also dark and often has an odd smell. She is on antibiotics which normally give her diarrhea but we haven't seen anything. Hospice feels that some of her body systems may be starting to shut down. We aren't positive of this but it's something we are definitely concerned about. Please keep Kaitlyn in your thoughts and prayers.

Exactly a year ago today- we moved into our new house! I could still hold Kaitlyn like this. What I would give to hold her like this again without hurting her.

Our sweet girl today

God is our refuge and strength, an ever-present help in trouble. Therefore we will not fear, though the earth give way and the mountains fall into the heart of the sea, though its waters roar and foam and the mountains quake with their surging. (Psalm 46:1-3)

*A note to NPD parents- fingernail polish has been proven to cause inaccurate pulse/ox readings so make sure you take the polish off of the big toes before you hook your child back up to a pulse/ox.

11/11/11 Challenge

Please consider taking the 11/11/11 Challenge and donating $11 to the Niemann-Pick Disease Foundation in honor of Kaitlyn Bourgeault, Type A. You can donate easily online or send in a check. Click here to donate

Here is the video I put together for the Challenge that shares the beginning of our story. Please share this with as many people as you possibly can and encourage them to also donate.

Niemann-Pick Disease is so rare that there aren't many people fighting for this disease. A disease that will certainly take our daughter's life. A cure needs to be found and helping to fund the research is the only way we will be able to prevent other families from going through the heartbreak that we face. Thank you in advance!

The Bourgeault Family
Deanna, Chip, Kaitlyn and Cole 

Sunday... a pain in the ear!

This morning I checked Cole's ears with an otoscope. Yes, I have an otoscope... the thing that the doctor uses to look in your ears. He had been coughing last night and I wanted to see if his ears were bothering him too.  Cole's ears looked fine but since I had it out I figured I would check Kaitlyn's too just for the heck of it.

This is Cole's latest antic... "I'm king of the world!! (Titanic-style)

Good thing I did cause her left ear canal was almost swollen shut! I called hospice and with my description of what I could see, they decided to start her on an antibiotic.

I feel so bad for her. Who knows how long her ear has been bothering her. With a neurological degenerative disease such as NPA, as the disease progresses it gets more and more difficult for Kaitlyn to communicate to us when something is wrong.

I have been trying to keep her upright in her chair to see if that helps the swelling go down but she hasn't been too happy about that. If we lay her on her right side, her oxygen concentration goes down, probably due to the large size of her spleen and liver pressing on other organs, like the lungs and blood vessels. So we have her on her left side a lot, which is the side that is swollen. I'm sure laying on that side a lot has contributed to the swelling. We are trying some Benadryl to see if that will help get rid of some of that fluid and some morphine for the pain. I can't imagine how bad that must feel. My ears hurt just thinking about it! 

Hopefully she will start to feel better soon. We are still going to try and have a fun day at home though. Maybe we will paint her toe nails since she's never had that done before. :-) Sounds like fun to me!

Daddy has been "quarantined"

It's like Where's Waldo... can you spot our chihuahua Lily?

Kaitlyn is still doing about the same. Since we increased her meds in the middle of the night her episodes don't seem as severe as they were. Hopefully this will continue to be the case and they won't bother her as much or take so much out of her.

Kaitlyn is now 2 years, 4 months old!

Daddy went on a trip to Wilmington on Wednesday and luckily he was out of town cause he came home not feeling well. He is now "quarantined" at his parents house. :-( I feel terrible that he can't be here with us but we can't risk Kaitlyn or Cole getting sick.

Cooking with Gigi

So we Skyped with daddy this morning and hopefully he will get better very very soon!

Skype snapshot from Daddy

Life can definitely throw some curve balls. But we can handle it. :-)

Halloween and Heartache

Hope everyone had a great Halloween!

Sweet siblings

Kaitlyn continues to have seizures multiple times a day and is on oxygen 24 hrs. She is also on antibiotics for a urinary tract infection that we suspected over the weekend. She is still less interactive than she used to be and seems to be getting more and more "out of it" as the disease progresses. She still hasn't laughed in a month and a half and smiles are scarce. She seems to be fairly comfortable though and happy from what we can tell so that is all that matters!

For Halloween, Kaitlyn was a princess (of course!) and Cole was her dragon protector.

We also celebrated Chip's birthday on Sunday. Happy Birthday!

Happy Birthday Daddy!!!

There was also a lot of heartache over the weekend too. We learned of the passing of two children with Niemann Pick Disease. Mia Walts, Type A, 2 1/2 years old and Gavin Lopez, Type C, 7 1/2 years old.

Our hearts and prayers go out to the Walts and Lopez families.  We have gotten to know both of these families over the past year and it hits really close to home as to how quickly things can change. Please pray for healing during this extremely difficult time for these families.You can also learn about Gavin and how he passed away on his mother's blog.

She mentioned the following quote, "to live in the hearts of those you leave behind is to never die".