Nicholas's Pulmonology Appointment

Here is Nicholas in his new EZ-On Vest which is a specialized adapted car seat for kids with SMA. The straps fit snugly around him and attach to the seat belt to keep him securely in place when traveling. It also allows him to lie flat which helps keep his mucus at bay for long car rides. We received the vest from Families of SMA at no cost to us, which was fantastic considering they cost a few hundred dollars. We will return it to them when Nicholas out-grows it so another child can use it. He was practicing his wave this afternoon and feeling pretty proud of himself!

Ella & Nicholas were both so happy to get out of our house! Because it is cold & flu season and because the flu hit our area fairly recently, I haven't been taking the kids out much at all. I think it has helped to keep them healthy, but it was also so nice to finally get out, even if it was just for Nicholas's pulmonology appointment at OHSU.

Nicholas has a new pulmonologist that we like very much! His name is Dr. Hsia (pronounced Shaw) and he has a good bedside manner and took his time in answering all of my questions during our visit. He suggested we schedule a sleep study for Nicholas to see if he is getting rid of his carbon dioxide gases while sleeping. I was hesitant to do this because of the logistics of it all...I am nursing Ella, Jeff is working swing shift, I would have to lug all of Nicholas's equipment with us (pulse/ox, cough assist, bi-pap, suction, feeding supplies, etc), and I just didn't see how it could be done. After a conversation explaining all of this, Dr. Hsia explained that we wouldn't need to bring any equipment with us since Nicholas could do the sleep study at Doernbecher's instead of a hotel (which was where we went last time) and my mom volunteered to watch Ella overnight, so it sounds much more appealing to me now. I am sure Nicholas will absolutely hate being in the hospital again, but it's really only for 12 hours, so I think he will manage okay.

We also discussed getting Nicholas a new ventilator, the Trilogy, because his current one is so darn loud and not very portable. Hopefully we can transition to the new ventilator very soon. We also discussed getting an ambu bag for Nicholas, as well as a nebulizer and portable oxygen. Dr. Hsia also discussed that we create a form which expresses our wishes for Nicholas health care and how it is handled in case of an emergency. He suggested that Jeff and I write it all out while Nicholas is stable so that we can be level-headed regarding all our our decisions about Nicholas's care and so that we can decide what will be in his best interest. This kind of discussion would have brought me to tears a year, even six-months ago. But I was proud of myself for keeping my wits about me and I totally agree with Dr. Hsia's advice. It will be so beneficial to Nicholas's care team to have a written document to follow when the unexpected happens. It's also nice to know that we can change or modify it at any time.

The last thing we discussed was whether to have Nicholas's trached (given a tracheotomy) when the time comes. I explained to Dr. Hsia that we would like to go the non-invasive route, which means Nicholas would not be trached and would be on a bi-pap most of the time when it becomes too difficult for his little body to breathe on its own. Dr. Hsia believes (as I am sure most pulminologists do) that a trach is the way to go. He said that it is easier for kids to communicate with the world and it is a better quality of life. I listened to his advice, but at this point, Jeff and I feel that a trach would be our last resort. This is a big debate in the SMA community and many kids have trachs and many do not. We continue to seek guidance from families we trust and our feelings may or may not change as we watch this terrible disease take over our son's body. However, we will always keep Nicholas's best interests at heart when we make all of our decisions in care for him. We only want to do what is best for him and what will give him the best quality of life. We hope that he will be able to experience life with as much love, laughter, and joy as possible and it helps to remember that as we make these difficult decisions for him.

Busy Baby Bee

Our six-month old girl is becoming a busy, busy bee! She is rolling everywhere and starting to get into things and, boy, does she love to play! She has also started squealing constantly which is very loud and hurts my ears if I am holding her. I try to ask her to "be quiet" when her brother is sleeping, but there is no reasoning with a six month-old. I am pretty sure she will have a tooth making an appearance soon, as she has been biting lately and chewing on absolutely everything. She has also been a grumpy pants the last couple days, which is not typical for her, so I assume her teeth are bothering her. The other day I let her chew on a bitter biscuit and she was as happy as can be sitting up in her high chair chewing away. Her personality is starting to come through and she is still our sweet little girl, but can definitely have some diva moments. She and Nicholas are starting to form an amazing little bond and enjoy playing together. The other night they passed a ball back and forth and watching them interact just warmed my heart.

Nicholas's Equipment

In the Gustafson household we have a love/hate relationship with all of Nicholas's medical equipment. We LOVE the fact that it keeps him healthy and at home, but we HATE that he has a disease which requires him to use so much medical equipment to live. This is Nicholas's pulse/ox. It has a sensor which wraps around his big toe and measures his oxygen and heart rate. This machine alerts us when his oxygen level drops under 91 and when his heart rate drops below 90 or goes above 200.
This is a photo of Nicholas getting a cough assist treatment. The cough assist gives him a big breath of air which helps to open up his lungs and then sucks the air out, producing a strong cough since Nicholas's cough is very weak. The cough assist brings up phlegm in his lungs, throat and nose.


This is what his cough assist looks like. We use this machine 2-3 times per day.

This is a photo is Nicholas's Mic-Key gastrointestinal tube which is how he is fed during the day. There is a small balloon inside his stomach that is filled with water which holds the button in place. The tube that comes out of the button can unattach.


This is Nicholas's pump and feeding bag. He has a Kangaroo Joey pump and feeding bags. This thing is attached to him most of the day as his body prefers not to do bolus feeds. The nice thing is that it is easily portable and we have a handy traveling bag for it.

This is Nicholas's suction. We use it with his cough assist. As the phlegm is cleared from his body with the coughing, this machine helps suck it all out.


These are the catheters we use to suction out the phlegm. He has a nasal aspirator and two different size throat catheters: 12 french & 14 french.

This is Nicholas's ventilator. He needs to use it whenever he sleeps, which means nap time and night time. We are hoping to get a different one after we visit his pulmonologist at the end of the month because his his current ventilator is so noisy and not very portable.Here is Nicholas wearing his bi-pap (ventilator) mask before bedtime tonight. He just got a new mask because his old one was hurting his nose. Believe it or not, he really doesn't mind wearing it and is quite used to it by now. He sleeps really well with it on and rarely wakes up during the night. It's hard to tell, but he's actually smiling for his photo!
So, our sweet, nearly three-year-old boy has a lot of medical equipment he uses every single day, but if you asked him, he would probably tell you that he doesn't mind one bit, because it helps keep him healthy and feeling good.

Daddy Cuddles

A couple of weeks ago Jeff got a new job and has been working 6 days a week, 10 hours a day. Needless to say, we are missing him a lot lately. Last night he spent some quality time cuddling with our kids in the evening before bedtime and they LOVED it! They adore their daddy and were so happy to spend some time with him. We appreciate how hard Jeff works for our family, and it makes us truly treasure the moments we get to spend together as a family even more!

Baby's Half Birthday

Our sweet Elizabeth is six months-old today! She grows and changes a little bit each day and it's hard to believe that in another six months we will celebrate her first birthday. She now weighs 16 lbs which puts her in the 65th percentile for weight and is 27 inches long which puts her in the 95th percentile for height. She's becoming more of a long and lean baby and less of a chunky monkey.
She has started to love playing Peek-a-Boo and giggles like crazy when we pop out and exclaim, "Peek-a-Boo, I see you!" She enjoys playing in her Exersaucer and stands up in it most of the time. When she's on her tummy she has started pulling her legs up under her belly, putting herself in a crawling position, but she has yet to go anywhere. She is sleeping a good 10+ hours at night now and takes 2-3 naps during the day. She likes to suck her thumb while holding on to a blankey or lovey and will often fall asleep that way. She's such a wonderfully delightful baby and we are so blessed that God chose us to be her parents!

Happy half-birthday, sweet Ella Rose!

Sitting Up (Almost)

Yesterday I got a couple photos of Ella on her changing table sitting up (with some support) but it is so exciting that she will probably be sitting up on her own soon! At the same time, I keep wondering how my little baby is growing up so fast. Before we know it she will be crawling everywhere and getting into things and turning our lives upside down. But I am so thankful that she is able to do these things, her physical movement is definitely not something we take for granted and we feel so much joy and happiness as she meets each milestone.

Good job, baby girl!

Ash Wednesday

Today is Ash Wednesday and the beginning of Lent. Ella, my mom, and I went to mass this afternoon and when we got home Jeff snapped this photo of Ella and I with our cross of ashes on our foreheads. Funny enough, Ash Wednesday is not a holy day of obligation in the Catholic church, but I attend mass every year anyway. It's just a nice way to begin Lent. I love the Lenten season. It's a time to repent and sacrifice to become closer to Jesus as we await Easter.

This year for Lent I am going to make a conscious effort to eat healthier, especially when it comes to snack choices. I will be trading chips for apples and cookies for granola bars. I am also going to cut out any fast food. I don't eat it all too often, but sometimes as I drive to work, I will stop and grab something, so that will also go for these forty days. I also follow the tradition of not eating meat on Ash Wednesday and every Friday during Lent. I am going to add some extra prayer time into my life and my prayer of choice is the rosary. I used to love to pray a decade with my students when I was teaching at Pacific Crest Academy, and so for Lent I am going to try to pray at least one decade every day and the whole rosary if I have enough time. Thankfully both kids' naps tend to overlap so I can fit in prayer time while they are asleep. I also would like to incorporate Nicholas's children's bible into our daily reading time as a way our family can celebrate Lent together.

Hopefully I can make this Lenten season a meaningful one for myself and my family.

DIY Project

I purchased a shadowbox for Ella's nursery long before she was born but saved it until she out-grew her adorable pink booties because I really wanted to display them. Last night I finally got around to putting everything together and I am very pleased with how it turned out. I am never quite sure of myself when it comes to doing crafty projects, but I feel like the more I do them and the less I think about it, the easier it is. Hopefully Ella will like it as much as I do!

photograph by Lyndsey Yeomans of Jack & Lola Photography

Happy Birthday to the Most Wonderful Father & Grandpa!

This is my smart, funny, kind, wonderful dad. Tomorrow (February 28th) he celebrates 60 years of life. He celebrates a full life: he has been married to my mom for thirty-three years and they continue to inspire us with their constant love and affection for each other. He raised three kids (my mom helped too) and taught us faith, morals, and good character. He taught us all the importance of a strong work ethic and worked very hard for his family every day of his life; in the office, in the backyard, or doing repairs on our home. He adores his grandchildren and enjoys playing with them and making them laugh. He enjoys a good meal and always compliments the cook. (I think I got that love-of-food gene from him). I have noticed that as he has grown older, he has a soft tenderhearted side to him that I did not see as much when I was a child, and I love that about him.
On Saturday evening, our family had a birthday dinner for him at my parents' home and we surprised him with his very own iPad! I got the idea to buy him one about a month ago when we took ours over to their house for Nicholas to watch Kipper on Netflix. My dad looked at some of the apps and thought they were easier to read than on his iPhone screen (he is getting older, after all, tee-hee) so I talked to my two brothers and our mom and we all chipped in to buy him one. He was thrilled and so surprised when he opened it!

Some of my favorite moments and most fond memories are with my dear, ole dad.

Here are a few:

• Dad carrying me into my Grandpa & Grandma Hargrave's house one Halloween when I was three because I was too scared to get out and walk on my own after I saw a ghost on the clothesline.
• Dad putting us in pillowcases when were kids and telling our mom, "Honey, I brought home a sack of potatoes for dinner!"
• One Saturday when we were kids my mom was out running errands, somehow we locked ourselves into my brother's bedroom and dad was fast asleep in his recliner in the basement and couldn't hear us shouting on the second floor. We opened the window and called to some people walking by on the street to come in and let us out (God only knows what they thought was going on). Dad woke up when he heard the door open and thanked the kind people and didn't get too mad at us (thanks for that, Dad).
• Having my dad drive me around the track in his corvette when I was nominated Homecoming princess and then escorting me onto the field.
• Dancing around the house boat at Lake Billy Chinook when I was a sophomore in high school to Stevie Nick's The Edge of Seventeen.
• Dad running back and forth between the two straight-aways on the track to tell me my times and give me words of encouragement when I was in junior high and high school running the mile.
• Dad attempting to teach me how to drive a backhoe so I could lift him up in the bucket to trim the 20 foot hedge on our property. Let's just say I am pretty sure he saw his life flash before his eyes that afternoon and I was relieved of my duties, much to my happiness.
• My dad walking me down the aisle and giving me away at our wedding. I got all choked up when he shook Jeff's hand and gave me a huge hug.
• The day Nicholas was born my dad came to the hospital after he got off work and he cuddled with his first newborn grandson and it was just the three of us watching Dancing With the Stars (which is a favorite of ours).
  

These are just a small sample of the many, many wonderful memories I have of my dad. We love you so very much and couldn't have asked for a better father/grandfather!

Wishing you a healthy, happy, fun-filled 60th year and cheers to many more years of fantastic memories!

xoxoxo

Kids, Colds & Cash

Here's what we've been up to this past week: Ella has been trying new foods the last couple weeks. So far she really seems to like green beans, applesauce, and pears. When she eats she will take a couple of bites then stick her thumb in her mouth for a bit which makes it hard to feed her. I have started holding her right arm down so I can feed her without her sticking her thumb in her mouth all the time, which can be more difficult than it sounds. I have ended up with baby food all over me lately!


Jeff got Nicholas's old Exersaucer out of the attic this week and Ella is happy to have a new form of entertainment. She likes exploring all the toys, but sometimes gets frustrated that they are attached because she cannot put them in her mouth, so we give her unattached toys to play with when she's in there as well.



When Ella and I returned from the retreat last weekend, we came home to a sick little Nicholas. Of course, anytime Nicholas is not feeling well, the worry starts to set in. He vomited on Sunday and Monday and his heart rate was unusually high. A typical pulse for him is between 118-130 when awake and his was between 140-180 all last week with short periods where it was lower or higher. He also had a runny nose and was running a low temperature. The highest his temp. got was 101.4. Thursday afternoon I took him in to see his pediatrician who couldn't determine what was plaguing our little boy. Nicholas's lungs were clear, he had no ear infection, no visible signs pointing to what was making his heart rate so high. On Thursday night around 2:00 a.m. he woke up crying and his heart rate was at 194 on his pulse/ox. His oxygen, however, was at 94 so Jeff and I just tried to calm him down and help him relax. I ended up laying in bed with him for a few hours which helped calm him down and he slept peacefully after that. On Friday he awoke feeling great and his pulse was much lower than the previous days. I think he is having some pain because he is cutting a molar and he also seems to have a bit of a cold, so we have been suctioning his nostrils on a regular basis and giving him Children's Advil to help with his low fever and tooth pain. This is a photo of him mid-week...he still manged a small smile, sweet little guy. I snapped this photo of him today and as you can see, he is starting to feel much, much better. His pulse has returned to normal...it has been around 120 all day, thank goodness. He also slept most of the night last night which was great for both him and for me.
I also spent a couple days this week going through the kids' medical records and calling to get reimbursed on over payments and co-pays. Good thing I keep such meticulous records of everything, because I am sure those companies were not going to reimburse us without my asking. So now we are looking forward to both our medical reimbursements and our tax return!