Tis the season to be hopeful

Christmastime---such a wonderful time of year. A joyful time to share with family. A time of giving, a time of sharing, and for being thankful for all the blessings in life. A time to get out with family and see the holiday lights, listen to jolly holiday music in the stores while Christmas shopping, and sipping hot cocoa at home while wrapping Christmas presents and watching Christmas classics. There are so many things I love about this season. But this time of year is also a time that brings a lot of pain and sadness. Many are grieving over the loss of loved ones and feeling the heartache and emptiness of missing friends and family members. So much heartache that seems to be exemplified at this time of year. My heart especially aches and goes out to those families that are suffering the loss of a child right now, especially those families that have lost their angels to SMA, several just recently. The fear of losing a child is something all parents experience but for families that have children with SMA it is more of a daily fear that accompanies the diagnosis of SMA. Things can change so quickly especially when illness strikes, and fear becomes reality. There are so many families hurting right now and my heart hurts along with them;  I don't know what they are going through, but I can only imagine. 

I feel that grief in general is part of the journey of having a child with SMA. Unfortunately, during this joyous season I’ve been feeling more of it lately too. The brokenness of what expectations were and what reality is, the hope that is getting stretched too thin----waiting for a cure, waiting for treatment. There are meds out there that are helping children with type 1 thrive (sit up, stand, and even eat orally)---amazing things that children with type 1 are not expected to do. Its heartbreaking to know its out there and that while some children that are lucky enough to get into trials and get access to it, other children are weakening, dying, and losing the battle to SMA. Yet, we can't lose hope or can't give up---we have to hold on to the hope that someday Lily and her friends will have access to this treatment and do everything we can to keep her healthy and strong so she thrives even more when she gets it.

It's not always easy, but we have to focus on the fact that she is a strong type 1 and grateful for all the things she is able to do, like smile, laugh, scowl, and make so many facial expressions, even sing (Adele's "Hello" is one of her favs.)

But some days the limitations of SMA are more visible and harder to distract from ---so sad and so frustrating. Maybe it’s the pregnancy hormones, increased tiredness, and business of Christmas season, AND starting nursing care to top it off all –but lately SMA seems to be a little more heartbreaking. Some days I just get so frustrated and sad that she can’t do typical two-year-old things. I don’t like to use the word typical or normal because I feel like Lily is a normal two year old----she is so smart and loves to do things other two year olds do, like color and play with dolls or stickers, watch movies and play games; however, her body just doesn’t allow her to do physical things that other kiddoes her age are doing---sit and play with toys, run, walk, scream, throw tantrums. Don’t get me wrong…she has her own way of throwing tantrums but the things that I grieve most about her diagnosis and SMA is that she can’t throw her arms around me and say I love you, she can’t run into my arms crying and I can’t console her in the way that every fiber of my being wants. I can’t hold her upright or against my chest and let her fall asleep on me when she wakes up at 3 am crying and upset. I can’t just lay with her in her bed because first of all, she’s in a crib and I’m largely pregnant, but she has bipap and her pulse ox and her feeding tubes and cords that make it difficult to do those things. Not impossible but very hard. Especially with our lack of space and small area we’re living in.  

However, dwelling on the limitations of SMA is not something that will change or better our situation, so I try not to stay in that place for long. But that being said, some days are just hard. I crave normalcy. I crave the days where I feel like I’m just constantly picking up messes that my two year old has made. I crave the days where my child is crying and screaming because she doesn’t get her way or she doesn’t want to come inside from playing. I crave the moments where she looks at me with chocolate all over her face and grins and I shake my head at her because she climbed on the counter and stole cookies. I crave to hear her talk to me and tell me everything going on inside of that smart little mind of hers. I crave, I crave, I crave just to hear her say "I love you mommy." I crave the moments that many take for granted or don’t give a second thought. There isn’t a day that goes by that I don’t wish for and pray for and hope for a little more normalcy. But on the other side, as I’ve heard other SMA moms say, if our life was normal-if our life was easier, then there would be a void---there would be an emptiness because that would mean that she wouldn’t be here with us. And every moment with her here (despite the challenges and sadness and limitations of SMA) could not compare to the sadness I would feel if she were not here. I would rather endure endless troubling, challenging days facing SMA than have one minute without her here. And during these times when I struggle and grieve over the things that are lost, the things that her body doesn’t allow her to do, I try to remember and hold on to the fact that she is HERE. A beautiful girl with a beautiful smile and so much personality. And though she has limitations, she is capable of so much. She is full of joy and has taught us the gift of love and she is daily reminding us of the true meaning of Christmas------the love of a Saviour that knows no limitations, sees no limitations, and loves and accepts us for who we are. That is a big Christmas blessing to be thankful for!!

                                                            

             
 



If you would like to pray for Lily and our family, here are some specific prayer requests!

1) Continued health throughout the winter and flu season
2) Available access to needed treatments and drugs for Lily and all her friends with SMA
3) Strength, patience, open minds as we adjust to having a nurse in our home helping us care for Lily as we anticipate Baby boys arrival in a few months
4) A healthy baby boy and smooth delivery
5) Healthy for family and friends
6) Peace and comfort for Levi's grandfather (Victor) and as he recently lost his wife
7) Being able to focus on what Christmas is truly about and the many blessings we do have -not the limitations or challenges we face

Riding the rails of life

I've had good intentions to blog lately and have felt the need to share but after Lily goes to bed, its hard to find the energy for anything else but sitting in the chair. However, when I started this blog about our journey to share with you all, I wanted to be as real as possible and talk about the reality of sma---the good, bad, and ugly. It's hard for me to be vulnerable and share my heart about things that hurt me to my core, but I want to be honest about all parts of our journey and share about hard days as well. The last few weeks have been hard. I’m not exactly sure why but it could be a) being pregnant and tired, (b) taking care of my 2 year old sassy toddler that needs constant care and supervision, (c) awful allergies all day long despite allergy shots and meds, (d) the start of harvest and dad getting home later, (d) thinking about having two children and how....I'm going to take care of them :-) or (e) or all of the above.

We have also started the process of looking for a nurse. Ugh. As my fingers type that, I just feel my stomach sinking. It may seem crazy to some--like why wouldn't you take the help if you can get it. BUT I have cared for my own daughter for 2 plus years and I have done all of her daily cares, all her treatments, all her meds, feedings, etc. It's very very hard to think of letting someone else do that. It’s letting go. Letting go of the fact that I know that I can’t do it all on my own, especially with another one on the way. Lily’s care is 24/7 and since she requires frequent suctioning and constant monitoring, unless she is napping and has her bipap on, (but even then) there is no break. I need to be close at all times so unless I’m really out of the house, its not a break. 

Nursing is not something I really want to do but there's been many days (more days lately) where Ive been tired, weary and worn and I know that it would be nice to have help or an extra set of hands during the day. Just hard to go through with it. Its hard trusting someone to take care of your child especially when there are special needs, many needs. I won’t ever have to leave if I don’t want, but it would allow me to get other things done at home like cleaning, laundry, make phone calls, setting up appts, ordering supplies, doing things that are just hard to get done while caring for Lily. Also, I could just be mom. I could still do her cares but I would’t have to do all of them and be “nurse” too.  Most likely it would take me a long time to feel comfortable leaving Lily but I wouldn’t have to. I suppose the reason I struggle so much is the letting go part of it. Its  hard to let go of control -to let go knowing it's something you need to do even when you don’t want to. It pushes you out of your comfort zone. I feel like since Lily's diagnosis, I am continually being pushed out of my comfort zone, forced to let go.  Letting go of what expectations were and what you thought life would be and instead taking things one day at a time --for what they are and accepting your new reality. 

Some days I don’t feel the loss or limitations of sma, I don’t see it as clearly as other days. Other days I feel such joy and pain within minutes of each other. It feels like living in two worlds at the same time, riding two rails of life at the same time. Trying to enjoy and cherish the moments that you have, at the same time knowing that in an instant things could change. Lately, it has been more of a struggle and the limitations of sma have been more visible. It may be due to the tiredness and all mentioned above but a lot it comes down to what my expectations for  Lily were and grieving the loss of those expectations, the milestones she has missed, and accepting what she is physically able to do. I know she is so intelligent and growing more independent by the day, wanting to do things on her own without moms help. That is great because its normal and typical. But its also a reminder that I have to help her with everything and her body doesn’t allow her what she wants to do. She can’t move without me, can’t eat without me, can’t itch her face, wipe her tears, or tell me why shes upset when shes crying. Its heartbreaking and it tears at my soul but that’s not a place I can stay for long. But easier said than done-some days it feels so defeating. Yet, I have to work on shifting my focus to what she can do and her personality, her laugh, her smile, her sassiness and that she is here with us today. She is here. Too many parents have had to experience the loss of their child due to sma. Thankfully there is great potential that someday, drugs other children with SMA are receiving through clinical trials, will be available to Lily and other children, hopefully sooner than later But it still doesn’t take away from all the families and parents that feel the void of missing and grieving over someone they love that is gone.

We feel that Lily is a very strong girl and strong for a type 1 and we want to help her conserve any muscle tone she has so hopefully one day she can sit upright and maybe by the grace of God even stand. It is happening. Other kids are doing it. It just has to become accessible for All kiddoes.

We have long days and some days are really hard. Just trying to get through the day is a challenge. But then I have these moments with her where I’m reminded of the gift of her life that we've been given. This sweet little girl with the most adorable little giggle and smile. I was able to hold her in the recliner for a little bit this evening and snuggle her while we watched some cartoons . Those moments make the limitations of SMA disappear and make the anger and frustration I feel towards SMA go away. Its just me and my girl sitting and watching cartoons. And I will cherish that time and those snuggles for as long as I am able to. Those moments help me to focus on what is important.

When I was on facebook earlier, I stumbled across a post from Proverbs 31 Ministries and I think whoever was writing was addressing me specifically. They were talking about God stretching our faith and taking us out of our comfort zones, how we are called to trust him and obey him during difficult seasons of life. I feel like we are in a difficult season of life and have been for awhile. And maybe it will always feel that way as we walk the journey of SMA. But especially as we go forth and try to find a nurse that is a good fit for our family, (despite much hesitation and stretching), I am reminded that I’m not alone and every day that passes, we are getting through with His strength and not our own. I really like the quote that Tracie Miles from Proverb 31 Ministries shared “God will never stretch us beyond what we are capable of in his strength.” Sometimes I wonder; I feel like I have been stretched and pulled and frayed beyond what I thought I could stand----but at the end of the day. I can say I made it through, I'm still standing. And I’ve got some pretty amazing blessings in my life to be grateful about. 

       

 If you'd like to support us in prayer, below are some specific prayer requests for our family! Thank you for you continued love and support!

1) Pray for HEALTH ---especially for Lily as we enter cold-flu season! A cold for her is detrimental to her health so pray that germs and illness stay away!
2) Pray that drug treatments children are receiving through clinical trials soon become available to us and kiddoes just like Lily
3) Pray that we are able to find a nurse that not only takes great care of our Lily girl, but enjoys spending time with her and becomes a great fit for our family
4) Pray for a safe and successful harvest for Levi, my dad, my father-in-law, and all those harvesting right now!
5) Pray for health for baby #2

Happy Birthday to my sweet 2-year-old Lilygirl!!

Feeling lots of emotions today as I think about my big girl turning 2 tomorrow. We weren’t even sure this day would come—we were told most likely it wouldn’t. According to statistics, 90% of children with SMA don't live to see their second birthday, however, with medical advances and increasing research, clinical trials and life saving equipment are more accessible than ever to help us win the fight. And thankfully, we can say that Lily is winning that fight. It is a day to day fight, but we are blessed and thankful to say that we get to celebrate her second birthday. Feeling grateful for that, yet also feeling sad and hurt for the families that don’t get to see their child reach their second birthday, or even their first. Yet another child with SMA passed away this week. Please continue to pray that the drugs available to children in trials now become accessible to all children soon. With SMA there are no guarantees ; that is why we continue to just take one day at a time.

Tomorrow we will celebrate Lily’s birthday at home and then we are having a big birthday party for her at Gpa and Gma Kramer's house on Saturday. It will be a lot of fun and Lily will get to see lots of family and play with her cousins, which she always enjoys. Last year for her first birthday, I was in a much different place (physically and mentally). I was worn out, tired, and just stressed/anxious. We had a crazy summer with Lily being sick,  in and out of the hospital, in addition to her gtube/nissen surgery, bipap trialing, etc. Levi and I were running on low. This year should be a different story. Not that I’m not tired and at times stressed :-) (who isn't!!) but we are in a new normal for us. We are in a good routine with Lily and we are “used” to SMA. We have embraced it, because really-what other choice do we have? We have chosen to do everything we can to keep Lily healthy and strong.

So looking back over the past two years and what we have been through, compared to where we are at now----wow, surprisingly we made it. Some days I truly wondered if we would. And we have a happy, healthy, sassy and increasingly independent two year old girl that brings us SO MUCH JOY!

Here’s to two year old Lily and some of my favorite things about my little girl:

1)   Her smile-thankfully Lily still has the ability to smile and whether its her toothy grin or just her smirky smile, it just makes me happy every time I see it. I hope she never loses the ability to smile at me but for now, every day I will cherish it.

2) Her giggle-her giggle may be easy to miss if you’re not around her much, but it is adorable---its not a hysterical cackly kind of giggle because she doesn’t have the strength, but it’s a subtle throaty giggle. I love that she has a humorous side to her and she can enjoy laughing at things that she likes (like kitties, cartoons, when mom runs into things, etc)

3)   her snuggles-due to her secretions and inability to swallow, Lily needs to be laying down the majority of the time with her head turned to the side, but she will tolerate short periods of being held (not completely upright of course) but snuggled in my arms. Nothing is better than being able to be close to her and smell her hair and kiss her sweet cheeks. Since I can’t pick her up carry her/snuggle her chest to chest, this is the absolute next best thing

4)   her growing independence- and I hesitate to write this because she has been testing my patience lately with her growing independence. But yet it also reminds me that she is just a toddler. She wants to do things on her own and she wants to do them at her pace. I was making a bday cake for Lily earlier today and had her help me pour the ingredients in the bowl and stir. When I took the bowl from her and the spoon to stir it and pour the batter in the pan—major fit! I need to remind myself that she is just a toddler and she doesn’t want mom to help or do things for her-----just so hard to meet that balance because she does require my assistance with pretty much everything!

5)   last of all, her strength. I was talking to my sister about this last night. Lily is such a strong girl and I know many parents say this about their kids that have SMA-they are strong. They are stronger than many adults you meet -they have generally been through a lot in their short lives already and yet, they persevere. They do not give up and they do not lose hope. Things do not come easy for Lily and she has to have help with everything. I can’t imagine how frustrating that would be. Of course, her toddler 'tude is becoming more apparent, but nonetheless, she is patient and she is so strong. What that girl lacks in muscle, she certainly makes up for in determination and mental strength-I can learn so much from her!

Can’t wait to celebrate my two year old girl tomorrow. So grateful for this milestone and the wonderful little person she is becoming.

After a hard & long labor, this was all I needed and all that mattered. 

                     

    

Oh God, You’re my deliverer
The One, the One who carries us
Oh God, You’re my deliverer
The One, the One who carries us

Matt Maher- "Deliverer"

Summer Lovin'

It's been several months since my last blog (yikes) so a lot has happened! I'll have to do a few posts to catch up to what we've been up to. So far, we've had a good busy summer and we've gotten outside a lot and enjoyed the beautiful weather! 

Lily's cousin Hailey (Schaa) was born at the end of March so we have enjoyed getting to spend time with her and her mom and dad, especially mom while on maternity leave. Initially, Lily wasn't quite sure what to think of Hailey. All she did was sleep and eat. ;-) But now she is much more entertaining and fun to watch since she is awake more, making lots of noises, and moving around! I can tell these girls will be great buddies as they grow up together!

We had Easter with the Kramer side of the family this year! Lily got to hang out with all of her crazy cousins' on the Kramer side and did her first Easter egg hunt as well!     

 
  Our beautiful Easter Lily!
 


We had the opportunity to dedicate Lily to the Lord at the Steamboat Rock Baptist Church (where Levi and I both grew up.) We were so grateful to be able to do this and for the support our church and community has shown us through everything!

                                                                                               So many grandmas!

 

This girl loves to be outside and loves crafts! So here is the best of both worlds!! She is a farm girl at heart!

      

Sand art is the best! Gotta love Hobby Lobby :)

Kitties, kitties, kitties! This girl can't get enough of her kitties. She is a little partial to the orange kitties because of Mr. Mustard. And I'm pretty sure the little kitty is quite fond of Lily too. Probably because he doesn't get mauled by Lily like he does with the cousins :) 

 

                      


As many of you know, Lily has had some trouble with reflux off and on. We tried numerous things to resolve it: feeding mix changes, changing the rate of her feed, medication changes, venting/not feeding feeds, positioning, etc. It happened off and on for months and she would have weeks without any problems, then some weeks where it was almost every day. It was to the point where it was clearly making her very uncomfortable when she would reflux and her feeding would come back out her gtube and at times out her mouth/nose. Horrible to watch and I felt terrible her having to go through that. In addition, the risk of Lily aspirating food into her lungs was very high due to her inability to swallow. Thus, we decided after much consideration and speaking with Dr. Schroth, (her pulmonologist) that it was best to try a gj tube (gastrojejunal time) that goes into her small intestine, rather than feeding her stomach. That would most likely resolve the reflux and prevent food from coming back up since it would be fed directly into the intestine. It was not what we wanted to do and not what seemed "normal" of course. But due to Lily's muscle weakness, inability to swallow, and high risk for aspiration (which could lead to more dangerous respiratory complications) we headed to Madison, Wisconsin for gj placement mid-May.

The actual gj placement went fairly well, considering Lily was not sedated and it was her first gj placement. However, things took a turn for the worst. We started feeds off slow and worked up overnight, which she tolerated perfectly. Then the morning after her procedure she started to vomit up large amounts of green bile, which you could tell was just so hard on her body and stressed us out because we had to make sure we were constantly coughing/suctioning/clearing her airway so it didn't settle in her lungs. Unforunately, for Lily the switch to the GJ made things worse for some reason, which was not to be expected at all. Lily's Dr. mentioned that she only knew a handful of kids that had not tolerated the switch from G-tube to GJ well. *Sigh* Apparently, Lily likes to march to the beat of her own drum. Not even 48 hours later, after the vomiting persisted, we made the decision to go back to a regular g-tube button.  We were nervous and we waited, but restarted up on feeds again slowly and Lily tolerated them very well. Hmmm. Crazy. And once we got her out of that hospital room and into her stroller, then into the truck, she was a new person. And (knock on wood) as wild and crazy as it seems, she has not had a single episode of reflux since we have been home. I have to laugh because that poor girl went through the mill and back just to get a regular gtube button placed. So 4 days later we returned home. Even though hospital stays are always rough and we are all sleep deprived and crabby, we had wonderful nurses, great care, and always felt supported in our decisions at American Children's Family Hospital-worth the drive to Madison!

 
Thankfully this girl will sleep in the car, otherwise it would make for a very long drive!

  

Lily was not a happy girl during our stay there! Can't blame her. 

                              
                                

And literally-the moment we got her into the truck, she was all smiles. Ready to go home mom and dad!



Lily has been doing very well and we are so grateful for her continued health and sweet personality. She is a joy to be around and we are so thankful its summer so we can get out more and enjoy the nice weather without being worried (so much) about germs!! Thank you for your continued support and prayers. The rest of the summer update will come soon!

Lily sends her love :-)

"My grace is sufficient for you, for my power is made perfect in weakness." 2 Corin 12:7

Diagnosis Day

One year ago tomorrow marks Lily's diagnosis day. It's hard to believe that one year ago we were getting stuff ready to head to Iowa City the next day to find out what the test results showed. I had read stuff online about SMA and in my mind feared the worst, hoping those weren't words we were about to hear. Here is a piece from my journal I wrote the night before we were diagnosed.

3/31/14

Iowa city appt tomorrow. Feeling nervous and feeling scared but trying to be positive. Knowing that God is in control and that He is bigger than anything that we could be faced with. Still the past few days have been harder and I have been thinking about it constantly and what kind of news were going to get. Ugh. Just want this wait of finding out to be over with. Still going to physical therapy in Waterloo and Lily has shown small steps of improvement (about the same). Oh that girl. I couldn’t love her more. Still, I just grieve for her every day, for those little things that she can’t do like kick, move her arms around, grasp, hold on to bigger toys, push buttons, etc. More than anything in this world, I want her to be stronger and to have strength in her arms so she can grab my face with her hands and for her head to be stronger so I can just prop her on my hip and walk around. Hopefully tomorrow will bring good things or at least a step closer to finding out what is going on with our sweet pea. 

It's hard to go back and read those words again and think about the fear and anxiety I was feeling that night and that next day. But it's also amazing to think about how we got through this last year since diagnosis. We received the worst news we could have heard, were told Lily had a terminal genetic illness (with no cure or treatment), and that most kids with Type 1 SMA had a life expectancy of up to two years of age. We were devastated. Sat in the speciality clinic in Iowa City with the neurologist and his nurse. Cried. Held Lily tight. Ugh. Thinking about that moment still makes me break down in tears. But...thinking about the fact that she is still here. That she will be TWO years old in August. That she is doing well. That she is the prettiest, sweetest little girl, and that for every day that she is here I get to be her mommy----that makes me happy, that makes me thankful, and that makes me feel so blessed. As I sit here crying writing this post I want to thank you-those who prayed, those who brought meals, those who visited, those who surrounded us in love and prayer, THANK you for your support. We would not have gotten through those following days without you. 

Looking below at the pictures from one year ago, you can see that Lily was such a happy, smiley girl and the same beautiful smile lights up our lives today. I am so proud, thankful, and full of joy for our blessing, Lillian Grace. And speaking of blessings, Lily became a big cousin today to beautiful little Hailey Marie Schaa, (proud parents Aunt Abbie and Uncle Michael Schaa). We are so thankful for this healthy, sweet little girl and Lily can't wait to meet her cousin! We love you guys! 




 

                                      

Happy Easter from our little bunny!!