Keeping Ellie's suctioning supplies clean is imperative. Without a streamlined system to keep our supplies clean and a good schedule for replacing catheters, Ellie's risk for infection would be much higher. Below I'm detailing what supplies we use, where to buy them and how to keep everything clean and rotated.
We like to color code our jars and lids and keep them different sizes to help avoid confusion. In the heat of the moment during an emergency you want everything organized and labeled as clearly as possible. We like to use the color green for the "dirty" jars/lids and purple for "clean". By using a shorter jar for nose suction, it helps avoid confusion so you know the nose sucker always goes in the short jar.
Our goal is to change out the jars and suction catheter twice daily. Once daily is the recommendation we were trained on but you can't be too clean with this stuff! We put the jars and lids in the dishwasher when we run our dishwasher at night and we make sure to run a sanitize cycle. We let the jars and lids air dry and then store them in a clean, dry cupboard until we need them. It's good to have at least 3 sets of jars/lids to rotate, two in the dishwasher and one in use each day.
Today I got a call from the genetics department. Ellie has a diagnosis that explains all of her symptoms. The our test from the whole exome sequencing was re-run to compare our results with current findings and data. Jeff, Ellie's dad, and I both carry the the genetic material responsible for Ellie's genetic variance with the TTN gene. Limb-Girdle Muscular Dystrophy type 2J also known as Salih Myopathy is newly discovered since Ellie's birth. We'll learn more when we meet with the geneticist in October. If you want to read more there is some info from the National Organization for Rare Disorders (NORD).
For Ellie, having a diagnosis will be helpful. For example, we're more likely to have help from organizations such as the Muscular Dystrophy Association and may be elegible for assistance for various things because she has a diagnosis where as before because she didn't have an MDA diagnosis we weren't eligible for support. For science and research, this is good. We have a name and something searchable. There are others diagnosed so we can learn from them however this because it's extremely rare there is little information so far.
For me a diagnosis is overwhelming. I have so many feelings. Before a diagnosis I could pretend, and hope and dream that some day all these difficulties would all disappear. That Ellie would walk and breathe on her own and we wouldn't need all this extra equipment and that she could be independent. She still might do all these things but instead of dream, I can research and learn how this disease/disorder has affected others. A diagnosis makes it real. It's scary and right now, I'm afraid to know more. It's a lot me to digest and a major change for me as far as mindset. I'll remain hopeful and still work very hard to provide and do the best I can for Ellie. That won't change. For today, I need time to grieve. Tomorrow is a new day. #TeamEllie
Happy Birthday Ellie!
Today you’re two! I can’t believe it. I can’t believe it’s been two years since the most terrifyingly miserable week of my whole life. Your birthday is a difficult day for me to celebrate. You'll understand some day.
I love you more than you know and I’m so proud of all you’ve accomplished in your short life. You’re determined to prove your abilities. You’re happy. You bring us joy. You radiate happiness.
It’s hard for me to sort out my feelings on your birthday. I’m so happy to have you in my life. You have made me a better person, a better mom, a better dietitian, and a better wife. But when your birthday rolls around I have flashbacks and relive the darkest, most lonely moments of my life. I walked the hospital halls, unable to hold back tears. I’d walk back and forth between the NICU and my room in the Mother/Baby unit. I passed by rooms filled with happy stories. Rooms filled with healthy, normal babies. Rooms filled with visitors and joy. All-day-long. My room was dark and lonely.
I visited you multiple times per day between force-feeding myself each meal. Between each pump session I would deliver milk to the NICU for you. I’d check on you and hold your little hands. I couldn’t hold you without sobbing uncontrollably. Your prognosis was a mystery. I feared I would lose you. I feared I’d have to make a choice to let you go. In a way, I had lost you. I lost the child I had envisioned I’d have.
You were supposed to be able to take trips to the grocery store with me hitching a ride in a baby carrier. You were supposed to be able to ride in a stroller and go for walks in the neighborhood or hang out in your stroller in the backyard while your big brother played and I tended to the garden. You were supposed to be able to breathe on your own. You were supposed to be cordless. You were supposed to come home with me and cry for me in the night because you needed your mom. Instead I had to leave you with complete strangers every single night. I’d wake up too scared to call and check on you because I was too scared to hear bad news. Every night I was scared I’d get a phone call saying you had died.
It took months but I learned that you could come home and live with your family where you belong. I learned how to take care of you. I made sure I was as practiced as much as possible spending 17 hours per day 7 days per week in the hospital with you so when you came home, I could keep you safe. It was my job to keep you alive. Your breath was literally in my hands. I learned to squeeze the ambu bag. I was breathing for you. It’s an amazing thing yet so, so scary. What if I didn’t do it right? What if I forget how to do CPR? What if I panic and can’t think clearly in an emergency?
Ellie you are so strong. You teach me what true strength is. Some days I wonder how long I can keep up this lifestyle. How can I keep going? Where will I find the energy to keep up with the demands of your needs? How will I be a good enough mom to your brother who is constantly overshadowed by your needs?
There is a saying “it takes a village to raise a child”. Without my village I would be unable to provide for you. Without our village I wouldn’t be able to care for myself or your brother Nathan or your dad. Without our village I would be alone in this journey. But I’m not alone. We’re surrounded by the best friends, family, community, supporters, caretakers and more love than I knew existed. My heart feels like it will explode on a regular basis.
I am grateful.
Ellie you have taught me,
To be strong.
To love unconditionally.
To adjust my sails.
You are amazing little girl. I’m so lucky to have you in my life. Just please don’t be upset that your birthday makes me cry, likely every birthday will. Most tears are tears of joy. Tears because my heart is so full it’s raining gratefulness. Yes, there are a few tears of sadness and fear but mostly today I’m remembering our journey. Our journey through the storm. We have braved so much and I’m so proud of us. I’m so grateful you’re here. You're my shining light.
Happy birthday Ellie.
It is NOT okay that my daughter STILL does not have adequate head support even though I have voiced my concern to many health professionals. It’s not okay that I have so many hoops to jump through to try to give my child ability to do a miniscule number of things that my other “normal” child can do. She can’t even turn the pages of a book or hold up her own head without support. How is it okay that it has taken this long to get help? Why is it okay that the stander that she very much needs for her development took so long to get (it’s not even here yet). We had to wait 3 months for an appointment and then it took a couple more months just for insurance to approve one for her. Umm… excuse me but I’m mortified that we have the technology in this country to help kids get into a sitting or standing position with special tools and support but we can’t get these tools to these kids in a timely fashion. Isn’t this the most critical time in their development?
I’m outraged that we don’t have a better system to help families like ours and even more upset that there isn’t more help for families less fortunate. I have a college degree and if I weren’t so fortunate to have married into a family who has been able to help support me in my educational, and career goals I’d be up a creek without a paddle. I still pay student loans yet I’m VERY limited in how much I can work. Again, I’m VERY fortunate because I was born and raised to be fierce and to give 110% to anything I strive for. I work long days and long nights to help our community and my family. I’m a Registered Dietitian Nutritionist and I’m so passionate about my job. I can’t even explain how much joy I feel when people come back to me and tell me that they have implemented something I taught them and that they are so much healthier now or that they feel better than they ever have. I live for the moments like the one I had the other day during a food demo. An elderly lady told me she doesn’t eat kale because she thinks it’s disgusting. I convinced her to try it and promised she could spit it in the trash if she didn’t like it. She tried it and she liked it AND took the recipe home to try it again at home. My point is, I volunteer in my community and I work but it is VERY hard for me to do these things and to do them regularly and consistently in my situation as Ellie’s mom. What does all this mean to you? We receive Medicaid. We are NOT a drain on the system. We work hard. We give back. We are like many other families out there who rely on assistance just to live an average life and give our children the best opportunities possible. We just want to be normal. Well, and sleep. We want sleep too.
We’re allotted 84 hours per week of nursing care. That’s 12 hours per day. In that 12 hours my husband and I have to sleep, work, run errands and grocery shop. And the kicker… it’s very rare we even have all 84 hours staffed. There is a nursing shortage and with the state of home care, many nurses don’t want to do the work. I can’t say I blame them based on some of the horror stories I’ve heard. However I know our nurses love working with us and they feel loved and appreciated. They are family now! More on that in another post… When we don’t have a nurse, either my husband or I have to be home with Ellie. Ellie must have a trained caregiver with her at ALL times. If we go somewhere in the car, there must be a driver, and a trained caregiver at Ellie’s side. Care doesn’t stop because we’re in the car. If Jeff and I want to go somewhere together we have to take both kids with us (it takes about 1.5 hours to pack Ellie into the car) or we have to have a nurse. This however, is an improvement because Ellie is growing stronger. Not long ago she was less stable and we always needed a second person on hand at home just in case we needed to call 911 or to have an extra set of hands to start oxygen or use the ambulatory bag while supplies were prepped for an emergency trach change. Just imagine how you’d plan your weeks without being able to get groceries or pick up prescriptions or pick up your second child from preschool while having to care for a child like Ellie. This is our reality.
For those of you who know my family personally, you know that we live a healthy lifestyle and avoid addictive and unhealthy substances (okay we do have a little sugar addiction but who doesn’t?). Ellie’s condition is genetic. It wasn’t an accident during birth. It wasn’t because I didn’t care for my body and the growing baby in my womb while I was pregnant. Everyone knows there is risk with having children but this lifestyle and Ellie’s limitations are not what most people think of when they make the choice to have a child. In fact, Ellie’s condition is so rare that she still remains undiagnosed. The doctors and geneticists will continue to study our family and seek answers and other families who may have the same condition. This is called SCIENCE. And thank goodness we can rely on science because without it, Ellie wouldn’t be here and a piece of our hearts would be lost forever.
This morning I finally found the inside part of Nathan’s bike helmet and it’s been repurposed. For now, it’s going to be used until Ellie can get a Headpod. I’m not an engineer but this is my best attempt at giving my child the ability to do more. Watch these videos and tell me it’s still not worth paying taxes to help children like Ellie.
I want to live in a world where people care for others and invest in the greater good.
I want to live in a country where our tax dollars are used wisely and therefore people willingly instead of begrudgingly pay their taxes, feeling like they’ve contributed to something important.
Thanks for listening. End rant.
It should not take this much time to get assistive technologies that can improve mobility, emotional stability, and cognitive development. The process needs to be easier and faster. Kids grow too fast and this time in Ellie's life is important. These tools are vital for her development. And haikus are fun ;)
Courtney is Ellie's mom. She and her villiage have been raising Ellie (and her big brother Nathan) and learning how to live with and manage life around Ellie's physical disabilities and special needs. It's not easy but our villiage is amazing. Thank you to all of you who support us, love us, and provide hope, wishes and prayers.