When you casually ask someone how they are doing, do you really want to know? I find myself wondering how someone would respond to me if I answered that question truthfully. I wonder how I would respond to someone else who answered me that questions truthfully. There are not many people in the world who want to sit and really hear how you’re doing. I am fortunate enough that I have a loving, strong family who support me and Christopher and Kierra through every single heartache. But guess what? They are living this too. It hurts them just as much. We all cried on the way home from Kierra’s appointment yesterday. I just keep hoping it will ‘let up’ somehow but it never does and I think I hit the realization yesterday that it may not. We may always get some kind of bad news at each doctor visit we go to. So… I need to get this off my chest. I need someone to hear me. We all have problems and heartache and bad days. I hope I’ve been an ear to someone who needed it at some point in my life because I need some ears now. There is no way I could talk about this in person with anyone because I cry….ugly cry. I can’t help it. So here goes the past few weeks of our life….
In January, Kierra had two MRI’s done. The first MRI was of her heart….the worst of it was the IVC placement and the low blood pressure worry. I (not a PhD mind you) came up with the idea to “Hey, maybe we should only give half of her heart medication before her next MRI since it lowers her blood pressure” doctor thought that was a good idea. Glad I came up with it. The results of Kierra’s cardiac MRI show that her mitral and tricuspid valves are mildly leaky, there is a tiny amount of backflow through the valves, and that her aortic root size is 3.1cm. To put that in perspective for you, the average size of an adult aorta is 2-3cm. Kierra is 16 months old. It has grown at every check up. We started a new medicine in September so it is my hope that it just did not have enough time to have a big enough effect on her heart. I PRAY that there is no aortic growth at her next check up. She will have to have her aortic root replaced surgically once it reaches 4.5cm – 5cm in diameter. Her valves will have to be repaired at some point as well. The rest of her heart appears to be in great shape.
The second MRI was of Kierra’s spine. While the IVC placement wasn’t wonderful, the entire process went smoother than the first MRI. I knew the scoliosis was getting worse, I could see the muscles in her back twisting and raising. I just did not think that both curves would have DOUBLED in their degree of curvature. DOUBLED. In 3 months. This is considered extremely progressive. The MRI also shows that Kierra has dural ectasia. This occurs in about 60% of the marfan population so it’s not uncommon. It is a widening in the dural sac around the spine. It can be responsible for severe lower back pain, leg pain, urinary incontinence, headaches, rectal pain, genital pain, weakness and numbness. Kierra does show signs of being very uncomfortable when she tries to sit. She doesn’t sit for more than a few moments at a time, she doesn’t like to be in her extra padded high chair for very long. It breaks me. I cannot describe the torture it is to know your child is hurting or uncomfortable in any way. Treatment options for the scoliosis…well I mentioned before in a previous blog about the MEHTA cast. We go in 6 weeks to attempt to put that on Kierra while under anesthesia and see how she does. If she does not do well with this cast, we will be using a brace. I spent a lot of time researching and talking to other parents that have dealt with infantile scoliosis about options etc. and there is a brace they do at the Salt Lake City Shriners, called an EDF brace, that might be a possibility for Kierra. Our doctor here is going to contact them about it. The brace has little hopes for being curative and the cast has less than 20% hopes for curing (within the marfan population, 40% in the normal population). These options are to possibly get some correction to the curves in her spine, but ultimately to prolong having rods surgically placed in her back for as long as possible. Dural ectasia has no treatment other than pain management.
This is our news. I keep thinking, we’re only 16 months into this marfan life for Kierra… what is it going to be like in 16 years? How many surgeries will she have had? God please… please give us the strength to handle this, to help Kierra in every way possible. I know there are many people out there in far worse health conditions, I know that. Maybe it’s not fair for me to complain that my child is hurting in these ways but I can’t help it. I do try to think of the positives, I rejoice that she hasn’t had any surgeries yet, some children have already had their heart surgery by this point. I rejoice that her pediatrician visit a few weeks ago went wonderfully. Kierra is above average in language and intellect. She is doing things an 18 month to 2-year-old would be doing and has been doing these things for quite some time. She knows how to produce animal sounds when asked, she knows a few of her colors (can point them out to you), she says words like caterpillar and avocado ( still very baby-like but the fact that she tires things with multiple syllables is amazing), she puts words (in sign language) together properly like “Daddy, more please” “No thank you”, points to dog’s bowls and signs “Dog water”, she knows where body parts are has for several months actually (nose, eyes, hands, fingers, ears, knees, feet, toes, tummy, belly button, hair, etc.) and the list goes on. She really WOWs us sometimes. And I will brag about this, rejoice in it, whatever you want to call it because I won’t ever be able to tell you that she scored a goal at a soccer game, or that she was first to finish the mile run at a track meet….so I will boast about her mind. Physically, she is still a little on the slow side…she walks with a bit of a limp, she has trouble standing up on her own, she has to pull up on something usually. So any time she does stand up all by herself, we all make a big deal about it and clap for her. She stumbles a lot (toddlers do anyways), her left ankle prolapses so bad that she walks on the very inside of her left foot. She has new orthotics to help with that, coming soon. Her chest is noticeably protruding now. It’s called pectus carinatum. She still has many sensory sensitivities, though she’s come a long way with that. Her sense of touch is very sensitive and she does not like certain textures, (like she freaks out about them), she doesn’t like her hand held (especially by strangers) unless you are helping her get up or walk, a few other things like that. We are still doing physical therapy twice per week, occupational therapy bi weekly. Add in all her doctor appointments and I try to work at the church a little bit every week, it is BUSY. I am tired. Not just physically but in every possible way I can be tired. My heart and mind are exhausted. Even if we’re not at an appointment, I’m thinking about all things related to Kierra. All her medical stuff plus normal stuff, is she being socialized enough? Am I teaching her enough? Am I spending enough time with her? Eating… oh I put a lot of effort into Kierra eating healthy. I look up all kinds of ideas and tricks to get her to eat the healthiest foods I can find. If she is going to only eat pizza today, well it’s going to have kale on it. I can’t remember the last time I actually fixed my hair, I manage to get a little make up on usually but my hair suffers. Ha. I have my own health issues I’m trying to manage. Christopher works so hard, he started a new job recently and it’s an hour drive each way to work. He puts in a lot of effort, time and love with his family. He is so devoted to Kierra. When he gets home, he kisses us both and then spends the rest of the evening playing, bathing, teaching, and loving our baby girl. He is the bomb diggity of Dads y’all.
One thing I count as the most amazing blessing: we are not alone. We have been incredibly blessed with a network of people who have already gone through these exact situations. To be able to get other’s experience and knowledge, it’s absolutely priceless. I thank all of the “Marf Moms” in my life. I could not do this without all of you.
Among all the heartache and sadness, there is Kierra. She is the sun. She is the moon. She is the stars. Every night as we lay her down to sleep, we pray over her and I always thank God for gifting me with my baby girl. First. Always thankful first. If you wanted to know whether or not I could go back and not have a baby….I would never undo it. NEVER. Does that make me selfish for wanting Kierra even though I know she’ll suffer through life… maybe. But I have her and her life is full of meaning. This world, without her smile, would be so dark. When she giggles, I hear angels singing. If I can do anything to make her happy, I do it. If I have to dance on my head, pick my nose, sing “The Wheels on the Bus” until I’m out of breath….consider it DONE. That’s what you do when you’re in love. You do anything. I try to be as strong as I can be for Kierra and in front of her. I constantly research, plan, write questions, e-mail faraway doctors who are specialists, and PRAY. Oh do I pray. I do question why still. I don’t understand. But I have faith that this is all a part of God’s intricate plan. I cannot wait to get to heaven with my little girl someday and find the lives that she touched just by living.
So there…I’ve poured out my heart for today. It feels better. I feel a little relief. I’m thankful that so many of you out there care enough to read all of this. I thank God for friends that call/text and check on us. I thank God for my family, Christopher’s family, giving us TONS of support. Among the thorns, there are roses…so many beautiful roses.