I get A LOT of questions about our life, both on social media or through emails and often repeats, so I decided to compile some of them in one place and actually answer. 😂
Q. What does an average day look like for Avery?
A. There's no short way to answer, so I might write a full blog post for those interested, but typically it is exactly the same: morning bath; trach care (done 2+ times a day to keep the skin under her ties in good condition- scheduled twice a day, but more often if she vomits or drools excessively); 3 extended bolus g-tube feeds; an hour of therapy with PT, OT or ST; one 3 hr nap; tummy time/play time with siblings; dinner at the table with the family (playing with/exploring dry or wet foods for sensory play); feeding therapy with yours truly (eating baby food/drinking from cup); outside time; evening bed-bath; sleeeeepy time.
A consistent schedule markedly reduces her anxiety and irritability. She participates better for every therapy session or activity when it's at exactly the same time every day. Kids thrive in consistency, even when the circumstances aren't ideal.
Q. What's her prognosis?
A. We're not entirely sure. Since we have no genetic answers, we don't know what kind of syndrome we're dealing with. Some syndromes can have an underlying cancer gene or are associated with organ failure by the teen years. If you think of a gene as a messenger, when a gene has mutated, it sends incorrect messages to the recipient (or part of the body). For cranio syndromes, the FGFR 2 (and others) gene sends the corrupted message to close the skull sutures, causing an abnormal skull and face shape. When you can isolate the mutated gene, you may be able to predict where the incorrect messages are being sent. In Avery's case, we can't find a mutated gene, so we have no idea what parts of her body could be receiving incorrect signals. My best case scenario is that Avery's mutation is in a yet-to-be discovered gene associated with Crouzon syndrome. If that is the case, her prognosis is excellent, with a full life expectancy, normal intelligence and relatively normal appearance by adulthood.
Q. Will Avery's trach be removed?
A. Yes! She's on a ventilator at night for apnea (her breaths-per-minute slows considerably when she's in a deep sleep), so we can't proceed until she no longer needs the vent, but once we have a sleep study (scheduled for September) that confirms she's no longer having apnea, decannulation talks can resume. During the day, we can cap her trach (which means she's breathing entirely through her mouth) with no issues, so that's a great sign. We've kept her trach size the same since she was 8 weeks old, so she has a lot of room around the trach to pass air through her airway. I've been firm about not up-sizing her trach since the beginning because I'm so passionate about her being able to use her speaking valve for most of the day, and that has worked out perfectly.
Q. Is Avery on oxygen?
A. Not at all. We have oxygen and emergency equipment in the house and in our travel bag at all times just in case, but I don't remember the last time she needed oxygen support.
Q. Do you have any help?
A. Yes! Avery has a night nurse most of the time (our weekends are spotty which means I've had to be awake to supervise her throughout the night maaaanny times) who we adore and a nurse who is with us in the morning that has also become like family. They administer meds, start feeds, change her linens and bath her. We have gone through 10+ morning nurses in the last year because home health is a horror show. It's AMAZING, LIFE-CHANGING when you find the right nurses. Don't settle.
Q. How did you create a new normal for your family?
A. That took so long and looks different for everybody, but it really happened when I became proficient in ALL of Avery's care. I remember being so afraid of being alone with all the kids by myself in case Avery had a plug in her trach or needed to be resuscitated or even have her trach ties changed, but once I mastered all those, being home with all the kids and no nurse felt so freeing, so normal. Avery's care certainly makes everything harder, so I can't even fold the laundry with her next to me without all of her equipment (picture all of your everyday tasks and add pounds and pounds of medical equipment and catheters and planning just to walk into the next room 😂), but I've found a rhythm that works. Planning is huge. Organization is huge. Getting up early is huge. Staying home and LOVING YOUR HOME, even when there's a stranger frequently present is huge. Home is not a prison. It's a place to learn a new way of life in safety, in control. Letting go of the need for perfect decor (helloooo ugly medical equipment) is hard but necessary. Do only the essentials while you're trying to find your new normal. Master those and then add in the extras over time. I think I'll write a book on this subject. 😉
Q. How do you balance mothering a special needs baby and other healthy children?
A. Time management and intentional quality time have been the focuses. My son's love language is quality time, so he NEEDS me to spend time with him, JUST him, in order to feel safe and loved. On some school mornings, we get up at 6am and go out for breakfast before I drop him off. I always tuck him in and lie down with him for a few minutes before bed every night (I put him to bed last so that there's no rush). I write him notes in his lunchbox so he knows I see him and think about him. We eat dinner together at the table every night and the focus is not only Avery so that the other children feel heard and noticed. Lolly is home with me all day, so we play, read, go to the gym, EAT (always, always eating with that girl 😂), go the park, etc. until we're absolutely sick of each other. She gets a lot of focus. I don't know what her love language will be, so I try to do it all for her at this stage. She gets lots of leg massages before naps and bedtime with her favorite lavender lotion (she is OBSESSED with lotion and oils and has been known to swipe lotion from the nurses' bags 😒). She helps me clean. We even get ready together in the mornings, as she mimics me brushing my teeth or applying make-up.
Q. How the hell do you stay sane?
A. This question in particular really cracked me up, because I'm not totally sure I am sane??! Kidding. I would say that self care is really important. I figured out what was fueling to my soul and incorporated a bit of that in my every-day life so I always felt like I had something to look forward to. There's not a single day that I don't visit the lobby Starbucks when Avery's in the hospital now because I discovered that that one luxury made the worst days feel a little more manageable. I'll put more of this in that book too, because I could write on this forever. 😉
Q. Do you want to have more kids?
A. No, no and no. I only want to experience one Lolly in my lifetime and birthing another is just too risky. 😂 But seriously, after having two children born with severe birth defects that don't seem to be related (but they MUST be because those odds are just ridiculous, right??? am I the only one who thinks that??!!), I am not convinced that my genes are entirely safe. Also, I have a heart for adoption, so I would like to pursue that at some point. I would love to adopt a baby with Down's Syndrome or Apert's Syndrome. After seeing what Avery has gone through WITH a loving family, I cannot imagine a child experiencing that kind of life alone. And I figured out that I'm pretty alright at this special needs parenting thing. 😊
Q. How did Macson have heart surgery with no scar?
A. 😂 I don't know why that question always makes me laugh, but it does. Maybe because I picture people zooming in on his photos, desperately searching for a jagged scar on his chest.
Because Macson was so much older and physically larger than the average child who is diagnosed with coarctation of the aorta (normally found in newborns that require a sternotomy), they were able to remove that section of aorta from an incision under his ribs (thoracotomy). He has an inch-long scar on his chest where his chest tube was placed and a 6-inch scar along his ribs on the left side.
Q. What surgery does Avery have coming up?
A. On August 3rd, Avery will undergo another cranial reconstruction, this time on the occipital portion of her skull. We've remodeled and partially corrected her forehead and one eye socket; removed some skull and bits of vertebrae from her neck (and various other brain surgeries), but this upcoming surgery will help improve her overall head shape and give her brain more space, which is always the main goal with craniosynostosis.
Well, it's been fun, you guys. If you are still reading right now, you seriously deserve a medal because this post is LONG. I know there are so many more questions that I should answer, so we'll have to do this again soon.