The Question

It happened, and I know it will only be the first of countless times.

Yesterday Harper's school had their annual 1st Grade Patriotic Program. It was so sweet. They sung songs and saluted the veteran relatives and friends of the families in attendance. Norah and I were especially excited to see that their Pledge of Allegiance song was accompanied by signs. (Not true ASL of course, but they were fairly accurate ASL signs using the frozen text of the pledge). I was such a proud mom to see my sweet boy on stage, knowing every word and giving me the occasional sly smiles and sign for I Love You. I also happened to sit next to someone that grew up in Jeff's neighborhood. It didn't take long into our conversation when she realized that she knew Norah's story. The kid's a celebrity, I tell you.

After the event, Norah and I joined Harper's class for some treats and a little social hour. Most everyone left, but Harper lingered with his friends while he also packed up his things to go home. One of his friends had been looking at Norah and I. He asked a few other kids if I was his mom. He finally asked Harper, to which he replied "Yes, that's my mom and my cute little sister." His friend quickly asked the question he was itching to ask someone, "What happened to your sister?" There it was. The first of many times, in just one of many forms that Harper will be asked about Norah's differences. In that moment, I was so proud of my son. He didn't understand what his friend wanted to know. Nothing happened to her. Nothing was wrong with her. He just knows that she is his cute sister. Harper's expression prompted the boy to repeat his question, this time pointing to his own neck. I could see Harper's brain scanning all of the things we've told him about Norah to help him understand that all people are different, and that the only things that make us "normal" are in fact, our differences. He told his friend that Norah needs help breathing with a mask. I of course didn't want to intervene. The boy wasn't quite satisfied, because he wanted to know what on earth was that gigantic white and blue thing around her neck. They ended up being distracted by another boy running around the classroom with a small flag, playing "good Army/bad Army".

There will always be something. There will always be stares and questions. There will be curious people, rude people, awful people, and wonderful people. My top goal aside from my kids' safety, health, and happiness is raising them to be confident in who they are, and I know much of that comes from example. I want us to be proud of one another. Our Deaf Mentor and I recently had a conversation about this - particularly regarding acceptance in being Deaf. She has told me before that watching Harper makes her proud, and that it is priceless to have a sibling who is so supportive of who you are. I don't know what the future holds, but I do have every confidence that this sort of support is going to go both ways. I like to think that Norah will be just as protective of Harper as he is of her. 

A Difficult Week

Those that didn't know me would have thought I was a mom going through a crisis for the first time. They probably would have felt pity for me -  a fearful mom in unexpected and new territory. It was partially true, but the deeper reality was that I was having some serious PTSD. I will however say that I received great comfort from family and friends, who helped me cope.

That first night post-op from Norah's c-spine fusion was awful (though her surgery itself went really well). She was in so much pain. She was able to sleep the next day, but in the middle of the second night, threw up and breathed a bit of it into her lungs. I can't tell you how many middle-of-the-night emergencies we've had with Norah. We moved to the PICU, which was when I started to feel the old nag of emotions bubbling up from living there so long. After some time in the PICU, we moved to the floor. But overnight, again, she began to decline all of a sudden. the respiratory therapist recommended a PICU consult. No. Not again. I didn't want it. "They'll take her. I know they will. I don't want them to come." Those were my exact words. But there was no stopping it from happening. She wasn't getting better, so I agreed to their consult. I was just relieved to see familiar faces come upstairs. It was becoming more and more clear that we were going to have to go back. The doctor who once told us three years ago that our daughter might die, was here to tell me that Norah needed to go back to that place. Back to that place that toes the line between being the most wonderful beautiful place and being the saddest, most horrible place. They wanted to put us in our old room, bed 14, but I asked them not to. I would have begged if necessary, but they had no problem finding another place for us.

Friday was awful. One of her most frequent and beloved caregivers there, a Nurse Practitioner, looked at her with the most genuine concern. I could tell he was worried. He looked at Norah as if she was his own child. I've never quite seen anyone look at her that way aside from Jeff. Sure, people love her, but this was different. It was so touching. He spoke with a somber but hopeful tone. We all had a hard time getting Norah stabilized on the vent. For a bit, I thought we were going to have to intubate her.

I saw something that I've only seen happen with her a few times during a code. No, she didn't code this time. But what I saw was a girl starting to give up. And it scared the shit out of me (no apology for language there). She and I had a talk. I tearfully told her that her mom was going to be selfish. That I couldn't stand it if she left. That I needed her to fight. She couldn't give up. Well, she didn't give up and I am so grateful. This last week+ has been one of the more difficult that we've had in a long time.

Based on her viral panel and presentation of symptoms, she was treated as having the nasty enterovirus d68. Don't worry - if you or your kids get it, it wouldn't likely do you you what it did to Norah - unless you also have airway issues and/or lung disease. It is because of these things that little colds can really do a number on her.

So we've come home with our old friend, the Trilogy ventilator. We're using it at night for non-invasive bipap. Norah was pretty upset when we put her on it last night. She thought that since we were home, that we were done with those shenanigans. The C-collar she has to wear (for four months) was also frustrating for her since she can't lie on her belly. Hopefully it just will take some time to acclimate to this new normal. Again. I'm just happy that we are all together again. I missed Jeff and Harper like crazy. And poor Jeff didn't exactly have an easy time trying to juggle between being supportive at home and the hospital.

As for Harper, the poor guy has been having a hard time. He told me yesterday that he had been sad because he thought that I wasn't going to come back. He was serious, too. We hadn't seen each other in person for over a week, so it was understandable. As much as we try to make things normal for him, it just isn't possible. So we' have some work to do with getting both kids to happy places. Hopefully it happens sooner than later.

Another Surgery

Norah had yet another trip to the OR on Monday. Many (if not most) special-needs moms could tell you exactly how many times their child has gone to surgery. I lost count long ago. This time she went in for her trach scar revision, and it'll be great to see how everything heals. I hope she grows to be proud of her scar and what she's overcome. Our ENT also noted that her mid-airway still has quite a bit of tracheomalacia (her airway doesn't stay rigid & open - think of a straw that is pinched half-way closed). He knows that I love pictures so brought some out to me. Hopefully she grows out of that floppy airway. This particular trip to the OR was particularly stressful because it was her first since having her trach out. But everything went smoothly without any issue. We walked away feeling relieved that Norah wouldn't need surgery for a LONG time.

We were wrong.

We went to Shriner's to follow-up with her Orthopedic doctor. We've known that Norah's neck isn't totally stable (she has excessive movement between C1 & C2) but we've hoped that over time, things would ossify and strengthen & she'd be fine. Turns out, it's gotten worse. We have a bunch of appointments next week, but I'm under the assumption that they're going to want to fuse Norah's neck within the next couple weeks.

I know that fusion will help her be stable, safe, and prevent spinal cord injury. But I am scared. I am scared of pain and her own fear. I am scared she won't want to come off the ventilator post-op. I am scared of permanent injury. I am scared of death. Of course, not getting this surgery could lead to those same things. So I know it is necessary. But that doesn't make it easier.

The thing is: I know Norah can do this. She has been through so much and has shown us that she isn't going to give up. I mostly worry about my own ability to hold it together. I hate seeing her hurt. I hate that I can do so little to protect her from the inevitable pain associated with her medical woes. I hate that our family will again have to taste what it is like to be apart. I hate that Harper has to see his mom cry this way. I hate that he has such worry about Norah in the hospital. He said to me recently, "But mom, I thought we got to keep her. I don't want her to go to the hospital."

So again we go through another challenge together. Part of me has forgotten that life in the hospital. I don't know how we endured those 23 consecutive months. But then the other part of me remembers that time with such distinct clarity that it adds to the anxiety and fear of going through that again.

I'll try to keep you all updated on her upcoming schedule. Here's to strength, bravery, and love getting us through.

Bare Neck

Tonight I saw Jeff doing something that I find myself doing quite often. He and Norah were playing on the floor, when he casually lifted her hair up off of her neck. "Are you admiring her bare neck," I asked him. He sure was. We both do it. We look at her in awe. No trach ties. No trach. No ventilator. No ointments, no skin breakdown, no trach smell. No secretions, no suction catheter, no drain sponges, no CPT. No more hoarding ten different types of wound care products. No more hoarding medical supplies at all in fear that we will run out (which you better believe happened and was AWFUL). I could go on and on.

Truthfully though, while we had it, the trach wasn't so bad. Neither was the vent. I'd go as far as to say that I loved them both. Without them, Norah wouldn't be alive today. Yet we sure love admiring how far we've come. The girl who was stuck in the hospital for 23 months on an ICU-only ventilator took less than a year to shake her life support. Life is good, and we're sure to notice those little things that make it so great.

Preschool Plans

Sometimes I can be pretty cavalier about things. The truth is that I'm not always so confident. Of course I knew that Norah would some day get off of the vent and decannulate. There was never a doubt in my mind. I don't really have a whole lot of doubts when it comes to Norah and what she can and will do. I guess my doubt is sometimes in my own ability to facilitate some of those triumphs. I also have doubts in my ability to cope with everything with a smile. Sometimes I'm not sure if my smile is genuine. I absolutely love my life. It is beautiful and wonderful, and I wouldn't trade it for any other life. I have found fulfillment in ways I never could have imagined. But sometimes when I look ahead, it all seems so daunting. Yes, we've gotten this far - and what a challenging journey it has been. Again, I don't ever doubt that we'll meet our goals. I suppose, like most people, I just get a little tired.

Though she is a very smart girl (sometimes too smart for her own good), Norah is pretty behind developmentally. She turns three in a few weeks but she has yet to walk, stand, or even get to a sitting position on her own (though she's close). She is improving, but her expressive communication is lacking too. We recently made the decision to enroll Norah into preschool at the Jean Massieu School of the Deaf in Salt Lake City. Norah is hard of hearing. Though we have a Deaf Mentor (one of the most awesome people) who comes weekly to teach us American Sign Language, Norah will need even more exposure to ASL if we hope for her to be fluent. As for us, we're working on our fluency too. I truly believe that at JMS, Norah will be a "normal kid". I picture her at a typical school. She tires quickly, so would be lying down a lot. She would have minimal communication with the other kids. They would treat her like a baby, running figurative (and possibly literal) circles around her. She is not a baby, and I remind people of that often. At JMS, she'll be just any other kid there. Those kids are so accustomed to "different" that different = normal. She'll be ready to join Harper at our local elementary school someday, but that isn't just yet. Next month we have another appointment at Shriners to see how her scoliosis and c-spine (neck) stability are coming along. Cross your fingers for us, because her safety at school will be directly related to her neck stability.

Norah's formal list of goals on file is a long one. Will it be hard? Yes. Will we get there? Yes. Heck, I'm just thankful that we are facing these educational decisions and goals. It wasn't too long ago that we were facing different sorts of decisions - those that were necessary for her very survival. It's a welcome change of pace to be thinking about education and development instead of breathing and surviving.

Hibernating

I had been hoping it wouldn't come to this, but it looks like it has become necessary, if not critical. This flu season is becoming progressively aggressive, so we're going to have to make sure that everyone that comes in close contact with Norah, or even in our house, is up to date on their flu vaccine.

The truth is, I didn't get my first flu shot until I was pregnant with Norah. After her diagnosis, my doctors stressed that she could have respiratory issues (ha!), so we needed to make sure our family was protected. I have received the shot every year since, and so have Harper, Jeff, and Norah.

In our lives we are always weighing risk and benefit. Some people have allergies or sensitivities that result in a choice against the flu vaccine. (You can get a preservative-free version, though there are other allergens too.) Others may not fully understand what can happen if they get ill. I truly hope that everyone does research on both sides of the topic to make a fully educated decision on whether or not vaccination is right for them. A good place to start is the CDC's website. We understand that our family lives in this unique world where we are apt to hear more scary details about what the flu can do to otherwise healthy, youthful people (hospitalization, heart and lung ventilation, even death).

I just know that for my family, we need to increase our "herd immunity". Jeff and I have decided that, for our family, we cannot afford risks. I will not tell anyone that they should or shouldn't get the vaccine. I may tell them to consider it, but I will not tell anyone that they have to. However, our company will be contingent on it. Our family has had to live in separate places for far too long to have to take any unnecessary risk of having to live that way again. For those that do not vaccinate, we still hope to keep in touch via other means, and look forward to coming out of hibernation in April!

I get by with a little help from my...

Today I did something probably long overdue. I met with a clinical social worker. The best part? She came to our house as part of the Medicaid waiver program. We already began to scratch the surface of my challenges, and I'm genuinely looking forward to meeting with her every other week. 

We talked about an array of things, and she was ecstatic to hear me rave about the relationship that Jeff and I have. Jeff and I aren't just husband and wife. We are partners. We are best friends. We laugh, giggle, flirt, cry to one another, vent to one another, comfort each other, and work together like peanut butter and jelly. Our strengths and weaknesses compliment one another so perfectly. We work so well because we came into this with a strong solid foundation. I could tell that hearing all of this was a breath of fresh air for our clinical social worker.

What else do "I get by with a little help from..."? The rest of my family and friends. Thank you all for such love, support, and understanding. As hard as it is for me to live in Utah, I sure am surrounded by amazing people, near and far.

Enough

It's been a rough couple of weeks for us: a hospital admission, pretty bad colds for all of us, impossibly painfully tight finances accompanied with lots of unpaid time off work, a huge unresolved misunderstanding with one of my family members (who's love and support I need right now more than ever), dietary issues, and now to cap it off, a really hard-to-swallow questionable diagnosis from one of Norah's doctors. He thinks that she needs spinal fusion surgery at the very top of her spine (C1 & C2). This is huge. This is life altering, and totally risky... and I just don't fully trust in the diagnosis.

So we are going through the motions for a second opinion in Delaware. Yep, Delaware. We are sending her radiology images to them to see if we can prevent a trip across the country with a girl that does not travel well or light. I'm not holding my breath. So, why Delaware? It's all for this brilliant man. His entire population of patients are those with a huge variety of skeletal dysplasias, where as the doctors here only have a small handful of patients with Norah's condition (the many different skeletal dysplasias can be vastly different from one another). So we don't just need specialty care - we need sub-specialty care. There are few places in this country with experts on skeletal dysplasias. People from all over the world travel to see this doctor, and with good reason. It's unfortunate that so few specialists understand Norah's condition... but we are so grateful for those that truly try (like our ENT). So if a costly trip to Delaware may save our girl from an irreversible invasive surgery, I'm all for it. She may still need surgery... but even if she does, I'll rest much easier knowing that only the best hands and minds are taking care of our girl.

So why the title of this post? I've had enough of being sad. I've had enough tears. I can't sustain it anymore. I'm putting on my big girl panties and facing issues with my head up. I'm going to focus on my health. I'm going to take care of my family. Yes, there will be moments of sadness, but I will hold onto the beauty and good in our world. I won't let others get me down. Jeff and I had a wonderful talk last night about what we want, how we want to feel, and how we will achieve our goals. We're so optimistic right now.  And what better teammate than my Jeffrey? I've had enough of the blue, and am ready for happier days. This seasonal change is the perfect time to start, since autumn is my favorite. I'll close this post with the motto of special needs families everywhere: I can do hard things.

A Quick Stay

That was a quick one. We were discharged from the hospital yesterday. It hasn't been the first time that I felt a nagging tug that they were going to make us stay. It is more the sort of feeling you get in a nightmare than a true suspicion. My brain knew they wouldn't make us stay, but my heart didn't quite get the message. I suppose that comes with having spent close to two years in the hospital.

Norah is doing much better now that we have albuterol to open up her lungs. Yes - the same albuterol given to asthmatics. She doesn't have asthma, but she sure benefits from the bronchodilator. Norah is so happy to be home. That night in the hospital, she (we) only slept for two hours then an additional hour and a half. The crazy girl fought sleep during the day, too. She was too worried that the masked and gowned people would come do something to her in her sleep. Of course the coughing and crying didn't help.

I'm so thankful that I listened to the mom-voice in my head. I simply knew she was going to need to go in. And I listened before things got too scary. What did it turn out to be? Rhinovirus. Yes, the common cold. But for us, the common cold is scary. It used to always mean a PICU stay. Norah is getting stronger, but apparently a cold still means a stay in the hospital. It was a mostly inevitable kindergarten cootie. I just wish I could program everyone's brain about illness prevention with hand hygiene, covering coughs and sneezes, sanitizing surfaces, etc. Alas, I know that I can't expect everyone to do it. So we'll continue to do our best to protect ourselves.

Thank you all for your well wishes these past couple of days. Though we'll be mostly hiding at home until we're completely better, we are definitely on the mend, and glad to be home.

THAT Feeling

It hit me while waiting for Harper to be dismissed from class. At about 1120 yesterday, I got the horrible feeling that Norah would be admitted to the hospital. She was starting to show hefty viral symptoms, and was inconsolable. I spent much of the day on the phone (in between kindergarten homework), and it was decided that Norah should at least go in to see her general pediatrician in Layton. 

To my surprise, at that very moment, one of my best friends Jaci was standing at my front door. We rushed to the pediatrician, but were late anyway. During the course of the appointment, Norah began to deteriorate. By the time we were loaded into the ambulance, Norah's heart rate was peaking at 180, and she was on 6 liters of oxygen. 

Though she stabilized a bit on the way there, she slowly became worse and worse. We decided to err on the side of caution by admitting her. I'm so glad we did. She had a rough night, spending more time crying and coughing than actually sleeping. 

So here we are just waiting. Waiting for her to get past this cold so we can take her home again. We were two days shy of three months at home without any admissions to the hospital. But here we are.. Hopefully not for long. 

There is a huge ray of sunshine though. My darling friends, the Packs, welcomed their three gorgeous daughters into this world. They are perfect and they are beautiful. They are at the hospital next door, but my desire to for them to be safe and healthy far outweighs my urge to run over there and snuggle on each of them. Hopefully they get to go home soon! 

Checking in

Yes, things have been beyond busy. Harper is loving kindergarten, and Norah is blossoming at home. She is more affectionate, sassy, and mobile than ever before.

Right now she has a cold, which, while dangerous, will hopefully be recovered quickly without any trips to the hospital. Her suction and oxygen needs have gone up, but lets just hope it stays manageable. 

We are still so appreciative of life at home with our complete family. Harper will randomly talk about how we get to "keep Norah". He often talks about how much he loves her, and how beautiful his sister is. 

Though blog updates may be sparse, you can always peek in on my Instagram photo feed to the right on the (non-mobile) site. Thank you all for your love and well-wishes!

PTSD

I've heard multiple parents of medically fragile children talk about PTSD (Post-Traumatic Stress Disorder). It's something I've learned a bit about over the last two years. I take anti-depressants and have lived with the chronic nightmares, but every now and then, PTSD rears its ugly head at unexpected times. I don't mind if this long post goes unread, I simply need to get out my thoughts and emotions.

Let me start by saying that private duty nursing (PDN) has been a huge help. Aside from some scheduling issues, we have been able to get (a little) more sleep, and pass off a few duties to Norah's nurses. Norah is pretty stable at night. She only needs monitoring, suctioning, humidifier refills, and assessing throughout the night. To that, we've added some cleaning items, a couple feedings, a respiratory treatment if she wakes before the end of the shift, and the like, to the nurses' duties. It is important that Norah gets plenty of rest at night. We give her more ventilator support at night than in the day. Every little activity during her waking hours are a workout, so we need her to conserve her energy as much as possible. After all, growing is a key part in her overcoming her respiratory issues.

So last night, we had a new nurse. I trained her but had the nagging feeling that I was forgetting something. Also, she had very few questions. (Yes, I am now going to put a written document together detailing Norah's needs so I don't forget anything in the future.) I fell asleep around 1230 or so, but woke at about 330 to the sound of Norah's labored breathing in the monitor. It was the "sound" of her crying. I went downstairs to find that our new nurse had just completed trach cares (cleaning the stoma/hole and neck, changing the trach ties, and placing a new drain sponge). What I neglected to tell the nurse, was that we simply let Norah sleep through the night. She's always been a great sleeper. She may wake a couple of times if she needs to be suctioned, but will otherwise snooze sweetly. We need her to.

She commented that Norah didn't seem to like trach care. Norah was sobbing. As I comforted her, I kindly said that she hated trach cares in the hospital, but since coming home, Norah doesn't mind when I do it. Jeff has said many times in these last weeks that he is amazed when watching me do Norah's trach cares. She looks up at me with trust in her eyes. If she cringes at all, I talk to her sweetly, or sing to her gently. She seldom cries when I do her cares.

So Norah was traumatized, taking in short gasping breaths. I was traumatized. I had found my girl beyond upset, and the nurse stepping away with the medical supplies. Something about it stabbed my heart. Then I realized what it was. You see, I don't blame the nurse. She was only doing her job to the best of her knowledge, training, and skill. After all, it had been my responsibility to tell her that I did the twice daily trach cares during Norah's waking hours. The distress that I felt was a culmination of all of the times that we've had scares with Norah. It was every time she turned blue. It was when she died for nine minutes. It was every ambulance ride. It was the middle-of-the-night phone calls from charge nurses, telling Jeff and I that we needed to come in. It was her inability to vocalize her fears and pain. It was helplessness while watching her struggle. It was every painful procedure she endured while we did what we could to soothe her. It was sheer exhaustion caused by two years of stressing about my daughter's life. It was PTSD. (I could link many more old posts, but you get the point.)

The nurse was quite well-intentioned when she verbally confirmed that we were new to private duty nursing. When I said that we were, she stated that when Norah gets "better" about trach cares, that it will help give us acuity points. For each item requiring the nurse's attention, Norah gets a certain number of points. These points are what help qualify her for the Medicaid waiver. It allows Norah to have medicaid due to her technology dependence instead of her family's financial status. There is no way I will put Norah through that in the middle of the night - even if it means that we had to give up nursing and Medicaid altogether. This isn't about relinquishing control. Norah needs security, confidence, and sleep much more than she needs those other things.

I like to think that we are very easy-going about Norah's cares. We aren't neurotic about specific things at specific times, or about nurses doing certain things a certain way. We've learned the hard way that the few "rules" that we have must be documented and outlined. Who would have thought that letting her have a good night's sleep was one of them?

A Long Day

With Norah's skeletal dysplasia, c-spine (neck) instability is a known risk. We had been told quite a few times that her x-ray films and MRI were "reassuring". However, we all wanted to formally clear her for c-spine stability, so we were referred to a pediatric neurosurgeon to take a look at the images of her neck. We generally prefer to cram in as much as we can when we visit Primary Children's... but this particular day was more taxing than most medical transports.

Little girl in a big machine

Prior to her appointments, Norah was happy and playful. Our first stop was imaging, for a CT scan of her neck. We had quite the entourage in there: a doctor, a nurse, a nurse practitioner, a respiratory therapist, two imaging techs, and the SDCH medic. She is really difficult to sedate. As expected, she resisted the initial medication, so we had to place an IV and give her something more. This second medication was really hard on her, as I'm pretty sure its effects were scary. Her heart rate shot up, but she was such a good brave girl. I had to hold her head while she went through the machine because she was bobbing her head with each breath, a definite sign of distress.

We spent a little time in sedation recovery, where she spiked a temperature. We stuck around there long enough for a dose of Tylenol and a quick (tube-fed) breakfast. We went to see the neurosurgeon next, who had ordered the CT scan. Despite having the detailed scan, he wanted additional xrays. By that time, we were running late for the orthopedic appointment, which was the original thing on our agenda for the day. So we went up there, took a hip x-ray in the clinic, then had our consult. We then went back down to imaging for the additional x-rays of her neck. Norah by this time was beyond upset, exhausted, and uncomfortable. She was tired of being poked, prodded, and forced into positions that she wanted nothing to do with.

We went back to neurosurgery yet again. After a long wait, we finally got back in to see the doctor. He gave us news that we did not expect. It was his opinion and diagnosis that while her cord looks to have plenty of space (so is not compressed), Norah has an unstable c-spine. We had gone there to get cleared, but were given the opposite result. He answered my questions (I could go into detail if you like, but this post is long enough as it is), which I greatly appreciated. But here's the thing: while I don't doubt that he is an extremely skilled doctor and surgeon, the reality is that there are no skeletal dysplasia specialists in Utah. Sure, this doctor may see every child in Utah that is even remotely similar to Norah... but that's going to be a tiny handful of patients.

Let me back-track a bit. What does c-spine instability mean? It means that she's at heightened risk for a spinal cord injury. What could happen with a spinal cord injury that high up her vertebral column? Well if something really bad was to happen, it could mean paralysis (from the injury all the way down) or death.

That isn't going to happen to Norah.

At this point, we've decided to stay the course (meaning no immediate surgery, and no collar). We'll go in every 3-4 months for more xrays (don't even get me started on the topic of radiation exposure) and a follow-up with that doctor. We'll make sure that Norah isn't looking worse. Hopefully she will improve. Hopefully she won't need fusion surgery. Hopefully this doctor is wrong altogether. Hopefully he just doesn't see enough of these skeletal dysplasia kids to understand that they are hypotonic, and every part of Norah's body is expected to be floppy for awhile. Hopefully he just doesn't understand that Norah's bones are and will be late to ossify, and his films don't show the cartilage  Hopefully we don't have to go all the way to Delaware for a second opinion.

Such a long day

One of the most draining aspects of the day was what it did to Norah. She was miserable. My heart broke every time she would reach for me to take away the sad, scary things that were happening to her. But I couldn't take those things away. All I could do was try to soothe her with words, try to distract her,  and tend to needs like suctioning, wiping tears, fanning her overheating body, and trying to help her be as comfortable as possible. I hate feeling helpless. Even worse, I hate that she wasn't getting the remedy from me that she was asking for. I'm supposed to protect her. Of course I know that no one can protect their children from everything... but it upsets me that she has to go through this. It upsets me that she doesn't understand why these things have to happen to her. It upsets me to think that this is her life, and this is all she knows. Our world lies within hospitals. These people and machines help us to keep Norah alive. It's a rocky start to what will be a trying life of more doctors, surgeries, interventions, and everything emotional that goes with it. But it's a life, and we do our best not to take that for granted. Though we often feel so weak, our strength comes from Norah's light. It comes from knowing that Norah is destined for greatness. For now, all we can do is fight for her and love one another with an intensity and purity like none other.

Stoma Revision

Every few weeks, Norah's trach tube gets changed out. It helps prevent infection while also helping make sure the tissues don't decide to grow too much around her trach. Norah is NOT a fan of trach changes. I think they are painful and a bit scary for her. Without going into gruesome detail, I'll just say that blood, sweat, and tears are almost always involved involved. A few weeks ago, when they respiratory department tried to change her trach, they could hardly get the old one out. Which naturally meant that the clean one could barely get in either. Remember what I just said about tissues growing around her trach? That had clearly happened.

They called our ENT, who got us into the operating room that very week. He is so amazing, and takes great care of us. We were surprised at how fast everything happened, but before we knew it, we were prepping for another overnight stay in the PICU. In addition to scopes and removal of that recurring granuloma tissue, our ENT did a full tracheal (airway) stoma (hole where her trach goes) revision, where he basically cut away scar tissue and opened her stoma wider. Hopefully this will slow down that dang granuloma growth, too.

Resting after some morphine.

Recovery was much more difficult than past trips to the OR (this is Norah's sixth, for those keeping track). Her stoma was raw, but is now healing nicely. She was in so much pain when she woke from anesthesia, that we had to give narcotics this time. She would just restlessly toss and turn in my arms, crying from the pain. Fortunately, she perked up right before I went home, and we had one of our most favorite nurses ever that night (love you, Andrea). I was able to go home and rest easy that night because I knew she was getting the best care possible.

Post-op playtime after finally feeling better

As far as progress goes on getting her home, she is up to 14 hours on the home ventilator, with 3 of those being in a mode that simply supports her breaths with pressure, but makes her do much more of the work. She still has a lot of growing to do before we can talk about a homecoming. But I will say that since it won't likely be before her second birthday, we're already thinking of a day trip home for her birthday in July.

I'll do a separate blog post soon, but many have asked me about the iheartcolum5k, benefiting our sweet Norah. The facebook page is here, and the website for registration is here. Hosted by the Pack Family, it will be held on Saturday June 29th at 8:30am, in American Fork, Utah.

Feeling Sick

This post is coming to you from a quiet, cozy corner of Norah's hospital room at SDCH. She's had intermittent viral symptoms over the last week or so. Today her heart rate was up, and she had a fever in the neighborhood of 102F. Right as we (Jeff, Harper, and I) were leaving, she started bawling, then she threw up.

Without a word, I held up my keys to trade with Jeff. We are all too familiar with this routine of division. He took Harper home so I could stay here with Norah. She's sleeping soundly in my arms now, but felt so crummy that she was shaking earlier.

Here in this moment, it's hard not to dwell on wishing I were rocking her in her bedroom at home. How I'd love to snuggle her all night, and be right by her side anytime she needs something. But soon I will have to slip her back into the hospital crib. I will have to rearrange her tubes and wires. I will gently kiss her cheek as to not wake her. I will have to whisper goodbye in her ear, tell her I love her, and that I will be back tomorrow. I can hardly stand to wait for the day that she comes home. Someday... At least I can say that much.

Back on Track

Norah's record for tolerating the home ventilator came before she had two trips to the OR, and before she came down with ventilator associated pneumonia in December. It was 13.5 hours continuous hours. She still hasn't beat that, but she is finally back on track. For the last couple of months, she has only been on it for 4-5 hours at a time. However, she is now tolerating 10 hours every day.

The plan is to re-evaluate her every two weeks, and increase her time on the home ventilator by one hour if she can tolerate it. With as well as she's been doing, I'm hopeful that we won't have a problem bumping her up when it's time. That is... so long as we don't have any sort of setback like we seem to keep having.

So she's that much closer to being able to come home. The unfortunate thing that we just found out, is that the FDA's rule for using the Trilogy (her home vent) at home is that she be 10 kg (about 22 lbs). Right now she weighs in the neighborhood of 7 kg (15.4 lbs), so we still have another 3 kg to go (6.6 lbs). That is a LOT for her. So we may not make our goal of getting her home by the time she's two. But we can still hope.

We have quite a few resources to help keep her weight gain on track. It's a little tricky, not only because of her skeletal dysplasia, but also because her work of breathing burns quite a few more calories than the average child her age or her size. Her growth goals are half that of a child her age. She still gets pretty much all of her calories from her g-tube, and we'll probably have it for quite sometime. I'm not so worried about it though. To be honest, it is really convenient. Of course we are still working on her oral feeds, but I'm quite the advocate of g-tubes when the application is right. We're taking the gentle approach with Norah. We encourage her (she LOVES Cheerios), but don't push too hard when it comes to anything oral. It isn't easy for her, and we don't want to push so hard that we end up going backwards.

I'll keep you all updated on her progress with the ventilator. Hopefully we'll continue to move forward!

Living on Life Support

Most people think of the term "life support" as the means to an end. Perhaps that is true in most cases, but not ours. To us, "life support" is the means to a beginning. Norah recently had her half-birthday, turning 18 months old. She is definitely a toddler. She throws fits. She gets frustrated. She gets annoyed with her family. She wants to do everything her way and on her own terms. But yes, she is still on life support. She has a trach, a ventilator, a long-term feeding tube, and enough gear to start her own medical supply store. She lives in the hospital still, and our hope is to get her home by the time she turns two years old (but who knows if that will actually happen). While we try to live life fully and happily now, we sure look forward to breaking free of life support.

Jeff and I often daydream about our future. There are so many unknowns, as with all lives. We wonder if Norah will still be dependent on these medical devices when she goes to school. We wonder if she will choose American Sign Language or spoken word. We wonder when we will hear her sweet voice for the first time. We wonder what it sounds like. We wonder if she'll grow to be proud of her challenges, or embarrassed by them. We daydream about the day she is decannulated - the day she gets her trach out. It will be an overnight stay in the PICU, and it will be coming full circle. We wonder how old she'll be. At first we pictured her toddling around, all of her favorite nurses chasing her around the unit. But now maybe she'll be coloring in her favorite coloring book. Maybe she'll be reading a Harry Potter book. Maybe she'll be painting her toenails and texting her friends about how bored she is.

We daydream about Norah and Harper taking care of one another. He will protect her, and she will protect him. They will fight, they will laugh, and I can only hope that they will be close.

There is certainly another sort of life support aside from the medical type. There is the life support that requires the love of those around you to lift you up and help you get through tough times. There is the type that creates wonderful memories to draw upon when life gets difficult. Right now we live on both sorts of life support- the medical and the emotional. It is difficult, frightening, and completely beautiful.

A very wire-and-tube-filled photo of Norah during her recent
PICU stay last week. Her hair is amazing.

Someone I Love

I recently read a lovely poem on a friend's blog that I felt compelled to share.


Poem by Lori Hickman

Someone I love relies on me in ways you will never understand.

Someone I love endures pain and challenges that break my heart and renew my spirit at the same time.

Someone I love is unable to advocate for themselves for things that most of us take for granted.

Someone I love will never have the opportunities that every child should have.

Someone I love will need unconditional love and support after I am gone - this frightens me to the core.

Someone I love encounters pity, stereotyping responses, and prejudice at every turn, because they look, act, and/or learn differently than others.

Someone I love has needs that require me to allow "outsiders" to have power and input in areas that should be mine alone to meet.

Someone I love will continue to look to me for everything in life long after other children are able to assume a place as part of the world.

Someone I love has needs that require more time and energy than I have to give.

Someone I love has needs that mean I am not able to meet basic needs of my own.

Someone I love has needs that have become the driving force behind major decisions my family makes.

Someone I love has changed me in ways I will never be able to describe.

Someone I love has taught me about love and about the really important things in life...

And still others don't understand what it is to be me.. they aren't living in my skin.

Tracheomalacia

Norah went in for yet another removal of tissue (granuloma) blocking her airway around her trach. Our ENT used a different method to remove it, this time actually taking out her trach tube to get at all the problem tissue. When he did, he noted that her tracheomalacia is much worse than he had originally thought. Basically, this means that her trachea (airway) sort of collapses instead of staying rigid and open like a straw. Every time he's looked in there before, it has been with the trach tube in there, so it helps stent open her airway. The only real cure is for Norah to get bigger and stronger. While growth is particularly slow for Norah and her diagnosis, it has definitely been the trend for answers to her medical problems.

What does this mean? Sigh, I don't fully know. But I do know that she will probably have the vent and trach longer than we had originally thought. I suppose we are already past that 12-18 month projected time with a trach. But if life with my girl means life with a trach and vent, then that's just the way it will have to be. We'll take her no matter what comes with it. At this point, it is all part of her, and these life-sustaining technologies have become a part of her that I am so very proud of.

Once in awhile, I am reminded of how great we have it. Other times, I am reminded that life is pretty tough. But it's not just our lives that are trying... life's difficulties are just part of the human condition. Everyone has their complaints - but as I've always said, should you ever feel down, take a visit to your local children's hospital. You will see hope and courage like you would not believe.

Recent losses in my and Jeff's extended family have reminded me that, while life is beautiful, it is also so sad, and so hard. So everyone, be careful out there. And take gentle care of those that you love. Life is fleeting, and we should do everything we can to take advantage of the beauty and love it has to offer.

A Beautiful Language

Norah is feeling so much better these days. She is more herself than she's been in several weeks. She's perky, playful, and is finally back to doing four-hour sessions on the home ventilator, twice each day. We've decided that a fair goal is to do everything we can to try to get her home before she turns two years old. Babies should get to come home before they are two years old, right? Well, I suppose that she isn't a baby anymore. She is very much a toddler (though she doesn't "toddle").

We're really grateful for many technologies that help our lives, including Norah's hearing aids. We just have to get better at putting them in consistently (us and her healthcare team included). Norah's hearing loss is moderate, which is simulated here if you're interested in getting an idea of what her world may sound like. Set your speaker volume by playing the "Normal" sample then adjusting as necessary. Then you can play the "Moderate" sample to see what various sounds are like for Norah without her hearing aids. Some of them make me a little sad, especially the music and nature sounds.

Like all parents, we want to give Norah everything that can possibly help her. Since she has been unable to speak with her trach (we've tried speaking valves to no avail), and has this hearing loss, we're learning sign language. She may decide to use it later in life, or she may choose to drop it. Either way, we want to give her every opportunity to learn and develop. To see if we were interested in their services, we were visited by a mentor from the Deaf Mentor program through the Utah School for the Deaf and Blind (USDB). Norah was nothing short of enthralled. She was enthusiastic about the deaf mentor, and even "babbled" by waving her hands around intentionally to simulate signing. Norah let us know that she NEEDED this. So we signed up, and should hopefully start soon. We'll be visited by a mentor once a week for three years... and it's a free service!

Norah's hearing loss is caused by her skeletal dysplasia. I could go into further science-y detail, but I won't bore you. I tend to geek out with this sort of thing. I'll give you the simple version, and just say that her hearing loss is permanent. So we're learning American Sign Language (ASL) from books, our USDB coordinator, speech therapist, my new favorite website Lifeprint, and soon we can add our deaf mentor to the list. We're really excited to learn this beautiful language, and Norah is too! Some of the ASL signs she has done are: eat (her first sign long ago), thank you, more, all done, bath, mama, dada, want, play, as well as her own version ("home sign") of "leave me alone". I have been teaching Harper how to say "Dad crazy". Hopefully Harper will have fun with it, too.

I hope you are all doing well during this exceptionally cold January (I heard that in Utah it's the third-coldest on January on record). My intention is to be back to blogging more frequently, but we'll see!