I have another daughter

I love the book Wonder. It's about a boy named August, who has a gene mutation that caused physical abnormalities in his face, and the various relationship dynamics in his life. There is a line from the viewpoint of Via, the sister, where she says, "August is the Sun. Me and Mom and Dad are planets orbiting the Sun. The rest of our family and friends are asteroids and comets floating around the planets orbiting the Sun." This quote strikes me to the core because I wish so hard it wasn't true. But so much of our life and my work and even this blog revolve around this stupid disease.

Growing up, I knew a girl named Sarah. We ice skated together and went to the same middle and high school. We weren't friends but we knew each other. She was in the popular crowd. She wasn't the prima popular, but the wing woman and side kick. I had known of her for probably 11 years before I knew anything about her family. As a senior, I worked at a videographer's studio and was putting together a photo montage that Sarah's family had ordered for her graduation. Her younger sister has down syndrome. And I never knew it. I probably hadn't paid attention to who was part of her family at school events and she didn't tout it openly.

It makes me think of Via in Wonder when she starts high school and doesn't talk about her brother and even tells her new friend that she's a only child. It makes me think of my other daughter, Morgan. She's an incredibly bright seven year old. Morgan was probably four years old the first time we explained that all the doctors visits were because Kylie's heart is special and it doesn't quite pump the way everyone else's does. When she was a little older, we added that there are arteries in her body that carry blood and the medication Kylie takes keeps those arteries open so her heart doesn't get too tired. And that medication is different than vitamins.

I still remember the day Morgan asked if Kylie was going to die. She and I had been talking and I had to explain to Morgan that Kylie isn't going to get better (unless researchers find a cure in the future). And it was so painful to see that veil of innocence taken away from her. It doesn't help that so much of my Facebook feed has pictures of kids in hospital beds. We were watching a video today of a machine based CPR and her question was, "Will Kylie need CPR?"

I worry too much sometimes at the toll it takes on her. She's often so aware of my exhaustion and I can tell she goes out of her way sometimes to avoid needing things from me. And it's not fair. She didn't choose this; none of us did. But I can't take it away either. I read in a two separate books that during childhood, we develop coping mechanisms for pain, disappointment and trauma and they often become some of our most natural and powerful strengths. I feel like I see glimpses of that when Morgan becomes so self reliant and resourceful. She almost has a innate reaction of not wanting to be instructed on how something should be done. She has melded these traits into another natural talent she possesses.

She has an imagination that is beyond my comprehension. She can create realms and stories and just disappears into a world of her choosing. Even when she was little, she was the kid that would take pieces from each play set and put them together into a new story (which drove my organizational mind crazy) and I'm so glad I let her. And she's an incredible artist. She also reads three grade levels above her. I can't keep up; I just hand her the bag at the library now and say, "Enjoy shopping."

She is generous and kind. She always wants to buy things for people or give her money to her brother or help us pay the bills (we don't let her pay our bills). I want to, and need to, push against the natural gravitational forces that this chronic illness bears downs on me. I want to carve more time out to spend with this amazing girl because as she grows up, she's going to be unstoppable in whatever world she creates.

So cool and over my head

One thing I love about the PHA conference is that they open it to medical professionals and patients and caregivers. It's this giant melting pot of super-knowledge, interaction, and feedback. In one of the dinner sessions, the speaker was talking about something so cool, I barely understand it. So I thought I'd share the microscopic amount I understood in the 30 minute presentation :)

Researchers are studying certain gene mutations on something called bmpr2 (bone morphogenetic protein receptor type II) and it's 33 relations that are located on chromosome 2. They seem to have a big impact on the development of pulmonary hypertension. It took years to find these receptors but now a research team is running four different trials to learn more about the role these receptors play in PH. This could lead to a better, faster, and less invasive way of diagnosing PH as well as a new avenue to explore cures to this awful disease.

Science is so cool! I should have paid more attention in biology....sorry Ms. Myers. 

The conference I attended was through the Pulmonary Hypertension Association (PHA)

New Hope

At the end of January, we went to visit a pediatric pulmonary hypertension specialist at the Lucile Packard Children's Hospital at Stanford. The visit was the result of 7 months of asking, pushing, paperwork and phone calls. And it was worth it all. We were in Palo Alto for about 24 hours. Our visit lasted over 5 hours but not once did we feel rushed, ignored, or uncared for.

We gained so much while we were there. The team confirmed the thoughts and recommendations of Kylie's cardiologist here in LA. It's comforting to know your doctor is knowledgeable. Kylie is now part of two research databases. It doesn't give her any immediate leg up right now, but the seeds are being planted along with over 1000 other children that hopefully we'll soon be able to see trends and links for kids with PH. I got emergency numbers if something would happen, and they're in our phones now.

The best take away is that we've expanded Kylie's team of care. There are now 6 more people who have met our little miracle and are going to walk alongside us on this journey. Their knowledge is expansive, their experience, immense. We left with our questions answered and a solid to-do list.

I asked Ethan on the way home what it felt like to him. He described it as being in a battle and then you're joined by a group of others that relieve the weight of fighting the enemy alone. Or grappling and then having someone come along and tag you out while they take over. You never realize how exhausting going it alone is until you're surrounded with support.

For me I felt like the girl who comes home and falls into the overstuffed arm chair and can't stop gushing about the new boy she just met. I was flying on uber-hope. The catharsis of emotion was so much I couldn't help but let the tears of release fall as our friend drove us back to the airport.

When we got home, there was work to be done. And from all that work, more work.

Never be lazy, but work hard and serve the Lord enthusiastically. 

Rejoice in our confident hope. Be patient in trouble, and keep on praying.

Romans 12:11-12

We met with Kylie's cardiologist and started the paperwork for Kylie's new medication. It is a specialty drug called Tracleer. It falls in a class of drugs called "ERA's" which stand for endothelin receptor antagonists.

"ERAs work by reducing the amount of a substance called endothelin in the blood. Endothelin is made in the layer of cells that line the heart and blood vessels. It causes the blood vessels to constrict (become narrower). In people with PH the body produces too much endothelin. This causes the blood vessels in the lungs to become narrow, increasing the blood pressure in the pulmonary arteries. ERAs reduce the amount of endothelin in the blood, therefore limiting the harm an excess of endothelin can cause."

There are some risks to the drugs. Kylie could sustain liver damage, so she has to get a blood draw every month to test her liver health. She also needs another blood test quarterly to test her blood count numbers. If Kylie had passed puberty, she would need a pregnancy test every month because ERA's can cause severe birth defects.

Six weeks after our trip to Stanford and four weeks after the paperwork was submitted, Kylie finally got her new med. Thankfully, it's another oral medication which doesn't complicate her ability to run, play, and swim. She's not a big fan yet, as it's a pill dissolved in water so it has a chalky consistency to it.

Kylie will take this new drug in addition to the Sildenaphil she's already taking. She can take multiple meds because they each attack the problem from a different angle. Sildenaphil is a phosphodiesterase 5 inhibitors.

"PDE 5 inhibitors stop a particular enzyme (phosphodiesterase type 5 [PDE5]), found in blood vessel walls, from working properly. PDE5 helps control blood flow to the pulmonary arteries. By stopping PDE5 from working, PDE 5 inhibitors (ie sildenafil and tadalafil) cause the blood vessels to relax. This increases blood flow to the lungs and lowers blood pressure."

The doctors also recommended Kylie get 21 other blood tests done to fill out the whole picture of her health. Since that would be too much to take all at once we're divided them up with the monthly draws she has to do anyway. It's so hard to watch.

But God is always giving us good gifts. A friend a our church, Mosaic, encouraged me to see if Kaiser has a child life specialist. Their job is to provide comfort and distraction. They also help children and families cope with the difficulties and challenges of hospital stays, illnesses and disabilities. Kaiser does have one and we met her and she's delightful! They have a play room that Kylie gets to play in before and after her visits. She also comes with us to the blood draw and last time brought bubbles and a doll. After the initial poke, Kylie was super calm while they drew out 8 vials of blood.

Kylie will also be getting a sleep study done at the end of the month to see if her PH is affecting her ability to get oxygen while she sleeps. We discovered on our trip that she snores (I guess the one plus side of having to share a bed with her). And she is still waking up during the night which is frustrating 2 1/2 years later.

And I'm learning and growing through all of this. Advocating takes time, determination, and patience. It took over 1/2 a year to get to Stanford and another month and a half to start the new treatment. There were days where I felt like I lived on the phone. But there is movement. As long as each day didn't end in a dead end, it was easier to begin the next one.

We go back in a few weeks to see how the medication is doing. Meanwhile, I'm letting my brain take a breather and bask in the warmer weather. I'm going to relish the million books my kiddos want me to read and soak in the swim season with them.

Hi, how are you?

I get up at 6. Kylie woke up twice last night. so instead of turning on my computer to work at keeping up on emails and paperwork like usual, I'm make my cup of tea and grab two magazines from my unread stack, hoping I can pitch both of them by the time my hour is over. The first is Pathlight, a magazine dedicated to Pulmonary Hypertension for patients and caregivers. I skim through some of the articles. I come across a pictorial timeline of the 14 drugs that have been approved to treat PH since 1995. I think, "I need to take a picture of that for reference." It gives me a hopeful feeling this early in the morning. And I'm grateful for Monica who connected me with the PH association who makes this magazine.

I keep reading and come across an article written by a woman in the Philippines and her words enrage me and break me. She only has across to one medication. A month's prescription costs her ten times her monthly salary. "This is not okay!" my stomach reels. We have the resources and capacity to heal the world, and we choose not to! Breathe. She describes that people in her country sometimes lie to get other medications through customs, calling them vitamins. She goes on for several paragraphs to list the names of other PH friends she's found over the years. Every paragraph ends with their death. Her hope was beautiful, though. She's working to create an PH Association in the Philippines and bring all the PHighters together, not only to help cultivate support, but to give them a voice in government to help create funding for their medication. And reading it wrecks me. It creates in me a hook to hang my pity on for our own situation. I have nothing to complain about.Yes, I have a child with a terminal, incurable disease...in a first world country. It humbles me to think when I dabble-dream about going overseas on a missions trip, I have not yet had to be nearly as brave or strong as the mothers there. Anything I feel I would have to say feels like a discarded balled up paper only good for shooting wastebasket shots. I'll have to work on rewriting that dabble-dream.

Then it's 7 and Ethan comes in and I get to vomit a bit of my emotional episode so that I can get on with my "Cinderella chores," as I like to call them. Dishes, laundry. I hear on the baby monitor, "Mommy, Mommy, Mommy." The two older kids find their way downstairs in the dark for breakfast. Morgan inquires on behalf of her little sister, "Will you go get Kylie?" I walk upstairs and pick up my little Kylie-boo. Our first stop as always is my bed, where I've learned from 600 repetitions to untie her snowboard boot in the dark (she was born with a club foot). Then downstairs to deal with the diaper.

A few of the lifestyle choices our family has made always makes this part of our morning one of our down and dirty tasks. We chose, back with our first child, to cloth diaper. So if it's dirty, you have to clean it. We also have gone mostly grain free, so there isn't a lot of rough bulk down there anyway. But Kylie's medication has this great ability to liquefy it. We always apologize and thank caretakers in advance when we know it's probably coming on their watch. (The extent to which I love my MOPS and Mosaic childcare workers is astronomical.)

Breakfast is served. I draw up Kylie's morning meds into a syringe. I give a quick prayer thanking God for my friend Heather, who is a pharmacist and saw a need a never new I had -- liquid medication adapters. They plug the opening of a bottle of liquid meds and have just a small opening, like a salad dressing bottle, that fits the syringe so I can tip the bottle upside down without spilling or wasting any of it. It's beautiful.

Ethan makes my breakfast, as always, so that I can eat one meal while it's still hot. Well, if I'm not already busying myself as most of us mothers do. Our children's training seems to be paying off as they all clean up their own breakfast dishes and run off to explore the wonder of the day.

Morgan and Ethan leave for school and work. And it's now just the three of us. I look and see we still have remnants of last year piled around the house: superhero cutouts on the wall from Sean's birthday (that was in July), paint and canvases from Morgan's birthday party (November--not as bad), and a Christmas decoration or two still needing to be packed away. The holidays and vacation have left my prayer wall and to-do list extremely out of date. I should update those....I give myself some minutes to tidy a few things, though I hold to my promise that toys are not "my things" to put away, so they stay. And now I consciously choose to be present with my kiddos: marbles are the activity of choice this morning. I whip out my degree in engineering (I don't have a degree in engineering) and we (well, I) build an impressive marble run course. Their fascination is endless.

I sit down to waste a few minutes on social media. Mixed in with a bunch of beautiful updated profile pictures and nearly envy-inducing vacation posts are pictures of my little PHriends in hospital gowns and posts about whether the IV site on their child looks "normal" when it's all red or yellow; you know, when normal is having a hole in your arm. Or your back. Some others post about still grieving yet another loss in the PH community. Some moms still have their profile picture as Alyana's angel wings (our PH periwinkle awareness ribbon with her name and angel wings). She had a stroke and got the flu and didn't recover. They aren't sure which happened first, but it doesn't really matter. She was nine.

The spot where I pause the longest is to read an update from Billie Jo, a mom of an 18 year old PHighter. We met them both at the PHA Conference last June. Brooke has been coughing up blood and they are still trying to figure out why. I'm hopeful in reading that her PH team in New York is working with the team at Mayo in Minnesota. Not always in agreement, but communicating none the less. It fills me with excitement as we are meeting our specialist team at Stanford next week.

I had the delightful pleasure of my friend Shauna coming over for lunch. Oh, the lovely life of my young motherhood. She and I sit trying to have a conversation while my four year old son, Sean, works to impress her with his new Christmas toys: a foam rocket launcher and his first Nerf gun. We take a pause because my phone alarm goes off at 1 o'clock: time for Kylie's meds, which she sucks down like a champ. We spend the remainder of our interrupted conversation with the game Headbandz adorned on our heads because Sean thinks we should play; although he always insists on looking at his card first so he knows what it is. I cherish that I have friends who will just go with our crazy, silly, fun life. Kylie during all of this has decided to climb in our toy box, diaperless, because she's killing the day-time potty training (thank you Brenda for the kick in the pants), and is hanging out among toy swords, baseball bats, and rollerskates. My thanksgiving prayer at the end is, "Oh thank you Jesus she didn't pee in there."

Now we pack up to go get Morgan from school. She has art class today, so we are picking her up from parks and rec, which is great because I don't have to get out of the car! This is such a blessing because we have surrendered the fact we have a toddler that doesn't nap. I'm sure this is her natural bent in life, but there are also 33% of children who have insomnia as a side effect from her medication. So I'm sure between the two, I'm destined to be tired for a very long time. But when she's strapped into the carseat, she seems to doze off. She's asleep when we pull up to the school yard, and Sean embraces his responsibility of sister-retriever.

Traffic to art class is AMAZING because it's been raining. People don't leave their house if they don't have to when it's raining in LA. So we get to art class and now I have two sleeping children! I thank God for the metered space that's right out front (and is usually always full). I pull out our handicap parking placard so we don't have to feed the meter. (It's one of the few "perks" of having a child with a disability). This is one of the rare moments where Morgan and I can talk uninterrupted. It's kind of nice. Her love language is quality time which I know I will never be able to give her enough of even if she were an only child. It's been nice to accept that.

She goes in to class and it's nice to close my own eyes for a few brief second. We normally go on "adventures" while she's in class. We've walked to a kids consignment shop where Sean is obsessed with the train table they have. We've gone to the library, walked and watched a car wash, gone to the grocery store with the "moving stairs" (escalator). But with the rain, we're just hanging out in the car. I even brought the game phones which we normally only bring when we go to the doctor (which is actually a lot).

At the end we get to go in and see what she's done. Today, she's finished a marker drawing of Hello Kitty. Kylie's in love with the picture. Hello Kitty's one of Kylie's favorite things. The others are "Lella" (Cinderella) and the Mickey Mouse characters. I love when they finally latch on to something at a young age!

We drive home and now it's dinner prep. While the kids play with cars, they also turn on a story that Amma introduced them to called Patch the Pirate; think radio theater for kids. It's louder than I'd like, but I don't say anything. Then Morgan asks to watch a video, debating that Sean watched it this morning. "When you're halfway done with your school project." is my reply. Then toy cars start to get driven off the table, crashing to our unforgiving stone floor. They receive the death glare because "I've told them before not to" and it's late and I'm hangry. Now Morgan wants to watch the video because she's met my requirement (which I didn't think she'd hit before dinner, but I need to follow through because that's what good parents do, right?). So I'm trying to find this video, which I can't in .2 seconds and I'm stressed about burning dinner, and the music is still loud, and Kylie crashes another car onto the floor. That's it! My sensory limit has been exceeded and my tolerance snaps like a rubber band. "YOU'RE DONE!" I belt as I wildly grab Kylie from the chair who immediately begins to cry. And instantaneously, I know I'm wrong and have terrified her and while she's still being swung through the air I start to sing, "I'm sorry, I'm sorry, you know that Mommy loves you," no longer caring about anything else but her tears. I thank God for Ashley who told me she sings to her kids when she's going to lose it.

I regain control of myself and focus on the things I need to be a good Mommy at the moment. Music down. Sorry Morgan, the video needs to wait but we'll find it. Kids, please clear the table for dinner. We eat without too much conversation because we can all see Mommy's tapped out. Then... Daddy gets home!! Yes, I love him and I'm glad that my husband has arrived back in my life. But usually the first thing that enters my mind is, "The reinforcements have arrived!!" Tonight they want to wrestle; that's Daddy's job. Even Kylie cries, "My chern! My chern!" It gives me a breather and I decide to sort through photos from the last year in our large camera dump folder on the computer, deleting what I don't want to keep. I love looking at them and relive those moments as I go through. I'm thankful for Tiffany, who told me she doesn't do dinner dishes until the morning. Blew my mind and it has changed my life.

Pajamas, teeth, and books in Mom and Dad's bed. Kylie's picked The Little Lost Penguin. Sean's in a Dr. Seuss phase and has chosen Horton Hears a Who and Morgan has a story from her Disney Fairies Collection. Dad is getting Kylie's diaper, meds and boot ready. Kylie interrupts the story with "No boo! No boo!" and begins to wail. She sees the end of her day coming. She still has to sleep in her snowboard boot until she's three. Eight more months, baby. We all take turns reciting our verse of scripture for the month. The kids like to stand on the bed when it's their turn. Morgan has moved into a new class this year, so they no longer share the same one. And they're a little harder. It's sometimes nice to see your child struggle a bit and be able to encourage her in it.

We go around and share what we're grateful for and ask if the kids want to pray for a friend tonight. Morgan chooses Kylie. "Dear Lord, please let Kylie's lungs feel better so that she can stop having to take the medication." It's beautiful and from her heart. We haven't yet explained to them the full extent of Kylie's disease. I almost cry for several reason. I love how compassionate my kids are. Morgan didn't choose to pray for Kylie not to scream for 45 minutes because she hates going to bed, which keeps the kids up. She chose to pray for the thing she can't see, only something she's been told. Kylie doesn't look sick and doesn't act sick. Currently, she doesn't display any of the symptoms of PH: fatigue, dizziness, fainting, or shortness of breath.

The other reason is that used to be my prayer too. And it may be yours too for her, but mine has changed. It would be amazing for her to be miraculously healed, and we could stop all this hospital business and medication. But if that happened, that's only good for our family; it doesn't help any of our PHriends. We would have gotten "lucky." No, my prayer has become for the person or team that will discover the cure for this beast. That God ignites a love of science and curiosity in them. That they have teachers that tell them just because it's never been done doesn't mean it's not possible. That they will possess qualities of tenacity, focus, and determination. That they will have champions who believe in them, support them, and finance their dreams. Because that will cause the greatest good.

Then it's hugs, kisses, high fives, and scruffles and off to bed. I fill their essential oil diffuser with Breathe tonight. I rotate between several different ones that help with respiratory health and immune health (Eucalyptus and peppermint, Melissa, Breathe, Lavender, On Guard). Balance is a new one I heard may help with Kylie's night terrors. Maybe in next month's order we'll have money for that one. I thank God for Lorrie and Cathy who have been so helpful in introducing me to oils and how they can help our little PHighter.

We turn off the light and Kylie's screaming begins. Tonight it's only about 40 minutes. It's date night tonight and Ethan has picked Couple Scategories. We've learned that in this season of life, going out at night is nearly impossible. Not just because of Kylie's screaming, but we're tired. Sleep is almost more important than socializing. Plus, it saves us the text messages from the sitter saying Kylie's still crying, should they do anything. And she's always been a screamer. I used to joke that it's hard to believe she has a lung problem with force like that. But it is on my list of growing questions for the specialist. I know some PH patients sleep with oxygen masks on at night.

Ethan and I laugh while we play and eat a large pile of dark chocolate he picked up. We tag team reassuring Kylie that we are still downstairs and that it's time to sleep. We finish and now it's our turn to sleep. Our prayers in this season are rather short; the morning comes early. Ethan's asleep in two minutes. I turn the flashlight on my phone and read through the next section in my book, The Whole-Brain Child which I heard about at MOPS. It's explaining so much of my own life, even though it's a book on how a child's brain develops and how we as parents can nurture that development and balance our expectations of our kids based on what they are actually capable of understanding. I'm thankful for all my MOPS moms who have been a lifeline these two years, supporting me when I'm about to lose myself and lifting me up to be the healthiest woman I can be.

My light goes out and I bury myself under the covers in my Eskimo-equivalent pajamas. I argue it's not 68 no matter what the thermostat says. Ten blankets or an electric blanket would be nicer, but I do this because it is the only way I will get myself out of bed when a child wakes up and I have to traipse through an uninsulated house when it's 38 degrees outside. And luckily tonight, I'm tired enough to actually fall asleep. Thank you, Jesus.

                                                            *****

The events in this post all happened last week, but I took the liberty of consolidating them into one day for the narrative. I wrote it for a few reasons. It reminds my that in every day there are good things that happen; I'm a good mom and I have great kids. It also reminds me that my life is difficult. Yes, I have a child with a rare and terminal disease; that's just life. Normal now operates on a slightly elevated level of hard.

Third, it was cathartic to write this. About a week and a half ago, our Pastor announced he has cancer, and immediately, my heart broke for his family because I believe it's sometimes harder to be the people who walk beside someone who is sick, because they have to make a conscious decision to stay checked in. And something he said resonated with me. He said, "Don't ask my family how they're doing. They're not ok." And that is often how I feel. So when I'm asked how I'm doing or how Kylie's doing, I literally have to block out half of our current life to say, "Good." So I ask that you be careful how you throw that question around. Yes, some days may be great for us, and I will readily tell you. But other days, even when Kylie is doing what every other two year old does, my mind is always loaded with information about PH and other families affected by it.

Both motherhood and PH have made me a more compassionate and grateful person. I'm more sensitive to people's pain and sadness, especially my kids. I'm eternally grateful for the women God has surrounded me with that speak wisdom, encouragement, helpful tips, and comfort into my life. And the things I find I appreciate the most are the small things.

Hugs: I need a lot of them. Because a hug when you mean it is like emotional oxygen.

Encouragement; motherhood is hard enough and we all need our tribe to champion us. But a mother who has a child with a chronic illness can never hear enough of, "You're doing great." "Your kids are wonderful" "Nothing your kids do will surprise me or make me think less of you." (Thanks Becky for that one!) "You give me so much hope." "You look great today."

Sitting with me in the chaos; the friends that will do life with me and my crazy crew are pure gold. I need you. I'm not superwoman. I possess no magical or supernatural powers. Yes, your hands will get dirty, but the joy and smiles you get in return can never be wiped away.

I love you all so dearly. Thank you for your prayers for our family and for a cure for our little Kylie-bear. To hope and the future!

We belong to a tribe called Mosaic that lives by faith, is known by love, and is a voice of hope.

The Pathlight magazine is published through the PH Association.

I attend MOPS which believes that Moms makes a better world.

To be filled with more

It's been just over a month since our visit with the cardiologist. And the news was less than desirable. Her levels had gone from 59 to the 70/80 range; although the doctor said the elevation may have been due to the fact she was more active on the table. (It's hard to rationalize with a 14 month old that they should lay flat on the table). But the bottom line is her pressures did not go down.

That was hard. Hard to hear. Hard to swallow.

But we moved forward. We had decided whatever the outcome we would go get coffee (and whip cream for the kids) afterwards. And we did. I had a mommy playgroup to go to after the appointment. Which we did. And there my tears were embraced by a group of women who loved on my kids, changed diapers and made me tea. They hugged me until oxytocin (your brain's happiness drug) was released into my body. And that was new to me.

A therapist had told me that we need to grieve in community. I was like, "Uh, no." I'm plenty happy (or honestly, content to be sad) grieving in my car, in my bed, in my shower. And what I realized in that moment was that there is a place for grieving by yourself. But what grieving in community does is allow the grieving to have it's moment and then you feel the ability to move past and move forward more quickly. I had my emotional overload and by the end of the day I felt we could start looking at the situation logically and make some new decisions.

After taking the weekend to reflect and pray we decided to use the next 4 months before our appointment in March to get informed and seek support. When I was first told about Kylie's situation prenatally, the doctor's told me to stay off the internet. Rarely do people blog about success stories and you'll just freak yourself out. So we followed the doctor's advice.

But now we need to know more.  The doctors had given us some general information as we've visited over the past year. We're grounded enough in our reality that we want to know the ins and outs of this condition and what others are doing to combat and cope with it. There seems like a mountain of information to sift through, but in my first go, these were some key points I found:

• There are two types of PAH (pulmonary arterial hypertension). Primary PAH's cause is unknown (also called Idiopathic). Secondary is a result of another problem (like heart disease). Kylie has primary.
• Idiopathic PAH is rare with an estimated incidence of 1 in a million per year, though its numbers seem to be increasing.
• IPAH patients have an increase in Endothelin production which is what the body normally produces to cause blood vessels to tighten.
• The tight blood vessels between the heart and lungs raise the pressure in the lungs and make the right side of the heart have to work harder to push blood into the lungs. Because of this the right side of the heart is normally enlarged. You can have a right-to-left shunt where the oxygenated blood is leaking backwards into the right side of the heart because there isn't enough force to keep it flowing in the proper direction.
• Prostacyclin is normally produced in the cells of the body to dilate the blood vessels. Her production is low or not enough to combat the raised Endothelin production.
• Kylie is asymptomatic, meaning she does not display any symptoms of IPAH like fainting, dizziness, racing pulse, or chest pains. We only know she has it because an echocardiagram says she does.
• Most people with IPAH take medication to help open the blood vessels and relieve their symptoms along with making dietary and lifestyle changes.
• Allicin (a component found in garlic) seems to be widely acknowledged as a natural anti-hypertension agent.

So I'm in a state of making lists. Lists of questions to research, questions for the doctors, information lists, action step lists. Looking at my list of 7 bullet points I know I have a lot more to learn. But we're leaning in and not too proud to ask questions and ask for help. Please always feel free to send us things you read or find that could be helpful. And we just pray we find the time with two other children and regular life to give our research the time it deserves.

Kylie's nutritionists, Heewon and Ellen (Ellen is taking over Heewon's practice as she is moving out of LA), have started Kylie on the supplement Allicidin. Here is a site describing how allicin is created in garlic; I found it quite interesting. Since she takes it twice a day I laugh that I have a garlic baby, because she really does smell a little like garlic. 

 As this is the beginning of a new year, Ethan and I were trying to pick themes for the year. I told Ethan I felt a little silly and embarrassed by what I thought my theme should be...positivity. It's something that is not a default for me; I would go as far as saying I'm not very good at it either. But a vision came crashing into my brain of the pamphlets you often find at doctor's offices about horrible diseases and conditions and there always seems to be a photo of a happy family on the front. I want to be that. The family that is full of joy no matter what the circumstances. That I can be stronger than the everyday frustrations and set-backs. That I can find the positive in every situation and be grateful. To go against myself. Because, honestly, I love me a pity-party. And there just isn't time for those anymore.

So we are looking positively into 2016. A year filled with possibilities. We know that our God will not leave us where we are and so we run forward with hope that we will go amazing places this year, learn incredible things, and bring the message of Jesus where we never would have thought we'd find ourselves. We'd love for you to journey with us. We love and are grateful for all of you!

Heewon and Ellen, Kylie's nutritionists

We belong to a tribe called Mosaic that lives by faith, is known by love, and is a voice of hope.

We started doing themes several years ago after hearing a talk by our friend, Hank Fortener. People often don't keep resolutions, so instead choose a theme to frame your year around. Check out his site! He's an incredible human being.

Ellen is the nutritionist we see and runs Points by Ellen.

Remembering to Breathe

Two weeks ago, I was beside myself in tears as I thought about Kylie's upcoming cardiologist appointment in December where she will have an echocardiogram to get a new measurement of the pressures in her lungs.

It will have been 5 months since we chose to not put Kylie on medication, but rather to attempt to fix her persistent pulmonary hypertension with natural healing. God has provided miracle after miracle in her and our lives that meds just didn't seem to be the right puzzle piece in this part of her masterpiece. There has been so much inside-out healing in our family this year that we made the decision to embrace the holistic healing path in a search for the root cause instead of treating symptoms.

We saw Heewon (our nutritionist) last Saturday. She couldn't find the bacteria in Kylie's body. It had begun in her lungs, moved to her blood, then liver. Now, it's gone. Gone. I had the biggest swell of joy. I was filled with so much gratitude I nearly started dancing and hugging her. Luckily, keeping Kylie from grabbing every vial in the office is a good distraction from over-celebrating :)

What we can't know yet is if this bacteria was the cause of her hypertension. So we still walk in the unknown.

And that's a scary place to be without faith and wisdom, which I pray for daily. I am not God. I am not the one who knows what is best for Kylie and directs her in the ways she should go. But God has entrusted her story to us. It sometimes feels like He has entrusted me with much when I feel I have not proven myself with the little. But I have to remember He qualifies the called; not the other way around.

And I'm not alone in this. I was slightly taken aback when Ethan mentioned he wants to be there on Friday when Kylie is tested. It was a thought that had not even crossed my mind. I was like, "You have work." But I had to breathe and let it sink in that we made these decisions together. He is her father as much as I am her mother. And if one of us feels the weight of foolishness (that's mostly me), the other can lift the fallen one. We walk together through this.

I wrote down the following in a journal to get it out of my head at the time:
"The decisions I made, I made them out of faith and love. I don't know if they are right.

In three weeks, I will stand in front of a doctor, an expert in a field that makes my understanding seem smaller than a grain of rice.

I will hold my breath as I present my daughter. It will feel so much weightier than a simple measurement reading. It will feel as though my worth, my beliefs, my choices will be weighed and judged."

And the comforting voice of God reminded me. Breathe. You are my child. Your worth is in me no matter how foolish the world thinks you are, how foolish one man thinks you are.

And then I saw the greater narrative. I thought about the end of time when we stand before our God and it will feel much the same. I saw myself in tears saying, "God, I don't know if the choices I made in my life were right or wrong. But they were made out of faith and love." And I won't know what will come through the fires of God, but it doesn't mean I don't keep pursing love just because I may not understand in this lifetime.

God loves correction and refinement. He loves to chisel at those who seek him. Love is made more perfect in Him. And perhaps the more I love, the more I may understand. Or not.

I've been reading Brené Brown's books Daring Greatly and The Gifts of Imperfection. They have brought about so much reflection and healing about the shame I heap on myself. There is a beautiful line in her book about keeping a list of names of whose opinions truly matter; it should be a short list. And that has been a hard practice for me to incorporate into my life because it doesn't mean that others' opinions don't effect me. They just can't define who I believe I am or the me that I and those closest to me love.

So even if Friday's results tell me that she is no different than she was three or six months ago, it does not mean we made a wrong choice. There was a bacteria in her lungs and it is no longer there. She is healthier because of the choices we made. We made a choice that brought about good even if it isn't the result we hope for in the end.

Another thing I'm learning from Brené's books is to shut down foreboding joy. I refuse to not make hope a big deal worrying that I may look foolish if I'm wrong. I refuse to not press into the discomfort of being proud of the choices I made. I will say I hope with abandon that the doctor will be amazed and she is healed and healthy. 

And if I'm wrong I will grieve with those who love me for those choices because I was courageous enough to let them know I had hopes and expectations. But I will not dwell on that possibility until it is a reality. Hopes met or unmet, Friday will come and go, and we will find our way with our God and our Tribe from there.

So we are praying for Plan A. We want her future to be one of health and joy. Please join us in praying for our Kylie-bear.

Yes. I'm a realist in transition hoping and learning to be an optimist.

We belong to a tribe called Mosaic that lives by faith, is known by love, and is a voice of hope.

Brené Brown is a researcher and storyteller. Her blog can be found here.

Mud bath...

..well not exactly.

At the end of September, Kylie went in for a mud therapy session. The mud is a combination of Volcanic and Kaolin Clays, Peat Magma, Shilajit, and Target Botanical Agent. It targets sites on the body that have accumulated toxins by creating a thermal effect to draw out deep seated toxins in the cells.

Chinese medicine describes your body as having energy flow and when there is disease or injury, it causes interruptions in the energy flow. Mudding can help remove the "interference fields" and restore chi flow. This results in cells being restored to their optimal cell resonance, having healthy circulation and lymphatic flow, and boosting the immune system.

Unlike a mud bath you think of at a spa, this kind of mud therapy targets specific sites. Kylie's mudding included 3 areas: hands and feet, vaccine sites, and her heart inflow point. The hands and feet were mudded because they are the sites through which energy recycles. The vaccine sites are mudded since vaccines contain chemicals and metals. Metals like aluminum cause cells to absorb water and swell so toxins can't be excreted from the cells. Her heart inflow point on her chest and back was mudded to help with the systemic bacteria that we've been dealing with. In talking with Heewon, the bacteria seems to have moved out of the lung tissue and into her blood. This is a good sign.

When we arrived, we were greeted by Ellen, who did the mud therapy for us. Before applying the mud, she had me make an "O" with my thumb and ring finger and the sites were tested for their strength, which were weak. Ellen applied the mud and then watched as Kylie tried to eat it and tried to climb off the bed in attempts to get brother and sister's toys. It got a little messy, but when the sites were retested, they were strong.

And that was it. I talked with a friend who had done mudding and she said the effects weren't immediate but in a month or so she felt a lot better. Heewon also told me we may need to mud more than once.

Then this past Saturday, about 3 weeks later, we had our normal follow up with Heewon. The heart and lungs were strong, yay! Now the systemic bacteria had moved into her liver. This is exciting because the liver clears the blood of harmful substances and excretes them into bile to be flushed out of the body. It looks as the though the body is getting ready to rid itself of this. Our hopes and prayers!!! Heewon had a little laugh that maybe next time we'll come in and the bacteria will be in the kidneys as its last stop on the Kylie train.

Heewon also found an emotional blockage in T6 of her vertebrae and I learned that there are nerve endings in each vertebrae that go to specific organs. One of the organs in T6 happens to be the liver. So hopefully now that it's cleared, and with the liver supplement she's on, her body will be that much stronger to fight this bacteria.

Some very sad news for our family is that Heewon is moving and closing her practice at the end of the year. I keep asking God why and what I'm supposed to learn through this turn in the road. I've pondered amazing as well as depressing options. But I'm learning again and again that understanding comes after action. So we keep praying and ask that you pray along with us that by the end of the year, this is done. We have another follow up with Heewon just before Thanksgiving. Then the big test comes in December when we go back to see Kylie's cardiologist for an echo to get new readings on her pulmonary hypertension. We fervently pray that with the bacteria out of her lungs, the pressures will have dropped.

Thank you so much for journeying with us in your support and prayers. We can't do any of this without the love, joy and reassurance from our Tribe.

We belong to a tribe called Mosaic that lives by faith, is known by love, and is a voice of hope.

Heewon is the nutritionist that founded and runs Healthee Life

Life on the unbeaten path

It's been 2 months since our first steps on the unbeaten path. Food has become a huge part of my life right now. After 6 weeks of mostly extracting things from our diet (losing an additional 9 pounds in the process), I'm finally getting a footing on adding new and exciting things to my family's menu and stomachs on a budget. I've encountered several new friends who have traveled this paleo road already and are helping me and being such an encouragement. 

Shopping is still the hardest part of my week, taking all three kids to shop for things we've never cooked with before which makes the trips much longer than they used to be. And we normally end up leaving the store without one or two things because they just don't carry them. But, I've learned customer service makes all the difference in my mood. We've switched grocery stores to one that's farther away just because the staff is so extremely empathetic and helpful. Tony in produce even brought fresh cut up fruit to my crying children and their determined on-the-verge-of-a-breakdown mother.

We saw Kylie's nutritionist in August and September, and both times Heewon told us her heart and lungs felt strong which is so exciting. Last week, we went in and saw Kylie's cardiologist for an echo. Her pressures had dropped from 63 to 59. According to doctors, it's minimal progress and he is still recommending the medication. To us, 5 points is 5 points and we're heading in the right direction. Our end goal are numbers in the low 30s so we still have a ways to go.

We have been doing castor oil packs with Kylie several times a week to try and help draw out the toxins in her lungs and now we're going to try mud packing at the end of this month to see if it can help as well.

We will go back to the cardiologist at the beginning of December to see if she's made more progress!

One update on Kylie's club foot - she's outgrown the shoes on her infant snowboard :) She will be getting bigger shoes to continue wearing at night until she's three, but the doctor said she will not need any surgery and can check in with him once a year until three!! Hurray!

Kylie turns 1 next week and it's crazy to think of everything that's happened in the last year! 

I'll post again after the mud packing in a few weeks! Until then, we still covet your prayers.

Waves and high fives to everyone!

We belong to a tribe called Mosaic that lives by faith, is known by love, and is a voice of hope.

Heewon is the nutritionist that founded and runs Healthee Life

Fork in the Road

[Originally written 7/15/2015]

Hello dear friends and family!

We are always so grateful to all of you for your continued prayers for our family and our little Kylie (who has started crawling now, so I guess she's not so little). Kylie had her CT scan at the end of June. The results were good in that the vein they thought was small and underdeveloped actually looked anatomically normal, according to the radiologist. Praise God! So structurally, nothing is wrong with Kylie. What her doctor did say is that, because the scan ruled out lung structure as the cause of her high lung pressure, there is some sort of substance problem with her lungs, like something with the tissue or fluid.

This presented a fork in the road. The medical recommendation is to put Kylie on medication that she would take 3 times a day for many years (sildenafil for those curious--yup). The medication would increase the blood flow through the arteries in hopes to drop the pressure in her lungs. After those many years, she could possibly titrate off the medication or outgrow the dosage but it's not guaranteed.

When we inquired what would happen if we did nothing, the doctor said that as Kylie grows older, she may begin to have fainting spells or her heart may give out if she's fairly active. We're not really excited about either road. So we've decided to take the unbeaten path.

A good friend of ours recommended a Nutrition Specialist (Healthee Life) and she is going to work with Kylie and Suzanne (since's she's still nursing) to see if adjusting and balancing what they eat will eliminate the hypertension. It's not a guarantee, but it's a path we want to explore before committing to medication. They've already had their first appointment and came to the same finding that Kylie's lungs (and large intestine) have low energy. Kylie's been put on some mineral supplements to help her immune and digestive health as well as an herbal supplement to help with systemic bacteria. She's also not supposed to have sugar.

Suzanne's nutrition has altered significantly. She is no longer eating milk, any grain, or eggs which has been an abrupt, difficult transition. Going through sugar detox, carb flu, and caffeine withdrawal (because why drink coffee without milk or sugar?) with the three littles was quite a battle. So now we're on basically a paleo diet and at the beginning stages of finding things we like to eat and reworking how and what we shop for.

Kylie's cardiologist is out on vacation right now, so we'll hear from him after next week about whether or not he wants to continue regular Echos during this period.

We always know that Jesus is our God and we travel this journey for a reason. It has brought along people we may not have met, given us a connectedness with countless others, and increased the impact of our story. We pray, as we walk this part of our story, that God continues to amaze us through our little girl, who everyone says is the happiest and most smiley baby they've met. And that means so much to us, and we love and are so grateful for her.

We continue in faith, hope, and love!

-Ethan, Suzanne, Morgan, Sean, and Kylie-bear

We belong to a tribe called Mosaic that lives by faith, is known by love, and is a voice of hope.

Heewon is the nutritionist that founded and runs Healthee Life

Bracing for a heart catheterization

[Originally written 4/30/2015]

Good morning, everyone!

Kylie has a big day coming up that we wanted to reach out and ask for prayers on. She's doing great in just about every way -- smiling, laughing, almost crawling, and generally being an adorable little girl.

However, she continues to have Persistent Pulmonary Hypertension (higher than normal lung pressure) and the tricuspid valve in her heart is leaking a moderate amount (meaning that blood is flowing backwards). Her cardiologist can't seem to find a reason. He even took her case to a meeting of all the Kaiser cardiologists, and they were all in consensus that she should have a cardiac catheterization.

Normally, this is done to confirm something that the doctor has already diagnosed, but as Kylie's diagnosis is unknown, this will be more of an exploratory procedure with hopes of finding a cause if there is one. They will be threading a tube through a blood vessel into her heart and lungs to take measurements and pictures. She will be under general anesthesia, and the whole procedure will be about 2 hours, while the catheterization and photos will be about 30 minutes.

The procedure is set for 7:30 AM next Tuesday morning (4/28). We would be most grateful for your prayers for the following:

-That the Spirit will be undeniable in that operating room. We know that God is in her story and there is power in her story. We think it's amazing that her story has reached all the cardiologists of Kaiser; something we never could have done. 

-For Kylie's safety and the doctor's skill. As the doctor talked about the procedure, he was honest that, as with any procedure like this, there are risks.

-For answers, wisdom and peace. That God gives an extra portion of wisdom to the doctor to read and understand the photos. No matter what the results, that we have a direction to move in and peace in that decision.

-Peace for Ethan and Suzanne as they wait in a waiting room.

-Childcare for the kids.

Thank you all so much for your love and prayers and support. You're part of Kylie's story, too!

Much love,

Ethan, Suzanne, Morgan, Sean, and Kylie

[followup]

Dear family and friends:

Thank you all so much for your support and prayers! The procedure went well and without complication. The doctors confirmed much of what they've been seeing on the chest echocardiograms that she's had in the past and they have our next step.

Her heart looks good and there was nothing surprising there. We are so thankful! The measurements of the pressure in her lungs was confirmed as much too high. They tried to give her a gas to drop the pressure and it had no effect. They did notice that one of her pulmonary veins (returns oxygenated blood from the lung back to the heart) looks small, like it's not fully developed. But because of the high pressure it was too risky to take pictures from inside as it could have spiked her

pressure to dangerous levels (we were thankful for that decision ).  

So, our next recommended step is a CT scan of her lungs and pulmonary veins in the next two weeks and we'll see what the radiologist thinks after seeing those scans. The Dr. feels like we've moved away from a cardiac mystery to a pulmonary one now.

Kylie is doing well in post-op and should be able to go home between 3-4 this afternoon.

Love and gratitude to you all, our Tribe!

Ethan, Suzanne, Morgan, Sean, & Kylie

Here we go!

"I did it, daddy..." Good job, kiddo. ❤ #perfect

Feeling so much better now that I've eaten!

We belong to a tribe called Mosaic that lives by faith, is known by love, and is a voice of hope.

Like normal people numbers

[Originally written on 1/30/2015]

Last Friday, the cardiologist said that Kylie's heart function was normal (like normal people numbers). Her cardiomyopathy is stable!! She still has the pulmonary hypertension (high lung pressure) but he said she's doing well so he really doesn't want to give her meds or do anything aggressive to bring the pressure down -- why interfere with a healthy baby? :)) So no heart catheter and no medication. We'll go back in 3 months. As for the foot surgery and anesthesia, he wants her to be at least 6 months. So when we go back she'll be 6 months and 3 weeks and then he can decide if she's ready. So, April it is! :) Love you all-- please thank everyone for praying for us!!

Love,

Ethan & Suzanne

We belong to a tribe called Mosaic that lives by faith, is known by love, and is a voice of hope.

It's never easy

[Originally written on 10/3/2014]

Hello, dear friends!!

As you all know, God has answered us and given us a beautiful new addition to the Ransom clan in our little Kylie. We're thrilled and overjoyed at His goodness and can't thank all of you enough for your love and support.

The hard work still continues, and we could really use your prayers in a few specific ways -- the hardest part of the journey may in fact be just ahead of us. :) It's a lot -- thank you for reading and even more for praying! (see bottom for blessings :) )

1. For Suzanne - she's still a high risk for post-partum depression, especially with her parents taking off next Monday and her having to figure out this new routine in the midst of sleep deprivation and hormonal changes. She'll have two weeks on her own (with whatever supplemental help from the calendar comes) before Ethan's parents come for a week. (When Sean was born, it took Suzanne 9 weeks before she could handle a full week with both on her own without extra help or asking Ethan to come home early.)

     Also, we are syringe feeding because it gives Kylie a better chance of learning to breastfeed which is our end goal. Syringe feeding becomes very time consuming as Kylie grows and requires more volume. Pumping is emotionally/hormonally draining for Suzanne.

     6 weeks post-partum is often the most common time that post-partum depression can surface. Even though Suzanne has been doing better and is already on anti-depressant medication, she is still at risk.

      Another layer that weighs on Suzanne is a big kids consignment sale that is on Oct 16th. It's a semi-annual sale that Suzanne goes to and gets all the kids' clothes, birthday/Christmas stuff for the next 6 months. She calls it her Black Friday and she's normally out for at least 6 hours. It will be overwhelming if she has to take the kids, but it's a lot to arrange care for too. She and her mother have been trying to sort through Morgan and Sean's clothes to see how much she has to buy. 

       Please pray for light, truth, hope, strength and sleep, and for progress with Kylie's challenges (detailed below). It's frustrating and difficult much of the time, even with Suzanne's parents here, so we desperately need God's and our tribe's help for these coming days. All the doctor's appointments are physically and emotionally draining for everyone -- please pray for supernatural strength and optimism!

2. For Kylie - she's got 5 small issues, most of which are normal for babies and resolve very quickly after birth. Kylie's have not and could become bigger concerns if they don't soon. This means extra visits to the hospital. And the whole being more than the sum of its parts, Suzanne is trying hard not being overwhelmed and anxious about all of it, and hopes that now that Kylie has passed her actual due date (10/2) that things will start to get better. Here's what we're looking at:

1. Heart - Kylie has a left to right shunt, which is the prenatal hole between the atria that should close after birth but has not, so her oxygenated blood is mixing with the deoxygenated blood. Please pray it closes soon!

2. Lungs - She has pulmonary hypertension (The pressure is the lungs is high so her heart has to work harder to pump blood through her lungs).

3. Liver/jaundice -Her overall bilirubin levels are decreasing. The liver is processing the bilirubin but it's not passing through the liver ducts to be flushed fast enough. If this doesn't resolve she may have biliary atresia (a narrowing of the bile ducts in the liver making it hard to flush bilirubin; sometimes requiring surgery).

4. Club foot -- we're set to begin casting/bracing Kylie's club foot a week from next Tuesday. Normally, there needs to be surgery at the end of that process before bracing begins, but with her heart/lungs where they are, she's not cleared for anesthesia, so the surgery is being postponed. There's a 5% chance that she wouldn't even need the surgery, however, and we are praying for that! Please pray that the casting/bracing would work very well.

5. Nursing - She's not latching so Suzanne is pumping and we're syringe feeding her. She has still not reached her birth weight though. The heart specialist seems especially concerned she put on weight. We feel pulled in different directions on pushing volume for weight gain and working on getting her to breastfeed where volume is unknown.

3. For help in the house - we have a helps calendar that looks pretty empty for October. Please pray that people would sign up so that Suzanne has some desperately needed reinforcements!

4. For Ethan - That he would have the strength to do well at work and well at home, for patience, kindness, and generosity with Suzanne and the kids. For Jesus to be powerfully present in our family as we struggle through this.

5. For us (Ethan & Suzanne) - This all definitely takes its toll on a couple. Please pray for protection from attacks, for honesty clothed in kindness, and the strength to give to each other when we feel we have nothing left to give. Pray we'd see the light at the end of the tunnel. :)

     Also, we have been so blessed to have care over the summer so that we could attend Midweek every week. We would love to continue going so please pray that we can find someone on a more regular basis so we're not searching each week for coverage.

6. For Morgan & Sean - They're being troopers, but it's really hard on them, too. Pray that they feel the love from us extra deeply so that the times we can't play/be with them are easier to bear.

There are of course many things we're grateful for:

Suzanne's parents have been super helpful and have been watching kids, fixing things, cooking, laundry, dishes, shopping. It's been awesome.

Friends have been hosting her parents so they can get good sleep.
Morgan and Sean play fairly well together and even by themselves so they don't always have to be entertained.
Kylie has been sleeping well at night (although tricky keeping her awake enough for a middle of the night feeding) but at least we're not up all night trying to comfort her.
The morning's and evening are cooler letting the kids get outside to play a little, even if just on our patio.
We have been so blessed and are beyond grateful for the food that's been dropped off and people who have sent money and gift cards for our current needs.

Thank you all again so much -- we couldn't do this without you!!!

Much love,

Ethan, Suzanne, Morgan, Sean, & Kylie

Kylie in her jaundice blanket. She looks like a June Bug

Kylie and her beautiful club foot

A present from our Mosaic tribe. The future IS full of hope.

We belong to a tribe called Mosaic that lives by faith, is known by love, and is a voice of hope. 

The calendar our dear friends set up is Lotsa Helping Hands and has an app.

The LA Kids Consignment sale I love here in LA is twice a year in 4 locations.