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)

The best group of people you never wanted to meet

Community is essential when you are a parent of a child with chronic illness. I was so fortunate when my friend Sophia introduced me to my first PHriend, Monica. Monica encouraged me, hugged me and said "Welcome to the family."

At conference we had a time to get together in support groups. There was a group specifically for Parents of children with Pulmonary Hypertension. My friend Sarah said to the group, "This is the best group of people you never wanted to meet." And it is so true because being a part of that group means that you share with us a huge pain and suffering in your life. But I've also never experienced such a true belonging with a group of strangers than those parents. We live 100s and 1000s of miles apart. An online support group keeps us connected day to day. And conference gives us time together to celebrate, support, and grieve.

In another session, a parent said to use the online group to vent your feelings and get support. Take your emotions there. There is no need to spout that off into your children. It was such a profound yet simple statement. My PH child and other children have no idea the stress and pain I feel so frequently. And they have no business at this age to have to carry a burden like that. They know her heart is special. And that she takes medication to help it work like theirs. And that's why we go to the doctor so much. That's all they need right now. I have a community to help carry of the load of I-can't-keep-it-together-right-now! And for that I am extremely grateful.


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

Join us to empower with hope (Kylie’s PH Conference)

Hello friends! In our mission to continually be educated as best we can about Kylie's 1 in a million disease (Pulmonary Hypertension) and surround ourselves with community who has shared experiences, insights, and advice, we are sending Suzanne to the PH Association's Conference at the end of June. Kylie may soon be switching medications and we'd love to learn more about them from the best in the medical field and talk with parents of children who have used them.
To alleviate some of the financial burden, we are only sending Suzanne (since the kids would be put into a conference day care all day) and her parents will be coming down to watch the kids so Ethan can continue to work. We have already received some donations so we only need $800. That's only $50 from 16 people! If you would like to donate and be a part of Kylie's and our continuing story, we would be so incredibly grateful. To avoid charges from crowd funding sites, we are just using personal transactions.

Feel free to share and forward to anyone who may be interested

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.

PH Conference

The PHA conference was three months ago, but I'm finally finding a few moments to write about it. It was amazing!

Here are some of our highlights!

We met a bunch of families! We all come from a slightly different circumstances and entrances into this family, but we are all here for each other to share our experiences, hope, frustrations, questions, and successes.

We found out there are 7 pediatric hospitals in the US that specialize in pediatric pulmonary hypertension. There is one at Stanford and we are working on getting Kylie there. We learned that even though she is not in a dire situation, it is best to have your specialist and know your team as soon as possible since PH is a progressive disease.

We saw the possible future. Kylie could end up with an intravenous line delivering medication straight into her blood stream or a subcutaneous line delivering the medication into the tissue. It was great to hear from parents their experiences with both and the struggles. We are grateful that Kylie still seems a ways off from that since she is responding to oral medication. We learned that if she ends up with an IV or SubQ that showering and swimming become very difficult as the site needs to be waterproofed and is very time consuming.

But the future is so hopeful. There are so many constant improvements in technology and discoveries in medicines that the pediatric world of PH may look very different in a few years, which is incredible.

Morgan and Sean got to interact with PH kids and ask us questions in a safe space. They saw a lot of people with oxygen and pumps, kids that were very thin, and people in wheelchairs and scooters. It was a great introduction for them (still being so young) to the disease and hopefully to have greater compassion for Kylie as she grows up. And even greater compassion for people in general.

We learned PH patients overheat more easily and get more fatigued. That definitely helped us with Kylie because we keep the house fairly warm. On really warm days, I've started running the air during her nap. Right now she is normally just sleeping in a diaper. We even speculated that maybe some of the insomnia was related to just being hot since she still needs to wear her foot braces (with socks) and pajamas. Once she is in school, we will most likely have to fill out special forms to have alternative options for her if being outside will be too hot--it is southern California and August and September get quite warm and most schools eat lunch outside.

We learned that with the fatigue, since she is so young, to err on the side of caution. We laughed along with other parents as they warned us that, yes, Kylie will take advantage of it and be lazy sometimes, but as the kids grow up they become fairly good at self regulating. There is a good chance she may not be able to participate in all the activities of a regular PE class in school.

On the plus side, Kylie will qualify for a few things. She will get a Make-A-Wish when she is older. She will receive a lifetime membership to US National Parks (that takes care of family vacations for the next 15 years ;) ). She also qualifies our vehicle for handicap parking.

The conference overall was just a beautiful three days of hope and celebration. We had the lucky chance of seeing the founding women of the PH Association at breakfast and got a picture with them and Kylie. This whole association was started by 4 women around a kitchen table. Three of them had PH and realized they needed to find others like them and help each other get the care and support they need.

And we are the grateful beneficiaries of their determination. We will benefit from having heard so many families' scary moments that they were willing to share with us. It has given me so much confidence that we can carry this responsibility and help Kylie become confident and strong.

So along with hope, confidence, and support, I came home with a long list of to-dos. We are working on getting Kylie Medicaid so she can visit Stanford. We need to create a medical binder to track Kylie's PH information so we are prepared for doctor's visits and emergencies. We need to order medical bracelets and get emergency hospital numbers. We need to apply for all the perks.

It's slowly coming along as we are balancing the rest of life and two other amazing kids. God gives us the strength we need day by day.

Kylie had her most recent appointment at the end of July. Her pressures are still in the upper 40s so we are working at titrating her dose from 5ml three times a day to 7ml three times a day over August and September. We will go back at the end of September. Hopefully by then we will have her Medicaid in place and can book an appointment at Stanford for October.

We always are so grateful for everyone who prays for our family and checks in to see how we're doing.

We're aware that she's rare!

Ethan's Mom flew in to help watch Kylie during the conference because she was too young to check in to childcare. Thank you Mom!

#phaconference

Staying cool in the hot Dallas heat

Two of the four founding PHA women

Empowered by Hope!

The conference we are attended is through the PH Association.

Surge

There's been a lot going on lately in life. And there always will be. But one of the biggest changes is Kylie is walking!! And she's 6 weeks on her medication.

And somehow the coming together of her walking and now beginning medication gave me great comfort. I had often explained to people when we were trying alternative treatments that if there was any time to try, it was this season because she's not extremely active or walking. And it's as if God has gently hugged me and said, "You did well. You trusted me and explored those options so that you can embrace this next phase with full confidence." And he's planted a peace is me that I have no shame. I don't fear the "told you so's" (not that anyone has said that besides my internal self-deprecation - which has become much quieter these days, Praise Jesus). And we are a stronger, healthier, more aware, understanding and compassionate family for the journey.

So, to back up and recap: in the middle of April, after the meeting with the head of cardiology, he and Kylie's doctored agreed on a dosage and we picked up Kylie's medication. She's on sildenafil. Here is a brief scientific explanation of how it works for those curious:

"The increased pulmonary artery (PA) pressure found in PAH is due to disturbances in key vascular mediator pathways including relative deficiencies of vasodilators such as nitric oxide (NO) and prostacyclin, as well as exaggerated production of vasoconstrictors such as endothelin and thromboxanes.  

     Sildenafil is a selective Phosphodiesterase-5 inhibitor that has been reported to be a potent pulmonary vasodilator. Sildenafil selectively inhibits phosphodiesterase 5 (PDE5 is abundant in pulmonary tissue) which leads to stabilization of cyclic guanosine monophosphate (cGMP).  cGMP is a second messenger of nitric oxide (NO). Stabilization of cGMP results in increasing nitric oxide (NO) at the tissue level leading to pulmonary vessel vasodilatation.^"
(link to article here)

We were told that the most common side effects are flushing, headaches, sensitivity to light, and diarrhea. Luckily she didn't seem to display any of those things.

And just because I need to laugh in all this, I wanted to share a fun story. Mom's make completely senseless random decisions sometimes. Ten days into her medication I decided to test her allergies of tomatoes, paprika, and black pepper...with Little Cesar's pepperoni pizza. They used to give her severe diaper rash and open sores. Well, it wasn't really a completed experiment because she threw it all up 3 hours later. And then she threw up lunch. And then she spiked a fever...and got a new tooth. So now I've quadrupled my confusion: is it meds? Is it food reaction? Is it illness? Is it just teething? Awesome, way to go, Mom. Looking back, I still think illness, but who really knows?

What I did notice more of a pattern in was that she is no longer sleeping as long or as well. I chalked it up to a developmental thing since she just started walking that maybe she's regressing into an attachment phase. But I just read one study that listed neurological side effects including insomnia and raised body temperature. It's something I'm working at being more aware of to discuss at our next appointment in July.

But before our next appointment, We're going to Dallas! We are attending a large Pulmonary Hypertension Conference in Dallas this June. There, we will hear researchers and doctors share their latest findings and patients talking about living with PH. We will also be able to meet other families with PH children.

I chuckle to think we're going to Dallas to find hope and community when I'm enveloped by it here at home at Mosaic. I think of it a little like Paul and his travels to the early churches. Driven by hope, but each community is different and offers unique things. And the hope that will spring from that room--a room where you walk in and everyone just gets it--I look forward to with great anticipation. God is always in the business of expansion because as humans we have a limited capacity. I love my tribe and wouldn't trade them, but I'm so excited at the idea of meeting people with a specific kind of hope for PHighters, PHamilies, and someday a cure.

But to send a family of 5 to a conference costs money; a lot of money we don't have. Ethan and I agreed that we would fund raise the money to go. I worry about a lot of things like finding parking spots, making people wait for me, that my son might get hit by a car, or that Kylie may die some day. But I never doubted that God would provide for us in this moment. I applied for a scholarship and got it! My travel, hotel, and conference fee is covered! So I estimated the rest that we would need to raise. And of course I estimated bare minimum, because God we just need to get there. I mean why would I ask for luxuries when it's our friends and family needing to sacrifice for us.

But God is showering us with love and teaching me how He is a generous God. He loves to give gifts to his children. We have received the amount in my tiny prayer almost twice over! Every time someone gives, I become overwhelmed with tears. Grateful and humbled.

I came to this thought once in the middle of my tears. It is belief in the character of one of us that moves someone to give. Whether it's Ethan's love and devotion to us, or Kylie's joyful smiley spirit, or our parents' years of faithfulness, or my upside down crazy beautiful life, it's belief in who we are that compels people. And it leaves me confused, humbled, speechless, and a little dumbfounded. Because who am I, God?

So many people believe that we should be there. We can accomplish so much together. I feel like we are going to be riding a tidal swell as we go to this conference.

Her disease is not a punishment. It is just an extended trial that in the end we may receive the crown of life. "We also glory in our sufferings, because we know that suffering produces perseverance; perseverance, character; and character, hope. And hope does not put us to shame, because God's love has been poured out into our hearts through the Holy Spirit, who has been given to us."

We have always prayed for Kylie's healing and it's hard to have hoped and not had it come through when you wanted it to. It's hard when you cry out for God move but instead He changes you and calls you to move with Him.  God constantly reminds me that He is her lifeblood, and it is Him who gives her breath life. And I cannot add a minute to her life that God does not have planned for her.

And some of you may be curious about the extra money. We are going to use some of the money to let the trip breathe a little. Like renting a car instead of taking shuttles to and from the airport. But the rest we want to donate to PH research to further making hope reality in finding a cure someday.

The post got a little long, but like I said, a lot has been going on. But we will surge with confidence wherever God will take us and our story. Love to you all!

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

The conference we are attending is through the PH Association.

Bring it all to Peace

I've never been at so much peace before, during, and after an appointment as I was today. I chalk it up to two things really. The first is the power of prayer. I was so touched by all the people who continue to pray not only for Kylie, but for me (and the rest of our family). When someone has a disease it affects the whole family and everyone has to adapt to some degree. For those familiar with Strengths Finder, Adaptability is not in my top 5 or probably even in my top 20 strengths. But together, we are learning how to make our family work. And my own prayers and confessions to God have become a daily practice of just releasing the weight of a burden I have no control over and allow my brain to focus on the pieces of our life that I can take action on.

The second things is self care. It's a phrase that's been around and it's one I've pushed aside and struggled with all my life. But I've embraced it recently because it's imperative for my survival. The list is always so simply put, but the execution takes so much discipline. Eat well, No sugar, Sleep, Play, Pray, etc. I've been working with our nutritionist Ellen this past winter on addressing a chronic stabbing pain in my side that I've had for over a year. (My kids and I affectionately call them "the roars"). A lot of it we've linked to liver, adrenal, and stress problems. When I'm on a stricter paleo diet, the more I've been able to manage stress and the less pain I'm in. The other part of self care is people for me. I can't work through my stuff with a 5 year old. Being a participatory voice in a community is hard for me; I know it will get easier the more I do it.

Anyway, so prayer and self care helped prepare me a lot for today. I did shed a few tears on the way to the hospital thinking of the most likely outcome that we would leave with a bottle of medicine. But I wasn't afraid. I knew it would be hard, but I wasn't afraid. I could feel the strength inside myself and it was a beautiful feeling. God brought to mind Phil 4:13, "I can do all things through Christ who strengthens me" which is literally when I find myself in a situation, I am actively being made stronger through the experience when I am connected with God.

We heard good things at the appointment: her oxygen levels are great, her height and weight are great, her heart function is great, still not displaying any symptoms (which keeps her at a class I).

But, her pressure levels are still between 73-85 (a normal person even working out shouldn't be above 40). Even though the Dr. added "rock steady", it's always deflating to hear. Between December and now we had tried different supplements and an essential oil regimen that all had vasodilating properties and it still wasn't enough.

Even though I had hopes for improvement, God had been speaking to me and it's like I knew. The the heavens didn't cave, and the floor didn't give out. He is before all things, and in him all things hold together. (Col 1:17). I think it's not a coincidence God gave me those two verses and that "all things" is the same word. Each and every thing. And HE IS before all those things were. Before this journey, He was. And when I am in Him it all stays together. My raft stays together. My brain stays together. In the Hebrew the word for heart and mind are the same. My heart is together and at rest. At peace.

And then of course the Doctor throws the curve ball. About a month ago, we had made an appointment to see the head of pediatric cardiology, but it's not until the middle of April. It was for a second opinion on Kylie. Since it's so close to this appointment, the Dr. recommended we wait on the meds until we see him just in case he recommends something different. Say what!? But God's peace was even there. It was like he immediately spoke to my heart that we wouldn't be dealing with side effects as we travel this next week. And I left the office not feeling on pins and needles, not defeated, not even delayed. I have to admit it was kind of strange, but in a really good way.

So we get to repeat this whole trip in about two weeks. And I'm okay with that. We did talk through the medication if she would start it then. It will possibly take weeks or months to get her to a proper dose. We would keep titrating the medication up until they see a drop in pressure on the echo, or she gets significant enough side effects that she would need a different kind of medication. It will also mean more regular trips to the doctor at this beginning stage. So I know more of what to expect if that's the outcome next time.

She was also cleared to fly since the rest of her health is so good! Thanks all so much for your prayers.

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

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

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.