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.
