A Central Line
This past weekend, Adahlia and I enjoyed some of the best of Seattle’s weather and spent some time appreciating our intact and strong bodies.
Adahlia makes a sand cake at Golden Gardens Park in Seattle Washington
Tomorrow, Adahlia will have a central line placed in her chest so that she can receive medications easily during her transplant, without multiple IV pokes. While it is a surgery (and therefore feels scary), it is ultimately a very good thing. Nobody likes getting vaccines and IVs. They hurt. The central line will allow nurses to give Adahlia medicine and IV support without having to continually harm her veins. The line is not permanent - it will come out as soon as she no longer needs that kind of medicine or fluid and nutritional support.
Adahlia isn’t looking forward to having a central line because she hasn’t had to have anything like this before. She has easily had over 100 blood transfusions. She had so many IV needles in her feet, arms, and hands that most kids with a condition like hers had ports placed in their chests years ago. (Ports are similar to central lines). We’ve both chosen and been able to avoid a port and this has allowed Adahlia to live relatively freely, a life of less confinement than typically experienced by someone with a condition like hers.
Adahlia laughs as we bike along the Burke-Gilman Trail in Seattle
As you know from this website, Adahlia plays soccer, surfs, scrambles mountains, and does other very physically intense things. She will have to take a break from it all for awhile - her body will need to be treated like it is fragile, because it will be for awhile. The central line is part of that - we will need to “baby” it. For example, she’ll be able to sit in baths and take showers, but she wont be able to soak or submerge under water. And she certainly won’t be participating in any rough or “contact” sports where a ball could knock into it. These restrictions will be hard on her. It will be painful physically and emotionally, and it will certainly be a challenge to someone so spirited as Adahlia.
But she and I are choosing to do this so that she can come back stronger. So that when she rejoins her soccer team and other life activities, it won’t be the fact that she is anemic or recovering from blood infusion that she winded or needs to rest. She will only be limited by how hard she trains; how badly she wants something. Adahlia will never be “just like everyone else” - and we wouldn’t want her to be. But we do want to make her life a little easier. We do want to open doors to opportunities that everyone else takes for granted, like opportunities to train hard and make a varsity sports team, or to take a job that involves international travel. As her mom, I have absolutely no idea if she’ll end up wanting to do those things in her life. But transplant will allow her to chose them if she wants to - to choose the life she wants to live - instead of a life dictated and limited by a body that simply can’t make red blood cells. In sum, she is choosing agency, the ability to direct her life. She is choosing temporary shackles because - together with our friends and medical team - we are betting on future freedom.
Let the medication begin
Adahlia and I aren’t big fans of taking many medicines, but she has already had one more medication added to her routine (taken twice a day) and today we will add in another.
Appreciating the flora and sporting her Colorado unicorn hat (and many rainbows!) as we wait for a smoothie and a milkshake before heading to the beach
All of this is preparation for transplant and based in research that shows what makes transplant safer. For example, the medication added a couple days ago is a protectant for the liver, because multiple rounds of multiple chemotherapeutic agents in quick succession will be intense for her liver to process. (The liver and kidneys work together to clean out the blood).
Today, she will start taking an antibiotic. While we all have bacteria in our bodies, and we need these good bacteria to digest food and keep us safe (this is called our microbiome - and it basically means that microscopic friends live on us as if we are their planet), during transplant she will have a very weak immune system, and it is more important to keep any bacteria (both good and bad) from multiplying out of control than it is for her to be a good planetary home to good bacteria. So, for the next couple of weeks, we need to flush out as much as possible.
The next two weeks will basically be bacteria Armageddon. (Sorry, guys. But your planet’s needs come first. Maybe there’s a lesson in there for humanity…)
Meanwhile, she is still taking her multi-vitamin and a medication to remove excess iron from her body due to all the blood transfusions. If this is news to you, an interesting bodily fact is that our bodies don’t have a mechanism for getting rid of extra iron. Iron is simply so scarce in nature, and so necessary to blood cell formation (in the center of all red blood cells is a molecule of iron), that our bodies never evolved a way, or mechanism, for getting rid of it. We pee out extra vitamins and other nutrients, but not iron. In fact, iron is so precious and the body so confounded on what it should do it with, that if we accumulate too much of it, it stores the extra iron in our organs.
Now, if you aren’t receiving blood transfusions (or don’t have a rare disease called hemochromatosis), don’t worry. You aren’t going to store iron in your liver, pancreas, and heart. But if you do have to get regular blood transfusions, like Adahlia, then your body WILL store iron in these organs. And I think we can all agree (even those without any medical training whatsoever) that having iron in your organs (and heart, especially!) is a really bad thing.
So we have to get it out.
And we have been. Adahlia actually has an astonishingly low iron count for someone who has had so many blood transfusions. Moreover, we were able to get the iron out without needing to resort to the iron removal (aka iron chelation) medicine that requires nightly needle sticks or spending 12 hours attached to a pump, like so many other kids with DBA. No needle sticks, no pumps. I’d say we’ve been lucky but we’ve actually been really thoughtful about it. Using integrative medical means, we’ve been able to maximize her chelation AND protect her kidneys (iron chelation medicine is hard on the kidneys - remember, like the liver, they clean the blood of medication).
During her last lab checks, we found out that her kidneys are doing better than they have all year.
Honestly, Adahlia’s health is nothing short of astounding given the body she drew in the lottery of life.
She’s a living miracle, a vibrant testimony to the power of what is possible.
No matter what the diagnosis.
No matter how bad the forecast.
And we intend to build on that.
What’s next
Today, Adahlia and I have FOUR medical appointments at the hospital today. The first one is to help her mentally and emotionally prepare for her central line surgery. The second and third is for me to learn how to care for and clean her central line and for us to go over sterilization procedures and food safety. The fourth is with Physical Therapy.
Like with Occupational Therapy, this will be a pretty fun appointment where the doctors and therapists will measure how strong and flexible she is through various exercises and games, so that we can be sure to keep her strength, flexibility, and coordination up during transplant. Getting the first set of measurements is called getting a baseline reading, and it is very important in the medical field. After all, if you don’t know where you’re at when you start, how can you tell if you’re improving or not?
This week we’ll have multiple appointments every day, including tomorrow 5/2, when she’ll have her central line surgery. Today is a non-invasive day, just talking, nothing scary, and we don’t even have to be at the hospital until late morning.
Tomorrow will be a very early morning for her surgery. That’s actually a good thing, because since you can’t eat before surgery, it means she’ll be able to eat a big dinner and not have to go very long without food the next day. If you pray or have been trained to send healing / light energy, Adahlia’s surgery is scheduled for 7:30 am pacific coast time (10:30 EST). We check-in for it at 6:00 am. Please pray and fill her surgical team with light and wisdom, so that her procedures are safe and wonderfully complete. I am certain she will also appreciate anything you can send to help her feel safe, confident, and loved as she enters surgery and then recovers quickly and beautifully.
On Thursday, 5/4 she will have another surgical procedure, called a bone marrow biopsy. She’s had three of these procedures so far in life, and hopefully, this will be the last one she ever has to undergo. She’ll be sore and in pain from both of these procedures this week, and still has to attend other medical appointments, such as provider consultations, an echocardiogram to measure heart function, an x-ray and ultrasound, and even a pre-transplant dental exam. It’ll make for a rough week.
But we have it all lined up, including a chiropractic appointment offsite, to get her spine feeling as great as possible before transplant. We don’t get a day off mid-week from hospital appointments this week like we did last week, but we are hoping for a quick recovery from her procedures and a wonderful weekend.
I’ll post more very soon.
We deeply appreciate all your prayers, light, and love. We also deeply value these days we have together, these opportunities to live and contribute to life with our own hearts and hands.
We love and look forward to seeing you again, stronger & brighter than ever. <3
