Where to start.
Let’s see. Well, Adahlia is now over 21 months old. She’s had roughly 24 transfusions in 21 months.
Her 24th transfusion was last week. The week prior, at 3 weeks since her last transfusion, I took her to get her Hb tested via a finger prick at her pediatrician (a relatively painless way to check for low blood levels, see Mama Bear for the battle I fought to get it done for her on a routine basis). Adahlia’s Hb was 8.1. Since we transfuse at 8.0, I figured we would definitely transfuse at our hospital appointment the following week.
So, we checked into the hematology/oncology clinic and Adahlia had an IV placed. It was more painful for her this time, and I’m not sure why. But it was one of those moments when I suddenly felt like I could feel what she was feeling, and the needle was sharp. Adahlia screamed and reached out and pulled away and arched her back and tossed her head and cried giant tears. I held.
We waited for her numbers to came back and I anticipated a Hb around 7.3. But it was only 8.0! To drop a tenth of a point over a week’s time is nearly unprecedented – perhaps back in November 2012 something similar happened, but that’s it. The other good news was that Adahlia’s reticulocyte absolute count (baby RBCs) was 16, which is higher than its been for at least the last 6 months. Her white cells were in normal range, too, which is wonderful, especially since the medication she’s on (exjade) can cause them to decline.
The doctors gave me the option to go another week before transfusion and I called Joe to conference about it, but we decided to go ahead and transfuse instead of waiting a week. Pros for waiting include fewer total transfusions, which means fewer risks associated with transfusions, including antibody reactions and iron overload. But, we are already doing medication for iron overload and the pros for transfusing: a happier and higher-functioning Adahlia with energy to eat and play and grow, outweighed the cons. Plus, I had a surgery myself scheduled in 10 days. A much-anticipated, tried-every-possible-other-solution, we-cant-guarantee-this-will-work-even-if-we’re-right-about-what-we-think-is-wrong-but-its-your-only-real-option-at-this-point, and you-wont-be-able-to-lift-your-daughter-for-a-month-if-we-can-do-this-laproscopically-or-two-months-if-we-have-to-open-you-up-but-we-wont-know-til-we’re-in-there type of surgery. I wanted Adahlia healthy prior to it.
All things considered, I was very happy about Adahlia’s numbers. The transfusion was going well. Then, while Adahlia was practicing climbing in and out of the little red car they have in the play area, her doctor came to see me.
“I got the numbers back for Adahlia’s ferritin and I need to talk to you about it — but I don’t want you to freak out,” she said.
Okay I thought. This was the last thing I expected. In fact, I had expected Adahlia’s numbers to be low enough to possibly take her off Exjade, the iron reducing medication. (Repeat blood transfusions can lead to a build-up of iron in the blood, which gets deposited in the organs, leading to things like heart and liver failure.) After a month of a half-dosage of the medication, Adahlia’s ferretin had dropped from the mid-800s to the mid-600s, and was last measured at 618. What was it now? 800 again? 900? It didn’t make sense for it to rise if she was still taking the medication but that’s okay, we could figure it out.
“Tell me, ” I said. “I wont freak out.”
She hesitated a moment before reading in my eyes that I meant it.
“Its 1680,” she replied.
I couldn’t help it. My eyebrows shot up. But my voice stayed firm.
A second passed, and then another.
“1680,” I said again, as if tasting the number on my tongue, to see if it was real.
“Yes, she said, and she moved towards me to show me on paper.
Then we were silent, the number hanging in the air like a badminton shuttle on a updraft.
“It has to be a mistake. Can they retest?”
“I called down and left a message to see if there was something weird with the sample, or maybe the machine’s acting up. It could be an error… ”
“Maybe they mistyped a “1” in front of the real number when they input it into the system,” I offered.
“Maybe,” she agreed.
Then, we were quiet again.
“If they have enough blood left from her earlier draw, I’ll see if they can rerun the test.”
I nodded. But I picked up Adahlia and returned us to our curtained bay, shaken.
What could this be?
I contacted Joe with the news, and confessed to him what I hadn’t told Adahlia’s doctor: I wasn’t entirely surprised. You see, the foremost experts on DBA are the doctors and nurses of a special team in NY, and I (like most parents of kids with DBA) consult with a member of their team before every major decision is made. The nurse on their team offers her consultation freely, because this disorder is so rare, so devastating, and they are desperately trying to collect patient information and connect the data points to understand it.
The NY nurse had warned me that a lesser dose of exjade (Adahlia was on a half dose instead of the standard dose) could result in iron getting redeposited back into the tissues. According to the nurse, the exjade would be enough to pull it out of the organ but not enough to flush it out of the body, and so it could get redeposited, making the situation worse.
Well, that didn’t make any sense to me. The half-dose was working — by every measure, her iron was dropping. We (her local doctors and I) had her on a half-dose because she was only 18 months when we started it, and exjade was only approved for children over age 2. I didn’t want her taking any more of it than was necessary, and since a half-dose was working, well, we’d stick with a half-dose. (Some children have suffered loss of hearing, loss of vision, stomach bleeding, intestinal hemorrhage, and even kidney failure from taking exjade.)
Later, during the transfusion, Adahlia’s doctor came back and reported that Adahlia’s ferretin test was re-run and came back at 1500. Still too high. I told her what I had been warned about by the NY experts. Adahlia’s doctor shook her head.
“I’ve never heard of that and it doesn’t make sense to me. Exjade attaches to the iron and carries it out of the body; the body flushes out the exjade with the iron attached. It shouldn’t redeposit it just because there’s not a lot of exjade in the system.”
I agreed that it didn’t make sense to me, either. She said she’d look into it further.
“So, if its not due to taking a half-dose, then what’s happening?”
“I think its possibly a normal fluctuation. Ferretin is not an exact measurement — it shows a trend over time. Let’s up her dosage to the standard dose and retest it in a week or two to see if it is dropping, or at least holding steady.”
And then I called NY.
I told them about the situation, expecting to be reprimanded about not heeding their warning, but much to my surprise, they didn’t seem to think it was due to a half-dosage.
“Has she had a fever recently? Is she teething or has she had a virus?” the nurse asked.
Yes, actually, she had a fever a couple nights ago because she’s getting in her last four teeth.
“Ferretin is an imperfect measurement. It really provides more of a trend over time than any hard data. If there is any inflammation in the system – due to a virus, or a teething fever – then the ferretin reading will go up. But, its nothing to worry about and will go back down.”
“When would you recheck it? Is a week too soon?”
“I’d actually wait until the next transfusion.”
“Really?” Again, I was surprised. “So, this isn’t a dangerous situation? I don’t have to worry about her going into organ failure?”
“Well, it is and it isn’t. She isn’t likely to go into organ failure because the kids who’ve gone into organ failure have been having transfusions for years, and its built up over that time. Now, if her iron continues to go up, then yes, its dangerous. But a reading of 1680 isn’t necessarily dangerous in and of itself. Some adults with DBA have ferretin counts in the 2000s and 3000s and they are okay. What really matters is how much of that iron is actually in the heart and liver. That you wont know until she gets a T2* test, which is done via an MRI, and we don’t recommend it for your daughter because she’s so little they’d have to sedate her for the test, and we don’t want to do that unless absolutely necessary, because there are risks with sedation.”
I tell you. Every single transfusion this game changes a little. Every few weeks, I learn something new. Sometimes, its contradictory. Usually, its apparent that the doctors are learning too. Always, it blows my mind and leaves me like a palm tree, standing tall but imperceptibly swaying, swaying.
On Monday, April 14th, it’ll be two weeks since her transfusion, and though we’ve considered taking her to get a ferretin reading, I won’t. They can’t get enough blood for a ferretin test via a finger poke, and I’m not willing to let them puncture her veins with a needle just for this reading — her veins are too precious — we are trying to keep them intact and avoid having to give her a port for as long as possible.
Besides, I also found out that though her ferretin has risen, her iron transferrin percent saturation has dropped from 90% to 70%. This is a good thing. This means that her blood has more “movers” available to carry out the iron — they are only 70% saturated with iron. This was accomplished with only the half-dose. Now that we are doing the full dose, I expect it will drop much further.
Now, according to NY, they don’t typically start seeing loss of vision and hearing with this medication until the saturation drops below 40%. I do expect it to drop to 40% over this month, since its already dropped so much with just a half-dose. If it does, I will most likely pull Adahlia off the medication (under the supervision of her local doctors, of course.) NY advises against it — they suggest I keep her on it indefinitely in order to prevent the iron from building up. But, I will not risk her vision and hearing. I would much rather simply keep monitoring her iron levels very closely, and once they start rising again, begin re-administering exjade to chelate the iron.
(It would also give her body a break from the medication, which could only be good for her liver and kidneys. It would give them a chance to rebound and heal a bit from all the work it takes to process exjade out of the body.)
The only “trick” to this is that the ferretin can fall to relatively low levels and there can still be dangerous iron overload in the organs. For whatever reason, the Exjade simply doesn’t work to remove the iron from the organs, and when a t2* MRI is finally done, the situation is found to be actually be life-threatening. But there is no way of knowing this until the MRI is performed. In such cases, a stronger chelation medication, administered via an IV over a period of several days, is given to the child immediately.
So perhaps, if we are so lucky as to find out that Adahlia’s iron has dropped very significantly, I will be able to pull her off Exjade for awhile, but we will have to have her sedated for an MRI, just to be safe. More consultations with the local doctors and NY will be done before determining all that, though.
And that brings us to this week.
Tuesday was the day before my scheduled and aforementioned surgery. As the day worn on, I began to feel increasingly anxious. It was a beautiful day and Adahlia and I had spent it together — playing in the sun, enjoying a children’s performer sing and play guitar, while we danced and I tossed her into the air. I carried her everywhere — relishing the fact that I could still, in fact, lift and carry her. I tried not to think about what would happen after my mom left in two weeks and I might not be able to carry her up and down our apartment stairs. I tried not to think about her reaching up for me and me having to decline to pick her up. I tried not to think about how she so often kicks my belly when we sleep together, and how I might be in too much pain to sleep near her and breastfeed her once I was released from the hospital. I tried not to think about how I would be admitted overnight and her calling out for me in the middle of the night… and for the first time ever, me not being there. Its not that I didn’t want to face these things. There was simply nothing I could do about them. The surgery had to happen. I had no choice.
But at 3:30, I couldn’t take it anymore. I packed up a bag and put Adahlia in the car. I was headed to the Sauvie Island beaches. I needed to ground my bare toes in the silty sand of the riverbank. I needed to see Adahlia laughing and playing by the water’s edge. I needed the lapping waves and moving current to clear my head and soothe my soul.
As I drove, Adahlia fell asleep. I turned my rearview mirror downward so I could keep an eye on her. Traffic behind me? Not nearly as important. I watched her head bobbing. It felt so right to be leaving the city behind and enter the farmland. And then it felt so right to leave the farmland behind and enter the wildlife refuge. I parked the car, Adahlia woke up, and I carried her over the sand embankment to the river.
We played in the sand and watched a boat come up the river. I did handstands and she laughed and I spun her in circles. I took pictures of us together. Then, my phone rang.
The hospital. Not Adahlia’s, mine.
“Hi,” she introduced herself. “Did you get the voicemail I left?”
“No,” I replied.
“I left a voicemail! Oh, well, at least I got ahold of you. Your surgery scheduled for tomorrow is cancelled. I wanted to call so you didn’t show up and find out here.”
Surely, I misheard.
“I’m sorry… you said its cancelled?”
“Yes, your surgeon hurt his back over the weekend and can’t perform surgery, and he’s the only one in the hospital who is able to do this type of surgery. We have to cancel all his patients for the next two months. We’re so sorry.”
I struggled to wrap my head around this news. To avoid premature celebration.
“Do you have any idea when its going to be rescheduled?”
“Well, you are high on our priority, but it would be June at the earliest.”
I suddenly had an image of my surgeon. I really liked him. A kind, dark-eyed, fatherly man.
“Is he okay?”
“Well, yes. I don’t really know that much about it so I can’t tell you much more. But he feels really bad about having to cancel your surgery at the last minute. He’s going to call you himself.”
We hung up the phone and for a moment, I did nothing. Then, I giggled. And laughed.
I still felt awful for my surgeon, but I was so, so happy for Adahlia and myself. It felt like a stay of execution.
Adahlia wouldn’t have to be sad. She wouldn’t need to cry and call for me and wonder where I was. She wouldn’t wonder why I couldn’t pick her up and I wouldn’t have to show her my stitches and she wouldn’t point at them and say in her concerned little voice, “hurt.”
It is wonderful. Adahlia still breastfeeds. She’s teething. By June, or July, or August, or whenever I have this surgery, she most likely won’t be. She might be able to walk up and down stairs by herself (she already can do it, if I am holding her hand.) Just as I’ve put off this surgery for 15 months now, stating that I cannot do it because I simply have to be here for Adahlia, I simply have to breastfeed her, especially when she’s too tired to eat real food, and I have to carry her and comfort her, especially during transfusions and when she’s low on blood and feeling awful. I’ve put it off and undergone three surgeries (one requiring a spinal tap, the others requiring general anesthesia) in less than a year to try to stabilize my kidney and preserve whatever function remains in it. But after the last stent failed to hold and I had to have an emergency stent placed, the doctors finally got me to agree that the time had come to “find-and-fix” the problem.
Except Adahlia still breastfeeds, especially at night. And is teething. And requires me to sing or hold her until she falls asleep. And needs me to help her up and down stairs, to hold and comfort her during transfusions.
Amazingly, this can all continue, as the surgery has been postponed. By an act of fate, or God, or…. Adahlia’s guardian angel wielding a golf club???… who knows. It’s been postponed nonetheless.
And though I think my surgeon is a dear man, I celebrate.
The time for surgery is not now. Adahlia’s world is secure. I am here for her in it.
And, oh, that makes me so happy!
It goes to show how a misfortune for one (or some) is a stroke of luck for another.
How everything can change in an instant.
How one action or event ripples outward, touching more lives than can be imagined, and forever altering the future.
How fast this world turns, turns, turns.
How fast the river flows, carrying time with it.
While we rest on its shore, holding each other and spinning, letting it race by.