The night before Memorial Day, I ambush Joe as he comes up the stairs.
“I just found something out,” I say, “about that Ayurvedic herb I wanted to try for Adahlia.”
“Oh,” he says. He can tell this isn’t going to be a light-hearted conversation. “What?”
“Well, as I told you, I was hopeful about it because it has steroidal components similar to hydrocortisone, which is one of two steroids used to treat DBA, and this herb has helped to raise testosterone levels — you know how boys with DBA sometimes go into spontaneous remission during puberty — this herb can raise or lower cortisol. It’s an adaptogen; it balances the body’s hormones by raising or lowering cortisol depending on the body’s needs, restoring strength and vitality. It’s even raised hemaglobin in a number of clinical trials.” I rattle off these promising facts while pacing the living room, gesturing at the walls as if they were an audience.
“Right. So what’s wrong?”
I turn to face him, my voice as heavy with its news as a construction crane swinging its load. “I was doing some more research on it, and I discovered that its also high in iron.”
He says nothing. I whirl.
“I’m so frustrated!” I say, shattering his non-reaction like a plate on tile. “Everywhere I turn, everything I try, it seems like it could potentially help cure her, but then I discover it might also kill her. Her body tested positive for needing the algae and the specialized probiotics, both of which were high in iron — granted, miniscule amounts compared to a transfusion — but now we know she’s got dangerous levels of iron in her heart, and we’re going to the hospital tomorrow for transfusion, which will only dump a massive amount of more iron into her! If I hadn’t tried the algae and the probiotics, would we be at this point now? Would we be already faced with nightly needles and pumps? I stopped doing the algae and probiotics as a precaution, given her iron situation. How can I give this new herb to her when I find that its also high in iron?”
He cuts me off briskly. “You can’t know if what you’ve done has made her worse. Like you said [the herbalist] would probably say, her body tested positive for the algae and probiotics, so it shouldn’t harm her, and the natural medicine community isn’t unanimous on the subject of whether the body would even store plant algae in a pathological way, anyway… some think it helps the body to dump the animal-sourced iron. Who’s to know? No one’s said that she is more iron overloaded than any other kid with DBA. It could just be the transfusions. No one knows. You’re not going to try the steroid herb now?”
“Oh, no,” I reply, “I’m going to try the steroidal herb. At least, if her body tests positive for it, or if it the testing shows that the sub-clinical infection is gone. Or maybe, even if it doesn’t. I don’t know…. I guess I have to try it. I know what you’re saying and I’m not blaming myself. I know why I did what I did, and I’d do it again. I stand by my decisions; I had to try. I’m not the sort of person who wouldn’t try… not when everything points to it being a functional problem… not when I know how the body works as a system, and I can see how it’s failing to work … I can’t just not do what might help her, not when there’s a possibility of it curing her, not when I know so much… when we know so little.”
Anger begins to surge through me. “I’m just so sick of it! Nothing is easy, nothing is clear. Every single therapy has a hook. Any choice I choose could cure her or end up killing her. I just realized the iron content of this steroidal herb. I haven’t been looking up the mineral composition of all of the herbs in her chinese formula. What about them? Do they all have a high amount of iron? I suppose I could look them up, I have to, maybe…” I drift off and then regain steam. “Tomorrow, the doctors’ll probably say we need to do desferal — to try a more aggressive approach — to get the iron out of her heart, which means I’ll have to stick her with a needle every night, and — as if that’s not bad enough — she’ll have to sleep hooked up to a pump, and how are we going to do that? You know how she is… moving around and crawling around the bed all night in circles… she’s going to lie still and be hooked up to a pump every night? For the next 5 years? For as long as she’s receiving transfusions? The rest of her life?”
I sink onto the edge of the sofa but don’t collapse — I am too rigid with emotion.
As if “normal” life weren’t already filled with enough suffering!
So much pain for a little person. Pain pain pain. Every day, I trick her into taking medicine that hurts her stomach… and even, like she told me the other day, hurts her gums. She’s constantly subjected to some sort of finger-prick test or a blood transfusion or other test or medical intervention. And she doesn’t realize it yet, but if my suspicions are correct, the doctors were indeed about to recommend desferal. And I had wanted a life as natural as possible for her! A gentle, natural, drug-free birth, and as few medical interventions and procedures and medicines as possible.
Her life has turned out to be full of everything I had not wanted for her.
Well, not exactly. She has our love. She is secure in our love. That is something.
Joe crouches, balancing meditatively, at the top of the stairs.
“You’re right,” he says. “She could end up dying a couple years early because you’re trying to cure her. But, who’s to say she wouldn’t die five years later from a different complication, from something the doctors give her? You can’t do this to yourself, Erika. Do you think the doctors at the hospital do this to themselves?”
“No,” I scoff. “They say: ‘These are the risks. This is what we think the best choice is.’ And if it goes south they just say, ‘Sorry. It was a risk that we warned you about.'”
“Exactly.” He pauses. “Erika, you’re trying your best. You’re doing everything possible. Everything does have a double edge with her. But, if she were cured right now, you’d be saying it was worth it, right?”
“Yeah.”
“You can’t beat yourself up over the outcome. If you have to try, you have to try. And it still might work out. If you want to try the steroid herb, I’m behind you. I support you. It’s either this herb or the actual steroids, and we already know the risks that come with those.”
Or a bone marrow transplant, I add mentally, thinking of the two kids with DBA in the transplant ward, one of whom is now over 20-days in the ward, fighting through sickness and life-threatening complications, as her parents hope for engraftment of the donated marrow… and the other little kid, a boy, from whose parents we have heard nothing in over a week. Not a good sign.
***
The next morning, today, I received a response from the DBA community. I had posted a question: “I know that there is a correlation and higher risk of cancer for people with DBA. Has anyone heard of a similar correlation between DBA and autoimmune disease?”
The replies blew me away. Two responders were persons with DBA who said that their mothers did not have DBA, but were eventually diagnosed with Lupus later in life. One responder was a mother who also did not have DBA, but she had a DBA child, and she was having auto-immune like symptoms, although, she had not yet had a full workup. A fourth respondent said that her DBA child had started showing some of the signs of Lupus, and doctors had begun treatment intervention before it could turn into full-blown Lupus.
This is not only interesting, it is phenomenal, and I believe, it is important. I had asked the question because I have been having autoimmune symptoms for the past few years, and my blood workup showed that I had two of four markers for Lupus. The rheumatologist couldn’t make sense of it — since all four markers weren’t lowered, I didn’t have Lupus, but my immune system was clearly doing something odd.
Why is this important?
Because DBA is extremely rare. EXTREMELY rare. There are an estimated 400 persons in North America with DBA. How odd was it that in this tiny community there were even three non-DBA mothers who were later diagnosed with Lupus with DBA children? Perhaps, the diseases shared common pathology, and if so, then that means there is a similar physiology — a way to restore proper, physiological health.
It cannot be denied that there is a genetic component to DBA: the mutant gene, in my view, is like a chink in armor. But I do not believe that any gene has the final say. An arrow has to pierce that chink. In this view, persons could be born with the gene (ie, carriers) and never know it. But, if the right arrow struck, if enough stress were placed on the system, the gene could be activated and symptoms of DBA appear.
Just like cancer. Just like auto-immune disease.
Of course, genes implicated in cancer and auto-immune disease might also potentially be activated for no reason whatsoever. Yet, I had done enough work with persons with such diseases, and read enough studies, to know that it was possible to turn such diseases around with natural medicine and healthy lifestyle.
The autoimmune connection to DBA is also interesting to me because I now know of two persons with DBA who had their spleens removed (and, eventually, their gallbladders), and once their spleens were gone, their anemic symptoms resolved to the point where they no longer need transfusions. (The spleen is highly involved in matters relating to the blood and to the immune system.) Of course, the steroidal treatment of DBA is also a potential connection to autoimmune conditions, because the only real treatment for most autoimmune disease is steroids, though, like with DBA, steroids are not a cure for autoimmune disease, either.
I do not know, yet, the full implications of a connection between DBA and autoimmune disease. But, it could be important to understanding the disorder, and, to finding a cure.
***
Adahlia has been amazing since my surgery, which happened just over a month ago. Immediately post-surgery, she would help gently roll me onto my side, and then she’d climb down from the bed to help lift and swing my legs over the edge of the bed, and then climb back up into the bed to push me from behind, up into a sitting position. Then she’d push me further until I was standing. She would get my cane for me, and push me from behind as I walked. She did this all on her own, with no prompting. She would ask to see “mama’s cuts” and say affirmatively: “mama’s cuts getting better.” She ate (natural) jello and watermelon with me. She slept by my side.
Tonight, we made chocolate chip cookies from scratch for the first time, ever. She helped pour the ingredients into the bowl, and place the dough on the cookie sheet.
A few days ago, Adahlia did her first series of “why?” Joe was driving the car as I answered five questions in a row about why it was important to eat a sandwich and not just chips. It went something like this:
“When we get to the sandwich shop, you need to eat the sandwich and not just chips, ok?”
“Why?”
“Because the sandwich has turkey and cheese and veges to help you stay strong.”
“Why?”
“Because there are vitamins and minerals and things your body needs in the sandwich that chips don’t have.”
“Why?”
“Because chips are a snack food, and they are tasty, but to be healthy, you need to eat real foods.”
“Why?”
“Because we want you to be as healthy and strong as possible.”
“Why?”
“Because we love you, goober-snick.”
Joe was astonished. “Isn’t it a little early for her to be doing this?” he asked. “I thought the ‘why’ thing started happening when they were, like, four?”
I laughed. I didn’t know.
But I do know that Adahlia surprises me every day. About a week ago, she surprised me again by starting to sing the “Part of your world” song from The Little Mermaid. She sang one line, I sang the following line, and then she sang the following line, and I sang the line after that… and on, and on, and on. When the bridge to the song came, I thought, Surely, she won’t know the next line, but she did. I could not believe that she had memorized the entire song! She doesn’t actually watch the movie that often.
For the two weeks post-surgery where I couldn’t really do anything because I was too weak, Adahlia and I spent a lot of time under the bed sheets, playing that we were under a tent. “Purple dadu-in!” she’d shout, pointing at the closet or out the bedroom door. “Oh no! Quick, hide!” I’d say. She’d dive under the covers and I’d pull them over us. She’d put her finger over her lips. “Shh… ” We’d lie together, acting scared, and giggling, for awhile. “Do you think he’s gone?” I’d ask. “You check.” I’d lift the sheet the slightest bit, and she’d squeal, “There he is!” or she’d say “All gone!” or she’d ask me to make him go away. In the latter case, I’d lift up the cover the slightest bit, exposing only myself, and chide the invisible dragon while Adahlia watched with big, amused, eyes: “Now, that’s enough Purple Dragon. Adahlia doesn’t want to be scared anymore. Go on, go play somewhere else!” Then I’d lift the cover further so she could see out, and ask: “Is he gone now?” “Yes!” she would announce, triumphantly throwing the covers off us both.
We still play under blankets and sheets often. She’ll say, “Mama, you come in, too! Both hide!” Its irresistible.
Sometimes, when the Purple Dragon comes into the kitchen, we feed him a snack, and when we do, he usually continues on his way, going out the back door of the house. Sometimes, its not the Purple Dragon but Seagulls and Dolphins that plague her. “Seagulls, leave me alone!” she says to the air, huffing and turning away.
Other times, the Dolphins and Seagulls are Adahlia’s friends. Sometimes, she plays as if she is hurt on the ground, crying. When I walk over to her, she says that the Dolphins come and lift her up when she’s sad. I ask her if she wants me to lift her up. She usually says yes, so I do.
These days, Adahlia’s favorite quiet-time activity is snapping a crayon into two pieces, and then peeling the paper wrapping off the wax. She’s currently working her way through a giant box of crayons. She works very diligently, every day, at breaking and peeling crayons. I can’t really blame her… there is something very alluring, very sensory-satisfying about the smooth waxiness of a cleanly peeled crayon. Yup, the paper has got to go.
Adahlia loves to share things with me. If she is eating something particularly good, she insists that I try a bite, too. Or, if she is having a special treat, she’ll insist that I take one for myself, too. Only then does she break into a big grin, satisfied we are both enjoying it.
It is my fault, entirely, but Adahlia has developed a love for tea. It started with sips of my own tea, and now she wants her own cup. We make it with honey and lots of milk. Her preferred method of drinking tea is to drink it by the spoonful. Its a meticulous process and takes 100 times longer than simply drinking the tea, but she loves to dip her spoon into the cup and draw it carefully to her lips. One of her favorite ways to show her love is to bring myself, Joe, or the cat, a pretend cup of tea from her wooden tea set and a piece of wooden cake. “You have some cake, Hamiya?” she asks the cat, though it is more of an order than a question. He looks at me, bewildered, allowing her to press it to his nose.
Adahlia is very expressive of emotions. When excited, she’ll laugh and stomp her feet and clap. “I’m so happy!” she’ll exclaim. When I tell her that she’ll have to have a blood transfusion in five days, and then, in the following days, I amend it to three days, and then two days, and then one day, (so as to prepare her for it), she replies, “It might hurt.”
“Yes, it will hurt. We have to do it though. You will feel better after it.”
She sighs. “I might be sad.”
“That’s okay, its okay if you’re sad.”
“I might cry,” she says.
“That’s okay, too. Crying is okay even if you’re very brave and strong. And you are.”
She sighs again, spies a bug, and the conversation turns.
Speaking of bugs, as I believe I’ve mentioned, Adahlia loves bugs. She loves Roly-Polys and earthworms and ladybugs. She once brought me a earwig but I couldn’t help myself – I knocked it out of her hand and told her that we don’t pick up those kinds of bugs. She thinks flies are bumblebees, and even though I’ve corrected her, she still calls them bees. She picks dandelions indiscriminately. By that, I mean that she doesn’t care if they are yellow flowers, or giant puffs of tiny, feathery seeds, or if they are closed. She picks them and carries them and stacks them in large piles. She keeps some, hands me one, and discards the others over her shoulder. She pulls opens the closed ones, shows me the fluffy interior, and says, “This one is Dub-Mai!” (Bob Marley). It means good things.
Adahlia reads to herself and her stuffed animals. Sometimes, she makes up things on the pages, and it usually goes something like this: “This one has puppies and kittens. This one also puppies and kittens. This one Dub-Mai.”
Other times, Adahlia actually sounds out the words. She knows all the letters (and can count almost continually to 20). She will say: “Gooo-ooo-d Ni—hi-hi-hight Moohooo-ooon.” Its very fun.
Adahlia will also “read” Hop on Pop to herself. She says almost all the correct words, such as “All Tall” or “Fall Off Wall” or “We Like to Talk”. Its as if she’s truly reading and its pretty impressive… in reality, of course, I know she’s just memorized the book. (Its been one of her favorites for years.) But, that’s pretty impressive too.
There are instances when we suspect that Adahlia may be a little dyslexic. For example, she says “pazzi” instead of “pizza” — switching the “i” and “a.” Its really cute, and I find myself having to resist the urge to start calling it pazzi, too.
Okay, last story.
I bought a dry erase calendar for myself. I thought it might be nice to put the calendar behind the table that serves as my desk. I thought it might be nice to plan things. But, I made the mistake of showing the calendar to Adahlia.
“Look,” I said. “See? You write the numbers in these little blocks, and in the big blocks, you can write what you have to do on that day.” I filled out the entire month of April. Adahlia was impressed. A few hours later, I saw that my chair had been pulled up to the wall so that she could reach the board, and the entire board had been erased, and it was now full of cryptic squiggles, small squiggles carefully placed in the tiny date boxes, and big squiggles filling the big boxes. She had even drawn a wavy line where the month was supposed to be written, and a couple additional wavy lines in the “Notes” section.
And it was my turn to be impressed.
Currently, the board is half-filled with animals. When I tried, failed, and realized I could not reclaim my board, it became hers. When she isn’t filling the board with her own version of a calendar, she is asking me to draw animals, trees, flowers, people, and other things in the various little boxes. If she asks someone else to draw something (such as her dad or a grandmother), and they do not draw it like I draw it, or the way she wants it drawn, she’ll scowl and erase it, and ask them to draw something else. But if she likes their drawing a lot for some reason, and it has a lot of value (to her), she will be protective of that drawing and make sure it doesn’t accidentally get brushed or erased. Such drawings needn’t even necessarily be ‘good’ – I find myself wondering exactly what she finds so appealing about a particular squiggle. But its very charming, and very reaffirming of the truth of how everything anyone is fond of is nothing more than something that happens to please our fancy. Not right, not wrong, not better, not worse. Just, pleasing.
Well, its an early morning tomorrow. I have to go to my hospital to get some post-surgery lab work done, and then we have an early-morning blood transfusion appointment. I am actually very thankful that I’ve had to have such intense medical intervention since her birth. In a way, I feel that my surgeries, hospitalization, various blood draws, and doctor consultations normalizes her experience. She doesn’t realize that the majority of the population doesn’t frequent hospitals as often as we do. And so I’m looking forward to my needle-stick tomorrow. She’ll watch fearlessly, holding my hand.
And then, an hour later, I’ll hold her.
Last, but certainly not least, I want to say that I take this Memorial Day to remember the children, young adults, and adults who have died from DBA and its complications. I mean no disrespect to veterans (I am a veteran myself) and those who have died in service. I merely mean to say that the experience of the loss of someone brave, strong, and loved is universal. There are many children who have died from this blood disorder. They were very brave, very strong, and very loved. There are many children and young adults fighting for their lives in intensive care, right now, due to this disorder. Adahlia could be among them tomorrow, or next year, or in three years.
I have to try. I will continue to try to cure her, working with her doctors and an integrated, holistic perspective, to free her from this disorder, and give her a healthy tomorrow.
But in every circumstance, and in every way, remember:
We have only today.
