One step forward, fifteen steps back

It's ridiculous how quickly our last, generally positive post became outdated. Christmas night was spent in the emergency room, after B's whole system freaked out, bringing on dizziness, blurry vision, and an inability to swallow. We (and her doctor) thought she might be having an allergic reaction to the new muscle relaxant she'd taken, so we rushed her to the hospital. As it turned out, it was not an allergic reaction, but a stress reaction—her body overreacted to the pain of the 2-and-a-half-hour-long muscle spasm she was having. So we were back home—at 2AM—with B stabilized. For the moment.

We'd hoped that maybe that was rock bottom, but it wasn't. After a particularly grueling PT session yesterday morning, B came home and took a nap. She woke up unable to move anything below her neck without extreme pain. Her right leg—the center of her RSD affliction—was suddenly the least painful part of her body. It was excruciating to bend her left leg. Her lower back ached too much for her to sit upright. Her elbows were so painful that she couldn't bring a cup of water to her lips to drink. Turning her head was agony. Noelle and I spent the better part of the day making emergency calls. We called every medical professional she had seen so far, and a few she hadn't even seen yet. A couple of them eventually called us back.

Turns out B is having an RSD flare-up, a total nervous system freakout that can be triggered when she is overtaxed either physically or emotionally. As one blessedly clear-spoken doctor reiterated to me, RSD is a neurological disorder—all the extreme pain comes from her nervous system misfiring. And it's that same nervous system that controls a body's stress and anxiety levels. The disease creates a vicious cycle (one that feels particularly cruel for young kids), in which pain creates stress, which in turn creates more pain, which increases the stress, which pumps up the pain levels, and so on. (So much for Schoolhouse Rock: "Every body understands those telegraph commands.")

Lesson learned from today's horrific experience: We can't beat this thing without treating both the physical and the emotional/psychological sides of it. Another lesson learned: We can't beat this thing on our own. We need to look into inpatient treatment.

We've been hearing for weeks—from doctors, PTs, parents of other kids with RSD—that the best surefire way to beat this disease is by enrolling in a very specialized, long-term, inpatient program with hours and hours of daily intensive physical therapy coupled with psychological counseling. There are a very few of these programs in the nation—and only one in our state. But as much as we've tried to avoid mentally going to that place, we've come to realize that we cannot fix this at home.

We've already begun making calls and have quickly realized that the logistical, red-tape challenge of getting into any of these programs will end up being the frustration icing on the crap-cake that is our life right now. They all have long waiting lists. Some have a prerequisite number of outpatient PT sessions that a patient must complete before they're even eligible to apply. And who the hell knows whether our insurance will even cover it. We've only managed to get an evaluation appointment at one so far—and that's not for several weeks yet. In the meantime, I hope and pray that B will wake up this morning with her system calm enough to let her get out of bed.

A Christmas Gift

Just thought we'd let you all know that after a really rough morning, during which Bryn was negative, frustrated, despondent and insisting she couldn't try any harder, her physical therapist kicked her butt into gear and got her onto the stationary bike. She achieved an 80-degree angle in the knee. Best yet. Merry Christmas.

Nothing is simple

Wonderful friends and family (you know who you are) ask me every day how B is doing, and I (Noelle) never know what to say anymore.

I could say that B is getting better. Her knee is bending just a few degrees farther every day. Since the nerve block, you can touch her leg without having her recoil in horror. A light breeze on her skin doesn't make her wince. She laughs at the Simpsons, while playing a board game, at Chris—sometimes even while he's pushing her to do ever-harder physical therapy exercises. At these moments, Chris and I feel like daily life is easing back into its normal shape. We can imagine an end to the chaos that has overtaken our household since November 20.

But then, you could ask me an hour later and I'd be far less positive. That knee, the one that (ironically) appears so lithe and graceful in photographs, refuses to bend again no matter how much pressure you exert on it. And when it finally does, she screams and begs us to let her swear, which we'll let her do (fuck it, why not) as long as her brother isn't around. Her face curls into an angry sneer: "Why do you keep making me do things I can't do?" "You don't understand what I'm going through." And her ankle just won't budge a millimeter: It's still stuck in the same damn Barbie foot position.

And then of course there are the nightly leg spasms, which start shortly after she turns out her bedroom light. They used to last about 10 to 15 minutes. Despite the meds she takes to control them, the spasms now last about an hour to an hour and a half. B and I lay there, trying everything to get them to stop: I shake her leg in time with the spasm; I massage the muscles; I distract her with the Colbert Report app or show tunes on YouTube; I make her lay on her belly, then her back, then her side; I have her hobble around the room on her crutches. Nothing makes the slightest difference. She wonders when this hell is ever going to end. I tell her, it will, don't worry. But I wonder myself. And I worry.

Still. As I keep reminding myself, fitful, imperfect progress is still progress. Case in point: About a week ago, the pain specialist and physical therapist both told us that, to aggressively treat her RSD, B might have to be hospitalized by late December/early January for up to two weeks. Reason being: RSD can spread to other parts of the body—it does in about 70 percent of cases. Also because her leg tendon is shortening the longer this goes on, and the damage can become permanent. And finally because her RSD is in an acute phase—so it's not yet considered chronic—and her best chance at remaining in remission is successfully treating this onset in the first three to six months. 

But as of the other day, both medical professionals concurred that B is making enough progress that we should be able to keep her at home—for the time being, anyway. It's not the Christmas miracle I might hope for (B bounding out of bed, fully restored and pain free), but it is enough for now.

I never thought I could experience this much joy simply from seeing someone bend her knee. But I tear up every time I see it. 

The hard work appears to be paying off. We've achieved what I'm estimating to be a 70-degree angle or so. Better yet, B can get there herself—she doesn't need us to bend it that far. We've been working at holding the bend for lengthier periods of time. So far, so good. This is by far the most hope we've had in a month. 

Yesterday may very well have been the hardest day of B's life so far. And it certainly wasn't one of our easiest either. The nerve block procedure was successful and has helped—to an extent. It has diminished the hypersensitivity in her leg, allowing us to finally touch the leg without causing immense pain. One of the most difficult parts of this for Noelle and me has been our inability to soothe the part of our daughter that hurts so much—we couldn't rub her leg or kiss her knee without making it feel worse. But those gentle caresses and soft pats that we, as parents, have been longing to give her can finally happen. For now, at least. Because the effects of the nerve block may prove to be only temporary.

The nerve block also did nothing for her mobility. Nor did it decrease the excruciating pain she feels upon anyone trying to bend her knee or ankle. And it was just such physical manipulation that B woke up to as she came out of anesthesia. The scene in the hospital yesterday morning is one I'll never forget, but would very much like to.

After being locked in a contracted position for so long, some of the tendons of her leg have already begun to shrink and shorten. This makes attempts to stretch them back into normal position all the more difficult and all the more painful. We can't let it continue to get worse. Which means more rigorous exercises, more frequently. Thankfully, B is now with a physical therapist who is not afraid to push her, and whom she likes and connects with on a personal level—someone B can still laugh with after that person just made her scream.

But the professional PT only happens once a day, and B needs to keep working on those joints throughout her waking hours. So the rest falls to us; stretches and bends (or attempts at bending, anyway) that have to be done constantly. And which alway hurt. A lot. But these exercises are the only way to make sure we get her leg back. I keep telling myself that as much as she hates all of this now, and as upset as she gets every time we say its time to work that leg, and as angry as she gets with us every time we tell her that, no, we can't stop doing this or no, we can't skip it his hour—she will look back on this in the future and thank us. I keep telling myself that because I have to. This is the hardest kind of love.

When B was little, and got a paper cut or a skinned knee, I'd ask her to squeeze my finger. "Mommy will take the pain away," I would say. "And then you'll be all better." We did this for years, because she always believed that this one simple gesture magically eradicated the hurt.

Two night ago, she asked to squeeze my finger in the midst of the longest pain spasm she'd ever had: 35 minutes of unrelenting torture. But then she stopped after about a minute, and just looked at me in that look I see so much now—in which her face is just twisted and contorted in pain. "It's not helping," she said bluntly. She released my finger and dissolved into tears, again.

Yesterday we saw the chief of pain management at a nearby hospital. He was a joy: He spoke with the average velocity of an Aaron Sorkin character, and the warm humanity of Dr. House. Within the first few minutes he informed me that B's case of RSD/CRPS was "very severe" and that he didn't understand how it could have been catalyzed by parvovirus (Fifth disease) because "there was nothing in the literature to indicate that was possible." He waved around a sheaf of papers. Had she had some other trauma? (No.) Was she stressed and did she want to avoid school? (A hundred times no: She loves school. The poor kid has been in tears over missing school. Though I would think even the most school-resistant child would have an awfully tough time keeping her leg extended and pointed 24 hours a day, 7 days a week, even during sleep, and screaming and weeping constantly.) "Well, then," he announced, "we will have to write this one up for the medical journals! This is unusual." He could barely contain his excitement.

Then, he was back to focusing on B. After bringing up (and abandoning) an idea to admit her into the hospital for the rest of the week so she could do physical therapy 3-4 times a day, he suggested giving her a sympathetic nerve block. I'd read about them, in reference to RSD patients: They're apparently a crapshoot with this disorder. Sometimes they deliver permanent pain relief to the affected area (pleasepleaseplease), other times they provide a degree of temporary relief. Sometimes they do nothing at all. So our fingers are crossed.

In early November, our daughter B was completely healthy and happy—just the same way she'd been the rest of her life. She was busily engaged in activities at middle school, from theater to orchestra to Model UN—and filming a movie she and her friends were writing together.

Her legs looked like this:

Normal.

On November 20, she complained of a sore back, a bad headache, and was running a high fever. Doctors were concerned about meningitis. By that night she landed in the ER with severe abdominal pains. Then doctors were concerned about appendicitis. Over the next few days, she suffered more headaches, more high fevers, and migrating pain. On November 22, she awoke and couldn't move below the neck, because the pain was so intense. It took both Chris and me (Noelle) to get her out the door to the hospital. Doctors, perplexed, tested her for Lyme disease, juvenile arthritis, rheumatoid arthritis, and parvovirus (fifth disease). Her test results came back positive for fifth disease, a normally benign childhood virus that results in (at most, typically) red cheeks and some joint discomfort. Heading into the Thanksgiving weekend, they told us not to worry, to wait it out and that she would get better. We were still distressed, but relieved.

In the meantime, B's leg and foot started to look like this:

They are in this locked, rigid position 24/7--even when she is sound asleep. The doctors agreed this was unusual, and not the typical presentation for fifth disease. A pediatrician friend suggested another explanation: a disorder called RSD (reflexive sympathetic dystrophy). Two days later, we say a pediatric rheumatologist who gave us that same diagnosis.

Chances are, like our family, you'd never heard of RSD. It's a rare, chronic neurological disorder that attacks the sympathetic nervous system.  Also called CRPS (complex regional pain syndrome), the disease is characterized by (and I quote the RSD Syndrome Association here):
• severe burning pain
• pathological changes in bone and skin
• tissue swelling
• extreme sensitivity to touch


B also experiences intense involuntary muscle spasms in her thigh and ankle a few times a day. How intense? Think labor pain-intense. And they last from five minutes (blessedly short) to 25 minutes (grindingly awful). She often has to bite down on a washcloth to keep from screaming.

A simple touch hurts her--at times, it hurts too much to keep a blanket on her leg, even when she is cold.

Right now, she gets around on crutches at home and in her wheelchair at school and in public. School for her has gone from being a delight to a challenge: Kids ask her what's wrong with her leg all day, and when she's going to get better. She never knows what to say. Her three hardcore pain medications (which so far do little to mitigate the pain, in my opinion) make her tired, and occasionally confused. Even though she has an accommodation plan in place, not every teacher seems to really understand what she's up against.

She is also in physical therapy five days a week. Plus we need to do exercises with her once an hour. Each time, they cause bouts of terrible agonizing pain, so it's hard on everyone. (The dog has been treated for stress and anxiety this week, and I am knocking back Pinot Noir some days at 3 PM.)

Despite all of this, B is keeping an upbeat attitude 90 percent of the time. We give her tremendous credit for that. She is just 11 years old, and was leading a perfectly normal life what feels like 5 minutes ago. Every night, after I coach her through the pain spasms, she thanks me and it breaks my heart just a little. Or what's left of it after watching her white-knuckle it through a trip to the kitchen, or after our dog merely brushes past her toes.

If there is any good news here, it's that we caught this early. Children with the best shot at full remission and no recurrences were diagnosed early and aggressively treated. There are no cures and no guarantees--many kids get flare-ups in years to come. And if love alone could bring her leg back to the way it was, she would have been healed weeks ago. But we are doing everything we can, and are determined to conquer this.

For more on RSD go to: http://www.rsds.org/aboutCRPS.html or http://www.ninds.nih.gov/disorders/reflex_sympathetic_dystrophy/detail_reflex_sympathetic_dystrophy.htm