The Birthmark

Yet, had Aylmer reached a profounder wisdom, he need not thus have flung away the happiness which would have woven his mortal life of the selfsame texture with the celestial. The momentary circumstance was too strong for him; he failed to look beyond the shadowy scope of time, and, living once for all in eternity, to find the perfect future in the present. Nathaniel Hawthorne, “The Birthmark”

Poor Nathaniel Hawthorne--The Scarlet Letter is pretty much a scarlet letter on his career. We hear “Hawthorne” and we typically think only of this piece of his canon; too often, this very prominent mark on his name is the only one discussed in high school courses.

This is unfortunate, because Hawthorne produced some pretty great work during his six decades of life. One of his short stories and the inspiration for this post is “The Birthmark.”

In the story, a scientist (or perhaps more accurately, a pseudoscientist or an alchemist), Aylmer, falls in love with a woman who is incredibly beautiful. Georgiana is perfect in every way save one: she has a small birthmark, shaped like a tiny hand, on her cheek.

This birthmark is of little consequence to Georgiana’s many male admirers and suitors; in fact, it is “often called a charm” and is said to be the mark of a fairy who “at [Georgiana’s] birth hour had laid her tiny hand upon the infant’s cheek, and left this impress there in token of the magic endowments that were to give her such sway over all hearts.”

When Aylmer and Georgiana wed, all seems initially fine. But over time, the birthmark slowly becomes all that Aylmer can see in his spouse. And because of his disgust, it becomes all Georgiana can see of herself, too. And as the story continues, this seemingly skin-deep flaw gradually comes to be called the “fatal birthmark” by both of them.

Georgiana begins to believe that only the removal of the birthmark will restore joy to her life.

“Again: do we know that there is a possibility, on any terms, of unclasping the firm gripe of this little hand which was laid upon me before I came into the world?”

“Dearest Georgiana, I have spent much thought upon the subject,” hastily interrupted Aylmer. “I am convinced of the perfect practicability of its removal.”

And so, she agrees to allow her husband to remove the hated birthmark in his lab. She understands that the procedure may be dangerous, but she has become so convinced that life is miserable that she is willing to risk anything in order to remove the blemish.

Several treatments come up short. In a final attempt to remove the birthmark, Georgiana drinks a potion prepared by her husband. She falls into a deep sleep and as Aylmer watches with delight, the birthmark begins to fade. Georgiana awakes and looks in the mirror to see the final stages of its removal, but she tells her husband that she is dying at the rate the birthmark is fading: the treatment has been as fatal as the birthmark was perceived to be. In the end, the birthmark is gone--but so is Georgiana.

Wow. What a brilliant story, right? Especially when you analyze its deeper meaning. Which I'm totally not going to do, except with a few words...

A rare and incurable disease is not the same thing as a superficial birthmark, but then again, even Hawthorne wasn't really writing about a superficial birthmark. In this great piece of allegory, the birthmark represents the imperfections that make us human. Rob us of our imperfections, and we lose our humanity, much like Georgiana sacrificed her life in an effort to rid herself of something that she had ironically deemed "fatal."

But I'm going to deviate from Hawthorne's deep meaning and be slightly more literal. Hawthorne was, after all, a transcendentalist thinker, and transcendentalism is just a tad too esoteric for my simple mind.

What I'm getting at here isn't that we shouldn't seek cures for rare diseases. In fact, I hope and pray every day that a cure for cystinosis is found in my lifetime. I believe it will happen, and even if this cure isn't available to me, I know I will be around to see diagnosed members of the younger generation live without the disease.

What I'm actually getting at is that we can't allow a disease or condition to overtake our lives and become all we see. While raising funds for better treatments and a cure, we have to be careful that we are keeping a beautiful reality in check: disease is actually a small part of our identity that shouldn't define us and certainly shouldn't make us metaphorically uglier or fatally flawed. While living in denial won't work (there are medicines to take and appointments to keep in order for someone with a medical condition to stay healthy), it is important to realize that if you allow any part of yourself to become a negative focus in your life, it could soon become all that you see. While having a cure as your priority certainly isn't bad in and of itself, to think that your life is terrible right now without one--as Georgiana did in this tragic tale--can lead to a pretty miserable existence.

Trifling as it at first appeared, it so connected itself with innumerable trains of thought and modes of feeling that it became the central point of all.

Likewise (and this extends beyond medical conditions), becoming razor-focused on someone else's allegorical "birthmark" (even if you think you are helping them) can lead to unhealthy thinking on their part--this is the whole reason why bullying is such a serious issue.

Until now he had not been aware of the tyrannizing influence acquired by one idea over his mind, and of the lengths which he might find in his heart to go for the sake of giving himself peace.

How we think about ourselves AND our flaws absolutely matters. Where we place our thoughts, there we place our lives. May life be rooted in joy (which is different than happiness and certainly allows for sadness) no matter the circumstances.

On Rhetoric

Many people know of my questioning of the status quo of disease rhetoric. Probably these same people all roll their eyes when they sense another rhetoric post coming. I continue to challenge the words we choose for ourselves and for others because words, beautifully crafted or unintentionally regurgitated, matter. Changing words are often either the first step or the first sign of changing attitudes.

This week in one of the Facebook groups I am a part of, someone posted a story about a man diagnosed with stage 4 cancer who chose to hike from Mexico to Canada on the Pacific Crest Trail. The story itself was a beautiful tear jerker that ended with his death - a death that he knew was coming regardless of what he did with his time left.

What really moved me, however, were the comments on the post. You see, this was not posted in a "support group" - it was posted in a closed group devoted to long-distance hiking. Therefore, some of the comments by those with unique health situations were obviously made without much premeditation, uncensored for a crowd that obviously shouldn't take any comments about illness personally.

One of the commenters, a woman who could directly relate to the man featured in the story because of her own long-term cancer diagnosis, said, "Choosing to live a normal life was the key to my survival. I didn't go to support groups - they sat around whining about their conditions. I didn't want to immerse myself in what I call the 'cancer culture.' I just wanted to live a normal life. And I have, for 35 years since my diagnosis."

This comment really got me thinking. Are support groups helpful? Ultimately, I think it depends on the person, and I have seen some people be completely lifted up by support groups. (The key word in such uplifting groups is, indeed, "support.") But a lot of times, these groups can turn into a sort of misery-loves-company kind of atmosphere, with people lamenting hardship (and these lamentations tend to be either contagious or competitive).

We dismiss those who keep themselves outside of these support groups, whispering words of judgment and throwing around phrases like "unhealthy denial."

It is a tricky balance. We need human interaction, support, and empathy directed our way. But to turn everything into a hardship is pretty dreary. Sometimes when I share, I internally recoil when others turn my experience into a hardship that I'm not, in fact, experiencing. What do I have to gain by seeing a learning experience as hardship?

I don't want to hurt anyone who relies on support groups for comfort. (Edit: or INFORMATION! I am entirely grateful for information I have learned through coming together with others.) It is very healthy to seek out support, and I am enormously grateful that such groups exist. I only want to gently encourage balance and perspective within these groups and also stress that what we see as "denial" may, in fact, not always be unhealthy. (Disclaimer: I don't consider "I don't really have an illness and therefore I don't need to take medicine" to be healthy denial. Realistic denial is understanding that when you are doing everything you can to stay healthy, you don't have to conform to someone's preconceived notions of who you should be.)

Something a friend and I were recently talking about was the use of the phrase "cystinosis patient" to refer to individuals with cystinosis. It's a tricky one, because people with cystinosis are generally followed by one or more physicians and have regular blood tests and medical appointments. On the other hand, so do lots of people who we don't refer to as "patients." My last overnight hospital stay was 15 and a half years ago, in August of 1999. My husband had a more recent overnight stay (also years ago), but I don't refer to him as a patient. Doctors and nurses did - while he was in the hospital. Is it helpful or damaging to automatically label people with cystinosis as patients? Does this phrase have a redundant eternal quality to it? (By that I mean, your cystinosis lasts a lifetime. Why suggest a lifetime of hospital stays as well?) Do we accept the patient mentality without questioning?

When you grow up with a rare disease, you are often exposed to your own exceptionalism. Sometimes I think the rhetoric reflects a desire to hold on to that, and we desperately apply it to entire communities in hopes that the suffering will stick, that people will notice our struggles and salute us. The truth is our communities are so varied, and while it has become (rightfully) wrong to apply universal stereotypes such as "black people can dance" and "Asians are good at math," it is still perfectly acceptable to say that "cystinosis patients suffer with ___________."

I will never deny you your suffering. That is yours to have, though I wish I could take it all away. But let's carefully consider how we apply words to an entire, albeit small, population.

I am hardly alone or original in these thoughts. For a great resource on people-first, positive language, see http://www.asha.org/publications/journals/submissions/person_first.htm. Notice that the first word listed under Principle Four is, in fact, patient.

Strengthening Trio

One of the side effects of cystinosis in adulthood can be muscle wasting. This is something that is so important to face head-on, and not just in the cystinosis community - the reality is that with age, muscle deterioration can affect almost anyone who isn’t diligent with a maintenance routine. This is just more true with cystinosis (often). The best defense against muscle wasting in cystinosis may be treating cystine buildup with a cystine-depleting medication such as Cystagon or Procysbi, but I don’t believe this is enough.

Here is a trio of products. As with anything, please check with your doctor if you plan on changing your regimen.

Strengthening Trio

Pulmonary Strength: Elevation Training Mask 2.0 This may sound extremely gimmicky, but according to a lot of people it isn’t - to many it is totally legitimate, used by professional athletes and coaches. The mask emulates high altitude conditions by creating pulmonary resistance and thereby forcing you to use available oxygen more efficiently.

There are two main muscle groups that assist with breathing, the diaphragm and external intercostal muscles. This mask seeks to strengthen these muscles, which also work with core muscles due to their positioning. (According the the Journal of Applied Physiology, for example, the diaphragm musculature can be thickened with sit-ups and bicep exercises.) So it stands to reason that if you forced the breathing muscles to work harder (by limiting oxygen) while also doing core muscle exercises, you'd get an extra benefit.

I'll be honest. The first time I put it on (set to 9,000 feet), I didn't feel like I was breathing at 9,000 feet (an elevation I am familiar with) - I felt like someone was suffocating me at 9,000 feet. I initially called it my "panic attack training tool" - how long can I calmly wear this thing before I rip it off due to hyperventilation in the face of ridiculous (and unfounded) fears regarding the fate of my life resting firmly in its grasp? But because it comes with different settings, you can easily start at a lower elevation and work your way up. And honestly, I got used to the 9,000-foot setting fairly quickly and feel comfortable doing exercise at that level (with plans to increase the resistance when I feel ready).

Grip Strength: DynaFlex Pro Gyro Powerball I first saw this hand strengthener being used by trainers at the rock climbing gym I frequent. It is designed to build wrist, grip, and forearm strength by way of a gyroscope mechanism that can spin up to 150,000 rotations per minute.

The concept is simple: keep the gyroscope moving by rotating your wrist while maintaining a firm grip on the ball at the same time. Simple concept, difficult exercise. Because my hands are fairly weak, I feel like the primary benefit to this product is its strengthening of my forearms to compensate. A lot of our grip strength is actually found in our forearms and upper arms.

Here's a short video of me using the product.

[video width="640" height="360" mp4="http://rollerskatingwithrickets.com/blog/wp-content/uploads/2015/01/powerball.mp4"][/video]

 

Overall Strength (via science or placebo): ZMA So here's the thing. I'm not sure I believe that this supplement does anything in terms of helping with muscle growth. Many claim it does, but (I hope) I am not that naive. At the same time, it includes zinc, magnesium, and vitamin B6, all found naturally in foods. I take two capsules at bedtime in hopes that it lives up to some of its performance-enhancing claims, but even if it doesn't, I'm okay with its contents. Note that women should take less than men.

Final Thoughts Different things will work for different people, and any sort of exercise enhancement that you don't use is a useless one, no matter how many people sing its praises. The most effective tools are the ones you will use. This is something I must daily remind myself of, especially in light of the fact that I believe that these products - especially the first two - can help, if only I stick with them.

Finally - and I hate to be a downer, but - it's important to remember that you cannot "spot strengthen" any isolated areas of your body. You really need to be working out your entire body in order to positively impact any one area. If gaining muscle were easy, there would be a pill for it and the media would be all over it. Sorry!

Have experience with any of these products? Drop me a line in the comments!

 

Lessons Learned from Hiking the John Muir Trail

This article first appeared in the Cystinosis Research Network’s Fall/Winter 2014 newsletter. I encourage you to check it out there, along with all the other wonderful articles. Cystinosis is the best thing that has ever happened to me.

I mean that not as a polarizing statement, but as a personal one. I have always enjoyed the gifts cystinosis has lavishly bestowed upon me—even those that come disguised as hardship.

It's a good thing that I feel this way, because cystinosis is written into my DNA. I can't just shake it off Taylor-Swift-style. It's good to avoid hating your DNA, because, well, you're kind of stuck with what you got. Literally. Unless you get those eye color-changing contact lens. Those are awesome.

But there is no denying that cystinosis can be destructive, and it has cost many people their lives. Too many. That is why the Cystinosis Research Network is so intent on raising funds, building awareness, and bringing families together for support. I am so grateful that such a community exists, and sometimes (OK, a lot of the time) I feel like I don't show that appreciation or give back enough in return. Maybe it's a zero-sum game: but I'm still trying.

My husband Wayne and I did a charity hike for CRN in September. This wasn't something that we planned far in advance; in fact, although hiking the 211-mile John Muir Trail (plus the combined 10 miles it takes to reach it and exit it) had been a dream of mine since I started hiking to combat pulmonary decline in early 2012, it wasn't something I thought we'd ever be able to schedule logistically.

99th Switchback

But a pretty crazy blessing happened in July: I lost my teaching job. The K-8 school where I started teaching in 2005 decided to close its doors to the upper grades and continue as just a K-5, leaving me to continue working at a job that I thought would be for the summer only.

My first thought—before I even stopped to feel sad about the loss—was that without the school year constraints, now I could make the John Muir Trail a reality in September! So I put in a request for two weeks off and Wayne and I got started planning.

We wanted to take the cystinosis community with us on this journey, in part because I only ever took up hiking and considered the John Muir Trail because of cystinosis. (Remember how thankful I am for this beautiful mess of a disease?) I wanted to have my heroes on my heart—so I literally put them on my bag, attaching buttons of individuals with cystinosis to my pack. We dedicated each mile hiked to an individual based on a sponsorship price of $20 per mile. Although not every mile of the trail was sponsored, we raised almost $3000 for cystinosis research and had many miles covered.

Many people we encountered on the trail asked about my pack. It was nice to be able to share about cystinosis.

There were truly moments when I didn’t want to continue. There was even a time when I had to come off the trail due to difficult circumstances and reenter after a few recovery days. I found it hard to eat and the cold almost unbearable. Most of the trail is above 10,000 feet and no section of it until near the end is flat—it is a constant up-and-down over mountain passes. We traveled south-to-north, starting with a hike to the top of Mt. Whitney (the highest point in the contiguous United States) and ending in Yosemite Valley (although a fire closed the final 12 miles of trail).

But those with cystinosis constantly motivated me. I kept aware of what mile we were on and knew who I was hiking for. A landmark would appear in the distance—Guitar Lake or Donahue Pass or Crabtree Meadow or Garnet Lake or any number of other places—and I would think about the individual whose mile it represented. Several hikers asked about the buttons on my pack, the most common question being, “Are those your kids?” (say what?! There were 40 buttons on that pack!), and it gave us a chance to describe cystinosis to a wide variety of strangers.

I wish I could share with you all the thoughts that went through my head as I thought fondly of those for whom each mile was dedicated, but those are not my stories to tell. Needless to say, from those who are on dialysis to those who have recently received kidney transplants and one sweet individual who recently passed away, each and every person inspired me.

Here are some things the John Muir Trail taught me about life, love, and cystinosis:

  1. It won’t be like walking on the moon.

One morning we crossed paths with a solo hiker headed the opposite direction and let him know we were headed toward Donahue Pass at 11,000 feet. We were around 9,500 feet at the time.

“Oh my,” he said. “You have a long way uphill.” He must have seen the melancholy written all over my face for he quickly added, “But it’s like walking on the moon. It’s beautiful up there. Otherworldly.”

Six hours later, I struggled to experience the moon walk. My pack was still heavy. My body was not weightless. Gravity still tugged at my every muscle, silently pleading, “Sit down.”

Weighing our packs at the Mt. Whitney trailhead. With all our food, camping equipment, and other necessities, W’s pack weighed 55 pounds and mine weighed 38.

But there is a force greater than gravity, a voice louder than Isaac Newton’s, and that is the voice that says, “Here you are, weight at all.”

The cystinosis life is not weightless. You cannot put down your heavy pack. But you should carry it. You must carry it. It’s not going anywhere unless you do. And with a little practice, you can carry it with joy.

The trail was rocky and almost constant up and down, but to wake up to the scenery every morning was incredible.

The alternative is to look at your heavy pack, plop yourself down on the couch and refuse to move, and complain about how heavy the pack sitting beside you is... and explain to others how it gives you a reason not to move.

  1. You’re always almost somewhere.

Another day, we passed a hiker who heartily patted me on the shoulder as he headed was down and we were headed up. “You’re almost there,” he said.

Almost where? I wondered. The audacity of this guy, assuming he knows my journey!

But we are always almost somewhere. Maybe it’s not always close to where we think our destination is, but we are always near the next landmark of our lives. We become so laser-focused on an important end goal (say, a cure) that we forget that our journeys are varied and complex, brimming with life, and worth experiencing 110%.

  1. The right way is usually the up way.

Whenever the trail forked, I knew our path was the one that went up. And lo and behold, that was also the path that led to the sweeping mountain passes and overlooks.

Thousand Island Lake

I hope the sting of this quote is lessened by the fact that I started hiking due to an unfortunate pulmonary diagnosis that I’ve since managed to turn around. John Muir said, “Few places in this world are more dangerous than home. Fear not, therefore, to try the mountain passes. They will kill care, save you from deadly apathy, set you free, and call forth every faculty into vigorous, enthusiastic action. Even the sick should try these so-called dangerous passes, because for every unfortunate they kill, they cure a thousand.”

  1. The toughest days are not necessarily the ones with the steepest paths.

One of the days I most dreaded while preparing for the JMT was Day 2. This day included the summit of the highest mountain in the Lower 48, which necessitated a 99-switchback, 2000-foot climb first thing in the morning.

I built this challenge up in my mind, but in the end, it wasn’t the one that took me down. It was the steepest path I had to walk, but I was also ready and on my guard.

It was Day 3, a day I had predetermined as “easy,” that I really struggled. An unexpected storm brought freezing cold and treacherous lightning. The altitude I had quickly attained robbed me of my appetite. And a single mile dragged on for hours.

We encountered a lightning storm and a couple of overnight freezes.

We don’t often anticipate the tough (unless you anticipate every day to be an upcoming battle—which, to be honest, I don’t recommend).

  1. If you did it yesterday, you can do it today. If you do it today, you can do it tomorrow. Kind of.

Obviously, this argument is fundamentally flawed; if it were true, we could live forever. But the idea behind this one is that you need to keep going, being ever diligent (while not seeing it as survival or a battle or work) as you live this life. Building off #4, you have to take care of yourself in order to continue on the trail day after day.

Johnson Meadows, one of the locations where we expected to find water to filter but instead discovered more of a stagnant puddle.

  1. Hike your own hike.

This is a popular mantra among hikers, backpackers, and peak baggers. In a nutshell, there is a lot of advice out there as to how to complete a thru-hike like the John Muir Trail. But it may not all fit your unique circumstances. Similarly with cystinosis—make your experience your own while never refusing to accept or give supportive words to others who may be experiencing things quite differently.

  1. Love the one you’re with.

This is your life. You can’t trade it. Love it for all it contains. This is a lesson that I learned (or rather, realized again) before I even hit the trail. We aren’t given many years on this planet, and losing a job reminded me of how much stuff there is to do in not-so-much time. How fortunate to be able to explore one more thing because of a circumstance others may see as unfavorable. How can I view cystinosis as unfavorable when it has propelled me to these adventures that afford me such tremendous views of creation?

On a more personal side, my love for Wayne only grew during the challenges of this experience. Not only did he carry our communal gear, but he also set up the tent each night and cooked the food. He was and is a huge support in everything, and I couldn’t be more grateful. He has humored me up Mt. Kilimanjaro in Africa, motivated me on the John Muir Trail, and encouraged me to challenge what it means to have cystinosis.

My partner in life and love.

We are all hiking a life trail every single day. There are mountains to summit and valleys to cross. I hope that the trail is smooth and glorious for every single one of you in the cystinosis community. But if that’s not possible, I hope it’s challenging and glorious.

“In this life we are all just walking up the mountain and we can sing as we climb or we can complain about our sore feet. Whichever we choose, we still gotta do the hike. I decided a long time ago singing made a lot more sense.” –Unknown

We Are Imperfect

As many of our friends and family know, W and I have made the decision to adopt. I can’t put my finger on when we first made this decision; W and both of his brothers were adopted, so obviously the thought has never been a foreign one to us. But I think it was when W’s parents died and we met his biological mother for the first time that I realized that adoption is something that can truly be a “twice blessed” scenario: we were blessed once with W’s family, and once again when his genetic family entered into our lives. I have mixed feelings about the adoption process itself. I’ve self-censored a lot of my thoughts due to the fear of being Googled by women considering us as possible parents, and as a result I’ve remained mostly silent. But I've noticed this silence on the part of other adopting couples, too. The home study, the profile, the wait, the personal feelings of inadequacy... no one seems eager to share much about the process until it's over. And truth be told, a lot of people who have not adopted or are not in the process of adopting make quite a few assumptions about their adopting friends. And maybe it's not benefitting the larger adopting community.

So here it is: we are imperfect. Incredibly, deeply imperfect. I have a genetic disease that many would like to label as life-limiting, and though I do live without limits (and we would not have passed our home study if my good health were not a provable fact), any child of mine is going to see his or her mama taking medicine. It’s never going to be a spectacle or show, but it’s going to be there. But more importantly, because of this small second-nature act on my part, I’m going to be there, too. (And according to my doctors, I’m going to be there for a very, very long time.)

But aside from this elephant, our imperfections extend even further, from our apartment lifestyle (we don’t have the huge house and big yard found in may couples’ profiles) to our somewhat camera-shy personalities (oh, how inadequate I felt when our adoption facilitator said we needed four more pages of photos in our profile!). We are not self-promoters and can only say whole heartedly and with complete integrity that what we do have to offer our children is love.

I’ve come to the conclusion that we cannot be afraid to hide our imperfections, while at the same time acknowledging that we must be careful about oversharing on the Internet, just as we would teach our children. As Jolie O’Dell so appropriately states, “…if you’re writing a story about yourself, well, just remember, the internet’s memory is long. Even if you delete your post, it’s guaranteed to be cached or archived on some server somewhere. So carefully consider how personal you want your personal stories to be.”

Image-1

I’d love to hear your experiences if you’ve been down this adoption road, carrying all of your imperfections with you. I’ll be honest and say that some elements of it seem so fake-candy-shell-colored pink, from the shiny profile booklet we had a graphic designer put together to the smiles we put on our faces when we are asked incredibly personal questions by a social worker conducting our now-thankfully-over home study. How have you let your authentic love take the lead in what can start to feel like an inauthentic process?

Look for at least one more future post on this process, but for now, this is what is on my heart.

How To Choose A New Doctor When You Have A Rare Condition

It's that time of year - open enrollment! If you're like me, change can be a little bit nerve-wracking. But if you are an advocate for yourself and your current health plan has not been meeting your needs, now is the time to take the plunge and make that change. So how do you go about choosing a new primary care physician (PCP) if your current doctor is not in your new plan's network? I wrote this a while back when working on a transition project with a nonprofit patient group but the guidelines are applicable any time you make a change.

Here are some things to consider when looking at doctor profiles:

  • In what area is he/she Board certified? (Please make sure he/she IS Board certified!) Because Internal Medicine is a more comprehensive certification than Family Practice, I generally go with an Internist. If you opt for a PPO rather than an HMO, you may be able to select a specialist, but I still like having one doctor who can serve as a central person for any issue, even if that means referring me out because the issue is beyond his or her expertise.
  • Does he/she have special certifications in addition to Internal Medicine? I look for a person with additional certification in endocrinology. Although the doctor will still be an Internist and not an endocrinologist, this means he/she probably has special interest in metabolic conditions.
  • Where did he/she attend medical school and complete his/her residency? Be on the lookout for doctors who have attended or worked at some of the top medical schools and hospitals in the country. U.S. News & World Report has listings for both top medical schools and the best hospitals.
  • How many years has he/she been practicing medicine? I occasionally go with newbies, because I feel that their enthusiasm for the field might be an asset. But usually, the more experience, the better.
  • Read carefully between the lines of an online bio to see what his/her strengths are. Look for lines like "enjoys problem solving challenging medical situations." (I've seen such a line.)
  • Go to www.vitals.com (or a similar site, preferably health specific and not yelp) and do a search for each person on the narrowed-down list of physicians that you are considering. Look for reviews from people with complicated cases. If there are negative reviews, read them and try to determine what makes them negative - I will usually forgive a negative review that seems solely based on the receptionist's bad attitude, but if a negative review mentions the doctor's lackadaisical demeanor, he doesn't make the cut. As with any reviews, be wary of reviewers who seem more interested in selling someone else rather than giving an honest appraisal of the doctor you are interested in.
  • Has the doctor ever seen another patient with your condition? Admittedly, this is not likely to be in his/her profile if your condition is rare. But it never hurts to place a call or shoot an email to the doctor's office asking (before making the selection) if he/she knows anything about your condition or is even interested in seriously taking on a patient with it. The response might be very telling.

Most of all, remember that you are in control. If you find that the doctor you selected is not meeting your needs, let your insurance plan know that you'd like to change.

HAWMC Day 6: Letters

Dear Cystinosis, When I hear people say they hate you, I get defensive on your behalf. Something inside me cringes.

I want to say, "Don't hate the hungry lion for being hungry. Look at his beautiful mane."

But I don't say it. I can't say it.

I have said it using other words before: just as I cannot hate my brown-sometimes-green eyes or my neither-curly-nor-straight hair, I cannot invite the sort of self-loathing that comes with hating a part of who I am. A part of who God created me to be. A part that has blessed me more than it has cursed me.

Sometimes I imagine being born without you. Would I push myself to climb mountains?

Maybe you would, a voice whispers. Because that is who you are. But maybe the mountains would look different. And maybe without the hungry lion chasing you, you wouldn't push as hard.

If brown eyes caused someone else pain, or wasted away their body, could I hate the trait without hating myself?

I'm coming to terms with the fact that maybe I could.

But I don't hate you in me. I hate what you do to others. But I can't imagine myself ever hating what you've done - and undoubtedly will do - to me.

Like a mother hen protecting her chicks, I hold you fiercely within my feathers and wonder why others just don't understand.

But maybe I am the one who doesn't understand. How can I possibly tell someone who has lost a child, a sister, a brother, a spouse - how can I possibly tell that person to love you?

I am the one who doesn't understand.

Yet when I go (whether that be many years from now of old age or tomorrow in a car accident), I want my husband to share not what you took from me, but what you gave to me. When weighed against one another, the latter falls heavy on the scales and the former remains lighter than a single grain of sand.

You have made me the hungry lion. Hungry for life, hungry for feelings of empathy that come too rarely, hungry for changing how we all tend to view the world around us. Hungry to push harder with no one but myself chasing me.

Because I know that the joy in hearing "Your legs must be very strong!" from someone else observing my movements isn't that it's true (though I hope-wish-pray-long for it to be true), but that no one would have said that to me a year ago.

For all that you're doing in my life and all that you've done,

Thank you.

Jessica

 

 

Hope is a Two-Word Phrase

I'm at a place in my life where I am not only content, but also at peace with the fact that I might die tomorrow for whatever reason. (I hear North Korea has some missiles pointed at us on the West Coast, y'all.) I'm grateful for what I've had and ready for what may come. But I don't accept cystinosis as a reason for imminent death. So I am very glad to see this, published by the NIH:

"Prior to the use of renal transplantation and cystine-depleting therapy, the life span in nephropathic cystinosis was no longer than ten years. With these therapies, affected individuals can survive at least into the mid-forties or fifties with satisfactory quality of life."

There is much to be hopeful about within this excerpt. But even more specifically:

Hope is a two-word phrase: at least.

And then there is this, published in a piece by Drs. Gahl and Nesterova: "Renal allografts and medical therapy targeting the basic metabolic defect have altered the natural hisotry of cystinosis so drastically that patients have a life expectancy extending past 50 years."

All the sad stories go viral very quickly within my community. Do you have the courage to share true hope - the hope that we don't have to wait for, the hope that exists in the here and now? In our understandable zeal to raise research funds for the children, dare we share?

Hope is a two-word phrase: at least.

I may not be the bravest person around, but I have at least this much courage.

School Trips

This week's remembeRED prompt was: School trips. We all go on them. What trip do you remember the most? Where did you go? Who was with you? How did you get there? Have you ever been back? The funny thing is, I didn’t really go on school trips until becoming an adult.

Oh, I went on field trips to museums. That’s about the only thing I remember. And not to criticize the educational value of these excursions, but museums seemed very mundane compared to the places some of my classmates were able to experience: the week-long trip to the great outdoors in the fifth grade. The eighth grade adventure in Washington, D.C. Yes, even senior prom aboard a boat in the San Francisco Bay.

It’s not that I didn’t want to go on these trips. In fifth grade I was on so many medications that had to be taken every six hours around the clock that I feared what my friends might think at the sight of it. By eighth grade, I was adamant: no one must know of the disease that was slowly shutting down my organs. And by senior year, every other day was consumed by a four-hour after-school trip of its own: my regular dialysis treatments at Stanford Hospital. Non-dialysis days were dominated by complete exhaustion after a full day at school.

There is no doubt that I could have gone on school trips, had I reallywanted to and hadn't cared so much about what others thought. When I have a will, I tend to make a way. But I just didn’t have the desire to be “that girl” on an excursion, the one who had to have special accommodations or needed to sit down and rest a little more often than everyone else.

Years later, when I became a teacher, myriad opportunities for school trips presented themselves. This time around, people still didn’t know of my chronic illness, but as a post-transplant adult, I felt freedom to take such trips – and I knew that should students or fellow teachers observe me taking medication, I would turn it into a proud lesson in the value of being “that girl.”

The first trip I took was to the great outdoors – an overnighter in the Muir Woods, just north of San Francisco. I bunked with a group of seventh grade girls, officially as their chaperone and unofficially as their confidante during late-night conversations. I experienced all that I had missed out on 15 years earlier – the rustic accommodations, the morning hikes, the afternoon nature lessons, the cafeteria-style food. At some point in the trip, I realized that I wasn’t living vicariously through anyone, although my students were the reason we were on this adventure. I was living this too.

And so, when we reached an obstacle course with an 80-foot ledge up one of the amazing redwood trees, I knew that my days of vicarious living were about to be truly incinerated forever. Unsure that I would even be able to climb the tree due to muscle wasting in my arms and legs (another side effect of having cystinosis), I surprised even myself when I made it to the ledge in record time. With my kids cheering me on – “Jump, jump, jump; you can do it!” – I walked to the edge with shaky legs and prepared to make my “Lion’s Leap.” I looked down at the smiling faces below me, though I soon heard only the loud clanging of my knees inside my head.

I turned my face upward and looked at the magnificence that was all around me. Redwood trees shot into the air as far as the eye could see. I knew that somewhere up there, just as my kids were doing from beneath me, God was smiling down at me from above. I had made it.

And with that, too frightened to leap and grab hold of the swinging bar that was about six feet out from the edge of the blank, I instead fell backwards and let the harness and ropes catch me. On this trip, I was content to simply have the new experience of making the climb. I knew that next time I would jump.

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