Spring has sprung!

Greetings, faithful followers. I am still alive and kicking…okay, well, I’m not really kicking, but I am alive.

I just had my six-month check up at Yale with the endocrinologist who is managing my XLH and it was an informative and helpful trip. I am tolerating the XLH meds well, so well, in fact, that my doctor wants to increase them to improve my numbers a little bit. Now I’m up to three calcitriol pills per day and 4 phosphorous pills per day. I will know within a couple of weeks if I can tolerate the increase. Then, in 8 weeks I will have blood work done to make sure my parathyroid hormone is under control. It is very important to monitor both the parathyroid and the kidneys when you are an XLH person on calcitriol and phosphorous.* (See footnote–pun intended!)

While doing my “medical tourism” last week, as my family doctor calls it, I also found out that I have a broken foot. I had wondered why it has been hurting so much for the last year. I mentioned it again to my endocrinologist on Tuesday, telling him it still wasn’t feeling right and so he sent me over to get an x-ray. He called me the next morning to tell me that it was broken and that I needed to go see my orthopedist when I get back to NC. He said it’s an insufficiency fracture.

So there go my soccer career plans.

Anyway, I made an appointment with my orthopedist and saw him a couple of days ago. He said that he wasn’t surprised that my foot had been hurting for about a year, because this particular location in a foot  is a “real booger to heal” when it is fractured. I love having a doctor who is a Southerner like me and who speaks my language!

Just so you know, an insufficiency fracture is a type of stress fracture. What is the difference? The cause. Athletes get stress fractures caused by abnormal stresses on their normal bones. My orthopedist rattled off several names of famous basketball players who had gotten them in this same spot on their feet.

Mine is an insufficiency fracture. Here’s a good definition I found for it: “Insufficiency fractures are a type of stress fracture, which are the result of normal stresses on abnormal bone” (From radiopaedia.org.). Yep–I’ve got the abnormal bone, for sure. So I cannot blame my athletic skills, like knitting and photography, for the broken bone. Darn.

My endocrinologist told me on the phone that the broken foot was another good reason to increase my medication. He said it would help speed up the healing process of the fracture.

I was feeling overwhelmed by all of this but decided to make myself feel better with a new pair of shoes. At this particular shoe store, referred by my orthopedist, I found out all about “neutral shoes” and that this type of shoe would be better for a bowlegged person like me. Most shoes, but not neutral shoes, help people to walk or run more on the outside of their feet, according to the salesman. A neutral shoe is more cushioned, too. Lord knows, I need more cushioning for my feet, and I don’t need to walk more on the outside of my feet than I do already! The fracture was on the left side of my left foot, another reason not to wear something that rolls my feet outward.

I have learned more about feet this year than I ever dreamed I would. I learned last year that my sore feet could only tolerate New Balance WIDE shoes, and only the ones made in Indonesia. The NB wide shoes made in China or Vietnam hurt my feet. Weird, huh?

So, my latest pair of shoes, which are “neutral” shoes, are made by Saucony. In addition to being wide enough, they are also the lightest weight shoes I have ever put on my feet! They’re expensive, though, so I hope they last forever. Well, at least as long as my feet last.

The next thing on the horizon is the XLH adult drug study of KRN-23 that may begin in the fall. I will be eligible for that. You can read more about that study at xlhnetwork.org. In the meantime, I will continue on my current meds and hope that I don’t get any more fractures, which we are prone to get!

All in all, it was a good 6-month checkup. It was sort of like life–a mixture of bad news and good news. Even the bad news (the foot fracture) was good in that I was glad to know I’m not crazy and imagining that my foot “ain’t right” as we say in the South. Life is like that sometimes. A mixture of the bad news and good news, rain and sunshine, thorns and blossoms.

Happy Spring everyone!

Copyright S. G. Hunter and Banjogrrldiaries, 2015. All Rights Reserved.

*If you’re a person with XLH reading this blog, please be sure to subscribe to the listserve of the XLH Network (xlhnetwork.org) so that you can be sure you’re being correctly treated for this disorder. There’s no need to make an already complicated disorder more complicated by being improperly treated! The XLH Network is a great resource for both patients and doctors, so take advantage of it. And, if you’re able, please support the network financially. It’s important to have groups like this who advocate for us on our behalf and there are some wonderful people who volunteer their time to do that for us. Many of those volunteers have XLH themselves or family members with XLH and spend time educating medical and pharmaceutical professionals about our rare genetic bone disorder.

Vitamin D

So, back in March, my endocrinologist told me to start taking 2,000 I.U.’s of Vitamin D (That’s 2 pills at 1,000 I.U.’s each) every day. He said that most folks are a little low in Vitamin D these days, because of using sunscreen, staying indoors, etc. and in my case, my body doesn’t metabolize it very well anyway. My blood test results in February said that my Vitamin D level was 9.1 ng/mL. The acceptable normal range for women my age is 30-100 ng/mL. Being a first born, and good at following the directions, being a “good girl” was not going to be a problem. I am pretty sure I didn’t miss a dose in the three months since his instructions. I had a lot of experience in taking Vitamin D pills, anyway, since the standard treatment back in the ‘60’s when I was diagnosed with XLH (called “Vitamin D-resistant rickets back then) was 50,000 I.U.’s of Vitamin D daily. He wanted me to take a mere 2,000 I.U.’s daily? Not a problem! (That doesn’t mean that I had to take 50 pills of 1,000 I.U.’s each, by the way…)

Yesterday, my family doc’s secretary called me to tell me the results of the blood test they did last week. She said the my Vitamin D level increased quite a bit (I could tell she was trying to sound really positive, so I was waiting for the “but…”) BUT it was still only 26 ng/mL. (I knew that “but” was coming!) “Dr. Familydoc wants to see you again in 3 months to recheck your Vitamin D level and also check your parathyroid hormone level.” Wacky parathyroid hormone levels are another challenge with XLH’ers.

Sigh. I had been so obedient, too! I really wanted an “A+” on my Vitamin D report card, but I should have known better. Living with XLH is a life-long commitment and isn’t curable, just manageable usually!

The secretary continued, “I’ll switch you over to Calendarlady and she’ll make an appointment for you.”

So, I spoke to Calendarlady. “Dr. Familydoc wants me to come in to have my Vitamin D level AND my parathyroid hormone level checked in 3 months.”

Calendarlady: “Oh, okay, that puts you in late October, the last week. What’s good for you?”

Me: “Monday, October 22 at 10 AM is good. And remember it’s for Vitamin D AND the parathyroid hormone level and is this also a non-fasting blood test?”

Calendarlady: “Well, honey, I don’t even know what in the world that parathyroid is, but the doctor will make sure that’s what gets tested and sure, it’s non-fasting.”

Sigh. You know, I have this particular mechanic that I like to use when I have car trouble and the calendar lady THERE would NEVER say to me, “Well, honey, I don’t even know what a distributor is, but the mechanic will know what to check.” That kind of response never instills confidence in a client. I wondered later if I should have spelled “parathyroid” for her…

Anyway, I put it on my calendar, and hung up the phone. Professorgrrl looked at me across the breakfast table…I guess I had that “I give up” look in my eyes because she said, “Don’t go there. It’s okay.” She knows that look and I admit, I was feeling defeated. I must be one of those people who occasionally sees the black dot on the white paper before noticing all the white surrounding the little black dot.

Again she said, “Don’t go there…I know you’re frustrated. We’ll get it up to where it needs to be! I’ll google this, we’ll read up on it.” And she did…everything online said that someone with low levels of Vitamin D should return to normal levels within 4-6 weeks after starting a Vitamin D pill regimen. Even Professorgrrl paused a moment when she read that. I had been working on this for 12 weeks.

On her way to work, she called me and said, “I’m going to do a little research and find out what foods are high in Vitamin D.” She loves to cook, and now she was on a mission!

Later she texted me, “Shiitake mushrooms are very high in Vitamin D!”

WHAT?!? Just last weekend we had gone to the local farmer’s market and bought a bag of freshly harvested shiitake mushrooms and later that evening, Professorgrrl made “Shiitake Mushroom Lasagna” inspired by the Barefoot Contessa, who has a cooking show on the food channel. Professorgrrl, though, altered it to be lower in fat and cholesterol, as she does most recipes, because I also inherited a body that makes cholesterol like Krispy Kreme makes donuts. Now, I don’t know what the Barefoot Contessa’s high fat Mushroom Lasagna tastes like, but Professorgrrl’s lower fat version was SO delicious.

Hearing this bit of news about the benefits of eating shiitake mushrooms really made my day, since the memory of that lasagna was still fresh on my palate. I asked her later if we’d go to the farmer’s market again this Saturday for more fresh shiitake mushrooms. “Oh yes,” she said.

So, I’m going to share the recipe with you, Professorgrrl’s version. This is for all of you who need a Vitamin D boost, but not a fat and cholesterol boost!

Ingredients
Sea Salt
Good olive oil
¾ pound dried whole wheat lasagna noodles
2½ cups skim milk plus 1½  cup of fat-free half and half
12 tablespoons (1½ sticks) Smart Balance butter, divided
½ cup all-purpose flour
1 teaspoon freshly ground black pepper
1 teaspoon ground nutmeg
1 ½  pounds Shiitake mushrooms
½ cup freshly ground Parmesan plus ½ cup 2% shredded Mozarella cheese

Directions
Preheat the oven to 375 degrees F.
Bring a large pot of water to a boil with 1 tablespoon salt and a splash of oil. Add the lasagna noodles and cook for 10 minutes, stirring occasionally. Drain and set aside.
For the white sauce, bring the milk/half and half combo to a simmer in a saucepan. Set aside. Melt 8 tablespoons (1 stick) of the butter in a large saucepan. Add the flour and cook for 1 minute over low heat, stirring constantly with a wooden spoon. Pour the hot milk into the butter-flour mixture all at once. Add 1 tablespoon salt, the pepper, and nutmeg, and cook over medium-low heat, stirring first with the wooden spoon and then with a whisk, for 3 to 5 minutes, until thick. Set aside off the heat.
Separate the mushroom stems from the caps and discard the stems. Slice the caps 1/4-inch thick. Heat 2 tablespoons of oil and 2 tablespoons of the butter in a large (12-inch) sauté pan. When the butter melts, add half the mushrooms, sprinkle with salt, and cook over medium heat for about 5 minutes, until the mushrooms are tender and they release some of their juices. If they become too dry, add a little more oil. Toss occasionally to make sure the mushrooms cook evenly. Repeat with the remaining mushrooms and set all the mushrooms aside.
To assemble the lasagna, spread some of the sauce in the bottom of an 8 by 12 by 2-inch baking dish. Arrange a layer of noodles on top, then more sauce, then 1/3 of the mushrooms, and 1/4 cup grated Parmesan/2% shredded Mozarella mixture. Repeat 2 more times, layering noodles, sauce, mushrooms, and Parmesan/Mozarella. Top with a final layer of noodles and sauce, and sprinkle with the remaining Parmesan/Mozarella.
Bake the lasagna for 45 minutes, or until the top is browned the sauce is bubbly and hot. Allow to sit at room temperature for 15 minutes and serve hot.

For the high-fat, artery-clogging version, just Google “Contessa mushroom lasagna.”

If you don’t like to cook, give this recipe to someone who loves you, and have them make it for you! It tastes even better when someone who loves you makes it for you!

One other thing: I would like to know how this whole Vitamin D thing works. Doctors say that we get Vitamin D from the sunshine. Shiitake mushrooms, which are very high in Vitamin D, grow in the SHADE!

Copyright S.G. Hunter and Banjogrrldiaries, 2012-2017

The MRI experience

Last Tuesday evening, I had my first, and I hope, only, MRI. Since this is the third time in the last 20 years that a doctor has recommended one, I figured I should go ahead and get it over with. It seems to be a rite of passage for all aging Americans with health insurance. The endocrinologist that I saw in May “ordered” it for my lumbar spine. He said he wanted to check on those possible calcifications on my spine that many XLH-ers tend to get on their bones. “I have one patient who is bedridden because of his calcifications!” he said. Gee, thanks for making my day…

Several years ago, a friend of mine had to get an MRI and she later warned me… “Banjogrrl, if you ever have to get one of these, be sure to wear earplugs! It was surprisingly loud.” I happened to recall that my friend had told me this, and I remembered to take my earplugs with me last Tuesday. We both work in a music profession that requires that we take very good care of our hearing. I’ve always done that anyway. When I was a teenager, I’d go to a concert and before getting in my seat, I would run to the bathroom and get toilet paper to stuff into my ears. I didn’t like loud music or noise.

I usually get a little anxious…just a little…before going to any medical appointments, but I was feeling pretty much like Jessie from Toy Story II. “No problem. I’ve got this under control!” Confidently optimistic. “Got my earplugs…I’m ready to go!”

I know that in my first-ever post, I said that I needed to get in touch with my inner warrior, but there is also an inner cowgirl in me. Even as a child, I didn’t limit myself! (I wish I still had that holster and gun!)

I sauntered, er, uh, strolled up to the check-in desk when I arrived to the MRI Depot, and, based on the amount of my co-pay, realized that this was not going to be a medical appointment but rather, a medical procedure. Yowza. I handed her my credit card.

Then the receptionist went through all the normal questions such as,

“Do you have a pacemaker?” No.

“Do you have any metal in your body?” No.

“Are you claustrophobic?” Yes.

“Are you pregnant?” Snort. No. That one always gets me. When I set up the appointment, I was asked that too. I guess these days, even an almost 52-year-old woman gets asked this question, since medical science has made it possible for women even older than I am to get pregnant, carry a baby to full term, then retire to rear their children on their social security checks.

Then I went to the back where I had to change clothes and be questioned by the assistant MRI technician. She had even more detailed questions to ask me. She used medical terms I had never even heard of, so I hope I answered them correctly. Then she said, “I guess there’s no chance you’re pregnant, is there?”

What I wanted to say was, “I did not have intimate relations with any man between the time that I arrived at the check-in desk across the hall 10 minutes ago and was asked this same question and this dressing room where we now stand.” But all I said was, “No chance!” She then pointed to the cotton drawstring britches that were custom made for Michael Jordan or any of his fellow basketball players and said, “Put those on and come out when you’re ready!” Awesome.

I carefully walked down the hallway, holding up my very long britches and headed towards the “open” MRI machine. It looked like a giant hamburger press to me. I sat on the table, stuffed in the earplugs (she agreed that some people seem to think that the machine is loud,) and she rolled me in, with that top part hovering very close to the tip of my nose. I was very glad I don’t have a long nose. The MRI assistant tech suggested that I turn my head to the side and look out the window, so I wouldn’t feel claustrophobic. She told me it would take 20 minutes. She left the room.

Then I heard a noise, like an Edgar Allen Poe story noise…a tap-tap-tapping. Silence. Then BAM! I jumped. I am pretty sure they did that on purpose so they could get all my nerves ready. They were now ready. Surely it wouldn’t get any louder than that. Then the tap-tap-tapping. Then BAM-A-LAM-A-LAM. And more BAM-A-LAM-A-LAM plus loud hums, etc. I found it to be…unpleasant. I think that Beaker from the Muppets best conveys my feelings at this point.

Ever-so-slightly horrified. It was VERY loud. I could feel my ribcage vibrating. I tried to decide if it was unpleasantly interesting or interestingly unpleasant. How could something with magnets be so loud? I played with magnets as a kid. It was very quiet play.

I tried to imagine that I was listening to a very loud and very boring punk rock band warm up before a concert. Lots of bass guitar drones (on one note) and lots of drums. LOUD drums. That made me think of Animal, the drummer from the Muppets. Which sent me to my happy place, since I love Animal.

Unfortunately, the “procedure” continued past the limits of my happy place. A few seconds of silence, then the tap-tap-tapping, (which I began to interpret as a warning of things to come) and then the extremely loud, monotoned rock band warm-up session. After 10 or 15 minutes my feet and calves started twitching and I thought, “Wonder what happens when someone goes into an all-out muscle spasm in one of these things? Hope I don’t find out.”

Finally, it was over. I put my better-fitting clothes back on, and walked into the waiting room where Professorgrrl was waiting for me.

“How was it?” she asked. I was still able to hear. That was a good sign.

“Root canal,” I said, “it ranks right up there with root canal in unpleasantness.”

“It hurt?”

“Of course it didn’t hurt! Root canals don’t hurt either if they’ve deadened your mouth enough, but they are highly unpleasant, with all the drill noise in your skull and keeping your mouth open for long periods of time. They are unpleasant. An MRI is unpleasant. And loud. Unbelievably loud.”

I found it ironic that not one single medical person mentioned to me beforehand that I should wear earplugs. I wondered if I should report them to the Audiology Police.

Through all of this, though, I had a song running through my head., which I credit for helping me to keep my sanity. I just recently downloaded the album by Adele called “21.” She is an incredible singer, and her music is so soulful and intense and heart-breaking. I had been listening to her album during the day on Tuesday while I drove out of town for my job. So, one song in particular was stuck in my head. I don’t know how, really, since I can scarcely understand her lyrics, but the intensity, the beat, the emotion, the tune was running through my head while the rock band drums of the MRI were beating in my ears. Adele got me through those 20 minutes of aural unpleasantness. When we got home, I looked up the song, since I didn’t know the name of it (and seriously, could only understand only about half the lyrics) and it’s called, “Set Fire to the Rain.” Hmm. That title is a paradox, really. Sort of like NOT telling your patient who makes her living by using her ears that you’re getting ready to expose them to dangerous levels of noise in a confined space.

I’m just going to step up on my soapbox and say that it is well documented that MRI’s can cause hearing damage. I can’t believe that not one single medical person or technician or ANYONE who was involved with my appointment mentioned to me that I should wear hearing protection, and of a noise reduction rating of at least 29 decibels. In fact, I should not even have to bring my own, but they should provide every single patient with ear protection for their MRI’s. Some places do this. Apparently the place where I went did not. Some websites even said that the noise is also loud enough to cause a person with an anxiety disorder to have an anxiety attack. I had actually thought to myself as I was lying there… “Wow…glad I don’t have an anxiety disorder.”

Okay. Stepping down off my soapbox. Back to Adele and her album, “21. ” It is REALLY good. I like to play it in my car while driving, with the volume turned WAY up.

© S. G. Hunter and Banjogrrldiaries, 2012-2017

An Ode to Bones

I have been at the beach. I love the beach! I enjoy fishing at the beach and I love to look for seashells. When I was a child, I used to get up very early, “at the crack of dawn” so the southern saying goes, and walk along the seashore looking for seashells, which are technically the calcified external skeletons of mollusks. I only wanted to find the perfect ones…sand dollars, olive shells, conch shells, etc. I did not want imperfect specimens!

The beach that we went to last week has mostly broken shells. The perfect ones are difficult to find, especially now that I am older and “getting up early” means after the sun and everyone else have been up awhile. I have to have a cup of coffee before I can think of what it is I want to do next.

Last week, however, was my week for finding BONES that had washed up on the beach. I am fairly certain they are fish bones. (At least, I hope so!) I know that they are not shark bones because sharks don’t have bones. They have skeletons of cartilage, which makes them very flexible creatures; however, they do have calcifications in their vertebrae. I caught two sharks last week. Bones or no bones, they DO have teeth, which makes them challenging to get off the hook! I successfully released them back into the water. The last time I was at this particular beach, I caught a shark that was about 30” long. More cartilage, bigger teeth.

Being an XLH-er, I have a particular interest in bones. My endocrinologist told me in May that he thought that it was ironic that folks with XLH can’t properly make good, hard bone, but our bodies are good (unfortunately) at forming calcifications outside of the joints, like around the knees or in the back, as we get older. I guess our bones are similar to the skeltons of sharks…more like cartilage! Soft…too soft, really. And those aggravating calcifications…

The bones that I found last week at the beach have a beauty of their own. I presume they are fish bones. If they are not, then I may have evidence from a crime scene. These look like part of a vertebrae to me.

Here’s a close up shot where you can actually see the porous texture of the bone.

Unfortunately, in order for me to enjoy the beauty of these ocean-worn boney remains, the fish had to meet their maker.

But be reassured…no fish met its maker while I was fishing last week. In fact, several were fed quite well with the bait that I supplied them!

So, here’s a toast to bones…soft ones, hard ones, normal ones, abnormal ones. Not only are they important to our bodies, the English language also has many idioms using the words “bone” or “bones.” Something can be “bone dry” and you can “work your fingers to the bone.” Ouch! You can have a “bone to pick” or you can “make no bones about it.” Children learn to say at an early age, “Stick and stones may break my bones, but words will never hurt me.” (Which, as adults, we know is not true!) A search of the Bible, including the Old Testament (Hebrew scriptures) and the New Testament, comes up with more than 75 verses that use the word “bones” in either a literal or metaphorical sense. I like Proverbs 14:30, which says, “A heart at peace gives life to the body, but envy rots the bones.” Note to self: don’t be envious!

Bones…take care of them! Reduce your caffeine intake, cut out the carbonated drinks (one website referred to sodas as “Osteoporosis in a Can”) and exercise as much as you are able (and it doesn’t get much better than a walk on the beach.) This advice I am giving to myself as much as anybody…summer is almost here and I do love an occasional cold diet soda. But I am going to try not to be envious or drink any more sodas…I definitely do not want to rot my bones, such as they are!

© S. G. Hunter and Banjogrrldiaries, 2012-2017