Books and Booze

Lady Rample visits Hollywood in her latest adventures. While there, she tries a delightful cocktail called the Southside. Of course, I had to join her.

First, bruise a sprig of fresh mint in the bottom of a cocktail shaker with a muddler. Top it with ice, and then put in 3 oz gin, 1 tsp simple syrup, 1 Tbsp water, and the juice of one lime. Shake until cold and strain into a chilled martini glass. Garnish with more mint.

This drink is barely sweet and dominated by lime and gin with just a hint of mint, a perfect refreshment for a summer evening!

Too Hot to Blog?

Temperatures have soared above 100 degrees Fahrenheit* this week, with even higher heat indices. The only activities that I deem tolerable are binging TV with appropriate AC and hanging out in neck-deep water in the pool.

It’s summer; why are you reading blog posts anyway?

*That’s above 38 degrees Celsius for those in other countries

In Training

Many years ago there was a bored little girl who needed entertainment. Grandma to the rescue! She wore a silver cuff that she slipped off, placed it between her palms, said “shake ‘em up,” and then hid in one hand. The child had to try and guess which hand hid the bracelet.

Sounds like a snooze when I describe it, but the game could keep me giggling for quite a while.

Details from the cuff

A few months ago, my mother announced she had something for me. I had to take it, even if I didn’t want it. I could dispose of it, but she didn’t want to know. It was the bracelet, Shake ‘Em Up.

Trial run to warm up the bracelet

I wore it for the first time today with a rather bohemian dress. Some friends of my mother’s parents went on a vacation to the west and brought her this cuff as a gift. Until my mother handed it to me, I remembered it as copper, probably because it was chronically tarnished.

I wonder if she ever dreamed of that young grandchild wearing that bracelet, anticipating the birth of her great-great-grandchild? That’s what I’m doing. I’m warming up Shake ‘Em Up for the next generation.

Cocktails by the Book

Last week I needed some distraction, a light fiction read. Amazon suggested a new mystery series that I downloaded and dug into. Lady Rample is a vicar’s daughter whose Aunt Butty rescues her from village life and brings her to London. She married an older Lord who has expired, leaving her rich. His distant cousin is the only male heir, and the former Lord left only the entitled country estate that is falling apart to “Bucktooth Binky,” making sure that the London townhouse, Riviera escape, and all fungible cash assets went to his wife. It is 1932 and she has just completed a year of mourning as we begin book 1, Lady Rample Steps Out

She and her gay BFF, Chaz, end up at a secret Jazz club, featuring an Amerian band. Murder ensues, and our heroine solves the case before the police, of course. Throughout the mystery, she imbibes a variety of cocktails, but her favorite is a highball, basically whiskey and ginger ale.

The second installment, Lady Rample Spies a Clue, sends her to a country estate to escape the unseasonable heat in the city. Of course, her eccentric aunt, Chaz, and a variety of other characters familiar from the first book attend. Of course, someone dies (English country manor homes must be the most dangerous places on earth), and Lady Rample solves the crime. In the heat of the summer, they switch drinks in the country, imbibing Aviation Cocktails, something I had never encountered.

The color of the wild, blue yonder…

I googled the drink, and it was as beautiful as described in the book. The link above will take you to the recipe. It presented two challenges, since I always have gin and lemon juice in the house: Liqueur de Violette and Maraschino Liqueur. Lucky for me I had to stop and pick up some stuff at shoe repair, right next to my liquor store*. They had both in stock!

After dining and finishing my work-related reading, I got out the shaker and made a batch of this lovely stuff. Blue is such an underused color in food, don’t you think?

The liqueurs are both sweet, but the gin and lemon keeps the cocktail on the correct side of candy. You still feel like you’re drinking dessert, but not in a bad way.

These mysteries are fun diversions. Aunt Butty is like a British Auntie Mame, and Ophelia Rample is a 30-something widow enjoying a well-funded life with few restrictions, thanks to a title and enough money to shut people up. Love interests hang about, but she really doesn’t want to give up any freedoms to be a “Proper Wife.”

I look forward to sprinting through the next installment. If I find a new cocktail as well, what a bonus!

*Yes, I found a liquor store and shoe repair center next to each other. Fate is kind, huh?

Stop Lauding Jerks

Last night I went to a dinner with a distinguished speaker, an emeritus professor, who began by talking about past mentors in his field. One of these leaders is memorialized by a statue of him at a chalkboard because he was such an “amazing teacher.” The floor of the statue around him is littered with dimes.

Why? Because when a student, resident, or other trainee answered a question wrong, he would hand them a dime and tell them to call their mother because she was the only one who would love them now.

What an asshole! Instead of being a real teacher who allows trainees to make mistakes and learn from them, he humiliated them in public. And someone felt a statue to lionize this behavior was in order?

Too many of academic medicine’s “elder statesmen” laud the “tough love” of the good old days. Just because some of us survived and thrived under such conditions does not mean they were good for us. I wonder about learners who looked to other fields of work because they were too stupid to follow in this “great man’s footsteps.”

Teachers should have respect for their learners. Public denigration is never the answer. You can correct an answer without humiliation.

Frenzied Female Parents

Back in 1987, the year I gave birth to my firstborn, Diane Keaton starred in Baby Boom, my first recollection of the Frenzied Female archetype. Her character, a driven yuppie known as the Tiger Lady, inherits a girl from distant relatives. This hyper successful businesswoman proves incapable of hiring a competent nanny. Ultimately, she ends up moving to a farm in the country. After hilarious escapades, she starts a successful business and falls in love with the local veterinarian and her “less complicated” life with child.

Yeah, right. Now I must admit children do strange things to you and your ambitions. Priorities can get reordered in a hurry when that bundle of joy (and excrement) lands in your arms. However, no one’s level of competence should be as compromised as the character Keaton portrayed.

There are other examples. In 2002 Allison Pearson brought us the life and times of a British hedge fund manager, Kate Reddy, who also seemed unable to hire adequate help with two young children at home. Of course, the opening scene of Kate “distressing” store-bought pies to make them seem homemade was hysterical. Her professional life remains more intact than the boss in Baby Boom, but she still seems to be one misstep away from getting sacked all the time. Really? A woman who can manage that many currencies across multiple time zones can’t keep her calendar in check? Of course, she does make a career change by the end of the story. We wouldn’t want her to succeed at all aspects of her life, would we?

I just finished a nice debut novel, The Queen of Hearts, by Kimmery Martin. This time the Frenzied Female is Zadie, a pediatric cardiologist who has managed to not only become a pediatric sub-specialist but also has 4 children with her investment banker husband. Her life seems to always be on the edge of chaos, with a delicate time balance of carpools and events, yet she always has time to drop everything for her BFF from college and medical school. Her spouse is constantly jetting off to some foreign meeting, leaving her to juggle their home life. Once again, they have not hired adequate help for the lives they live. I have some bones to pick with some of the medical stuff in the novel, but in general, it was a fun read that kept my tablet in my hand. It was also nice that Zadie’s career does not suffer through the novel as in the earlier examples.

I’m frustrated that we see the same Frenzied Female.  In 30 years, we still laugh at that poor woman who hasn’t figured out that she cannot manage it all.

Over the years, the Manic Pixie Dream Girl has become a common trope in books and media, despite no evidence that such creatures exist. Most MPDGs arise from the male imagination, while women writers were involved in all of the FFP examples above. There is a bit more truth to this archetype, but I do wish it would evolve. Could we admit that we have someone (looking at you Amazon) deliver stuff to our home, rather than making grocery runs all the time? Or hired a nanny who can actually do the job of hauling children around town?

The life of the FFP can be chaotic, madcap, and funny, but women do not lose their ability to organize and function while they raise children. All of these are women with money and resources, those best able to take on the child raising role. If we can’t picture these educated, well-paid women doing these tasks, what hope do working-class families have?

A Million Tiny Wounds

Everyone in the medical community seems to be concerned about physician burn-out and suicide. All the solutions I see seem to add to the problem:

  • More training modules!
  • Meditation!
  • Work-life balance tools!

Shall I go on? I believe part of the problem is we have so many boxes to tick off:

  • Value!
  • Patient satisfaction!
  • Maintenance of Certification!
  • Quality Improvement!
  • Meaningful Use!
  • Complete Billing!

We are not being killed by big changes in medicine; we are dying by a million tiny wounds, each inflicted with good intentions, but altogether just as fatal as a single stroke with a machete.

 

An Approach to Impostor Syndrome

xkcd for the win!

We have all had that self-doubt at times, wondering if we were really qualified to do what we do. In some people it becomes debilitating, preventing them from pursuing opportunities.

Most of us suck it up and struggle through the self-doubt, usually with some version of “fake it till you make it.”

I heard a new approach yesterday in an interview with Todd Herman, an internationally recognized coach. He suggests having an alter-ego who has qualities that you lack. For example, he is often indecisive, but his business alter-ego has no such problem. He assumes his other identity by putting on glasses (which he does not need).

This reminds me of when my daughter had to do unpleasant tasks, like cold-calling for donations for fundraisers. She has a theater background, and she became “Fundraiser Jenny.” People weren’t rejecting Jen; they were saying no to the other character.

I’m going to try this for some upcoming situations and see how it works. How about it? Do you ever assume a secret identity?

Free to Pee etc.

An Illinois Public Radio story caught my eye on Twitter this week. It dealt with a major player in Chicago charter schools and their disciplinary policies. Limitations on bathroom usage meant many young women were bleeding through the mandatory khaki pants during their periods, a less than ideal situation for all involved:

“We have (bathroom) escorts, and they rarely come so we end up walking out (of class) and that gets us in trouble,” she texted. “But who wants to walk around knowing there’s blood on them? It can still stain the seats. They just need to be more understanding.”

They go on to defend the policy, noting that girls who bleed through their pants can tie a sweater around their waist to cover up the damage. Of course, since this is not a usually acceptable part of the dress code, they then announce the names of the girls who are allowed to wear this aberration.

Yeah, please announce to the world the name of the menstruating students. Nothing about that will make them feel self-conscious or awkward.

I haven’t worried about bleeding through for a while now, but I do worry about bathroom access in schools. As a pediatric nephrologist, I take care of a lot of children who would benefit from easier access to the restroom.

  • First up are those children with frequent urinary tract infections (UTIs). One of our defenses against UTI is completely emptying our bladders on a regular basis. This action flushes out any bacteria that have made their way into the interior space. In addition to that, holding urine can cause the bladder to lose efficient function. Children may not be able to empty completely, meaning bacteria are more likely to get a foothold in the bladder and cause trouble. 
  • Second, we must consider children with constipation. A large wad of poop can put pressure on the bladder, its outlet, and its nerves, preventing proper sensation and function. These children must be cleaned out with aggressive stool softening. How inconvenient if the bathroom escort is not available when the poop is ready to pop! Holding it in not only makes constipation worse but further worsens bladder function and makes UTI likely. Adequate fluid intake can also prevent constipation. 
  • Third, a lot of children get kidney stones. Some of these kids have biochemical problems that can be treated, but even those stone-formers could likely prevent such things if they drank enough water. For adults, we recommend enough water to produce 2 liters (66 oz) of urine daily. This means drinking 2-2.5 liters of fluid. At least part of this should be consumed during the school day, necessitating bathroom use. Kidney stones produce debilitating pain, and in the long-run can lead to permanent kidney damage. 

Other considerations include keeping bathrooms clean and functional and safe.

I would like to declare that all people, even students, have the right to use the bathroom when necessary. Not only is holding pee and poop in harmful, but I cannot imagine being able to learn when I’m worried about losing control or bleeding through my clothing.

Join me in showing support for the right to hygienic elimination! You can buy a “Let Kids Pee” ceramic cup or stainless steel travel mug on Amazon (my design is featured above in this post). You will help support this website and the battle we pediatric nephrologists fight on this front.

 

Sickling Hemoglobinopathy and the Kidney #ExpBio

HbSS from Wikipedia

This year a number of abstracts about sickle cell and the kidney caught my attention, having just initiated dialysis on a patient with HbSS. Since the science forces of the universe seemed determined to focus my attention on this disorder, I gave into their wishes.

Hemoglobin (hb), the molecule that carries oxygen to tissues in our body, is composed of two alpha protein chains and two beta chains. In the HbS mutation, a single change in the beta chain changes the structure of the protein so that when oxygen levels drop, it becomes straighter instead of round, stretching red blood cells into a crescent or sickle shape. Abnormally shaped red blood cells are prone to damage (hemolysis) and may clog the smallest vessels in the body, resulting in organ damage and pain.

Actual Sickle

If a child gets one copy of the HbS gene, then they are a carrier; this condition is also called sickle cell trait and occurs in 1 in 13 African American babies. Most people with trait have no symptoms, although under conditions of low oxygen their disorder may be unmasked. For example, the central part of the kidney (the medulla) has much lower oxygen tension normally. Cells often sickle there and cause damage, so individuals with otherwise asymptomatic sickle trait may not be able to fully concentrate their urine.

If a child gets a copy of HbS from both parents, then they have HbSS, the full-blown sickle cell anemia disease. This happens to about 1 in 365 African American children. These individuals have anemia because the sickling cells don’t last as long as those with normal Hb (3 weeks vs 3 months). The abnormally shaped cells may also impair blood flow to organs cause acute pain episodes, what most people think about with sickle cell disease. In addition, other organs may be damaged by these events, including the brain, heart, lungs, and kidneys.

For more general information about sickle cell disease, including other associated hemoglobin disorders, the NIH has an excellent resource here.

Kasztan M, Fox BM, Speed JS, Townes TM, and Pollock DM:  KIM-1 as a new biomarker for glomerular hyperfiltration and chronic kidney disease in humanized sickle cell disease mice

The first poster on my list dealt with KIM-1 (Kidney Injury Molecule 1) as a new biomarker for kidney damage in a humanized mouse model.They followed glomerular filtration rate (GFR) and urinary biomarkers in HbSS mice and genetic controls every 4 weeks for 24 weeks starting at 8 weeks of age. At that starting point, no differences in GFR or proteinuria were demonstrated. By 12 weeks, the HbSS mice had a significant rise in GFR and proteinuria. By 32 weeks of age, GFR was lower in HbSS than Hb AA mice, even as proteinuria climbed higher. Urine biomarkers demonstrated early KIM-1 as a potential predictor of loss of GFR later in the course. Not included in the abstract was a cohort of patients with HbSS, some of whom have developed elevated KIM-1 excretion. This has the potential to drive important translational studies in the future.

Taylor CM, Kasztan M, Yoder B, Pollock JS, and Pollock D:  Reduced Renal Primary Cilia Expression in Humanized Sickle Cell Mice

The next poster looked at renal cilia in this same mouse model. Ciliary disorders often result in cysts in the kidneys, and patients with HbSS have an increased risk of cyst formation. Kidneys from HbSS mice have reduced ciliary proteins in their kidneys, suggesting reduced numbers or size of cilia in this model. Additional studies applied hypoxic stress to the mice, a maneuver that increased expression of the proteins under study. These experiments are still preliminary, but I would never have guessed that cilia would be a problem in sickle cell kidneys.

Eshback ML, Kaur A, Rbaibi Y, Agarwal Y, Zhang Q, Nolin TD, Tejero J, and Weisz OA:  Hemoglobin Inhibits Uptake of Filtered Proteins by Proximal Tubule Cells: Implications for Sickle Cell Disease and Vitamin D Status

The final poster examined the role of Hb uptake in tubular cells in nickels cell disease. Much of this study occurred in vitro, examining what happened to tubular transport when free Hb gets filtered and the tubules reabsorb it. It competes for transport with a number of other proteins, such as albumin and the vitamin D binding protein. Patients with HbSS have reduced red blood cell lifespan and episodic hemolysis, so they do filter higher amounts of free Hb. They also seem to be much harder to make replete with Vitamin D. These basic science observations may help explain some interesting clinical observations, even though these phenomena would seem unrelated at first glance.

Sometimes an abstract just grabs me; this time, three of them jumped me and made me write about them. All were really good science with excellent presenters, and a lot more information than I’ve included here (click the links, people). Best of luck helping us understand this difficult consequence of a fairly common disorder.