Go See the Brooklyn Cyclones and Fall in Love with New York

Just when you think you couldn't love New York City any more than you already do, you go to a Brooklyn Cyclones game on Coney Island. And fall in love all over again.

How could you not?

You're watching great baseball sitting next to the Coney Island Boardwalk overlooking the Atlantic Ocean. That's right - you can see the ocean from your seat in the stands. And because the stadium seats only about 7500 fans, every seat feels like it's right on the field.

There are couples on dates and families with kids and buddies drinking beers, and in the ladies' room line an old lady with teased yellow hair who is sitting in a wheelchair and wearing a Cyclones T shirt tells you the score.

You get to sing "Take Me Out to the Ballgame" at the top of your lungs and are transported back to your childhood.

While standing in line for a Nathan's foot long, you strike up a conversation with a big guy from Africa who's at his first Cyclones game, and when you say it's your first game too, the two of you look at one another and just smile.

There's so much going on - not a second is wasted between plays. Every minute of down time on the field is a chance to shoot more t-shirts out to the crowd, to sing more songs, to watch the cheerleaders dance, to have the kids come down to the field for a pitching contest. This is baseball with A.D.D., and it's fun!

And then there's that great triple in the bottom of the ninth that won it for the home team.

Not to mention the Ocean sunset.

And the post game fireworks.

And the walk along the beach and the boardwalk on the way back to the subway.

And the Wonder Wheel.

The most fun I've ever had at a ball game.

Go.
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This is another post in a special TBTAM series "Shh! Don't Tell the Tourists!"

Congenital Adrenal Hyperplasia - Something to Consider

Adrenal Steroid Pathways (from Wikipedia)

The New York Times this week has a very nice article about Congenital Adrenal Hyperplasia or CAH, as it is called. I thought the article was very well-written, but might be confusing for folks who don't know the basics about CAH, and might lead some women to over-diagnose themselves with what is a rather uncommon condition. So let me see if I can give you the basics and help you put the article in perspective.

Congenital Adrenal Hyperplasia (CAH)

CAH is caused by a genetic enzyme abnormality in the adrenal gland. In women, this can lead to an over-production of testosterone, which in turn can cause irregular menses, acne, hirsutism (excess hair growth) and infertility.

Clinically, CAH is classified in decreasing order of severity as -

• Classical salt-wasting CAH (Early onset): Presenting at birth with varying degrees of genital ambiguity in females (normal genitals in males), severe adrenal insufficiency and life-threatening salt wasting (both males and females). It is treated with lifelong steroids. Females have normal internal genital structures and with treatment can have normal menstrual cycles and normal pregnancies. Hirsutism can be problematic but is usually quite treatable. Surgery is usually done to correct the genital abnormalities.
• Non-classical, virilizing CAH - Enough adrenal steroids are produced to prevent adrenal insufficiency and genitals are normal at birth. Elevated testosterone levels can causes early puberty and in girls, excess hair growth and clitoral enlargement.
• Late-onset, non-classical CAH - Presents in the late teens and 20's with menstrual irregularities, severe acne or hirsutism, and sometimes, infertility. Can also cause early puberty, though this is less common. Can have no symptoms at all.

The condition the NY Times article is addressing is late onset or non-classical CAH. So that's what we'll be talking about in the rest of this post.

Genetics of CAH

The gene affected in CAH is called CYP21A2, and it codes for the enzyme 21-hydroxylase. This enzyme is part of the adrenal production pathway for cortisol, and catalyzes the conversion of 17-hydroxyprogesterone to 11-deoxycortisol. (It's the second vertical green bar on the top in the steroid production pathway up there.) If that enzyme is blocked, levels of 17 hydroxyprogesterone build up, and then steroid production tends to preferentially head down the other path towards testosterone.

Think of it as a construction delay at the Manhattan-bound Lincoln Tunnel, with cars backed up all the way to the Jersey Turnpike. Traffic is so hemmed in that you can't get over to the right lane, and you end up on Rte 3 headed to Secaucus (testosterone) instead of Manhattan (cortisol). Not exactly where you wanted to go, was it?

CYP21A2 is a recessive gene, meaning that an individual usually has to carry two abnormal gene copies to be affected. There are several different known mutations of the gene, some leading to more severe enzyme deficiencies than other, and various combinations of these mutations in an individual can lead to varying degrees of severity of the condition. The correlation between a specific CYP21A2 mutation and clinical presentation is not always predictable, and other genes are thought to influence the phenotypic presentation.

How common is late-onset or non-classical CAH?

The Times article states that CAH is much more common than realized, and not diagnosed or treated as often as it should be.

Dr. New, who has studied the disease among New Yorkers, said she found it in 1 out of 100 people, but more often in certain ethnic groups — 1 in 27 Ashkenazi Jews, for example, and 1 in 40 Hispanics. It is the most common of the autosomal recessive diseases, in which a child inherits two copies of a recessive gene from his parents — a class that includes sickle cell anemia, Tay-Sachs and cystic fibrosis.

Remember that Dr New screens a select population of women and New Yorkers, so those numbers are not necessarily representative of the US population at large. I've been screening for late-onset CAH for over 20 years whenever an adolescent or adult woman presents to me with irregular menses and acne or hair growth, and I've diagnosed it in maybe 2 or 3 patients. It just is not very common. Be careful also not to confuse the incidence of the gene defect with the clinical condition - not all women who have the gene defect have any symptoms.

As an aside, I've probably seen more classic CAH patients in my career than most gynecologists, having done the pelvic exams as part of a long term study of classical CAH patients conducted by Dr New.

Screening for late-onset CAH

The screen for late-onset CAH is a simple blood test for 17 hydroxyprogesterone - that hormone builds up as a result of the mild enzyme block in the adrenals. It should be done whenever a woman presents with menstrual irregularities and signs of excess androgens such as hirsutism or severe acne. (The test has a very low yield in the absence of signs of androgen excess, but may be useful in evaluating infertility if severe menstrual irregularities are present or there is no response to standard treatments.).

Testing for 17 hydroxy-progesterone is best done fasting and in the latter part of the menstrual cycle. If the result is abnormally high, then a confirmatory test is done called an ACTH stimulation test. The patient is given a hormone that stimulates the adrenal gland to make more steroids, leading to more back up behind the enzyme block and a further rise in 17 hydroxy progesterone. (Think of the ACTH stim test as causing rush hour traffic in the analogy I gave above.)

How is CAH Treated?

Treatment of late-onset CAH depends on the desired outcome and severity of symptoms. If the menstrual cycles are fairly regular and hirsute symptoms mild, then no treatment is necessarily needed. Birth control pills are the mainstay of treatment for mild forms of the condition, especially in sexually active women who want to prevent pregnancy. More severe forms will respond to steroids with or without oral contraceptives. Women who want to conceive may be treated with steroids or not depending again on how severe the condition is and how well she responds to standard ovulation induction.

What about Genetic Testing?

Because the CYP21A2 gene is recessive, individuals who carry the gene may not be aware of it. If two carriers have a child, there is a 25% chance they will have a child with the more severe classical form of the disorder.

Which leads of course to the question - who should be screened for the CAH gene defect?

I'd recommend screening if anyone in your immediate family has CAH - you could be a gene carrier. If you are, then your husband can be screened before you get pregnant to determine if you are at risk for having a child with the classical form of CAH. Given the incidence of CAH in Ashkenazi Jews, I suspect at some point we may start offering CAH testing along with Tay Sachs and other genetic prenatal screens. Right now, however, it is not a recommended routine test in this population.

For more information on CAH

• Info from the NIH - good links here
• CAH - A Handbook for Parents : Info from the Texas State DOH
• E Medicine CAH Chapter

The Dinosaur Comes out of Hiding

So to speak.

Our beloved Dinosaur Doc has written a book, and with its publication, comes out of a long anonimity.

Question: What do anonymity and virginity have in common?

Answer: You can only lose them once, so make it count.

I am pleased, proud and thrilled to announce the upcoming release of my first book.

Many will be surprised to find out that this tough, biting and sometimes foul-mouthed family doc is a girl. I myself had thought she was a he, but Lucy set me straight a few years back. (Yes, I am proud to say I was one of the privileged few who has known of her identity for some time now...) Gender mix up seems to be my specialty - I had thought NHS Blog Doctor was a girl, but he set me straight pretty quickly on that count.

I for one can't wait to read the book. You can pre-order it on Amazon.

Congratulations, Dr Hornstein!

Surgexperiences and Bharatanatyam

See some gorgeous photos of  Bharatanatyam, traditional Indian dance, along with a great compilation of posts from the medical blogosphere in this week's Surgeperiences, hosted by Vijay.

This is what I love about the blogosphere - where else can you get such a wonderful mix of medicine and the arts?

Alternative Medicine - It's Not So Funny

This British comedy sketch about an Emergency Room in a Homeopathic Hospital is damed funny. (Make sure to read the signs on the corridor walls.)

But change the patient from a trauma to a cancer patient, and it's a tragedy.

The patient still steadfastly refused all surgery, chemotherapy, and radiation. Against all evidence that the course she had chosen thus far had not resulted in the elimination of her tumor that she expected, she nonetheless insisted on continuing with various alternative medicine treatments. Against all evidence to the contrary, she continued to refuse any form of "conventional medicine." She still believed that her " healer" could save her life, even though she now had a large, bleeding, stinking mass in her breast stuck to her chest wall that had three years ago been a pea-sized cancer that could have easily been excised with a small surgical procedure. She was well on her way to dying in the horrific way that so many women died of this disease 100 years ago. And the cost was more than just the growth of the tumor. The woman had three small children at the time of her diagnosis.

(Hat tip to Dr Val and Orac)

Is This a News Article or an Ad for Estroven?

How to write a bad medical news article -

Lead your article on alternative treatments for menopause with an unsubstantiated anecdote by an anonymous woman who claims Estroven use led to a 20 lb weight loss without changing her eating habits. Show a photo of several brands of "natural" remedies for menopause, none of whose brand name is clearly visible except the Estroven.

Never mention in the rest of the article that there is no good data to support Estroven's efficacy in treating any of the conditions for which it is marketed, and certainly no data that it leads to effortless weight loss.

I'm surprised - the New York Times usually does a much better job than this.

Emergency Contraception Use Does Not Adversely Affect Pregnancy Outcome

Plan B

Pregnancies conceived in a cycle when levonorgesterel emergency contraception (Plan B) was used have no increased risk of adverse outcomes.

In a prospective study, researchers compared the outcomes of over 300 pregnancies in which Plan B was used during the cycle of conception to a similar number of pregnancies in which no exposure to Plan B occurred. Outcomes were the same, with about a 10% miscarriage rate and 1% rate of major malformations in both groups.

The study outcomes are not surprising. Plan B uses levonorgestrel, a progestin component of hormonal contraceptives for many years, long ago shown to be safe if pregnancy occurs despite its use. Despite the fact that plan B uses levonorgestrel doses that are higher than in traditional oral contraceptives, no adverse impact has been reported to date on pregnancies conceived despite Plan B use. Having yet another reassuring study is always good news.

A couple of things to remember when interpreting data from studies exploring the association between exposure to a medication or chemical and pregnancy outcome -

• The background rate of miscarriage traditionally was thought to be about 15%, but we now know can be as high as 50% if one includes very early miscarriages. Once a heartbeat is established on sonogram, that rate drops to 5% or less. So if you are studying pregnancy outcomes, how you diagnose the pregnancy will affect your miscarriage rate - will you use hcg levels or clinical diagnosis? If you enroll women in your study after 6 weeks, your miscarriage rate will be lower since the early miscarriers will not make it to your study.
  
  
• The background rate of birth defects in the general population is 3-4% - that includes major birth defects like spina bifida and minor birth defects like an extra digit.
  
  
• Subject recall of medication or other exposure can be skewed depending on the pregnancy outcome. If things go wrong, we tend to look back and remember everything that occurred, looking for a possible cause. If things went well, we tend to forget little things like that aspirin we took for that one-day headache. But retrospective studies are much much easier to do that prospective studies. So if you are looking for a link between an exposure and a birth defect, the first step is retrospective cohort analyses. If something comes up there, you need to confirm it in a prospective fashion (if you can).

Seattle's Green Bag Campaign

In an attempt to reduce plastic bag waste in Puget Sound, the Seattle City Council has passed an ordinance imposing a 20 cent fee on disposable shopping bags. The fee is optional - you only pay if you decide to use a disposable plastic bag instead of bringing your own reusable shopping bag to the market.

The American Chemical Council, representing plastic manufacturers, has spent almost $200,000 in a signature gathering campaign to force the tax measure onto the August 18 citywide primary ballot.

The Seattle Green Bag Campaign is fighting to get the ordinance passed on the ballot and is looking for donations and publicity to get their message out. I encourage you to spread the word and donate if you can. If you buy your reusable shopping bags at Reusablebags.com between now and August 18, they will donate 1% of their proceeds to the cause.

I think Seattle has the right approach
I've done a bit of my own research and agree with the Seattle City Council that taxing bags is the way to go if they want to reduce disposable bag use. That city's move is just a small part of a national and global trend to reduce plastic bag use and waste.

• Palo Alto, California has banned plastic bags, and will be considering a tax similar to Seattle's later this year. San Jose already has a tax ruling up for city council ruling this summer.

• San Francisco passed a measure in 2007 banning the use of disposable, non-recyclable plastic bags in chains grossing over $2 million yearly. But that move may have been too limited, and appears to have had little overall impact on the problem of plastic bag pollution in San Francisco.

• Ireland passed a 22 Euro cents tax on plastic bags in 2002, and since then use of plastic bags in that country has dropped 90%. (The American Chemical Council disputes this widely touted statistic.)

• China imposed an outright ban on free disposable plastic bags in 2008. That move has saved almost 2 million tons of oil and cut use by 40 million bags in the first year, but has been blamed for the closure of the state's largest plastics manufacturer just about a month into the ban, a time frame that makes me wonder if there was really a cause and effect.

• Ikea started charging for plastic bags in US stores in 2007, a move that led to a 92% reduction in use. (I myself gave in and bought several large Ikea plastic totes last time I shopped at Ikea, and find them to be a wonderful bag for many, many uses.)

• New York City had initially taken a different tactic, requiring large retailers to collect and recycle the plastic bags they give out to shoppers. The Bloomberg administration proposed a tax on plastic bags in January 2009, but has since backed down on the issue.

Speaking as a New Yorker, I can say I had have never recycled plastic bags at the market, and would support a tax on the bags here. When I shop at Costco, a store that does not supply bags, we use old boxes to carry our goods home. At Ikea, I but their reusable tote. But when I hit Gristedes or Food Emporium or even the Farmers Markets, I take the plastic bags they give me without thinking twice. I don't think consumer behavior in this area will change voluntarily unless it is made either too costly or too inconvenient to do otherwise.

The American Chemical Council is aggressively fighting Seattle's and other local measures aimed at reducing plastic bag use. In Seattle, they used out of state paid workers to gather signatures against the measure. In smaller municipalities, their tactic is to threaten lawsuits that cities cannot afford to defend.

It's another case of the big corporation using their financial and political weight to bully local governments. But with the advent of the Internet, individuals now have the resources to counter the corporate giants.

So spread the word. And start using reusable shopping bags whenever you can.
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If you want to cut back on your plastic bag use, you'll need another bag. Here are some resources -

• Reusablebags.com
• Ecobags
• Minimaxi shopper
• Use it Again Sam
• Baggubag
• Envirosax
• Flip and Tumble
• Kim Valee reviews the reusables

Joplin's "Solace" (Mexican Serenade)

Mike Nelson's "Solace"

After I saw the movie The Sting in Bryant Park last week, I couldn't get Scott Joplin's soundtrack music out of my mind. Especially the lovely Mexican Seranade 'Solace' that plays as Redford and Newman lay down with their respective lady loves the night before they pull off the big sting.

Determined to learn the song on the piano, I went in search of the sheet music online. And stumbled across this hauntingly beautiful guitar arrangement by Mike Nelson that beats any piano playing of the song I've heard to date. Certainly better than my amateur piano pickings...

I commented on Mike's You Tube page suggesting he record it. He informed me he already has - I picked it up on ITunes.

Enjoy.

Farmers' Market Foray

I find the challenge for us as a busy family is incorporating local foods into a busy lifestyle. Our neighborhood Farmer's market is only open on Saturdays, and we don't live near enough to Union Square to go there on a regular basis during the week.

And then, of course, there is the fact that Mr TBTAM likes to go to Fairway on the way home from work to see what's there to inspire tonight's dinner. So it was a bit of a tussle between us on Saturday as I convinced him to change it up a bit - see what's at the Farmer's Market on Saturday when we have time to go together, and plan the week's menus around that.

We decided to start small. Really small. With a bunch of lovely garlic ramps and some baby anise from the Union Square market.

Our first dinner tonight with Victor, who is with us for two weeks from Barcelona on an exchange student visit, provided the perfect opportunity to use these ingredients in a meal that would show him some traditional American cuisine - Chicken and potatoes. "Ramped up" a bit, of course.

Ramps n' Taters

Ramps, or wild leeks, are quintessentially American. Appalachian to be more specific. Native to North America and growing wild in the woods, they provide spring sustenance for early Native Americans and mountain folk for generations. Ramps with potatoes and bacon is a traditional Appalachian dish. We cut back significantly on the bacon, but traditional recipes will use up to a pound of it. Add eggs at the end to make it a complete meal.

3 slices Bacon
1 bunch of garlic ramps, thoroughly washed
6 potatoes, washed and cut into bit sized pieces
Salt, pepper and paprika to taste

Wash the ramps well. Cut off the tip of the root and slice into 1/4 inch pieces. Spin or towel dry

Fry the bacon in a skillet till crisp and remove to a paper towel to drain.

Add the potatoes to the bacon fat and cook for 3-4 minutes. Add the ramps and fry till done. Crumble and toss in the previously fried bacon and serve.

Sauteed Chicken with Fennel and Rosemary

This is a modification of a recipe from Food and Wine. I know it is probably a bit more Mediterranean than American in flavor, but we used the Farmer's Market fennel and that made me proud. This dish has a very light and delicious sauce.

2 tablespoons olive oil
4 bunches baby fennel, cut into 1/2-inch slices
2 tbsp chopped fresh rosemary
1/2 teaspoon salt
1/2 cup chicken broth
4 organic chicken legs, split into thighs and drumsticks
1/4 teaspoon fresh-ground black pepper
2 cloves garlic, minced
2 tablespoons chopped fresh parsley

Heat 1 tablespoon of olive oil is a large saute pan over moderately high heat. Add the fennel, 1 tbsp rosemary, and 1/4 teaspoon of the salt. Cook, stirring frequently, until the fennel is golden brown and almost done. Add the broth and bring to a boil. Cover, reduce the heat and simmer until the fennel is tender. Remove the fennel and the cooking liquid from the pan.

Wipe out the pan and heat the remaining 1 tablespoon oil over moderate heat. Season the chicken with the remaining 1/4 teaspoon salt and 1/8 teaspoon of the pepper. Add the chicken to the pan with the remaining 1 tbsp of rosemary and cook until brown, about 5 minutes. Turn and cook until almost done, about 3 minutes longer. Add the garlic; cook, stirring, for 30 seconds. Add the fennel and its cooking liquid and the remaining 1/8 teaspoon pepper. Bring to a simmer. Cover the pan and remove from the heat. Let steam 5 minutes. Serve, scattering parsley atop the plate.