Saturday, January 12, 2013

Book Review: Living with Sickle Cell Disease (UPDATE)

Gate City Woman Beats Medical Odds

Book Title: Living with Sickle Cell Disease: The Struggle to Survive

Author: Judy Gray Johnson with Leroy Williams

Publisher: (order your copy there)

ISBN: 978-1-105-58199-1

Length: 146 pages

Illustrations: black-and-white photos

Date: 2012

Quote: "It...felt as though one hundred serrated steak knives were piercing my body from within."

[UPDATE: The computer shows that readers aren't finding Judy Gray Johnson's comments on this review, which are long enough to be a separate post. That post is here:]

When part of a healthy body is not getting adequate oxygen from the blood because the circulation is blocked for a few minutes, the "pins and needles" pain is brief and bearable. When part of a body is not getting adequate oxygen from the blood because blood cells that should be soft, round, and slippery are becoming hard, sticky, and dead, the pain can be intense. In between pain episodes, the patient is anemic, weak, and chronically tired. This is the sickle cell disease with which Judy Gray Johnson has been living since 1943.

Sickle cell disease is caused by a gene. People who inherit one sickle cell gene and one gene for normal, healthy blood are resistant to malaria, and pass the sickle cell gene on to some of their children. People who inherit two sickle cell genes have the most serious form of sickle cell disease, sickle cell anemia, and usually die young. People who inherit one sickle cell gene and one gene for a different kind of blood abnormality have sickle cell diseases that are more specifically known, in medical literature, by alphanumeric codes. Johnson has the most common of these milder sickle cell diseases.

The sickle cell gene seems to be most common in sub-Saharan Africa but is not race-specific. It has also been traced to South America, the Mediterranean and Middle East, Scandinavia, and India. Judy Gray Johnson traces her sickle cell gene to Cameroon.

Since I've never known anyone with sickle cell disease well, when I saw a brand-new copy of Living with Sickle Cell Disease at the library, I thought "Leave that for someone who has the disease." I was looking for Susan Cain's Quiet, which other people keep checking out, and was about to sit down with a frivolous celebrity biography when a librarian who knows my real-life identity said, "Have you seen that new local book?" and handed this book to me. However, I soon learned that an African-American with sickle cell disease, and an Irish-American with gluten intolerance, have something in common. "I often felt tired, yet...I did not do any work around the house...I was lazy, plain and simple." Clearly this was a story to which I could relate! I read on, and I'm glad I did.

Unfortunately, sickle cell disease is not cured by simply eliminating a food from the diet. It is not cured at all. Those who live with sickle cell disease are considered better off than those who die from sickle cell anemia, but they are always tired, often in pain, in and out of the hospital. Their average life expectancy is still less than fifty years. "Fortunately," Johnson observes, "I have beaten those odds." She further observes that one reason why she's lived so much longer than other patients with the same disease may be that, unlike many "sicklers," she has resisted the temptation to self-medicate with street drugs. So many "sicklers" become addicts that at least one doctor ticked Johnson off by seeming to assume that Johnson must be an addict too.

A minor contributing factor in what is basically a success story is that Johnson grew up in my home town of Gate City, Virginia, which is known (to the extent that it is known) for having a small, poor school that regularly beats the odds and wins state championships of all sorts. She also had the blessing of being mentored by Mary Wolfe Coley, a remarkable local character. Like most people who went to Shoemaker Elementary School between 1975 and 1980, I have only good memories of Mrs. Coley, but I never would have guessed (though Johnson was not the first to tell me) that (a) Mrs. Coley had been the principal of another school, (b) she was born in 1913, or (c) she was officially paid as the assistant librarian. Mrs. Coley was obviously the senior librarian...and she was also modest; a real Southern Lady.

However, only one short chapter in Living with Sickle Cell Disease is about Gate City; like many of us, Johnson left at an early age. The next chapters tell us about her life with this chronic disease. Going to work early, in order to rest from the commute before starting to work. Going to bed immediately on arriving home from work. Asking for a classroom near a bathroom, when she was teaching, because drinking plenty of water helps extend the time between sickle cell "crises" and hospital visits...then letting herself become dehydrated, anyway, because good teachers don't leave children unsupervised while the teachers use the bathroom. Propping her feet up on an empty chair, under the table, during meetings. And having the man who'd promised to cherish her in sickness or in health disappear as soon as he realized the extent to which it was going to be "in sickness," leaving her alone with a toddler to raise.

Nevertheless, she survived, reared a healthy daughter, was eventually able to set aside an early plan to pass for normal by "being a job hopper," and stayed in Fairfax County, Virginia, long enough to become president of the teachers' union. Approaching age seventy, Johnson admits a medical profile that makes her sound "older" than many 69-year-olds, but she's still active...and learning...and maintaining a web site.

Living with Sickle Cell Disease is an eye-opener for those of us who, well, aren't living with it. How do we know the extent to which another individual is disabled by this disease, which certainly is a disability, but interferes with a patient's work to different degrees on different days? We ask. It's worth asking.

What's not to love about this book? Well...when I was the first to "crack" the new library book, the cover and flyleaf literally started to crack away from the printed pages. This valuable little book deserves a second edition with a real library-quality binding.

Who should read Living with Sickle Cell Disease? Anybody who's likely to meet a person who has the disease. That would mean anybody living in North or South America, Africa, the Middle East, Europe, India, or Australia...China seems to have very few cases of sickle cell disease.

Who especially needs to read Living with Sickle Cell Disease? I can think of six categories of readers who need this book:

(1) Anybody in Virginia. Whether we want to claim her as a Gate City Girl, a Blacksburg alumna, a successful teacher in Fairfax, or a former resident of Richmond, all Virginians can be proud of Judy Gray Johnson.

(2) Especially our state legislators, who regularly pass Resolutions of Commendation of persons and organizations. I think this book is a commendable achievement.

(3) Any school or other organization that observes Black History Month and/or Women's History Month.

(4) All teachers.

(5) All single parents.

(6) Anyone who took The Bell Curve and similar stereotypes seriously. Well...if the words "Baltimore's Shameful Mile" recall vivid pictures to your mind, you know where these stereotypes originated in reality. (Foreign readers: where Route 1 runs through Baltimore, Maryland, for more than a mile it's lined with shabby, ugly buildings in front of which one always used to see sick-looking adults sprawling about on steps, in broad daylight.) These people certainly look lazy, stupid, and apathetic. Probably most of them are stoned. After reading Living with Sickle Cell Disease you will have some idea why they are stoned, and how and where some of them might, possibly, begin to help themselves.

Other books about sickle cell disease exist. The ones I've seen were written by and for doctors, but a quick web search reveals that books on the topic have been written for children. Living with Sickle Cell Disease is a book for the general public. It contains more medical details, and more disrespectful references to Michigan, than our contract allows this web site to display, but I wouldn't hesitate to recommend it to middle school students.