Last minute charitable giving? Consider Shriners

For those of you looking for a worthy recipient of a last minute, end of the year charitable contribution, we'd suggest Shriners Hospital for Children. Our family made Shriners the major focus of our charitable giving this year. This blog has cataloged just a few of the numerous stories about the organization and its efforts on behalf of children with limb differences and other related conditions.

You can specify funds for a particular hospital or donate to the charity in general. We directed our support to the Twin Cities facility.

Scottish Rite Hospital patients giving back through race

The Dallas Morning News had a couple articles recently on patients and patient families helping to give back to the hospital that has helped them through the White Rock Marathon. From the December 7, 2008 story Running for a cause at White Rock:

"We knew our baby had hand and feet differences," said Butts, 27, from her home in Midland. "We were scared to death. Scottish Rite made us feel safe."

...Butts knew she could support Scottish Rite by participating in the race after passing the marathon display case on her initial tour of the hospital. During the car ride home, she contacted running friends, who helped her organize a team.

...The Butts' mission now is to do as much for the hospital as they can. "We're committed to giving Will the best we can," she said. "What a relief it was to have a place that is top in the world. Everyone there goes above and beyond with every detail. And, they don't charge a cent. We want to raise money to give back."

Limb Difference Web Round-up: Pistorius Banned; Range of Motion Project

A collection of recent limb difference related items from around the web:

• Oscar Pistorius likely banned from Beijing Olympics
• Chicago Public Radio updates its previous coverage of the Range of Motion Project and its efforts in Guatemala

Limb Difference Web Round-up: Alaska Parity Campaign

A collection of recent limb difference related items from around the web:

• Spina Bifida sufferer and amputee completes the Ironman Triathalon
• ChickSpeak Blog profiles actress and congenital amputee Aimee Mullins
• ACA launches Alaska prosthetic parity legislative campaign

Limb Difference Web Round-up: Staring; Prosthetics in China

A collection of recent limb difference related items from around the web:

• Born Just Right blog explores the "staring" challenge
• Will hosting the Paralympics boost availability of advanced prosthetics in China?
• Choral singer forming limb loss and congential limb difference support group in upstate New York

Limb Difference Web Round-up: Nerve Powered Prosthetics

A collection of recent limb difference related items from around the web:

• Chicago physiatrist advances nerve powered prosthetics
• Health columnist focuses on challenges for the amputee community
• ACA expands its advocacy department

Limb Difference Web Round-up: New ACA Headquarters; i-Limb Hand

A collection of recent limb difference related items from around the web:

• The Amputee Coalition of America celebrates its new headquarters
• Project Valour-IT seeks voice activated laptops for wounded troops
• The Onehandedblogger updates on his i-Limb hand

Limb Difference Web Round-up: Illinois Couple Honored; AP Covers Prosthetic Parity

A collection of recent limb difference related items from around the web:

• Boeing donates to a limb bank in Canada
• The Associated Press covers the federal "prosthetic parity" push
• Bethgo posts her letter to her son's preschool
• An Illinois couple is posthumously honored for their support of Shriners Hospital for Children
• Greg gets a new prosthetic leg approved by insurance

Iowa boy receives prosthetic arms

We first blogged about Aaron's story back in May. KCCI in Des Moines has the latest on Aaron in this story from a couple weeks ago:

A 10-year-old boy who has captured the hearts of Iowans continues working with his new prosthetic arms. Aaron Hynes was born with Holt-Oram syndrome that causes deformities in the upper limbs. Aaron said he loves having the new prosthetics. It's also an adjustment.

Insurance Won't Pay For Breast Birth Defect Surgery

From WSET in Roanoke, Virginia:

If you pay health insurance every month, you expect the coverage to be there when you need it. One Roanoke mother is frustrated because her claim has been denied twice by her provider. Her 14-year old daughter has, what her doctor calls a birth defect. But the insurance company says the procedure the doctor wants to do is cosmetic so they won't cover it.

Dr. Briener - "I can't remember a time when I haven't had a patient covered by insurance for this type of deformity."

Doctor Briener has diagnosed his young female patient with Poland's Syndrome. That's an absence or underdevelopment of the chest muscle on one side of the body...

There is also a video of the news report that you can view on the WSET website.

5-year-old appears in new Tom Hanks movie

From SouthCoastToday.com:

Five-year-old Kenzie Waskiewicz from Dartmouth has what she calls a "little hand" and a "big hand." Kenzie was born without most of her left hand.

She also has luminous eyes, a beautiful face and a fearless personality — all of which earned her a part in the upcoming Tom Hanks and Julia Roberts film.

Due out in December, "Charlie Wilson's War" is about the effects of a Texas Congressman's covert dealings in Afghanistan to assist rebels against the Soviet Union. Kenzie plays a wounded refugee...

...When Kenzie's parents, Eric and Dayna Waskiewicz, found out that their daughter might have an opportunity to shine on the silver screen, they didn't have a portfolio for her and they weren't pushing her towards stardom. Because they are members of the Helping Hands Foundation — a nonprofit for children with "upper limb differences" — they were alerted to a special casting call...

I finished the book about a week ago. As several of the professional critics noted, it's an amazing story that requires you at times to remind yourself that it's non-fiction. For those interested in the book, know that there's some rough language and several adult only stories. But for those whose sensibilities aren't offended, it's a whirlwind account of how an unlikely cadre of individuals set in motion a series of events that for better or worse changed (or at least expedited) the course of history.

Senators introduce CARES Act

Senators Mary Landrieu and Norm Coleman have introduced a Senate version of the CARES Act. A House version was introduced in March of this year.

NY Times: Clearly, Frankly, Unabashedly Disabled

The New York Times had this article last week on how the perception of people with disabilities is changing in the media and in popular culture. From the Times piece:

The public image of people with disabilities has often hinged on the heroic or the tragic. But Mr. (Josh) Blue, 28, represents the broader portrait of disability now infusing television and film. This new, sometimes confrontational stance reflects the higher expectations among many members of the disabled population that they be treated as people who happen to have a disability, rather than as people defined by disability...

...Hollywood’s embrace of a franker depiction of disabilities is mirrored in everyday life in trends such as the jettisoning, by both child and adult amputees, of cosmetic covers for prosthetic legs. Instead, prosthetics experts say, many patients wear their legs openly, often customizing them with designs that are flaunted like tattoos.

(Hat Tip: NerdyMom)

Treatment in Australia

For those seeking consultation and treatment for childhood limb deficiency or loss in Australia, the Royal Children's Hospital -- Melbourne has a Plastic and Maxillofacial Surgery department that offers treatement for a variety of conditions including a Hand Clinic.

Tot's dad designs unique prostheses

From the Atlanta Journal Constitution:

Michael Haag was born with a rare birth defect that left him with only one fully developed hand. But it's not keeping him from doing just about everything other children do.

"Yay, I caught Bobbi," the precocious 3-year-old exclaimed Monday morning, as he used a specially designed prosthetic fishing rod to reel in his favorite stuffed bunny in his family's living room. "She's heavy!"

Thanks to his gadget-minded father, other disabled children one day may have similar experiences.

So far, Robert Haag has crafted two "helping hands," which are child-friendly prostheses that allow his son to have greater use of his left hand — and have a little fun along the way.

Haag recently teamed with a nonprofit group called the Open Prosthetics Group to try to mass-produce his inventions...

...Michael was born with congenital limb loss, a birth defect that each year afflicts about 400 children born in the United States alone, according to Children's Healthcare of Atlanta.

Instead of settling for standard prosthetic options, Robert Haag went to work inventing his own...

He figured out how to disassemble the standard adult-sized prosthetic limbs and reattach kid-friendly things like a dinosaur head, whose mouth opens and closes when Michael moves his arms, allowing him to grasp toys and other items between its "jaws."

Michael's fishing rod is a store-bought Spider-Man reel attached to the end of a prosthetic arm. Michael used it to catch several fish on a recent trip to Florida.

"They were blowfish," he explained Monday, showing off his red and blue fishing pole.

Through Open Prosthetics Group, families like the Haags share information on the Internet in hopes that a manufacturer will step forward.

"If they can take our ideas and use them to help other kids, God bless them," Robert Haag said.

PA legislation seeks better coverage for artificial limbs

From the Pittsburgh Post-Gazette:

Some health insurance plans in Pennsylvania evaluate coverage for prosthetics on a case-by-case basis or limit coverage to as low as $1,000 per year and one prosthetic over a lifetime, even for young children who will outgrow the devices, said the Amputee Coalition of America, which is advocating for better coverage.

State Rep. Bernie O'Neill, R-Bucks, wants to change that.

He has introduced legislation that would prohibit insurance companies from instituting lifetime caps on prosthetic devices. The change would set a uniform standard across the state, Mr. O'Neill said at a recent Capitol press conference

Without legislation ensuring coverage, insurers may reduce or eliminate coverage for prosthetics, he said.

People who are born without limbs or lose them to amputation face many obstacles, he said. "Not being able to pay for limb replacement to return to work or lead productive lives shouldn't be among them," he said.

PBS Kids Video

Came across this PBS Kids video at limbdifferences.org

How Doctors Think

This evening's PBS News Hour featured an interview with Dr. Jerome Groopman about his new book How Doctors Think. The interview gives viewers a fascinating insight into the methodology of how doctors often go about making a diagnosis.

The premise of the book is that, according to Dr. Groopman, physicians are prone to quickly latching onto a specific diagnosis without taking the time (or being allowed to take the time) to consider other factors that could lead to a different conclusion. In the interview, Dr. Groopman details his own experience with misdiagnoses by multiple hand surgeons which would seem mildly humorous if it wasn't at the same time so disconcerting.

I've ordered the book and will post my reaction once I've completed it. The Amazon page dedicated to the book includes a Publisher's Weekly and Washington Post review.

You can download an audio podcast of the interview here. You can also view the video via streaming video on the PBS News Hour website.

Scientific team delivers advanced prosthetic prototype

A couple weeks ago, the Johns Hopkins University Applied Physics Laboratory issued this press release detailing the delivery of the first prosthetic prototype developed by the Revolutionizing Prosthetics 2009 team. The project is underwritten by the Defense Advanced Research Projects Agency (DARPA) Revolutionizing Prosthetics Program with a goal of providing state of the art prosthetics for military personnel who have suffered limb loss in the line of duty. From the APL release:

An international team led by the Johns Hopkins University Applied Physics Laboratory (APL) in Laurel, Md., has developed a prototype of the first fully integrated prosthetic arm that can be controlled naturally, provide sensory feedback and allows for eight degrees of freedom—a level of control far beyond the current state of the art for prosthetic limbs...

... The advanced degree of natural control and integrated sensory feedback demonstrated with Proto 1 are enabled by Targeted Muscle Reinnervation (TMR), a technique pioneered by Dr. Todd Kuiken at RIC that involves the transfer of residual nerves from an amputated limb to unused muscle regions in appropriate proximity to the injury. In this case, the nerves were transferred to the pectoral area of the patient’s chest. This procedure provides for a more intuitive use of a prosthetic arm and allows for the natural sensation of grip strength and touch.

During clinical evaluation of the limb at RIC, Jesse Sullivan, a patient of Dr. Kuiken, demonstrated substantial improvements in functional testing, such as the ability to reposition his thumb for different grips, remove a credit card from a pocket, stack cups while controlling his grip force using sensory feedback verses vision, and to walk using the free swing mode of the limb for a more natural gait. Harshbarger says that critical to Proto 1’s development was closely working with patients such as Sullivan to help the team understand the attributes patients look for in new prosthetic limbs. The limb system also includes a natural-looking artificial covering that was created using photographs of the patient’s native limb taken before the accident.

Military innovations have often found their way into commercial products. This is certainly a worthwhile undertaking on behalf of injured soldiers and I hope whatever breakthroughs come as a result are made available for commercial development so civilians with limb deficiency and loss can benefit as well.

Oregon Health & Science University

For those seeking a treatment option in the Pacific Northwest, the Oregon Health & Science University's Plastic & Reconstructive Surgery Clinic specifically identifies these categories of services related to congenital disorders:

• Pediatric reconstructive surgery for children with cleft lip/palate and other congenital deformities of the head, face, ears, chest and extremities.
• Hand surgery related to congenital disorders, carpal tunnel and traumatic injury

The University's website also provides this overview of congenital hand deformities. I've added OHSU to the list of Hospitals & Treatments Centers listed in the right-hand-side of the site.

Iowa boy's story captures media's attention

The Des Moines Register had this article a couple days ago on Aaron Flat. Aaron is a 10-year-old boy who suffers from Holt-Oram syndrome which has left him "with two holes in his heart, and arms that are just small flaps near his armpits, with three fingers each." As if that wasn't enough, Aaron, his older brother and younger sister (who also suffers from a milder form of the same syndrome) "grew up in a troubled home and was placed in foster care." Aaron and his siblings are now living with their Aunt and Uncle who have begun adoption proceedings.

From the Register article:

"Aaron has moved in with his aunt and uncle in Des Moines, now that there is hope that medical professionals can fit him with prosthetic arms...

The arms will cost $100,000, with lifelong care for follow-ups and adaptations adding another $1 million...

Two months ago, Des Moines prosthetist Jonas Chladek told the family that the latest technology - a prosthetic device that uses the body's muscles to operate arms - would be expensive...

By the end of last week, Chladek finally got a call from Medicaid, the health care insurance for low-income Iowans. Aaron's prosthetics will be covered, and Chladek will donate his services.

The Shriners also are meeting with the family this week to explore their options. And Iowans have begun to donate to a fund set up for his long-term care.

Once he is fitted for the arms, which include a shoulder joint attaching muscles to electrodes that will move his elbow and wrist and rotate his hand, Aaron will be able to pick up items as heavy as a suitcase.

Upcoming ACA Volunteer Night

If you happen to be in the Washington, DC metro area, The Amputee Coalition of America is organizing another volunteer night for Wednesday, May 16 from 6:30 p.m. – 8:30 p.m. ACA is located at 1436 U Street, NW, Suite #104 , between 14th and 15th Street. You can RSVP to Jeremiah Perez at (202) 742-1885 or jperez@amputee-coalition.org

ACA needs volunteers to help with activities in support of prosthetic parity legislation it is currently advocating for in several states.

Specifics on our diagnosis

We received our follow-up letter that details the diagnosis I referenced in a recent post. I'm posting excerpts here verbatim:

The child has a right hand anomaly with transverse deficiency, symbrachydactlyly with a stable thumb and small finger and nubbins on the index, long and ring finger. She has a strong pinch and stable thumb.

CHEST: She has absence of the clavicular head of the pectoralis major muscle, consistent with Poland's syndrome.

She has a full range of motion of the right arm.

I'll be doing additional posts specifically on the symptoms and the condition itself.

Research project underway on orthopedic birth defects

The Washington University in St. Louis School of Medicine has a research project underway with a goal of better understanding the possible genetic links behind certain orthopedic birth defects. The project is being led by Christina Gurnett, an assistant professor of neurology and pediatrics. Gurnett and team will be evaluating children who are being treated for congenital limb differences at St. Louis Children's Hospital's orthopedic clinic.

From the press release:

Gurnett is collaborating with Matthew Dobbs, M.D., associate professor of orthopaedic surgery. Together, they have established a DNA databank of musculoskeletal disorders that includes more than 700 DNA samples of patients with clubfoot; scoliosis; kyphosis, a curving of the spine that causes a bowing of the back, which leads to a hunchback or slouching posture; congenital vertical talus, a common cause of rigid flat foot; triphalangeal thumb, where the hand has another finger in place of a thumb; polydactyly, which causes the hands to have more than five fingers; and patients with other limb abnormalities.

Gurnett's objective is one that will certainly strike a cord with those who have children with limb differences and those who are living with a congenital deficiency.

One large family Gurnett is working with has a history of split hand malformation, a congenital limb malformation characterized by a deep cleft of the hand. Gurnett and her colleagues set up a genetics pedigree chart that shows which family members had the cleft hand.

"The third-generation women in this family are fearful of having children," Gurnett said. "We want to find the gene to help them determine their children's odds of having the disorder so we can find a way to prevent it. We may not find it the next day or the next week, but maybe in five to 10 years we will be able to use our advances in the lab to help patients in the clinic."

Mordecai Brown

A recent comment on this post alerted me to the story of Mordecai Brown. The commenter is the co-author of a book on the turn-of-the-century Chicago Cubs pitcher whose hand was disfigured in a farming accident. The authors also maintain this site dedicated to Brown.

Reviewing the site, you get a sense that Brown was an exceptionally determined individual as well as a great athlete. Teammate and fellow Hall of Famer Johnny Evans has this quote about Brown:

"You haven’t space enough to tell of the good deeds of Brownie on and off the field. Plenty of nerve, ability and willingness to work at all times under any conditions. Crowds never bothered him. There was never a finer character-charitable and friendly to his foes and ever willing to help a youngster breaking in."

The foreword for the book is provided by fellow Cubs pitcher and Hall of Fame member Ferguson Jenkins. An excerpt from that foreword is definitely going to be bulletin board material for our youngest daughter.

"When you start out with a disadvantage, you have to work harder to do what others take for granted In the end, that gives you an advantage."

A bit of Easter Sunday inspiration...

Came across the story of Keiron McCammon who blogs at A One Handed Blog. McCammon lost an arm in a paragliding accident over a year ago and since has been blogging about his experiences. He's recently started receiving some well deserved media coverage. I'd recommend reading this piece on the San Francisco Chronicle website that details McCammon's accident as well as his drive to maintain the momentum behind his startup company. From the Chronicle piece:

Today, McCammon has not only adjusted, he is thriving. He swapped his Jeep Wrangler for a Toyota Prius with the license plate 1 HANDED, commuting 80 miles round-trip from his Danville home in the carpool lane. He types with four fingers on one hand rather than pecking at the keyboard with two fingers on each. He has experimented with prosthetics so he can get back to the activities he enjoys, from practicing yoga to riding a bike to playing the guitar. This winter, he hit the slopes to snowboard again. As an amputee, he gets half-price lift tickets.

McCammon blogs about his experiences at www.onehandedblogger.com. He uses Kaboodle's software to compile resources for others who have lost a hand.

That passion for technology's life-changing potential has convinced McCammon that within five to 10 years, he will have a bionic hand, a transplant or the ability to grow a new one. In the meantime, his wife's love and his ambition for this tiny startup lift his spirits, much in the same way the wind used to propel his glider.

"You have to have something that drives you to get out of bed and get back to life as quickly as possible," McCammon said. "For me, it was Kaboodle."

(Hat Tip: Bethgo)

POSNA summary of congenital upper limb deficiencies

The Pediatric Orthopedic Society of America (POSNA) has this summary of "congenital deficiencies of the upper limb". The discussion section, while a little technical, is nicely condensed and provides a very general overview of conditions, rates of occurrence and treatment approaches. Included is this explanation of the classification system for congenital anomalies:

The classification system developed by Swanson is now internationally accepted, and divides congenital anomalies into seven categories, failure of formation (transverse or longitudinal), failure of differentiation, duplication, overgrowth, undergrowth, constriction bands, and generalized skeletal abnormalities. A number of anomalies of the upper limb are associated with cardiac anomalies, hematologic problems, and VATER syndrome. Poland's syndrome includes hypoplasia of the pectoralis major.

For those with access to (and a desire to review) medical literature, a reference section is included at the bottom of the page. There is also a PDF version of the entire summary.

Philadelphia prosthetist's work highlighted

Jeff Eichhorn, who serves as director of orthotics and prosthetics at the Shriners Hospitals for Children in Philadelphia, was featured in this article in the Connellsville Daily Courier. The article includes a quote from Eichhorn on how cultural differences play into prosthetic treatment.

Eichhorn's treatment can differ depending on whether a child lives in America or abroad. Cultural distinctions matter.

"Here, in the United States, we stress function over cosmetics. In a lot of countries, cosmetics are important," he said. "In Iraq, an arm missing could indicate you are a thief, even though you did nothing illegal. A cosmetic arm is not functional, but being normal in their society is vital."

CARES Act

I was alerted to the introduction of the Children’s Access to Reconstructive Evaluation and Surgery Act -- or CARES Act -- via this American Society of Plastic Surgeons press release. The legislation was introduced by Reps. Carolyn McCarthy (D-NY) and Patrick Tiberi (R-OH). Below is the ASPS release in its entirety. I've bolded the highlights.

ASPS Applauds Introduction of Legislation to Increase Access to Reconstructive Surgery for Children
For Immediate Release: March 23, 2007

ARLINGTON HEIGHTS, Ill.—The American Society of Plastic Surgeons (ASPS) today announced its support of the Children’s Access to Reconstructive Evaluation and Surgery Act—or CARES Act. This bi-partisan legislation was introduced today in the House of Representatives by Reps. Carolyn McCarthy (D-NY) and Patrick Tiberi (R-OH).

In response to an increasing number of insurance companies denying coverage for reconstructive procedures to correct deformities in children, the CARES Act would require insurance companies to provide coverage for the treatment of a child’s congenital or developmental deformity or disorder due to trauma, infection, tumor, or disease. Seven years ago, President George W. Bush signed comparable legislation into law while Governor of Texas.

“The ASPS and Coalition for Coverage of Children’s Deformities believe that a statutory requirement for insurance coverage of children’s deformities is vital to correct the growing problem of insurance companies denying care on the grounds that the care provided is cosmetic in nature,” said Roxanne Guy, MD, ASPS president. “The American Medical Association defines reconstructive surgery as being performed on abnormal structures of the body caused by congenital defects, developmental abnormalities, trauma, infection, tumors or disease. Reconstructive surgery is generally performed to improve function and approximate a normal appearance. The CARES Act identifies the distinction between cosmetic and reconstructive surgery.”

Lauren Mandel, now a teenager, was denied such coverage when she was a little girl. Insurance covered the life threatening procedures for her arterial venus malformation (tumors made of blood vessels) but denied surgery to fix her jaw and teeth, stating it was considered a cosmetic procedure. Lauren finally had the surgery, which was paid for by her parents.

“My daughter deserves that chance as does every child in this country,” said Bernadette LaPoppa, Lauren’s mother. “No family should have to face the obstacles we have faced or experience financial hardship to receive necessary care from the appropriate medical specialist.”

According to the March of Dimes, 3 percent of babies born annually (120,000) suffer from birth defects. A birth defect is an abnormality of structure, function, or body metabolism present at birth that results in physical or mental disabilities or is fatal. Of the 120,000 children born annually with birth defects, approximately 40,000 require reconstructive surgery.

Examples of these deformities include cleft lip, cleft palate, skin lesions, vascular anomalies, malformations of the ear, hand, or foot, and other more profound craniofacial deformities. Although surgeons are able to correct many of these problems, an increasing number of insurance companies are denying access to care by labeling the procedures "cosmetic" or "non-functional" in nature. A survey of ASPS members revealed that nearly 54 percent of respondents indicated they had pediatric patients who have been totally denied insurance coverage, or had experienced significant and deleterious obstacles in obtaining approval for coverage of surgical procedures.

“The CARES Act is common sense legislation that will improve the current delivery system and restore patients’ and families’ trust and confidence in their health plans. Too many American families are being denied access to health care that would prevent long term physical and psychological injuries. The procedures used to treat these children are, by definition, reconstructive, and should be covered by insurance,” said Dr. Guy. “Speaking on behalf of the ASPS membership, we thank the sponsors of this proposed legislation and appreciate their support. As plastic surgeons, we recognize this disparity on a personal level with our young reconstructive patients and their families.”

Shriners sponsors Maryland patient screening

From the Frederick News-Post:

"Volunteering their time to help children with orthopedic or burn problems, 19 employees of the Mid-Maryland Musculoskeletal Institute offered a patient screening clinic Saturday.

Members of the Ali Ghan Shrine in Cumberland, a branch of the Shriners of North America, used the facility on Thomas Johnson Court for five hours to give parents with children needing medical assistance an opportunity to apply for treatment at no cost. No appointments were needed.

Children were accepted with conditions including burns; scoliosis; clubfeet, a common birth defect; leg length discrepancies; limb deficiencies; orthopedic problems associated with cerebral palsy; congenital hip, neuromuscular, hand and back problems; sports injuries; and cleft palette."

6-Year-Old Undergoes Toe to Hand Transfer

From the Baltimore Examiner:

Hector Salas endured nine hours of surgery Tuesday and emerged sporting a new ring finger.

The 6-year-old underwent a procedure that removed a toe from his foot and added a finger to one of his hands, which only had a thumb and “nubbins.”

Complications usually surface within the first five days following surgery, and the first 24 hours hold the most danger, said Dr. James Higgins, a surgeon who performed the operation at Union Memorial Hospital’s Curtis National Hand Center.

Helping Hands Foundation Aids Romanian Boy

The Capitol (Annapolis, Maryland) had an article recently about Paul Lacatos, a 17-month old Romanian boy who suffers from complex syndactyly along with other issues in his legs.

The Helping Hands Foundation helped bring Paul to Annapolis from Romania. The foundation has covered travel and medical costs along with finding a host family for Paul and his mother.

The article itself is comprehensive and thoughtful. It touches on almost every aspect of the story from potential environmental causes of the condition to the emotional bond that Paul's host family developed for him. Most importantly, it demonstrates how the generosity of these individuals has resulted in a tangible benefit for Paul.

New Diagnosis for Poland's Syndrome

On Wednesday we took our six-month-old daughter to see Dr. Steven Moran at Shriners Hospital in Minneapolis. He quickly diagnosed her with Poland's Syndrome. According to this overview by the National Organization for Rare Disorders:

Poland Syndrome is a rare condition that is evident at birth (congenital). Associated features may be extremely variable from case to case. However, it is classically characterized by absence (aplasia) of chest wall muscles on one side of the body (unilateral) and abnormally short, webbed fingers (symbrachydactyly) of the hand on the same side (ipsilateral).

In those with the condition, there is typically unilateral absence of the pectoralis minor and the sternal or breastbone portion of the pectoralis major. The pectoralis minor is a thin, triangular muscle of the upper chest wall; the pectoralis major is a large, fanlike muscle that covers most of the upper, front part of the chest.

Affected individuals may have variable associated features, such as underdevelopment or absence of one nipple (including the darkened area around the nipple [areola]) and/or patchy absence of hair under the arm (axilla). In females, there may be underdevelopment or absence (aplasia) of one breast and underlying (subcutaneous) tissues. In some cases, associated skeletal abnormalities may also be present, such as underdevelopment or absence of upper ribs; elevation of the shoulder blade (Sprengel deformity); and/or shortening of the arm, with underdevelopment of the forearm bones (i.e., ulna and radius).

Our daughter appears to have the classic symptoms of the disorder minus any skeletal abnormalities in her ribs and shoulder blade. I based this last comment on an x-ray she had at three-months-old that didn't appear to show anything abnormal. It is certain she's missing at least one of her pectoral muscles.

There is likely to be surgical procedures down the road that are both functional and cosmetic in nature. I'll cover more on the condition, treatment options, and related topics in subsequent posts.

As for our daughter's hand, Dr. Moran was as optimistic about her function and prognosis as the other specialists, orthapedists, hand surgeons and pediatricians we've met with.

Cleveland Clinic

I came across this overview from the Cleveland Clinic that includes a good straight-forward explanation of the most common congenital limb conditions, treatment options, and information for relevant staff at the Clinic. I dug around in the Cleveland Clinic site to try and find a page dedicated to the Clinic's approach and resources for treating limb differences. The closest I could come up with is the Pediatric Plastic Surgery Section and the Orthopedic Surgery Section.

I haven't read or heard anecdotally about the Clinic's reputation for pediatric plastic or orthopedic surgery, surgical rehabilitation or the specific experience its specialists have with limb differences. While it's always risky to make assumptions about an institutions ability to diagnose and treat rare conditions, given the Clinic's national reputation it's probably fair to assume that if your seeking initial information and treatment options you'll be well served. This is especially true if you need to seek treatment in the Cleveland area and don't have access to other treatment centers in the Midwest or East Coast.

Back to Curtis

Last week we took our daughter back to see Dr. Thomas Graham at Curtis National Hand Center. This was our first real appointment at Curtis. Our first visit was during the monthly clinic the Center offers for those seeking initial consultations for children with congenital hand differences.

As I previously detailed, our daughter has a small thumb and a pinky finger on her right hand. She is missing her index, middle and ring finger. The growth of the hand so far has allowed her to form a very functional pincher grip.

Overall the discussion was as positive as we could've hoped for. Dr. Graham was as optimistic about our daughter's hand function as he and the others physicians were during her initial clinic visit. It was Dr. Graham who first diagnosed her condition as "transverse failure to form." Meaning it is not connected to any other conditions and is very unlikely to have been passed on genetically. In his words, it was a "lighting strike."

The bottom-line from this appointment is that there is really nothing more to do in the short-term other than continue watching our daughter develop. We are seeing one more specialist at Shriners later this month. Provided his guidance doesn't dramatically differ from the 1/2 dozen others doctors we've seen, my wife and I have agreed that we've heard enough to be confident that our daughter is lucky to have the level of function she has and that there are no procedures we need to plan for in the near future.

2007 O&P Extremity Games

From the Extremity Games Press Release:

"The O&P Extremity Games is an extreme amateur sporting competition for individuals, 13 years and older, living with limb loss or limb difference. The event allows participants to demonstrate skill, persistence and passion while competing for cash and other prizes - proving There’s No Replacement for the Competitive Spirit. The inaugural 2006 O&P Extremity Games rocked with over 500 participating athletes, sponsors, spectators and volunteers. That number is expected to triple in 2007. "

The 2007 Extremity Games take place July 19 - 21 at the Orlando Watersports Complex.

Phillies pick up polydactlyly pitcher

From phillyburbs.com:

He looks like a modern-day giant. He's 6 feet 5, wide-bodied and tips the scales at 250-plus. His head is the size of a large pumpkin, his 1970-ish afro is big and his long goatee adds intimidation.

Of course, what this Goliath is known best for is the extra finger on each hand, which you could say makes new Phillies reliever Antonio Alfonseca twice the man as Hall of Fame pitcher Mordecai “Three Finger” Brown.

The Dominican-born right-hander, who also has six toes on each foot, isn't the least bit embarrassed about his rare condition known as polydactyly. His grandfather had it, too, and quite frankly, he's proud of who he is and what he has.

“People want to see it,” Alfonseca said after working out with the Phillies for the first time the other day. “It doesn't bother me. Jesus gave this to me, so what can I do? I gotta take it.”

Studio looking for cast members with "limb differences"

From the Signal (Santa Clarita, CA):

Universal Pictures has an open cattle call for people - NO ACTING EXPERIENCE NECESSARY - who'd like to be in a film with Julia Roberts and Tom Hanks. Yes. THAT Julia and Tom. We ain't foolin'. Call 310-364-3661. The location is at 11009 East Washington Ave., El Cajon, CA 92019. The production office is specifically looking for "Men, Women, and children who have limb differences or Unique Phiscial Characteristics."

ACA Survey of Prosthetic Coverage Restrictions

The Amputee Coalition of America has released a survey that found a wide disparity between what private insurers will cover for prosthetic devices. The ACA is advocating at the state and federal level for legislation that requires all private health insurers to provide coverage for the "fitting, repair or replacement of a prosthetic device and/or component."

Did da Vinci have a hand deformity?

The San Diego Union-Tribune had this article from last week on a California man who believes Leonardo da Vinci had a hand deformity.

(Ben) Sweeney's research convinced him that da Vinci had a congenital defect – a webbed, or syndactyl, hand. The artist's drawings of his own hand, Sweeney contends, seem to depict a webbed third and fourth finger, characteristic of syndactyly, and a pointed little finger. Sweeney also singles out similarly deformed left hands in other da Vinci artworks – including the hand of Jesus Christ in “The Last Supper.”

Sweeney shared his research with Dr. Joseph Upton at Harvard. Upton is one of the country's leading hand specialists.

“What he has shown me is certainly very believable,” Upton said of Sweeney's research.

While no one will ever be able to prove the theory, “you can't rule it out,” Upton said. He noted that the malformation of the ring finger and third finger depicted in da Vinci's art matches several hand casts in his office.

Upton concluded, “If he didn't have the deformity himself, he certainly saw people who did.” He also said the deformed left hand of Christ in “The Last Supper,” thought by some art experts to be a depiction of da Vinci's own hand after he suffered a stroke, is inconsistent with that of a stroke victim.

Sweeney is in the early stages of his research and it will be interesting to see if his findings get any traction with da Vinci experts and other art historians.

Basketball star doesn't sweat extra digits

The Athens Banner Herald has this article on Auburn Tiger basketball player Frank Tolbert. Tolbert is a starting guard for the Tigers and also has polydactyly. Excerpt from the article follows:

Tolbert doesn't set himself apart with his high tempo alone. He also has a small extra finger protruding from the side of both hands, a birth defect called polydactyly. It doesn't give him any edge gripping the ball.

"People come up and ask me, 'Can I move it? Does it affect me?' I always just tell them, no," said Tolbert, whose father and grandmother also have extra digits. "It's just there."

He's not sensitive about the subject, laughing off the occasional heckling from opposing fans. He particularly liked one visiting fan's sign that read: "Hey, Frank. High Six."

"It just makes me laugh," Tolbert said. "I always joke around about my fingers anyway."

Visit to the Geneticist

A couple weeks ago we took our daughter to see a local geneticist at the University of Nebraska Medical Center. While they didn't do blood work during this first appointment, the doctor did examine her hand. As with the majority of doctors we've seen, he did not think it was amniotic band syndrome -- do to tell-tale signs like the formation of finger nails.

His best initial guess was an atypical version of ectrodactyly, which is the medical term for the unfortunately labeled "lobster claw." The timing is ironic given that this condition is in the news courtesy of Bree Walker's appearance on Nip/Tuck.

Based on previous consultations and our own research, both my wife and I are skeptical it's actually ectrodactyly. That condition usually means the third metacarpal bone is missing and the hand forms a deep cleft where that bone should be. While we haven't had a truly conclusive x-ray on our daughter's hand, the hand surgeon we saw in Omaha thought he felt that all the metacarpal were present and during our initial consultation at the Curtis National Hand Center, I recall that team also ruling out ectrodactyly.

I'll be covering genetics in future posts as more tests are done over the next few weeks.

As an aside, in our appointment we were joined by about half-a-dozen medical students. The more the merrier as far as my wife and I were concerned. We know the rarity of this condition dictates that it's not often seen by most physicians, especially when they train and practice in a smaller city. Hopefully the subsequent discussions about my daughter's condition will help familiarize more local physicians with congenital hand deformities and improve their ability to diagnose and counsel other parents who will be faced with this situation in the future.

ACA Legislative Agenda

The Amputee Coalition of America is pushing legislation in 21 states in 2007 that would require insurers to provide coverage that allows policyholders to receive the prosthetic device they need. Here's how ACA frames the issue:

"In return for premiums paid for group health insurance, consumers expect to be covered for catastrophic illness or injury. Sadly, without legislation to ensure coverage, many people living with the loss or absence of a limb are facing discouraging obstacles when trying to obtain prosthetic care. Current changes in insurance plans are having a devastating effect on amputees and their families..."

The Coalition's online advocacy center includes a wealth of information on the issue and this initiative, including the status of legislation or coalition building in each state. The organization is also maintaining a blog specificly focused on its legislative campaigns. In a previous post I highlighted an upcoming volunteer night in DC that will help support the overall legislative effort. For more information on these efforts you can contact the ACA's Advocacy Dept. at (202) 742-1885 or appll@amputee-coalition.org.

ACA Volunteer Night in DC

The Amputee Coalition of America is organizing a "volunteer night" in DC on Wednesday, January 17th. According to the ACA advocacy page, the organization is pushing for prosthetic insurance legislation in 21 states in 2007 and is looking for volunteers to help support that effort. For more information you can contact Jeremiah at (202) 742-1885 or jperez@amputee-coalition.org.