Discussion Point 2: Burrying your head in the sand?

Discuss the roles that honesty and deception play in this novel. How do the characters lie to themselves? To each other? Is it sometimes better not to know the truth?

The truth behind the disease “Handle with care”

Osteogenesis imperfecta (OI)

Osteogenesis imperfecta (OI) is a genetic disorder characterized by fragile bones that break easily. It is also known as “brittle bone disease.” A person is born with this disorder and is affected throughout his or her life time.

In addition to fractures people with OI often have muscle weakness, hearing loss, fatigue, joint laxity, curved bones, scoliosis, blue sclerae, dentinogenesis imperfecta (brittle teeth), and short stature. Restrictive pulmonary disease occurs in more severely affected people.
OI is caused by an error called a mutation on a gene that affects the body’s production of the collagen found in bones, and other tissues. It is not caused by too little calcium or poor nutrition.
OI is variable with 8 different types described in medical literature.
The types range in severity from a lethal form to a milder form with few visible symptoms.
The specific medical problems a person will encounter will depend on the degree of severity.
A person with mild OI may experience a few fractures while those with the severe forms may have hundreds in a lifetime.
The number of Americans affected with OI is thought to be 25,000-50,000.
The range is so wide because mild OI often goes undiagnosed.

Genetics
The majority of cases are caused by a dominant mutation to type 1 collagen (COL1A1 or COL1A2) genes
Other types are caused by mutations of the cartilage-associated protein (CRTAP) gene or the LEPRE1 gene. This type of mutation is inherited in a recessive manner.
OI occurs with equal frequency among males and females and among all racial and ethnic groups.
Approximately 35% of children with OI are born into a family with no family history of OI. Most often this is due to a new mutation to a gene and not by anything the parents did before or during pregnancy.

Testing and Diagnosis
Diagnosis for OI is primarily based on signs seen in a doctor’s examination. When there is uncertainty about the diagnosis, it is best to consult a physician who is familiar with OI. Genetic testing is available to confirm a diagnosis of OI through collagen or gene analysis—a skin sample or a blood sample are used to study the amount of Type I collagen or to do a DNA analysis.

Types
Since 1979, OI has been classified by type according to a system based on mode of inheritance, clinical picture, and information from x-rays. The characteristic features of OI vary greatly from person to person, even among people with the same type of OI, and even within the same family. Not all characteristics are evident in each person. The OI type descriptions provide general information about how severe the symptoms probably will be. Health issues frequently seen in children and adults who have OI include:

Short stature
Weak tissues, fragile skin, muscle weakness, and loose joints
Bleeding, easy bruising, frequent nosebleeds and in a small number of people heavy bleeding from injuries
Hearing loss may begin in childhood and affects approximately 50% of adults
Breathing problems, higher incidence of asthma plus risk for other lung problems
Curvature of the spine
See Types of OI for a detailed description.

Treatment
Doctors who see children and adults with OI include primary care physicians, orthopedists, endocrinologists, geneticists and physiatrists (rehabilitation specialists). Other specialists such as a neurologist may be needed.

Treatments focuses on minimizing fractures, maximizing mobility, maximizing independent function and general health
Treatments include:
Physical therapy and safe exercise including swimming
Casts, splints or wraps for broken bones
Braces to support legs, ankles, knees and wrists as needed
Orthopedic surgery, often including implanting rods to support the long bones in arms or legs
Medications to strengthen bones
Mobility aids such as canes, walkers, or wheelchairs and other equipment or aids for independence may be needed to compensate for weakness or short stature.
Treatments Being Studied
Medications
Bisphosphonates such as ©Aredia (pamidronate), ©Fosamax (alendronate) or ©Reclast (zoledronic acid)
©Forteo (teriparatide injections) for adults only
Growth Hormone
Increased vitamin D intake
Physical activity
Potential for gene therapy
At this time, there is no cure.

Prognosis
The prognosis for a person with OI varies greatly depending on the number and severity of symptoms.

Life expectancy is not affected in people with mild or moderate symptoms.
Life expectancy may be shortened for those with more severe symptoms.
The most severe forms result in death at birth or during infancy.
Respiratory failure is the most frequent cause of death for people with OI, followed by accidental trauma.

Despite the challenges of managing OI, most adults and children who have OI lead productive and successful lives. They attend school, develop friendships and other relationships, have careers, raise families, participate in sports and other recreational activities and are active members of their communities.

Managing OI
Techniques for safe handling, protective positioning and safe movement are taught to parents
Infancy, early childhood and the pre-teen years are often challenging
Growth and hormonal changes can affect the frequency of fractures
Children and youth learn which activities to avoid and how to practice energy conservation
The number of fractures usually decreases in adulthood
Following a healthy lifestyle including not smoking, and maintaining a healthy weight is beneficial
History of OI in Medical Literature
There is evidence that OI has affected people throughout history. OI has been recognized in an Egyptian mummy dating from 1000 BC. It has also been identified as the medical condition suffered by Ivan the Boneless who lived in 9th century Denmark. Prince Ivan, according to legend, was carried into battle on a shield because he was unable to walk on his soft legs.

Case studies of fragile bones and hearing loss have appeared in the medical literature since the 1600s.The term “osteogenesis imperfecta” was originated by W. Vrolik in 1849, and the condition was loosely divided into “congenita” and “tarda” by E. Looser in 1906. Van der Hoeve in 1918 described the occurrence of fragile bones, in combination with blue sclera and early deafness as a distinct inherited syndrome.

In the 1970s, Dr. David Sillence and his team of researchers in Australia developed the system of categorization using “Types” that is currently in use. His original four classifications (Type I, Type II, Type III and Type IV) combine clinical symptoms with genetic components. This listing is based on the number of people in the study who had similar symptoms. The types do not go from mildest to most severe. This classification system has been generally accepted world wide since 1979 but continues to evolve as new information is discovered. In recent years, evidence from bone biopsies and other research led to the addition of Types V, VI, VII and VII

A bit of non fiction to educate the mind

I love books which is the reason I set up this blog in the first place to be able to share that with others. I am currently on holiday which gives the perfect opportunity to both read and look at books and i have twice found myself lost in a book shop this week.

During these spells of freedom I have found two great finds, or atleast I hope they will be. The first being “Trust No One” by Teresa Cooper. One girl’s harrowing and disturbing tale of the abuse she suffered in care and how she fought back against all odds to survive.  A true story and I am sure will bring me to tears.

The second purchase was maybe partly through recent conversations with a good friend as well as a personal interest in the topic, this being “Three Cups of Tea” written by Greg Mortenson and David Oliver Relin. One man’s mission to promote peace in Pakistan and Afghanistan through building schools.

“Here we drink three cups of tea to do business: the first you are a stranger, the second you become a friend, and the third you join the family, and for our family we are prepared to do anything – even die.”

Haji Ali, Korphe Village Chief, Karakoram Mountains, Pakistan.

I will be sure to update you on my progress with these personal reads.

Discussion Point 1: All about the person

On Page 38. Charlotte says “I would never have wished for an able-bodied child, because that child would have been someone that wasn’t you.”

If with the right partner and in a loving stable relationship does that equipt you to deal with anything? Thoughts please……..

For everyones attention!

“You men out there who think Ms. Picoult is a chick thing need to get with the program. Her books are an everyone thing, and the current offering — about a little girl whose bones are so brittle that they break almost at a puff of wind — is her best since My Sister’s Keeper. It’s a legal/medical thriller, but at bottom it’s a story about the American heart of darkness: a small-town marriage under stress. Picoult writes with unassuming brilliance and never descends into soap opera.”

—Stephen King

“Told through multiple points of view, this suspenseful story explores questions of medical ethics and personal choice, pinpointing the fragile and delicate fault lines that span out from personal tragedy and disability.”

—Kirkus Reviews

Synopsis of “Handle with Care”

When Charlotte and Sean O’Keefe’s daughter, Willow, is born with severe osteogenesis imperfecta, they are devastated – she will suffer hundreds of broken bones as she grows, a lifetime of pain. As the family struggles to make ends meet to cover Willow’s medical expenses, Charlotte thinks she has found an answer. If she files a wrongful birth lawsuit against her ob/gyn for not telling her in advance that her child would be born severely disabled, the monetary payouts might ensure a lifetime of care for Willow. But it means that Charlotte has to get up in a court of law and say in public that she would have terminated the pregnancy if she’d known about the disability in advance – words that her husband can’t abide, that Willow will hear, and that Charlotte cannot reconcile. And the ob/gyn she’s suing isn’t just her physician – it’s her best friend.

Handle With Care explores the knotty tangle of medical ethics and personal morality. When faced with the reality of a fetus who will be disabled, at which point should an OB counsel termination? Should a parent have the right to make that choice? How disabled is TOO disabled? And as a parent, how far would you go to take care of someone you love? Would you alienate the rest of your family? Would you be willing to lie to your friends, to your spouse, to a court? And perhaps most difficult of all – would you admit to yourself that you might not actually be lying?

The first read

Now this is where the story gets muddled from the very first word, it was initially agreed that we all read “Nothing to lose” written by Lee Child, however this got off to a slow start, and when I say slow I mean glaziers have been formed faster. So the book was written off and  Jodi Picoult’s “Handle with care” was started. Watch this space……

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