Yesterday, Oprah aired the second part of her interview with author James Frey. As you may recall, Frey came to his celebrity when he published a best selling book, A Million Little Pieces, which was presented as a memoir of his experiences during his treatment for alcohol and drug addiction at a rehabilitation center in Minnesota. The book came to the attention of Oprah Winfrey- who was so blown away by it that she made it one of her Oprah Book Club selections, thereby skyrocketing sales even further. It was later revealed that Frey fabricated large parts of his memoirs, including details about his criminal record. Oprah invited Frey back on her show for what turned out to be a confrontation about his lying to her about the book. As part of her final season, Oprah once again sat down with Frey to discuss the whole controversy and how they have finally made peace with each other.
One of the most poignant parts of the two-day series was when Frey opened up to Oprah about the death of his son, Leo, at eleven days of age. Leo died of a neuromuscular disease called Spinal Muscular Atrophy (SMA). SMA is a disease where nerve cells that control muscles (“motor neurons”) are damaged.
Spinal Muscular Atrophy (SMA) belong to a group of hereditary diseases that cause weakness and wasting of the voluntary muscles in the arms and legs of infants and children. The disorders are caused by an abnormal or missing gene known as the survival motor neuron gene (SMN1), which is responsible for the production of a protein essential to motor neurons. Without this protein, lower motor neurons in the spinal cord degenerate and die.
According to Families with Spinal Muscular Atrophy (FSMA):
SMA is one of the most prevalent genetic disorders.
One in every 6,000 babies is born with SMA.
SMA can strike anyone of any age, race or gender.
One in every 40 people carries the gene that causes SMA. The child of two carriers has a one in four chance of developing SMA.
7.5 million Americans are carriers.
There are 3 types of SMA (I, II, or III), which are determined by the age of onset and the severity of symptoms. Type I (also known as Werdnig-Hoffman disease, or infantile-onset SMA) is evident at birth or within the first few months. Symptoms include floppy limbs and trunk, feeble movements of the arms and legs, swallowing difficulties, a weak sucking reflex, and impaired breathing.
Type II (also known as juvenile SMA, intermediate SMA, or chronic SMA), has an onset between 6 and 18 months. Legs tend to be more impaired than arms. Children with Type II are usually able to sit without support if placed in position. Some may be able to stand or walk with help.
Type III (also called Wolhlfart-Kugelberg-Welander disease, or mild SMA) can begin as early as the toddler years or as late as adolescence. Children can stand alone and walk, but may have difficulty getting up from a sitting position. It has been postulated that physicist Stephen Hawking suffers from this form of the disease.
Is there any treatment?
There is no cure for SMA. Treatment consists of managing the symptoms and preventing complications.
What is the prognosis?
The prognosis is poor for babies with SMA Type I. Most die within the first two years. For children with SMA Type II, the prognosis for life expectancy or for independent standing or walking roughly correlates with how old they are when they first begin to experience symptoms – older children tend to have less severe symptoms Children with onset after 18 months are often able to walk and are fully functional for years before they need assistance. They may have a normal life expectancy. Mental functioning in all groups remains normal.
For more information about SMA- click here to go to the Resounding Health Casebook on the topic.