The Social Security Administration established the Compassionate Allowances program in an attempt to expedite cases where individuals have medical conditions that are very severe and would qualify under one of the listings. However, not all diseases are met under a specific listing, but still may be approved under the CAL.
Alpers disease is a progressive neurologic disorder that begins during childhood and is complicated in many instances by serious liver disease. Symptoms include increased muscle tone with exaggerated reflexes (spasticity), seizures, and loss of cognitive ability (dementia). Diagnosis is established by testing for the POLG gene. Symptoms typically occur months before tissue samples show the mitochondrial DNA depletion, so that these depletion studies cannot be used for early diagnosis; about 80 percent of individuals with Alpers’ disease develop symptoms in the first two years of life, and 20 percent develop symptoms between ages 2 and 25. The first symptoms of the disorder are usually nonspecific and may include hypoglycemia secondary to underlying liver disease, failure to thrive, infection-associated encephalopathy, spasticity, myoclonus (involuntary jerking of a muscle or group of muscles), seizures, or liver failure.
Individuals who have been diagnosed with Alpers typically die before they reach the age of 10, usually caused by unrelenting seizures. The course of the disease is usually rapid and eventually the combination of the diseased brain and increasing physical weakness becomes too great to sustain life, and death usually occurs within a year. Parents will be aware of the child’s increasing frailty, and death is usually relatively peaceful and expected when the time comes.
Alpers may be diagnosed under SSA Listings 111.02, 111.06, and 112.02. SSA suggests that there be clinical history and examination that describes the progression of neurological and cognitive decline from the treating primary care physician, neurologist, or psychiatrist, EEG reports, and lab tests consistent pf hepatic failure, to adequately assess.