Affected individuals inherit one copy of the gene that is not functioning properly from each parent. Each parent is a called a carrier and has one copy of the gene that is functioning properly and one that is not. Siblings of individuals with Farber disease have a 25% chance to also have Farber disease, a 50% chance to be a carrier like the parents, and a 25% chance of being unaffected and not a carrier.The ASAH1 gene is also known to cause a condition called spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME). Farber disease and SMA-PME have been classified as separate disorders, however more recent case reports have identified some individuals with overlapping symptoms of both disorders. Some individuals with SMA symptoms without seizures have also been described. Diagnosis
Disease onset is typically in early infancy but may occur later in life. Children who have the classic form of Farber disease develop symptoms within the first few weeks to months of life. Individuals with moderate or attenuated forms may develop symptoms at any time in childhood. Sometimes it is difficult to diagnose Farber disease because the symptoms can be misdiagnosed as Juvenile Idiopathic Arthritis (JIA). Diagnosis is confirmed by molecular genetic testing of the ASAH1 gene or by measuring acid ceramidase enzyme activity. Treatment
There is no disease specific treatment for Farber disease. Anti-inflammatory medications, specifically tocilizumab (an interleukin-6 receptor inhibitor), has been shown to improve inflammation and pain in some patients.
In October 2020, the FDA updated its safety concerns about CBD, acknowledging the unknown effects of protracted use, how it affects the developing brain, fetus or infants during breastfeeding, whether it interacts with dietary supplements or prescription drugs, whether male fertility is affected, and its possible side effects, such as drowsiness. Mislabeling and poisoning
Studies conducted by the FDA from 2014 through 2019 have determined that a majority of CBD products are not accurately labeled with the amount of CBD they contain. For example, a 2017 analysis of cannabidiol content in oil, tincture, or liquid vape products purchased online in the United States showed that 69% were mislabeled, with 43% having higher and 26% having lower content than stated on product labels.In 2020, the FDA conducted a study of 147 CBD products and found that half contained THC.As of September 2019, 1,085 people contacted US poison control centers about CBD-induced illnesses, doubling the number of cases over the 2018 rate and increasing by 9 times the case numbers of 2017. Of cases reported in 2019, more than 33% received medical attention and 46 people were admitted to a hospital intensive care unit, possibly due to exposure to other products, or drug interactions with CBD. 2018 Farm Bill and hemp
The 2014 Farm Bill legalized the sale of "non-viable hemp material" grown within states participating in the Hemp Pilot Program which defined hemp as cannabis containing less than 0.3% of THC.