The diagnosis is by estimation of the enzyme activity in a suitable sample.On May 17, 2013, the Secretarys Discretionary Advisory Committee on Heritable Diseases in Newborns and Children (DACHDNC) approved a recommendation to the Secretary of Health and Human Services to add Pompe to the Recommended Uniform Screening Panel (RUSP). The HHS secretary must first approve the recommendation before the disease is formally added to the panel. Classification
There are exceptions, but levels of alpha-glucosidase determines the type of GSD II an individual may have. More alpha glucosidase present in the individuals muscles means symptoms occur later in life and progress more slowly. GSD II is broadly divided into two onset forms based on the age symptoms occur.Infantile-onset form is usually diagnosed at 4–8 months; muscles appear normal but are limp and weak preventing the child from lifting their head or rolling over. As the disease progresses, heart muscles thicken and progressively fail. Without treatment, death usually occurs due to heart failure and respiratory weakness.Late or later onset form occurs later than one to two years and progresses more slowly than Infantile-onset form. One of the first symptoms is a progressive decrease in muscle strength starting with the legs and moving to smaller muscles in the trunk and arms, such as the diaphragm and other muscles required for breathing. Respiratory failure is the most common cause of death. Enlargement of the heart muscles and rhythm disturbances are not significant features but do occur in some cases. Treatment
Cardiac and respiratory complications are treated symptomatically.
I can’t go round in a raincoat and fedora looking over my life saying I did it my way — well, for 10 minutes in some American bar over a gin and tonic you might be able to get away with it. But Sid Vicious’s rendition takes in everybody; everybody is messed up like that, everybody is the mad hero of his own drama. It explodes the whole culture this self-presentation can take place in, so it completes the song for me. The 1986 film Sid and Nancy features a scene where Gary Oldman, portraying Vicious, performs his version of "My Way" while filming the songs music video.Viciouss version of this song appears in Martin Scorseses 1990 film GoodFellas, where it plays over the end credits. Margaret Mackie and Jamie Lee Morley
In December 2019 footage of Margaret Mackie, a resident of Northcare Suites Care Home in Edinburgh who suffers from dementia, performing "My Way" with staff member Jamie Lee Morley, went viral after being posted online by Mackies daughter.Morley later arranged to have the song professionally recorded and it was released in January 2020 as a charity single to raise funds for The Alzheimers Society and Dementia UK. The single peaked at number seven in the iTunes top 40 UK Pop Songs live chart and number five in the Amazon best seller chart. Yuzo Kayama
In Japan, Yuzo Kayama who is usually called the Japanese Frank Sinatra, performed My Way in 2008 in English.