Along with these general signs and side effects, patients can have trouble healing.Women who are pregnant should be warned about things such as pre-labor rupture of membranes, drop in blood pressure with anesthesia, precipitate birth (very fast, active labor), malposition of bleeding, and more. New mothers with hEDS should pay extra attention to taking care of their babies. Mothers may have trouble taking care of babies because of the risk of dropping a baby due to weak connective tissue in arms and legs, falling, postpartum depression (more than the general population), and healing from the birthing process. Genetics of Hypermobile EDS
While 12 of the 13 subtypes of EDS have genetic variations that can be tested for by genetic testing, there is no known genetic cause of hEDS. Despite the unknown gene, hEDS appears to follow an autosomal dominant pattern of inheritance. This means that in order to be affected, a person needs a mutation in only one copy of the responsible gene in each cell. This mutation can be inherited from a parent or a de novo (new) mutation. A person with hEDS has a 50% chance of passing their mutated gene on to their children.Recently, several labs and research initiatives have been attempting to uncover a potential hEDS gene. In 2018, the Ehlers–Danlos Society began the Hypermobile Ehlers–Danlos Genetic Evaluation (HEDGE) study. The ongoing study has screened over 1,000 people who have been diagnosed with hEDS by the 2017 criteria to evaluate their genome for a common mutation.
If left untreated, severe decrease in cardiac output, vascular collapse, and hypoperfusion of body including the brain results in death. Pathogenesis
The cause is believed to be an autoimmune response against damaged cardiac tissue. This is supported by excellent response to immunosuppressive (steroid) therapy.This condition is a febrile illness caused by immune attack of the pleura and the pericardium. Possible cell mediated immunity led by Helper T-cells and Cytotoxic T-cells is postulated to be important in the pathogenesis of this condition. There is also possibility of anti-cardiac antibodies created idiopathically, or due to concurrent cross-reactivity of the antibodies produced against viral antigens, however the latter assumption is not fool-proof or completely reliable due to conflicting studies. Diagnosis
A chest X-ray might depict pleural effusion, pulmonary infiltration, or pericardial effusion.During medical doctor examination, a pericardial friction rub can be auscultated indicating pericarditis. Auscultation of the lungs can show crackles indicating pulmonary infiltration, and there can be retrosternal/pleuritic chest pain worse on inspiration (breathing in). Patient can also depict sweating (diaphoresis) and agitation or anxiety. Treatment
Colchicine
Colchicine has been used effectively to prevent pericarditis, and inflammation that follows surgery of the pericardium. Although no current drug on the market prevents post-pericardiotomy syndrome, colchicine seems to provide an effective and safe way to treat pericarditis by reducing inflammation. Colchicine is a natural product extracted from plants, and is a secondary metabolite (an organic compound not directly related to growth and development in an organism).Colchicine interferes with the inflammatory process by altering several important steps in the pathway.