tag:blogger.com,1999:blog-7423880838207203660.post2958801786483647502..comments2024-01-10T09:56:49.324-05:00Comments on Respiratory Therapy Cave: What is genetic emphysema?Rick Freahttp://www.blogger.com/profile/01132949384071592216noreply@blogger.comBlogger2125tag:blogger.com,1999:blog-7423880838207203660.post-67684759381351255462012-05-25T21:25:02.207-04:002012-05-25T21:25:02.207-04:00Those with genetic emphysema can get alpha 1 antit...Those with genetic emphysema can get alpha 1 antitrypson infusions. Here is an extract from the American College of Chest Physician's journal:<br /><br />"Based on these data, it has become the standard of care to treat individuals with lung disease related to AATD with augmentation therapy. The usual dose is 60 mg/kg given weekly by IV infusion over 30 to 90 min. Side effects are rare but those reported include fever, leukocytosis, transient flu-like symptoms, asthenia, injection site pain, headache, dizziness, rash, transient hypertension and tachycardia, chest pain, increased cough, dyspnea, paresthesia, and pruritis, all in <1% of patients (based on product literature and prescribing information from augmentation therapies available in the United States)."<br />http://www.chestnet.org/accp/pccsu/%CE%B11-antitrypsin-augmentation-approaches-and-benefits?page=0,3<br /><br />It is expensive though. It also says:<br />"Virtually all of the circulating AAT protein is synthesized in liver hepatocytes... Liver transplantation “cures” AATD because the transplanted liver will produce and secrete normal levels of the protein, but it does not correct the abnormal gene in germ cells or other organs."<br />I read that 10-15% of all liver transplants are due to this antitrypson deficiency, which also often ruins the liver.Anonymoushttps://www.blogger.com/profile/10144285227118921415noreply@blogger.comtag:blogger.com,1999:blog-7423880838207203660.post-786610486490729002012-05-25T21:11:35.283-04:002012-05-25T21:11:35.283-04:00Those with genetic emphysema can get alpha 1 antit...Those with genetic emphysema can get alpha 1 antitrypson infusions, especially at an early age. Here's an extract from the American College of Chest Physician's journal:<br /><br />"Based on these data, it has become the standard of care to treat individuals with lung disease related to AATD with augmentation therapy. The usual dose is 60 mg/kg given weekly by IV infusion over 30 to 90 min. Side effects are rare but those reported include fever, leukocytosis, transient flu-like symptoms, asthenia, injection site pain, headache, dizziness, rash, transient hypertension and tachycardia, chest pain, increased cough, dyspnea, paresthesia, and pruritis, all in <1% of patients (based on product literature and prescribing information from augmentation therapies available in the United States)."<br />http://www.chestnet.org/accp/pccsu/%CE%B11-antitrypsin-augmentation-approaches-and-benefits?page=0,3<br /><br />It is costly though. Treatments currently being studied include recombinant and inhaled forms. Since alpha antitrypson is made in the liver, a liver transplant cures the deficiency.Anonymoushttps://www.blogger.com/profile/10144285227118921415noreply@blogger.com