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A malfunctioning immune system could imply anything from a death sentence (inherited immune system disorders) to lifelong malaise (allergies, autoimmunities and chronic inflammatory disorders).In recent decades, scientists have made giant strides in treating inherited immune system disorders.In 1971, David Vetter - Wikipedia was the second child born to a Houston couple. His older brother suffered repeated infections and died a few months after birth, shortly after he was diagnosed as having SCID, Severe combined immunodeficiency - Wikipedia.Back then a child born with such a severe immunodeficiency lived an unimaginably difficult life and faced imminent death. Limited treatment options were merely palliative and only extreme physical cocooning minimized risk of catching infections. Also a SCID, David was placed into a sterile plastic isolator as soon as he was delivered by C-section, and lived in permanent isolation the rest of his brief and unbelievably surreal life.By the time he died at the age of 12 in 1984, David spent his entire life inside a plastic, germ-free chamber, never having physically touched anyone nor being touched, becoming famous as the original Bubble Boy (see below from 1). According to his mother, Carol Ann Demaret, one of David's dreams had been to 'walk barefoot in the grass' (2). Obviously, he never did.Contrast with today when highly effective and even curative treatments such as bone marrow and hematopoietic cell transplants have transformed the lives of those born with an incurable and serious immunodeficiency such as SCID. At this point, >500 transplant centers around the world have carried out >1 million HCTs for a variety of such immune system disorders.At the same time IVIG or Immunoglobulin therapy - Wikipedia has emerged as standard therapy for treating B cell and antibody deficiencies while gene therapy is making a comeback as a viable treatment option for many inherited immune system disorders.Meantime scientists continue to identify new genes involved in PIDs. At the last count >120 genes and >150 PIDs (3).A remarkable pace of progress considering even something as simple as the diagnosis of primary immunodeficiencies (PIDs) wasn't simple and most doctors weren't even adequately trained to do so. After all, even the cheat-sheet called 'Ten Warning Signs of Primary Immunodeficiency' was only developed in the early 1990s (see below from 4).However, there's a long way to go with allergies, autoimmunities and chronic inflammatory disorders (e.g., inflammatory bowel disease).Prevalence of these conditions increased dramatically in recent years, especially in older industrialized countries, even as their accurate diagnoses and treatments lag behind. For long only glucocorticoids, other immunosuppressives and non-specific painkillers were the mainstay Rx.In recent years many biologics such as mAbs, Monoclonal antibody - Wikipedia, and small molecules that target specific enzymes and cytokines have rapidly gained regulatory approval. However, such therapies only better target symptoms and don't address root causes even as they're found to work optimally only in subsets of patients.Poorly predictive animal models and poor clinical definitions are the major hurdles that prevent faster progress. For example, it's entirely possible that diseases such as MS, Multiple sclerosis - Wikipedia, and SLE, Systemic lupus erythematosus - Wikipedia, are more accurately syndromes, the upshot being subsets of patients could differ in key disease features. In the case of most autoimmunities, even some basic questions such as the identity of target antigen(s) remain unknown.Not knowing the antigen(s) that are the target(s) of the autoimmune responses makes it difficult to precisely treat an autoimmune disease in such a way as to ensure minimal collateral damage.As for allergies and chronic inflammatory disorders, more effective treatments can only come when their underlying causes are better understood, which is not yet the case.Bibliography1. Chinn, Ivan K., and William T. Shearer. "Severe combined immunodeficiency disorders." Immunology and Allergy Clinics 35.4 (2015): 671-694.2. David's Story. William T. Shearer, Carol Ann Demaret. Etzioni, Amos, and Hans D. Ochs, eds. Primary Immunodeficiency Disorders: A Historic and Scientific Perspective. Academic Press, 2014, pages 313-326.3. Picard, Capucine, et al. "Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency 2015." Journal of clinical immunology 35.8 (2015): 696-726. Primary Immunodeficiency Diseases: an Update on the Classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency 20154. 10 Warning SignsThanks for the R2A, Robert Troxell.

I was asked to answer this question, but since it is not my field of expertise (I am a dentist not a hematologist), I will link to a webpage on Medscape and quote what is says about ITP. Medscape has excellent information, and is meant for professional use:http://emedicine.medscape.com/article/779545-medication#showallThis answer is meant only as a reference to what Medscape lists as "Medication" for ITP. Please consult you doctor if you want to learn more about this disease.Prehospital CarePrehospital care focuses on the ABCs, which include providing oxygen, controlling severe hemorrhage, and initiating intravenous (IV) fluids to maintain hemodynamic stability.Prehospital airway control may be necessary for a large intracranial hemorrhage.EMS providers should be aware of the potential for serious bleeding complications in patients with idiopathic thrombocytopenic purpura (ITP).Emergency Department CareLife-threatening bleeding requires conventional critical care interventions.In the patient with known ITP, high-dose parenteral glucocorticoids and IV immunoglobulin (IVIg), with or without platelet transfusions, are appropriate.Platelet transfusion is indicated for controlling severe hemorrhage. Send a blood specimen to the lab for type and screen in case platelet transfusion is necessary.Platelet survival is increased if the platelets are transfused immediately after IVIg infusion.A consultation with a hematologist may be required to make a decision regarding the transfusion of platelets.Guidelines for transfusion dosage6-8 U of platelet concentrate, or 1 U/10 kg1 U of platelets to increase count of a 70-kg adult by 5-10,000/mm3 and an 18-kg child by 20,000/mm3Splenectomy is reserved for patients in whom medical therapy fails. Emergent splenectomy is indicated in patients with life-threatening bleeding in whom medical therapy fails.In patients without life-threatening complications, focus ED care on confirming the diagnosis, if possible, and initiating therapy as needed.Most patients with undiagnosed thrombocytopenia and purpura will need admission for further evaluation and treatment, since ITP is a diagnosis of exclusion.ConsultationsConsult a hematologist for assistance in confirming the diagnosis or, in the patient with known ITP, arranging disposition and follow-up care, if appropriate.Consult a neurosurgeon for intracranial hemorrhage. Consultation by other surgical specialists may be required for extensive hemorrhage at other sites.Medication SummaryGlucocorticoids and IVIg are the mainstays of medical therapy. Indications for use, dosage, and route of administration are based on the patient's clinical condition, the absolute platelet count, and the degree of symptoms. Consultation with a hematologist may be needed prior to starting therapy.Children who have platelet counts >30,000/mm3 and are asymptomatic or have only minor purpura do not require routine treatment. Children who have platelet counts < 20,000/mm3 and significant mucous membrane bleeding and those who have platelet counts < 10,000/mm3 and minor purpura should receive specific treatment.Adults with platelet counts >50,000/mm3 do not require treatment. Treatment is indicated for adults with counts < 50,000/mm3 with significant mucous membrane bleeding. Treatment also is indicated for those adults with risk factors for bleeding (eg, hypertension, peptic ulcer disease, vigorous lifestyle) and in patients with a platelet count < 20,000-30,000/mm3.IV anti-(Rh)D, also known as IV Rh immune globulin (IG), was not recommended by the 1996 American Society of Hematology practice guidelines. However, recent studies using higher dosages of IV RhIG in acute ITP in children and adults show platelet count increases at 24 hours faster than medicating with steroids and at 72 hours similar to IVIg. Although generally less toxic than IV steroids, IV RhIG is more expensive than IV steroids. Studies in children with chronic ITP show that escalating or elevated doses of IV RhIG have comparable responses to those of high-dose IVIg therapy in children. This therapy is not appropriate for patients who have undergone splenectomy. Acute intravascular hemolysis after infusing IV RhIG has been reported, with an estimated incidence of 1 in 1115 patients.Steroid use and immunosuppressives and splenectomy may be undesirable because of their associated complications. For long-term steroid use, this includes osteoporosis, glaucoma, cataracts, loss of muscle mass, and an increased risk of infection. For immunosuppressive therapy and splenectomy, risks include worsening immunosuppression and infection or sepsis. Studies of the use of multiagent therapies in refractory patients are ongoing. Some small studies have shown limited success. According to one study[3] , using a combination of weekly vincristine, weekly methylprednisolone, both until platelet counts reached 50,000/mm3, and cyclosporine orally twice daily until the platelet count is normal for 3-6 months seems promising, though larger prospective studies are needed.Other therapies, such as cyclophosphamide, danazol, dapsone, interferon alfa, azathioprine, vinca alkaloids, accessory splenectomy, and splenic radiation have been studied. Many case series discussing these treatments are too small to show sufficient evidence of a clinically significant reduction in bleeding or mortality rate; however, they serve as additional therapeutic measures in ITP refractory-to-primary therapy (eg, glucocorticoids, IVIg immunoglobulin, splenectomy). Newer studies on rituximab suggest that this agent is an effective treatment option in splenectomized refractory or relapsed ITP patients.[4, 5]Clinical trials have shown promise for agents that directly stimulate platelet production, such as thrombopoietin (TPO) receptor-binding agents. Two new agents, eltrombopag and romiplostim, are available to patients with chronic ITP who have failed other therapies.[6, 7] Both of these agents require registration in a database. While they show promise for raising platelet counts, there are potential safety concerns such as thrombocytosis and rebound thrombocytopenia. It is unlikely that emergency physicians should be prescribing these agents without being under the recommendation of a hematologist.GlucocorticoidsClass SummaryThese agents are used to treat idiopathic and acquired autoimmune disorders. They have been shown to increase platelet count in ITP.View full drug informationPrednisone (Deltasone, Orasone, Sterapred)Useful in treating inflammatory and allergic reactions; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. DOC for all adult patients with platelet counts < 50,000/mm3. Asymptomatic patients with platelet counts >20,000/mm3, or patients with counts 30,000-50,000/mm3 with only minor purpura, may not need therapy; withholding medical therapy may be appropriate for asymptomatic patients, regardless of count.Methylprednisolone (Solu-Medrol, Depo-Medrol)Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased permeability. Used as alternative glucocorticoid of choice for all patients with severe, life-threatening bleeding or children with platelet counts < 30,000/mm3. Careful observation without medical treatment may be appropriate in some asymptomatic children.Blood productsClass SummaryAdministration of IVIg may temporarily increase platelet counts in some children and adults with ITP. Consider IVIg if the situation requires a rapid, temporary rise in platelet count.Intravenous immune globulin (IVIg)DOC for severe, life-threatening bleeding or for children with platelet counts < 20,000/mm3 with minor purpura; can be used alone or in addition to glucocorticoid therapy.Thrombopoietic AgentClass SummaryThese agents directly stimulates bone marrow platelet production.[8]Eltrombopag (Promacta)Oral thrombopoietin (TPO) receptor agonist. Interacts with transmembrane domain of human TPO receptor and induces megakaryocyte proliferation and differentiation from bone marrow progenitor cells. Indicated for thrombocytopenia associated with chronic idiopathic thrombocytopenic purpura in patients experiencing inadequate response to corticosteroids, immunoglobulins, or splenectomy. Not for use to normalize platelet counts but used when clinical condition increases bleeding risk.Prescribers must enroll in Promacta Cares program. Only available through restricted distribution program. Program phone number is (877) 9-PROMACTA (877-977-6622).Romiplostim (Nplate)An Fc-peptide fusion protein (peptibody) that increases platelet production through binding and activation of the thrombopoietin (TPO) receptor, a mechanism similar to endogenous TPO. Indicated for chronic immune (idiopathic) thrombocytopenic purpura in patients who have had an insufficient response to corticosteroids, immunoglobulins, or splenectomy.Only available through the Nplate NEXUS (Network of Experts Understanding and Supporting Nplate) program, a program designed to promote informed risk-benefit decisions before initiating treatment. For more information, see www.nplate.com or call (877) NPLATE1 (877-675-2831).

Thank you for the A2A.The variable part of Common Variable Immune Deficiency, or CVID, applies here. Sometimes people with CVID develop difficulty walking, but oftentimes they do not.People with CVID have a higher than normal chance of developing a number of different co-morbidities. One of the most frequent co-morbidities are auto-immune disorders, such as rheumatoid arthritis or lupus. These auto-immune disorders often lead to difficulties walking, but not always.Fatigue so profound that walking is difficult, can also be a problem with CVID. This is especially true before the CVID has been diagnosed, and thus, before immunoglobulin treatments have been initiated. This fatigue can also become severe during times of illness, or when the patient’s IgG level is low (such as the period before the patient’s next IVIG, or SQIG infusion). Not surprisingly, the symptoms of auto-immune disorders, including difficulty walking, are also greatest before diagnoses and treatment, during illness, and when IgG levels are at their lowest before infusions.I had my most recent IVIG infusion this morning. I was so fatigued, and my joints hurt so badly this morning, that I could barely walk into the infusion canter. By the time I walked out three hours later, it was as though I was a different person. I was tired from the infusion, but the crushingly severe fatigue had ended. My joint pain had also improved, and will likely continue to improve even more over the next couple of days.I get my IVIG infusions every three weeks. I used to get them every four weeks, but I was so incapacitated, and my breathing problems became so severe over that fourth week, that that I was frequently in danger of losing my life. Now, unless I get sick (which sadly, happens more than I would like), I can live fairly normally for almost three weeks. (I never stay healthy for the full three weeks though. Two or three days before my next infusion, I begin to fall apart once again, and walking, once again, becomes difficult.)Please feel free to message me if you would like. I volunteer with the Primary Immune Definitely Foundation, and despite needing to retire from my profession a bit earlier than I had hoped, I am still technically an RN. I would be happy to answer any questions you might have, provide you with resources, share more of my own experience with CVID, or be there for you while you share yours.