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Alpha-1 Antitrypsin Deficiency (A1AD)

About the disease

Alpha-1 antitrypsin deficiency (A1AD) is a genetic disorder that may result in lung disease or liver disease. Onset of lung problems is typically between 20 and 50 years old. This may result in shortness of breath, wheezing, or an increased risk of lung infections. Complications may include COPD, cirrhosis, neonatal jaundice, or panniculitis. A1AD is due to a mutation in the SERPINA1 gene that results in not enough alpha-1 antitrypsin (A1AT). Risk factors for lung disease include cigarette smoking and environmental dust. The underlying mechanism involves unblocked neutrophil elastase and buildup of abnormal A1AT in the liver. It is autosomal co- dominant, meaning that one defective allele tends to result in milder disease than two defective alleles. The diagnosis is suspected based on symptoms and confirmed by blood tests or genetic tests.

Treatment

Treatment of lung disease may include bronchodilators, inhaled steroids, and when infections occur antibiotics. Intravenous infusions of the A1AT protein or in severe disease lung transplantation may also be recommended. In those with severe liver disease liver transplantation may be an option. Avoiding smoking is recommended. Vaccination for influenza, pneumococcus, and hepatitis is also recommended. Life expectancy among those who smoke is 50 years old while among those who do not smoke it is almost normal.

Products CSI Pharmacy offers

Glassia

More Information

please visit the Alpha-1 Foundation at https://www.alpha1.org/

Crohn's Disease

About the disease

Crohn's disease is a condition of chronic inflammation potentially involving any location of the gastrointestinal tract, but it frequently affects the end of the small bowel and the beginning of the large bowel. In Crohn’s disease, all layers of the intestine may be involved and there can be normal healthy bowel between patches of diseased bowel. The Center for Disease Control and Prevention (CDC) reports that symptoms include persistent diarrhea (loose, watery, or frequent bowel movements), cramping, abdominal pain, fever, and, at times, rectal bleeding. Loss of appetite and weight loss also may occur. However, the disease is not always limited to the gastrointestinal tract; it can also affect the joints, eyes, skin, and liver. Fatigue is another common complaint. The exact cause of Crohn’s disease is unknown. People with Crohn’s disease have ongoing (chronic) inflammation of the gastrointestinal tract (GI tract). Crohn’s disease may involve the small intestine, the large intestine, the rectum, or the mouth. The inflammation causes the intestinal wall to become thick. There are different types of Crohn’s disease. The type depends on what part of your body is affected.

Treatment

  • Aminosalicylates (5-ASAs) are medicines that help control mild to moderate symptoms. Some forms of the drug are taken by mouth; others must be given rectally.
  • Corticosteroids (prednisone and methylprednisolone) are used to treat moderate to severe Crohn’s disease. They may be taken by mouth or inserted into the rectum.
  • Medicines such as azathioprine or 6-mercaptopurine quiet the immune system’s reaction.
  • Antibiotics may be prescribed for abscesses or fistulas.
  • Biologic therapy is used to treat patients with severe Crohn’s disease that does not respond to any other types of medication.

Products CSI Pharmacy offers

Cimzia

Entyvio

Humira

Infliximab

Octreotide

Simponi

Stelara

Xifaxan

More Information

please visit the Crohn's & Colitis Foundation at http://www.crohnscolitisfoundation.org/

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

About the disease

The term Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) has been used to identify patients with a chronically progressive or relapsing symmetric sensorimotor disorder with cytoalbuminologic dissociation and interstitial and perivascular endoneurial infiltration by lymphocytes and macrophages. It can be considered the chronic equivalent of acute inflammatory demyelinating polyradiculoneuropathy, the most common form of Guillain-Barré syndrome. CIDP typically starts insidiously and evolves slowly, in either a slowly progressive or a relapsing manner, with partial or complete recovery between recurrences; periods of worsening and improvement usually last weeks or months. Most experts consider the necessary duration of symptoms to be greater than 8 weeks for the diagnosis of CIDP to be made. Pertinent physical findings are limited to the nervous system, except when the condition is associated with other diseases.

20% of CSI Pharmacy patients have CIDP.

Treatment

  • Immune Globulin Therapy
  • Corticosteroids

Products CSI Pharmacy offers

Immune Globulin Therapy

Corticosteroids

More Information

please visit the CIDP-GBS Foundation International at https://www.gbs-cidp.org/

Guillain–Barré syndrome (GBS)

About the disease

Guillain–Barré syndrome (GBS) is a rapid-onset muscle weakness caused by the immune system damaging the peripheral nervous system. The initial symptoms are typically changes in sensation or pain along with muscle weakness, beginning in the feet and hands. This often spreads to the arms and upper body, with both sides being involved. The symptoms develop over hours to a few weeks. During the acute phase, the disorder can be life-threatening, with about 15% developing weakness of the breathing muscles requiring mechanical ventilation. Some are affected by changes in the function of the autonomic nervous system, which can lead to dangerous abnormalities in heart rate and blood pressure. The cause is unknown. The underlying mechanism involves an autoimmune disorder in which the body's immune system mistakenly attacks the peripheral nerves and damages their myelin insulation. Sometimes this immune dysfunction is triggered by an infection or, less commonly by surgery and rarely by vaccination.

Treatment

In those with severe weakness, prompt treatment with intravenous immunoglobulins or plasmapheresis, together with supportive care, will lead to good recovery in the majority. Recovery may take weeks to years. About a third have some permanent weakness.

Products CSI Pharmacy offers

Immune Globulin Therapy

Corticosteroids

More Information

please visit the CIDP-GBS Foundation International at https://www.gbs-cidp.org/

Huntington's disease (HD)

About the disease

Huntington's disease (HD), also known as Huntington's chorea, is an inherited disorder that results in death of brain cells. The earliest symptoms are often subtle problems with mood or mental abilities. A general lack of coordination and an unsteady gait often follow. As the disease advances, uncoordinated, jerky body movements become more apparent. Physical abilities gradually worsen until coordinated movement becomes difficult and the person is unable to talk. Mental abilities generally decline into dementia. The specific symptoms vary somewhat between people. Symptoms usually begin between 30 and 50 years of age; but can start at any age. The disease may develop earlier in life in each successive generation. About eight percent of cases start before the age of 20 years and typically present with symptoms more similar to Parkinson's disease. People with HD often underestimate the degree of their problems.

Treatment

There is no cure for HD. The best evidence for treatment of the movement problems is with tetrabenazine and austedo.

Products CSI Pharmacy offers

Austedo

Tetrabenazine

More Information

please visit the Huntington’s Disease Society of America at https://hdsa.org/

please visit the Movement Disorder Society at https://www.movementdisorders.org/

Immune Thrombocytopenia (ITP)

About the disease

Immune thrombocytopenia (ITP) is a type of thrombocytopenic purpura defined as isolated low platelet count (thrombocytopenia) with normal bone marrow and the absence of other causes of thrombocytopenia. It causes a characteristic purpuric rash and an increased tendency to bleed. Two distinct clinical syndromes manifest as an acute condition in children and a chronic condition in adults. The acute form often follows an infection and has a spontaneous resolution within two months. Chronic immune thrombocytopenia persists longer than six months with a specific cause being unknown. ITP is an autoimmune disease with antibodies detectable against several platelet surface antigens.

Treatment

  • Antibiotics
  • Anti-D
  • Monoclonal Antibody (Rituxan)
  • Chemotherapy
  • Corticosteroids
  • Immune Globulin Therapy
  • Immunosuppressants
  • Modified Testosterone
  • Platelet Growth Factors
  • Splenectomy

Products CSI Pharmacy offers

Immune Globulin Therapy

CellCept

Corticosteroids

More Information

please visit the Platelet Disorder Support Association at https://www.pdsa.org/

Multifocal Motor Neuropathy (MMN)

About the disease

Multifocal motor neuropathy (MMN) is a progressively worsening condition where muscles in the extremities gradually weaken. The disorder, a pure motor neuropathy syndrome, is sometimes mistaken for amyotrophic lateral sclerosis (ALS) because of the similarity in the clinical picture, especially if muscle fasciculations are present. MMN is thought to be autoimmune. It was first described in the mid-1980s. Unlike ALS which affects both upper and lower motor nerves, MMN involves only lower motor nerves. Nevertheless, definitive diagnosis is often difficult, and many MMN patients labor for months or years under an ALS diagnosis before finally getting a determination of MMN. MMN usually involves very little pain; however, muscle cramps, spasms and twitches can cause pain for some sufferers. MMN is not fatal and does not diminish life expectancy. Many patients, once undergoing treatment, only experience mild symptoms over prolonged periods, though the condition remains slowly progressive. MMN can however, lead to significant disability, with loss of function in hands affecting ability to work and perform everyday tasks, and "foot drop" leading to inability to stand and walk; some patients end up using aids like canes, splints and walkers.

Treatment

Multifocal motor neuropathy is normally treated by receiving intravenous immunoglobulin (Immune Globulin Therapy), which can in many cases be highly effective, or immunosuppressive therapy with cyclophosphamide or rituximab. Steroid treatment (prednisone) and plasmapheresis are no longer considered to be useful treatments; prednisone can exacerbate symptoms. Immune Globulin Therapy is the primary treatment, with about 80% of patients responding, usually requiring regular infusions at intervals of 1 week to several months. Subcutaneous immunoglobulin is under study as a less invasive, more-convenient alternative to IV delivery.

Products CSI Pharmacy offers

Immune Globulin Therapy

More Information

please visit the GBS/CIDP Foundation International at https://www.gbs-cidp.org/

please visit the Neuropathy Action Foundation at http://www.neuropathyaction.org/

Multiple Sclerosis (MS)

About the disease

Multiple sclerosis (MS) is a nervous system condition that affects the brain and spinal cord. It damages the myelin sheath, the material that surrounds and protects your nerve cells. This damage slows down or blocks messages between the brain and the body. While MS can sometimes cause disabilities for those diagnosed with it, advances in treatment and patient care have made it possible for most people with MS to enjoy a normal life expectancy. There are four general classes of MS including: Relapsing-Remitting MS, Secondary- Progressive MS, Primary-Progressive MS, and Progressive-Relapsing MS.

Treatment

There is still no cure for MS, but there are treatments for initial attacks, medications and therapies to improve symptoms, and recently developed drugs to slow the worsening of the disease. These new drugs have been shown to reduce the number and severity of relapses and to delay the long-term progression of MS. These treatments may include injectable steroids, oral steroids and plasma exchanges as a secondary option in certain types of MS. In addition, drugs are available to reduce relapses in patients with relapsing MS called disease modulating drugs and immunomodulator medications impact the immune system to treat patients with relapsing MS.

Products CSI Pharmacy offers

Ampyra

Avonex

Corticosteroids

Copaxone

Extavia

Gilenya

Imune Globulin Therapy

Ribif

More Information

please visit the Multiple Sclerosis Association of America at https://mymsaa.org/

Myasthenia Gravis (MG)

About the disease

Myasthenia Gravis (MG) is a chronic autoimmune neuromuscular disease characterized by weakness of the skeletal muscles. Common symptoms include weakness of the muscles that control the eye and eyelid, facial expressions, chewing, talking, and swallowing. Weakness tends to increase during periods of activity and improve after periods of rest. The condition results from a defect in the transmission of nerve impulses to muscles, which is due to the presence of antibodies against acetylcholine. The exact reason this occurs is not known. Some cases have been linked to tumors in the thymus gland. Researchers believe that variations in certain genes may increase a person's risk to develop MG, but other factors likely also play a role. There is no cure for MG at this time, but treatment can significantly improve muscle weakness. Some cases may go into remission temporarily, and muscle weakness may disappear so that medications can be discontinued. People can develop MG at any age. For unknown reasons, it is most commonly diagnosed in women younger than age 40 and men older than age 60.

20% of CSI Pharmacy patients have a form of Myasthenia Gravis.

Treatment

  • Cholinesterase Inhibitor
  • Corticosteroids
  • Immunosuppressants
  • Monoclonal Antibody
  • Plasma Exchange
  • Immune Globulin Therapy
  • Soliris
  • Thymectomy

Products CSI Pharmacy offers

Immune Globulin Therapy

CellCept

Corticosteroids

More Information

please visit the Myasthenia Gravis Foundation of America at http://www.myasthenia.org/

please visit the Myasthenia Gravis Hope Foundation at https://mghope.org/

Myositis

About the disease

Myositis is a collection of several rare diseases that share the common elements of muscle weakness and/or pain along with an autoimmune component. In adults, these diseases include polymyositis (PM), dermatomyositis (DM), necrotizing myopathy (NM) and sporadic inclusion body myositis (sIBM). There is also a juvenile form of DM that affects children (JDM). Myositis signs and symptoms include: muscles weakness, sometimes with muscle pain; general tiredness and fatigue; trouble climbing stairs, standing from a seated position or reaching up; difficulty swallowing or breathing; rash on knuckles, chest, face and back; persistent dry cough.

20% of CSI Pharmacy patients have a form of Mysoitis.

Treatment

  • Corticosteroids
  • Immunosuppressants
  • Monoclonal Antibody
  • Immune Globulin Therapy

Products CSI Pharmacy offers

Immune Globulin Therapy

CellCept

Corticosteroids

More Information

please visit the The Myositis Association at https://www.myositis.org/

please visit the Cure JM Foundation at http://www.curejm.org/

Osteoperosis

About the disease

Osteoporosis is a disease where increased bone weakness increases the risk of a broken bone. It is the most common reason for a broken bone among the elderly. Bones that commonly break include the vertebrae in the spine, the bones of the forearm, and the hip. Until a broken bone occurs there are typically no symptoms. Bones may weaken to such a degree that a break may occur with minor stress or spontaneously. Chronic pain and a decreased ability to carry out normal activities may occur following a broken bone.

Treatment

Bisphosphonates are useful in decreasing the risk of future fractures in those who have already sustained a fracture due to osteoporosis.

Products CSI Pharmacy offers

Alendronate 70mg(75mL) Oral Liquid

More Information

please visit the National Osteoporosis Foundation at https://www.nof.org/

Pemphigus & Pemphigoid

About the disease

Pemphigus and pemphigoid are rare, autoimmune diseases that affect a very small percentage of the population. There are many different autoimmune diseases, and they can each affect the body in different ways. Pemphigus vulgaris, the most common of the pemphigus diseases, affects the skin and mucous membranes.

Treatment

  • Corticosteroids
  • Immunosuppressants
  • Immune Globulin Therapy

Products CSI Pharmacy offers

Corticosteroids

CellCept

Immune Globulin Therapy

More Information

please visit the International Pemphigus & Pemphigoid Foundation at http://www.pemphigus.org/

Primary Immunodeficiency Diseases (PI)

About the disease

Primary immunodeficiency diseases (PI) are a group of more than 350 rare, chronic disorders in which part of the body’s immune system is missing or functions improperly. While not contagious, these diseases are caused by hereditary or genetic defects, and, although some disorders present at birth or in early childhood, the disorders can affect anyone, regardless of age or gender. Some affect a single part of the immune system; others may affect one or more components of the system. While the diseases may differ, they all share one common feature; each results from a defect in one of the functions of the body's normal immune system. For people living with PI, infections may not go away or can come back often, even with the use of medications. Infections may be common, severe, long-lasting, hard to cure and may become life threatening.

Treatment

  • Antibiotics (long-term)
  • Immune Globulin Therapy
  • In some cases, bone marrow transplants, gene therapy, or other alternative treatments may be appropriate.

Products CSI Pharmacy offers

Immune Globulin Therapy

More Information

please visit the Immune Deficiency Foundation at https://primaryimmune.org/

please visit the Jeffrey Modell Foundation at http://www.info4pi.org/

Stiff-person syndrome (SPS)

About the disease

Stiff-person syndrome (SPS), also known as stiff-man syndrome (SMS), is a rare neurologic disorder of unclear cause characterized by progressive rigidity and stiffness. The stiffness primarily affects the truncal muscles and is superimposed by spasms, resulting in postural deformities. Chronic pain, impaired mobility, and lumbar hyperlordosis are common symptoms.

Treatment

  • Baclofan
  • Corticosteroids
  • Diazepam
  • Plasma Exchange
  • Immune Globulin Therapy
  • Monoclonal Antibody

Products CSI Pharmacy offers

Immune Globulin Therapy

Corticosteroids

More Information

please visit the National Organization for Rare Diseases at https://rarediseases.org/

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