hep-c, hereditary angioedema, alpha-1 antitrypsin deficiency

Hepatitis C

Hepatitis C (HCV) is an infectious disease affecting primarily the liver, caused by the hepatitis C virus. When people refer to HCV, they are actually talking about a family of related virus strains.

Today, there are six major genotypes (or strains) of HCV and more than 50 subtypes. These genotypes share some common gene sequences (or traits) with other HCV genotypes, but differ in others.

Approximately 3 million people in the United States have chronic HCV, most of them born from 1945-1965. Most were likely infected during the 1970s and 1980s, before the donor blood supply was screened for HCV.

HCV is transmitted mostly through large or repeated exposures to infectious blood or body fluids such as:

• Injection drug use
• As a recipient of donated blood, blood products, and organs (before 1992)
• Needlestick injuries in the health care setting
• Mother to childbirth transmission from an HCV-infected mother
• Unprotected sex with a person infected with HCV
• Unsanitary tattoo procedures with contaminated equipment

Many patients experience no symptoms when they are initially infected. Patients may experience non-specific, mild symptoms that are unlikely to prompt a visit to their physician. When symptoms occur, they can include:

• Fever
• Fatigue
• Dark urine
• Clay-colored stool
• Abdominal pain
• Loss of appetite
• Nausea and vomiting
• Muscle aches and joint pain
• Jaundice (yellowing of skin and eyes)

Of those that are exposed to HCV, 75%-85% have chronic HCV. For the remaining 15-25% the body will naturally clear the virus without treatment and no chronic infection results.

Those with chronic HCV experience gradual liver damage, scarring (or cirrhosis) and loss of function. In some cases, those with cirrhosis will go on to develop complete liver failure (resulting in the need for transplantation), liver cancer or life-threatening esophageal and weak gastric veins.

Because HCV infection doesn’t typically cause symptoms, patients are often not diagnosed until their donated blood is screened or when elevated liver enzymes are found during a routine health exam.

Because symptoms of the disease are often silent, the U.S. Centers for Disease Control recommends that all people born between 1945 and 1965—as well as other at-risk populations—be screened for HCV. It is important to discuss your individual risk for infection and the need for testing with your physician

The following specialty medications are available at Albrighton Pharmacy, a specialty pharmacy for hepatitis C.         

• Copegus® (ribavirin) 
• Daklinza™ (daclatasvir) 
• Epclusa® (sofosbuvir 400 mg/velpatasvir 100 mg) 
• Harvoni® (ledipasvir and sofosbuvir tablets)  
• Intron® A (interferon alfa-2b)
• Moderiba™ (ribavirin, USP) 
• Mavyret™ (glecaprevir/pibrentasvir)‎ 
• Olysio™ (simeprevir)  
• Promacta® (thrombocytopenia) 
• Rebetol® (ribavirin)
• Ribapak® (ribavirin) 
• Ribasphere® (ribavirin) 
• Sovaldi™ (sofosbuvir) 
• Technivie™ (ombitasvir, paritaprevir, and ritonavir tablets) 
• Viekira XR™ (dasabuvir, ombitasvir, paritaprevir, and ritonavir tablets)
• Vosevi® (sofosbuvir/velpatasvir/voxilaprevir) 
• Zepatier® (elbasvir and grazoprevir) 

Financial assistance coordination may be available to help with your medication costs, including manufacturer and community programs. Albrighton Pharmacy representatives are available to help find a program that may work for you.

• Good Days® | 1-877-968-7233
• OPUS Health | 1-800-364-4767
• Patient Access Network Foundation | 1-866-316-7263
• Patient Advocate Foundation | 1-800-532-5274

There are many organizations that support research and advocacy for HCV and liver disease. See below for a few of those organizations.

• American Association for the Study of Liver Diseases
• American Gastroenterological Association
• American Liver Foundation
• HCV Advocate

• Centers for Disease Control

 Hereditary Angioedema

Hereditary angioedema, or HAE, is a rare, potentially disabling and life-threatening genetic disorder characterized by episodes of severe swelling (angioedema) in the hands, feet, genitals, stomach, face and/or throat.

Hereditary angioedema (HAE) is a very rare and potentially life-threatening genetic condition that occurs in about 1 in 10,000 to 1 in 50,000 people.1

The main symptom of HAE is swelling (angioedema). Swelling (angioedema) occurs or reoccurs in the hands, feet, gastrointestinal tract, genitals and throat that can develop over several hours and can last for two to five days. Painful swelling in hands and feet can decrease daily activities. When swelling (angioedema) involves the face or throat, seek immediate medical treatment. Swelling of the throat is dangerous due to blockage of the airways of the lungs and could be deadly. Swelling in the stomach can result in severe pain, vomiting, nausea, diarrhea and dehydration. These symptoms vary in frequency, severity and location from patient to patient.2

The diagnosis of hereditary angioedema is based upon patient history and physical findings during episodes of swelling (angioedema) combined with unexplained abdominal pain, family history and X-rays. Blood and genetic samples are also required to diagnose and distinguish between the two different types of hereditary angioedema, Type I and II.

2. https://www.haea.org/WhatIsHAE.pdf

1. https://www.haea.org/HAEdisease.php

The following specialty medications are available at Albrighton Pharmacy, your specialty pharmacy, for the treatment of hereditary angioedema.

Berinert® (C1 esterase inhibitor) 

Cinryze® (C1 esterase inhibitor)            

Haegarda® (C1 esterase inhibitor [subcutaneous])             

Ruconest® (C1 esterase inhibitor [recombinant])             

Firazyr® (icatibant injection)            

Kalbitor® (ecallantide)    

Financial assistance coordination may be available to help with your medication costs, including manufacturer and community programs. Albrighton Pharmacy representatives are available to help find a program that may work for you.

• US Hereditary Angioedema Association (HAEA)

 Overview

If you are diagnosed with alpha-1, it means your blood and lungs have low levels (a deficiency) of a protein called alpha-1 antitrypsin (AAT). You need AAT to have healthy lungs; a deficiency can lead to lung and/or liver disease.

Alpha-1 is widely under-diagnosed. Of the 100,000 individuals in the United States estimated to have this condition, only about 10 percent have been identified. Many times, the symptoms are misdiagnosed as chronic obstructive pulmonary disease (COPD) or even asthma.1

Some experts estimate as many between 65,000 and 110,000 people (1 in 3,000 – 5,000) in the United States have alpha-1. As many as 85% are undiagnosed. Alpha-1 is found in all ethnic groups, but it is most common in Caucasians of Northern European ancestry.1

You can reach your Albrighton Pharmacy alpha-1 antitrypsin deficiency team, anytime, day or night, seven days a week by phone or online.

You might see signs of alpha-1 in your lung or liver functions or, in rare cases, your skin. Unfortunately, alpha-1 often is not discovered until there has been significant tissue damage, or until a patient has been diagnosed with emphysema, chronic obstructive pulmonary disease or treatment-resistant asthma. This is why testing is so important. Some of the most common signs of alpha-1 include, but are not limited to, the following list of symptoms.

• Abdominal swelling and/or leg swelling
• Dark urine
• Intense itching
• Jaundice (yellowing) of the eyes and skin
• Pain or tenderness in the upper right abdomen
• Unexplained liver problems and/or elevated liver enzymes
• Hardened skin with painful patches or lumps (panniculitis)

• Cough with or without mucus
• Decreased exercise tolerance
• Increased supplemental oxygen needs
• Nonresponsive asthma or year-round allergies
• Recurring respiratory infections or chest colds
• Shortness of breath
• Wheezing

• Hardened skin with painful patches or lumps (panniculitis)Hardened

Alpha-1 can be detected by a simple test that determines the level of AAT in the blood.

The American Thoracic Society and European Respiratory Society both recommend alpha-1 testing for people who have been diagnosed with any of the following:

• Chronic obstructive pulmonary disease
• Emphysema (diagnosed at age 45 or earlier)
• Liver disease with no known cause
• Bronchiectasis (in adults)
• Asthma that does not respond to traditional treatment
• Unexplained panniculitis (skin disorder) and Wegener’s granulomatosis
• Family history of Alpha-1, emphysema, bronchiectasis, liver disease, or panniculitis
• Difficulty breathing and persistent cough among family members (same or multiple generations)
• Testing should also be done for family members of individuals with Alpha-1

The following specialty medications are available at Albrighton Pharmacy, your specialty pharmacy, for the treatment of alpha-1 antitrypsin deficiency.

Aralast NP® (alpha1-proteinase inhibitor [human])

Glassia® (alpha1-proteinase inhibitor [human])

Zemaira® (alpha1-proteinase inhibitor [human])

Financial assistance coordination may be available to help with your medication costs, including manufacturer and community programs. Albrighton Pharmacy representatives are available to help find a program that may work for you.

• Patient Access Network Foundation | (866) 316-7263
• Good Days (Chronic Disease Fund) | (877) 968-7233

There are many organizations that support research and advocacy for alpha-1 antitrypsin deficiency. See below for a few of those organizations.

• Alpha-1 Foundation
• AlphaNet