WHAT IS DIRA?
DIRA is an autoinflammatory disease first reported in 2009 and is characterized by the absence of functional interleukin-1 receptor antagonist (IL-1Ra) protein. Patients with DIRA have life-threatening systemic inflammation with skin and bone involvement. DIRA is a genetic disorder inherited in an autosomal recessive manner, meaning that a mutation in both copies of a gene is needed to cause the disease.
DIRA is a very rare disease with fewer than 50 patients reported to have the disease worldwide. Cases have been identified in families originating in Puerto Rico, Newfoundland (Canada), the Netherlands, Palestine/Lebanon, Germany, Turkey, India, and Brazil. In Puerto Rico, the incidence of DIRA in some regions may be as high as 1 in 6300 births. Although difficult to estimate, mortality is approximately 30% in early infancy.
DIRA is characterized by a neonatal onset, with patients presenting at birth or in the first days of life with prominent skin and bone manifestations. Some of the more distinctive clinical features of the disease include:
- Large, fluid-filled, blister-like areas of the skin
- Painful areas of damaged bone
- Joint swelling
Children with DIRA experience intense chronic pain. This pain may lead to feeding challenges and subsequent growth delays. If left untreated, DIRA can escalate to life-threatening inflammation, with the development of systemic inflammatory response syndrome (SIRS), multiorgan failure, and death in early childhood.
Please visit the Autoinflammatory Alliance to learn more about DIRA.
ARCALYST is indicated for the maintenance of remission of DIRA in adults and pediatric patients weighing at least 10 kg.
DOSING AND ADMINISTRATION
ARCALYST is a once-weekly, self-administered subcutaneous injection
Your first injection of ARCALYST will be given with the help of a healthcare provider in the doctor's office, or with support from an ARCALYST Nurse Educator.
Your first injection of ARCALYST will be given with the help of a healthcare provider in the doctor’s office or with support from an ARCALYST Nurse Educator.
Do not try to give yourself ARCALYST injections until you are sure that you understand how to prepare and inject your dose.
The recommended dose for DIRA is shown below.
It is important to use ARCALYST exactly as prescribed by your healthcare provider.
Adults
(18 years and older)
Pediatric Patients
(weighing at least 10 kg)
After training on ARCALYST, you will self-administer a once-weekly injection.
See Instructions for Use for detailed information on the preparation and administration of your injection.
Step-by-step administration
Resources to help guide you
In addition to what you have learned from your healthcare provider or ARCALYST Nurse Educator, the resources below can help you better understand how to prepare and inject ARCALYST.
Learn more about how to use ARCALYST.
Frequently Asked Questions
Find the answers you need
You may have some questions about treatment with ARCALYST. Whether you are considering starting or already taking ARCALYST, we are here to help guide you through some frequently asked questions and provide you with the answers you are looking for. As always, be sure to discuss your treatment options with your healthcare provider and contact Kiniksa OneConnect™ at 1-833-KINIKSA (1-833-546-4572) with further questions and for finding support.
ARCALYST is a once-weekly, self-administered medication given by subcutaneous injection (under the skin).
Take ARCALYST exactly as prescribed by your healthcare provider.
ARCALYST is given by injection under the skin (subcutaneous injection) 1 time each week. The Kiniksa OneConnect™ program can work with you to set up one-on-one injection training sessions with an ARCALYST Clinical Educator, so you are familiar with the administration process. Do not try to give yourself ARCALYST injections until you are sure that you understand how to prepare and inject your dose. Call your healthcare provider, pharmacist, or your Kiniksa OneConnect™ Patient Access Lead at 1-833-KINIKSA (1-833-546-4572) if you have any questions.
Other resources include the Step-by-Step Injection video, the Step-by-Step Injection Manual, and the ARCALYST Instructions for Use.
Keep ARCALYST in the carton it comes in to protect it from light. Store ARCALYST in the refrigerator between 36° F and 46° F (2° C and 8° C). Refrigerated ARCALYST can be used until the expiration date printed on the vial and carton. ARCALYST may be kept at room temperature after mixing with sterile water for injection and should be used within 3 hours of mixing.
Keep ARCALYST away from light, and keep ARCALYST, injection supplies, and all other medicines out of the reach of children.
If you miss a dose, you should speak with your healthcare provider. For more information, please see the full Prescribing Information.
Your Kiniksa OneConnect™ Patient Access Lead will help you coordinate the delivery of your medication and supplies.
Before starting treatment with ARCALYST, tell your healthcare provider if you:
- Think you have an infection
- Are being treated for an infection
- Have signs of an infection, such as fever, cough, or flu-like symptoms
- Have any open sores on your body
- Have a history of infections that keep coming back
- Have asthma. People with asthma may have an increased risk of infection
- Have diabetes or an immune system problem. People with these problems have a higher chance for infections
- Have tuberculosis (TB), or if you have been in close contact with someone who has had tuberculosis
- Have or have had HIV, hepatitis B, or hepatitis C
- Take other medicines that affect your immune system
Before you begin treatment with ARCALYST, talk with your healthcare provider about your vaccine history. Ask your healthcare provider whether you should receive any vaccines, including the pneumonia vaccine and flu vaccine, before you begin treatment with ARCALYST.
Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements. Especially tell your healthcare provider if you take other medicines that affect your immune system, such as medicines that block tumor necrosis factor, interleukin-1, or corticosteroids.
You should not take medicines that block tumor necrosis factor (TNF), such as Enbrel® (etanercept), Humira® (adalimumab), or Remicade® (infliximab), while you are taking ARCALYST. You should also not take other medicines that block interleukin-1 (IL-1), such as Kineret® (anakinra), while taking ARCALYST. Taking ARCALYST with any of these medicines may increase your risk of getting a serious infection.
For patients taking CYP450 substrates, such as warfarin, the dose of these types of medications may need to be adjusted upon initiation of ARCALYST.
You should not receive live vaccines if you take ARCALYST.
Talk to your healthcare provider if you experience any side effects that bother you or that do not go away. Please report side effects by contacting Kiniksa Pharmaceuticals toll-free at 1-833-546-4572, Option 4, or contact FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
If you believe you are experiencing a medical emergency, please call 911.
Tell your healthcare provider if you are pregnant or plan to become pregnant:
- It is not known if ARCALYST will harm your unborn child. Tell your healthcare provider right away if you become pregnant while taking ARCALYST
- If you are breastfeeding or plan to breastfeed. It is not known if ARCALYST passes into breast milk. Talk to your healthcare provider about the best way to feed your baby during treatment with ARCALYST
ARCALYST can affect your immune system. ARCALYST can lower the ability of your immune system to fight infections. Serious infections, including life-threatening infections and death, have happened in people taking ARCALYST. Taking ARCALYST can make you more likely to get infections, including life-threatening serious infections, or may make any infection that you have worse.
You should not begin treatment with ARCALYST if you have an infection or have infections that keep coming back (chronic infection).
After starting ARCALYST, if you get an infection, any sign of an infection, including a fever, cough, flu-like symptoms, or have any open sores on your body, call the healthcare provider right away. Treatment with ARCALYST should be stopped if you get a serious infection.
You should not take medicines that block tumor necrosis factor (TNF), such as Enbrel® (etanercept), Humira® (adalimumab), or Remicade® (infliximab), while you are taking ARCALYST. You should also not take other medicines that block interleukin-1 (IL-1), such as Kineret® (anakinra), while taking ARCALYST. Taking ARCALYST with any of these medicines may increase your risk of getting a serious infection.
Before starting treatment with ARCALYST, tell the healthcare provider if you or your child:
- Think you have an infection
- Are being treated for an infection
- Have signs of an infection, such as fever, cough, or flu-like symptoms
- Have any open sores on your body
- Have a history of infections that keep coming back
- Have asthma. People with asthma may have an increased risk of infection
- Have diabetes or an immune system problem. People with these problems have a higher chance for infections
- Have tuberculosis (TB), or if you have been in close contact with someone who has had tuberculosis
- Have or have had HIV, hepatitis B, or hepatitis C
- Take other medicines that affect your immune system
Before you begin treatment with ARCALYST, talk with the healthcare provider about your vaccine history. Ask the healthcare provider whether you should receive any vaccines, including the pneumonia vaccine and flu vaccine, before you begin treatment with ARCALYST.
See Prescribing Information for additional safety information for ARCALYST.
The Kiniksa OneConnect™ program is made up of a team of dedicated Patient Access Leads who provide personalized one-on-one support at each step of your treatment journey with ARCALYST. You can find services to help you with starting treatment, financial assistance, and treatment support. You can contact a Kiniksa OneConnect™ team member at 1-833-KINIKSA (1-833-546-4572) Monday-Friday (8 AM - 8 PM ET).
ARCALYST Nurse Educators are qualified personnel who can assist you with in-person or virtual administration training to help you better understand the self-injection process.
ARCALYST can cause serious side effects, including:
- Serious Infections. ARCALYST can affect your immune system. ARCALYST can lower the ability of your immune system to fight infections. Serious infections, including life-threatening infections and death, have happened in people taking ARCALYST. Taking ARCALYST can make you more likely to get infections, including life-threatening serious infections, or may make any infection that you have worse.
- Risk of Cancer. Medicines that affect the immune system may increase the risk of getting cancer.
- Allergic Reaction. Stop taking or giving ARCALYST and call the healthcare provider or get emergency care right away if you get any of the following symptoms of an allergic reaction while taking ARCALYST:
- Rash
- Swollen face
- Trouble breathing
- Changes in your blood cholesterol and triglycerides (lipids). Your healthcare provider will do blood tests to check for these changes.
Common side effects:
- In people with CAPS and RP, the most common side effects of ARCALYST include:
- Injection-site reactions including: pain, redness, swelling, itching, bruising, lumps, inflammation, skin rash, blisters, warmth, and bleeding at the injection site.
- Upper respiratory tract infections
- Joint and muscle aches in RP
- In people with DIRA, the most common side effects of ARCALYST include:
- Upper respiratory tract infections
- Rash
- Ear infection
- Sore throat
- Runny nose
These are not all the possible side effects of ARCALYST. Tell your healthcare provider if you have any side effect that bothers you or does not go away. For more information, ask your healthcare provider or pharmacist.
Kiniksa OneConnect™
Your personalized treatment support starts here.
We are a team of experienced individuals, with knowledge of insurance plans and healthcare networks, known as Patient Access Leads.
Patient Access Leads
Once you are enrolled in Kiniksa OneConnect, you will be paired with a dedicated Patient Access Lead to receive personalized one-on-one support throughout your entire treatment experience.
While your healthcare provider is your go-to resource for all of your medical needs, we have unique expertise to help navigate:
- Insurance coverage and benefits investigation
- The prior authorization process
- Treatment logistics
- Options for injection training
- Ongoing education and support
We’ll be here for you from the very beginning.
Understanding your insurance coverage
We will guide you through the insurance process by helping explain your coverage and by partnering with your healthcare provider to assist in the case a prior authorization is needed.
If eligible, we may be able to help you begin treatment while you are waiting for an insurance coverage determination.
Injection training
After coordinating your first treatment shipment, we’ll work with you to set up one-on-one virtual or injection sessions with an ARCALYST Nurse Educator. They can provide guidance and training on the injection process.
We think your focus should be on your health, not treatment costs.
Kiniksa Copay Assistance Program
If you are eligible, the Kiniksa Copay Assistance Program may be able to help lower out-of-pocket costs related to receiving treatment, such as copays, coinsurance, and deductibles to as little as $10 per month.*
*To be eligible for the Kiniksa Copay Assistance Program, you must have commercial insurance, must not have Medicare, Medicaid, or other government insurance, and must meet other eligibility criteria. You also must agree to the rules set forth in the terms and conditions for the program. Please visit kiniksapolicies.com/copay to review additional eligibility criteria.
Patient Assistance Program
If you struggle with high out-of-pocket costs, do not have insurance, or find that your treatment is not covered by insurance, our Patient Assistance Program may be able to help. We will work with you to see if you qualify for this program and discuss any questions you may have.†
†To be eligible for the Kiniksa Patient Assistance Program, you must meet certain financial eligibility requirements. Please visit kiniksapolicies.com/pap to review additional eligibility criteria.
We’re always here for you.
Product shipping
We partner with select specialty pharmacies to provide refill reminders and deliver prescriptions directly to your door. These specialty pharmacies also provide access to items, such as sharps disposal containers to ensure that you have the necessary supplies for each injection.
Product support
We can assist you with product-related questions and provide access to educational tools and resources for additional information about your treatment.
Resources and support
Throughout your treatment journey, we will check in with you to make sure we’re staying up to date on your needs. If there are any changes in your situation (new insurance, a change of address, upcoming travel, etc.), we can help you find ways to avoid treatment interruptions. If you need additional information and support, we can also direct you to patient advocacy and support groups.
