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Hemophilia is a bleeding disorder that prevents blood from clotting normally due to a lack of blood-clotting proteins. Patients may bleed longer after an injury. They may also require special care to prevent more serious complications.
About 70% of hemophilia cases are inherited. This means a parent who either has hemophilia or carries the gene passed it on to the child. In the remaining 30%, hemophilia occurs where there is no family history of it. This is usually associated with pregnancy, autoimmune conditions, cancer, multiple sclerosis, and/or drug reactions.
The most common types of inherited bleeding disorders are hemophilia A, hemophilia B, and Von Willebrand disease. Patients with hemophilia A lack sufficient levels of clotting factor VIII, and patients with hemophilia B lack sufficient levels of factor IX.
Symptoms depend on how much clotting factor is missing from the individual’s blood. Those with little to no clotting factor experience frequent, prolonged bleeding. These episodes may occur as often as twice a week and require clotting factor infusions. Those with a higher percentage of clotting factor may not experience any symptoms until a major injury occurs or they undergo a surgical procedure.
Treating hemophilia requires increasing or replacing factor levels. Prolonged bleeding episodes are treated with infusions to replace and enable blood to clot more effectively. Other therapies may also help the body produce more of the missing factor.
Those with severe hemophilia may prevent bleeding or reduce their severity through routine prophylactic infusions. Infusions can be received in a specialized treatment center, through a home-nurse visit, or by self-infusing. Many of our patients self-infuse for the convenience and freedom it provides.
Most cases of hemophilia are genetic or inherited (passed down from parent to child). The “hemophilia gene” or genetic mutation responsible for clotting factor deficiencies is found on the X chromosome, making hemophilia an X-linked disorder. Males, who have just one X chromosome, are much more likely to inherit hemophilia than females, who have two X chromosomes.
As an X-linked disorder, hemophilia is only inherited if all X chromosomes have the genetic mutation. For a male, who only has one X chromosome, the chance of inheriting hemophilia is 50% if his mother has the defective gene. For a female to inherit hemophilia, she must receive the hemophilia gene from her mother and her father must also have the disease.
A female with the hemophilia genetic mutation is called a carrier. While a carrier does not have hemophilia, her male children will have a 50% chance of inheriting hemophilia and her female children will have a 50% chance of becoming carriers themselves.
Sometimes hemophilia occurs spontaneously, meaning it is not passed down from parent to child. When this happens, hemophilia is classified as an autoimmune disorder where the body mistakenly attacks clotting factors.
Hemophilia is diagnosed from blood samples that measure the level of clotting factors present in the blood. Those with low levels of clotting factor VIII are diagnosed with hemophilia A. Those with low levels of clotting factor IX are diagnosed with hemophilia B.
The severity is linked to the amount of clotting factor missing from the blood. Normal factor levels range from 50%-150% (measured in international units per milliliter of blood, 0.50-1.5 IU/ml). Those with mild hemophilia have a factor level between 5%-40% (0.05-0.40 IU/ml). Individuals with moderate hemophilia have a factor level between 1%-5% (0.01- 0.05 IU/ml). Those with severe hemophilia have a factor level of less than 1% (0.01 IU/ml).
People with moderate to severe hemophilia are usually diagnosed as infants or young children. Patients with mild hemophilia may not be diagnosed until they undergo a dental or surgical procedure that results in prolonged bleeding.
Pregnant women with hemophilia or who are carriers usually undergo screening to determine if their unborn child has the disease. Prenatal testing can be performed between 11-14 weeks through chorionic villus sampling, or between 15-20 weeks by obtaining a fetal blood sample.
Hemophilia impacts every aspect of a patient’s life. We offer a holistic approach to treatment and disease management. We provide the right solutions for meeting its challenges. A patient’s treatment team includes skilled and educated medical staff. This includes hematologists, physical therapists, dentists, and health professionals like the Fidelis Specialty Pharmacy staff. We assist with health insurance matters and obtaining treatment.
Von Willebrand (VW) disease is a bleeding disorder which occurs when the VW factor is either deficient, abnormal, or absent. VW factor is a protein that helps blood to clot. This is vital for bleeding to stop. VW disease is the most common bleeding disorder. In most cases, it is genetic and passed on from parent to child.
VW occurs equally in males and females but can be more severe in women due to bleeding from menstruation and childbirth.
VON WILLEBRAND DISEASE TYPES
There are three types of von Willebrand disease: 1, 2, and 3. Knowing which type a patient has can assist in obtaining appropriate and effective treatment.
Type 1 is the most common type. People with Type 1 have lower than normal levels of VW factor.
People with Type 2 have enough VW factor, but it doesn’t function properly.
Type 3 is the rarest and most severe type of VW disease. Patients have very little or no VW factor in their blood. They also have low levels of factor VIII, the clotting factor missing in hemophilia A.
Symptoms vary greatly from person to person. Some individuals have no symptoms at all. Others may have moderate to very severe bleeding complications. When symptoms first appear they also vary from infancy to adulthood. Symptoms may include persistent and frequent nosebleeds or blood in the urine or stool. Additional symptoms include easy bruising, excessive bleeding from an injury, surgery, or minor cut, and bleeding from the gums. For women, symptoms can include a heavy period, the presence of large blood clots, and/or longer than normal bleeding after childbirth. Anemia, fatigue, and shortness of breath are other possible symptoms of VW disease.
Anyone with the above symptoms should go to the doctor immediately. In addition to exploring the family’s medical history, the doctor will perform a physical exam and have blood drawn for laboratory analysis to determine the level and efficacy of the VW factor.
While an individual may not experience symptoms, the faulty gene can still be passed on to children. A child whose parent is a carrier for VW should be checked for the disease. Early diagnosis is vital for a healthy, active, and normal life.
Treatment focuses on stopping or preventing bleeding episodes. This is done by using medications that assist with blood clotting. Depending on the diagnosis, treatments may be administered orally, through injections, by nasal spray, or applied topically. In mild cases, medication may only be required for surgery or dental work.
Common von Willebrand disease treatments include:
VW factor production stimulation
- Desmopressin (DDAVP, Stimate)
Typically used in Type 1 mild to moderate VW disease
Coagulation factors to replace low or missing VW factor
- Recombinant von Willebrand factor (Vonvendi)
- Antihemophilic factor/von Willebrand factor complex (Alphanate, Humate P, Wilate)
Antifibrinolytics to stabilize clots
- Aminocaproic acid (Amicar)
- Tranexamic acid (Cyklokapron, Lysteda)
Contraceptives/estrogen to reduce menstrual bleeding severity
- The estrogen that is contained in birth control pills can increase VW factor and factor VIII activity can also help control bleeding that occurs with menstrual bleeding.
Topical treatments for external injuries
- Fibrin sealant (Tisseel VHSD)
Actively managing symptoms leads to better outcomes. This includes following treatment plans laid out by doctors or specialists, taking medications consistently, and obtaining infusions when needed.
Although there are many developments in gene therapy, there is still no cure for hemophilia. With the right care and support, patients can lead long and healthy lives. From preventative treatments to replacement therapy, our team of experienced pharmacists and registered nurses can create a customized care plan.
We are here to help 24 hours a day. Call the Fidelis team at (866) 643-2042 to discuss the best course of action for your family.
Regular physical activity offers great benefits to bleeding disorder patients. In a study by Ruth Mulvany, PT, DPT, MS, 20 bleeding disorder patients between 7 and 57 years old took part in a supervised exercise program for six weeks. Before the study, 65% of participants reported participating in minimal exercise and 50% indicated a fear of exercise-induced bleeding, pain or physical impairment. During the study, each patient participated in an active program with strength, flexibility, and cardiovascular training. Only positive changes resulted. No exercise-induced injuries, pain, edema, or bleeding episodes were reported. Significant improvements occurred in joint motion, strength, and distance walked in six minutes. The greatest gains were among the individuals with the most severe joint damage and coexisting illness. The benefits of moving your body through exercise are worth considering! “In certain cases, exercise can be as effective as antidepressants when treating depression,” shares Lawrence Henry, Pharm. D, BCACP, CSP Pharmacist at Fidelis Specialty Pharmacy.
- Anxiety and depression
- Weight gain
- Cardiovascular fitness
- Muscular fitness
- Bone health
- Body composition/ healthy weight
When it comes to bleeding disorders and sports, there is no standard rule of thumb. Every activity is different. Decide what is best for you based upon your doctor’s advice. Weigh the potential risk of the activity with your desire to participate. As you get older, some sports become more physical and riskier. For example, soccer and basketball are often safer for young children because there is less physical contact. But these sports can result in serious injury in teens and adults.
Learning to self-infuse clotting factor is an important milestone. This can be an important step for the child’s independence, and a big relief to caregivers.
Most experts agree that by age nine a child may be ready. Some kids may be eager to start, while others may need more time. The key is creating consistent and clear communication with your healthcare team. At Fidelis, we create a dialogue with the child, their parents, nurse, social worker, and hematologist. Together, we can map out a plan for success.
Here are some things to consider that may indicate that your child may be ready:
- Do they have a chore that they manage without help?
- Can they use a calendar and logbook? Do they understand when their next infusion will be?
- Do they like to set goals and achieve them?
- Do they stay calm during an entire infusion?
- Do they know the name and severity of their bleeding disorder? Do they know their medication names, doses, and when to take them?
- Can they name all the supplies needed? Including the syringe, factor, gloves, tourniquet, sharps container, cotton swabs?
- Do they understand the treatment’s product label? This includes the medicine’s name, dose, and expiration date.
- Do they help to prepare the equipment and clean the skin?
- Can they mix their factor correctly on their own?
- Can they find a vein easily?
- Are they pushing the infusion and marking it in a log?
- Can they troubleshoot if something goes wrong?
For more information on self-infusion, visit www.hemaware.org or call the Fidelis team to discuss the best course of action for your family.
The primary symptom is excessive external or internal bleeding. This may include frequent nosebleeds, heavy bleeding from an injury, bleeding from dental or surgical procedures, and unusual bleeding after a vaccination.
Symptoms of internal bleeding can include blood in the urine or stool, large bruises, joint pain, and/or swelling. In cases of severe hemophilia, the brain may bleed spontaneously, resulting in symptoms such as double vision, fatigue, vomiting, and prolonged headache.
People with factor levels ranging from 5%-40% (0.05-0.40 IU/ml) often do not experience symptoms. Mild hemophilia accounts for about 25% of people who have the disease.
Those with factor levels between 1% and 5% (0.01-0.05 IU/ml). Roughly 15% of people with hemophilia experience moderate symptoms.
These patients have blood with less than 1% clotting factor activity (0.01 IU/ml). These patients can experience spontaneous, frequent bleeding episodes of 1-2 times a week.
A proactive approach is best for long-term health. By making healthy choices and carefully adhering to treatment plans, patients will enjoy a better quality of life.
Learn more about how the services provided by Fidelis Specialty Pharmacy support hemophilia patients to help them achieve the best possible treatment.
Uncontrolled bleeding can cause severe damage. These guidelines can help avoid potential bleeding episodes and ensure proper treatment.
GENERAL SAFETY GUIDELINES
- Wear protective gear before riding a bicycle, skating, etc.
- Keep a seatbelt on during car rides.
- Wear a medical bracelet.
- Keep emergency contact information easily accessible.
- Follow your prescribed treatment plan.
- Maintain a prophylactic infusion schedule.
- Have enough factor on hand to last for at least one week.
- Properly storing and rotating your factor helps ensure that the factor you have at home does not expire before you have a chance to use it.
Emergencies can happen despite taking safety precautions. Preparing for an emergency is important for timely intervention.
- Know the symptoms of spontaneous bleeding episodes and what to do.
- Seek medical attention for head or neck trauma.
- Have emergency procedures on hand.
By managing symptoms, taking preventive treatments, and making healthy lifestyle choices, patients can live long and full lives. With comprehensive approaches to treatment, prevention, and self-infusion, patients have more freedom and independence than ever before. Learn more about our treatment approach and the benefits of home infusion and self-infusion.
Community groups offer support and encouragement for patients and their loved ones. Fidelis proudly partners with “Powering Through.” This organization hosts speakers to address how to overcome living with life’s challenges. We also support “Hope for Hemophilia,” a philanthropic organization that provides assistance for patients in need.
The two most common types of inherited hemophilia are hemophilia A and hemophilia B. The type is determined by which blood clotting factor a person is missing or is deficient in. Hemophilia A patients have low levels of clotting factor VIII and hemophilia B have low levels of clotting factor IX.
BLOOD CLOTTING FACTORS
Blood clotting factors are proteins that stop bleeding. After a blood vessel is damaged, the body releases chemical signals to aid in forming a clot using clotting factors to control and stop the bleeding.
The level of a clotting factor deficiency determines how severe a patient’s symptoms are and what treatment regimen is needed. Those with a relatively mild clotting factor deficiency may only need treatment when undergoing dental or surgical procedures. Those with blood with very low levels of clotting factor may need regular infusions and other treatment to prevent severe hemophilia complications.
The most common type of hemophilia. Patients have low levels of factor VIII. Hemophilia A occurs in approximately 1 in 5,000 live births and affects mostly males. Roughly 20,000 people in the United States have hemophilia A.
Also known as Christmas disease. This occurs when levels of clotting factor IX are absent or deficient. Considerably rarer, this occurs in about 1 in 30,000 live male births and is about one fifth as common as hemophilia A.
Typically, a milder version and often affects individuals of Jewish Ashkenazi descent. Hemophilia C occurs in both sexes.
Treatment depends upon the type and severity of the disease. Individuals with mild hemophilia may only need treatment before a surgical or dental procedure. Those who have severe hemophilia may require routine infusions.
For individuals with missing or low levels of a particular clotting factor, treatment involves replacing the factor intravenously as needed to stop a bleeding episode in progress. They may also require routine factor infusions to prevent bleeding episodes from occurring.
Hemophilia treatment plans can be therapeutic to treat current symptoms or prophylactic to prevent symptoms from developing.
PROPHYLACTIC HEMOPHILIA TREATMENTS
Routine clotting replacement treatments are often recommended for those with severe hemophilia to prevent bleeding episodes. Replacement treatments are performed either at treatment centers or at home using self-infusion.
ADDITIONAL TREATMENT OPTIONS
Other treatment options that may help manage symptoms and prevent bleeding episodes may include:
- Hormone drugs to stimulate clotting factor production
- Antifibrinolytics to keep already-formed clots from breaking down
- Topical treatments to promote clotting
- Physical therapy for joints damaged by internal bleeding
Patients who manage their hemophilia proactively by maintaining a healthy lifestyle and obtaining preventative treatments have a better long-term prognosis than those who don’t. To learn more about which hemophilia treatment options are right for you, including self-infusion and home infusion, we invite you to speak with a patient support specialist at Fidelis.
Please contact us if you have any questions, concerns or errors concerning order status, copay amount, claims submissions, benefit coverage, and to access the Patient Management Program. If you have any adverse effects to the medication you were given, please contact your prescribing physician or your pharmacist.
You can recognize an adverse effect as undesirable reaction to a medication that may or may not be expected.
Fidelis Specialty Pharmacy
Phone: Las Vegas, NV (866) 643-3547 and Fax: (702) 815-0801
Phone: Arcadia, CA (866) 643-2042 and Fax:(626) 447-6433
Email: email@example.com (Usual response time is next business day)
NEVADA: 5275 Arville St, Suite 156, Las Vegas, NV 89118
CALIFORNIA: 1002 S Baldwin Ave, Arcadia, CA 91007
These telephone numbers are intended for all communications, including emergency medication access during natural or man-made disasters.
(Fidelis Specialty Pharmacy is closed door only and does not accept walk-ins.)
A Fidelis Specialty Pharmacy employee is available to answer any questions you may have, either during normal business hours in person at our locations, or through 24-hour Pharmacist-on-Call support.
Business Hours of Operation:
- Las Vegas, NV: Monday through Friday, 8:30am to 6:00pm PST
- Arcadia, CA: Monday through Friday, 8:30am to 5:00pm PST
- 24-hour coverage at (866) 643-3547 (LV) or (866) 643-2042 (CA)
- A Pharmacist-on-Call is available 24 hours a day, 7 days a week for any question you may have. Pharmacists are available for emergency and clinical situations such as side effects and medication assistance, as well as complaint resolution. If you are having a medical emergency, please call 911.
They also have access to all pharmacy systems and can answer any questions regarding order and copay status, claims submissions and benefit coverage. In addition, Pharmacists have access to Fidelis Specialty Pharmacy facility if patient need would require support from the systems and/or prescription fulfillment process.
- Your prescriber must send a valid prescription to our pharmacy via their preferred means. When a valid prescription is on file, you may call into our pharmacy to place your order and set up a shipment during regular business hours.
- During business hours, you can call the pharmacy to speak with a staff member.
- After hours, you can follow the prompts to request a refill by leaving a message for the pharmacy staff, requesting medication refill. Please include in your message: medication name, your first and last name, address, date of birth, daytime phone number, and additional requested information by the answering service.
- Your doctor can e-prescribe or fax a new prescription to us. Please note, certain controlled substance medications cannot be faxed.
- Your prescription may be filled with a generic equivalent substitution based on state law, equivalency rating, and in accordance with company policy. Please ask a pharmacist if you have any questions or concerns.
*If a different generic from what was previously dispensed is required by your insurance, or due to vendor availability issues, the pharmacist will attach a sticker to your prescription bottle or container stating “This is the same medication you have been getting. Color, size, or shape may appear different.”
- A Fidelis Specialty Pharmacy employee will let you know if Fidelis Specialty Pharmacy is unable to fulfill the medication request. A Fidelis Specialty Pharmacy employee will reach out to either you and/or your physician by phone to provide guidance on how you may access the medication. This may result in your prescription being transferred to another pharmacy who may be able to supply the medication.
- Our standard processing time at Fidelis Specialty Pharmacy is normally 24 to 48 hours. This does not include delivery time. If processing time is delayed longer than 24 hours, we will contact you to notify you of your options, so you don’t go without medication.
- A Fidelis Specialty Pharmacy employee will immediately let you know if there are any issues that may delay fulfillment, such as prior authorizations or quantity limits imposed by your insurance company. Fidelis Specialty Pharmacy employees will work with you and your physician to try and get any Prior Authorizations completed as quickly as possible. If your insurance company will not allow a quantity override due to unforeseen circumstances, a Fidelis Specialty Pharmacy employee will help determine the best way to get the medication you require.
- If shipped medications are sent via FedEx, Priority Overnight is available or required for some medications.
- Prescriptions are shipped Monday through Friday for next-day delivery, including Saturday if permitted by the delivery zip code. Medication delivery is a complimentary service at no additional charge to you. All medications will require your signature for delivery. A Fidelis Specialty Pharmacy staff member will coordinate with you to schedule the most convenient delivery time to ensure your availability to sign for the prescription.
- Fidelis Specialty Pharmacy will call to schedule your refill order 7 to 10 days before you should run out of medication.
- If you run out prior to Fidelis Specialty Pharmacy contacting you, or you would like to go ahead and order your refill, please contact us. Please have your prescription number(s) available to place your order.
- Please immediately let a Fidelis Specialty Pharmacy employee know if you need a vacation supply, or run out of refills and/or medication and would like us to call your physician for a new prescription.
- Please remember to always inform Fidelis Specialty Pharmacy and billing staff of any insurance, address, or health changes.
- If you need your prescription immediately, please let a Fidelis Specialty Pharmacy employee know so your order can be expedited. If you cannot wait for a shipment, you may ask about having your prescription transferred to a local pharmacy. The prescription can be transferred back to Fidelis Specialty Pharmacy the next time it is needed.
- Prescription costs will vary depending on your insurance.
- Because drug pricing can change daily, a final determination of your co-pay cost cannot be made until your claim is processed. You may also call the Member Services phone number on your prescription insurance card to get the most current information.
- If you are unable to afford the out-of-pocket cost for your prescription, Fidelis Specialty Pharmacy will work to identify co-pay card assistance, patient assistance programs, or other support and/or charitable organizations.
- The cost may also vary depending on the quantity of medication. Your prescription will be filled for the amount of medication that the physician prescribes. Please be sure to advise your physician to prescribe for the maximum amount/days’ supply allowable by your insurance coverage (days allowed may vary by plan). Fidelis Specialty Pharmacy will provide you with written information (either through delivery or mail) regarding deductibles, co-pays, co-insurance, in-network, and out-of-network costs.
- Reimbursement staff can assist you in determining and understanding your options. The staff can also let you know if Fidelis Specialty Pharmacy is in or out of your health benefit plan’s network. They can also show you how this might affect your cost.
- Fidelis Specialty Pharmacy accepts all major credit cards, checks, or money orders. If mailing payment, please do not mail cash.
- Visit the following website to view a list of medications that can safely be flushed down the toilet, or see the handouts given in the Welcome Packet: https://www.fda.gov/drugs/safe-disposal-medicines/disposal-unused-medicines-what-you-should-know
- If your medication is not on this list, please see the handout included in your Welcome Packet on how to properly dispose of your unwanted or expired medications.
- You will be notiﬁed by a Fidelis Specialty Pharmacy employee if there is a recall on your medication, and you’ll be given instructions on what to do.
- The Patient Management Program is included at no cost to you, and you are automatically enrolled as a patient of Fidelis Specialty Pharmacy. You may opt-out at any time.
- Pharmacists will work with you on any problems, concerns, or questions you may have regarding your medication therapy. Issues discussed include disease overview, medication, dose, dose frequency, interactions, side effects, physical assessments, and coordination of care with your physician when appropriate, etc.
- The potential health beneﬁts of this program include managing side eﬀects, improved overall health, increased disease and medication education and awareness, increased medication compliance, and when coordination of care with your physician is necessary, your pharmacist will have all the information needed to help make informed decisions regarding what is best for you as the patient.
- The potential limitations of this program are dependent on you as the patient. You must be willing to follow the directions of your physician and pharmacist, be compliant with taking your medication, and willing to discuss the details of your disease, medical history, and current practices with your pharmacist so he/she can have a full understanding of the situation.
- Please let your physician know you are a patient of Fidelis Specialty Pharmacy and are enrolled in their Patient Management Program. A good relationship between your physician and your pharmacist will benefit everyone involved in your care.
- To contact the Patient Management Program, please call Fidelis Specialty Pharmacy.
Fidelis Specialty Pharmacy provides each patient with a patient drug monograph to supply each patient with evidence-based health information provided by the manufacturer. Other information about common conditions, diagnosis, treatment diagnostics, and interventions are available on the Center for Disease Control (CDC)’s website, here.
Hemophilia Foundation of Northern California:
6400 Hollis Street, Ste 6, Emeryville, CA 94608
Phone: (510) 658-3324
Fax: (510) 658-3384
Hemophilia Foundation of Southern California:
959 East Walnut Street, Suite 114, Pasadena, CA 91106
Phone: (626) 765-6656
Fax: (626) 765-6657
Nevada Chapter of the Hemophilia Foundation:
222 S. Rainbow Blvd, Suite 203, Las Vegas, NV 89145
Phone: (702) 564-4368
Fax: (702) 446-8134
National Hemophilia Foundation:
7 Penn Plaza, Suite 1204, New York, NY 10001
Phone: (212) 328-3700
Fax: (212) 328-3777
Fidelis Specialty Pharmacy trains consumer facing staff such as customer service representatives and/or marketing on all aspects of patient related services.
We're Ready to Hear More
We understand that challenges faced by patients with chronic disorders can occur at any time. Our experts are ready to assist you 24 hours a day, 365 days a year. Our specially trained pharmacists and nurses are experts on your condition and can give personalized counseling and answer questions about your specialty medications and treatment plan.
A Fidelis Specialty Pharmacy employee is available to answer any questions you may have either during normal business hours or through 24-hour Pharmacist-on-Call support.
Fidelis Specialty Pharmacy
1002 S. Baldwin Avenue
Arcadia, CA 91007
Phone: (866) 643-2042
Fax: (626) 447-6433
Fidelis Specialty Pharmacy
5275 Arville Street, Suite 156
Las Vegas, NV 89118
Phone: (866) 643-3547
Fax: (702) 815-0801