Management of Hemophilia in Older Adults

Introduction-

Hemophilia is a problem in which a person bleeds longer than a person without hemophilia. The blood travels through the body in tubes called arteries, veins, and capillaries. Normally when there is a tear or hole in one of these, bleeding starts, and blood cells called platelets gather at the opening, and the clotting factor of the blood holds the platelets together to form a clot that stops the bleeding. When a person has hemophilia, they lack this clotting factor and the ability to form a clot which makes them bleed longer than expected.

What Are the Types of Hemophilia?

There are thirteen clotting factors that work with the platelets to stop bleeding at the site of injury. The most common type is Hemophilia A, and it occurs as a result of a deficiency in clotting factor 8. Hemophilia B occurs when there is a deficiency of clotting 9. It is also called Christmas disease. Other types of hemophilia result from a deficiency in clotting factor 11, and parahemophilia is due to a deficiency in clotting factor 10. In very rare cases, hemophilia can develop after birth, and that is called acquired hemophilia. Hemophilia is also associated with cancers, autoimmune disorders, and pregnancy.

How Does Hemophilia Affect Men More Than Women?

Because of the genetics involved in the way the sex of a child is determined, men are more vulnerable to hemophilia than women. Everyone inherits two sex chromosomes, one from each parent. Females inherit two X chromosomes, one from each parent, and males have an X chromosome from the mother and a Y chromosome from the father. Hemophilia is caused by a defect in the X chromosome. If a girl is born with one defective X chromosome, her other X chromosome can compensate for it, and she will be the carrier of hemophilia but will not suffer from the condition herself. If both her X chromosomes are defective, she will suffer from hemophilia. On the other hand, if a boy is born with a defective X chromosome, he does not have the second X chromosome to compensate for it and will suffer from hemophilia.

What Are the Signs and Symptoms?

Hemophilia can also result in internal bleeding in the knees, ankles, and elbows, which can damage organs and tissues and may have life-threatening complications. This bleeding can be seriously devastating if it occurs within the brain as it results in headaches, seizures, decreased level of consciousness, lethargy, vomiting, double vision, and paralysis.

The level of clotting factor determines the severity of symptoms, which include-

• Many large bruises.

• Blood in urine or stools.

• Excessive and unexplained bleeding from cuts or injuries and after surgery or dental procedures.

• Unusual bleeding after vaccinations.

• Unusual nosebleed without a known cause.

• Pain or swelling around the joint.

How Is Hemophilia Diagnosed?

• On suspicion, the physician will ask about family history to help identify the cause.

• A blood test may give information about the level of the clotting factor to check if the clotting factor is missing and the time it takes for the blood to clot.

• Pregnant women who are carriers may have the fetus tested for the condition after ten weeks of pregnancy.

What Are the Treatment Options?

The main treatment is replacement therapy. This therapy focuses on the replacement of the specific clotting factor that is missing and is given intravenously. This treatment can be derived from human blood or synthetic blood produced in a lab. Synthetically produced factors are recombinant clotting factors and are considered the treatment of choice as they reduce the risk of transmitting infection carried in human beings. Other treatments may include-

• Desmopressin, also called man-made vasopressin (hormone in the body that balances salt and water).

• Antifibrinolytics (medicines that prevent excessive bleeding).

• Fibrin sealants.

• Physical therapy.

How to Manage Hemophilia in Older Adults?

The older hemophilia population is more at risk as they are associated with other medical conditions such as diabetes, hypertension, etc., and they are more susceptible to chronic joint arthropathy (joint disease associated with blood disorder) that increases the chances of falls and fractures, and other complications that are related to hepatitis and HIV.

• Prophylaxis is considered the standard of care for severe hemophilia, but patients with mild or moderate hemophilia may not be on prophylaxis. These patients are treated on demand or when a bleed occurs.

• Participation in physical activities is recommended, which improves self-esteem and decreases muscle and joint bleeding.

• Non-contact sports such as swimming, biking, golf, and tennis are recommended.

• There are comprehensive hemophilia treatment centers available where an older person can seek care.

• Avoid the use of non-steroidal anti-inflammatory drugs (NSAIDs) like Aspirin. An alternative should be opted after consultation with a physician.

• Pain management with cyclooxygenase (COX-2) inhibitors.

• Regular checkups with physiotherapists specializing in hemophilia.

• Educating the patient and creating awareness about the disease.

• Managing comorbidities of the patient.

• Patients with hematuria (blood in urine) should be referred to a urologist.

• Screening for other conditions like mobility, impaired vision or hearing, and depression.

• Hemophilia is also associated with low mineral bone density. About two-thirds of older patients with moderate to severe hemophilia have osteoporosis, so a proper evaluation of joint diseases is important, and the use of vitamin D supplementation can improve musculoskeletal health.

• Avoid the use of alcohol and smoking.

• Managing hypertension as it increases the risk of intracranial (within the skull) bleeding.

• Hemophilic patients with obesity suffer from delayed healing, especially in the case of orthopedic surgery, so weight management is crucial.

• Counseling older individuals as they are more likely to isolate themselves in a condition like hemophilia, which leads to depression.

• Engaging them in social activities that include interacting with other hemophilic patients can help them overcome the fear associated with this disease.

Conclusion-

Older hemophilic patients have a higher chance of morbidity than the general population as they pose more risk for age-related diseases. Hence they should be kept a priority while receiving treatment and should be encouraged to live with a positive attitude toward life by adopting a holistic approach and comprehensive care available.