Hemophiliacs among us

CEBU, Philippines – Over a thousand people in the country have been confirmed to be hemophiliacs as of July 2012;       944 of them suffer from Hemophilia A, while the other 145 from Hemophilia B.

Dr. Lynda Mae Lepatan, a hematologist, shared this information in marking the first of a series of public dissemination seminars here in the city, together with barangay health workers, officers of groups involving the differently abled, nurses, midwives, the Cebu City Health Department officials, and members of the media.

Of the 1,159 documented cases, 109 are here in Region 7.

According to Dr. Lepatan, well coordinated efforts are needed to properly manage this often misunderstood genetic disorder.

"I have to admit that cases of misdiagnosis happen because of the lack of awareness on hemophilia even among doctors. In fact, there was once a case of hemophilia diagnosed as arthritis because of swollen knee joints," she said.

Hemophilia (also spelled as haemophilia after the Latin words "haemo" to mean blood and "philia" to mean "take care of") is life-long bleeding due to a disorder of the blood clotting mechanism. "It is the deficiency of a particular clotting factor resulting in excessive bleeding from injury or without obvious reason at all."

One cannot catch, contract, or acquire hemophilia in his or her lifetime. One is born with it. The reason for this is that a hemophiliac has little or almost no clotting factor.

This clotting factor, it was further learned, is a protein needed for normal blood clotting. Such proteins work with platelets to help the blood clot. When blood vessels are injured, clotting factors help platelets stick together to plug cuts and breaks at the site of the injury and stop bleeding. With deficiency in clotting factor, normal blood clotting can't take place.

Dr. Lepatan, in her presentation, pointed out that Hemophilia A patients are those with Factor VIII deficiency while those categorized as a B type have factor IX deficiency.

The normal activity of clotting factor (Factor VIII or IX) in the blood is 50 percent or much higher. In hemophilia, the clotting factor deficiency varies in three severities:

• Severe hemophilia (less than one percent). Patients are prone to spontaneous bleeds or they bleed for no clear reason of injury. Bleeds often occur into the muscles or joints (mainly elbows, knees, and ankles) and also occur after undergoing surgery. Recovery from bleeds is very slow.

• Moderate hemophilia (1% to 5%). Bleeds are usually caused by injury or after undergoing surgery. Bleeds might happen about once a month. Recovery from bleeds is slow.

• Mild hemophilia (5% to 30%). Bleeds are only likely to happen when badly injured or after undergoing surgery; does not bleed often or might never have a bleeding problem. Recovery from bleeds is still slower than the normal clotting.

Bleeding can happen inside, outside the body

According to the Haemophilia Association of the Philippines Inc., the most common symptom of hemophilia is bleeding in the joints and muscles. Symptoms include pain, swelling, and limited movement especially the elbows, knees, and ankles.

Bleeding outside the body is seen after getting a cut, tooth extraction, or surgery. Patients with hemophilia are not advised to undergo surgery as they cause bleeding for a long period of time.

First aid

Dr. Lepatan mentioned the RICE mnemonics to remind caregivers that "whether or not treatment is available," administration of RICE is advised:

• Rest. Most injuries should be rested for a while.

• Ice. Ice pack or cold compress can alleviate the pain and can aid in healing bruises and swelling.

• Compress. Use a splint or bandage to keep the joint still. It is advised never to wrap the joint too tightly.

• Elevate. Lift the joint or muscle higher than the chest level and rest in on something soft.

Sad thing is that there is no known cure yet for hemophilia. However, good nutrition and a healthy lifestyle can help cope with hemophilia and also help avoid bleeds.

• Regular exercise. A regular exercise program guided by a physiotherapist will ensure patients of strong joints, muscle strength and flexibility. Maintain physical fitness to prevent bleeding. Swimming is especially recommended. Contact sports like basketball, soccer, and boxing are contraindicated because of the danger of injury. Consulting a physician for an appropriate exercise program is advised.

• Dental care. It is important to take good care of teeth to prevent problems and complications. Tooth extractions and dental surgery can cause a major bleeding.

• Aspirin and other pain relievers. Do not take pain relievers that contain ASA (acetylsalicylic acid) or Aspirin as they can cause more bleeding. NSAIDS (Aleve, Motrin, Ibuprofen) also decrease the blood's ability to clot. Pain relievers also merely alleviate the pain; they do not help increase the clotting factor activity for bleeds to heal.

• Intake of foods high in uric acid. Limit the intake of foods such as nuts, soy, tofu and preservatives as they have high uric acid content. They can cause joint pains. But since uric acid is also found in meat and other common food groups as well, it is advisable to regulate the uric acid level by eating lots of raw fruits and vegetables.