Factor IX Tips

Rare Disease Day is an observance held on the last day of February to raise awareness for rare diseases and improve access to treatment and medical representation for individuals with rare diseases and their families.
Hemophilia is an inherited genetic disorder that impairs the body's ability to make blood clots because it lacks sufficient blood-clotting proteins (clotting factors).

Epidemiology
Hemophilia is rare, and it has only about one instance in every 10,000 births for hemophilia A and 1 in 50,000 births for hemophilia B. Hemophilia usually occur in males.

Types
There are two main types of hemophilia:

Hemophilia A: It occurs due to unavailability of enough clotting factor VIII.
Hemophilia B: It occurs due to unavailability of enough clotting factor IX.
Other types include:

Hemophilia C: It occurs due to unavailability of enough clotting factor XI.
Parahemophilia: It occurs due to unavailability of enough clotting factor V.
Acquired hemophilia: It is associated with cancers, autoimmune disorders, and pregnancy.
Causes
When a person bleeds, the body pools blood cells together to form a clot to stop the bleeding. The clotting process is encouraged by certain clotting factors.

Acquired hemophilia is a rare variant of the condition. It occurs when a person's immune system attacks clotting factors in the blood. It can be associated with the following factors:

Pregnancy
Autoimmune conditions
Cancer
Multiple sclerosis
Signs and Symptoms
It may vary, depending on the level of clotting factors. If the clotting factor level is mildly reduced, the patient may bleed only after surgery or trauma. If the deficiency is severe, the patient may experience spontaneous bleeding symptoms which include following signs and symptoms:

Unexplained and excessive bleeding from cuts or injuries, or after surgery, or dental work
Unusual bleeding after vaccinations
Blood in urine or stool
Nosebleed
Many large or deep bruises
Pain, swelling, or tightness in joints
In infants, there is presence of unexplained irritability
Bleeding into the brain leading to painful and prolonged headache, repeated vomiting, sleepiness or lethargy, double vision, sudden weakness or clumsiness, convulsions, or seizures

Complications
Deep internal bleeding
Damage to joints due to internal bleeding causing arthritis or destruction of the joint
Infection due to blood transfusions
In few people with hemophilia, the immune system has a negative reaction to the clotting factors used to treat bleeding. The immune system develops proteins (known as inhibitors) that inactivate the clotting factors, which make the treatment less effective

Diagnosis
Can be made with positive family history
A blood test can show a clotting-factor deficiency in children and adults
Some people come to know they have hemophilia after they bleed excessively during a surgical procedure

Treatment
Replacement of the specific clotting factor that body needs
Desmopressin (DDAVP): This hormone can stimulate your body to release more clotting factor
Clot-preserving medications (antifibrinolytic)
Fibrin sealants: Applied directly to wound sites to promote clotting and healing

When you give some of your blood for someone else, it is called blood donation. It usually helps people who have lost quite a bit of blood in accidents or suffer from diseases, such as cancer or hemophilia, etc.

Like all activities related to medical conditions, there are myths related to blood donation too and some of them are:

1. Myth: Because you're a vegetarian, you don't have enough iron in your blood; so your blood isn't useful as donated blood.
Fact: The fact is that vegetarians can donate blood and their blood has enough iron, just as much as non-vegetarians.

2. Myth: Infections, such as HIV can be contracted from donating blood.
Fact: This does not have a practical basis except for the fact that using contaminated needles might lead to contraction of the infections. However, usually fresh needles are used; therefore this never happens much.

3. Myth: Your health can deteriorate after you donate blood.
Fact: Your body produces new red blood cells as well as white blood cells after donation; hence your health doesn't deteriorate at all, except in cases where there is no rest taken at all after donation.



4. Myth: If you take medications, you cannot donate blood.
Fact: This is entirely dependent on your physician's advice. In the case of some medications, you need to halt them in order to donate blood. In most cases though, taking medications does not prevent you from donating blood.

5. Myth: You will be unable to partake in physical activities after donating blood.
Fact: Heavy lifting should be avoided. Apart from this, donating blood does not stop you from physical activities. You need to rest for a while, but there is nothing about blood donation putting a check on most physical activities.

6.Myth: People with more weight have more blood to donate.
Fact: This is an entirely baseless assumption. Overweight or obese people are often unhealthy, so donating more blood has nothing to do with weight. Hence, obese people do not have more blood in their bodies as compared to their thinner counterparts.

World Hemophilia Day is held annually on April 17 by the World Federation Of Hemophilia. It is an awareness day for haemophilia and other bleeding disorders, which also serves to raise funds and attract volunteers for the World Federation Of Hemophilia. It was started in 1989; April 17 was chosen in honour of Frank Schnabel's birthday.

Haemophilia is a type of disorder in which blood does not clots easily or in a normal way. Haemophilia A is one of the common types of disease which causes due to deficiency of blood clotting factor VIII. Haemophilia B is another type which is caused due to the deficiency of blood clotting factor IX. Haemophilia is a lifetime condition which has no exact cure but can be managed by treatment. Haemophilia is a hereditary disorder which is mostly seen in men but is also observed in women sometimes if they carry the gene.

Symptoms of Haemophilia
Symptoms of haemophilia can vary depending on the severity of the disease. In most cases, bleeding is a common symptom of haemophilia. Sometimes, haemophilia causes internal bleeding which if left untreated can cause joint pains. There are some other general symptoms seen in people suffering from haemophilia. They are listed here.

Bleeding through the nose
Bruising
Bleeding seen in faeces or urine
Continuous bleeding when removal of tooth, surgery and when injured
Unprompted bleeding
Bleeding seen in joints which lead to swelling and pain
Bleeding seen in urinary and gastrointestinal tracts
In some cases, internal bleeding happens in the brain which may come into notice during an injury. Such type of internal bleeding can be detected by the following symptoms.

A severe headache
Drowsiness
Neck pain
Severe stiffness in the body
Abrupt weakness
Problems in walking
Treatment for Haemophilia
The standard treatment of haemophilia involves mainly in replacement of missing clotting factor. As haemophilia is caused due to deficiency of factor VIII, the treatment involves the injection of factor VIII concentrates into the body which can be identified depending on the severity of bleeding, the site of bleeding and the age of the patient. Depending on the seriousness of the disease, factor VIII concentrates are given to the patient before going for surgery or dental extractions. This can help in preventing bleeding.

As haemophilia is a hereditary disease, it is advisable to concentrate on prevention rather than cure of the disease. In order to prevent the disease, it is wise to undergo genetic counselling.

Knee replacement is a surgery wherein an artificial joint is used to replace a diseased, damaged or worn out knee. This surgery is common among people who fall in the age group of 60-80, but recent trends seem to suggest that younger people are opting for this surgery as well. The lifetime of the artificial knee joint is around 20 years, provided the knee is well cared for.

Why do you need Knee Replacement?

Most common reason is high grade osteoarthritis due to wear and tear of the knee joint. The pre-hospital study of the Knee joint is mandatory and would decide what kind of Replacement is suitable to the patient. If there is diabetes or hypertension associated with this, then it should be controlled well before undergoing surgery. Hemoglobin of at least 10 gm% is required.

If the mobility in your knee joint is reduced leading to impaired functioning of the knee joint, then you might need a knee replacement surgery. You may experience pain while walking, sitting and, in some cases, resting as well.

Some of the common reasons why you may opt for this particular surgery are:

Gout, where, small crystals are formed inside the joint.
Rheumatoid Arthritis, an autoimmune disorder, wherein the immune system of the body attacks the body s healthy tissues.
Hemophilia, wherein, the blood ceases to clot normally.
Injuries to the knee
Disorders that cause unusual bone growth (bone dysplasias)
Death of bone in the knee joint following blood supply problems (avascular necrosis)
Knee deformity with pain and loss of cartilage
Unusual growth of bones in the knee joint
Knee replacement surgery is classified into:

1. Partial Knee Replacement: In this surgery, only one part of the joint is replaced.

2. Total Knee Replacement: Total knee replacement surgery involves replacement of both sides of the knee joint.

The usual hospital stay period is around 2-3 days after the surgery is completed. Initially, you will require the help of crutches to walk for at least 2 months. You may also be asked to do gentle knee strengthening exercises. It may take up to two years to recover completely from a knee replacement surgery.

When you give some of your blood for someone else, it is called blood donation. It usually helps people who have lost quite a bit of blood in accidents or suffer from diseases, such as cancer or hemophilia, etc.

Like all activities related to medical conditions, there are myths related to blood donation too and some of them are:

1. Myth: Because you're a vegetarian, you don't have enough iron in your blood; so your blood isn't useful as donated blood.
Fact: The fact is that vegetarians can donate blood and their blood has enough iron, just as much as non vegetarians.

2. Myth: Infections, such as HIV can be contracted from donating blood.
Fact: This does not have a practical basis except for the fact that using contaminated needles might lead to contraction of the infections. However, usually fresh needles are used; therefore this never happens much.

3. Myth: Your health can deteriorate after you donate blood.

Fact: Your body produces new red blood cells as well as white blood cells after donation; hence your health doesn't deteriorate at all, except in cases where there is no rest taken at all after donation.



4. Myth: If you take medications, you cannot donate blood.
Fact: This is entirely dependent on your physician's advice. In case of some medications, you need to halt them in order to donate blood. In most cases though, taking medications does not prevent you from donating blood.

5. Myth: You will be unable to partake in physical activities after donating blood.
Fact: Heavy lifting should be avoided. Apart from this, donating blood does not stop you from physical activities. You need to rest for a while, but there is nothing about blood donation putting a check on most physical activities.

6.Myth: People with more weight have more blood to donate.
Fact: This is an entirely baseless assumption. Overweight or obese people are often unhealthy, so donating more blood has nothing to do with weight. Hence, obese people do not have more blood in their bodies as compared to their thinner counterparts.