Hereditary Angioedema

Hereditary Angioedema is a rare genetic condition that causes episodes of edema (swelling) in various parts of the body intermittently. The main target areas are usually limbs, throat, lips, eyelids and abdomen. These attacks may or may not occur frequently. But if left untreated, it can be life-threatening. However, the patient feels completely normal in between the attacks. This condition occurs as result of either deficiency or improper function of protein C1 esterase inhibitor (C1-INH) in plasma. C1 esterase inhibitor is responsible for regulating the production of a hormone called bradykinin. Higher amount of bradykinin is produced in the body when protein C1 esterase inhibitor does not function normally. Increased bradykinin hormone levels leak the fluid from blood vessels into the body tissues resulting in edema.

There are three different types of Hereditary Angioedema.

In Type I HAE, blood contains low level of C1 esterase leading to excess production of bradykinin, resulting in swelling attack.

With Type II HAE, blood contains normal levels of protein C1 esterase inhibitor but fails to function normally leading to to inflammation and swelling episodes.

Type III HAE is very rare, and isn't associated with functioning of C1 esterase inhibitor.

HAE is caused due to the mutations inherited from a parent. The gene is dominant, which means one abnormal copy is enough to cause HAE. However, one fourth of the Hereditary angioedema cases aren't inherited. They occur from spontaneous mutation that takes place at conception.

Symptoms of Hereditary Angioedema

Most often Hereditary Angioedema manifests into cutaneous swelling, on the face, hands and extremities. Though not common, it also causes abdominal swelling and the swelling of airway passage in few cases. The disease may become potentially life-threatening when upper respiratory system of the patient is attacked - asphyxiation. Early suspicion, recognition and treatment of this condition are important.

Trigger factors

Certain triggers have been identified that leads to the episodes of swelling. Try and identify your triggers - certain foods, environmental factors, medications, anxiety or certain physical activities. They include physical injury, medical or dental procedures, psychological stress, menstruation, pregnancy, infections and certain medications such as exogenous estrogen (OCP) and angiotensin-converting enzyme inhibitors. Some patients do take preventive medication, after consulting their doctor. After a bout of HAE, take rest and hydrate yourself. Log your attacks.

Diagnosis and treatment

Since edema can indicate host of other conditions, it is difficult to diagnose HAE with clinical symptoms alone. Taking note of family history of swelling attacks and the frequency of attacks in the patient himself should be the starting point to diagnosis. Further, laboratory analysis of blood samples for C1, C2 and C4 levels along with genetic testing is required to confirm Hereditary Angioedema.

Currently there is no cure for Hereditary angioedema. However medicines are available for treating patients with acute HAE attacks to address the symptoms during attacks. HAE is also treated with prophylactic therapy (preventative HAE treatment).

Tranexamic Acid

Tranexamic acid is used in the control of heavy bleeding or menorrhagia during periods. Since, this acid helps to stop blood clots from breaking down, bleeding is reduced. The lining in the womb clots and thus when you are having a period, it will reduce the heavy bleeding, though it will not stop the period altogether. By controlling heavy bleeding, it reduces the amount of blood loss with minor side effects. Tranexamic acid is an Antifibrinolytic medicine and is also called Cyklokapron which is available both in tablets and injection form.

Normally, when you bleed, your body forms clots to stop bleeding. In some, these blood clots break down and the bleeding continues and it is here that Tranexamic acid comes into play to stop the clots breaking down and thus reduce unwanted bleeding.

Conditions in which Tranexamic acid is used

Apart from heavy bleeding during periods or menorrhagia, Tranexamic acid is used in conditions such as unwanted or heavy bleeding post surgery, such as that on the prostrate, bladder and cervix, nosebleeds, bleeding inside the eye, during tooth extraction when bleeding is more than normal, and in conditions like Hemophilia and hereditary angioedema.

Precautions before consuming Tranexamic acid

Not all medicines suit all persons and with certain conditions, sometimes a medicine may only be used if extra precaution is taken. It is imperative to inform the medical practitioners before starting on Tranexamic acid:

• If you are pregnant or breast-feeding or planning to become pregnant.
  
• If there are problems in kidneys and blood in urine.
  
• If there has been a recent blood clotting problem.
  
• If you have had fits
• If you are on any medication, herbal medicines or contraceptive pill.
  
• If the time between the start of periods is less than 21 days or more than 35 days.

Use of Tranexamic acid is not advised if:

• You are allergic to any ingredient in Tranexamic acid
  
• If there are blood clots in the leg, lung, eye, brain and a history of blood clots or conditions that risk blood clots such as heart valve problems, and irregular heartbeat etc.
  
• If there is bleeding in the brain, blood in urine or bleeding related to kidney problems
  
• Disturbance in color vision
  
• Irregular menstrual period bleeding of unknown cause
  
• Using birth control that contains estrogen and progestin
  
• Using Medications which promote bleeding - eg. aspirin
  
• Using medicines to help blood clot such as factor IX complex concentrates or anti-inhibitor coagulant concentrates.

Taking Tranexamic acid

It is essential to read the manufacturer's information leaflet before you start treatment. The tablets have to be taken exactly the way your medical practioner has prescribed, on the dosage and also the number of tablets to consume daily. The dose will vary from person to another. The tablets have to be swallowed drinking water - do not crush or chew them. You can instead crush the tablet and mix it with a small amount of soft food such as yogurt, honey or jam. In case of liquid medicine, the right amount using an oral syringe or medicine spoon has to be measured. You cannot use a kitchen spoon as it will not give the right amount. Tranexamic acid can be taken before or after food. The medicine will start working right away and will reduce the bleeding on the first day.

In case you have forgotten, better to go on with the next dose, and not to take the missed dose. Only take Tranexamic acid after periods have started. Not more than three doses can be taken (6 tablets) within 24 hours. It should not be taken for more than five days within any menstrual cycle. Thrice daily means, this should be once in the morning, once in the early afternoon and once in the evening. Ideally, these times are at least six hours apart, say, 8 am, 2 pm and 8 pm.

It is also important to keep regular appointment with the doctor. Tranexamic acid is taken for short duration only. In case you are undergoing any operation or dental treatment, remember to tell the doctor about taking Tranexamic acid.

Side effects of Tranexamic acid

Though not all experience these, some do suffer these side effects due to Tranexamic acid consumption:

• Diarrhea,
  
• Eyesight problems, such as with color vision
  
• Back pain, headache, joint pain, muscle pain
  
• Spasms, cramps
  
• Nasal congestion
  
• Stomach pain
  
• Severe allergic reactions such as itching and difficulty in breathing
  
• Swelling in mouth, face, lips and tongue, and flushing of face
  
• Chest pain
  
• Confusion
  
• Coughing up blood
  
• Decreased or difficulty in urination
  
• Eye problems
  
• Numbness in arm, leg or one sided weakness, seizures
  
• Unusual tiredness or weakness

If you are feeling sick, stick to simple meals, and avoid rich and spicy food. Also, it would be better to take tablets after meals. In case of diarrhea, take plenty of water to replace the lost fluids. Never take more than the prescribed dose and do not use outdated medicines. Seek medical attention right away if the side-effects persist.

Tranexamic acid precautions

It is imperative to keep medicines out of reach of children and pets; and also store in a cool place, away from direct sunlight. Tranexamic acid is stored at room temperature and should be kept away from heat, moisture and light. Do not store in the bathroom.

As this acid may cause dizziness, and it may worsen if it is taken with alcohol or certain other medicines, it is better to use it with caution. It is recommended not to drive until you know how you react to it. If your symptoms do not get better after two menstrual cycles and instead get worse, it is better to stop the acid and instead check with the doctor.

The risk of heart attack, stroke, or other blood clots may increase with Tranexamic acid use with hormonal birth control pills. This risk is even greater if you are overweight, or a smoker and are older than the age of 35. Tranexamic acid should be used with utmost caution in children younger than 18 as well as the elderly.