Hemophilia: Symptoms-Complications-Management

Hemophilia is a rare genetic disease where blood doesn't clot normally due to the deficiency or the
lack of blood clotting factors (Factor 8 for Hemophilia Type A and Factor 9 for Hemophilia Type B ) associated with unexplained bleeding episodes and eventually internal bleeding causing critical and sometimes lethal complications.

Symptoms
Painful and stressful Symptoms of hemophilia have a great impact on patients social and emotional life due to outgrowing complications

1. Unexplained excessive bleeding from minor cuts or dental work 
2. Social stigmatization due to large deep bruises and frequent nose bleeds
3. Joints Pain stiffness tightness and swelling due to internal bleeding forcing patients to skip a lot of activities and be very careful practicing daily life.
4. A simple bump can be critical for hemophilia patients by causing brain hemorrhage, hence serious complications associated with symptoms like vomiting, pain, double vision, convulsions weakness, and lethargy

Complications
Hemophilia patients are at high risk of developing serious complications due to untreated bleeding episodes damaging internal organs and tissues and affecting joints health causing stiffening and severe pain (Knees, Elbows, and Ankles)

Serious outgrowing complications of Hemophilia 

1. Deep internal bleeding puts pressure on limbs causing numbness and severe pain and is capable of developing arthritis or destroying the joints.
2. Infections acquired by blood transfusion can be lethal in some cases who need a frequent blood transfusion.
3. Patients' immune system starts some sort of rebellion & Negative reaction against factor treatment and releases some sort of proteins called inhibitors impairing the function of clotting factor making them inactive treatment which urged the switch to non-factor Treatment in many cases.

 Management

It can be managed by substituting Clotting factors that have been reduced with  Factor Treatment.

The patients should have the full support of family, friends, and caregivers to beat this vicious enemy and live with its frustrating restrictions related to daily life activities and Protection.

Hemophilia Patients should avoid any medications aggravate bleeding and blood-thinning medications.

It Can be diagnosed in early life by blood tests and suspected cases can be screened out as embryos for discarding the gene (Genetic screening).

Replacement Therapy is used to manage acute cases of bleeding and to protect patients from upcoming bleeding episodes as prophylaxis by keeping Clotting factors 8 & 9 circulating the blood normally.
Treatment Options

• EHL -Extended half-life coagulation factors managed to maintain normal status for longer periods than SHL- standard half-life agents which encouraged the switch from SHL to EHL rapidly.
• Recombinant factor products are not made from human blood, developed in a laboratory using recombinant technology to avoid potential blood-borne transmission of infectious diseases offering a safer option than plasma-derived products.
•  Clotting factor Hormone releasing agents prevent clots from breaking.
• Medication applied directly ( topically) on wounds are used to enhance clotting and healing

Support
All Hemophilia patients must have regular access to factor medications, so Medical Authorities have to secure the factor all over the year.

Every City should have a fully equipped comprehensive medical center or clinics to receive benign hematology cases including Hemophilia patients 24/7.

It is preferred to use the help of a Comprehensive team including hematology consultants & orthopedists, a pain team, nurse educators, social workers, prosocial specialists, and physiotherapists; in such medical centers for providing patients with ideal optimum treatment.