Dealing with Severe Hemophilia

Severe hemophilia is characterised by clotting factor activity being below 1%. Normal clotting factor activity is usually above 50%. The low level of clotting factor delays clotting which can be fatal and thus requires extra precautions and care.

Individuals with hemophilia have been known to bleed profusely and for a prolonged period.  Soft tissues and areas with thin fibrin are more prone to such bleeds and allow easy injuries.  Bleeding in joints, followed by an inflammation is another sign of hemophilia. Such internal haemorrhages can lead to permanent deformities. Site of an internal haemorrhage can be anywhere from the urinary tract, to knee joins and intestine. No sooner than the symptoms are noticed should the patient be given professional medical help.

Recurring bleeds in the same site can chronically damage the joint too. Blood deposition between joints that bleed frequently erodes the sides of the joints causing permanent deformities. Such damage can be checked and controlled if patient undergoes replacement therapy. The missing clotting factor is infused intravenously to boost the clotting factor count. This promotes clotting.

In case of severe hemophilia the patient might have to undergo prophylactic therapy which is also called the preventive therapy. To control the incidents of internal bleeding, clotting factors is given to the haemophiliacs as a preventive measure at regular intervals. In such cases the patient and his family need to be familiar with the infusion process because of the frequency of these infusions. Patients here generally inject the clotting factors themselves. These factors can be stored in your home for your convenience and used as per your needs. Even prior to dentistry or a surgery, clotting factors are infused to promote clotting, in the absence of which a patient might bleed to death.

Availability of recombinant factor has made such replacements a much safer process as compared to the past. Before the 1980s, cases where patients were injected HIV or Hepatitis infected blood had led to many a deaths. Since the introduction of recombinant factors, this issue has been dealt with completely.

Amongst all the talk of bleeds, we generally forget about the pain that is associated with the bleed. More often than not, these pains become a part of the life of a patient suffering from hemophilia. Always consult your doctor, if your acute pain is becoming chronic to ease it out.

While the doctors would burden you with an unending list of do’s and don’ts, some very common and basic things that people with severe hemophilia forget are:

  • Not to carry heavy weight, since this may adversely affect the joints.
  • Know how to self infuse clotting factors to enable a faster healing process in case of an injury
  • To stay away from contact sports
  • To take added fitness measures and develop a strong immune system
  • Be regular with your visits to the doctor and keep him updated with your condition.

Those with severe hemophilia should take vaccines against hepatitis B since it can be transmitted through blood which is namely disinfected. No vaccine for Hepatitis C is available. Since, patients with severe hemophilia are so frequently infused with blood, such vaccines are indispensible.

It has been observed that women are rarely diagnosed with severe hemophilia. Symptomatic carriers exhibit traits similar to those of mild hemophilia patients, but women suffering from severe hemophilia is a rarity, if not impossible.

Patients with severe hemophilia are the ones who are most susceptible to injuries and traumas. They are also the ones who are known to live in the shadow of fear. However severe the symptoms might be, new medications empower you to conquer all of it. All that you need is a little bit of care.

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