Diva's challenge #171

Dear Laura,

When I saw the weekly challenge about the pearls of courage the first person who came to my mind was my grandson Tristan.

Tristan is 8 year old now, but when he was 2 days old he was diagnose with severe Hemophilia B.

Hemophilia B is a rare genetic disorder which effects approximately 1 in 20,000 newborn males worldwide.

There are 3 levels of gravity, mild, moderate and severe. As I wrote earlier, Tristan is severe, means that he has less than 1% of the factor IX in his blood.  After genetic and blood tests, we know that his body makes no factor IX.

That means that he is bruising very easily, but most of all, it means that if his level of factor IX is below 1%, he is susceptible to spontaneous bleeds in the joints.  He could be playing legos and one of his joints might start bleeding internally, even without bumping himself. He also may bleed internally during his sleep….. Kind of scary situation.

The first 17 months of his life, my daughter took him to the ER so many times, the nurses had so many difficulties to stick h

im (one time, after 7 or 8 none successful sticks, my daughter told the nurse to stick him in the vein he has on his head, at that point he was crying so hard, the vein was very obvious).

So she decided to ask the Hematologist to do a surgery, in order to give to Tristan an implanted port. From that time, we all learn how to stick him (we also call it infuse him), my daughter, Tristan’s father, me (the grandmother), my husband (Tristan’s grandfather) and my second daughter. From that time, no more runs at the ER, except for 2 changes of the port, one in 2010 and the last one in 2014, last March because port have a limited “lifetime”.  One average, Tristan’s ports last about 3 years. We give him the factor he is missing every Sundays, Tuesdays and Thursdays. With this routine he is “safe”.

But if he falls or bumps himself hard we give him an immediate infusion of 100% of the factor. We were told not to wait :”when in doubt it is better to stick than later on regret to not have infused and Tristan has to deal with bleeding in his joints or having a huge hematoma (a bruise with accumulation of blood under the visible bruise)”.

The bleeding in the joints (elbows, knees, ankles, shoulders) are very dangerous, because the blood destroys the cartilage and the joint may become swollen and be damaged for the rest of his life.