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.
