The 19th June is World Sick­le Cell Day, the day des­ig­nat­ed to raise aware­ness of sick­le cell at a na­tion­al and in­ter­na­tion­al lev­el. On 22nd De­cem­ber 2008, the Unit­ed Na­tions Gen­er­al As­sem­bly adopt­ed a res­o­lu­tion that recog­nis­es sick­le cell dis­ease as a pub­lic health prob­lem and “one of the world’s fore­most ge­net­ic dis­eases.” The res­o­lu­tion calls for mem­bers to raise aware­ness of sick­le cell on June 19th of each year at na­tion­al and in­ter­na­tion­al lev­el. So let’s ven­ture on this jour­ney of aware­ness.

What is Sick­le Cell or Sick­le Cell Dis­ease (SCD)?

Sick­le cell dis­or­ders are a group of ill­ness­es which af­fect your red blood cells that car­ry oxy­gen from your lungs to your tis­sues. Sick­le cell is a ge­net­ic con­di­tion, which means it is passed on from your par­ents and you are born with it; you can­not catch it from oth­er peo­ple.

Sick­le cell dis­or­ders cause your nor­mal­ly round and flex­i­ble blood cells to be­come stiff and sick­le shaped, stop­ping the blood cells, and the oxy­gen they car­ry, from be­ing able to move freely around the body and caus­ing pain as the body’s tis­sues be­come starved for oxy­gen. This can cause episodes of se­vere pain. These painful episodes are re­ferred to as sick­le cell cri­sis. They are treat­ed with strong painkillers such as mor­phine to con­trol the pain.

Peo­ple with sick­le cell are al­so at risk of com­pli­ca­tions of stroke, acute chest syn­drome, blind­ness, bone dam­age and pri­apism (a per­sis­tent, painful erec­tion of the pe­nis). These or­gans are acute­ly af­fect­ed by sick­ling as they are like­ly to be starved of oxy­gen re­sult­ing in dam­age.

Over time peo­ple with sick­le cell can ex­pe­ri­ence dam­age to or­gans such as the liv­er, kid­ney, lungs, heart and spleen. Death can al­so re­sult from com­pli­ca­tions of the dis­or­der. Treat­ment of sick­le cell most­ly fo­cus­es on pre­vent­ing and treat­ing com­pli­ca­tions.

Symp­toms of Sick­le Cell

Chron­ic (long term) anaemia – when there are not enough red blood cells or Hb in your body.

Un­pre­dictable Pain (cri­sis) – When the blood ves­sels get blocked, it can cause pain and swelling in the area, al­so known as a cri­sis. Cri­sis may be pre­cip­i­tat­ed but things such as stress, de­hy­dra­tion and in­fec­tion.

You may get tired eas­i­ly or ex­pe­ri­ence fa­tigue – con­stant tired­ness, feel­ing weak or lack­ing in en­er­gy be­cause of the anaemia.

Jaun­dice – yel­low­ing of the whites of the eyes and skin.

Facts About Sick­le Cell Dis­ease

1. SCD is in­her­it­ed from both par­ents; sick­le cell trait is in­her­it­ed from one par­ent.

2. SCD can af­fect any­one, al­though it pre­dom­i­nant­ly af­fects peo­ple from African and Caribbean back­grounds.

3. A sim­ple blood test will tell whether you have sick­le cell trait or the dis­or­der

4. Chil­dren with SCD are at in­creased risk for stroke, the risk is high­est be­tween the ages of 2

5 .Episodes of pain may oc­cur in sick­le cell dis­or­der and are gen­er­al­ly re­ferred to as a cri­sis

The on­ly pos­si­ble cure for the dis­or­der is bone mar­row trans­plant but this is on­ly pos­si­ble for a lim­it­ed num­ber of af­fect­ed in­di­vid­u­als who have a suit­able donor. A med­i­cine called Hy­drox­yurea, can sig­nif­i­cant­ly re­duce the num­ber of painful crises.

The Sick­le Cell Trait (SCT)

Sick­le cell trait is in­her­it­ed when on­ly one of your par­ents has passed on the sick­le gene, and will nev­er de­vel­op in­to sick­le cell dis­or­der. You do not have symp­toms from sick­le cell trait, so it is a good idea to have a blood test to see if you have sick­le cell trait. If you have the trait, the ma­jor­i­ty of red cells in the blood are nor­mal round shaped cells. Some sick­le shaped cells may be present un­der cer­tain con­di­tions.

The trait is not an ill­ness, but if you are plan­ning to have chil­dren, then cer­tain fac­tors have to be con­sid­ered.

If your part­ner does not have sick­le cell trait, then any chil­dren you have will not have sick­le cell dis­or­der, but they could have the trait (50% chance).

If you and your part­ner both have the trait, there is a 25% chance that any child con­ceived may have sick­le cell dis­or­der and 50% chance they will have the trait.

Of­ten, in­di­vid­u­als who have per­sis­tent anaemia dis­cov­er that SCD or SCT may be hered­i­tary in their fam­i­lies and a sim­ple blood test can di­ag­nose the pres­ence of dis­ease.

For more in­for­ma­tion please vis­it the Sick­le Cell So­ci­ety of T&T at https://www.onescd­voice.com/place/sick­le-cell-so­ci­ety-trinidad-to­ba­go/

Dr. Visham Bhimull

MBBS (UWI)

Diplo­ma in Fam­i­ly Med­i­cine (UWI)