Photo credit: the free dictionary. com — Medical dictionary
Today, I will be writing on the ‘Habitus of Sickle Cell Disease’
Habitus of SCD are physical signs of sickle cell disease which may be seen/observed in the body build of a patient with the disease. These features are physical manifestations of problems of SCD, as a result of the abnormally shaped cells (sickled red blood cells) blocking the flow of blood through the tissues of the body. The tissues with impaired blood and oxygen circulation then suffer damage. Damage to tissues and organs of the body may result to moderate to severe disability in patients with sickle cell anaemia. The patients endure episodes of intermittent crises of variable frequency and severity, depending on the degree of organ involvement.
The following are some of the habitus of sickle cell anaemia:
Frontal bossing; seen as prominent frontal bone (fore head bone) in patients with sickle cell anaemia. This occurs due to marrow hyperplasia of the skull bone. The extent of this prominence depends on the severity and frequency of patient’s crisis. There may also be associated pariental bossing.
Squinting on one eye which may result from sickle cell retinopathy on the other.
Yellowness of the eyes; too frequent damages to red blood cells result to frequent hyper haemolytic crisis, which consequently present as jaundice.
Pale conjunctiva; which results from anaemia. Through regular clinic visits, every sickle cell anaemia patient is advised to know his/her stable state PCV (steady state Packed Cell Volume or Haemoglobin level). The stable state PCV for SCD patients is that PCV at which the patient is not in imminent crisis (otherwise stable), usually between 18 and 25%, compared to normal range of 36-48% for adult females and 40-52% for adult male individuals carrying normal Hb genotype.
Breathlessness; this is noticed as nose flaring or if the patient is undressed, signs of subcostal and intercostal recession may be observed. Commonly seen in Acute Chest Syndrome, a type of Sickle cell crisis
Gnathopathy; observable prominence of the maxilla. Commonly seen as prominent upper jaw bone.
Skinny body resulting from poor growth and development
Tall physique; because of the flattening of the vertebrae, the arms and legs appear longer than normal.
Whitish skin; SCD patients may present with pale and whitish skin which may result from chronic anaemia or from complications of chronic renal failure. Cutaneous manifestation of uraemia may be noticed as uraemic frost.
Abdominal swelling; there may be features of protruding/ distended abdomen, which may result from enlargement of the spleen/ or from complications of end organ damage—kidney damage may cause generalized body swelling.
Swelling of the hands and feet; known as dactylitis. It is a painful hand and foot condition usually noticed at age 6months to 8years.
Clubbed fingers; rare feature but may occur in chronic end organ damage.
Limping walking gate may be noticed in patients that have had recurrent osteoarthritis of the hip joint. Avascular necrosis of the head of femur may also result into walking difficulties.
Leg ulcer; chronic leg ulcer may be seen as a result of recurrent infection. Ulcers may refuse to heal as a result of immunosuppression from auto-splenectomy.
Kindly refer to my post on ‘Is She Truly A Sickler‘ to read more on the disease.