SICKLE CELL: Reporting Early Symptoms (2)

ACCORDING to Dr. Olufunto Kalejaiye, consultant haematologist at LUTH, “Other systems that can be affected include the central nervous system where cerebrovascular accidents (strokes) could occur. Transcranial Doppler (TCD) screening effectively identifies children at highest risk of clinical stroke. There could be eye involvement (sickle cell retinopathy), which is more common in HBSC disorder.

Kidney involvement includes a reduced ability of the kidneys to concentrate urine and may often be faced with the problem of bedwetting, and haematuria (blood in urine) from renal papillary necrosis. Acute complications of sickle cell anaemia are the vaso-occlusive crises,-the most common of which is the bone pain crises. Other well recognized acute complications include priapism ( painful sustained penile erection in the absence of sexual activity or desire , lasting more than 2 to 4 hours ;mean age of onset is about 12-15 years) and acute chest syndrome which usually presents with chest pain , breathlessness fever and cough and can be fatal if not managed promptly.”

She informs that other sickle crises include haemolytic crises (usually caused by infection) where there is increased red cell lysis worsening anaemia and deepening jaundice, sequestration crises (sudden enlargement of the spleen and sometimes the liver and trapping a significant proportion of the circulating cells and can cause a sudden severe anaemia) and aplastic crises (usually by parvovirus B19 infection which suppresses erythroid precursors) also causes a profound anaemia.”

What are the circumstances that give rise to sickle cell crisis?
Several factors have been recognized to precipitate sickle cell crises. These include: Dehydration, fever (from infection,) hypoxia, extremes of temperature, excessive physical exertion (competitive sports, labour) pregnancy to mention a few. Often, the precipitant of crises may be unknown.

In managing or controlling the disease, the expert said that persons with sickle cell disorders are advised about adequate hydration, (at least three litres of water a day,) malaria prophylaxis, (animalarials for malaria prevention as malaria is endemic our environment), daily folic acid supplements (as there is increased demand for folate by the bone marrow in response to haemolysis). Pneumococcal vaccinations to prevent invasive pneumococcal disease may also be given.

Infections should also be recognised early, and treated promptly.“Mild bone pains can be managed at home with analgesics (given by prescription by the managing physician) but when they do not abate or increase in intensity will have to be referred to hospital. Treatment consists of hydration (intravenously), warm packs to the affected area(s), and a combination of analgesic and anti-inflammatory medications and underlying cause of crises e,g infections sought for and treated. Blood transfusions are given when indicated.”

On how parents can help children with sickle cell disorder at home, she says, “Parents first should be educated/ informed/counselled as best as possible about sickle cell disorder, the clinical symptoms and complications, crisis prevention measures, nutrition, and hospital referrals as indicated.

It is also important to emphasize that sickle anaemia is not a death sentence and can be well managed and therefore their children should not be deprived of education or other necessities of life.

Parents should as best as possible ensure that their children are well hydrated, receive malaria prophylaxis and folic acid supplements as prescribed by the managing physician/hematologist. The role of good nutrition also cannot be overemphasised. Other medications when necessary or indicated will be prescribed by the physician/haematologist at the out patients clinic.

Regular out patients’ clinic visits should also be kept for clinical, drug and laboratory test reviews. It is also important to recognize and report symptoms early and when they do not abate on prescribed medication so that they can be evaluated and treated promptly. Acute complications like bone pain crises, require hospitalisation to be properly managed.

Parents in turn have to be able to educate their children about the disorder, explain reasons for prevention of acute complications, reasons for medications, and the need for clinic visits and life style modifications.

They must also individualize limits to exercise/physical activity tolerance. They also have to encourage and give their children good moral support and encourage their children to participate in peer groups like sickle cell clubs where they can meet with others with the disorder where challenges, experiences can be discussed and shared freely, she advised.
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