MAJOR ILLNESSES AFFECTING RAINBOW CHILDREN
Blood disorders result from the defects in the blood vessels or from abnormalities in the blood itself. There are different types of blood disorders such as Haemophilia, an inherited bleeding disorder in which the blood fails to clot normally; Anaemia, a condition which occurs when there is an abnormally low amount of red blood cells.
Haemophiliacs lack a specific clotting factor (usually “Factor VIII”) in their blood. They bleed easily and for a longer time even with very minor or no trauma at all. Their daily physical activities are therefore severely limited. They require an injection of this very expensive clotting factor to stop any bleeding, especially if they have sustained an injury.
The most common blood disorder in Club Rainbow is Thalassaemia Major, which is an inheritable blood disorder that occurs due to the inability of the body to manufacture normal haemoglobin, a protein housed in the red blood cells, which is needed to carry oxygen. As a result, insufficient oxygen is carried to many organs in the body. A child with TM may be normal at birth but usually suffers from severe anaemia in infancy. With this the child can develop heart failure that may be life threatening if not treated promptly. In the long run, the child will have a stunted growth and eventually, multi-organ failure. The TM child requires frequent and regular blood transfusions on a long-term basis to correct his/her anaemia. However, as a result, iron gradually accumulates in various parts of the body, causing multi-organ dysfunction such as heart failure, diabetes mellitus, liver damage and delayed puberty. To remove the excess iron from the body, the child has to be on a long-term, almost daily, expensive medicine, such as Desferral. The only hope of a possible cure is a bone marrow transplant which costs about S$150,000 on average, and is not without risks as well.
Gastroenterology relates to disorders of the gastrointestinal tract, liver and pancreas. Club Rainbow (Singapore) services a high number of beneficiaries with Biliary Atresia (BA). BA is a disease characterized by the absence of bile ducts, which normally drain bile from the liver to the intestines. Many have to undergo surgery (Kasai Procedure) which involves connecting a segment of intestine to the liver’s bile ducts to re-establish the flow of bile away from the liver. A third may survive to early childhood but suffer from liver failure. Eventually, a liver transplant will be the optimal but is costly and involves risks/complications.
Congenital Heart Disease (CHD) with its incidence of approximately 6 – 8 per 1000 live births, is the single most common major congenital abnormality. This is a defect in the structure of the heart and great vessels of a newborn. Most heart defects either obstruct blood flow in the heart in an abnormal pattern. The more severe cases are the “blue babies”. Many will require some form of surgical correction and without treatment, the majority of the children with severe forms of CHD will die before adolescence. However, the situation has improved thanks to the advances in medical and surgical care during childhood, and a majority can expect to survive into adulthood. Nevertheless, many of these children continue to suffer from poor health and a poor quality of life, especially since not all heart defects can be cured by surgery.
Children as young as infants can develop kidney failure. When the kidneys fail to excrete unwanted toxic metabolites and excess water at a sufficient rate, both fluid and dangerous waste products accumulate in the body. When the kidneys completely cease to function, the child is in End Stage Renal Failure (ESRF) and requires lifelong dialysis, unless a kidney transplant can be arranged. Nephrotic Syndrome disease involves the kidneys in which there is leakage of a large amount of proteins from the kidneys into the urine. This result in a disruption in the distribution of the body’s fluid balance and a child with Nephrotic Syndrome will appear swollen in the face, abdomen and the limbs due to fluid accumulation. Most will require long term steroid medication and many will suffer from their side effects.
VERY LOW BIRTH WEIGHT PREMATURE BABIES
Prematurity is a common problem in Singapore. The incidence of infants born with very low birth weight (less then 1500g) is about 1 to 1.2% of all live births. A newborn is considered premature if born before 37 weeks of gestation. With the advent of medical sciences, babies as early as 24 weeks of gestation can be saved. However the more premature they are, the more complications they will face as their organ systems are not sufficiently developed to allow them to face the life in the outside world. Most of these babies will encounter various combinations of respiratory, cardiovascular, neurological, visual and nutritional problems.
Neurologic disorders affect the body’s nervous systems. Some symptoms include paralysis, muscle weakness and poor coordination. Cerebral Palsy (movement disorders that appears in early childhood) and Muscular Atrophy/Dystrophy (progressive loss of mass/weakness muscle mass) are common diagnosis affecting mobility of a child. From the spectrum of disorders, one of the most severe is Duchenne Muscular Dystrophy (DMD). Some affected children never learn to walk, while some gradually get weaker and lose their ability to even stand or walk. Most children are either wheelchair-bound or bedridden. One of the most severe complications is when they become too weak to use their muscles to breathe adequately, eventually entering into respiratory failure. Without proper respiratory support, many at this stage will suffer from frequent chest infections and also succumb to the illness.
RARE GENETIC DISORDERS
A genetic disorder is a disease caused by abnormal expression of one or more genes in a person. There are a number of possible causes for genetic defects. It may be caused by a mutation in a gene, affecting its function; or by an abnormal chromosome number, and other causes. The vast majority of these disorders are quite rare and affect one person in several thousands or millions. The group is composed of different kinds of genetic disorders such as Williams Syndrome, Achondroplasia, Pradar Willi and Velocardiofacial Syndrome.
Diabetes Mellitus (DM) is a metabolic disorder where the body has high blood sugar levels over a prolonged period. It is further segregated into Type 1 and Type 2. DM Type 1 results from the pancreas’s failure to produce enough insulin and is more common in children. DM Type 2 is where cells fail to respond to insulin produced by the body. Insulin is the hormone necessary to control the body’s sugar level. These children require long-term daily treatment consisting of multiple insulin injections, and frequent finger pricks to monitor their blood sugar level. However, despite treatment, both short and long term complications may still arise.
Immunological disorder is a dysfunction of the immune system. Frequent occurring disorders include: Systemic Lupus Erythematosus (SLE) which is an autoimmune disease where the body’s immune system attacks healthy tissue. Affected areas can be the skin, joints, kidneys, brains and other organs. Juvenile Arthritis results in the inflammation of joints. Chronic Eczema is prolonged inflammation of the skin leading to rashes, dryness and blistering of the skin.
Asthma is a common long term inflammatory disease of the airways of the lungs. Symptoms include episodes of wheezing, coughing, chest tightness and shortness of breath. With medical advancement, most cases can be controlled with medication.