Favism and its rescue and nursing measures

Many people may be relatively unfamiliar with favism, and once it occurs, they don’t know how to prevent it. Today, I will introduce it to you:

Favism:

It is the most common genetic disease among Chinese people. It is inherited in a sex-linked recessive manner, so the main victims are males. It is estimated that 2~3% of the people in Taiwan have favism, and most of them are descendants of southerners. It is not a disease unique to the Hakka people.

The genetic lesion of favism is the "glucose-6-phosphate dehydrogenase (G6PD) gene" occurring at the Xq28 locus of the X chromosome, which leads to insufficient activity of the G6PD enzyme in red blood cells. Therefore, when children come into contact with camphor balls (commonly known as stinky balls, camphor balls), gentian violet, certain antipyretics, anti-inflammatory drugs, or eat broad beans, it will cause red blood cells to rupture and hemolysis, causing jaundice, anemia, hemoglobinuria (large amounts of hemoglobin are excreted in the urine) and other conditions that may be life-threatening.

Currently, newborn screening can be used to detect G6PD deficiency in babies. Babies with favism should be careful of excessive jaundice during the neonatal period and avoid contact with the above-mentioned harmful substances, so that they can live a healthy life like ordinary people.

Other common sex-linked recessive genetic diseases include color blindness, hemophilia, mucopolysaccharidosis type 2, and Duchenne/Becker muscular dystrophy.

Rescue and nursing measures

2.1 Timely transfusion of fresh blood: The main symptom of favism in children is acute intravascular hemolysis. The key to first aid is emergency blood transfusion, which is beneficial to replenish blood volume, correct anemia, provide platelets and coagulation factors, and prevent hypovolemic shock. All 25 children received blood transfusions, a total of 62 times. Four cases with hemolytic crisis received an average of 3.5 blood transfusions. During the 62 blood transfusions, we carefully implemented three checks and seven comparisons, strictly implemented aseptic technical operations, and no children in the whole group had transfusion reactions.

2.2 Oxygen supply: Oxygen supply is beneficial to correct hypoxemia and accelerate the separation of hemoglobin from toxins. In this group, except for 16 cases with mild symptoms, the other 9 cases were all given oxygen supply. The respiratory tract and oxygen catheter should be kept unobstructed during the oxygen supply process.

2.3 Application of glucocorticoids: Its effect is to stabilize the cell membrane and prevent the continuation of hemolysis.

2.4 Closely observe changes in vital signs: For children with severe anemia, high fever, shock, and heart and kidney dysfunction, T, P, and R should be measured every hour, and changes in consciousness and complexion should be observed and recorded. After the condition stabilizes, the observation and recording should be changed to once every 2 to 4 hours. Accurately record the 24-hour urine volume.

If the amount is less than 300ml, it indicates the possibility of acute renal failure. If there is shock, renal failure, or high fever, it should be treated immediately. Physical cooling should be actively adopted for patients with high fever, and antipyretic drugs should not be abused.

2.5 Diet and medication care: Most of the children in this group have varying degrees of hepatosplenomegaly and loss of appetite. They should be encouraged to eat a high-protein, low-fat, high-carbohydrate diet. Oral medications are mainly iron preparations, which stimulate the gastrointestinal tract and should be taken after meals. Tea should not be consumed during iron preparations, and large amounts of milk should not be consumed to avoid affecting the absorption of iron preparations.

2.6 Discharge instructions: When the child was discharged from the hospital, the family members and children were informed to avoid eating broad beans and broad bean products, and the parents were warned to prevent the child from going to places where broad beans were grown. Due to the repeated publicity, no second attack was found in the child.