Haemolytic Neonatal Disease

Morbus haemolyticus neonatorum is a serious pathological disorder of the unborn child and newborn. The cause is a rhesus intolerance.

Haemolytic Neonatal Disease

What is neonator haemolytic disease?

Morbus haemolyticus neonatorum is also known as fetal erythroblastosis or fetopathia serologica. The disease usually occurs before birth and is therefore also called Morbus haemolyticus fetalis. Due to blood group incompatibility, the child develops anemia in the womb. The entire organism does not receive enough oxygen. See fun-wiki for What is Hydrophthalmos used for.

The heart is not fully functional and effusions occur in the abdomen and chest. If the disease is caught early, the child can be treated with a blood transfusion in the womb. Rhesus incompatibility as a cause is prevented in Germany by giving all mothers with a negative Rhesus factor antibodies against the Rhesus blood group characteristic.

Causes

The main cause of Morbus haemolyticus neonatorum is Rh incompatibility. Rhesus factor is a surface protein found on the cell surface of red blood cells (erythrocytes). If a person has the Rhesus factor D antigen, they are Rhesus positive. If a person lacks this antigen, they are Rhesus negative. When a Rhesus factor negative person comes into contact with Rhesus positive blood, the body produces antibodies against the Rhesus factor D antigen.

Since the body does not know this antigen from its own blood, it considers it foreign and fights it. During childbirth, blood usually passes from the newborn to the mother. So if the child is Rhesus-positive and the mother Rhesus-negative, she forms antibodies against the Rhesus D antigen. As a rule, this is the first antigen sensitization. Thus, the first child is usually not affected by Morbus haemolyticus neonatorum.

In rare cases, fetal erythrocytes enter the maternal blood during the first pregnancy. Antibodies are then also formed. Since the amount of flushed erythrocytes and thus also the amount of antibodies formed is rather small, they are not sufficient to trigger Morbus haemolyticus neonatorum in the firstborn.

In a second pregnancy with a Rhesus-positive child, the mother’s antibodies against the Rhesus factor D antigen reach the unborn child via the placenta. Since the antibodies are haemolytically active, i.e. they destroy the red blood cells, the child develops haemolysis. Anemia develops due to hemolysis.

Another cause of Morbus haemolyticus neonatorum is ABO incompatibility. It is based on the same principle as Rh incompatibility.

Symptoms, Ailments & Signs

The disease shows its first symptoms in the womb. The fetuses store more water in the tissues, resulting in effusions in the abdominal and pleural cavities. The accumulation of water in the abdominal cavity is also known as ascites. The amniotic fluid is significantly increased. If the amniotic fluid index (AFI) is more than 20 centimeters, it is polyhydramnios.

The pumping weakness of the heart is already evident in the womb. If all symptoms of Morbus haemolyticus neonatorum are fully developed, the full picture (Hydrops fetalis) is present. Anemia can be diagnosed after birth. The edema is clearly visible. The children’s abdomen is distended due to the edema. Severe newborn jaundice (icterus neonatorum) can also be observed.

Diagnosis & course of disease

A blood group determination is already carried out in early pregnancy as part of prenatal care. In addition, an antibody search test for irregular blood group antibodies is carried out. If the result of the antibody screening test is negative, the test is performed again at 25-27 weeks of pregnancy. On the basis of regular ultrasound check-ups, developing hydrops fetalis can be detected at an early stage.

Blood can be taken from the umbilical cord if Morbus haemolyticus neonatorum is suspected. This is how anemia can be diagnosed. After birth, the Coombs test is carried out in the laboratory. This test can be used to detect incomplete antibodies, the so-called IgG, against erythrocytes. In order to be able to record the extent of damage to the blood cells, hemolysis parameters such as reticulocytes or LDH are determined in the laboratory.

Reticulocytes are precursors of erythrocytes. Since the body produces more erythrocytes to compensate for anemia, there are more reticulocytes in the blood of the newborn. LDH, L-lactate hydrogenase, is an enzyme found in all cells. As a laboratory parameter, it indicates cell damage, in this case damage to erythrocytes.

Complications

In most cases, the Morbus haemolyticus neonatorum causes various symptoms and complications before birth. Those affected suffer from water retention that is located directly in the abdominal cavity. Likewise, the patient’s heart is often affected by Morbus haemolyticus neonatorum, so that it only shows a weakened pumping capacity.

In the worst case, the patient can die directly if heart failure occurs. The so-called newborn jaundice can also set in after birth. In most cases, neonatal haemolytic disease can be diagnosed before birth, so that treatment can be initiated immediately after birth. Usually there are no further complications.

The symptoms can be reduced with the help of medication and therapy. In most cases, the course of the disease is positive and the patient’s symptoms are completely reduced by the treatment. The patient’s life expectancy is also not reduced by Morbus haemolyticus neonatorum. In some cases, however, those affected are dependent on blood transfusions.

When should you go to the doctor?

Pregnant women should always take part in the preventive and check-up examinations that are offered during pregnancy. In this way, the current state of health of the fetus is checked and documented. However, if changes and abnormalities occur outside of the check-up appointments, an additional visit to the doctor is advisable. If the mother-to-be has the vague feeling that something is wrong with her health or the development of the growing child, a doctor is needed. Changes in the complexion, inner restlessness, heart palpitations, sleep disturbances or feelings of insecurity should be discussed with the attending doctor. Swelling, severe unusual weight gain, or a general feeling of being unwell should be checked out by a doctor.

If, despite all the examinations and controls that have been carried out, no abnormalities can be found during the pregnancy, the newborn child is examined independently by the obstetric team immediately after the birth. In the first routine postnatal tests, midwives or doctors present detect any irregularities in the infant’s state of health. Parental intervention is not necessary. Disorders of the heart rhythm, discoloration of the skin or swellings on the body are noticed through visual contact with the newborn and in the first examinations to determine the bodily functions. If there is a need for action, further tests are carried out and the responsible doctors are informed. This happens automatically in the case of an inpatient birth or a birth accompanied by a midwife.

Treatment & Therapy

If the Morbus haemolyticus neonatorum develops during pregnancy, blood transfusions can be carried out via the umbilical cord. With early therapy, hydrops fetalis can be avoided. Half of all newborns with rhesus incompatibility have only neonatal jaundice. This usually does not require treatment. Phototherapy may be done.

The child is illuminated with light from the blue area. The unconjugated bilirubin, which causes the skin to turn yellow, is thereby converted into water-soluble bilirubin derivatives. These can be excreted without any problems. The other half of newborns have severe jaundice that needs to be treated with blood exchange transfusions.

In individual cases, the administration of immunoglobulins can alleviate the haemolytic symptoms. Full-blown neonatal haemolytic disease, hydrops fetalis, is an emergency that requires intensive care treatment. The children are intubated and artificially ventilated.

Outlook & Forecast

The sooner the risk of Morbus haemolyticus neonatorum can be recognized, the better the chances of recovery. In most of the affected children, the disease subsides without therapy or with the use of special light therapy. However, in some particularly severe cases, the disease can still progress to life-threatening proportions, especially if appropriate treatment is not given. Then there is a risk that affected children may die from the complications of the disease.

If left untreated, the progressive haemolysis leads to dangerous hyperbilirubinemia after childbirth and can thus damage the body through indirect bilirubin. Muscle weakness in newborns in particular indicates the onset of encephalopathy. As brain damage progresses, patients develop seizures and generalized spasticity. Respiratory insufficiency and pulmonary hemorrhage often occur.

Around 25 percent of the diseased fetuses develop signs of pronounced anemia as a result of the anti-D as early as the 18th to 35th week of pregnancy, with a dangerous hemoglobin concentration below 8 g/dl. Left untreated, this leads to acidosis, hypoxia, spleen enlargement, and liver damage. This in turn leads to a massive tendency to edema in the affected fetuses. Other complications such as pulmonary edema and pulmonary bleeding can also lead to early death.

Prevention

For prevention, Rhesus-negative pregnant women in the 28th week of pregnancy and after the birth of a Rhesus-positive child receive anti-D immunoglobulin injections. The antibodies bind to the erythrocytes that are transferred from the child. The affected erythrocytes are broken down in the mother’s spleen before the mother’s immune system can produce its own antibodies.

Aftercare

In Morbus haemolyticus neonatorum, the aftercare measures are usually significantly limited. It is a congenital disease that cannot be completely cured. For this reason, the affected person should consult a doctor at an early stage in order to prevent the occurrence of other complications and symptoms.

Self-healing is also not possible, although if the person concerned wishes to have children, they should primarily have a genetic examination and counseling carried out in order to prevent a recurrence of Morbus haemolyticus neonatorum. In many cases, those affected are dependent on phototherapy.

In many cases, the help and support of one’s own family is also very important with this disease. Psychological support also has a very positive effect on the further course of the disease and can limit depression and other psychological symptoms. Contact with other patients with Morbus haemolyticus neonatorum can also be useful here, as this leads to an exchange of information, which can make everyday life easier for those affected.

You can do that yourself

The therapy of Morbus haemolyticus neonatorum must be carried out in a pediatric intensive care unit by experienced staff. Parents of the little patients quickly feel helpless. Seeing your own child surrounded by hoses and machines evokes unwanted negative emotions, which are mainly dominated by fears.

In such moments, many clinics offer professional psychological support that can help to better process the stressful experience. It is important here to involve both partners and, if necessary, also siblings and to discuss feelings openly with one another.

However, the actual patient is and remains the sick child. More than anything else, he needs the closeness and attention of his parents during this difficult time. Therefore, whenever possible, both partners should participate in the care of the newborn and communicate this wish to the professional care team. As a rule, this request is gladly complied with. Regular contact with parents has been shown to contribute to the child’s health and effectively combats one’s own sense of powerlessness.