Blue Baby Syndrome – Causes, Symptoms and Treatment

A mother looking after her unwell child

Does your baby look somewhat blue off late? Have you noticed a blue, dusky or purple hue in body parts where the skin is thinner? Watch out, your baby may be suffering from Blue Baby Syndrome!

What is Blue Baby Syndrome?

You may have a blue baby at birth because this syndrome occurs due to congenital reasons. This condition is characterised by bluish or purple skin and this is known as cyanosis. In Greek, ‘cyanoses’ literally means ‘dark blue’. The blue tinge is caused by lack of oxygen and de-oxygenated blood flowing in the body.

The bluishness begins around 2-3 months. Initially, the bluish tinge is seen when the baby is cranky or passing bowel. Later the baby always tends to be blue.

This condition may also be accompanied by blue baby heart defect in some cases.

Causes of Blue Baby Disease

A mother looking after her unwell baby

Blue Baby Syndrome is caused by caused by environmental and congenital/genetic factors like associated dysfunction of the heart, lungs and blood pigment. These dysfunctions are explained below:

Heart problems are caused by:

Congenital down syndrome in babies

This is an abnormality in one chromosome that usually leads to physical and mental developmental delays.

Type 2 diabetes in the mother that is not well controlled.

Congenital Cyanotic Heart Disease without any other specific reasons. These are a group of heart diseases and not a single one.

(Please note that not all heart diseases cause cyanosis).

Methemoglobinemia caused by nitrate poisoning

Methemoglobinemia blue baby syndrome occurs when you mix baby’s formula milk or food with nitrate-rich well water or from homemade baby food rich on nitrate (spinach, beet etc.). It is common in rural areas where soil nitrates are used as agricultural Nitrate when not properly processed in the baby’s underdeveloped system hinders sufficient oxygen flow in the blood. Blue Baby Syndrome can be caused by other chemicals and antibiotics in food and water.

It occurs mostly in under 6-month old babies because their gastrointestinal system is not fully formed. So, they cannot convert methemoglobin, an enzyme that cannot carry oxygen, to haemoglobin that carries oxygen throughout the body. The bluish tinge occurs due to this insufficient oxygen in the blood.

These babies tend to retain nitrate that reacts with blood haemoglobin and creates high amounts of methemoglobin. Blue baby syndrome nitrate decreases the oxygen-carrying capacity of the blood and gives the baby the blue pigment. Sometimes, Methemoglobinemia is congenital and is a serious condition that needs immediate treatment.

  • Tetralogy of Fallot (TOF)

This is a major cause of blue baby syndrome. It is congenital and includes four heart defects. These defects affect blood circulation in the lungs. This, in turn, results in oxygen deficient blood flow in the entire body.

  • Transposition of Great Arteries (TGA)

This is a defect in the two main arteries of our body that stem from the heart. It causes deoxygenated blood to circulate in the body and oxygenated blood cannot come out of the lungs. It requires urgent treatment.

  • Other diseases

There are few other conditions that cause blue baby syndrome like Tricuspid Artesia, Persistent Truncus Arteriosus, Hypo-plastic left heart syndrome, Dextro-transposition of the main arteries and total anomalous pulmonary venous connection.

Symptoms of Blue Baby Syndrome

There are many other symptoms of this disease other than the baby blue face.

  • Always cranky and crying.
  • Excess salivation.
  • Feeding tantrums
  • Weight loss.
  • Cold baby
  • Slow growth.
  • Racing heartbeat.
  • Rapid breathing.
  • ‘Clubbed’/swollen fingertips and toes
  • Murmuring sounds in baby heart and poor pulse detected by the doctor.
  • State of shock and fainting in extreme cases.
  • Fever, headache, joint pain.

How is the Diagnosis Done?

The best way to diagnose this condition is with these medical tests prescribed by your doctor:

  • Blood examinations.
  • Tests for lungs:
    1. Chest X-ray
    2. Oxygen saturation
  • Tests for the heart:
    1. Electrocardiogram (EKG).
    2. Echocardiogram
    3. Cardiac Catheterization

Treatment

Blue baby syndrome cure depends on the underlying disease that is causing it.

1. Medication

Methylene blue that supplies oxygen to blood is used to cure Methemoglobinemia.

2. Surgical Procedure

Blue baby heart surgery is sometimes required for TOF and TGA though it is high-risk intervention on a newborn.

Surgery for TOF: It is an open-heart surgery called Blalock-Thomas-Taussig shunt. It is performed for ‘Total Correction.’ Surgery is required in TOF because it involves:

  • A hole in the heart that has to be is surgically closed
  • There is an obstruction in blood flow to the lungs that has to be surgically corrected.

The surgery lasts six to eight hours and is done under general anaesthesia. The recovery period is typically two weeks.

Surgery for TGA: Surgical intervention called Balloon Atrial Septostomy is often required to correct a natural hole in the baby’s heart. It is done under with anaesthesia. The surgical procedure is completed with another intervention in two to three weeks of age and best completed within a month of baby’s birth. It is called the Arterial Switch Operation which restores normal circulation. These procedures improve circulation of deoxygenated and oxygenated blood.

How can you Prevent this Condition?

In many cases the causes are unknown and so prevention can be difficult. However, as a thumb rule:

1. Don’t use well water

Don’t make baby formula milk or cook baby food with well water. Boiling water does not remove nitrates. The only way to avoid this is to get information about the nitrate levels in local water. You may get the well water tested with help from local health officials. It should ideally not be more than 10 mg/L. According to WHO, more than 50mg/ litre of nitrates can cause methaemoglobinaemia.

2. Reduce food full of nitrates

Foods that have a lot of nitrates (that come from the soil) are broccoli, spinach, beets, and carrots. Try to limit them till your baby is 7 years old. It is best to use them in frozen form rather than fresh.

3. Avoid medications like Sulphonamides and Phenacetin

Sulfonamides are mostly antibiotics and some are used as medications for ulcerative colitis. Phenacetin is a painkiller and medication for fever. These drugs are however rarely prescribed these days.

4. Avoid illegal Drugs/Smoking/Alcohol/certain Antenatal Medicines

This may help to prevent congenital heart defects.

5. Control Diabetes

If you have antenatal diabetes it should be controlled properly.

What’s the Outlook for Infants with this Disorder?

This is a rare disorder in babies caused by an underlying disease. The doctor can start immediate treatment with medication or in extreme cases surgery. After a good cure blue babies glow pink with good health. They can lead normal lives with few health concerns.

If you ever notice your little one turning a little blue, it is time to consult a doctor immediately.