Breath holding spells

Definition

Breath holding spells are episodes of brief, involuntary cessations of breathing that occur in children in response to stimuli such as anger, frustration, fear , or injury.

Description

A breath holding spell (BHS) is a reflexive response that occurs in some healthy young children, usually between the ages of about eight months and two years. A typical breath holding spell lasts between two and 20 seconds. The child cries or gasps, forcibly exhales, stops breathing, and turns either blue (cyanotic form) or pale (pallid form). In a simple BHS the child may faint or briefly lose consciousness. If the child recovers before fainting, some physicians do not consider it to be a true breath holding spell. In a severe or complicated BHS, the child may have a small seizure while unconscious. The entire episode usually lasts less than one minute. The child regains consciousness and normal breathing, and color resume immediately.

The frequency of breath holding spells varies from several times a day to perhaps only once a year. About one-third of affected children have two to five spells daily whereas another one-third of affected children average one spell per month. It is not uncommon for a child with only sporadic or rare breath holding spells to have several in a single day.

Cyanotic form

About 60 percent of affected children have the cyanotic form of BHS, in which the skin and lips turn bluish. This type of breath holding spell also is called type 1, red-blue form, or cyanotic infantile syncope. It usually occurs when the child is angry or frustrated and may be a component of a temper tantrum.

Pallid form

About 20 percent of affected children have the pallid form of BHS in which the child turns pale. It also is called type 2, white breath-holding, reflex anoxic seizure, or pallid infantile syncope. The pallid form of BHS typically follows a frightening or painful experience.

An additional 20 percent of affected children have both types of breath holding spells at different times.

Demographics

Breath holding spells occur in about 5 percent of healthy children between the ages of six months and six years. They are most common between six and 18 months of age. Most affected children have had their first episode before the age of 18 months and almost all affected children have had an episode by the age of two. Breath holding spells are rare before six months of age. Although they are less common after the age of five, some children continue to have episodes until age six or seven.

Breath holding spells appear to occur equally among boys and girls. However, some studies have suggested that boys are more susceptible and that the average onset of BHS in boys is earlier (13–18 months) compared with girls (19–24 months). Studies have found no significant behavioral differences between children who have breath holding spells and those who do not. Nor is there a correlation between the frequency of spells and scores on behavioral profiles. Cyanotic breath holding spells typically begin when children are in a negative or oppositional phase and are starting to assert their independence. They typically end as the child learns to express emotions in words.

Causes and symptoms

Causes

Although the exact physiological mechanism of breath holding spells is not understood, they are an involuntary reflex caused by the interplay between the respiratory control center of the central nervous system, the autonomic nervous system, and the cardiopulmonary system.

CYANOTIC BHS Cyanotic BHS may be a component of a temper tantrum or a child's attempt to gain control over a situation. A cyanotic breath holding spell may also occur in response to the following:

• anger or frustration
• failure of the child to get its way
• a scolding or some other upsetting event
• pain
• being startled
• fear
• a confrontational situation
• a traumatic event

A breath holding spell is an involuntary reflex because it follows exhalation rather than inhalation. In a cyanotic BHS the long exhalation following crying causes breathing to stop. However, in some situations a child may learn how to trigger a cyanotic BHS. A child over the age of two with daily spells may have learned that intense crying or a temper tantrum can trigger a spell. If past breath holding spells have earned children lavish attention or enabled them to get their own way, the children may intentionally cause the spells to trigger an episode.

PALLID BHS Pallid breath holding spells are unpredictable. They usually occur in response to being startled, frightened, in pain, immunized, or injured, particularly after hitting the head.

In a pallid BHS the brain sends a signal via the vagus nerve that severely slows the heart rate, leading to a temporary cessation of breathing and loss of consciousness.

Risk factors

There appears to be a genetic component to at least some breath holding spells. About 25 percent of affected children—particularly those who experience pallid BHS—have a family history of BHS or fainting.

In some cases breath holding spells may be associated with anemia (a reduced number of red blood cells) caused by an iron deficiency, although this is controversial. Treatment may decrease the number of spells in some anemic children; however, treatment with iron increases the frequency of spells in children who are not anemic.

Symptoms

CYANOTIC BHS Once a parent or caregiver has witnessed a breath holding spell the symptoms are obvious. Children may do the following:

• cry vigorously for less than 30 seconds
• hyperventilate (over-breathe)
• have a pause in breathing followed by a long forced exhalation
• turn red in the face from anger
• stop breathing (apnea)
• have a strained face as if they are crying, although there is no sound
• turn bluish-purple, particularly in the face and around the lips, due to lack of oxygen in the brain.

The spell may end at this point and the child resumes breathing. Alternatively, the following may occur:

• The child may faint, become limp, or lose consciousness, usually for just a few seconds, due to a lack of oxygen reaching the brain.
• If breath holding lasts ten seconds or more, the unconscious child may experience muscle twitching, one or two jerky movements, back arching, body stiffening, or a true seizure.
• The child takes a deep breath and resumes normal breathing within 30 to 60 seconds.
• Consciousness and normal skin color return.

Most children recover completely within less than one minute after the start of the episode and resume normal activities. Some children may cry or scream for a period, and other children may fall asleep for an hour.

PALLID BHS In a pallid breath holding spell a child do the following:

• gasp and the lower jaw may quiver, but there is little or no crying
• experience a slowing heart rate or the heart may even stop briefly
• turn pale
• sweat
• stop breathing
• lose muscle strength and go limp
• faint or lose consciousness
• experience muscle twitching or body stiffness while unconscious
• have a seizure

Following these responses, the child's heart speeds up, breathing resumes on its own, and consciousness returns. The child usually recovers completely within one minute but may feel sleepy.

Seizures are much more likely with the pallid form than with the cyanotic form of BHS. Seizures during breath holding spells are more likely if breath holding lasts longer than usual. A child may vomit or urinate during a seizure.

When to call the doctor

Breath holding spells may have symptoms in common with various seizure disorders or other medical conditions. Therefore, a physician should be consulted if any of the following occurs:

• It is the first time a child has had a breath holding spell.
• The child is under six months of age, particularly if the spells occur during feeding or diaper changing.
• The child has a first breath holding spell at four-and-a-half years of age or older.
• The spells become more frequent.
• The spells become more severe.
• The pattern of the spells changes.
• The pallid form of BHS occurs frequently.
• The spells last more than one minute, with continuous body stiffening and relaxing.

Diagnosis

Diagnosis of breath holding spells usually is based on the medical history of children and their families and on complete physical and neurological examinations to rule out other causes. Breath holding spells usually are diagnosed in the following way:

• a child's history of breath holding spells
• the exact sequence of events, which can be written down or videotaped
• lack of incontinence
• lack of post-convulsion symptoms
• blood tests to determine if a child has iron-deficient anemia

In addition the physician inquire about the following:

• if the child has ever been diagnosed with a medical condition
• if there have been recent changes in the child's behavior that are cause for concern
• if there have been recent changes in the child's life such as moving, a new sibling, or divorce
• if the parents have concerns about how other people may be treating the child

Ruling out other causes

Medical conditions that may cause breath holding spells include the following:

• Rett syndrome, a rare genetic disorder affecting girls
• Batten disease, the juvenile form of a group of progressive neurological disorders known as neuronal ceroid lipofuscinoses
• Riley-Day syndrome, a rare genetic disorder
• familial dysautonomia, a rare genetic disorder that can cause involuntary breath holding spells in a child who is already seriously ill

Breath holding spells can be distinguished from epileptic seizures using the following criteria:

• BHS are provoked by an event or situation.
• BHS seizures are brief.
• Recovery from BHS is rapid.
• The change in skin color and loss of consciousness with BHS occur before any seizure-type jerking.
• With epilepsy, convulsions and muscle weakness precede the loss of skin color.
• An electroencephalogram (EEG) that records electrical activity in the brain is normal in all forms of BHS, whereas it may be abnormal with epilepsy.

An electrocardiogram (ECG, EKG) that records the electrical activity in the heart may be used to check for heart rhythm abnormalities, such as long QT syndrome, in children who have had a pallid breath holding spell. Children with long QT syndrome may have breath holding spells in response to exertion or excitement. However, because long QT syndrome is so serious, some physicians recommend that all children with breath holding spells have a baseline EKG.

Treatment

The primary treatment for BHS is to reassure the parent or caregiver that the spells are completely harmless and that they usually disappear by the age of two or two-and-a-half. The child may be put in bed to rest after recovering from the spell. The only treatment for cyanotic BHS is to not encourage or reward the behavior. It is possible that behavior therapy may help a child who suffers from frequent cyanotic spells.

If a child is anemic, iron (at 6 mg per kg [2.2 lb]) of body weight per day for at least three months) may reduce the frequency of breath-holding spells. If pallid breath holding spells are frequent and severe, a preventative anti-cholinergic medicine such as atropine sulfate may be prescribed, in consultation with a neurologist or cardiologist. The dosage is usually 0.1 mg of oral atropine three times daily. Anti-convulsive medications have no effect on breath holding spells.

Prognosis

There are no long-term effects of breath holding spells. Both types of BHS cease without treatment as the child's brain and body develops and matures. The cyanotic form usually peaks at about two years of age and is rare past the age of five. Both types of BHS disappear by the age of four or five in about 50 percent of affected children and in 90 percent of children by the age of six.

Up to 17 percent of children with pallid BHS will experience syncope (fainting spells) as adults, usually in response to fear, injury, or emotional stress. Children with cyanotic episodes are not at a greater risk for syncope as adults.

Prevention

As of 2004 there is no known prevention for pallid breath holding spells since the trigger for such spells is unpredictable. It sometimes may be possible to prevent or interrupt a cyanotic spell by doing the following:

• avoiding situations or events that may lead to tantrums or have caused previous breath holding spells
• distracting the child
• intervening in temper tantrums with soothing words and gestures
• encouraging the child to express emotion with words
• placing a cold cloth on the child's face, particularly within the first 15 seconds

Parenting strategies that may help avoid cyanotic BHS include the following:

• ensuring that the child gets plenty of rest, including daytime rest periods and adequate sleep at night
• not allowing the child to become too hungry, because hunger can contribute to frustration
• minimizing unnecessary frustration
• avoiding unnecessary discipline
• helping the child to learn other means of expressing anger and frustration
• maintaining a regular daily routine
• maintaining a calm home atmosphere
• allowing the child to make simple choices
• praising accomplishments and good behavior
• helping the child to feel secure
• helping the child to become more independent and self-confident

Parental concerns

Breath holding spells can be extremely frightening for parents, siblings, and caregivers. Families need to be reassured that BHS is not a harmful or dangerous event and that no treatment is needed. It is important that caregivers understand the cause of breath holding spells and the proper response.

During a breath holding spell parents should:

• Protect children from injury and prevent their arms, legs, and head from hitting something hard or sharp.
• Lay children down on their back or side, preferably on a padded surface such as a carpeted floor; this increases blood flow to the brain and helps prevent muscle jerking.
• Check for food in the mouth if the child ate just before a spell. Parents should not try to remove the food; rather the child's head should be turned to one side so that the food can come out on its own.
• Touch and talk to the child.
• Allow children to wake from the spell on their own.
• Time the spell with a watch.

Following a breath holding spell, parents should do the following:

• Acknowledge the child's behavior and emotions.
• Reassure any other children present that everything is okay and it is not their fault.
• Hug the child and walk away.

Parents should NOT do the following:

• overreact
• call 911 or use mouth-to-mouth resuscitation or cardiopulmonary resuscitation (CPR)
• place anything in the child's mouth which could cause choking or vomiting
• give the child any medications during the episode
• do anything that could reinforce the behavior, including paying undue attention to the child, making a fuss about the episode, or giving in to the child's demands
• try to keep children from all frustration by overprotecting or sheltering them

KEY TERMS

Apnea —The temporary absence of breathing. Sleep apnea consists of repeated episodes of temporary suspension of breathing during sleep.

Cyanosis —A bluish tinge to the skin that can occur when the blood oxygen level drops too low.

Electrocardiagram (ECG, EKG) —A record of the electrical activity of the heart, with each wave being labeled as P, Q, R, S, and T waves. It is often used in the diagnosis of cases of abnormal cardiac rhythm and myocardial damage.

Electroencephalogram (EEG) —A record of the tiny electrical impulses produced by the brain's activity picked up by electrodes placed on the scalp. By measuring characteristic wave patterns, the EEG can help diagnose certain conditions of the brain.

Pallor —Extreme paleness in the color of the skin.

Syncope —A loss of consciousness over a short period of time, caused by a temporary lack of oxygen in the brain.

A parent who cannot watch a child having a breath holding spell without intervening should leave the room. Parents who have difficulty dealing with a child's frequent breath holding spells may choose to seek counseling.

If a child does not begin breathing on his or her own within one minute, it is not a normal breath holding spell. The parent should call 911 or other emergency services and begin rescue breathing to maintain the child's air passage until help arrives.

Resources

PERIODICALS

Anderson, Jane, and Daniel Bluestone. "Breath-Holding Spells: Scary but not Serious." Contemporary Pediatrics 1 (January 1, 2000): 61.

DiMario, Francis J., Jr. "Prospective Study of Children with Cyanotic and Pallid Breath-Holding Spells." Pediatrics 197, no. 2 (February 2001): 26–59.

ORGANIZATIONS

American Academy of Pediatrics. 141 Northwest Point Boulevard, Elk Grove Village, IL 60007–1098. Web site: <www.aap.org>.

WEB SITES

Greene, Alan. "Breath Holding." Dr. Green.com, April 30, 2002. Available online at <www.drgreene.com/21_1039.html> (accessed December 20, 2004).

Grover, Geeta. "Breath Holding Spells." The Pediatric Bulletin. Available online at <http://home.coqui.net/myrna/breath.htm> (accessed December 20, 2004).

Heins, Marilyn. "Breath-Holding Spells." ParenTips. Available online at <www.parentkidsright.com/ptbreath.html> (accessed December 20, 2004).

Jennette, Robert. "Breath-Holding Spells in Children: How to Distinguish the Benign Type from Serious Conditions." Postgraduate Medicine, May 2002. Available online at <www.postgradmed.com/issues/2002/05_02/jennette.htm> (accessed December 20, 2004).

Terra, Richard P. "Breath-Holding Spells" PeaceHealth, July 19, 2004. Available online at <www.peacehealth.org/kbase/topic/mini/hw31827/overview.htm> (accessed December 20, 2004).

Margaret Alic, Ph.D.