Health Insurance

Health insurance is insurance that pays for medical expenses. It is sometimes used more broadly to include insurance covering disability or long-term nursing or custodial care needs. It may be provided through a government-sponsored social insurance program, or from private insurance companies. It may be purchased on a group basis (e.g., by a firm to cover its employees) or purchased by individual consumers. In each case, the covered groups or individuals pay premiums or taxes to help protect themselves from high or unexpected healthcare expenses. Similar benefits paying for medical expenses may also be provided through social welfare programs funded by the government.

By estimating the overall risk of healthcare expenses, a routine finance structure (such as a monthly premium or annual tax) can be developed, ensuring that money is available to pay for the healthcare benefits specified in the insurance agreement. The benefit is administered by a central organization such as a government agency, private business, or not-for-profit entity.

How it works

A health insurance policy is a contract between an insurance company and an individual or his sponsor (e.g. an employer). The contract can be renewable annually or monthly. The type and amount of health care costs that will be covered by the health insurance company are specified in advance, in the member contract or "Evidence of Coverage" booklet. The individual insurered person's obligations may take several forms:

• Premium: The amount the policy-holder or his sponsor (e.g. an employer) pays to the health plan each month to purchase health coverage.
• Deductible: The amount that the insured must pay out-of-pocket before the health insurer pays its share. For example, a policy-holder might have to pay a $500 deductible per year, before any of their health care is covered by the health insurer. It may take several doctor's visits or prescription refills before the insured person reaches the deductible and the insurance company starts to pay for care.
• Copayment: The amount that the insured person must pay out of pocket before the health insurer pays for a particular visit or service. For example, an insured person might pay a $45 copayment for a doctor's visit, or to obtain a prescription. A copayment must be paid each time a particular service is obtained.
• Coinsurance: Instead of, or in addition to, paying a fixed amount up front (a copayment), the co-insurance is a percentage of the total cost that insured person may also pay. For example, the member might have to pay 20% of the cost of a surgery over and above a co-payment, while the insurance company pays the other 80%. If there is an upper limit on coinsurance, the policy-holder could end up owing very little, or a great deal, depending on the actual costs of the services they obtain.
• Exclusions: Not all services are covered. The insured person is generally expected to pay the full cost of non-covered services out of their own pocket.
• Coverage limits: Some health insurance policies only pay for health care up to a certain dollar amount. The insured person may be expected to pay any charges in excess of the health plan's maximum payment for a specific service. In addition, some insurance company schemes have annual or lifetime coverage maximums. In these cases, the health plan will stop payment when they reach the benefit maximum, and the policy-holder must pay all remaining costs.
• Out-of-pocket maximums: Similar to coverage limits, except that in this case, the insured person's payment obligation ends when they reach the out-of-pocket maximum, and the health company pays all further covered costs. Out-of-pocket maximums can be limited to a specific benefit category (such as prescription drugs) or can apply to all coverage provided during a specific benefit year.
• Capitation: An amount paid by an insurer to a health care provider, for which the provider agrees to treat all members of the insurer.
• In-Network Provider: (U.S. term) A health care provider on a list of providers preselected by the insurer. The insurer will offer discounted coinsurance or copayments, or additional benefits, to a plan member to see an in-network provider. Generally, providers in network are providers who have a contract with the insurer to accept rates further discounted from the "usual and customary" charges the insurer pays to out-of-network providers.
• Prior Authorization: A certification or authorization that an insurer provides prior to medical service occurring. Obtaining an authorization means that the insurer is obligated to pay for the service, assume it matches what was authorized. Many smaller, routine services do not require authorization.
• Explanation of Benefits: A document sent by an insurer to a patient explaining what was covered for a medical service, and how they arrived at the payment amount and patient responsibility amount.

Prescription drug plans are a form of insurance offered through some employer benefit plans in the U.S., where the patient pays a copayment and the prescription drug insurance part or all of the balance for drugs covered in the formulary of the plan.

Some, if not most, health care providers in the United States will agree to bill the insurance company if patients are willing to sign an agreement that they will be responsible for the amount that the insurance company doesn't pay. The insurance company pays out of network providers according to "reasonable and customary" charges, which may be less than the provider's usual fee. The provider may also have a separate contract with the insurer to accept what amounts to a discounted rate or capitation to the provider's standard charges. It generally costs the patient less to use an in-network provider.

Health plan vs. health insurance

Historically, HMOs tended to use the term "health plan", while commercial insurance companies used the term "health insurance". A health plan can also refer to a subscription-based medical care arrangement offered through HMOs, preferred provider organizations, or point of service plans. These plans are similar to pre-paid dental, pre-paid legal, and pre-paid vision plans. Pre-paid health plans typically pay for a fixed number of services (for instance, $300 in preventive care, a certain number of days of hospice care or care in a skilled nursing facility, a fixed number of home health visits, a fixed number of spinal manipulation charges, etc.) The services offered are usually at the discretion of a utilization review nurse who is often contracted through the managed care entity providing the subscription health plan. This determination may be made either prior to or after hospital admission (concurrent utilization review).

Comprehensive vs. scheduled

Comprehensive health insurance pays a percentage of the cost of hospital and physician charges after a deductible (usually applies to hospital charges) or a co-pay (usually applies to physician charges, but may apply to some hospital services) is met by the insured. These plans are generally expensive because of the high potential benefit payout — $1,000,000 to 5,000,000 is common — and because of the vast array of covered benefits.

Scheduled health insurance plans are not meant to replace a traditional comprehensive health insurance plans and are more of a basic policy providing access to day-to-day health care such as going to the doctor or getting a prescription drug. In recent years, these plans have taken the name mini-med plans or association plans. These plans may provide benefits for hospitalization and surgical, but these benefits will be limited. Scheduled plans are not meant to be effective for catastrophic events. These plans cost much less than comprehensive health insurance. They generally pay limited benefits amounts directly to the service provider, and payments are based upon the plan's "schedule of benefits". Annual benefits maximums for a typical scheduled health insurance plan may range from $1,000 to $25,000.

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Pneumococcal Vaccine (PCV)

The pneumococcal conjugate vaccine (PCV) protects against pneumococcal infections. The bacterium is a leading cause of serious infections, including pneumonia, blood infections, and bacterial meningitis.

Children under 2 years old, adults over 65 years old, and people with certain medical conditions are most susceptible to serious pneumococcal infections. The pneumococcus bacterium is spread through person-to-person contact. The vaccine not only prevents the infection in children who receive it, it also helps stop its spread.

Immunization Schedule

PCV immunizations are given as a series of four injections starting at 2 months of age and following at 4 months, 6 months, and 12 to 15 months. Kids who miss the first dose or may have missed subsequent doses due to vaccine shortage should still receive the vaccine, and your doctor can give you a modified schedule for immunization.

Why the Vaccine Is Recommended

The most serious infections affect children younger than 2 years old, and the vaccine will protect them when they're at greatest risk.

PCV also is recommended for kids between 2 and 5 years of age who are at high risk for serious pneumococcal infections because they have medical problems such as:

• sickle cell anemia
• a damaged spleen or no spleen
• HIV/AIDS
• cochlear implants
• a disease that affects the immune system, such as diabetes or cancer
• receiving medications that affect the immune system, such as steroids or chemotherapy

In addition, these high-risk children may also receive the pneumococcal polysaccharide vaccine (PPV) in addition to the PCV when they're older than 24 months.

The PCV vaccine should be considered for all other unvaccinated 2- to -5-year-olds, especially those who are under 3 years of age; are of Alaska Native, American Indian, or African American descent; or who attend group childcare centers.

Possible Risks

Children who receive the PCV vaccine may have redness, tenderness, or swelling where the shot was given. A child may also have a fever after receiving the shot.

When to Delay or Avoid Immunization

• if your child is currently sick, although simple colds or other minor illnesses should not prevent immunization
• if your child has had a severe allergic reaction to a previous dose of the vaccine

Caring for Your Child After Immunization

The vaccine may cause mild fever, and soreness and redness in the area where the shot was given. Depending on the age of your child, pain and fever may be treated with acetaminophen or ibuprofen. Check with your doctor to see if you can give either medication, and to find out the appropriate dose.

When to Call the Doctor

• if your child missed a dose in the series
• if a severe allergic reaction or high fever occurs after immunization

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Hepatitis B Vaccine (HBV)

Hepatitis B virus (HBV) affects the liver. Those who are infected can become lifelong carriers of the virus and may develop long-term problems such as cirrhosis (liver disease) or cancer of the liver.

Immunization Schedule

Hepatitis B vaccine usually is given as a series of three injections. The first shot is often given to infants shortly after birth. If the mother of a newborn carries the hepatitis B virus in her blood, the infant needs to receive the first shot within 12 hours after birth, along with another shot (HBIG) to immediately provide protection against the virus. If a newborn's mother shows no evidence of HBV in her blood, the infant may receive the hepatitis B vaccine any time prior to leaving the hospital. It may also be delayed until the 1- or 2- month visit to your doctor.

If the first dose is given shortly after birth, the second shot is given at 1 to 2 months and the third at 6 to 18 months. For infants who don't receive the first shot until 1 to 2 months, the second shot is given at 3 to 4 months and the third at 6 to 18 months. In either case, the second and third shots are usually given in conjunction with other routine childhood immunizations.

Why the Vaccine Is Recommended

The hepatitis B vaccine usually creates long-term immunity. Infants who receive the HBV series should be protected from hepatitis B infection not only throughout their childhood but also into the adult years. Eliminating the risk of infection also decreases risk for cirrhosis of the liver, chronic liver disease, and liver cancer. Young adults and adolescents should also receive the vaccine if they did not as infants.

Possible Risks

Serious problems associated with receiving the HBV vaccine are rare. Problems that do occur tend to be minor, such as fever or redness or tenderness at the injection site.

When to Delay or Avoid Immunization

• if your child is currently sick, although simple colds or other minor illnesses should not prevent immunization
• if a severe allergic reaction (called anaphylaxis) occurred after a previous injection of the HBV vaccine

Caring for Your Child After Immunization

The vaccine may cause mild fever, and soreness and redness in the area where the shot was given. Depending on the age of your child, pain and fever may be treated with acetaminophen or ibuprofen. Very young infants should not be given either medication, but for older infants or children, you can check with the doctor about the appropriate dose.

When to Call the Doctor

• if you're not sure of the recommended schedule for the hepatitis B vaccine
• if you have concerns about your own HBV carrier state
• if moderate or serious adverse effects appear after your child has received an HBV injection

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Varicella (chickenpox) Vaccine

The varicella vaccine protects against chickenpox (varicella), a common and very contagious childhood viral illness.

Immunization Schedule

The varicella vaccine is given by injection between the ages of 12 and 15 months, followed by a booster shot at 4 to 6 years of age for further protection. Older kids under the age of 12 years who have not had chickenpox may also receive the vaccine, with the two doses given at least 3 months apart. Kids 13 years or older who have not had either chickenpox or the vaccine would need two vaccine doses at least 1 month apart.

Why the Vaccine Is Recommended

The varicella vaccine prevents severe illness in 95% of children who are immunized. It's up to 85% effective in preventing mild illness. Vaccinated kids who do get chickenpox generally have a mild case.

Possible Risks

Serious reactions are extremely rare. Possible mild effects are tenderness and redness where the shot was given, fever, fatigue, and a varicella-like illness. A rash can occur where the shot was given or elsewhere on the body up to 1 month after the injection. It may last for several days but will disappear on its own without treatment.

When to Delay or Avoid Immunization

• if your child is currently sick, although simple colds or other minor illnesses should not prevent immunization
• if your child has an allergy to gelatin or to the antibiotic neomycin that has required medical treatment
• if your child has recently received gamma globulin or a blood transfusion
• if your child has immune system problems related to cancer; is taking prednisone, steroids, or other immunosuppressive drugs; or is undergoing chemotherapy or radiation therapy

Caring for Your Child After Immunization

Pain and fever may be treated with acetaminophen or ibuprofen. Check with your doctor about the appropriate dose.

When to Call the Doctor

• if you aren't sure if the vaccine should be postponed or avoided
• if there are problems after the immunization

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Influenza Vaccine

Influenza, commonly known as "the flu," is a highly contagious viral infection of the respiratory tract.

Immunization Schedule

These groups, who are at increased risk of flu-related complications, should receive the flu shot every year:

• all children between 6 months and 18 years old, especially those 6 to 59 months old
• any child or adult with chronic medical conditions, such as asthma, cystic fibrosis, diabetes, sickle cell anemia, and HIV/AIDS
• children and teens on long-term aspirin therapy
• anyone age 50 and older
• women who will be pregnant during the flu season
• anyone who lives or works with infants (especially those under 6 months old)
• residents of long-term care facilities, such as nursing homes
• health care personnel who have direct contact with patients
• out-of-home caregivers and household contacts of anyone in any of these high-risk groups

In the past, there have been times when there were vaccine shortages and delays. So talk with your doctor about availability.

For kids younger than 9 who are getting a flu shot for the first time, it's given in two separate shots a month apart. It can take about 2 weeks after the shot is given for the body to build up protection to the flu.

Another non-shot option called the nasal mist vaccine came on the market in 2003 and is now approved for use in healthy 2- to 49-year-olds. But this nasal mist isn't for everyone, and can't be used by high-risk children and adults or pregnant women.

Why the Vaccine Is Recommended

The flu vaccine reduces the average person's chances of catching the flu by up to 80% during the season. Getting the shot before the flu season is in full force gives the body a chance to build up immunity to, or protection from, the virus.

The shot usually becomes available between September and mid-November. Although you can get a flu shot well into flu season, it's best to try to get it earlier rather than later, if your doctor thinks it's necessary. However, even as late as January there are still 2 to 3 months left in the flu season, so it's still a good idea to get protection.

Even if you or your child got the vaccine last year, that won't protect you from getting the flu this year, because the protection wears off and flu viruses constantly change. That's why the vaccine is updated each year to include the most current strains of the virus.

Possible Risks

Given as one injection in the upper arm, the flu shot contains killed flu viruses that will not cause someone to get the flu, but will cause the body to fight off infection by the live flu virus. Getting a shot of the killed virus offers protection against that particular type of live flu virus if someone comes into contact with it.

Some of the most common side effects from the flu shot are soreness, redness, or swelling at the site of the injection. A low-grade fever and aches are also possible. Because the nasal spray flu vaccine is made from live viruses, it may cause mild flu-like symptoms, including runny nose, headache, vomiting, muscle aches, and fever. Very rarely, the flu vaccine can cause serious side effects such as a severe allergic reaction.

When to Delay or Avoid Immunization

People who should not get the flu shot include:

• infants under 6 months old
• anyone who's severely allergic to eggs and egg products because the ingredients for flu shots are grown inside eggs. Tell the doctor if your child is allergic before he or she gets a flu shot.
• anyone who's ever had a severe reaction to a flu vaccination
• anyone who's had Guillain-Barré syndrome (GBS, a rare medical condition that affects the nerves) within 6 weeks of getting a flu shot
• anyone with a fever

Caring for Your Child After Immunization

Pain and fever may be treated with acetaminophen or ibuprofen. Check with your doctor about the appropriate dose. Some doctors recommend a dose just before the immunization. A warm, damp cloth or a heating pad also may help minimize soreness. Moving or using the limb that has received the injection often reduces the soreness as well.

When to Call the Doctor

• if you aren't sure if the vaccine should be postponed or avoided
• if there are problems after the immunization

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Hepatitis A Vaccine (HAV)

The hepatitis A virus (HAV) causes fever, nausea, vomiting, and jaundice, and can lead to community-wide epidemics. Childcare centers are a common site of outbreaks.

The vaccine is recommended for children 12-23 months old, followed by a second dose 6 months later. The vaccine is also recommended for older kids and adults who are at high risk for the disease, including those who are traveling to locations where there are high rates of HAV.

Why the Vaccine Is Recommended

Vaccination against HAV can help stop epidemics from developing in the community, in addition to protecting the individual child. Some infected children do not have any symptoms, and can spread the virus to others. The more young children who are vaccinated against HAV, the more limited the spread of disease will be in a community.

Possible Risks

Side effects are usually mild fever, and tenderness, swelling, and redness at the site of the injection.

When to Delay or Avoid Immunization

• if your child is currently sick, although simple colds or other minor illnesses should not prevent immunization
• if your child had an allergic reaction to the first dose of hepatitis A vaccine

Caring for Your Child After Immunization

Your child may experience fever, soreness, and some swelling and redness in the area where the shot was given. Pain and fever may be treated with acetaminophen or ibuprofen. Check with your doctor about the appropriate dose.

When to Call the Doctor

• if you aren't sure if the vaccine should be postponed or avoided
• if there are problems after the immunization

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Rotavirus Vaccine

Rotavirus is a common virus that causes diarrhea, especially in infants and young children. Childcare centers are a common site of outbreaks.

The vaccine, which is a liquid given by mouth, is recommended at ages 2, 4, and 6 months.

Why the Vaccine Is Recommended

Rotavirus can cause dehydration secondary to large amounts of diarrhea, and can result in children requiring hospitalization. Vaccination against rotavirus can help stop spread in the community, in addition to protecting the individual child.

Possible Risks

Side effects can include diarrhea and vomiting, in addition to fever.

When to Delay or Avoid Immunization

• if your child is currently sick, although simple colds should not prevent immunization
• if your child had an allergic reaction to a previous dose of the vaccine
• if your child has an abnormality of the digestive system or a gastrointestinal disease
• if your child has a history of intussusception, a type of serious bowel blockage
• if your child has recently received gamma globulin or a blood transfusion
• if your child has immune system problems related to cancer, leukemia, or lymphoma; is taking steroids or other immunosuppressive drugs; or is undergoing chemotherapy or radiation therapy

Caring for Your Child After Immunization

Depending on the age of your child, fever may be treated with acetaminophen or ibuprofen. Check with your doctor to see if you can give either medication, and to find out the appropriate dose. If vomiting or diarrhea occur, make sure to give your child small, frequent amounts of fluid and watch for signs of dehydration, such as less urine than usual.

When to Call the Doctor

• if you aren't sure if the vaccine should be postponed or avoided
• if there are problems after the immunization

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HPV Vaccine

Human papillomavirus (HPV) is a sexually transmitted disease (STD) that causes genital warts and changes in the cervix that can result in cervical cancer.

The vaccine is recommended for girls 11 or 12 years old, as well as for older girls who are unvaccinated. It is given as a series of three shots over a 6-month period.

Why the Vaccine Is Recommended

Because HPV can cause serious problems such as genital warts and cervical cancer, a vaccine is an important step in preventing infection and protecting against the spread of HPV. It works best when given before a girl becomes sexually active.

Possible Risks

Side effects are usually mild fever and tenderness, swelling, and redness at the site of the injection. Dizziness, fainting, nausea, and vomiting may also occur after the shot.

When to Delay or Avoid Immunization

• if your daughter is currently sick, although simple colds or other minor illnesses should not prevent immunization
• if your daughter had an allergic reaction to the first dose of HPV vaccine
• if your daughter has had a severe allergic reaction to yeast
• if your daughter is pregnant
• if your daughter has a bleeding disorder (discuss with your hematologist)

Caring for Your Child After Immunization

Your child may experience fever, soreness, and some swelling and redness in the area where the shot was given. Pain and fever may be treated with acetaminophen or ibuprofen. Check with your doctor about the appropriate dose.

When to Call the Doctor

• if you aren't sure if the vaccine should be postponed or avoided
• if there are problems after the immunization

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Immunization Schedule

This immunization schedule is a handy reference to help you keep track of which vaccines your child needs to receive and when.

Types of Vaccines

Four different types of vaccines are currently available:

1. Attenuated (weakened) live viruses are used in some vaccines such as in the measles, mumps, and rubella (MMR) vaccine.
2. Killed (inactivated) viruses or bacteria are used in some vaccines, such as in IPV.
3. Toxoid vaccines contain a toxin produced by the bacterium. For example, the diphtheria and tetanus vaccines are toxoid vaccines.
4. Conjugate vaccines (such as Hib) contain parts of bacteria combined with proteins.

Immunizations for Travel

Specific information about which immunizations are required by travelers to each country worldwide is available directly from the Centers for Disease Control and Prevention (CDC). Ask your doctor for more information.

Depending on the type and length of travel, some vaccines may be recommended. Most immunizations should be given at least 1 month before travel. Take your child's immunization records with you when you travel internationally.

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MCV4

The meningitis vaccine protects against meningococcal disease, a serious bacterial infection that can lead to bacterial meningitis.

The vaccine is recommended for kids at age 11 or 12 years, at ages 13 to 18 years if not previously vaccinated, and for older teens who are entering college and will be living in a dormitory setting.

Why the Vaccine Is Recommended

Bacterial meningitis, an inflammation of the membrane that protects the brain and spinal cord, is a rare but highly contagious disease that can spread rapidly among kids who are in close quarters. It can be life-threatening if not promptly treated.

Possible Risks

Some of the most common side effects are swelling, redness, and pain at the site of the injection, along with headache, fever, or fatigue.

When to Delay or Avoid Immunization

• if your child tends to have allergic reactions to the DTaP vaccine or to latex
• if your child has a history of Guillain-Barré syndrome, a disease of the nervous system which causes progressive weakness
• if your child is currently sick, although simple colds or other minor illnesses should not prevent immunization

Caring for Your Child After Immunization

Your child may experience fever, soreness, and some swelling and redness in the area where the shot was given. Pain and fever may be treated with acetaminophen or ibuprofen. Check with your doctor about the appropriate dose. Some doctors recommend a dose just before the immunization.

A warm, damp cloth or a heating pad also may help reduce soreness. Moving or using the limb that has received the injection often reduces the soreness.

When to Call the Doctor

• if you aren't sure if the vaccine should be postponed or avoided
• if there are problems after the immunization

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Hepatitis B Vaccinations

At birth, infants have protection against certain diseases because antibodies have passed through the placenta from the mother to the unborn child. After birth, breastfed babies get the continued benefits of additional antibodies in breast milk. But in both cases, the protection is temporary.

Immunization (vaccination) is a way of creating immunity to certain diseases by using small amounts of a killed or weakened microorganism that causes the particular disease.

Microorganisms can be viruses, such as the measles virus, or they can be bacteria, such as pneumococcus. Vaccines stimulate the immune system to react as if there were a real infection — it fends off the "infection" and remembers the organism so that it can fight it quickly should it enter the body later.

Some parents may hesitate to have their kids vaccinated because they're worried that the children will have serious reactions or may get the illness the vaccine is supposed to prevent. Because the components of vaccines are weakened or killed — and in some cases, only parts of the microorganism are used — they're unlikely to cause any serious illness. Some vaccines may cause mild reactions, such as soreness where the shot was given or fever, but serious reactions are rare.

The risks of vaccinations are small compared with the health risks associated with the diseases they're intended to prevent.

The following vaccinations and schedules are recommended by the American Academy of Pediatrics (AAP). Please note that some variations are acceptable and that changes in recommendations frequently occur as new vaccines are developed. Many of these vaccines are available as combinations to reduce the number of shots a child receives. Your doctor will determine the best vaccinations and schedule for your child.

Recommended vaccinations:

Hepatitis B

Hepatitis B virus (HBV) affects the liver. Those who are infected can become lifelong carriers of the virus and may develop long-term problems such as cirrhosis (liver disease) or cancer of the liver.

Immunization Schedule

Hepatitis B vaccine usually is given as a series of three injections. The first shot is often given to infants shortly after birth. If the mother of a newborn carries the hepatitis B virus in her blood, the infant needs to receive the first shot within 12 hours after birth, along with another shot (HBIG) to immediately provide protection against the virus. If a newborn's mother shows no evidence of HBV in her blood, the infant may receive the hepatitis B vaccine any time prior to leaving the hospital. It may also be delayed until the 1- or 2- month visit to your doctor.

If the first dose is given shortly after birth, the second shot is given at 1 to 2 months and the third at 6 to 18 months. For infants who don't receive the first shot until 1 to 2 months, the second shot is given at 3 to 4 months and the third at 6 to 18 months. In either case, the second and third shots are usually given in conjunction with other routine childhood immunizations.

Why the Vaccine Is Recommended

The hepatitis B vaccine usually creates long-term immunity. Infants who receive the HBV series should be protected from hepatitis B infection not only throughout their childhood but also into the adult years. Eliminating the risk of infection also decreases risk for cirrhosis of the liver, chronic liver disease, and liver cancer. Young adults and adolescents should also receive the vaccine if they did not as infants.

Possible Risks

Serious problems associated with receiving the HBV vaccine are rare. Problems that do occur tend to be minor, such as fever or redness or tenderness at the injection site.

When to Delay or Avoid Immunization

• if your child is currently sick, although simple colds or other minor illnesses should not prevent immunization
• if a severe allergic reaction (called anaphylaxis) occurred after a previous injection of the HBV vaccine

Caring for Your Child After Immunization

The vaccine may cause mild fever, and soreness and redness in the area where the shot was given. Depending on the age of your child, pain and fever may be treated with acetaminophen or ibuprofen. Very young infants should not be given either medication, but for older infants or children, you can check with the doctor about the appropriate dose.

When to Call the Doctor

• if you're not sure of the recommended schedule for the hepatitis B vaccine
• if you have concerns about your own HBV carrier state
• if moderate or serious adverse effects appear after your child has received an HBV injection

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Thalassemias

What Are Thalassemias?

Thalassemias are genetic disorders that involve the decreased and defective production of hemoglobin, a molecule found inside all red blood cells (RBCs) that transports oxygen throughout the body.

As frightening as thalassemias can be, the outlook is encouraging. In the past 20 years, new therapies have greatly improved the quality of life and life expectancy in kids who have these diseases.

Thee two types of thalassemia are alpha-thalassemia and beta-thalassemia. Their names describe which part of the hemoglobin molecule that is effected, the alpha or the beta chain. Hemoglobin contains two different kinds of protein chains named alpha and beta chains. Any deficiency in these chains causes abnormalities in the formation, size, and shape of RBCs.

Thalassemia can cause ineffective production of RBCs and their destruction. As a result, people with thalassemia often have a reduced number of RBCs in the bloodstream (anemia), which can affect the transportation of oxygen to body tissues. In addition, thalassemia can cause RBCs to be smaller than normal or drop hemoglobin in the RBCs to below-normal levels.

Kids who have with different forms of thalassemia have different kinds of health problems. Some only have mild anemia with little or no effects, while others require frequent serious medical treatment.

Causes

Thalassemia is always inherited, passed on from parents to children through their genes. A child usually does not develop symptoms unless both parents carry a thalassemia gene.

If only one parent passes a gene for thalassemia on to the child, then the child is said to have thalassemia trait. Thalassemia trait will not develop into the full-blown disease, and no medical treatment is necessary.

Many families have thalassemia carriers, but the trait often goes undiagnosed because it produces no or few symptoms. Frequently, thalassemia is not diagnosed in a family until a baby is born with it. So if someone in your family carries a thalassemia gene, it's wise to have genetic counseling if you're thinking of having children.

At one time it was believed that the disease affected only people of Italian or Greek descent, but it's now known that many people with thalassemia also come from or are descended from Africa, Malaysia, China, and many parts of Southeast Asia.

Because of a recent pattern of migration from Southeast Asia, there has been an increase in the past decade of thalassemia in North America. Testing for thalassemia is generally recommended for anyone from Southeast Asia with unexplained anemia.

If your doctor determines that your child is at risk for thalassemia, prenatal tests can find out if your unborn child is affected.

Types of Thalassemias

Alpha-Thalassemia

Children with alpha-thalassemia trait do not have thalassemia disease. People normally have four genes for alpha globin, two inherited from each parent. If one or two of these four genes are affected, the child is said to have alpha-thalassemia trait.

A specific blood test called a hemoglobin electrophoresis is used to screen for alpha-thalassemia trait and can be done in infancy. Sometimes, alpha-thalassemia trait can be detected through routine newborn blood screening, which is required in most states in the U.S.

Often, results of the hemoglobin electrophoresis test are normal in people who have alpha-thalassemia trait and a diagnosis of alpha-thalassemia is done only after other conditions are ruled out and after the parents are screened. The disease can be harder to detect in older kids and adults.

Kids who have the alpha-thalassemia trait usually have no significant health problems except mild anemia, which can cause slight fatigue.

Alpha-thalassemia trait is often mistaken for an iron deficiency anemia because RBCs will appear small when viewed under a microscope.

Other cases can cause more severe anemia where three genes are affected. People with this form of alpha-thalassemia may require occasional blood transfusions during times of physical stress, like fevers or other illnesses, or when the anemia is severe enough to cause symptoms such as fatigue.

The most severe form of the disorder is called alpha-thalassemia major. This type is extremely rare, and women carrying fetuses with this form of thalassemia have a high incidence of miscarriage because the fetuses cannot survive.

Beta-Thalassemia

Beta-thalassemia, the most common form of the disorder seen in the United States, is grouped into three categories: beta-thalassemia minor (trait), intermedia, and major (Cooley's anemia). A person who carries a beta-thalassemia gene has a 25% (1 in 4) chance of having a child with the disease if his or her partner also carries the trait.

Recently, some kids have successfully undergone bone marrow transplants to treat thalassemia major; however, this is considered only in cases of severely disabling thalassemia disease. There is considerable risk to bone marrow transplants: the procedure involves the destruction of all of the blood-forming cells in the bone marrow and repopulating the marrow space with donor cells that must match perfectly (the closest match is usually from a sibling).

The procedure is usually done in children younger than 16 years of age who have no existing evidence of liver scarring or serious liver disease. Results have been encouraging so far, with disease-free survival in many patients.

Blood-forming stem cells taken from umbilical cord blood have also been successfully transplanted, and research using this technique is expected to increase. Currently bone marrow treatment is the only known cure for the disease.

Talking to the Doctor

If you know the thalassemia trait exists in your family, it's important to meet with your doctor, particularly if you notice any of the symptoms of thalassemia major — anemia, listlessness, or bone abnormalities — in your child.

If you're thinking of having children, speak with a genetic counselor to determine your risk of passing on the disease.

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Sickle Cell Disease

Sickle cell disease is an inherited disorder in which red blood cells (RBCs) are abnormally shaped. This abnormality can result in painful episodes, serious infections, chronic anemia, and damage to body organs.

These complications can, however, vary from person to person depending on the type of sickle cell disease each has. Some people are relatively healthy and others are hospitalized frequently.

But thanks to advancements in early diagnosis and treatment, most kids born with this disorder grow up to live relatively healthy and productive lives.

A Closer Look at Sickle Cell Disease

The different forms of sickle cell disease are determined by the genes inherited from the person's parents.

Someone who has the disease has inherited a sickle cell gene from each parent (hemoglobin SS disease, also called sickle cell anemia).

A person can also inherit a sickle cell gene from one parent and a different kind of abnormal gene from the other and end up with a different form of sickle cell disease, such as hemoglobin SC disease or hemoglobin S-thalassemia.

Someone who inherits only one sickle cell gene and a normal gene from the other parent will have the sickle cell trait, but not the disease. A blood test can determine whether you have sickle cell disease or carry the sickle cell trait.

People with sickle cell trait don't have sickle cell disease or exhibit any signs of the disorder, but they can pass the gene for the disease to their children. When both parents have the sickle cell trait, there's a 25% chance that a child will have sickle cell disease. But when one parent is carrying the trait and the other actually has the disease, the odds increase to 50% that their child will inherit the disease.

Who Is Affected?

In the United States, hemoglobin SS disease (sickle cell anemia) affects mostly African Americans. Some forms of sickle cell disease may occur, although less frequently, in people with different ethnic backgrounds, such as those whose ancestors came from Mediterranean countries (including Turkey, Greece, and Italy), East India, or Middle Eastern countries.

Causes of Sickle Cell Disease

A child with sickle cell disease has inherited two defective hemoglobin genes. The hemoglobin can take on an abnormal shape, distorting the shape of the red blood cell. The cells change from a normal round, doughnut shape to the elongated shape of a sickle, or the shape of the letter "C."

Unlike normal RBCs, which move easily through small blood vessels, sickle cells are stiff and pointed. The sickle shape means that they have a tendency to get stuck in narrow blood vessels and block the flow of blood. This can cause episodes of pain and can also lead to organ damage because the cells aren't getting enough oxygen.

Sickle cells have a shorter-than-normal life span, which leads to anemia (low red blood cell count). A normal red blood cell lives for about 120 days, whereas a sickle cell lives for only 10 to 20 days.

Diagnosis

Usually, sickle cell disease is diagnosed at birth with a blood test. This can be done in conjunction with other routine newborn screening tests. If a child tests positive on the screening test, a second blood test is usually performed to confirm the diagnosis.

Because kids with sickle cell disease are at an increased risk of infection and other health complications, early diagnosis and treatment is important. Currently, more than 40 states require newborn screening programs for sickle cell disease.

Signs and Symptoms

Symptoms of sickle cell disease vary and range from mild to severe, and symptoms may be less severe or different in children who have inherited a sickle cell gene from one parent and a different abnormal hemoglobin gene from the other.

Most kids with sickle cell disease have some degree of anemia and might develop one or more of the following conditions and symptoms as part of the disorder:

Acute chest syndrome: Inflammation or trapped red blood cells in the lungs cause this syndrome. Signs include chest pain, coughing, difficulty breathing, and fever.

Aplastic crisis: This is when the bone marrow temporarily slows its production of RBCs due to infection or another cause, resulting in a severe drop in the red cell count and severe anemia. Signs include paleness, fatigue, and rapid pulse.

Hand-foot syndrome (also called dactylitis): This painful swelling of the hands and feet, plus fever, may be the first sign of sickle cell anemia in some infants.

Infection: Kids with sickle cell disease are at increased risk for certain bacterial infections. It's important to watch for fevers of 101° Fahrenheit (38° Celsius) or higher, which could signal an infection. Children with sickle cell disease and fever should be seen by a doctor immediately.

Painful crises: These may occur in any part of the body and may be brought on by cold or dehydration. The pain may last a few hours or up to 2 weeks or even longer, and may be so severe that a child needs to be hospitalized.

Splenic sequestration crises: The spleen becomes enlarged by trapping (or "sequestering") the abnormal RBCs. This leads to fewer cells in the general circulation. Early signs include paleness, weakness, an enlarged spleen, and pain in the abdomen.

Stroke: Poor blood flow in the brain can occur when the sickle-shaped cells block small blood vessels. This may lead to a stroke. Signs can include headache, seizures, weakness of the arms and legs, speech problems, a facial droop, and loss of consciousness.

Other possible complications include leg ulcers, bone or joint damage, gallstones, kidney damage, painful prolonged erections in males (priapism), eye damage, and delayed growth.

Treatment

Aside from a bone marrow transplant, there is no known cure for sickle cell disease. Transplants are complicated procedures and aren't an option for everyone. To qualify, a child would need bone marrow from a "matched" donor with a low risk of being rejected. Even then, there are significant risks to the procedure and there's always the chance of rejection of the transplanted marrow.

But even without a cure, kids with sickle cell disease can lead relatively normal lives. Medicines are available to help manage the pain, and immunizations and daily doses of penicillin (an antibiotic) can help prevent infection.

Infection used to cause many deaths in infants with sickle cell disease, but thanks to penicillin and appropriate immunizations, children with sickle cell disease are much more likely to live longer, healthier lives. Although penicillin isn't a cure, it can help prevent life-threatening infections due to bacteria that cause serious infections such as sepsis (a severe infection in the blood), meningitis, and pneumonia.

Infants and kids with sickle cell disease usually require two daily doses of penicillin, as prescribed by their doctors, until they're at least 5 years old (and often older). In addition, they should be fully immunized with the regular childhood vaccinations, including the pneumococcal vaccine and influenza and meningococcal vaccines.

Most doctors also prescribe daily vitamin supplements. Folic acid, in particular, can help a child with the disorder produce new RBCs.

In addition, kids who develop serious complications (such as acute chest syndrome, severe anemia, or stroke) may receive regular transfusions of red blood cells to prevent or treat these complications.

In 1998 the U.S. Food and Drug Administration (FDA) approved the drug hydroxyurea for use in adults with sickle cell disease; while it still has not been officially approved for use in children, it can be used by specialists in some circumstances.

Hydroxyurea increases the amount of fetal hemoglobin in blood cells, which interferes with the sickling process and makes red blood cells less sticky. This helps decrease the number and intensity of painful episodes and other complications. The drug has been proven to decrease pain and other complications in children as well as adults. Research is continuing to determine the drug's long-term effects and safety.

When to Call the Doctor

Seek emergency medical attention immediately if your child develops any of the following:

• fever of 101° Fahrenheit (38° Celsius) or higher
• pain that isn't relieved by oral medication
• chest pain
• shortness of breath or trouble breathing
• severe headaches or dizziness
• severe stomach pain or swelling
• jaundice or extreme paleness
• painful erection in males
• sudden change in vision
• seizures
• weakness or inability to move any part of the body
• loss of consciousness

Caring for Your Child

In addition to the primary care doctor, your child should receive regular care from a hematologist (a blood specialist) or a sickle cell specialty clinic. It's important to share your concerns and discuss any new symptoms or complications with your child's health care team.

Your child also should drink lots of fluids, get plenty of rest, and avoid extreme temperatures.

Most people with sickle cell disease now live into their mid-40s and beyond. A critical time is the first few years of life, which is why early diagnosis and treatment are so important.

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Port Wine Stains

About Port-Wine Stains

About 3 out of every 1,000 kids are born with a port-wine stain — a birthmark that literally looks like maroon wine was spilled or splashed on the skin. Though they often start out looking pink at birth, port-wine stains tend to become darker (usually reddish-purple or dark red) as kids grow.

Port-wine stains (also known as nevus flammeus) can be anywhere on the body but most commonly are found on the face, neck, scalp, arms, or legs. They can be any size, and usually grow in proportion with a child. They often change in texture over time, too — early on, they're smooth and flat but they may thicken and feel like pebbles under the skin during adulthood.

For most kids, port-wine stains are no big deal — they're just part of who they are. And some port-wine stains are barely noticeable, especially when they're located somewhere other than the face.

But port-wine stains often get darker and can sometimes become disfiguring and embarrassing for children. Port-wine stains (especially on the face) can make kids feel self-conscious, particularly during the already challenging preteen and teen years when kids are often more interested in blending in than standing out.

Although port-wine stains won't go away on their own, they can be treated. In fact, laser therapies can make many port-wine stains much less noticeable and give kids' self-esteem a much-needed boost.

Cause

Port-wine stains happen when an area of skin doesn't get any (or an insufficient) supply of nerve fibers, which normally help keep blood vessels narrow. When there's a lack of nerve fibers, small blood vessels (called capillaries) keep expanding, allowing a greater amount of blood to flow into the blood vessels, causing a stain to form under the skin. Birthmarks that form like this are called vascular birthmarks.

Port-wine stains can't be prevented — and they're not caused by anything a mother did during pregnancy.

Diagnosis

Your doctor can sometimes tell if your child has a port-wine stain or a different kind of vascular birthmark by looking at your child's skin.

Vascular birthmarks called macular stains (also known as salmon patches, angel kisses, or stork bites) may resemble port-wine stains. They're faint red marks often found on the forehead or eyelids, the back of the neck, or on the nose, upper lip, or on the back of the head. These often fade on their own by the time a child is 1 to 2 years old.

Port-wine stains also may be confused with hemangiomas. Superficial (formerly called "strawberry") hemangiomas are bright red, raised birthmarks. Deep hemangiomas (once called "cavernous") are a bluish-red, puffier birthmark. Hemangiomas, which are also found on the head or neck, grow quickly during infants' first 6 months or so, but usually shrink back and disappear by the time a child is 5 to 9 years old.

Port-wine stains are usually nothing more than a harmless birthmark that doesn't cause any problems or pain. However, they're sometimes (though very rarely) a sign of other medical conditions.

For example, port-wine stains on or near the eye or on the forehead need to be monitored. That's because they may be associated with a sort of "stain on the brain" (a very uncommon neurological disorder called Sturge-Weber Syndrome that causes problems like seizures, developmental delays, and learning disabilities). Stains on the eyelids may also, rarely, lead to glaucoma — increased pressure inside the eye that can affect vision and lead to blindness if left untreated.

If there's a concern about the location of a port-wine stain or accompanying symptoms, your doctor may order tests (such as eye tests or imaging tests like an X-ray, CT scan, or MRI) to see what's going on and rule out an underlying problem. If your child has a birthmark anywhere on the body it's important for your doctor examine it to see what type it is and what kind of monitoring and treatment it needs, if any.

Treatment

Although some port-wine stains are small and hard to see, some can be really upsetting for kids, especially if they're large, dark, or on the face. And any birthmark can take a toll on a child's self-confidence, no matter how large or small the mark might be.

The good news is that lasers (highly concentrated light energy) can make many kids' port-wine stains much lighter, especially when the birthmark is on the head or neck. Dermatologist or plastic surgeons usually give several treatments with the laser of choice for port-wine stains called a "pulsed-dye" laser.

Laser treatment is often started in infancy when the stain and the blood vessels are smaller and the birthmark is much easier to treat. But that doesn't mean laser treatments can't help older kids or teens, too — it's just that the longer someone has had the stain, the harder it might be to successfully treat it.

Laser therapy doesn't hurt a lot, but can be uncomfortable. During the treatment, kids can usually be given an anesthetic (given as a shot, spray, or ointment to numb the area to so it doesn't hurt as much). Young kids may also be given general anesthesia to help them sleep or relax during the procedure. After treatment, the area might be swollen and bruised at first, but it will be back to normal in 7 to 10 days.

For port-wine stains that have become bumpy, thick, or raised, doctors sometimes need to use another type of laser or surgery. Port-wine stains can also develop grape-like growths of small blood vessels called vascular blebs — these aren't usually cause for concern but they often bleed and may need to be removed.

In the past some people have opted for other treatments, too (like freezing, tattooing, even radiation). But these aren't as effective — or as safe — as laser therapy. Laser surgery is the only treatment that works on port-wine stains with less risk of damaging or scarring the skin. Sometimes, though, laser treatments may make the area look lighter or darker than normal, although this usually is just temporary.

And laser treatments may not get rid of the birthmark entirely (though a few kids' birthmarks do disappear altogether after treatment). Plus, over time the birthmark may come back and need to be retreated.

For a small number of kids, laser treatment might not work at all. Every child's port-wine stain is different, so whether or not the treatment works well will be different for each child, too.

Care

Port-wine stains can get very dry sometimes, so it's important to use a moisturizer. Also be sure to call the doctor if your child's port-wine stain ever bleeds, hurts, itches, or becomes infected. Like any injury where there's bleeding, make sure to clean the wound with soap and water and use a gauze bandage to place firm pressure on the area until the bleeding stops. If the bleeding doesn't stop, call your doctor.

If your child's port-wine stain has been treated with laser surgery, avoid rubbing or scratching the area, and gently cleanse it with lukewarm water. Your doctor may prescribe an ointment to aid in healing and help prevent infection.

Helping Kids Cope

As with any birthmark, port-wine stains (especially on the face) can make kids feel different and insecure about how they look. If it's clearly visible, people might ask questions or stare, which can be hurtful for both you and your child. Even at a young age, kids watch how their parents respond to these situations and take cues about how to cope with others' reactions.

Practice responses so your child will feel more prepared when asked about it. It can help to have a simple, calm, nonchalant explanation ready like, "It's just a birthmark. I was born with it."

Talking simply and openly about a birthmark with kids makes them more likely to accept it as just another part of themselves — like their height or eye color. It's also important, emotionally, for kids to be around supportive family and friends who treat them like everyone else.

Of course, it's still natural for kids to want to do whatever they can to minimize a birthmark. In addition to laser treatments, special cover-up makeup can camouflage the stain and make living with it a little easier.

Still, kids with port-wine stains (or any birthmark, really) need to know that they're no different from other kids. If anything, it may help to tell your child that kids born with a port-wine stain are unique in a good way — it's a special, colorful part of themselves that few other people have.

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Mitral Valve Prolapse

Mitral valve prolapse (MVP), a very common heart condition, occurs when one of the heart's valves doesn't work properly. MVP can be frightening because it involves the heart and can cause sharp chest pains, but it isn't a critical heart problem or a sign of other serious medical conditions.

Because MVP often doesn't produce any symptoms or interfere with everyday life, in many cases it isn't diagnosed until adulthood. But with kids who are diagnosed, it's important to know what the symptoms are, so that you can distinguish them from signs of any other more serious heart issues.

What Is the Mitral Valve?

To understand mitral valve prolapse, it's helpful to review some basics about the way a healthy heart works.

• The heart is made up of four distinct chambers: two atria (the two upper chambers) and two ventricles (the two lower chambers).
• During circulation, blood flows from all over the body into the heart's right atrium.
• From there the blood travels to the right ventricle, which pumps the blood to the lungs to receive oxygen.
• Once the blood has been infused with oxygen, it returns from the lungs to the heart's left atrium.The oxygen-rich blood then passes into the left ventricle, which pumps it out to the body through a large blood vessel known as the aorta.

The mitral valve is located between the left atrium and the left ventricle and helps control the flow of blood as it passes from the left atrium into the left ventricle. The valve has two flaps of tissue — known as leaflets — that open and close together like a pair of swinging doors. Each time the heart beats, the left ventricle pumps blood out to the body and the flaps of the mitral valve swing shut to prevent the blood in the ventricle from flowing backward into the left atrium.

In cases of MVP, one or both of the mitral valve's flaps bulge back into the atrium when they are shut, a bit like a balloon. This may occur because one of the flaps is abnormally shaped or a little too large.

In some cases, when the flaps do not close evenly, blood is allowed to leak back into the left atrium. This is called mitral regurgitation. A tiny amount of mitral regurgitation is normal. In some cases, there's more leakage, which can cause a heart murmur a whooshing sound between the normal lub-dub of the heartbeat. (Because of these sounds, MVP is sometimes called click-murmur syndrome, floppy valve syndrome, or balloon mitral valve.)

In most cases, the cause of MVP is unknown. Sometimes kids are born with the condition. In other cases, it develops after some sort of inflammatory condition, like endocarditis, an inflammation of the inner lining of the heart, or rheumatic fever, an inflammation that can affect the joints and the heart.

MVP may be diagnosed in people who have other health conditions that affect the body's connective tissue, such as Marfan syndrome. Some research has shown that kids with MVP may be more likely to also have an arrhythmia, an irregular heartbeat.

Signs and Symptoms

Many people with MVP have no symptoms. In some cases, though, the flaps of the mitral valve make a clicking sound when they close. A doctor may be able to hear this noise when listening to the heart with a stethoscope. Someone who has MVP and mitral regurgitation may also have a heart murmur, a sound caused by some blood moving backward into the left atrium. When a click and a murmur are heard together, the click happens first, as the flaps close, followed by the murmur as the blood leaks back into the atrium through the improperly closed valve.

Kids with MVP also may experience:

• dizziness or lightheadedness
• fatigue
• shortness of breath or trouble breathing after exertion
• a feeling that the heart is skipping beats or beating very quickly
• chest pain that comes and goes

The chest pain is often sharp but it can vary from person to person. Learn to recognize the symptoms, and call your doctor if your child has chest pain that:

• consistently occurs during physical exertion (exercise or sports)
• creates pressure and a crushing sensation
• occurs with other symptoms (palpitations that last more than a few seconds, dizziness, fainting, or shortness of breath)

Diagnosis and Treatment

In most cases, MVP is diagnosed during a routine exam when a doctor listens to the heart with a stethoscope and hears a different sound. If the doctor hears a click or a murmur that indicates MVP, your child may be referred to a pediatric cardiologist, a doctor who specializes in diagnosing and treating heart conditions in kids.

The cardiologist will perform a thorough physical exam and listen to the heart. Then he or she may order tests — including an echocardiogram (echo) and an electrocardiogram (EKG or ECG) — to find out what's causing the sound. An echo uses sound waves to create a picture of the heart and its blood flow, and an EKG records electrical activity in the heart. If a child has MVP, the bulging valve flaps will probably be seen on the echo when the heart beats.

Kids who are diagnosed with MVP usually require no medical treatment. In some cases where MVP causes significant regurgitation, blood pressure medication is prescribed to control how hard the heart muscle must work. (With blood leaking back into the atrium, the heart works harder to pump the normal amount of blood out to the body.) A child who has an arrhythmia in addition to MVP may need to take medicine to help regulate the heart's rhythm. In rare cases, surgery may be done to repair a very leaky mitral valve.

Caring for a Child With MVP

Kids with MVP who have no other medical conditions typically require no special care. A child with MVP who plays competitive sports will be able to continue doing so as long there's no mitral regurgitation or active symptoms from the MVP. A child who does have regurgitation or symptoms will have to be cleared by the doctor to participate in sports. This may involve some additional tests.

Although any heart condition can be frightening, mitral valve prolapse likely will not have any impact on your child's everyday life and activities. If you have any questions or concerns, speak with your doctor.

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Heart Defect in Child

It can be frightening to learn that your child has a congenital heart defect (one that's present at birth). But congenital heart disease is a relatively common condition that affects almost 1 in every 100 newborns in the United States. Medical knowledge, technology, and experience can offer accurate diagnosis and treatments that allow nearly every form of congenital heart disease to be treated.

Most defects today are treated with surgery, catheter procedures, and sometimes medication. Thanks to advances in techniques, surgical and heart catheterization procedures can treat congenital heart defects that once could not be treated effectively.

Cardiac surgery and cardiac catheterizations are now being performed on younger children — in fact, it's common for them to be done during infancy or even the newborn period. This has resulted in many long-term advantages for these children.

Although nothing can be guaranteed with 100% certainty, most kids with heart problems can enjoy happy and healthy futures.

Why Do Some Babies Have Congenital Heart Defects?

The human heart begins to form as a single tubular structure at about the fourth week of pregnancy. By the eighth week, this tube will gradually increase in length, eventually twisting upon itself. A wall, or septum, grows to divide the upper (atrial) and lower (ventricular) chambers into left and right sides. Four valves made of tissue develop, which will keep blood moving forward through the cardiac chambers, lungs, and body as the heart pumps.

Because the placenta (and not the fetus' lungs) does the work of exchanging oxygen and carbon dioxide, it's possible for even severe developmental abnormalities of the heart to exist without causing difficulties for the fetus. Such abnormalities may become important only after the fetus' circulation transitions to the newborn state after birth (when the umbilical cord is clamped at the time of delivery, the placenta is no longer involved in the baby's circulation).

The newborn becomes dependent upon the lungs and circulatory system for the oxygen and blood flow needed to survive outside of the womb. The right side of the heart receives oxygen-poor blood flowing back from the body and pumps it to the lungs, where the circulating blood picks up more oxygen. The left side of the heart receives oxygen-rich blood from the lungs and pumps it out to the body.

Multiple genetic and environmental factors interact to alter the development of the heart during the early stages of a fetus' development (the first 8 to 9 weeks during pregnancy). Sometimes, the cause of a congenital heart defect is known. Certain environmental exposures during the first trimester of pregnancy may cause structural abnormalities (including anticonvulsant medications such as phenytoin, the dermatologic medication isotretinoin, or lithium salts for manic-depressive illness). Uncontrolled diabetes, alcohol or drug abuse, or exposure to industrial chemicals during pregnancy can also increase the risk of congenital heart malformations. But most of the time, the specific cause of congenital heart disease is not known.

Over the past 25 years, advances in ultrasound imaging techniques have led to the availability of sophisticated tools such as fetal echocardiography, making it possible for many congenital heart malformations to be diagnosed as early as the 12th to 20th week of pregnancy. The use of such imaging has reassured many parents-to-be that their baby's heart is normal. For others, it has offered an opportunity to know long before the birth that there's a malformation. This gives the family and doctors the ability to make well-informed decisions about the best treatment options.

Signs and Symptoms of Congenital Heart Disease

After birth, the first sign of congenital heart disease is often the presence of a heart murmur. A murmur in itself is not a disease, but simply a sound. As the heart pumps blood, it sometimes creates vibrations that are heard through the doctor's stethoscope as a noise, or murmur.

Not all heart murmurs are signs of abnormalities — in fact, heart murmurs usually don't indicate the presence of any heart problem. Sometimes, a doctor can determine with the stethoscope alone whether a particular murmur is a sign of heart disease. In other cases, additional tests — such as chest X-rays, electrocardiograms (EKGs), or echocardiograms — are performed to help determine the exact nature of a murmur.

Although many children with minor forms of congenital heart disease may not require any treatment, some can have serious symptoms early on that will require medical or surgical treatment within the first year of life. One such symptom can be breathing difficulties from lung congestion. This is usually the result of excessive blood flow from the left side to the right side of the heart through abnormal connections between the two sides of the circulation, such as holes in the heart (as in ventricular septal defect, atrial septal defect, atrioventricular canal, and patent ductus arteriosus).

Or the congestion could be the result of obstructions to blood flow on the left side of the heart, resulting in a backup of blood in the blood vessels returning blood from the lungs (such as in aortic stenosis, coarctation of the aorta, and hypoplastic left heart syndrome). The shortness of breath in these babies may interfere with their ability to feed and may result in an inability to gain weight adequately. Such babies may require medical treatment or a procedure such as surgery or cardiac catheterization within the first weeks of life.

Other symptoms of congenital heart disease relate to an inadequate amount of oxygen carried within the blood. These infants usually appear to have blue skin, a condition called cyanosis. This can be due to an obstruction of blood flow to the lungs (such as in tricuspid atresia or pulmonary atresia) or due to a hole within the heart that allows oxygen-poor blood to flow from the right to the left side of the heart and out to the body (such as in total anomalous pulmonary venous return or Ebstein's anomaly). It can also be related to an abnormal positioning (transposition) of the arteries leaving the heart. In any of these cases, less red oxygenated blood comes from the lungs and more blue unoxygenated blood is carried to the body, causing the blue skin color.

Treatment for Congenital Heart Defects

Many heart abnormalities (including patent ductus arteriosus, ventricular septal defect, truncus arteriosus, atrioventricular septal defect, tetralogy of Fallot, and transposition of the great arteries) can be corrected with a single operation in early infancy. More complex abnormalities (including hypoplastic left heart syndrome and tricuspid atresia) may require a series of two or three operations beginning in the newborn period and completed at approximately 3 years of age. With most complex abnormalities, the children spend the majority of their time in the care of their parents at home, with occasional visits to the pediatric cardiologist (a heart specialist) as well as to the child's primary care doctor.

Less invasive procedures done in the cardiac catheterization laboratory, rather than the operating room, may be used to treat some conditions. Such treatments may include balloon angioplasty or valvuloplasty to relieve an obstruction of a blood vessel (such as in coarctation of the aorta) or a valve obstruction (such as in pulmonary or aortic stenosis). In these procedures, a pediatric cardiologist inserts a catheter, a thin plastic tube with a special balloon attached, into a blood vessel. The balloon is then inflated to stretch open the narrow area of the blood vessel or heart valve. Another procedure called transcatheter device occlusion may be used to close abnormal openings or holes within the heart or blood vessels (such as in patent ductus arteriosus, atrial septal defects, and ventricular septal defects) without requiring surgery.

Some abnormalities, such as small- or moderate-sized ventricular septal defects, may close or decrease in relative size as your child grows. While waiting for the hole to close, the doctor may prescribe medicines for your child, which some kids also need to take after surgery.

Whether treated surgically or medically, your child will need to regularly visit a pediatric cardiologist. At first, these appointments may be fairly frequent (perhaps every month or two), but after treatment, they may be cut back, sometimes to just once a year. Your child's cardiologist may use tools like X-rays, electrocardiograms, or echocardiograms to monitor the defect and the effects of treatment.

Preventing Infection

Kids with congenital heart disease are at risk for the development of bacterial endocarditis, an infection of the tissue that lines the heart and blood vessels. This serious illness requires prolonged treatment with intravenous antibiotics in a hospital setting.

Any time a child has a surgical procedure, the surgical incision can introduce bacteria into the bloodstream. Although the white blood cells of the body usually destroy these germs before an infection can occur, the rough surfaces that may be present within a congenitally malformed heart may allow some germs to survive and reproduce, resulting in an infection of the heart lining.

Fortunately, the risks of bacterial endocarditis can be greatly reduced by taking a dose of specific antibiotics before any scheduled medical procedures that have a risk for introducing germs into the bloodstream. This includes dental work and certain types of surgery.

However, some parents misinterpret this to mean that dental visits and cleanings are potentially dangerous and that they can avoid risk by avoiding the dentist. This is incorrect! In fact, the riskiest thing to do is to ignore dental health, which may allow teeth to develop cavities and gums to become infected. Along with taking antibiotics correctly, it's important for children with heart defects to take good care of their teeth by brushing and flossing properly. Your child should begin visiting a dentist as early as possible, and those visits should be as frequent as the dentist recommends.

Taking measures to prevent bacterial endocarditis is recommended for kids with almost all congenital cardiac malformations (except in the case of isolated ostium secundum and atrial septal defect). Discuss these preventive measures with your child's doctor, pediatric cardiologist, and dentist. Local chapters of the American Heart Association (AHA) or your pediatric cardiologist can give you free wallet cards detailing the recommended antibiotics and their appropriate doses.

If You Suspect a Problem

Although sudden serious downturns during or after cardiac treatment aren't common, you should watch for certain signs that could signal a need for medical attention. If your child appears to be working harder than normal to breathe, call your child's doctor right away.

Other signs that warrant immediate medical attention include:

• a bluish tinge or color (cyanosis) to the skin around the mouth or on the lips and tongue
• an increased rate of breathing or difficulty breathing
• poor appetite or difficulty feeding (which may be associated with color change)
• sweating while feeding
• failure to thrive (failure to gain weight or weight loss)
• decreased energy or activity level
• prolonged or unexplained fever

Call the doctor immediately if your child has any of these symptoms.

Caring for Your Child

Parenting kids with heart defects includes learning about basics like feeding, giving medicines, and watching for signs of trouble, but it also involves encouraging kids to become involved in their own care.

Because most congenital heart defects are now treated during infancy, it's often necessary to explain to an older child what happened in the past. When your child is old enough to understand, explain why he or she has a surgical scar, needs to take medication, or needs to visit the pediatric cardiologist. Describe the treatment in a way your child can understand and don't try to hide the details.

If kids believe they have a role in their care, they're likely to be more confident and positive. Your doctor may be able to suggest ways to discuss these issues.

Participation in some physical activities may be limited, but kids can still play and explore with friends. Always check with your child's cardiologist about which activities your child should or should not be doing. Certain competitive sports may be restricted, for example.

Although it's tempting for parents to be overly protective, sheltering kids can make them feel isolated and stigmatized — which may do more harm than a heart defect in the long run. So do everything you can to make sure your child leads as normal a life as possible.

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