The Challenges of Raising Children With FH

As a parent, you only want to give your children the best. Unfortunately, when it comes to hereditary high cholesterol, whether or not you pass it on to your child it is out of your control. Familial Hypercholesterolemia (FH) is a genetic condition.  If one parent with FH passes the gene to his or her child, the child will also inherit the disorder.

Assessing Your Child’s Risk

Children with one parent with FH have a 50 percent chance of inheriting heterozygous FH (HeFH).  If both parents have FH, their children are at risk for a more serious form of the disease, homozygous FH (HoFH). In addition to the 50 percent chance of inheriting one FH gene from one parent, these children have a 25 percent chance of inheriting two FH genes – one from each affected parent.  Homozygous FH is a more aggressive form of the disorder, according to the National Institutes of Health. It is important that both parents be tested to determine if just one or both of them have FH.

Both types of FH lead to early heart disease caused by narrowing or blocked arteries and plaques, including heart attacks, strokes, bypass, angina, stents, and congestive heart failure. Children with HoFH can experience a serious cardiac incident or even death before the age of 20. Identifying and treating FH as early as possible is critical to the successful management of the condition to slow down or even prevent progressive heart disease.

Treating FH in Children

While you may be reluctant to start your child on medication, prescription medication is recommended for the treatment of hereditary high cholesterol to reduce LDL cholesterol, even in children. Unlike high cholesterol that is the result of an unhealthy lifestyle, FH is a genetic disorder that usually can’t be resolved through lifestyle changes alone.  Each family needs to consider the information available and make their own decision about when and how to treat FH, in consultation with their healthcare provider.

People with FH are exposed to very high levels of LDL cholesterol from birth, and by the time they reach the age of 12 many will already have measurable atherosclerosis. Cholesterol-lowering medications can dramatically reduce the risks of hereditary high cholesterol. Observational studies from Europe have found that long-term cholesterol-lowering therapy with statins decreases the excess lifetime heart disease risk associated with FH.

Current recommendations from both the National Lipid Association (NLA) and American Academy of Pediatrics (AAP) recommend beginning cholesterol lowering medications at around the age of 8 for children with FH. Several statin medications have been approved by the FDA for use in children 8 or 10 years and older. Your child’s healthcare provider might also suggest other cholesterol lowering medications.

Getting Your Child To Eat a Healthy Diet

It can be a challenge to get children to eat healthy foods, especially if they are teenagers. Keep low-fat snacks readily available, such as fruit and cut-up vegetables. Sneak healthy foods into meals, such as incorporating whole wheat flour into recipes, soy milk or blended silky tofu into soups and sauces. Model healthy behavior by eating a balanced diet low in saturated and trans fats. Keep your home stocked with nutritious choices, avoiding pre-packaged snacks and carefully reading food labels to watch out for hidden trans fats and hydrogenated oils.

Making Exercise a Habit

Today’s children are surrounded by screens and technology, including smart phones, computers, televisions and video game systems. Getting them to be physically active is more difficult than ever, but regular exercise is important for heart health, especially for children with inherited high cholesterol. Make a family commitment to get up and move for 30-60 minutes most days of the week, whether it’s by taking daily walks together, playing family soccer in the backyard, or turning up the music and having a dance-off in the living room.

Eliminating Exposure to Tobacco Smoke

If you are a smoker, quit. Smoking is a major contributor to cardiovascular conditions and strokes, and children with FH already face a 20 times greater risk of developing it. Children model their parent’s behaviors, and one of the best ways to ensure that your children remain life-long non-smokers is to be a non-smoker yourself. According to the U.S. Department of Health and Human Services, exposure to secondhand smoke also damages blood vessels and can trigger a heart attack or stroke, increasing the risk in children who are already vulnerable to cardiac incidences due to their inherited high cholesterol.

Dealing with HoFH

Children with homozygous FH (HoFH) inherit two abnormal chromosomes, one from each parent. HoFH is a more serious and aggressive form of FH. These children are often diagnosed at around the age of two, when they develop xanthomas (orange cholesterol deposits on the elbows, buttocks, legs, ankles, on the tendons of the fingers and between the fingers). In children with HoFH, the LDL cholesterol is often over 500 and can be as high as 1000 mg/dL.

If your child has HoFH, he or she will require immediate cholesterol lowering medications. Your child’s response to the medications depends on their particular genetic mutation. Some children will respond quite well to high dose statins combined with exetimibe (Zetia®) and a bile acid sequestrant. Other children will require more heroic measures such as LDL-apheresis. This is a procedure in which LDL cholesterol is physically removed from the blood every week. Children with HoFH require close follow-up with a cholesterol specialist.

Children with untreated HoFH develop very early atherosclerosis, often experiencing heart attacks in their teens. It is therefore crucial to treat children with HoFH early and aggressively. Because the aortic valve (all the blood in the heart passes through this valve on its way to the body) can develop cholesterol plaque in HoFH, children with this diagnosis often undergo much more involved heart evaluations than do children with HeFH. This can include heart catheterizations and echocardiograms. The future is bright for children with HoFH – there are many new treatments being studied which have the potential to profoundly lower their LDL level profoundly.

Visit the website of the Family Heart Foundation at or call 626-465-1234 to learn to more about familial hypercholesterolemia.

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