Does Hemophilia Mean Brittle Bones? Part 2

By Paul Clement

In part one of this two-part series on bone health, we discussed osteoporosis and how it causes bones to become weak and brittle. We also reviewed some risk factors for developing osteoporosis—and having a bleeding disorder is a risk factor for developing osteoporosis. But just because you have bleeding disorder does not mean you are destined to develop osteoporosis—you can do many things to lower the risk of developing osteoporosis or to lessen its severity.1

Front view of a leg bone with cut section showing bone marrow.

So, what can you do? First, if you smoke, stop! If you drink several alcoholic beverages or three or more cola soft drinks a day, reduce your consumption of these beverages. Eat a healthy diet containing adequate amounts of calcium and vitamin D. (Many people are deficient in vitamin D, which helps the body absorb and retain calcium and phosphorus. Check online for daily calcium and vitamin D requirements and recommended foods). And exercise!

Bone growth changes in response to stress and strain applied to the bones. If you are sedentary, such as sitting around all day or sitting in an office, your bones are not subjected to regular mechanical stress. So the remodeling process tilts towards resorption and bone loss occurs. The opposite is also true. When bones are subjected to regular stress or mechanical loading, such as when walking or running, then over time, the remodeling process tilts towards new bone formation, which keeps your bones healthy and can help prevent, delay, or reduce the severity of osteoporosis.

Exercise is key! Healthy lifestyle choices are important, but they alone cannot prevent osteoporosis unless you add exercise. And not just any exercise routine—although swimming and bicycling tone muscles and provide a great cardio workout and are often recommended for people with hemophilia, they do not stress the bones sufficiently to promote new bone formation. To develop and maintain healthy bones you need to engage in regular weight-bearing exercise, such as brisk walking, dancing, aerobics, stair climbing or jogging as well as resistance exercises such as light weight lifting or use of elastic bands. (Resistance exercises are especially important for bones of the upper body, including the arms and shoulders.) Note that higher-impact weight-bearing activities, such as brisk walking or jogging, provide more benefit to bone health than lower-impact activities, such as a leisurely stroll. And although exercise is crucial in maintaining bone health throughout your lifetime, the greatest benefit is seen in childhood through age 20, when the skeleton is more responsive to exercise than in adulthood and old age, when bones become less sensitive to exercise. Greater bone strength when you are young pays lifetime benefits in the form of greater bone strength over a lifetime.

So, if you have a teenager who likes to watch TV or play video games for hours on end, get them outside and exercising. If they don’t play sports, give them chores that make them walk or carry things. Walking the dog works! Their bones will thank you!

Of course, if you have not engaged in any exercise for a while or have painful joints, you should discuss your health status with your healthcare provider before starting any new exercise program. A physical therapist associated with a hemophilia treatment center is an excellent resource to help you get started.

  1. There are no outward signs that you have osteoporosis—until you break a bone. The U.S. Preventive Services Task Force recommends screening for osteoporosis in women 65 years or older and men age 70 years and older, and anyone who has broken a bone after age 50 years. However, if you have hemophilia, you may already be on the path to osteoporosis in your 30s. Because of this, it is often recommended that people with hemophilia have a bone density scan earlier rather than later. (A bone density scan is a simple and fast procedure which measures bone mineral density using a special type of X-ray scan called dual energy X-ray absorptiometry, or DEXA scan.)

Does Hemophilia Mean Brittle Bones?

By Paul Clement

Many studies have linked having hemophilia to a significantly increased risk of developing osteoporosis, or “holey bones” (osteo means “bone” and porosis means “porous”). Osteoporosis causes bones to become weak and brittle—so brittle that a fall or even mild stresses such as bending over or coughing can cause a fracture. But just because you have hemophilia does not mean you are destined to develop osteoporosis—you can do many things to lower the risk of developing osteoporosis or to lessen its severity.

Bones are living tissue and are constantly being “remodeled”—old bone is removed (resorption) and new bone is deposited (ossification). This remodeling process is relatively rapid when we are young: a baby’s entire skeleton may be replaced in a year. It slows as we age: about 10% of an adult’s skeleton is replaced in a year. Until age 25 to 30, more new bone is added than is removed and bones become more dense. From about age 30 to about age 50, the bone remodeling process is balanced, with new bone formation equaling the amount of bone removed. From about age 50 onwards, the remodeling process tilts towards resorption, and more bone is removed than new bone formed, causing your bones to become less dense and weaker as you age. (This is especially true when women enter menopause; the drop in the hormone estrogen—a key regulator of bone metabolism—significantly speeds bone loss, increasing the risk of osteoporosis.)

At a microscopic level, bones have an internal structure with many holes, similar to a honeycomb, which makes them rigid yet relatively light. In addition to allowing us to move by providing a rigid framework for the attachment of muscles, bones also protect our organs, make blood cells, and serve as a reservoir a number of minerals, especially calcium and potassium (bones contain 99% of the calcium in the human body). Calcium and potassium are critical for the functioning of your body and the concentration of these minerals in the blood must be tightly regulated: if your diet contains insufficient levels of these minerals, then your body pulls them from your bones, which is why a balanced diet is important. In osteoporosis, resorption pulls minerals from your bones faster than new bone is formed, leaving the holes in your bones larger (thus the name of the disease) and leaving a lower concentration of minerals, which causes our bones to become less dense: all of which weaken the bones, making them more prone to breaking.

What are risk factors for developing osteoporosis?

Many things—some we can control, others we cannot change—can tip the remodeling process towards bone removal/resorption, increasing the risk of developing osteoporosis. Some risk factors include family history of osteoporosis, older age, being female, being post-menopausal and not taking estrogen, being small/thin boned, and being of Caucasian or Asian ethnicity. Some diseases increase the risk of osteoporosis, including having type 1 diabetes, rheumatoid arthritis, chronic kidney or liver disease (such as HCV infection) and anorexia nervosa. And some medications are known to cause bone loss, including corticosteroids such as Prednisone, various anti-seizure medications such as Dilantin and certain barbiturates, and high-dose thyroid replacement drugs. And there are life-style choices that we can control that affect your risk of developing osteoporosis, including smoking, heavy alcohol consumption, being sedentary, and a diet low in calcium and vitamin D.

And having hemophilia—both hemophilia A and B of any severity—as well as being a carrier of the gene for hemophilia or having von Willebrand Disease, are all risk factors for developing osteoporosis, and for those with severe hemophilia A, developing it an early age. For many years it was believed that the increased risk of osteoporosis in people with hemophilia was primarily due a more sedentary lifestyle as a result of joint bleeds/joint pain and avoidance of exercise or sports that might cause a joint bleed. However, in recent years it has been found that factor VIII plays a role in bone health by binding to a protein that inhibits bone resorption, allowing resorption to proceed at a faster rate. And it is also speculated that thrombin, an enzyme created by the clotting process that helps form a fibrin clot, may also play a role in bone health. Bone metabolism is complex, and the many processes involved are not fully understood, but it is apparent that factor VIII, and perhaps other clotting factors, play a role in maintaining health bones. But having a bleeding disorder does not mean you are destined to develop osteoporosis.

Next week: Part 2! How to maintain healthy bones

PS Order this book for your kid with hemophilia, by Dr. Roy A. Meals!

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