LIVING WITH CYSTIC FIBROSIS Nutrition Bone Health and Cystic Fibrosis People with cystic fibrosis (CF) are living longer than ever. As people CONTRIBUTING AUTHORS AND REVIEWERS age, bones get weaker and break easier. This pamphlet talks about bone Danett Guy, RN disease in CF and different ways that it can be prevented with nutrition Nurse Coordinator, Adult CF Center and exercise. Vanderbilt University Nashville, TN Bone Disease and CF Karen Maguiness, MS, RD, CSP Two common bone diseases are osteoporosis* Dietitian, Pediatric Pulmonology (ostea-o-pour-o-sis) and osteopenia Riley Hospital for Children (ostea-o-pea-knee-a). People with CF are prone Indianapolis, IN to osteoporosis and osteopenia. Bone health is Melanie Meyers, RN vital for health and normal growth. Coordinator, Adult CF Center Bones have a thick outer shell and a strong University of Cincinnati inner mesh. This mesh looks like a “honeycomb.” Cincinnati, OH It contains collagen, protein, calcium and Normal Osteoporosis Michelle Noschese, MS, other minerals. There are blood vessels and Image Copyright 2001, David W. Dempster, PhD. CRNP, CDE bone marrow in the other spaces of the bones. Nurse Practitioner Having holes in your bones does not make them osteoporosis make bones more fragile and prone Leslie Hazle, MS, RN, CPN weaker! As with bricks or cement blocks, holes to break or fracture. Director, Patient Resources strengthen bones. Since people with CF can have low bone Cystic Fibrosis Foundation Some of the minerals that build bones are minerals, they are prone to osteopenia. In CF, Bethesda, MD calcium, phosphorus, magnesium and fluoride. it can happen at any age but becomes more With osteopenia, bones do not have enough of common in the later teen and adult years. these minerals. This is often called low mineral Fractures hurt. A broken chest bone makes it density. This makes the bones weak and brittle. hard to breathe deep and do airway clearance. With osteoporosis, the holes you normally have in Broken bones affect lung health. your bones get too big. This is why osteoporosis Osteopenia or osteoporosis can keep people means “porous bones.” Both osteopenia and from getting organ transplants. Poor bone health can cause big problems after a transplant. *Brief definitions of the words that appear in bold can be found on page 4. Nutrition: Bone Health and Cystic Fibrosis Causes of Bone Disease in CF There are many reasons why people with CF SOME MEDICINES CAN AFFECT BONE HEALTH get osteoporosis or osteopenia. It is important Ask your CF care team how your or your child’s to remember nutrition, lung disease and bone medicines or supplements affect bone health. health are all related. When people do not feel • Steroids can help lungs but hurt bones. Long- Bone Disease: Causes well, they eat less. and Risk Factors term steroid use can make bones thin and Poor food digestion, called malabsorption, weak. Your CF care team should monitor them. causes malnutrition. With malabsorption, the • Low body weight • Depo-Provera® (i.e., medroxyprogesterone body may not get the vitamins and minerals acetate) is birth control medicine. It may raise • Low vitamin D it needs. Vitamin D, a fat-soluble vitamin, and your bone disease risk. It should be avoided if calcium are vital to bone health. The right • Low calcium intake possible. amount of pancreatic enzymes and food are • Malabsorption necessary for a good diet, good body weight and • Megace® (i.e., megestrol acetate) is used to • Not enough pancreatic healthy bones. Low body weight can lead to low increase appetite. It may affect testosterone enzymes bone density and fractures. levels and raise the risk of bone loss. If you are taking Megace®, your CF care team should • Lack of exercise, Poor nutrition and lung disease can slow puberty monitor its use. especially weight- too. A lot of bone is made during the teen years. • Medicines that suppress the immune system bearing exercise Since this is the bone you will have for the rest that are used after transplants raise the risk • Tobacco, alcohol and of your life, slowed puberty can lead to less bone being made. This sets the stage for future bone of osteoporosis. caffeine use disease. • Aluminum-containing antacids can block • Moderate to severe lung Prompt diagnosis and care for CF-related calcium absorption. Avoid them. Some illness diabetes (CFRD) is vital for bone health. Diabetes antacids provide calcium. Ask your CF care • Chronic infections treatment makes it possible for the body to use team or dietitian which antacids to take. • Chronic bone and joint the vitamins and minerals in food for building • Herbal medicines and “natural supplements” swelling and maintaining bones. can block the absorption of nutrients. Talk with your CF care team or dietitian before • CF-related diabetes Lung infections cause inflammation. This can also lead to bone loss. Treating illness early may using them. • Organ transplant lessen bone loss. Talk with your CF care team and dietitian to learn • Long-term steroid use When people are sick and inactive, muscles and about bone health and the risk of bone disease. • Low sexual hormones bones weaken. Activity helps bones get strong They can teach you how to keep bones healthy and stay strong. and strong. Caffeine, alcohol, tobacco and some medicines can make you prone to bone disease too. REGULAR EXERCISE – Weight-bearing exercise and resistance exercise like walking, jogging or weight lifting are great for bone health. Exercise early in life may build more bone for life. Your CF care team can help you build an exercise program. GOOD NUTRITION – A balanced diet, enough Bone Health DO’s... calories, the right nutrients and enough pancreatic enzymes are vital for growth and health. ALCOHOL AND CAFFEINE –They lower bone density. CARBONATED DRINKS – Instead of sodas, drink whole milk for calcium, vitamin D and calories. TOBACCO – Smoking hurts your lungs and bones, Bone Health DON’Ts… causing osteoporosis and leading to fractures. 2 LIVING WITH CYSTIC FIBROSIS Screening Your CF care team will screen for osteoporosis or VITAMIN D osteopenia by checking height and weight. They Vitamin D helps our bodies absorb calcium to will track pubertal development. Some yearly make bone. Fortified milk and cereals, egg yolks Take Vitamin D lab tests screen for bone disease. If you take and fatty fish are good sources of vitamin D. Every Day! extra vitamin D because of low levels, lab tests People with CF don’t easily absorb vitamin D will be done more often to check vitamin D levels. from foods. They should take vitamins made just • 11 years and older, You may have a dual-energy X-ray absorptiometry for people with CF like Vitamax™, AquADEK® or take 800-2000 IU. (DEXA) scan. This checks bone mass or density SourceCF®. These vitamins have water-soluble • 1-10 years, take and can diagnose osteoporosis. It is painless. vitamin D, which is easier to absorb. 800-1000 IU. It uses an X-ray to check bone thickness Vitamin D levels in the blood should be checked • 12 months and younger, throughout the body. It can find weak bones once a year and after taking extra vitamin D as take 400-500 IU. before they break. It can predict the chance of prescribed. If the amount of vitamin D in your future breaks for you or your child. All people • Make sure the form of blood is low, you may need to take more. with CF should have a DEXA scan by age 18. The Vitamin D is cholecalcif- scan is done every one to five years. If you or Your body makes vitamin D from being in the erol or Vitamin D3. your child are prone to, or have, osteoporosis, sun. Being in the sun for 5-10 minutes, 2-3 times the DEXA scan will be done more often. a week, can raise vitamin D levels. Be careful not to stay out in the sun too long to avoid sunburn. Nutrition OTHER VITAMINS AND MINERALS Nutrition is vital for bone health. Low weight and • Vitamin K is a fat-soluble vitamin needed for poor nutrition cause weak bones. It is important bone density. to gain and keep your child’s weight up and eat • Low zinc levels can cause poor growth and healthy. Calcium, zinc, magnesium, proteins and slow puberty. vitamins D, K and A are needed to build and keep strong bones. • Low magnesium blocks the body’s use of calcium. CALCIUM • Proteins, also known as amino acids, are the Calcium plays a big part in forming bones and building blocks for all body tissues, even bones. keeping them strong. Children and teens need the most calcium because bones grow fast in Treating Bone Disease in CF those years. People with CF age 9 years and up The CF care team may prescribe calcium and/or should get 1300-1500 mg of calcium a day. Your vitamin D supplements if your levels are low. CF team can tell you how much calcium younger kids should get. BISPHOSPHONATES It is best to get your calcium from foods such as In osteoporosis, medicines called Bone Health Nutrients those listed in the table on page 5. Milk and milk bisphosphonates strengthen bones. These medicines help bone-building cells work better. products are great sources of calcium. Ask your • Calcium CF dietitian about other foods high in calcium They can stop osteoporosis from getting worse • Vitamin D and whether you or your child should take more or reverse it. They are not often given to kids calcium in a pill. under age 7 years. They should only be used in • Vitamin K those with known fractures from weak bones or • Vitamin A Calcium can decrease how well some organ transplants.