Welcome to Nutrition Management of Cystic Fibrosis by Lolita McLean, MPH, RDN; Connie Martin, MS, RDN; and Claire Stephens, MS, RDN...

Transcription

1 Welcome to Nutrition Management of Cystic Fibrosis by Lolita McLean, MPH, RDN; Connie Martin, MS, RDN; and Claire Stephens, MS, RDN...all of Alabama s Children s Rehabilitation Service, or CRS, part of the Alabama Department of Rehabilitation Services. 1

2 At the conclusion of this presentation, participants will be able to to: describe cystic fibrosis (CF) and how it is diagnosed; discuss related medical and nutritional implications; conduct appropriate nutrition assessment, diagnosis, and intervention related to CF; and explain the use of nutritional supplements and pancreatic enzyme replacement. 2

3 Cystic Fibrosis is a genetic disease primarily affecting Caucasians. Only 4% of African Americans and 7% of Hispanics are affected. Cystic Fibrosis is autosomal recessive, meaning that a person must receive a defective gene from both parents to have Cystic Fibrosis. The affects of Cystic Fibrosis are widespread but frequently have a predominant focus on the respiratory system, gastrointestinal system, and sweat glands. 3

4 As said in the previous slide, Cystic Fibrosis is autosomal recessive, meaning that a person must receive a defective gene from both parents in order to have Cystic Fibrosis. This slide includes a visual illustration of how Cystic Fibrosis is inherited. Each pregnancy of 2 carriers of the defective gene has a 25 percent chance of producing a child with Cystic Fibrosis, a 50 percent chance of producing a carrier, and a 25 percent chance of producing a child who neither has Cystic Fibrosis nor carries a defective gene. Carriers do not have Cystic Fibrosis but can pass along the defective gene to their offspring. 4

5 The defective gene in Cystic Fibrosis negatively impacts cellular chloride channels, causing the mucus in the body to have a higher salt content and lower water content. This makes the mucus abnormally thick. This mucus obstructs the airways, promoting bacterial infection. The mucus obstructs the passage of enzymes through the pancreatic duct for use in the digestion of food. Cystic Fibrosis is characterized by the presence of chronic pulmonary disease, pancreatic enzyme deficiency, abnormally high sweat electrolytes, and cirrhosis of the liver. Cystic fibrosis can also cause sinusitis and affects male reproductive organs, leading to infertility in 98% of males. Females with CF have normal fertility rates. The diagnosis of Cystic Fibrosis is typically made within a few weeks to a few months after birth. 5

6 Alabama was the last state to make newborn screening for Cystic Fibrosis mandatory by statewide law. Cystic Fibrosis can also, however, be diagnosed at a later age. In the past, prior to newborn screening, many people were diagnosed later in life as a result of chronic pulmonary and/or gastrointestinal issues. Chronic pulmonary issues include chronic cough, bronchospasm, repeated bronchial infections, chronic pneumonia, etc. Gastrointestinal issues include malnutrition, steatorrhea (or excessive fat in stools), rectal prolapse, and intestinal obstruction (especially in newborns caused by meconium stool). In clinic, I find that it is easier for patients and families to discuss pulmonary issues than gastrointestinal issues. Frequently, children go to the restroom alone and can t (or don t want to) comment on the quality of their stools. This is an area where parents need to stay more involved and be more informed than they would if their child did not have Cystic Fibrosis. Teenagers often don t feel comfortable discussing their stools, so they need to be educated, over time, that this is an unavoidable and important part of the conversation that needs to happen at every clinic visit. 6

7 Once newborn screening points to Cystic Fibrosis, a Cystic Fibrosis Center performs a thorough diagnostic evaluation, which includes a sweat chloride test. If the results of the test show possible Cystic Fibrosis, rather than a diagnosis of Cystic Fibrosis, a repeat sweat chloride test will be performed. Currently, Children s Hospital in Birmingham is the only Cystic Fibrosis Center in Alabama where that diagnostic evaluation can occur. Once the diagnosis is made, any active pulmonary infections are treated with appropriate medications and gastrointestinal health is evaluated and managed. The goal is to begin appropriate pancreatic enzyme replacement therapy, supplement fat soluble vitamins, and maintain the patient at greater than or equal to 50% of their ideal body weight and length for children less than 24 months of age. 7

8 The appropriate management of Cystic Fibrosis requires an interdisciplinary team approach. Team members typically include nurses, physicians, nutritionists, respiratory therapists, physical therapists, and social workers, among others. Other team members, sometimes found outside the clinic setting, include occupational therapists, clinical psychologists, counselors, parent advocates, and others. Multiple, large clinics are held each week, in order to accommodate the large number of patients in the state and their complex needs. 8

9 Turning our attention to the nutrition implications of Cystic Fibrosis, children with Cystic Fibrosis have increased energy needs. Individuals with Cystic Fibrosis expend an increased amount of energy breathing. They need more protein and significantly more calories than children without Cystic Fibrosis, so they frequently struggle with protein/calorie malnutrition. Calories from carbohydrates and fat may need to increase in order to spare protein. There are often behavioral issues around oral intake. Their gastrointestinal problems also typically include large, foul smelling, greasy stools which are a result of inadequate digestion of food. Eventually, gastrointestinal issues often include the development of CF-Related Diabetes and liver disease. 9

10 As part of our nutritional assessment, anthropometric measurements are obtained every 3 months at the child s quarterly Cystic Fibrosis clinic visits, or as often as directed by the health care team. These measurements include weight, height or recumbent length, and head circumference (if the child is less than 24 months of age). Every team member s annual visit assessment is more detailed. 10

11 At every clinic visit, or as directed by the health care team, the following should be plotted on every patient s growth chart: (1) the weight-for-age percentile; (2) the head circumference-for-age percentile, if the child is less than 24 months of age; (3) the height (or length)-for-age percentile; (4) the weight-for-length percentile, if the child is less than 24 months of age; and (5) the body mass index (or BMI)-for-age percentile, if the child is greater than 2 years of age. 11

12 Calculate the patient s target height range by noting the height of both parents, also known as the mid-parental height. For girls, take the father s height and subtract 5 inches. Then average that total with the mother s height. For boys, take the mother s height and add 5 inches. Then average that total with the father s height. Plot this estimated patient target height at 20 years of age on the height-for-age growth chart. 12

13 So, in a nutshell, individuals with Cystic Fibrosis require a high calorie/high protein diet. They experience malabsorption which may be characterized by a variety of challenges. They may have frequent daily bowel movements that are bulky, greasy, and foul-smelling. Because of frequent coughing and hard to pass stools, they may experience rectal prolapse. As more of their food passes through their gastrointestinal system without being properly digested, they may experience excess gas, bloating (which may be accompanied by a full feeling), and stomach discomfort. Many with Cystic Fibrosis experience weight loss or difficulty gaining weight. These issues may make it challenging to be around others at school, as kids with Cystic Fibrosis sometimes get teased. School packets and a teacher s guide are provided in the summer for all children attending school. It is recommended that families complete an Individualized Education Plan, or IEP, to ensure that the child s daily health needs are met. Although it may seem easier to children to hide the fact, at least at school, that they have Cystic Fibrosis, it will likely be better, in the long run, to educate those around them and learn to advocate for themselves, with help from parents, siblings, the health care team, etc. 13

14 Across the board, nearly all children with Cystic Fibrosis struggle to either gain or maintain weight. Pancreatic enzyme replacement therapy, or PERT, is employed (and adjusted, when needed) to relieve malabsorption. The dose is dependent upon the child s weight and age, pancreatic function, postsurgical anatomy, and dietary fat content. Some examples of commonly used pancreatic enzymes include Creon, Pancreaze, and Zenpep. These enzymes are taken by mouth just before eating any snacks and meals or at the same time the initial bites are taken, as enzymes and food should arrive in the small intestine at the same time. Often, pancreatic enzyme replacement therapy is available to children free of charge or at a reduced rate. If oral food intake and weight are insufficient, gastrostomy feedings may be required. 14

15 The Cystic Fibrosis Foundation provides guidance for weight-based enzyme dosing that recommends 1000 lipase units per kilogram per meal for children under 4 years of age and 500 lipase units per kilogram per meal for those 4 years and older. Older patients may require lower doses of lipase units per kilogram per meal, since they weigh more but are likely to ingest less fat per kilogram. Half the standard dose is given with snacks, and the total daily dose should be based on approximately 3 meals and 2 to 3 snacks per day. It is very important that pancreatic enzyme replacement therapy be in effect for all ingested snacks and meals, as these enzymes aide digestion. If an individual begins to see a change in or worsening of some of their gastrointestinal issues, it s time to alert the doctor, as a change in pancreatic enzyme replacement therapy may be warranted. Adjustments should be based on weight gain and clinical symptoms of malabsorption. 15

16 Individuals with Cystic Fibrosis struggle with maldigestion and malabsorption, so they don t absorb all of the available energy from the foods they eat. In addition, dealing with their respiratory challenges (increased lung infections, work of breathing, coughing, etc.) demands more energy. They need to protect energy stores for lengthy and more frequent periods of illness. As they are less able to eat large quantities of food, they need calorie-dense foods, which often means adding fat to their diets and adjusting their pancreatic enzymes accordingly. If nutritional needs are still unmet, tube feeding might have to be considered to increase protein and calorie intake. 16

17 People with Cystic Fibrosis need vitamin and mineral supplementation because they do not adequately absorb the vitamins and minerals they need from the foods they eat. They require fat-soluble vitamins made specifically for people with Cystic Fibrosis such as Vitamax, AquADEKs, or MVR Complete. They also need extra salt, since it is lost due to the high sodium chloride content of body fluids (especially sweat) in Cystic Fibrosis. Even infants require extra salt because of the low salt content of breast milk, infant formula, and baby foods. For infants, salt should be added to formula or baby foods. Children, adolescents, and adults should use the salt shaker liberally, eat foods high in salt, and drink plenty of fluids. The health care team can help patients determine the right amount of extra salt to meet their needs. 17

18 A handy way to increase calories is to consume nutritional supplements like Pediasure, Boost products, Ensure products, Carnation Instant Breakfast, Scandishakes, Opt2Thrive, etc. One of the problems people with Cystic Fibrosis deal with is that, not long after they find a supplement they like, they grow weary of it. Supplements can be expensive, although possibly available through assistance or state programs. Supplements can also reduce a child s appetite, which is an overreach of their desired effect. 18

19 To make the most of mealtime, I encourage the families I work with to allow the kids in the family to participate in gardening, meal planning, grocery shopping, and meal preparation. Add spices and flavorings to food and try new recipes. Try to make a habit of eating meals around the table, avoiding the television. Make sure and pack extra snacks for use during the day, especially at school, and always provide a bedtime snack. 19

20 The case example on this slide focuses on a 10 year old male with Cystic Fibrosis named PB. At this visit, PB weighs 27.1 kilograms, which puts his weight at the 7 th percentile for his age. PB is centimeters tall, which puts his height at the 17 th percentile for his age. PB s body mass index is 14.8 or at the 9 th percentile for his age. At this visit, PB has averaged a weight loss of 4.5 grams per day for the past 133 days. His gastrostomy button came out last week and he hasn t used it since. Previous to this, PB was only receiving feedings via gastrostomy tube at 1:00 am for a 250 milliliter bolus feeding of 1.5 calorie Peptamen Junior because he had complained that continuous night feeds caused tummy aches and clogging. 20

21 At this visit, PB reports having a good appetite and eating a lot of food. He also drinks an 8-ounce Boost or Pediasure nutritional supplement daily at bedtime. PB takes 2 Creon 12 enzymes with meals and 1 with snacks. This provide 866 units of lipase per kilogram per meal. Asking PB about his usual stools could help determine malabsorption and the potential need to increase enzymes. He also takes Cyproheptadine, Vitamin D, AquADEKs chewable, and Miralax daily. It is not a surprise that PB can report these items on his own. Kids with CF are often some of the best educated, best prepared kids when it comes to the ability to manage typical transition-related issues (like understanding and communicating their medication and vitamin use). In addition, the official website of the Cystic Fibrosis Foundation, has every resource you would ever need when it comes to understanding something or trying to teach a concept to others (complete with downloadable handouts, etc.). It is very well organized. 21

22 The significant nutrition-focused physical findings from this visit include the fact that PB s weight is down. Looking at comparative standards, PB s energy needs are estimated by multiplying the recommended daily allowance, or RDA, by which equals kilocalories per kilogram, grams of protein per kilogram, and 1500 to 1800 total milliliters of fluid. PB has increased energy expenditures related to the increased work of breathing he experiences as a result of having Cystic Fibrosis. His nutrition prescription includes a high calorie/high protein diet with 3 meals and 3 snacks per day, no sugar-sweetened beverages, and either 4 cans per day of 1.0 calorie Boost or 3 cans per day of 1.5 calorie Boost. To help monitor and evaluate PB s progress, a desired growth pattern and goal weight should be set to be met by the next visit. 22

23 The case example on this slide focuses on a 3 month old male with Cystic Fibrosis named CA. At this visit, CA weighs 5.33 kilograms, which puts his weight at the 2 nd percentile for his age. CA is 58 centimeters long, which puts his length at less than the 2 nd percentile for his age. CA s weight to length ratio is in the 42 nd percentile. Over the past 21 days, CA has gained 0.67 kilograms, which is an excellent average daily weight gain of 32 grams per day. CA has a history of significant gastrointestinal complaints. His mother reports that he often strains for up to 30 to 60 minutes to have a bowel movement. CA has a history of skin breakdown on his bottom due to frequent, loose, oily stools. Surgery is following him for a possible rectal prolapse. 23

24 CA started out using Enfamil Gentlease, a standard infant formula, then moved to Similac Total Comfort, a partially hydrolyzed protein formula, and finally transitioned to Elecare Infant, an amino acid-based formula for his gastrointestinal issues. He currently takes ounces of Elecare Infant 27 kilocalories per ounce 7 times daily with occasional spitting up. CA also takes Creon 6 enzymes (1 1/3 capsules with each feeding), AquADEKs, and Omeprazole. 24

25 The significant nutrition-focused physical findings from this visit include the fact that CA is having approximately 3-4 stools daily. His diaper rash is improved at this visit. Looking at comparative standards, CA s energy needs are estimated by multiplying the recommended daily allowance, or RDA, by which equals kilocalories per kilogram, grams of protein per kilogram, and milliliters per kilogram per day. CA has increased energy expenditures related to the increased work of breathing he experiences as a result of having Cystic Fibrosis. His nutrition prescription includes Elecare Infant 27 kilocalories per ounce by mouth as desired with a goal of approximately 28 ounces per day, which equates to ounce bottles per day. To help monitor and evaluate CA s progress, a desired growth pattern and goal weight should be set to be met by the next visit. 25

26 This final slide contains some helpful references used in this presentation. 26