Welcome to Hemophilia and Nutrition Considerations by Connie Martin, MS, RDN; Lolita McLean, MPH, RDN; and Claire Stephens, MS, RDN...

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1 Welcome to Hemophilia and Nutrition Considerations by Connie Martin, MS, RDN; Lolita McLean, MPH, 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

At the conclusion of this presentation, participants will be able to: describe the types, prevalence, and treatment of hemophilia; discuss considerations for patients with hemophilia such

2 At the conclusion of this presentation, participants will be able to: describe the types, prevalence, and treatment of hemophilia; discuss considerations for patients with hemophilia such as weight, exercise, and bleeding; categorize the different levels of acceptable and unacceptable activity; and explain indicated medical and nutritional considerations and interventions. 2

3 Hemophilia is a rare bleeding disorder in which the blood does not clot normally. It is an inherited, X-linked recessive disease. There are different types of hemophilia: Hemophilia A, or Classic Hemophilia, is a Factor VIII deficiency; Hemophilia B, or Christmas Disease is a Factor IX deficiency; and Hemophilia C is a Factor XI deficiency. In 1944, researchers reported a case in which, when the blood from 2 different hemophiliacs was mixed, both were able to clot. This could not be explained until 1952, when researchers made a discovery that led them to believe there were 2 types of Hemophilia. They had been studying a 10- year-old boy with Hemophilia named Stephen Christmas who didn't seem to have the "typical" disease. They called his version Hemophilia B, or "Christmas Disease, and the more prevalent version Hemophilia A, or Classic Hemophilia. Christmas Disease affects only 15-20% of people with Hemophilia. 3

Hemophilia A occurs in 1 in every 5,000 live male births and is 4 times more common than Hemophilia B. About 20,000 people in the United States have Hemophilia.

4 Hemophilia A occurs in 1 in every 5,000 live male births and is 4 times more common than Hemophilia B. About 20,000 people in the United States have Hemophilia. Although the worldwide incidence of Hemophilia is not well known, it is estimated that over 400,000 people have it, and 75% of those have inadequate care or treatment. 4

Von Willebrand Disease is included in this presentation because it is another bleeding disorder that is covered in Alabama by the Children s Rehabilitation Service.

5 Von Willebrand Disease is included in this presentation because it is another bleeding disorder that is covered in Alabama by the Children s Rehabilitation Service. It is not a form of Hemophilia but is treated similarly. Von Willebrand Disease is the most common inherited bleeding disorder. It is caused by missing or defective Von Willebrand Factor, which is a clotting protein that acts like a glue to help platelets stick together and form a clot. Von Willebrand Factor binds Factor VIII, a key clotting protein, and platelets in blood vessel walls, which help form a platelet plug during the clotting process. There are 3 types of Von Willebrand Disease: Type 1, Type 2 and Type 3. Often times, people do not know that they have Von Willebrand Disease until something happens; for example, they don t stop bleeding after childbirth. Sometimes you will hear people who have not yet received a diagnosis say something like nosebleeds run in our family. 5

6 In adults, the standard approach is to manage symptoms and prevent future bleeds with education. In children, the standard approach may include prophylactic Factor injections or infusions along with multidisciplinary education. In mild Hemophilia A, more specific treatments include a slow injection of the hormone desmopressin (or DDAVP). In moderate to severe Hemophilia A or B, bleeding may only stop after an infusion of Clotting Factor, and repeated infusions may be necessary. In Hemophilia C, plasma infusions, and sometimes antifibrinolytics along with Clotting Factor, help prevent clots from breaking down. Sometimes with Hemophilia, small rural hospitals may not have the Factor or feel entirely comfortable providing care. A lot of advanced planning can go a long ways, especially when it comes to working with patients who live in rural areas. Patients with Hemophilia can often receive real benefit from the services of a care coordinator. 6

In Alabama we have Camp Clot Not, which is a camp for kids with Hemophilia where they can go and participate in activities in which they may not normally be able to participate because of the dangers

7 In Alabama we have Camp Clot Not, which is a camp for kids with Hemophilia where they can go and participate in activities in which they may not normally be able to participate because of the dangers of bleeds and accidents. At camp, they get to talk with a variety of professionals like nutritionists, physical therapists, occupational therapists, physicians, nurses, etc. to learn more about their disease, how to take care of themselves, injections and infusions...things they need to know about Hemophilia. 7

The following are common topics that must be addressed with individuals with Hemophilia, especially in the adult population, where some pretty complicated cases can be found: managing bleeds most

8 The following are common topics that must be addressed with individuals with Hemophilia, especially in the adult population, where some pretty complicated cases can be found: managing bleeds most appropriately; promoting a healthy weight based on age; exercising safely and appropriately; managing co-existing conditions; taking medications, supplements and herbs; addressing poor nutritional intake; and managing social issues. 8

9 The young man pictured in this slide is experiencing a bleed at his knee. The majority of bleeds occur in the muscles and joints. These can be spontaneous or the result of direct trauma. As our size increases, more stress is placed on our muscles and joints to perform activities of daily living. 9

So why is promoting a healthy weight based on age important in the management of Hemophilia? First, the joints of overweight people suffer significantly more stress.

10 So why is promoting a healthy weight based on age important in the management of Hemophilia? First, the joints of overweight people suffer significantly more stress. Less stress on joints equates to fewer joint bleeds. Second, it can sometimes be difficult to find usable veins in overweight people when Factor infusions are needed. Third, there are increased healthcare costs associated with being overweight. And fourth, overweight people are at an increased risk of developing chronic conditions such as cardiovascular disease, diabetes, and hypertension. More information about assessing weight in adults, teens, and children using Body Mass Index can be found on the Centers for Disease Control and Prevention website using the link provided here: World Health Organization and Centers for Disease Control and Prevention growth charts, including the Centers for Disease Control and Prevention s BMI-For-Age growth charts, can be found on the Centers for Disease Control and Prevention website using the link provided here: 10

Health data entered into the Universal Data Collection (UDC) system, created by the Centers for Disease Control and Prevention, indicate that higher rates of obesity and inactivity are occurring in

11 Health data entered into the Universal Data Collection (UDC) system, created by the Centers for Disease Control and Prevention, indicate that higher rates of obesity and inactivity are occurring in people with bleeding disorders. People who are overweight are more likely to have joint damage, regardless of the severity of their bleeding disorder or even the existence of a bleeding disorder. 11

The Centers for Disease Control and Prevention report that people with Hemophilia and Von Willebrand Disease 13 to 19 years of age are twice as likely to be overweight than the general population.

12 The Centers for Disease Control and Prevention report that people with Hemophilia and Von Willebrand Disease 13 to 19 years of age are twice as likely to be overweight than the general population. Also, range of motion is typically more limited as weight and body mass index increase, as there is increased stress on joints. People will often overprotect their joints by moving and exercising less, which actually causes additional joint problems due to lack of use. 12

13 Regular exercise can help with weight loss and weight maintenance. Strong muscles protect joints, reducing the number of bleeds. As a nutritionist, I cannot tell you how many times I have seen patients, weighed them, measured them, answered some questions, and discussed healthy eating, only to have the patients steer the conversation towards exercise. My response is usually to confirm that they are scheduled to see a physical therapist and to go ahead and transition them to that team member. Then, their questions about exercise can be posed to the most appropriate professional. The physical therapist will evaluate their range of motion and provide the most appropriate exercise recommendations. At most, I might suggest parking the car in the last available parking space before walking into the supermarket. Once they ve gotten their exercise-related questions answered, they are often more open to discussing nutrition-related information. 13

14 For people with bleeding disorders, activities are divided into 5 levels of sports ratings ranging from safe to dangerous. Level 1 activities, labelled safe, include archery; aquatics, including swimming, water exercise, and water aerobics; elliptical machines; exercise bikes; fishing; golfing; hiking; Tai Chi; and walking. 14

15 Level 1.5 activities, labelled safe to moderately safe, include bicycling, rowing machines, ski machines, treadmills, circuit training, body sculpting classes, spin classes, frisbee golf, pilates, and weight/resistance training. 15

16 Level 2 activities, labelled moderately safe, include aerobics, bowling, step machines, dance classes, recreational diving, cardio kick boxing, ultimate frisbee, jumping rope, indoor rock climbing, roller skating, running and jogging, cross country skiing, t-ball, tennis, and yoga. 16

17 Level 2.5 activities, labelled moderately safe to dangerous, include baseball and softball, basketball, canoeing, cheerleading, gymnastics, horseback riding, ice skating, inline skating, jet skiing, kayaking, Kung Fu and Tae Kwon Do, mountain biking, skateboarding, downhill skiing, soccer, track and field, volleyball, and water skiing. 17

Level 3 activities, labelled dangerous, include BMX racing, boxing, competitive diving, football, hockey, lacrosse, power weight lifting, outdoor rock climbing, rugby, rodeo, snowmobile, trampoline,

18 Level 3 activities, labelled dangerous, include BMX racing, boxing, competitive diving, football, hockey, lacrosse, power weight lifting, outdoor rock climbing, rugby, rodeo, snowmobile, trampoline, and wrestling. It is advised that people with Hemophilia not participate in Level 3, or dangerous, activities. Although these recommendations come from the National Hemophilia Foundation, health care professionals ought to consider the rationale and evidence-base behind recommendations before passing them along to clients and colleagues. 18

The Hemophilia Clinic team here in Alabama s Children s Rehabilitation Service is comprised of a nurse, laboratory staff, a nutritionist, a physical therapist, a dentist, a medical doctor (a UAB

19 The Hemophilia Clinic team here in Alabama s Children s Rehabilitation Service is comprised of a nurse, laboratory staff, a nutritionist, a physical therapist, a dentist, a medical doctor (a UAB Hematologist), a parent consultant (in the pediatric Hemophilia Clinic), and a social worker. The Hemophilia Clinic social worker may not be the patient s actual care coordinator, as their care coordinator is going to be located in the district where the patient lives. 19

20 In the population of adults with Hemophilia, we see a lot of patients with Hepatitis C and concurrent liver issues. Of course, avoiding alcohol; junk foods; and maintaining a balanced, healthy diet are very important. Also, unless their doctor specifically recommends it, we do not generally recommend supplementation in people with Hepatitis C because of the potential for liver damage. In our adult patients with Hemophilia, we also see people with Hypertension and/or HIV (some of which are underweight and have nutritional issues). We also see Type II Diabetes in our adults with Hemophilia and, in an effort to support their adherence, I make it a practice to ask if they are checking their blood sugar regularly and when they last checked it. 20

There are other issues that influence nutritional intake and, therefore, nutritional status in patients with Hemophilia. Low income patients may find themselves food insecure.

21 There are other issues that influence nutritional intake and, therefore, nutritional status in patients with Hemophilia. Low income patients may find themselves food insecure. Many of the patients we see in the Children s Rehabilitation Service clinics are very poor and have little to no insurance coverage. We help them survive by providing Factor and medical care, but that doesn t make it so that they necessarily have money for food. Many of our patients live in rural areas with few grocery stores and limited food availability. Even when there is money for food and available grocery stores, joint damage limits their ability to perform activities of daily living like grocery shopping and food preparation. 21

22 Many medications, supplements and herbs are contraindicated for those with Hemophilia. Medications such as aspirin and non-steroidal anti-inflammatories like Naproxen (also known as Aleve) and Ibuprofen (also known as Motrin and Advil) affect blood clotting. Topical agents such as Bengay and Icy Hot contain Menthol and Methyl Salicylate which act like aspirin, also affecting blood clotting. Supplements such as Vitamin E and Fish Oil should be avoided because Fish Oil is a natural blood thinner and Vitamin E can antagonize Vitamin K, which aides in blood clotting. Herbs such as Ginkgo, Garlic in the supplement form, Ginger in the supplement form, Ginseng, Horse Chestnut, Turmeric, and White Willow can make bleeding more severe. This is not a complete list of the medications, supplements and herbs that people with Hemophilia should avoid. 22

23 In Hemophilia, bleeds and the resultant nutritional issues lead to anemia and transfusions, among other things. Active bleeds are painful. Increased pain levels often decrease appetite and lead to an increased use of pain medication, which can cause constipation. A decreased appetite can result in weight loss and poor nutritional status. Increased pain levels will result in decreased movement, which can lead to weakness and depression. Many patients experiencing this kind of pain are doing well to even be able to stand up on the scale! Depression can lead to a decreased appetite, setting up a vicious cycle that can be difficult to break. 23

24 In the Children's Rehabilitation Service Hemophilia Clinic, nutrition counseling is provided for every patient. Depending upon the doctor s recommendations, we generally recommend that our adult patients use a nutrition supplement like Ensure, Ensure Plus, Boost, or Boost Plus if weight loss tends to be an issue. We sometimes use iron supplementation and, occasionally, appetite stimulants. However, as dietitians, we always recommend food first! 24

25 Thank you for your attention! A list of helpful resources can be found on the last slide in this presentation. 25

26 This slide contains some helpful resources. 26

Use this info if you're counting steps and want to convert activities that are not easily measured by the pedometer.

1 of 9 Use this info if you're counting steps and want to convert activities that are not easily measured by the pedometer. PLEASE NOTE: Activity Conversions are estimates; your actual steps may vary Bicycling