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Prophylaxis, Teens and Adherence

Tune in to learn more about the full potential for prophylaxis, exercise and a healthy weight to ensure joint health and enhance the lives of teens with hemophilia.

Transcription of "Prophylaxis, Teens and Adherence"

ANNETTE GOSLIN (Moderator): Good evening, everyone, and welcome to Prophylaxis, Teens and Adherence, part of the National Hemophilia Foundation’s Hemophilia Webinar Series.  This new series is supported by an educational grant from Novo Nordisk. 

Our presenters this evening are Jim Munn and Dr. Ruth Mulvany.  Jim is the hemophilia treatment center program nurse coordinator at the University of Michigan Health System in Ann Arbor.  He has served as both regional representative and chair of NHS Nursing Working Group. He is treasurer of the International Society of Thrombosis and Haemostasis Nurses Forum and has presented lectures and authored several publications of bleeding disorders care.  Jim has also worked closely with the World Federation of Hemophilia serving on the organization’s Nurses Committee, and most recently as program committee co-chair with the WFH 2016 World Congress.  The congress, which was held in July 2016 in Orlando, Florida, included more than 5,000 participants from around the globe. 

Dr. Ruth Mulvany retired in 2014 as an associate professor at the University of Tennessee Department of Physical Therapy where she was involved in teaching research and patient care.  For more than 30 years she served as physical therapy consultant to the university’s Hemophilia Treatment Center and Arthritis and Rheumatology Clinic.  She has published and presented on research and management of bleeding and musculoskeletal disorders nationally and internationally.  Dr. Mulvany has worked extensively with the Centers for Disease Control and NHS as a member of the Physical Therapy Working Group and as co-chair of four NHS annual meetings.  In 2002 she received the NHS Physical Therapist of the Year Award, and in 2014 she was inducted into the Tennessee Physical Therapy Association Hall of Fame.

Welcome, Jim and Dr. Mulvany.

Our program will conclude with a question and answer segment.  To ask a question, please go to the area in the far lower left of your webinar screen and type your question in the field just to the left of the Send button, which is located in the pod or area labeled Chat.  Click the Send button when you are finished typing your question.  However, please note that your question will be addressed during the question and answer session at the end of the presentation. 

There are no handouts for this presentation, however, a recording of this webinar will be available shortly on the National Hemophilia Foundation website at www.hemophilia.org

Without further ado, I would like to turn things over to Jim. 

JIM MUNN:  Thank you very much, Annette, for the kind introduction, and to NHS for inviting both Ruth and myself to speak tonight.  I also want to really thank everyone who was able to join tonight with this webinar.  Hopefully we’ll be able to impart some information to you and get some questions answered that you might have out there.

As you can see from the objectives slide, we were tasked with two things.  The first one I’m going to address, and that’s describing the risks and consequences that are associated with having hemophilia, and then the importance that prophylaxis might be playing in the prevention of bleeding.  Ruth then will go on to discuss both the evolution of joint damage, which is arthropathy, and particularly the morbidities that can be associated with each stage as the evolution progresses and how exercise, physical therapy and maintenance of a healthy weight can help maintain mobility, especially in the teenage population. 

Essentially you’ve probably all been told about the risks and consequences of hemophilia A and B at some point in your life.  Everyone knows that the biggest thing to worry about of course is bleeding.  Bleeding in hemophilia is most often found in joints, muscles and soft tissues.  Obviously it can happen anywhere that there happens to be a blood vessel in the body. 

What we see is that when treatment doesn’t occur and bleeding happens, especially into joints, you can get progressive joint damage over time.  As a result of having hemophilia A or B, we have seen a number of patients who have had increased absences from work and from school just because of the fact that their hemophilia and their joint issues have played a role.  We can address those concerns when we talk later on about treatment that’s available for patients with hemophilia.

Some of the other consequences associated with hemophilia A and B happen to be that activity levels drop off once a bleeding episode is present, and that makes sense because you’re being told by your treatment center to rest the affected joint to make sure that re-bleeding doesn’t occur.  So, as those reduced activity levels happen, it sort of circles that progression of joint destruction over time, which Ruth will address later on.

There have been a number of studies that have looked at responses from both parents and patients with hemophilia, and many have described psychosocial and emotional concerns as well as the financial constraints that can occur with this disease. 

This is a picture, as you can see, of a joint that has had damage over time.  What’s happened is repeat bleeding has occurred into this left knee.  Normally we like a little bit of a wider joint space.  It has been narrowed here because of the destruction over time.  The smooth surface that you would expect in a joint has gone away.  You can see it’s a little bit irregular.  We start to get some erosion of bone over time as well.  These occur as a result of repeat bleeding, especially in scenarios where someone is not treated appropriately or hasn’t decided to move on to a prophylaxis regimen.

The big question is, is there a way to prevent these risks and consequences?  Happily I can say yes. 

The biggest thing that we’ve been able to find from all the research that has been done is that prophylaxis can prevent all of the long-term consequences of hemophilia.  We have seen that in a number of studies.  What happens in patients who are not experiencing prophylaxis is that the total number of bleeds, and especially joint bleeds, will increase as time increases with factor levels that fall below 1%. 

As you can see in the chart here, this is the pediatric patient population compared to the adult population here on this graph.  They’re both looking at the predicted number of bleeds that will happen in a year at every hour in a week that someone stays less than 1%.  You will see that that increases.  These are pediatric patients less than six years of age, and these are adults.  In the middle is the comparative chart which looks at the pediatric patients on the top less than six versus adult patients over eighteen, and the potential or the probability of having no bleeds in a year.  That drops off precipitously with the number of hours that you stay less than 1%.

There are a number of ways to treat hemophilia.  You guys are very familiar with them.  First and foremost, you may decide to choose to treat episodically, which is on demand, and that’s only at the time bleeding occurs.  I’m hoping that most of you have been exposed to treaters who are recommending prophylaxis, because we’ll notice later on how important that is.  But there are different types of prophylaxis that are out there.  I’m not sure if you’re familiar with all of them.  The prophylaxis regimens can be continuous, meaning that that happens from the time it starts to forever. 

The continuous prophylaxis can fall into three separate categories.  There can be a continuous primary prophylaxis, which is started before a second large joint bleed and before three years of age.  There is secondary prophylaxis, which is a regular continuous approach that’s started after two or more large joint bleeds, but before the onset of joint disease.  Tertiary prophylaxis is what we used to call secondary prophylaxis.   It’s when people have established some joint disease over time, and it’s used to prevent further damage.  This is something that’s very important in our adult population who didn’t have access to prophylaxis when they were younger.  The last term is intermittent prophylaxis, or periodic prophylaxis.  That’s the scenario in which a patient might choose to do prophylaxis during a short period of time, such as after surgery or with a sports season, for instance. 

What is included in prophylaxis consideration?  Dr. Oldenburg from Bonn, Germany came up with a beautiful diagram that spells out this concept very nicely.  You can see the three things that you need to consider before deciding on prophylaxis.  What trough level and interval of treatment do I need, which essentially results in cost?  What are the bleeding triggers that I might have?  What is my physical activity level?  Am I a very physically active person, or am I a couch potato who has probably less chance of bleeding because of that?  Do I have already-established joint problems, or is there chronic synovitis present?  The other part to the consideration for prophylaxis is the number of acceptable joint bleeds that I might have.  If you think about this concept, if you have unlimited access to factor, you could probably do some fairly aggressive things, because your physical activity can go up and you might be able to eliminate joint bleeds, but you would do that at an extremely high cost.  Those are all the pieces of information that are taken into consideration when prophylaxis is recommended for someone to treat their hemophilia.

Who do we think needs prophylaxis?  The answer has been quite clear for MASAC for a long time.  What they have decided is that in view of the demonstrated benefits that this treatment process has, it should be started at a very young age in persons with hemophilia A and B, and that it be considered the optimal therapy, especially for individuals who have severe hemophilia A and B. 

But are those the only people who need prophylaxis?  The answer is quite simply no.  There are tons of other people who may need prophylaxis, or at least benefit from it.  If you’ve had any previous history of an intracranial hemorrhage, you should be placed on prophylaxis.  If there is the potential for inhibitor development, there have been recommendations that some patients should consider going on to prophylaxis to see if that will help minimize or prevent the inhibitor development.  That’s still a very controversial approach, but these are some things that treaters have used as far as decision making for prophylaxis. 

Of course, we want to make sure that anyone who has the presence of joint damage is considered for prophylaxis.  If you happen to be a person who is one of the couch potato people whose weight has increased and don’t have severe disease—you could have moderate or mild hemophilia—we may make a suggestion that prophylaxis be included just because of the amount of stress that excess weight puts on joints.

If you happen to have other comorbidities, such as hepatitis C or HIV, those patients might need prophylaxis as well, because other types of bleeding can occur, especially when there is insult to the liver. 

We want to make sure that patients who have been having problems with school or work absences be considered for prophylaxis so that we can avoid that issue all together.  And of course, prophylaxis is best to treat patients who are very, very active.  We are seeing that more and more in our younger kids.

So, is prophylaxis the same for everyone?  As much as you have heard that the same sort of treatment regimen occurs for prophylaxis for everyone, that’s not the case.  We have a number of studies who have looked at prophylaxis.  The first one, which was the Hallmark Study, was done in Malmo, Sweden.  What they did was they looked at a number of severe patients with hemophilia A and B, and they put them onto regimens that you see listed here.  Essentially a 48% to 80% correction to hemophilia A patients three times a week, and if you had hemophilia B you got 25% to 40% correction twice a week.  They started this treatment between the first and second years of life in patients that were on the study.  They looked at both orthopedic and radiologic scores.  They were evaluated by the WFH recommendations at that time.  What they found was that it really appeared to be possible to prevent joint damage if you were on an effective continuous prophylaxis regimen and you prevented the factor VIII or IX from falling below 1% of normal. 

From the Hallmark study came the pivotal study.  The Hallmark Study wasn’t a randomized controlled clinical trial that compared patients who were put onto prophylaxis versus those who were not.  This study that was done by Dr. Manco-Johnson and several other investigators across the U.S., called the Joint Outcome Study, was what we call a pivotal study.  The reason is that it has given us information that has allowed us to go to our insurance companies in order to get prophylaxis covered for treatment.  They assigned boys randomly to either prophylaxis or an episodic arm.  They were treated with an enhanced approach.  Not with just one infusion, but every bleed that a child had was treated with an 80% correction as soon as the bleeding was noted.  Then they were given a 40% correction the second day and then two days after that.  They would continue that treatment every other day up to four weeks until the bleed was completely controlled.  We know that from this study outcomes were much better on the prophylaxis arm such that the statement that was concluded was that prophylaxis with recombinant factor VIII can prevent joint damage, and it decreases the frequency of joint and other hemorrhages in young boys with severe hemophilia A.

The Italians came up with their own study to compete with the U.S. study.  Theirs was a prophylactic regimen that was given twice a week, and episodic treatment occurred very aggressively as well.  They found the same information.  The outcomes were much better on the prophylaxis arm.

The Canadians came up with an approach that looks at what we call a dose escalation program.  It started once a week, and dosing advances when you have a certain number of bleeding episodes.  After thirteen years on this study, the Canadians took a look, and the median follow up was more than seven-and-a-half years.  What they found was that the age at the first joint bleed really made an important indicator of whether the more severe bleeding was going to occur.  So, when you’re designing a prophylactic regimen for young boys with severe hemophilia A, you really need to take into account how young they were when they got their first bleed.  The Canadians have been able to say that boys treated with this primary prophylaxis approach and exhibit minimal joint damage on physical examination, they have minimal functional disability as well. 

There has been an incremental dosing approach in which the Europeans started very similar to the Canadians where they did once-a-week prophylaxis with 500 units of factor VIII, and they did it until it was feasible for families to be able to do infusions on their children.  The parents were being taught that incremental dosing increased to two to three times a week, and eventually it went to every other day when the patients were on home therapy.  Interestingly enough it was an incremental step, but the dosing remained the same, so 500 units.  In that period of time kids can get bigger, and you would think that maybe the dose should be adjusted upward as their weight increases, but they were still able to find that there was no clinical influence if you maintained a trough level less than 1%, because there were some patients who, even though their troughs were less than 1%, still had good outcomes.  The lowest effective factor VIII and IX really needs to be determined for each patient when you decide to do primary or secondary prophylaxis. 

A nurse out of Sweden did a study looking at daily dosing with factor, which required thirteen patients to go onto what we call a crossover study.  The first twelve months they did their usual prophylaxis regimen, and the second twelve months they did daily prophylaxis.  What they did was made sure that the trough level remained the same or higher between both regimens.  The Swedes are very regimented in making sure that trough levels remain above 1%, so that was targeted the same here.  What she was looking for was the feasibility of how much concentrate was consumed and was it efficacious.  Interesting enough, it certainly reduced the cost of factor by 30%, but in the patients who went onto daily dosing, they reported more bleeding events, and they felt that they had a decreased quality of life and increased stress based on the fact that they were having to do an infusion every single day.

The last type of prophylaxis approach is a pharmacokinetic dosing approach.  This was a study that was done—again, a crossover in which you did on-demand therapy for six months and then you followed up with prophylaxis, either the standard dosing, which you had seen before, which is an every-other-day approach versus a PK-tailored approach looking at your pharmacokinetics in which your factor was given only every three days.  What they found was that there was comparable safety and efficacy for both prophylaxis regimens, and that prophylaxis significantly reduced bleeding compared to the on-demand six months prior, and that PK-tailored prophylaxis is an alternative to standard prophylaxis in the prevention of bleeding.  That’s where some of the new products are coming on board.  The pharmacokinetic dosing with those products might be able to be achieved at doses twice a week or even sometimes just once a week if you’re a factor IX-deficient patient. 

How does adherence tend to affect treatment and my life?  We know that adherence, which is a combination of a worked-out plan between patients, families and the treatment center team, is the extent to which patients then adhere or stick to that regimen.  In basic terms, many people would say it’s the extent to which patients take the medicine as prescribed by their healthcare providers.  According to the World Health Organization, rates of nonadherence with any medication can vary anywhere from 15% to 93% with the average estimated rate of 50%. 

So, adherence can be an issue, especially in scenarios where treatment paradigms are very, very complicated.  We do know that adherence to prophylaxis is very critical to success, and that it’s most effective if factor levels are continuously maintained above the target range, which in some patients may be more than 1%.  Depending on how active you are, we may be targeting you higher.  When you skip or miss doses, it causes the clotting factor to fall below the target level, which of course increases the risk of bleeding, which we saw before.

The real issues that can occur with adherence are outlined here.  It may sound as if it’s gloom and doom, but it’s not in the sense that all of these things are preventable, and you have the ability to take control of these issues yourself.  The more that you have your own independence and self-advocacy, the more likely you are to shy away or to steer away from some of these concerns.  We do know that there is a higher divorce rate in marriages in which one partner has a chronic disease.  Hemophilia falls into that category.  Does that mean that every patient with hemophilia is going to be divorced at the same rate?  Absolutely not. 

The same with the second bullet point, which suggests that depression happens to be six times higher in spouses who become caregivers.  What we’re trying to say is that, as teens, if you can develop a partnership where you have control independently and don’t put as much emphasis or work onto your partner, the chances of these things drops off.  We do know that many adolescents still depend on parents for daily hemophilia care, and we want that to change.  We’re working more and more on transition issues for patients in hopes that they will move towards their own independence.  During the teen and early adult years not only does adherence decrease, but when patients actually choose to go from prophylaxis to on-demand therapy, their bleeding increases and their quality of life decreases. 

What are some of the barriers and motivators to adherence?  We had some investigators that looked at these concerns.  What they found was the things that motivate people to have great adherence are generally age.  We know in our younger population parents are very good at making sure their children get prophylaxis.  As the teens start to take over their own care, sometimes they slip off on that, so that becomes a barrier as well.  As far as motivators, if you’re able to recognize your symptoms, you’re more likely to adhere to your treatment, because you want to get those symptoms taken care of.  If you believe in the necessity of the treatment, you also are more likely to adhere.  Believe it or not, if you have a good relationship with your healthcare provider, that also is a strong motivator for adherence. 

Besides age, as barriers are concerned, infrequent or absent symptoms may also occur. What we have found sometimes is in the prophylaxis age patients and families who have never experienced a bleeding episode might forget to call the treatment center when bleeding occurs and unfortunately not do a treatment that might be necessary, so essentially falling off from an adherence standpoint there.  It certainly is a time-consuming process, and so that becomes a barrier for some patients, especially when venous access is an issue and you have to use a central venous access device, for instance.  Cost also can become a barrier to adherence. 

Does adherence really matter in the long run?  The answer is a simple yes.  We know that physicians have been reluctant to actually prescribe prophylaxis in patients that come across as non-adherent.  We know that better adherence leads to less pain in patients.  It results in more self-reported joint bleeds in those who are not adherent.  We have fewer missed days from school or work in patients who are better at their adherence.  And if you are not as adherent, then you get lower physical health scores in the pediatric patient population.  So, adherence does make a difference.

At this time I would like to turn over the slide deck to my partner, Ruth, who is going to be talking about, besides prophylaxis, what other things we can do to help. 

DR. RUTH MULVANY:  Thank you very much, Jim, and thank you to everybody who has joined us, and of course to NHF, who is having us make this presentation available. 

Jim did a wonderful job of talking about prophylaxis and how that can affect joint health.  I would like to talk with you about four other things that are very important.

The first thing I believe is that if you have a condition that is chronic and life long, you need to really know about it. You need to be able to manage it. You have to have a good deal of knowledge.  I’m going to start out by talking about your musculoskeletal system—what that is and how it gets injured.  Then we’re going to talk about how to stay strong, flexible and physically active.  The third thing we’re going to talk about is achieving and maintaining a healthy weight.  Then the final one is building a good team with your HTC physical therapist, or, if you live far away and don’t have someone readily available, how you can build a team with someone near you.

So, understanding your musculoskeletal system.  To me, as a physical therapist, this is a beautiful thing—these pictures of bones, muscles, joints, cartilage and ligaments. Those are the structures that make up your musculoskeletal system.  The first picture on the left is a beautiful picture.  If we took the skin away and now we’re looking at this big, beautiful quadriceps muscle.  This is the muscle that straightens your knee.  It’s the muscle you hear a lot of talk about from physical therapists.  You will see that the muscle then has this white sheath that runs all the way down and connects the quadriceps to the bone.  When muscle connects to a bone, it’s connected by a tendon. Then this big structure right here is the kneecap.  You also hear it called a patella.  These white glistening structures around here are ligaments.  Ligaments are like duct tape that holds the joint together.

If we move over to the picture on the right, we have taken the skin away and now we’ve taken the muscle away.  We’ve got just a little bit of muscle left attached to the patella or the kneecap, and a tendon attached to the bone.  Let’s look at this knee as if someone is sitting in front of us and we’re looking straight on at their knee and their knee is bent.  Here is the femur.  This is what the surface of the femur—your thighbone—looks like.  If you notice in this illustration, it’s covered with white, which is a beautiful illustration of the articular cartilage that acts like Teflon in a frying pan.  It’s smooth.  It’s glossy.  It’s well hydrated.  It glides over the tibia, so you have nice, smooth movement.  Then if we look at the blue area here, this is an example of the meniscus.  You have one on either side—a little cartilage disk to make it all move smoothly.  Here in the middle—you probably hear ACL injuries discussed in terms of football or basketball—this is your anterior and posterior cruciate ligaments—your ACL and PCL in PT lingo.  You have a ligament on either side of your knee.  These are critically important structures. 

If someone turned sideways and we cut right through the knee and we’re looking at the inside of the knee from the side, here is the femur.  Here is your shin bone or your tibia.  Here is that beautiful cartilage that’s so important that makes everything glide smoothly on both sides.  This structure is underneath the kneecap, and is part of the bursa.  What The word synovium starts coming up.  Synovial fluid is kind of like WD-40 in the joint.  Synovial membrane—here is the culprit to joint bleeds.  The synovial membrane is a thin membrane that is inside the capsule of ligaments that stabilize your joint.  The problem with the synovial membrane is that it is highly vascularized—lots and lots and lots of blood vessels.  So, when it gets pinched or twisted, it bleeds.  As we know with hemophilia, the bleeding goes beyond what an ordinary person would have in terms of a joint.  So, inside the capsule all these structures get bathed in blood, which is very destructive to all these critically important musculoskeletal structures. 

The synovium also can become inflamed.  Whenever you hear the word itis in a medical term, it means inflammation.  Then you can have synovitis.  Above the knee of this youngster you can see this is all bulky and inflamed, and there is a condition of synovitis, which is also very destructive to all these important structures. 

If you get the chances to look at an x-ray, it’s really helpful to know what you’re looking at.  Here is that same femur—thighbone.  Here is the tibia.  Jim mentioned that it’s really important to have a space between these.  When we look at this on an x-ray, what we’re hoping to see is this nice space.  This is a normal knee. The space is created by that healthy cartilage that we have there.  That’s a wonderful illustration of a knee.  I believe this is a knee that Jim showed you.  This is a knee that has a lot of destruction of the cartilage.  You have bone on bone.  This is a very severe level of arthritis in this knee, and very typical changes that are described in some of the ways that we rate x-rays.

This one is the end result when the joints actually fuse.  There is little to no motion at all.  So, this is absolutely where we don’t want anybody to end up.

The long-term effects of all these bleeds are that there is loss of motion.  A person ends up with weakness and atrophy in all those muscles, impaired sensation, stiffness and pain.  As physical therapists, we like to talk a lot about function.  The important thing is can you stand up and sit down from a chair?  Can you go up and down stairs?  Get in and out of a car? 

This young guy’s arm has a serious problem.  He only has a little bit of bend in his elbow.  He doesn’t have enough to feed himself very well.  If both elbows run into trouble, he’s got some problems using his arms, scratching his head, button his shirt.  These are functional problems that we’d like to avoid. You can see with this older gentleman here, he’s got some pretty visible problems with his knees. 

In the musculoskeletal system with hemophilia, the most common joints affected are knees, ankles and elbows.  Muscles are also a big part of the picture.  A muscle bleed can have some very painful and some very serious problems.  Some of the common areas that are problematic for bleeds in hemophilia are the forearm, the hip, the thigh and the calf.  These are also extremely important and factor needs to be utilized for those as well.

How do all these structures get injured to start the bleed?  Impact, like jumping off of a structure.  A basketball player going up for a layup landing on one leg.  Lifting too hard or straining really hard.  Compression and twisting are problematic in terms of pinching that synovium.  It keeps going back to that synovium in the joints.  Once it has bled, then it swells, which makes it even more likely to be vulnerable for more bleeds.  So, when we talk about vulnerability, if we could avoid joint bleeds all together it would be great, because each bleed makes the next one more likely to happen—more possible, because that synovium gets swollen and pinched.

That’s sort of a quick run through of the musculoskeletal system and how bleeds happen and the affect they have.  On the positive side, let’s talk about staying strong and flexible.

Multiple studies have been done on people with hemophilia.  There is lots of evidence that says if you can exercise and if you can stay strong, you will improve your range of motion.  I did a study a couple of years ago with colleagues with people with really advanced arthritis and problems in their joints.  We found that they improved in range of motion and strength.  They had a decrease in pain.  They could walk further.  Their cardiovascular system was better.  Across the board exercise really worked, and multiple studies have shown that.

We want to develop a personal plan that involves stretching, strengthening, cardiovascular function—doing things that you enjoy.  For that, the last point that we’ll talk about is teaming up with your physical therapist and getting a good plan for you to establish for yourself.

Participating in regular activities.  What are some of the things that anyone can do, and that are especially considered good options for people with hemophilia?  Walking is certainly a big one.  There are a number of recommendations about walking up to 30 minutes a day, 45 minutes if that’s possible.  If walking is painful and difficult, cycling or swimming or some other activities, like Tai Chi might be appropriate for some of our older gentlemen.  Silver Sneakers is a great program that involves a variety of activities.  You can do it at just about any level.  For some of the older guys who aren’t quite sure how to get back into fitness, Silver Sneakers is a really good option. 

I mentioned Tai Chi.  Tons of research has been done on Tai Chi and how effective it is for balance and strength and flexibility, even for cardiovascular function and pulmonary function.  Pilates is popular and is a very good form of exercise, because it emphasizes flexibility and core strengthening, which the core is the name of the game in a lot of the fitness routines now.

Another thing is if you can’t get out and exercise, you don’t belong to a gym and you don’t feel like you have time to do things, DVDs—getting good exercise videos or downloading good exercise routines at home is a way to do it.  Set up your computer.  Set it up on your iPad and your television.  There are a number of great exercises.

The last thing I have is commercial breaks.  If a person will just stand up in every commercial break and do squats or do curls with weights beside them, if they’ll walk around, if they’ll do stretches—if you think about how many hours of your life you spend waiting for this commercial to be over so you can get back to your ball game or your TV show, we’ve got tons of time to exercise.

Another break that I like to think about is the microwave break. You put your coffee in there to warm it up.  You set it on 30 seconds.  You’re making your bag of popcorn.  You’ve got a couple of minutes.  You can do a variety of exercises.  Toe raises, squats, stretching—a number of things.  So, we can all find time to work it in.

Let’s talk about sports.  Probably the most frequently asked question that I get is what about sports.  Number one is selecting a safe sport.  Low impact, low collision, low contact are the best sports for people with hemophilia.  That eliminates football, ice hockey and some of the ones that are known to be high impact with a lot of injuries.

The other thing is to use your prophy wisely.  Talk with your doctor if you want to be involved in sports.  Schedule prophylaxis for your most active or high-risk times.  These are wise ways to make good use of your prophylaxis. 

Play a lower-risk position.  In basketball, for example, the guy that really gets a lot of elbows to the face and roughed around is typically the center.  Under the basket is where you see a lot of contact occurring.  People go up for a layup.  They land on one knee, twist their leg.  A lot of falls.  So, under the basket is really not the smartest place to be if you have hemophilia.  I recommend to the guys that I work with that to develop their outside shot.  If they could be the point guard, they’re in a little bit safer position. 

Soccer—obviously you don’t want to be the goalie.  That’s pretty clear.  Baseball—the catcher has a lot of stress on his knees and is very close to the ball and the bat, so it’s a relatively dangerous position.  The short stop and the pitcher are also pretty close to fast-coming balls. The pitcher has a lot of stress on the elbow.  So, for baseball I like to recommend that people are in the outfield, second or third base. I think those are lower-risk positions than some of the others. 

Then get assessed by your HTC physical therapist before starting, because therapists can recommend to you pre-sport conditioning.  If they know that you have a bad knee on the left or you’ve had bleeds on your elbow on the right, you can really focus on pre-sport conditioning, especially the core and the hips.  We’re learning a lot now about sports medicine.  Also utilize recommended equipment, like good shoes, good orthotics that have been recommended, elbow or knee pads or different joint supports.  And remember that rehab after every bleed is essentially.  One of the worst things you can do is try to jump back in a sport before your joint has healed. 

So, finding the best fit for fitness.  My mantra is everyone can exercise.  Avoid exercise when your joints are swollen or inflamed.  Stay in the pain-free zone.  If you want to exercise, but you get to a certain point with your elbow where if you go any straighter it’s going to hurt, then don’t go there.  Stay in the pain-free range.  If you’ve got 20 degrees of motion with your elbow, then lift your weight in that pain-free range and work in that level.  What we found previously with our research study was that pain-free zone increased.  After a few weeks the person now had 25 degrees, or they had 30 degrees of elbow motion. 

Definitely learn correct form and equipment usage.  Go slowly and build gradually.  One of the hard things for people with bleeding disorders when they start working out is they really need to progress gradually.  Everybody wants to be great and to look great immediately.  Rate your joints and muscles daily.  I’m going to show you a slide that talks about that. 

Consult your HTC doctor, physical therapist or staff.  Only progress when you’ve completed each exercise level before the bleed.

Other things:  forget no pain no gain.  Listen to your body.  If it hurts, don’t do it.  You can expect a certain amount of soreness after exercise, but that’s not to be confused with doing joint damage.  If it hurts in the joint, if you have swelling and heat afterwards, then that’s how you know you did too much.  Stay with low-risk/high-gain activities.  Remember the hips, abdomen and the spine.  The core is essential to good function, especially if you want to be in sports.  And pace yourself.  Have the patience, even though it’s hard, to start slowly and increase gradually.  Exercise about three days a week for 30 to 60 minutes at a time.  And have fun.

Rating your joints.  A hard thing about prescribing exercise for people with hemophilia is their joints can change from one day to the next.  Their right elbow may be perfectly fine with no problem.  Their left elbow may have had a lot of bleeds.  So, prescribing exercise sort of depends on where each joint or muscle is.  This is from the research study that we published in American Physical Therapy Journal.  Level zero is a joint or a muscle that’s had recent or frequent bleeds.  It’s swollen, stiff, warm or painful.  As we said before, that’s really vulnerable.  We really have to protect it and mobilize it.  Not exercise. Not weight bear.  These are your acute bleeds.  That’s not the time to exercise or go back to sports. 

Level one is a joint or muscle that is fragile.  It’s had the bleed.  It may even be a target joint.  It’s had previous injury.  It can be painful with active or passive motion or weight bearing, but it hasn’t had a bleed in the last couple of weeks.  So, this is the joint that’s resolving from bleeds or maybe weakened.  At this point we can start gentle, active motion.  We still want to avoid a lot of stress or loading, but we just want to gently get this joint moving again. 

Level two is a joint or muscle that has had a history of bleeding and it has mild to moderate impairment.  There is pain, stiffness and swelling.  There is a little weakness.  Your left elbow is not as good as your right, but it still works pretty well.  That’s your level two joint.  No bleeding in the past six months.  With this joint you can go ahead and start with active motion and then slowly add weights in one- to two-pound increments.  Exercise in the pain-free range. 

Level three is that joint or muscle that’s really had minimal, if any, history of bleeding.  No signs of impairment.  No pain, swelling, stiffness or weakness.  This one I would usually advise people to go ahead and start conservatively.  It’s going to be trial and error to get to your appropriate level.  Discuss this with your physical therapist. 

Here is an example of an exercise program for resistance that was in the study that we did that we came up with.  If you have a joint that’s a level zero, this one has got to be protected.  No exercise.  Protect it.

Now it has started to heal, so now it’s in the level one range.  At level one range let’s say that this is an elbow and you were a ten rep maximum before you had the bleed, you could do twenty pounds ten times.  You can move through full range ten times, and then you are pretty tired.  That’s your repetition maximum.  If you can do it ten times, it’s your ten rep maximum—RM.

At level one let’s say you could do 10% to 20% of your ten rep maximum.  At this point you could start doing ten reps with two to four pounds.  Week three you’re advancing.  Week four 40% to 50%.  Now you could do ten to twenty repetitions with eight to ten pounds for three sets.  A set is where you’ve done ten to twenty and now you’re resting.  That’s set number one.  The next set you do ten to twenty, now you’re resting.  That was set number two, etcetera. 

Rate each joint.  This elbow may be a level one.  The other one might be a level three.  You can see you’ve got a different game plan here.  It’s not perfect, but it’s kind of a guideline to help you see.

Where do you start with sports and exercise?  I’ve provided hopefully some pretty useful references for you here.  National Hemophilia Foundation of course has got some great information.  World Federation of Hemophilia—that’s typo there.  WFH.org.  Hemophilia Federation of America.  AAP is the American Academy of Pediatrics.  They have a great website called Letsmove.  There is another one, Protect The Ones You Love:  Childhood Injuries Are Preventable.  That’s the Center for Disease Control.  I have the website there.  Healthychildren.org.  American College of Sports Medicine.  The Arthritis Foundation is great, especially for older folks who have a lot of these advanced joint changes which are arthritic.  This is a wonderful website.  It talks about pain, pain management and a number of things.  WebMD is fit.webmd.  So, those are some resources to get you started.

Keeping your weight in a healthy range.  The impact of weight on arthritis and joint damage is well known.  There is a multitude of studies out there that talk about if you even lost 5% of the weight that you have, you can improve on your joints.  The Center for Disease Control has a wonderful guideline talking about body mass index, weight and obesity that I highly recommend.  There is a myriad of health problems that go with being overweight.  Those include heart disease, stroke, type 2 diabetes, high blood pressure and even cancer.  We know for a fact that carrying more weight promotes more arthritis.  It promotes more joint damage.  So, keeping your weight in a healthy range is really important. 

BMI is body mass index.  That’s not a perfect standard, but it’s a way that we talk about weight.  For body mass charts for teenagers you can go to the CDC, the Center for Disease Control, or American Pediatric Association and get their recommendations.  If you want to calculate what your body weight is, you can go to this website that I’ve given you here.

Let’s talk about body mass index.  In the chart that I’ve got up here now, find your height.  Let’s say that you’re five-foot-eight.  Across here find your weight.  If you’re five-foot-eight and you weigh 110 pounds, your body mass index is seventeen.  All these in blue are considered underweight.  If you’re five-foot-eight and weigh 110 pounds, you have a body mass index of seventeen, you’re considered underweight.  If you’re five-foot-eight and you weigh 140 pounds, you have a body mass index of 21.  This diagonal row of black represents normal weight.  You could even go up to 160 and you would have a body mass index of 24.  But the holidays are coming up and often it starts creeping up.  You’re 170.  Now you’re in the overweight zone.  Now you’re running into areas that are problematic.  Whether it looks pink or red on your computer screen, your body mass index is 26 and you’re overweight now based on this chart.  You can see that as your weight goes up, your body mass index moves up.  When you’re at 29.9, you’re still overweight, but when you go up to a body mass index of 30, you’re considered to be in the obese range.  Thirty to 34 is the obese range.  Thirty-five to 39.9 is considered level-two obesity.  Then when you go up above 39, that’s considered morbid obesity.  These are very, very dangerous levels to be at for all of those health risk issues that we mentioned.

Where do you start with good nutrition?  We know that we’ve really got to have the whole family committed to healthy nutrition.  The young kids are not the ones that buy the groceries. They’re not the ones that cook it.  It requires a whole family commitment to having good nutrition.  I’ve given you a number of websites here:  Dietary Guidelines for Americans, Choosemyplate.gov, American Academy of Pediatrics has great information, Slow Go Whoa from Center for Disease Control, and then there is a really interesting 5-2-1-0 Daily guide which says you should have five fruits and vegies a day.  The two means you should limit your screen time to just two hours a day.  The one is physical activity.  Try to get physical activity for at least an hour a day.  That can be getting up during the commercials and exercising while you’re wasting time with commercials.  Or it can be the microwave stretches that you do.  Zero is zero sugary, sweet soda drinks or juices.  The American Pediatric Association has waged war on sugary beverages and the problems that they cause for us.  

Teaming up with your physical therapist.  Your physical therapist, especially if you have one at your HTC, who knows you well is your best ally for this second portion of preserving your joints.  If you don’t have a physical therapist at your HTC, we’re going to talk a little bit about what you need to do.  Here’s how you get started.  You talk with your doctor.  You get exercise approval.  And you can get a prescription for a physical therapist—either the one at your HTC or one that you know—to be able to work with you to provide a good, solid foundation. 

Get an individualized evaluation.  We found out in our study that that’s critically important.  You can’t really give a blanket exercise prescription for someone with hemophilia.

Know that you may need to modify and adapt some of the activities.  We had folks who wanted to do cardiovascular, but they couldn’t ride a bike.  They didn’t have enough motion in their knees, so we got them into swimming.  We also got them into cross country ski machines, because they could do really good activity with a limited amount of range of motion in their knees.  So, get your sports recommendations, splinting, bracing, protective gear and go for it. 

Teaming up with your physical therapist.  Communicating is so important.  Let the person know you’re thinking about going into a sport.  Let your therapist know that you really do want to start toning up.  You want to start dropping some weight.  You want to start getting more fit and more healthy.  Let your therapist work with you.  Set goals together.  Make a plan and then stick with the plan.  If you have a physical therapist in your community and you don’t live near your HTC and you can get a prescription from your doctor, that person can help you.  First you’ve got to be the communicator.  You need to provide resources to that therapist, because, as most of you know, not everybody knows about hemophilia.  Once the physical therapist has been connected with the National Hemophilia Foundation, Physical Therapy Working Group, they’ve got an ally. Those of us in that group are there for people to be able to call us and for us to be able to give advice, give suggestions and give references.  NHF and the other sites that I gave you—World Federation of Hemophilia, Hemophilia Federation of America—they all have really good information, so share that with your physical therapist.  Get instructions from your own HTC therapist who can work with you to provide information to that person. 

The take home message teens—time to listen up.  Respect your joints and muscles.   I know so many older individuals who say, “I wish I would have listened.”  Maintain a strong, flexible musculoskeletal system.  Know that everybody can exercise.  If you’re overweight, if you’re stiff, if you have bad joints it doesn’t matter.  You can exercise and be physically active.  I promise you that can be done.  Choose your sports wisely.  Maintain a healthy weight.  Team up with your physical therapist. 

Those are my suggestions.  I wish everybody the very best of luck.  We’ve got a little bit of time now to open up for questions. 

ANNETTE GOSLIN:  Ladies and gentlemen, to ask a question via the web presentation, please select the Chat Pod located in the lower left corner of your screen, then type and send your question.  If you would like to ask a question live via your phone, please press star 1 on your telephone keypad.  I will announce each caller prior to bringing you into the conference.  Please remember that if you have your phone on mute, take it off mute when you are selected to ask your question.  Once again, to ask a question via the web presentation, select the Chat Pod in the lower left corner of your screen, then type and send your question.  To ask a question live, please press star 1 on your telephone keypad. 

We do have one question.  Jim, this is directed towards you.  Do you think adherence in teens might improve in the future when there are treatments that do not require venous access? 

JIM MUNN:  Thank you very much for the question who submitted that.  I think that we’ve always had this desire to improve routes of administration.  The desire to go, for instance, from venipuncture to sub-Q injections, to maybe even oral preparations have always been on our docket, so to speak, because we do believe that when you make any regimen easier that adherence improves.  From that standpoint I do believe that teens are going to get better with doing the actual sub-Q injection instead of needing to do venous access.  I do believe that is probably going to improve, but I think we’re going to fall back onto what we found as a barrier earlier, and that was when you have infrequent bleeding episodes or you don’t recognize what’s happening, adherence tends to drop off.  So, my fear is that, although patients may be taking their subcutaneous injection, if they’re not recognizing bleeding episodes and then treating those appropriately, because these products that are going to be given sub-Q may not prevent all bleeding, and if you have never recognized it for a few years and all of a sudden you have tweaked your ankle, you may go to bed thinking it was just an ankle sprain and I’m fine, and wake up in the morning with a devastating ankle bleed going on.  So, my fear is that we’re going to potentially change one scenario for another, which is what has happened with prophylaxis, as I mentioned in the presentation, that even prophylaxis has changed that piece for some families.  Families who grow up without any hemophilia in their family, who have never experienced bleeding episodes, who have had their child on prophylaxis for years and years sometimes forget that injuries do result in bleeding that needs to be treated.  We still occasionally get calls from families who didn’t let us know that, for instance, a hip was bothering somebody for a couple of days before they called us, and then we find out that there is a hip bleed going on.  So, adherence falls into a couple of things.  Yes, I think they’re going to be adherent to the actual subcutaneous injection regimen, but they may not be adherent to be treating when they have a bleed that they are not recognizing as really a bleed.

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Thank you for attending.  This concludes our program.