Jan Stephen Tecklin is Professor of Physical Therapy and past Chairman of the Department of Physical Therapy at Arcadia University (formerly Beaver College), Glenside, PA. Professor Tecklin is also physical therapist at the Cystic Fibrosis Center at The Children’s Hospital of Philadelphia . He has been very active in care, treatment and research for individuals with cystic fibrosis for his entire 35 year career in physical therapy. Included in his scholarly work are two textbooks, each in its 4^th edition. They are entitled Cardiopulmonary Physical Therapy and Pediatric Physical Therapy, with combined sales approaching 100.000 copies. In addition to hisbooks, Tecklin has many additional articles, refereed papers, chapters and international presentations to his credit. Tecklin has also been very active in the American Physical Therapy Association for which he has served two terms as President of the Section on Pediatrics, six years as an Associate Editor of Physical Therapy, the national journal of the profession and one year as Chairman of the Editorial Board for the journal.

Physiotherapy has become known these days as airways clearance. Try to clear the airways of secretions. But virtually every technique that we use as physical therapists, around the world was begun for children or adults who have Cystic Fibrosis. I am going to go through this very quickly, because Dr Ntountounakis was already speaking about the disease. When a child is born, when an infant is born with Cystic Fibrosis we think, although there is some questions now, that their lungs are histologically normal. There is no lung disease when they are born. So, chronic bronchitis and then as the disease progresses in later years, they will develop bronchiectasis, which is a disease where the airways, where the breathing tubes actually are destroyed. But, that’s a long way down the line for your children. So, the treatment approaches are primarily in terms of pulmonary disease to reduce infection, whether it will be by oral antibiotics intravenous, inhaled antibiotics which are very popular now, to decrease inflammatory disease in the lungs, because by decreasing the inflammation we think will help decrease mucus and also physiotherapy or airway clearance. And again I am just focusing now on the pulmonary disease not on gastrointestinal or nutritional problems.

Doctor Couch form Toronto said that physiotherapy for CF is like environmental protection. Everyone knows that it is important but very few people agree around the world on exactly how it should be done. So, there have developed over the last 20 years 8 or 10 different approaches to physiotherapy. And I am going to discuss each of them very briefly. There were few studies by physicians who are now retired and they suggested that physiotherapy was good. Reduces sputum, reduces coughing, improves lung function. Those are all good things that we want to do, but nothing about complications. In the 1970’s there were several more studies of airway clearance suggesting that airway clearance was good. The first two that I wrote with my former boss and mentor were published earlier on 1975 and 1976 and several others into the 80’s. And these studies all clearly showed that physical therapy for Cystic Fibrosis was beneficial. It improved flow rates of lung function, it helped to reduce the mucus in the lungs and it helped them to function better on a day to day basis. And that is really what this is all about. Improving the lung function and helping these kids to go through childhood and be the happy kids that we want them to be without bad lung disease. And it is hard work. And again, as the doctor said earlier, this is difficult work. But, I don’t have to tell you that. You know that this is difficult work. So, from the early studies we know that traditional chest physical therapy will improve the removal of sputum, will improve pulmonary function and will help maintain pulmonary function over the months and over the years. It will reduce the dropping of pulmonary function or keep pulmonary function up. One concern in recent years and this is a 5 years study, done by my friend physiotherapist from Melburne, named Brenda Button. She was concerned that when children babies with Cystic Fibrosis were tipped head down that they would have reflux, they would vomit and get it into their lungs. And she studied these children for a 5 year period of time and this was the 5 year study completed into the year 2003 and she found, in fact, that children babies who were treated without head down, flat, had better pulmonary function than babies who were treated head down. And she believes now, very strongly, that infants with Cystic Fibrosis should not be put in a head down position but they should be treated flat on the bed for their chest precaution all around. In US the Cystic Fibrosis Foundation agrees with this research and today it is very unusual to see a child, a baby with Cystic Fibrosis tipped in a head down position. And that is a recommendation that I would make based on this research. So, physical therapy with chest precaution and vibration and positioning was the only real opportunity for airway clearance until the 1980’s.

One of the things that begun to happen in US and other parts of the world in the 1980’s is children because of better medical care, better antibiotics, better nutrition, better airway clearance they begun to live longer. This is a treatment called Autogenic Drainage. This is used for older people with Cystic Fibrosis in their teens or above. It involves having them breath at different lung volumes. First at a very low lung volume, so we have them blow out all the air and breath for several minutes. And a bigger breath. And all this breathing loosens mucus. And then, when we hear the mucus or feel the mucus with our hands, as physiotherapists, we go to very deep breaths. And at that point the child can bring the mucus up without a big cough. One cough and mucus comes up. It works well. It is called autogenic drainage. It is a little bit difficult to teach. But, usually, in one or two sessions of teaching, the physiotherapist can teach the child how to do this. The benefit is that the child can do this anywhere. No equipment, no any clapping on their chest. They can be driving to work as an adult doing autogenic drainage and doing the airway clearance. So, it is time saving as well. There is one or two research studies about autogenic drainage and in fact it works as well as the traditional chest physiotherapy and some of the others that I am going to be talking about.

Positive expiratory pressure. This was originated with a mask. A mask that makes it a little bit difficult to blow out. And that little bit of difficulty is the positive pressure. You are blowing out against pressure. Why is this good? How is it work? It works because as we blow out against pressure our airways will be kept open, in an open position. Normally, if we blow out, our airways close down, our breathing tubes close down. But if we blow out against pressure it keeps the airways open. Why is it important. It is important because as we blow out mucus is dragged, is moved from our lungs up to the larger airways to a place where the child can cough it out much more easily. So, keeping the airways open be blowing out against pressure is positive expiratory pressure and your physiotherapist all know about this. This is a very effective technique. But again, people have to know how to do it. And that is one of the reasons I am here with you this evening telling you about these techniques. And what this does. It not only keeps the airway open, because it is positive pressure, but the fluttering shakes the mucus loose from the airway wall.

If you look over here, that fluttering shakes the mucus loose as the ball moves up and down. And loosen the mucus, with the positive pressure keeping the airways open, can then move out of the lungs much more easily. Very effective, not particularly expensive. The problem with it is people forget to use it. They put it in their drawer in their desk and they forget about it. This can be used anywhere (image). In school, if you are feeling congested, go to the bathroom, use your Flutter, cough, back to class. I would recommend this for children who are about 5 or 6 years old. There is a certain way you have to hold the flutter, that it is a little bit difficult to learn. But when the child blows in the flutter, you can feel, if you put your hands on the child’s chest, you can feel the sensation that is loosening the mucus. It is like doing this, only much faster. So, that is the flutter. Again, it works, it doesn’t work better than anything else, it works well.

There are two different Acapella. One is called the Acapella, one is called the Acapella choice.

The Acapella choice, that is part of the name and you can see at the bottom of the slide, is newer and is better for people with Cystic Fibrosis. The reason it is better, it is much easier to disinfect and clean. To get rid of any bacteria. So, if someone recommends an Acapella, I don’t know if they have them in.

This is called intra-pulmonary precausive ventilation. I have personally not used this very much at all. But essentially what this does, this is a system run by compressed air, as you can see, so it is probably used in a hospital or clinic. And the child breaths into this unit while the unit is doing two or three things. One, the compressed air is providing positive pressure. So, we have four different devices that provide positive pressure.

High frequency chest wall oscillation. You might hear of this as the Smart Vest. I am going to show you some pictures of this. This is a vest, a garment that is worn by the child and into the garment and I will show you a picture of it, is an air hose, it sends bursts of air that shakes the chest wall, it oscillates the chest wall. And this is the device. This is the vest. And inside the vest is a blotter or a balloon enough strong and into that balloon you can see this tubing. The single tube comes from this air compressor. This air compressor or air pulse generator send bursts of air. Up to 20 bursts a second into the vest. And you can control the speed of those bursts. You can also control the pressure, how hard it squeezes you. But essentially what this does, it provides oscillation from the outside of the chest. It basically makes like this, up to 15 or 20 times a second. Again there are devices down here on the control panel where you vary the speeds, so maybe it is only 10 times a second, like this. Or maybe it is 20 times a second. But you can vary it depended on the size of the child, depending on the comfort. We know from research, going back as far as 1991, that this is effective in the long run.

There was a study by Dr. Waring from Minnesota in US who actually invented the original vest system that found a children for 21 months using the vest as physical therapy and found the pulmonary function either stayed the same or improved, it did not deteriorate. And every time the vest squeezes it will cause a burst of air from her lungs and that burst of air does three things. Number one it will make the secretions less thick. It will liquefy them. Number two it will push them up in the lungs. Number three, it will cause an increased air flow out of the lungs to draw the secretions that have been loosened in this oscillation fashion.  Today we believe in this too grade degree.

I work in children’s hospital in Philadelphia where we have 300-350 children and young adults who have Cystic Fibrosis in our clinic. And there probably 200 of them who are using high frequency chest wall oscillation on a regular basis.  It is effective, it is very easy for the child to do. A child of 6 or 7 years old can do it itself. You don’t need someone to do it for you. And it enhances the nebulizer. The nebulized medications will go deeper into the lungs when the smart vest is being used. So, all this is positive. Families love it. We usually start using the vest at about 5 ears of age. And families are begging “can I use the vest”? Because there is not a small one for infants. The one downside to the vest is its cost. It costs between 13000 and 17000 dollars US. It is expensive. The smart vest that you can see in is the 13000 dollar vest system. My colleagues and I, we followed a group of 102 children for a year using the vest versus 55 children getting the traditional chest PT at home. And the vest was as good as the chest physiotherapy at home. People say why should we spend so much money if it is no better, if it is only as good as. The reason I say and the reason that it is used so much in US is the thing quality of life which can be measured in Cystic Fibrosis. And the quality of life has been showed to be so much better with the vest, than you trying to chase your child down and holding him or her in a place when you do chest physikotherapy and they yell and they scream. They tend to really like this. You put it on a 5 year old and that will be laughing. And there is not a lot of CF therapies that starts with the child’s laughing.

So, those are the various techniques that are commonly used. It is hard to generalize one from the other. There have been lots of work, lots of studies down at the last 20 years, that are trying to see which one of these is better. And I will come back to that point in just a minute. There have been more than 300 research paper written about physiotherapy in Cystic Fibrosis. Despite these 300 papers, none has been shown to work particularly better than another. And I think I know why. Not because I am so smart, because I am so old. I have been doing this for 36 years, almost 37. Why not? Because there is no best treatment overall. What I believe is it will depend on the age of the child, the severity of the disease, the preference. You will like Acapella better than you will the flutter. You may like the SmartVest better than you like the chest PT. And that is fine. You should use whatever you prefer. Because if you prefer it, if your child prefers it he or she are more likely to do it. Very simple. And there is research that shows that. If you like something you are going to choose it. The other consideration, and I always love to talk to groups of parents and people with CF, because it is not mentioned often enough by medical professionals, physicians, nurses, therapists, nutritionists, whoever. We need to be more aware of the time and the physical demands and the emotional demands that Dr. Ntountounakis spoke of earlier. You, people who have children with Cystic Fibrosis or if you have Cystic Fibrosis you work hard, 2-3 hours a day is what most of the research says, treating the disease, trying to keep yourself well. And that is hard work. Doing physical therapy early in the morning is important but it is difficult. Kids are going of to school, husbands and wives are going off to work, your house is probably in chaos in the morning just like mine used to be, when my kids lived at home. And on top of that you got to do an hour of treatment. It is difficult. So, we need to work with you. The aging of condition of the patient and the care giver. It is very difficult for someone who might have syndrome or bad arthritis in their hands and arms to do chest precaution. Work with them. We, as therapists, we have to have reasonable expectations. I will teach you a technique, do I expect you to do it three times a day, seven days a week, 365 days a year? No. I would be a fool if I thought that. But, what I expect is you are going do it most of the time, whatever that is. And I am going to meet with every six months or every year to talk with you, to make sure you understand it, to see if you have any questions, so that in fact you will do it most of the time. And to foster independence in our children. As your children get to be 10 and 12 and 15 years old they are going to want to develop their own life. And their airway clearance their medications and the nutritional needs, need to be independent. They need to be participating probably from around pre-teen years. They need to start understand, may be even before that, how and why they are getting all these medications, 12, 13, 14 different medications. “What do they do? Why I am taking them?” In age appropriate language. So, that is my talk on airway clearance for people who have Cystic Fibrosis. I’d like to thank you for this opportunity. It is truly an honour and a pleasure to visit and I would like to thank the people who invited me, the Smart Intermed company for inviting me, with Ms Stella Mitsios and her colleagues in Smart Intermed. Thank you all very much.

 

 

 

 

 

 

 

back....