For the first time in its history the EAMDA Youth Organization (EYO) arranged a seminar, which took place from October 6 to 9, 1994, and which at first glance had a medical theme: "Respiratory Support and Young People with Muscular Dystrophy"
When the idea of arranging a seminar with a medical theme was first mooted late 1993, agreement upon the theme was soon reached: "Respiratory support and young people with NMD".
Why this subject?
Respiratory support is a type of treatment, which directly affects the lives of young people with a muscular disease.
In this report you can among other things read about the experience, which some of the participants at this meeting have gained in connection with respiratory support. The medical aspects and the practical experience related to respiratory support are highlighted. What makes this report so interesting is, however, that there are so many and different experiences.
You can further see that the most important conclusion reached at the seminar was that the national muscular dystrophy associations must give the question of respiratory support top priority. „After all, everyone has the right to live, and it is impossible to live without being able to breathe air. Air to live".
As a result of this EYO seminar EAMDA must soonest possible come up with a clear and practical programme regarding respiratory support, a programme which will really enable the affiliated associations to take action. In order to achieve results within a reasonably short time the participants in the EYO seminar propose the following:
That an information package filled with new and already known information about respiratory support be produced, and further to make sure that this information will be available for all the people, who are involved in the aspects of respiratory support.
In all this the EYO must be sure to observe what is in interest of the young people, and to make sure that the information provided is understandable and not too professionally oriented, that patients are able to identify themselves with what they read and are able to use the information given. In our opinion this report is a step in the right direction.
The participants in the EYO realise that all this is not a simple task, and that it takes time. Nevertheless, in their opinion, everyone has a right to be informed correctly in order that he/she can finally „breathe with relief".
Dick Cochius
EYO Chairman
The role of the national muscular dystrophy association:
In the area of respiratory support the national muscular dystrophy association plays a very important role. In our opinion the association has first and foremost the obligation to provide information and guarantee contact between its members. Among those members are the young people within the association. Especially these members need to be well informed by their association.
The role of EAMDA:
With a Europe without borders it is the task of EAMDA to advance the same medical treatment in every country for people who needs respiratory support. Furthermore to advance that this group is given the opportunity to live an independent life with the equipment and help they need. In the chair: Evald Krog, President of EAMDA and National Chairman of Muskelsvindfonden
Professor, Bent Juhl, M.D., former head of the respiratory centre at Århus Kommunehospital, started the day by giving an introduction to the normal functions of the lungs and then to the specific problems in relation to muscular dystrophy.
The lungs are not affected when you have muscular dystrophy. The respiration problems are a result of the reduced muscle power, only. The treatment is, therefore, aiming to compensate for the poor muscle power. You might say that the aim of the treatment is very simple - to secure an improved functioning of the lungs."
Symptoms of reduced lung function:
The patients are often complaining about many different problems. Some of the quite young patients cannot remember how they were when their respiration was good. First when they have obtained a good respiration again it is possible for them to remember how they were several years ago.
The most common symptoms are problems with intermittent sleep, nightmares, headaches, abdominal problems, etc.
Prevention of respiratory problems:
It is very important that the body is trained. God or nature have designed the lungs to be used. It is important to move as much as possible, and if you are unable to do so yourself then you must have someone to help you.
Another important aspect is to inhale deeply now and then so the lungs unfold completely. If you are unable to do so yourself it is advisable to apply a C-PAP (continuous Positive Airways Pressure). The C-PAP operates like a reverse vacuum cleaner and it provides "breaths" of 20 to 30 litres.
Treatment:
The respirator treatment depends on the muscular dystrophy diagnosis and thus on the degree of power reduction in the muscular groups which affects the lung function.
It was previously quite normal to apply an iron lung or a suit which could create a negative pressure having the effect on the lungs that they were alternately being extended or pressed together.
In connection with the major polio epidemic in 1952 it got common to apply tracheotomy which enabled the application of positive pressure. The air was now blown direct into the lungs - initially by help of a balloon. Later the machines were applied.
It is important to remember, though, that the treatment was only offered to people with acute respiratory problems.
Not until the muscular dystrophy organisations fifteen years ago launched the debate about respirator treatment of patients with a neuromuscular disorder the medical world started to consider respirator treatment. In Denmark this treatment has become commonly recognized in the nineties - amongst other things because a new law came into force which stated that there should be two respiration centres in Denmark, one in the eastern part and one in the western part.
One of the therapy methods which we often apply in Denmark, is the so-called B-pap. It is based on a change between pressure and non-pressure. The equipment can feel when the patient intends to breathe, and it then follows him/her. If the patient does not breathe then the respirator takes over the breathing.
To-day the respirator is very noisy and it is difficult to keep a sufficient humidity. Great efforts are made to improve on this.
Questions and discussion:
Gregor Schwarz: How is it that our respiration centre does not inform us sooner - and that several of us have to collapse before we realize how serious it is with our respiration.
Bent Juhl: The body slowly gets used to the poor respiration. You can say that it comes to depend on a high concentration of carbon dioxide. The body is already filled with CO2 so you don't feel it.
Nurse Laura Verley from Home Mechanical Ventilation in Utrecht, The Netherlands, told how muscular dystrophy patients with respiration problems are treated in the Netherlands.
I am a nurse at the Centre for Home Mechanical Ventilation in Utrecht. This is one of four centres in the Netherlands, which assists people who are in chronic need of respiratory support. Today I want to tell you about chronic ventilation problems starting with some definitions, after which I will try to explain how we work at the centre.
We tend to consider respiration to be quite normal and necessary body function, and we do not realise that there is a group of people, who are so unfortunate that their respiration is not normal and who have to work very hard to keep their respiration up to standard.
What is respiration ?
The function of respiration is to ensure that an efficient exchange of gases takes place so that the body is supplied with oxygen (energy). Carbon dioxide (waste product) leaves the body via respiration. It is important for the lungs to be well ventilated (the air must circulate freely or it will become stale, as in a poorly ventilated room). There has also to be an efficient exchange of gases at the level of the air cells in the lungs. The respiratory muscles are responsible for good ventilation, by enlarging the chest cavity so that plenty of fresh air enters in. This is done by the muscles of the chest and the midriff.
If this is not entirely successful the condition is known as hypoventilation.
The symptoms of hypoventilation are as follows :
- Headache on awaking
- Restless sleep, often awake
- Dozing off by day due to lack of sleep at night and rising C02 levels
- Lack of concentration
- Excessive sweating at night
- Difficulty with shouting and singing
- Difficulty with long sentences, unable to count to ten in one breath
- Depression or euphoria
- The C02 level in the blood may become so high in the end that the patient will go into coma and die if not ventilated.
Mechanical ventilation
Mechanical ventilation is artificial ventilation of a patients' lungs with and without the aid of equipment designed for that purpose. Ventilation is applied to patients whose lives are threatened by a temporary respirator failure, where recovery may reasonably be expected sooner or later.
Ventilation is also applied in the case of patients whose own respiration is not likely to recover in the light of their symptoms. This is what we call chronic ventilation. The quality of life is expected to improve or at least to remain stable as a result of ventilation.
The object of chronic ventilation is to take over the function of the respiratory muscles, for instance in a patient who is completely paralysed as a result of a lesion of the spinal cord . But it is also given to patients with a neuromuscular disorder where the muscles are weakened and have to work ever harder to keep respiration up to standard. Exhaustion eventually causes the respiratory muscles to fail, resulting in respiratory failure. These people are often able to breathe just sufficiently by day with the aid of the accessory respiratory muscles (these are the muscles of the neck and shoulder girdle). When they go to sleep they stop using these muscles consciously and respirator failure is the result. Given mechanical ventilation at night, the muscles can rest and the C02 level in the blood does not go up during the day.
In the Netherlands chronic ventilation is given in the following cases:
- People with a high cervical lesion of the spinal cord, which also causes paralysis of the respiratory muscles.
- People with a neuromuscular disorder such as Duchenne, Pompe's disease, atrophy of the spinal muscles and Amyotrophic lateral sclerosis.
- People with respiratory failure as a result of poliomyelitis, especially those with a kyfoscoliosis.
- People with Chronic Non Specific Lung Disease.
- People usually elderly who remain dependent on mechanical ventilation after major surgery (oil the thorax or abdomen).
Chronic ventilation
Chronic (intermittent) ventilation is a comparatively unknown and to many a controversial form of ventilation. It's originated in the fifties after the epidemics of poliomyelitis (1956 in the Netherlands) which left a number of people so dependent on mechanical ventilation that they were receiving chronic ventilation. Some of them were admitted to units set up at that time for chronic ventilation (,,De Ark", Rotterdam 56, ,,De schelp" Scheveningen) others stayed in hospital an a few were sent home.
At that time it was rare for patients with other neuromuscular disorders to be chronically ventilated.
There was an increasing call for alternative accommodation for these people and this was certainly not due to the lack of IC beds alone. The people themselves were in a stable condition so that they were out of place in an IC unit. The Centre for Home Mechanical Ventilation at Utrecht began removing chronically ventilated people from the hospital as from 1970. They either went home, or to at nursing home, or into a form of sheltered housing (where people can live independently but with a care attendant on call) or to a rehabilitation centre.
When a person decides that he or she wants to be ventilated the first concern is to see if ventilation is possible in their accommodation. I may be necessary to go into a nursing home if ventilation at home is no longer feasible because of the added strain of care. Not every nursing home is willing and able to admit patients with chronic ventilation. There are financial matters too, which will have to be settled. Home mechanical ventilation is paid for by medical insurance in the Netherlands unless the patient is in a nursing home, so firm agreements must be made in advance. At the moment, together with the VSN and the other centres for home mechanical ventilation we are looking into the possibility of selecting some ten or so nursing homes in the Netherlands to house ventilation patients.
We now have four centres for home mechanical ventilation in the Netherlands (in Groningen, Rotterdam, Maastricht and Utrecht) assisting over 300 people. Our team in Utrecht consist of:
- three doctors (internist)
- three nurses
- two secretaries
- two technicians
- a dietician
We also co-operate regularly with the paediatrician of the Wilhelmina Children' s Hospital, the neurologist, the ENT specialist, the rehabilitation specialist and the orthopedic surgeons (concerning corrective surgery for scoliosis).
The main tasks include:
a. providing information
b. out-patient examination
c. admission for screening
d. preparation for mechanical ventilation
e. instruction
f. assistance at home
g. inspection
a. Providing information
Providing information in the earliest stage to people who are expected to need respiratory support in the future. This is done by:
1. Joint consultation in the various rehabilitation centres which are held regularly in the presence of the rehabilitation specialist, the orthopedic surgeon and an internist attached to the centre for home mechanical ventilation.
2. Publications in journals including that of VSN.
3. Attendance at and/or organisation of information sessions.
4. Fellow-sufferers - many with muscle disorders know each other via school and sport and come into touch with ventilation in this way.
b. Out-patient examination
The functioning of the lungs is examined; the resulting values and patient's symptoms are an accurate guide to the deterioration in the functioning of the lungs and an indication as to when exactly to-start ventilation.
If the lung-function-values show a marked deterioration, and the blood-gas-values and the PaC02 level are higher (normal values Pa C02 35-45 mm HG = KPascal,) or if the symptoms clearly indicate hypoventilation, then the patient is admitted to IC for blood-gas-measurement at night by means of an arterial line. This is usually carried out during two or three nights while the patient sleeps.
c. Admission for screening
Unfortunately we are still obliged to admit these patients to an IC-ward because blood gases are removed via an arterial line, and this is not allowed on a general hospital ward. It is rather unpleasant for the patient and generally noisy so that he/she does not always sleep well. That is why they have to stay in for at least two nights and, if possible, in a small room.
Blood gases are removed via an arterial line during the night and ventilation is started if the PaC02 is higher than 55 mm HG.
d. Preparation for mechanical ventilation
Chronic ventilation can be given in three ways :
1. By means of a curas, also known as indirect or negative pressure-ventilation. The curas is a shell made to measure to fit over the chest wall and abdomen. An appliance is used to create. a vacuum between the shell and the chest causing the chest to rise (as when inhaling) and air is sucked in. Then the negative pressure (vacuum is released and the chest returns to its original position while the air is exhaled. This is a modern version of the iron lung.
2. By means of a nasal mask. Air is blown into the lungs via the nose by means of a nasal mask which is made to measure (the mouth is left free), This is important to keep the mouth closed to prevent leakage. A chin stap or a mouth plaster can be applied.
Nasal mask-ventilation is usually applied to start with unless there is a contraindication such as one of the following:
- problems with sputum
- recurring bronchial infections
- difficulty with swallowing
- ventilation for longer periods by night and day
- insufficient passage of air through the nose due to a crooked septum
Disadvantages:
- sputum cannot be removed by suction
- some people suffer from excessive wind in the stomach; this is usually passes in time. I.E ratio of the mechanical ventilator
- eating and talking are very difficult during ventilation
- not suitable for lengthy ventilation (more than 12 hours per day).
Advantages:
- no stoma
- time to get used to equipment.
3. Via a tracheal cannula. An opening called a tracheotomy is made surgically through the neck into the windpipe. A tracheal cannula is placed in the opening.
Air is blown through the cannula straight into the windpipe to the lungs. Ventilation is usually easier to regulate in this way. One big advantage of the tracheal cannula is that it makes it easier to remove sputum by suction.
After the operation patients are first fitted with a cannula with cuff which makes speech impossible while the cuff is inflated. This is because the tracheotomy is placed underneath the vocal chords (just under the Adam's apple) and air has to pass along the vocal chords before they can produce a sound. The cuff prevents this. That is why the cannula used must not be too big so that when the cuff is empty air can pass alongside the cannula. It is best if the cannula can be entirely sealed off during spontaneous breathing and the patient can breathe ,,normally" via the nose and mouth. The cuff remains inflated for the first five days after the portion to give the wound a chance to heal and to prevent blood from entering the lungs. Swallowing is sometimes difficult at first and the cuff also prevents sputum and water from entering the lungs. Difficulty with swallowing may also be caused by the cuff (dia) and the nasal catheter with which patients are fitted in the operating theatre for the first few days food supply.
After this ventilation will be attempted without the cuff meaning that the air which is blown in via the cannula is not only blown downwards into the lungs, but that some of the air can also leak away along the cannula via the nose and mouth (that is why we call this leak-ventilation). It will depend on the rigidity of the chest wall and the muscles surrounding the neck whether this is successful. If ventilation succeeds without the cuff and there are no problems with swallowing then a cannula without the cuff will be inserted. A silver cannula is preferable because it is beautiful material, durable, and with an inner cannula. An inner cannula is pleasant because it prevents the cannula from clogging and it can be removed for cleaning.
Sometimes it is not possible to insert a standard silver cannula due to the anatomy of the windpipe (often applies to people with a serious kyfoscoliosis). It might cause damage to the wall of the windpipe. A plastic cannula may be chosen instead, or adjustments may be made to the silver cannula.
If ventilation without the cuff is unsuccessful we try to give the patient a cannula during the hours that he does not need ventilation so that he can talk.
e. Instruction
The patient and his/her care attendant are given instruction as soon as possible. If the patient lives in sheltered accommodation or a nursing home the care attendant has to be authorised because suction is a medical operation in the Netherlands which can only be carried out by doctors.
Authorisation entails that the care attendants are trained and considered qualified to carry out these tasks.
The patient can only be allowed to go home when everyone knows how to use the equipment and the situation is stable.
f. Assistance at home
Nurses from the centre will accompany patients to their homes and give any additional information to the General Practitioner and district nurse if this has not already been done at the hospital.
Once the patient is at home the nurse will call in regularly to see if everything is going well. In addition the technical department and the doctors and nurses are available 24 hours per day to deal with defects in the equipment and medical problems, respectively. We try to arrange for patients to visit the clinic once a year to be seen by the internist of the centre and the dietician. The lungs are x-rayed, an ECG is made and blood is taken for tests at the same time.
g. Inspection
At least twice a year the settings of the ventilator are checked at the patient's home by means of an oxicap measurement. This appliance is delivered by the nurse during the day and the patient connects it himself at night. All night long information is registered concerning the oxygen level in the blood, the carbon dioxide content of the exhaled air, and the frequency of the heartbeat and the respiration. Next day the oxicap is collected again and interpreted and if necessary adjustments are made to the ventilation.
Conclusion
There are now over 3OO people in the Netherlands living at home or in sheltered housing, a nursing home or a rehabilitation centre, who receive respiratory support. This all works very well from a technical point of view. But the strain, both emotional and physical, especially on the parents of young people living at home and the partners of elderly patients, is sometimes too great to bear. There is room for many improvements, for instance additional relief facilities for a holiday for parents or during illness, especially for the people who live at home. A fund enabling them to engage people so that they can go out sometimes, etc.
It is sad that the present developments in the Netherlands health service are not likely to promote improvements.
Lene Lauritsen, a student of psychology, who herself suffers from rheumatism, told about the psychological aspects of being handicapped.
"As a handicapped I think that we are living through several, strong crises our entire lifetime. We experience more existential crises amongst other things because we are now and then confronted with the death. But we get used to it. "
When you are suffering from progressive muscular atrophy/muscular dystrophy crises will occur from time to time.
The word crisis originates from ancient Greek and means turning point/dramatic peak.
You can talk about different kinds of crises. The existential crisis and the acute crisis.
The existential crisis
Everybody has experienced it. In periods of your life with fundamental changes. Life conditions change: biologically (puberty, getting older) our special condition make us more vulnerable but also stronger. Psychologically (growing up). Socially: education, raising a family, work.
The Swedish psychologist Cullberg speaks about natural crises for each seven years of a person's life.
What's the meaning of life, who am I, what do I want with the feeling of being vulnerable, insecure, confused about my identity, emotionally unstable, afraid.
The acute crisis
Cullberg has the definition: a man is in crisis when he is in a life situation where his previous experience and ways of reaction aren't sufficient to understand and psychologically cope with the new situation.
There has been an event in the outside world. A crisis situation. For example death, sickness, divorce, disablement. Those situations don't necessarily come to a crisis. You can be sad, but it depends on your social network and how you have previously coped with difficult situations. A crisis is always limited in time. You can't be in crisis all the time, max. for one year. The crisis is a normal reaction to a traumatic event.
Three facts have to be present to talk about crises:
1. loss or threat of loss
2. the feeling of being overwhelmed by emotions
3. confusion and lack of ability to solve problems.
Your individual reactions depend on earlier experience, and what is important in your life. It's much easier for you if your are prepared or if it's possible for you to influence the crisis.
In our culture we don't like to talk about illness and the dark sides of life - in general about what can hurt. Therefore it can be a lonely experience to be seriously sick. At the same time the person in the centre has a great need to talk about what has happened again and again. This must be acceptable and understandable to anyone.
When you have come well through a crisis you can start thinking realistically about what you have actually lost. And what you have gained, how you can see new opportunities.
A crisis can be turned to something positive. There is a potential for development all through life.
This is often forgotten when it's concerning people with weaknesses like seriously handicapped/sick persons.
You can have some new opportunities when passing through a crisis. You can throw away an old role, make closer contacts to other people, get increased knowledge about yourself - be self-realistic about what you can and can't handle.
It's also important to remember that not two people are alike. What would cause a major crisis in one person's life is just a sad incident in another person' s story.
Having muscular dystrophy can bring up crises during your entire lifetime. Old crises may pop up again when you come to a new crisis, and the re-occurring old crises may make the new ones even more complicated.
And a crisis, which you have not quite come through can cause bitterness.
Questions and discussion
Ivan Jakobsen: For me it has always been important to be together with others having the same disease and to see others in a situation similar to that of mine. I have also always been good to advise my friends, but I am unable to listen to the good advice offered me by others.
Christian Fischer: The most difficult for me is when I fall in love with a girl. It is much more difficult for me than it is for others to get in contact with a girl - and this cannot be changed.
I have had a girlfriend who didn't want to keep me because of my illness. Nobody can help me in this respect. The biggest problem for me has for many years been that I don't have a girlfriend.
Dick Cochius: It may be because they are afraid of your future that you will become more ill and shall have to have a respirator ! No future, so to speak.
Inger Stoltze: Sometimes I think it is difficult for us to make friends, to let them come close to us. Anyway I have experienced it so. Until one day when someone said to me that now I should start using my friends and come out of my shell.
Dick Cochius: When I should have a tracheotomy it was very nice having some friends to whom I could phone.
Ivan Jakobsen: I was afraid of dying. It was my first worry. The next was what kind of respirator I was going to have. But I have got used to it as if it were a pair of glasses, I was going to wear.
Gregor Schwarz: I had to force myself the first time I was to go out wearing my nasal-mask. One day in the city a little boy asked his mother what it was I had on my nose. "It's a respirator," the mother answered. "Can he dive with it", the boy asked again without getting an answer.
Actually it is most difficult with my own mother. She worries always a lot and doesn't think I can manage on my own.
Lene Lauritsen: I think parents feel guilty because we are as we are. But I also think that we are too afraid of telling our parents what we are thinking about our lives. We are doing so in order to protect them. But of course our parents can manage to hear about our lives.
When I moved from my home it was a relief for my mother. She had always been so worried for me and so each and every day when seeing me. Not until two years after my move she dared to tell me how much she had worried.
Frank Schut: Sometimes I hear some parents say that they do not hope their child will decide for a respirator. They are just fed up with caring for you any longer.
Dick Cochius: They had imagined that they should care for us until we died, but then the ,,damned machine" came. But we mustn't blame our parents. You must remember that in many countries - also in The Netherlands and Germany - they don't have the helper arrangements which are necessary before you can speak about a real choice.
I got polio in 1953, when I was one and a half years old. Polio is a virus which attacks the muscles and in some cases also the lungs and the heart. Thus the capacity of my lungs and heart has been reduced to 50%.
The dramatic event, which I will now tell about, took place four years ago. I was nearly unconscious because there was extremely little oxygen in my blood. I was in a black tunnel and saw a tiny little spot of a shining, white light at the end of the tunnel. But somehow I couldn't reach the shiny doorway.
Anyway, I am still here today, because I got a ,,little help" from the specialised doctors at the very top of the respiration-area, and a little help from all my friends, too.
I had been a person, who never paid much attention to the signals from my body. I had been a teacher for 13 years, and I had been walking and standing in front of children four to eight hours each day talking and listening to them. It was a job, which demanded a lot from you, but funny and interesting. It meant a lot to me to be doing what I did. Some years back a doctor (and my body) had told me, that I ought to stop working. But I didn't listen or didn't want to do so. I ignored my tired body and the doctor's words and continued to force myself to work.
Although my breathing had troubled me for some time, I suddenly realized that something very seriously happened with me during a study trip with my school to London. I couldn't breathe freely in the plane. But I didn't tell anybody. I thought I was only tired, but 14 days later I contacted my own doctor and he answered that ,,perhaps I had been nervous to fly". I was quite sure, however, that this wasn't the reason. Nevertheless he refused to examine my lungs further. (I have to tell you that he didn't know me very well as he had only been my doctor for three years).
Then I myself went to the hospital. It was established that my heart was too big. OK, something was really wrong, and I stopped to work.
One week later I collapsed and was then connected to a respirator for four days at the intensive care at the hospital.
Up to this I had been very afraid of going to sleep because every morning I woke up with a terrible headache and I feared that maybe one day I wouldn't wake up at all. But still I didn't tell anybody about my anxiety. I stayed at home, was very quiet, watched TV a lot - and I was always very tired and felt drowsy.
Most of all I didn't understand a bit. I had never heard about polio-people who had symptoms like this. I had never known anybody like this, and I didn't identify myself with the people around me with muscular dystrophy. No doctor ever told me that I suffered from post-polio and pneumonia in both my lungs.
I prepared myself to die. And it was too late to listen to myself. So I collapsed but I didn't die.
Never before had I learned so much about myself as I did during one month at the hospital - and the subsequent years, too. The most important thing that I learned was that I had to be responsible for my own life.
I woke up on May 1, four years ago. In a way I was very happy, because I could hear somebody breathe in the same room as me. That was nice, I was not alone in the room, I thought. Some hours later, I slowly noticed the sound from my own breathing through the respirator. I was a the hospital and ,,someone" was breathing for me. Nice, but very strange. I had many questions - what had happened and things like that. All my friends crowded round my bed, but I was unable to talk with them I could only write on a piece of paper. I got all the answers, some very happy, but I remember one of my best friends was: very angry at me. Why haven't I told them that I was that sick ? Who knows you, by the way ? Does anybody know you at all ? Do you let anybody come close to you ?- and I understood why she said so. But at that time, I didn't know myself and my body very well, so how could I tell other people what happened to me. I was just very confused and I only knew, that I had to start a new life. To hire some helpers, get an electric wheelchair and a new car, so that I could drive the car myself sitting in my electric wheelchair.
Half a year later I got pure oxygen 14 hours a day and had to stay at home by the oxygen-machine. It was shocking and very boring for me. I had been used to go to work every day, this was no life. And in that half year, the doctors didn't allow me to drive the car because I still had too little oxygen.
Well, time can change. I met professor Bent Juhl whom you saw here yesterday. After some consultations, examinations and very careful information Bent Juhl offered me the BiPAP (respiratory support).
He became my hero, I was and still am a big fan of this man.
The trouble with me, he said, was my high C02 during the nights. And the muscle in my stomach wasn't working very well. The BiPAP-machine would help me to breathe nicely during nights. In the daytime I could do without the BiPAP and lead a normal life again.
But I decided to quit my job as a teacher.
After three and a half years with my ,,BiPAP" friend, I have had many nice hours - without headache in the evenings, nights and days. Now I can be concentrated for more than one hour, if I want to. Now I am more in contact with my own organisation PTU (the Polio Institute in Copenhagen). Each second year I am trained at the Institute. And I benefit very much from that which the muscular dystrophy people finds about respiration. But I must admit, that still I have difficulties to introduce new friends to the ,,BiPAP respiratory support".
I mean, you might look a lot better if you didn't have to wear this silly hat in the night-time. One of my best friends, with whom I was in love, and who was going to spend the first night with me said with a big smile ,,Inger it is OK with me." You look like an elephant and sound like an aeroplane".
I mean, where do you find any romantic aspects of this machine ?
I'm still very shy, when I put it on and sleep together with another person. But I do it.
At the beginning for me it sounded like an ocean . Really - now it is more or less gone, this ocean. But I can't hear the birds in the morning. I can't hear if it rains or the wind is blowing in the trees. I even can't enjoy thunder in the nights.
That is unacceptable. All your life you have been used to wake up with these sounds around you. It's the start of your day. I want the nature back again. I am a very romantic soul. And even when you walk in the woods in daytime, you can almost only hear the sound of your electric wheelchair.
Noise in the night, noise in the daytime. I won't accept it. But I am very happy with my new breathing. I have got a new life and I became more aware of my body after the first year, four years ago. That is very important and I accept that. I'm painting now, and is a student at the art-academy, which I have always dreamt about.
I have started a theatre - and culture association ,,Korona", and I am very much involved in this work. We make workshops with dance-improvisations for people in wheelchairs, art-exhibitions etc. This Korona is for a mixed group of able-bodied and disabled persons. Here I can use some of y organisational qualifications from my teacher- job. I can be teaching again, but I miss the children.
Now I am quite happy with my life again. The next step will be to get a full-time respirator. OK, let it come, I have said to the doctors. I don't need it as yet, but I think I will accept it, too, if future shows that I shall need it.
Let in the clean air - for heavens sake - and for my sake.
I am 28 years old. I have muscular dystrophy of a type called Kongenit Myopati. When I was three months old I got difficulties with my breathing so I had to stay at the hospital for the first three years of my life. There I got a tracheotomy, and during these three years I learnt to breathe without any mechanical aid.
I then came home to my parents' farm where I felt like a stranger in new surroundings, but pretty soon I got happy about my sister, brother and the animals, too.
At the age of nine I got the diagnosis MD at the hospital in Århus. The doctors didn't give me much hope for my situation. But after ,,the down period" on the farm, life was normal again. My parents decided to treat me just like they treated my sister an brother, and soon I was given responsibilities at the farm just like them. My breathing was fine except when the wind blew direct into my face.
20 years old I moved to Århus to get an education as a computer programmer, and hence I worked six years as a system planner. Three years ago Maria became my girlfriend, and we are going to be parents very soon.
And now the story about my respirator, which is one and a half years old.
I joined the ,,Green Concert" tour of Muskelsvindfonden for a period of fourteen days, which was very hard from a physical point of view. Much too little sleep, too much work and too much alcohol to drink. Two days later Maria, I myself and three helpers started our three weeks' holiday trip to Greece. The last day in Greece I collapsed in Athens.
It was very hot, and we were extremely stressed because of all the things we had to manage before travelling back home. Suddenly I didn't feel well. I couldn't feel my feet and arms, and the sounds around me became unclear. My pulse was high and I couldn't breathe. Next, I found myself in a taxi on my way to a hospital. I thought I'd had a stroke, but the doctors told me it was stress and wanted to give me heart medicine. I didn't dare to take the medicine because of muscular dystrophy.
Next day we left Greece and back in Denmark I went to see my own doctor. He told me, that I suffered from a severe case of hypoventilation, but as I still felt extremely bad even after fourteen days I insisted on going to hospital to be properly tested.
After having been told that I was a hypochondrial and nervous I felt relieved when they found out that I had too much C02 in my blood. I ended at the Respiration-centre in Århus. Many tests were made during several nights, and the result to life of these tests couldn't be misunderstood. ,,Mechanical aid for breathing needed".
I was certainly totally depressed, but at the same time I felt relieved because they found an explanation of my illness.
Bent Juhl and I had some good conversations, and we discussed tracheotomy and BiPAP.
Sleeping with a machine that is fitted with tubes, which irritates when you want to change position during nights, and which is just as noisy as a vacuum cleaner, and a humidifier blowing water in your head when you have finally fallen asleep - it was certainly not an easy thing which to live. The mask scratches your nose - and answer me this question: How do you hold your girlfriend tight, when you look like an elephant ?
During the first weeks with my new friend "BiPAP" I almost lost the will to live. I couldn't feel any progress whatsoever except for the pain in my nose. My friends, among which two are BiPAP users gave me confidence by giving me some advice. Compared to my contact lenses the BiPAP suddenly became more important for me. Now I have more energy and I almost feel that my new situation gives me some new values. Further I have become more grateful to life.
Today I know that if tracheotomy is necessary again then it just has to be done because now I know that I can manage my own situation. What I can see today is that really, I needed my BiPAP years ago. Before the BiPAP - I was even more tired, when I woke up, than I was when I went to bed.
In 1991 I visited Bent Juhl - an ordinary check. At that time I had no problems, but I was told that I would be needing a respirator in a year or two. I went back home contemplating this. I was quite aware that it was my prospects with my diagnosis . But two years were for me some distant future.
Half a year later I returned for control. There was still no reason to worry. But I phoned one of my friends telling him about the respirator and decided that I would accept it when it was needed. I continued my full-time job at Muskelsvindfonden while I planned my summer. First I was going on Green Concert and then to Greece in August. After the Green Concert tour I was very tired. I couldn't get up in the morning, I had re-occurring colds and felt I had problems with my lungs.
Instead of going to Greece I was hospitalized. At the hospital Bent Juhl told me that I should have a respirator now. I started crying. I had thought that I had prepared myself for this situation, but now I couldn't use it for anything. Again and again I had been thinking that it was way out in future.
I was at the hospital for one month. The respirator wasn't the worst - but all that I had to learn. In the beginning it took three hours to get ready. I thought I would never be able to go to work again. When I was home again I needed desperately a hope - something to look forward to. Since my years at college I have wanted to go to Australia. I decided that now it was time for fulfilling my dream. I would go to Australia before I died. The incident with the respirator I took as an omen that life is not everlasting. In the autumn of '93 I left and toured Australia for two and a half month. It was a fantastic tour.
By the way, I started also to make a youth theatre and worked as a volunteer at Muskelsvindfonden. Suddenly I was very active and no longer tired and without energy.
To-day I use approximately 20 minutes extra because of my respirator. With my helper, my technical aids etc. I can live like anybody else. The respirator has enabled me to have a good life. In retrospect, I must admit that I had hardly sufficient energy to go to work the last two years before I got my respirator. I nearly spent all my time sleeping and working. I died a little every day.
Questions and discussion
Frank Schut: I can recognise that story from myself. It is so easy to say yes to a respirator the first time you hear about it but when the time comes it is extremely difficult.
Dick Cochius: It is so very difficult to confront reality. There is a new day to-morrow then I can do it. You keep postponing your decision.
Klaus Bach: It is very important that you yourself can decide when it has to be.
Dick Cochius: Yes, but suddenly to be informed that now it must be. I was told so on a Monday, the next day I was hospitalized, and on the following Wednesday I was operated.
Maria Overgaard Hansen: Klaus is my hero now. He is always so full of life and energy. Two years ago we talked about how quiet and boring he was, we didn't count on him any longer. Now I and others have realised that it was a symptom of his respiration problems.
I have always been handicapped and have never been able to ambulate. My diagnosis is spinal type 2.
In 1979 I was very ill because of a severe pneumonia. I had been at the hospital for some time and a doctor then suggested to try this C-pap system at me. It hadn't been used for many years. It helped, and I have managed with the C-pap morning and evening to have my lungs thoroughly ventilated. Except when I am suffering from pneumonia - then it has been necessary to ventilate my lungs more often.
As I have heard others say, too, I have always been good of advising others. But it was of no concern for me myself. Therefore, when I was introduced to my B-pap I was certainly not happy, on the contrary. It was difficult for me to imagine this machine in exactly my life. Difficult to see how it could be transported round with me. Initially I was told that I wouldn't have to use it each night - but right after the first night it was a miracle. My life changed radically. Previously I had to be turned up to six times each night - now only approximately twice. As you will all know it gives you a much better sleep and thus a far better everyday life. If you don't have enough air you will loose your muscle power - because the muscles need oxygen.
In Muskelsvindfonden we have decided that we must endeavor to have the respiration aids improved. They must so to say have a nicer look, and they must be noiseless and functional. We must develop a prettier design.
To-day it is of course wonderful that we - anyway in Denmark - can have the respirator treatment we need. But time has come to have the design of the respiration aids improved.
I won't accept that my B-pap is so noisy.
A quick survey of the situation within the respiration area in the individual EAMDA-member associations revealed that we are not out of the wood until we can say that respiration treatment will be offered to all of the members of EAMDA.
In Ireland, for instance, there is no tradition whatsoever for treating muscular dystrophy patients with respirator.
In Germany the attitude to respirator treatment is slowly changing but the individual must continuously fight to avoid being sent to an institution.
To-day it is possible to get respirators in The Netherlands, but as yet there is no real choice, because the necessary help doesn't follow the respirator. This means that you have to live at a special institution for respirator users.
In general there are also problems with the attitude to respirator treatment. Briefly said: Why give a respirator to individuals with muscular dystrophy - isn't it better "to let them have peace instead".
Evald summed up the "trip" in three main themes, with which the individual countries must work if conditions are to change:
He also stressed that by experience he knows that it is a long process.
"In Denmark they also had I don't know how many excuses for not giving us a respirator. We gathered all the doctors at seminars with a few years interlude - and they changed attitude. Bent Juhl, whom you have met here yesterday, was previously opposing to respirators for us „muscle swindlers", but to-day he is boasting of all the people, he has helped" Evald Krog said.
The last debate at the seminar focused on the role of EAMDA in relation to the problems of respirator treatment. The President of EAMDA, Evald Krog stressed in this connection that preferably EAMDA should develop into an information centre, but that it would still take some time before such decision could be accomplished.
"I think it is now about time to ask EAMDA to strongly animate the steering group being responsible for the respiration treatment to take action. I am aware that we haven't much money in EAMDA, but it must, nevertheless, be possible to have this work started", Evald Krog said.
Dick Cochius: Information is the foundation. It needn't be so very expensive to have information disseminated. It may for instance be done in connection with workshops. As a way of doing it I think it is a good idea to arrange meetings and seminars with participation of both users and general practitioners. In The Netherlands we have often meetings about respirator treatment. But it is seldom that people, who use respirators, participate. We should not only participate in the discussions but also make the decisions ourselves.
Maria Overgaard Hansen: In Muskelsvindfonden we have many courses for young people. It is a good way to have information communicated and to get some ideas of what it is all about and what is possible.
Christian Fischer: We must begin communicating the information that we have got at this seminar to our relevant organisations and to our friends.
Laura Verwey: It has been so positive to see the respirator users living such an active life. Many doctors are in need of much more information. They think that the respirator is only a question about a little oxygen. Furthermore, The Netherlands need a helper system if it shall be possible to live a good life with a respirator.
Gregor Schwarz: We must show some examples to demonstrate what sort of life - and a good life - it is possible to live with a respirator. Perhaps there should be some sort of network to which you could phone when you wanted to know more about respirators. Respirator treatment saves lives, it must therefore be given top priority by EAMDA.
And we must insist that the question of respirator treatment is also given top priority in our countries.
Conclusion
The national muscular dystrophy associations must put respiratory support at the top of their priority list. When the talk is about respiratory support we are thinking of a subject which can greatly influence the life of a person with NMD. It is of the utmost importance that it is commonly accepted that we all have the right to live even if we are suffering from a progressive muscular disorder.
Furthermore the knowledge of and information on respiratory support is certainly insufficient. The correct medical information is not available, and the psycho-social aspects of this treatment are not fully acknowledged.
What remains to be done
In the short run EAMDA must come up with a concise and practical programme dealing with respiratory support, a programme which enables its associations to carry on the work planned. In order to achieve results within a limited period of time the participants of the EYO seminar suggest as follows:
- That an information package dealing with the following subjects should be produced:
On the medical side:
On the psycho-social side:
Amongst other things EYO will take care of the interests of the young people by making sure that also the youngsters and not only the professionals can understand the information provided. Further, when the young have read it they can see how it applies to them and how they can make use of it in practice. We could carry on like this, in vain. The participants of the EYO seminar were of the opinion that everyone is entitled to the correct information and at the end to be able "to breathe with relief"
The first comprehensive survey of the living conditions for Danish respirator users establishes that respirator treatment of people with muscular dystrophy is a great success.
by Joergen Jeppesen
Muscular dystrophy sufferers that use respirators are feeling well. Surprisingly well.
Muskelsvindfonden has known this for a long time, but has been unable to prove it. Thanks to the first comprehensive survey of the living conditions for Danish respirator users it is now possible to do so.
The survey has been carried out under the guidance of former Chief Physician at Rigshospitalet, Henning Sund Kristensen, who is one of the most outstanding experts within the field of respirator treatment in this country. He was one of the physicians that introduced respirators to the polio patients in the 1950s. The nurses Grethe Nyholm, Rigshospitalet, and Tove Agertoft Nielsen, Århus Kommunehospital have also contributed to the survey.
"It is very surprising to see that respirator users suffering from Duchenne's and Becker are feeling so well as they are. It surprises me, how well they are actually functioning", Henning Sund Kristensen says to Muskelkraft. He immediately adds that the Danish helper system is a factor which contributes to the success of the respirator treatment. If it didn't exist the users would be far worse off, he thinks.
"In this country we are extremely privileged thanks to the helper system," he says.
Based on the results of the survey it can be established that everyone or at least a great number of patients with muscular dystrophy, who are potential respirator users, are to-day offered a respirator - and that most of them accept the offer.
It is a development which can only be described as a revolution for the persons in question! They are alive - and according to as well themselves as to others they are living a good life.
Five years back there were three respirator users having muscular dystrophy of the Duchenne's or Becker type. The survey was finished in the second half of 1993 and at that time the number was 28. From the report, quote: „Until the late 1980s the opinion was that respirator treatment would not help people with Duchenne's or a similar type of muscular dystrophy."
In front within the field of muscular dystrophy
The survey is a result of a co-operation between Muskelsvindfonden, Århus Kommunehospital and Rigshospitalet, and Muskelsvindfonden has financed it. All Danish respirator users, and not only the muscular dystrophy sufferers, have been surveyed, and thus it has become the first comprehensive analysis of permanent respirator users in this country.
In Denmark there are approx. 120 persons that use respirators during part of or the entire 24 hours of the day and night. 110 of these have been visited and questioned about their disability, level of activity, family conditions, way of living, and about helpers. They were also asked to characterise their life quality.
The report concludes, quote: "The main impression is very positive. In spite of their severe disabilities the users' activities are only slightly reduced. To a large extent the young respirator users start independent households thanks to the Danish Social Security Act that provides for publicly paid helpers."
Also from an international point of view the survey is apparently rather unique. Henning Sund Kristensen informs that similar surveys are hardly known in countries, which use respirator treatment extensively, for example France and the US.
For this reason it is difficult to assess whether Denmark lies in front of others, but specifically as regards the group of muscular dystrophy sufferers Henning Sund Kristensen dare say that Denmark is leading.
"In Sweden, for example, hardly any muscular dystrophy patients have got a respirator," he informs.
The niceties of bureaucracy
One of the objectives of the survey was to find out whether the treatment was inadequate or incomplete. The report mentions a few unimportant deficiencies as regards the treatment itself. In this connection it is also said that the two Danish respirator centres have not enough resources, that there are various problems as regards the training of the helpers, their employment, their wages. Furthermore there are inappropriate administrative procedures when respirator users go to hospital, etc..
"It can best be characterised as niceties of bureaucracy causing difficulties in the daily working life. Friction between the various public bodies, which causes annoying delays and makes it all more expensive. But no fundamental problems," Henning Sund Kristensen says..
He hopes that the relevant bodies will take necessary steps to correct the problems mentioned. In this connection Henning Sund Kristensen regrets very much that not a single person from the sectors, which have to „solve" the problems, participated in the survey.
"Our survey would have been much more efficacious if representatives from the administration and the directorate of social services had participated. They would then had been much more committed," Henning Sund Kristensen thinks.
He expects that the survey will arouse professional discussions amongst doctors and therapists especially regarding one question which is of no specific interest for the muscular dystrophy patients. Only two persons in Denmark suffering from the disease Kyfoscoliosis are using respirators, whereas you will find 55 respirator users with this disease in Sweden, for example. Perhaps Denmark lies far behind other countries in this respect ?